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ENT audiology news www.entandaudiologynews.com IAPO Exclusive Content 18-20 November 2011 Sao Paulo, Brazil Included inside: articles from the recent paediatric issue (July/August 2011) Plus added bonus paediatric articles published since 2009 welcome to your IAPO 2011 Exclusive ENT & audiology news content featuring articles from the recent paediatric issue (July/August 2011) PLUS added bonus paediatric articles published since 2009 Paediatric articles published in the JULY/AUGUST 2011 issue ENT FOCUS 5 The Scope of Paediatric ENT 19 Iain Bruce 8 Paediatric ENT: why choose this subspecialty? Congenital Midline Nasal Masses Compared Mary-Louise Montague 22 Sujata De Virus Infection of the Paediatric Airway and Chronic Disease Effects Adam J Donne and Michael P Rothera 10 Combining Adult and Paediatric Practice – is it still feasible? 25 Konstance Tzifa and Kate Hanvey Fiona B MacGregor 12 Failed Extubation in Children: assessment and management options The Rehabilitation of the Deaf Child 27 Serious Complications of Acute Otitis Media: mastoiditis and intracranial sepsis William PL Hellier Joe Grainger and Michael Kuo 31 15 17 Paediatric Nasal Emergencies Reassembling the Auditory World in Children with Cochlear Implants Tom Beech and Ann-Louise McDermott Blake C Papsin, Sharon L Cushing and Karen A Gordon Neck Abscesses in Children 34 The Future of Paediatric ENT Surgery Gavin Morrison Louise Melia and Haytham Kubba AUDIOLOGY MATTERS 38 Paediatric Audiology 45 Josephine Marriage 39 41 Post Newborn Hearing Screening ABR: quality assurance and the role of peer review Priya Singh Guy Lightfoot, Graham Sutton and Sally Wood Amplification Options for Mild Bilateral Hearing Loss & Unilateral Hearing Loss in Children: a literature overview Helping Families Accept Technology Cherilee Rutherford Jane R Madell 43 Unilateral Hearing Loss in Early Childhood: what are we doing about it? 48 Auditory Verbal Therapy Provision in the UK 52 Paediatric Tool Development: developing a rationale for empowering children with hearing loss Elizabeth Tyszkiewicz Melanie Gregory BONUS ARCHIVE: Paediatric articles published since 2009 ENT FOCUS AUDIOLOGY MATTERS 55 69 European Society of Paediatric Otolaryngology John Graham May/June 2009 57 Doris-Eva Bamiou and Tony Sirimanna Jan/Feb 2009 Paediatric Audiology at Alder Hey Children's NHS Foundation Trust 72 Ezeddin El Tabal Sept/Oct 2009 58 Imaging the Paediatric Airway 75 The Challenges Ahead in Paediatric Audiology Jane Madell Mar/Apr 2009 Bone Anchored Hearing Aids in Children 78 Patrick Sheehan Mar/Apr 2010 65 Managing Children with Auditory Processing Disorders in the Educational Environment Pauline Grant Jan/Feb 2009 Laura Coleman, Helen Williams, Kate Parkes and Michael Kuo Jan/Feb 2010 62 A Clinical Service For Children and Adults With Suspected APD New Developments in Hearing Aids for Children and Adults Josephine Marriage May/June 2009 Epidemiology of Paediatric Sleep Disordered Breathing 80 Michelle Wyatt Jul/Aug 2010 Cochlear Implantation in Early Childhood: what is happening in the long-term? Sue Archbold Jul/Aug 2009 83 New Frontiers: auditory brainstem results in adults and children Martin O’Driscoll Jul/Aug 2009 87 An Approach to the Dysmorphic Child with Deafness Melissa Lees Sept/Oct 2010 PLUS Copyright: No part of this journal may be reproduced without written permission from the Publishers. The comments of the reviewers are their own and are not necessarily endorsed by the Publisher, Editor or Editorial Board. Accordingly, the Publisher, Editor or Editorial Board accept no liability for the consequences of any such inaccurate or misleading data, opinion or statement. The Editor’s decision is final and no correspondence will be entered into. The utmost care has been taken to ensure accuracy of detail, but the publishers cannot accept responsibility for errors or omissions. Note: The work of the Editorial Board is wholly honorary and without any direct or indirect commercial or financial interest. pinpoint scotland ltd Rosaleen Shine Tel: +353 (0)91 635 890 (Ireland) Email: rosaleen@ pinpoint-scotland.com 90 Special Focus on Distribution 93 International Circulation SALES EXECUTIVE Helen Stuart Email: helen@ pinpoint-scotland.com EDITORIAL AND JOURNAL REVIEW CO-ORDINATOR Joe Crossland Email: editorial@ pinpoint-scotland.com OR CONTACT US ON: Tel: +44 (0)131 478 8401 (ENT & audiology news direct line) Tel: +44 (0)131 557 4184 (switchboard) Fax: +44 (0)131 557 4701 features Paediatric ENT The Scope of Paediatric ENT Paediatric ENT: why choose this subspecialty? Combining Adult and Paediatric Practice – is it still feasible? Failed Extubation in Children: assessment and management options Paediatric Nasal Emergencies Neck Abscesses in Children Congenital Midline Nasal Masses Compared Virus Infection of the Paediatric Airway and Chronic Disease Effects The Rehabilitation of the Deaf Child Serious Complications of Acute Otitis Media: mastoiditis and intracranial sepsis Reassembling the Auditory World in Children with Cochlear Implants The Future of Paediatric ENT Surgery The Scope of Paediatric ENT Mr Iain Bruce, MD, FRCS(ORL-HNS), Consultant Paediatric Otolaryngologist. Correspondence Royal Manchester Children’s Hospital, Manchester, UK. E: [email protected] Declaration of Competing Interests None declared. he range of services provided by paediatric ENT surgeons continues to evolve with the requirement to manage isolated pathologies and the ENT manifestations of other disease processes (for example nasal polyposis in cystic fibrosis) and syndromes (for example OSA in Mucopolysaccharidosis). The majority of secondary level ENT problems in childhood continue to be managed successfully in District General Hospitals, under the guidance of lead clinicians for paediatric ENT. This article will focus on tertiary level services provided by dedicated paediatric ENT surgeons, often but necessarily exclusively, working in children’s hospitals or large teaching hospitals. An effective working relationship between tertiary and secondary level paediatric ENT services is obviously important if the highest possible standard of care is to be provided. Paediatric ENT as a subspeciality is unique as it necessitates the management of complex otology, rhinology and head and neck problems, with a consultant often being skilled in each of these areas (Table 1). Therefore, there is often an overlap with the skill set of adult subspecialists, with this providing a potential source of strength as it facilitates the recruitment of the most appropriate local team to ensure effective management of certain pathologies. The amount of input from adult subspecialists will vary between hospitals, but an effective relationship is important. This interaction is most noticeable when presented with a child with a head and neck malignancy. Although the child may initially be reviewed by a paediatric ENT surgeon or paediatric oncologist, thyroid or salivary gland malignancy will often be best managed by a cancer surgeon. Children will also be referred with secondary level ENT problems complicated by significant comorbidity. T The management of childhood airway obstruction is a significant component of a paediatric ENT surgeon’s workload. The ability to perform a diagnostic rigid airway endoscopy (MLB, DLTB) is a basic requirement of a paediatric ENT surgeon. A wide variety of airway pathologies may be encountered as illustrated in Figure 1. Management may include endoscopic surgical treatment (for example subglottic cysts), a surgical airway (tracheostomy) or an open airway augmentation procedure (for example laryngotracheal reconstruction with a costal cartilage graft). Access to the airway may be further complicated by abnormalities such as micrognathia in Treacher Collins syndrome. The management of an airway emergency requires advanced airway skills and close cooperation with the paediatric anaesthetist. The management of childhood chronic otitis media, with or without cholesteastoma, is particularly challenging. Effective management of this clinical problem involves disease control or eradication, but an increasing emphasis is also being placed on the rehabilitation of hearing loss once the disease has been treated, to limit the effect on educational performance. Collaboration with paediatric audiologists is important and bone anchored hearing aids (BAHA) and middle ear implants (MEI) may also be indicated. Particular childhood pathologies present specific otological problems, such as hearing loss in children with cleft palate, craniosynostoses, microtia and canal atresia. Although the majority of cochlear implants in children are performed by subspecialist otologists, paediatric ENT surgeons now perform cochlear implantation in several centres in the UK. An established paediatric ENT / anaesthesia team may be more appropriate when the child also has complex comorbidity, particularly involving the airway. Paediatric ENT surgeons will manage congenital head and neck anomalies An effective working relationship between tertiary and secondary level paediatric ENT services is obviously important if the highest possible standard of care is to be provided ENT & audiology news | www.entandaudiologynews.com 5 feature Figure 1: Laryngeal pathology in children. Subglottic haemangioma Epiglottitis Laryngeal cleft MPS deposits Post-intubation changes Foreign body Supraglottic cyst RRP Laryngomalacia Subglottic stenosis Subglottic cyst Collaborative working with paediatricians, neurologists, intensivists, anaesthetists, audiologists, respiratory physicians and cardiologists is an essential and rewarding aspect of working as a paediatric ENT surgeon 6 ENT & audiology news | www.entandaudiologynews.com feature Table 1. Summary of childhood tertiary level ENT problems. including lymphovascular malformations (for example cystic hygroma), branchial abnormalities (for example branchial sinuses), thyroglossal cysts and teratomas. Treatment will often comprise surgical resection but may also include airway management in large cystic hygromas and teratomas. Various congenital abnormalities may present with nasal obstruction or a mass in early childhood. Neonates are obligate nasal breathers and airway obstruction in this period may present as an airway emergency. Pathologies may include choanal atresia, nasal dermoids, gliomas, teratomas and encephaloceles. The surgical approach will be dictated by the nature, site and extent of the disease and the options include endoscopic transnasal, transpalatal, and combined transnasal and transcranal in conjunction with a neurosugeon. A paediatric ENT surgeon must be able to manage the complications of childhood head and neck sepsis. This will include deep neck space infections, orbital complications of rhinosinusitis and the intracranial and intratemporal complications of acute and chronic otitis media. A paediatric ENT surgeon must have demonstrable skills in this area that constitutes, along with airway obstruction, a significant component of on-call commitment. As paediatric ENT departments expand the services provided to children are becoming increasingly comprehensive. To meet these varying clinical demands paediatric ENT surgeons are often required to have undertaken a paediatric ENT fellowship during their training. Collaborative working with paediatricians, neurologists, intensivists, anaesthetists, audiologists, respiratory physicians and cardiologists is an essential and rewarding aspect of working as a paediatric ENT surgeon. n Anatomical sites Pathologies Neck Deep neck space infections Congenital abnormalities Thyroglossal cysts Branchial abnormalities Lymphovascular abnormalities Teratoma Malignancies Rhabdomyosarcomas Thyroid Salivary gland Airway Oropharygeal OSA Micrognathia Adenotonsillar hypertrophy (for example MPS, Down syndrome) Macroglossia (Beckwith Weidermann syndrome) Lingual thyroid Supraglottic Laryngomalacia Vallecular cysts Cystic hygroma (microcystic) RRP Glottic Vocal cord palsy Laryngeal webs RRP Post-intubation injury Laryngeal clefts Subglottic Subglottic stenosis (congenital or acquired) Haemangioma Subglottic cysts Trachea and bronchus Tracheobronchomalacia Foreign bodies Ears OME in children with comorbidity Chronic otitis media Complications of AOM and COM Microtia and atresia Surgical rehabilitation of moderate to profound hearing loss Bone anchored hearing aids Middle ear implants Cochlear implants Nose Congenital nasal obstruction and masses Choanal atresia Nasal dermoid Glioma Teratoma Encephalocele Craniosynostosis Have you visited our website? • Over 1,300 diary listings online • Society links • Over 500 journal reviews archived • Resources for ENTs and Audiologists www.entandaudiologynews.com • Bookmark us now! ENT & audiology news | www.entandaudiologynews.com 7 Paediatric ENT: why choose this subspecialty? Miss Sujata De, FRCS(ORL-HNS), Consultant Paediatric ENT Surgeon. Correspondence Alder Hey Children’s Hospital, Eaton Road, Liverpool, L12 2AP, UK. E: Su.de@ alderhey.nhs.uk Declaration of Competing Interests None declared. he vast majority of ENT surgeons have some element of paediatric practice – indeed our ‘bread and butter’ has historically comprised grommet insertion and adenotonsillectomy. The old adage ‘children are not little adults’ remains true and Paediatric ENT as a distinct subspecialty was born of a necessity to recognise this in ENT. Essentially paediatric ENT is the practice of ENT surgery in children. This encompasses the common procedures as mentioned above but also the management of conditions that are unique to infants and children such as congenital anomalies and airway disorders that are sequelae of prolonged intubation and / or prematurity. In any case children are not little adults and so it is only fair that their ENT needs are addressed accordingly. Paediatric ENT is a relatively young subspecialty in its own right. It is only in the last 20 years or so that it has had its own subspecialty association – BAPO in the UK, ESPO in Europe, and ASPO in the United States. As a result the paediatric ENT community is a relatively small one with the resulting ease of exchange of ideas, and rapid uptake of innovation. An example of this is the speed at which the treatment of subglottic haemangioma has been revolutionised by the use of propranolol. Paediatric ENT has been incorporated into the assessment and examination process of most ENT training programmes. Specialist experience can be T 8 gained by seeking fellowships both in the UK and abroad. There are two types of paediatric ENT surgeon; one who works in general ENT practice but who perhaps takes on the role of paediatric lead in a District General Hospital (DGH). A large proportion of children who require an ENT opinion or management can be seen and managed in a DGH. This paediatric ENT surgeon in this case would be responsible for ensuring that children are treated according to the highest standards possible and to create referral pathways with the local tertiary unit for conditions that cannot be dealt with at a DGH either because of lack of suitable anaesthetic cover or lack of nursing / surgical expertise or ICU backup. The other type of paediatric ENT surgeon works in a tertiary paediatric hospital and in addition to dealing with common childhood ENT disorders in both ‘normal children’ and children with other challenging conditions, also manages conditions specific to children. The tertiary ENT consultant is a ‘generalist’ in that he / she will have to manage all ENT conditions in children. These are fairly evenly spread between otology, rhinology, laryngology and head and neck surgery. One can therefore put to use all the skills accumulated during years of ENT training. There are specialist areas such as paediatric airway, congenital anomalies, cochlear implantation that require specific skills and ENT & audiology news | www.entandaudiologynews.com feature expertise. In addition, a lot of collaboration is required particularly in managing children with challenging conditions such as Down’s syndrome, craniofacial conditions and neurological conditions. One has the great privilege of working closely with paediatricians, neonatologists, geneticists and intensivists. Of course the main advantage of being a paediatric ENT surgeon is the opportunity to work with children and to make a real difference to the quality of life of individuals who have the whole of their lives ahead of them. I still remember the surgeon who took out my tonsils. Trivial operation maybe, but he’s the person who inspired me to be a surgeon! One of the challenges of paediatric practice is the lack of a strong evidence base for much of our practice. This has led some to question whether what we do is necessary at all!1 Clinical/ pharmaceutical research in children is obviously much more difficult than in adults. We sometimes have to rely upon evidence from adult research to guide our management principles. Even good research conducted in children has to be interpreted carefully as conclusions cannot be generalised across the board. For example OME poses a very different management challenge in a two year old when compared to a 13 year old with Down’s syndrome! Of course life is not without its challenges and working with infants and children inherently means having to deal with their parents and carers. Infants and younger children cannot communicate and are therefore completely reliant on their parents or carers to express their needs and concerns. Taking a history therefore is a challenge as the parent or carer may bias the explanation of symptoms towards a course of action that they prefer. At some point in time (and this varies from child to child), the child becomes quite capable of expressing him / herself; and then the consultation can be like a game of ping pong with the child claiming one thing while the parents claim another. It is the clinician’s job to decipher the real story and to make an appropriate decision that is in the child’s best interest. Most of the time managing parents and their expectations is a positive part of the experience but there will be occasions when there is disagreement between you and the parents as to what is in the child’s best interests. Most conflict can be resolved with good communication, careful explanation and if necessary mediation by another member of the team. It is always unfortunate when management plans do not result in a favourable outcome. It sometimes becomes necessary particularly in children with complex morbidity to take a step back and decide whether less intervention is a kinder course of action. The loss of a young life is particularly harrowing. Thankfully, in paediatric ENT mortality is rare but it does occur and can be heartbreaking. On the whole paediatric ENT is a very rewarding subspecialty, one that makes a difference to its patients and their families, and one that challenges one’s clinical skills, both outside and inside the operating theatre. n References 1. Spence D. Bad medicine: paediatric ear, nose and throat surgery. BMJ 2010;341:c6560. British Association for Paediatric Otorhinolaryngology [www.bapo.org.uk] promote your event in courses & conferences Ensure your event features in ENT & audiology news, REACHING APPROXIMATELY 16,500 POTENTIAL DELEGATES across the world per issue RAISE AWARENESS by announcing your event details to our readers well in advance! ENHANCE your PROFILE by submitting an eye-catching advert to us. . . . . . or USE OUR DESIGNERS to create a striking advert for you. For further details contact: Helen Stuart T. +44 (0)131 478 8401 (direct) or +44 (0)131 557 4184 E. [email protected] www.entandaudiologynews.com European Society of Pediatric Otorhinolaryngology [www.espo.eu.com] American Society of Pediatric Otolaryngology [www.aspo.us] ENT & audiology news | www.entandaudiologynews.com 9 Combining Adult and Paediatric Practice – is it still feasible? Fiona B MacGregor, MBChB, FRCS, FRCS(ORL), Consultant Otolaryngologist and Honorary Senior Lecturer. Correspondence Gartnavel General Hospital, Great Western Road, Glasgow, G12 0YN, UK. E: Fiona.MacGregor@ ggc.scot.nhs.uk O ne of the factors that originally drew me to ENT surgery over 20 years ago was the opportunity to work with both adults and children. Indeed, most consultants would see adults and children in the same clinic and combined operating lists were commonplace. Practice has changed since then and children are usually seen in dedicated paediatric clinics by ENT surgeons with a paediatric subspecialty interest. Operations are carried out on paediatric lists with the involvement of paediatric anaesthetists, nurses and other healthcare professionals. Almost all would agree that this is a significant improvement in the delivery of ENT care for the paediatric population. Declaration of Competing Interests None declared. There has been much debate about paediatric ear disease and who should manage it Royal Hospital for Sick Children, Glasgow. 10 Specialising solely in paediatric otolaryngology was unheard of until around 20 years ago. Now, some children’s hospitals in the UK are staffed by a number of ENT surgeons whose sole practice is paediatric. However, there remain a significant number of surgeons who work between the adult and children’s sectors both in teaching and district general hospitals. Generally speaking, specialist paediatric ENT surgeons will turn their hands to all things paediatric – choanal atresia, subglottic stenosis, thyroglossal cysts and even cholesteatomas. In the adult sector, ENT surgeons are often specialised in terms of otology, rhinology, or head and neck surgery. Partly as a consequence of this many ENT surgeons have become reluctant to cover children in the on call setting – often citing a concern about managing the challenge of the difficult airway in a child. This concern has resulted in debate, disagreement and even downright disorder in some ENT departments in the UK! Some would argue that having some experience in adult ENT surgery can enhance a surgeon’s paediatric practice and vice versa. Others believe that it is in the interest of both children and clinicians if the two are entirely separate. At The Royal Hospital for Sick Children, Glasgow, I work with four full time paediatric ENT colleagues in addition to an associate specialist and a consultant audiological physician. My practice is split approximately 30% paediatric and 70% adult with a specialist interest in head and neck surgery, both benign and malignant, and voice disorders. Three of my colleagues share the complex paediatric airway work and hence I do little in the way of assessment and intervention in the paediatric airway. I cover both adults and children when I am on call and for me (and hopefully my patients) this works, but it has its challenges. In this article I hope to illustrate the advantages and disadvantages of a split workload through my own experiences. I will start with one of the most contentious issues… On call The common paediatric emergencies include post-tonsillectomy bleeds, tonsillitis, neck abscesses, a variety of foreign bodies in the nose and ear, traumatic injuries and less commonly periorbital cellulitis and mastoiditis. Inhaled foreign bodies are uncommon and are usually referred to specialist paediatric centres. A few of the larger paediatric ENT departments in the UK have separate adult and A child-friendly environment in a paediatric centre. ENT & audiology news | www.entandaudiologynews.com feature paediatric on call rotas. However, this is more difficult to organise in smaller centres where there may be only two or three ENT surgeons with specialist paediatric skills. Working a ‘1 in 2’ or ‘1 in 3’ is no longer accepted by most and a compromise is usually sought. In Glasgow we now have a hybrid rota with three consultants covering both adults and children, a further four colleagues covering children only and the remainder covering adults only. Does this work? Well, it’s not perfect. It’s difficult to make a swap when you cover both rotas and forward planning is essential. It is no doubt busier for those of us who cover both rotas and it is not possible to be in two places at once. Personally, I have to ensure that I maintain my core skills in paediatric emergencies (for airways please read on). Some major ENT centres remain committed to involve all consultants in both the paediatric and adult rotas. There is certainly the very reasonable argument that consultants should endeavour to maintain their core skills in all subspecialties including paediatrics. After all we still expect otologists to deal with epistaxis, rhinologists to manage neck trauma and head and neck surgeons to drain a mastoid abscess in the emergency situation. Fortunately most of us are in the position that we have helpful colleagues who can assist us when we encounter challenging patients. To maintain skills in paediatric airway intervention I would suggest attending the occasional specialised paediatric airway list and instructional paediatric airway course. It is necessary to understand how to perform a tracheostomy on a young child and to recognisie the differences from the adult procedure, in addition to being comfortable in assembling a paediatric bronchoscope. From a personal point of view I very much enjoy seeing routine paediatric ENT patients and it is a refreshing contrast to my adult head and neck workload in the West of Scotland! Head and neck surgery Fortunately, the majority of head and neck pathologies in children are benign – congenital cysts and sinuses, cervical lymphadenopathy and so on; interesting and rewarding to manage. Malignancies in the head and neck region in children are rare and I have found my experience in the adult sector to be invaluable in this situation. Regularly performing for example thyroidectomies and neck dissections in adults is extremely useful when faced with the rare thyroid carcinoma in a child. Having access to the adult head and neck managed clinical network has been invaluable when considering the treatment of rare head and neck tumours, perhaps more often seen in adults. For instance I managed a 12 year old boy with a laryngeal synovial cell sarcoma who ultimately required a total laryngectomy. Decision making was gratefully shared with the network and my adult head and neck team including the theatre staff and speech and language therapist were invaluable in the management and rehabilitation of this young man. Airway I work in the recognised national paediatric airway centre for Scotland and the vast majority of paediatric airway work is performed electively. Three of my colleagues take on most of this work and I am fortunate that they make themselves available to deal with the problems that sometimes arise. Otology General paediatric ENT surgery The vast majority of children seen in ENT clinics have problems related to tonsillitis, middle ear effusions, sleep disordered breathing and rhinitis. This is of course managed extremely well by the majority of general ENT surgeons in teaching and district general hospitals. Indeed, the limiting factor in continuing to see children outwith specialist centres is often down to the anaesthetists and their desire to maintain their paediatric anaesthetic skills. No doubt this has been frustrating for many capable surgeons and has resulted in an increased workload of routine ENT procedures in specialist centres. But that is a subject for another day… There has been much debate about paediatric ear disease and who should manage it. Traditionally, adult otologists have provided this service to paediatric hospitals but the workload has in general been taken up by a number of paediatric surgeons with a particular otological subspecialty interest. What is well recognised is that surgery should be performed by those familiar with the anatomical and pathological variations in young children – and that surgery should be something that he or she performs on a regular basis. In Scotland we have one national cochlear implant centre which is situated at Crosshouse Hospital in Ayrshire. There the surgeons operate on both adults and ENT & audiology news | www.entandaudiologynews.com children. However, a small number of the very young and children with complex problems are operated on by these same surgeons at Yorkhill because of the availability of paediatric ICU and specialist anaesthetists and paediatricians. This situation works well for us and we have a good relationship with our implantation colleagues. The insertion of bone anchored hearing aids and aural reconstructions have often been performed by otologists working in the adult sector but an increasing number of paediatric surgeons have developed a specialist interest and expertise in this field. Rhinology Apart from the snotty nose, adenoidal hypertrophy and allergic rhinitis much of the pathology found is unique to paediatrics – choanal atresia, nasal dermoids, encephaloceles and so on. Functional endoscopic surgery is rarely required (for example cystic fibrosis) and one could argue that this might be an instance where the adult rhinologist may have specific skills to offer. Logistics Working between the two sectors usually means working at more than one site. Not uncommon these days but there are logistical challenges associated with having two secretaries, two IT systems, two destinations for postal mail, and the supervision of two surgical teams. In addition, I find myself asked to commit to teaching, training and administration commitments in both sectors... Summary In practice most UK paediatric ENT centres are staffed by a combination of those who work solely with children assisted by a variable number of surgeons who have a combined adult / paediatric practice. The challenge of providing an adequate paediatric on call rota continues. Surgical expertise should be provided by those with specialist paediatric skills but that does not necessarily mean these skills cannot be provided by a surgeon who also works in the adult sector. In my opinion and from personal experience combining adult and paediatric practice is feasible. I find it enjoyable and rewarding despite the challenges. It does however depend on having a good working relationship with your colleagues and I am indebted to mine. I thank my paediatric colleagues throughout the UK who have provided me with information regarding their local paediatric services and their (differing!) opinions on the feasibility of combined practice. n 11 Failed Extubation in Children: assessment and management options Mr Joe Grainger, FRCS, Consultant Paediatric ENT Surgeon. D espite neonatologists’ and paediatric intensivists’ best efforts to reduce laryngeal injury during periods of intubation, approximately 5% of children spending time on the Paediatric Intensive Care Unit (PICU) will require an unanticipated reintubation within 48-72 hours of being extubated. One of the roles of the paediatric ENT surgeon is to assist in the assessment and management of the child who ‘fails’ extubation, usually following critical illness or major surgery. This article aims to provide an overview of the assessment of these children and look at some of the current management strategies. Mr Michael Kuo, FRCS, Children’s Ear, Nose & Throat Surgeon. Correspondence Birmingham Children’s Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK. E: [email protected] Declaration of Competing Interests None declared. Initial assessment Although the child who has failed extubation is likely to have been reintubated long before you as an ENT surgeon arrives on PICU, there is a lot of information to be gleaned from both the staff on the unit and the parents of the child. The history surrounding the intubation episode will give you a significant insight into the likely causes of the failure and whether or not an endoscopic examination of the larynx is likely to be helpful. What do you want to know? A reasonable approach would be to find out about three areas: the child’s history prior to intubation, the reason for the intubation and what happened around the time of the trial of extubation. Were there problems during the pregnancy or following delivery? If the child spent time on the neonatal unit, try and ascertain if they were intubated and if so how long for. Parents frequently get confused between intubation for ventilation and the passing of a nasogastric tube so try and be as clear as possible. Did the child have noisy breathing of any kind and was this ever investigated? If they are slightly older, have they had issues such as recurrent croup as this may suggest underlying airway disease? Was this admission and intubation related directly to an airway problem or was it for another reason? Frequently, children who fail extubation are not known to have a pre-existing airway problem – they may have been intubated for surgery or for a medical reason such as severe sepsis. These children may have an underlying mild airway problem that has been 12 exacerbated by intubation, or may have developed an airway issue solely as a result of the intubation episode. Upper airway factors such as micrognathia will also have a role and an assessment of the oropharnx should form part of the assessment process. What happened during the admission and around the time of extubation is crucial. A knowledge of the child’s ventilatory requirements and oxygen requirements will indicate whether there is an underlying chest problem. Ideally, prior to extubation, the child should require an FiO2 of less than 0.35 and minimal pressure support. High pressures and a large oxygen requirement may suggest a lower respiratory tract problem. The endotracheal tube size and whether there is an air leak will give an indication as to the size of the subglottis, although with the increasing use of low pressure cuffed paediatric tubes, this information is becoming less useful. Overall, prior to extubation the child should be as well as possible from a cardiac and respiratory point of view in addition to having as little sedation on board as possible. A good relationship between the ENT surgeon and PICU is vital. Whilst in the past it was commonplace for intensivists to attempt extubation multiple times before contacting their local ENT surgeon, it is now becoming more common to involve the ENT surgeon at a much earlier stage. A further trial of extubation may be appropriate if there are factors that can be corrected or improved. However, repeated extubation and reintubation cycles should be avoided as these are likely to result in further laryngeal damage. Twenty-four to ENT & audiology news | www.entandaudiologynews.com feature Table 1. Common causes of failure to extubate. Respiratory Cardiac Neurological Above the larynx choanal atresia Pierre-Robin sequence Laryngeal laryngomalacia bilateral vocal cord immobility granulation tissue/oedema subglottic cysts subglottic stenosis Below the larynx repeated extubation and reintubation cycles should be avoided tracheomalacia bronchomalacia Figure 1: Intubation related granulation tissue of the glottis – small areas will usually resolved spontaneously following extubation. Figure 2: Subglottic cysts. 48 hours of dexamethasone (100 micrograms / kg 8 hourly) along with anti-reflux medication (ranitidine 4mg / kg 8 hourly or omeprazole 750 micrograms / kg 12 hourly) may facilitate extubation by reducing oedema and is generally recommended. Airway endoscopy Assuming that the child's cardiorespiratory status was optimal for their trial of extubation, an airway endoscopy in theatre is likely to be required if the child needs reintubation. This allows the diagnosis to be established, appropriate treatment to be commenced and provides reassurance to those looking after the child. However, if there are factors that can be corrected, these should be addressed prior to considering endoscopy. It is always preferable to perform an assessment on an airway that has not been recently intubated. A formal endoscopic assessment of the airway is performed following removal of the endotracheal tube in the operating theatre. An assessment of vocal fold mobility should be made. The Storz ventilating bronchoscope provides a safe means of assessing the airway whilst at the same time facilitating ventilation if necessary. The summary of the potential causes of failed to extubation can be found in Table 1 although this list should not be regarded as exhaustive. Figure 4: Early soft subglottic stenosis. Figure 3: Image of the airway seen in Figure 2, following endoscopic deroofing of cysts. laryngeal rest. The child is intubated for a period of around two weeks without a further trial of extubation during this time. A smaller tube size is utilised and anti-reflux treatment is optimised. Histological studies have shown that mucosal ulceration may heal without stenosis and therefore, laryngeal rest may represent an alternative option to the cricoid split. However, a two week period of intubation on PICU is not without its problems and this option is therefore used infrequently. Removal of granulation and deroofing of cysts Figure 5: Balloon dilatation of the subglottis for a soft granular stenosis. Initial management Once the diagnosis has been established, treatment can be tailored accordingly. The therapeutic options include the following: A period of 'laryngeal rest' Providing there is no established firm subglottic stenosis, minor ulceration and oedema may settle with a period of ENT & audiology news | www.entandaudiologynews.com Intubation related granulation tissue and cysts are a common finding (Figures 1 and 2). Small areas of granulation can be removed endoscopically following the application of topical epinephrine. Subglottic mucous retention cysts can be carefully de-roofed aiming to leave an area of undamaged mucosa in between cysts to prevent circumferential fibrosis (Figure 3). Balloon dilatation Extensive granulation and soft stenosis around the endotracheal tube responds well to balloon dilatation (Figures 4 and 5). Radial dilatation crushes granulation and reduces damage to normal mucosa. Balloon dilatation may also be used where 13 feature Figure 6: Suggested algorithm for the management of children who have failed attempted extubation. there is an element of firmer stenosis after radial division of the fibrous tissue along with local infiltration of corticosteroid. Cricoid split The anterior cricoid split was first described by Cotton and Seid in 1980. It is best utilised in neonates that have failed extubation because of significant oedema and developing subglottis stenosis as an alternative to tracheostomy. The cricoid ring is divided anteriorly, allowing an age appropriate endotracheal tube to be passed and any glottic oedema to disperse. The larynx is then rested for five to seven days before a further trial of extubation. The original procedure was described without a graft, however, both thyroid and hyoid cartilage are readily available in the operative field and may be used as in a laryngeal reconstruction to hold open the cricoid ring. Up to 90% of children will be successfully extubated after an anterior cricoid split, thus avoiding the need for tracheostomy. The cricoid ring can also be split endoscopically using a sickle knife. This technique is best performed by those with significant endoscopic airway experience in order to prevent complications and ensure adequate division. Tracheostomy Whilst a tracheostomy may be regarded as a failure by some, for children who have 14 significant chest disease, severe laryngeal disease, bilateral vocal cord immobility or have failed extubation attempts multiple times, it provides a means of providing a secure airway that can be managed on a ward and avoid the associated morbidity of prolonged ventilation. A tracheostomy enables the larynx to settle and enables airway surgery to be performed electively. facilitate successful extubation. Therefore, unless there are correctable cardiorespiratory issues, diagnostic endoscopy should be utilised early in cases of extubation failure in order to prevent further mucosal damage. A suggested algorithm for the management of children who have failed extubation is shown is Figure 6. n Subsequent management Even following successful extubation, the subglottis will continue to evolve over the following weeks as areas of ulceration heal and any residual granulation tissue fibroses. Subsequent follow-up of these children is therefore required until their airway is stable in order to identify developing stenosis. Many will have sustained significant mucosal damage and may require more definitive surgery including laryngotracheal reconstruction. Summary ‘Failure of extubation’ may be due to many causes and it is important that cardiorespiratory function is optimised prior to further attempts. A detailed history relating to the child’s preintubation status, their reason for intubation and the events surrounding their extubation attempt are important to establish. The paediatric ENT surgeon has, at their disposal a number of endoscopic and open techniques to ENT & audiology news | www.entandaudiologynews.com FURTHER READING Albert D. Post intubation laryngotracheal stenosis. In Pediatric ENT. Edited by Graham JM, Scadding GK, Bull PD). Berlin: Springer; 2007:223-7. Baisch SD, Wheeler WB, Kurachek SC, Cornfield DN. Extubation failure in pediatric intensive care: incidence and outcomes. Pediatr Crit Care Med 2005;6(3):312-8. Cotton RT, Seid AB. Management of the extubation problem in the premature child. Anterior cricoid split as an alternative to tracheostomy. Ann Otol Rhinol Laryngol 1980;89(6 Pt 1):508-11. Eze NN, Wyatt ME, Hartley BE. The role of the anterior cricoid split in facilitating extubation in infants. Int J Pediatr Otorhinolaryngol 2005;69(6):843-6. Lusk RP. Congenital anomalies of the larynx. In Ballenger’s Otorhinolaryngology. Edited by Snow JB, Wackym PA. Connecticut: BC Decker Inc; 2009:815-28. Paediatric Nasal Emergencies Miss Ann-Louise McDermott, BDS, FDSRCS, PhD, FRCS(ORL-HNS). N asal and sinus problems are very common in children however clinicians should be mindful that they do have a risk of serious morbidity and even mortality if not managed appropriately. The following article highlights the main paediatric emer- gencies and their management. 1. Choanal atresia Mr Tom Beech, MSc, FRCS(ORL-HNS). Correspondence Miss Ann-Louise McDermott, BDS, FDSRCS, PhD, FRCS(ORL-HNS), Department of Paediatric Otorhinolaryngology, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK. E: [email protected] Declaration of Competing Interests None declared. Although nasal and sinus problems are very common in children, clinicians should be mindful that they do have a risk of serious morbidity and even mortality if not managed appropriately Choanal atresia was first described by Roederer in 1775 and is now accepted as one of the more common congenital abnormalities of the nose.1 It is thought to affect 1:8,000 live births and females are more commonly affected. Choanal atresia may be bilateral or unilateral. The unilateral case may not become apparent until later childhood and may only cause difficulties during breast feeding when the unaffected side is occluded by contact with the breast. Association with other malformations is reported and CHARGE syndrome is the most widely reported. Paediatric assessment and screening for CHD 7 mutation is recommended for infants with bilateral choanal atresia. Bilateral choanal atresia typically presents as a respiratory emergency very soon after birth as neonates lack the ability to mouth breathe. Previous diagnostic tests have included bilateral failure of nasal misting on a metal spatula and failure to pass a soft suction tube through each nasal passage. The gold standard test however is computerised tomography (CT). This confirms the diagnosis, indicates whether the atresia is bony, membranous or mixed and furthermore the thickness and relation to other anatomical structures can be assessed. The airway needs securing in the first instance, usually by means of a endotracheal airway although modifications of a nipple from a feeding bottle have been described.2 Definitive surgery is recommended in the first week of life to create bilateral choanal patency. Orogastric tube feeding is necessary if surgery is delayed. Endoscopic intranasal choanotomy techniques are the most commonly performed and the removal of the posterior vomer is common practice. Surgery for choanal atresia has a significant risk of re-stenosis. It is reported to be higher in children with CHARGE syndrome. It is estimated that a mean of 1.5 to 3 operations are required per patient.2,3 The application of mitomycin C and the use of choanal stents are still popular with many surgeons in an attempt to reduce the re-stenosis risk. 2. Anterior pyriform aperture stenosis Congenital nasal pyriform aperture stenosis is an unusual cause of nasal emergency presenting as respiratory distress in neonates. The problem is caused by bony overgrowth of the nasal process of the maxilla. The clinical presentations mimic those of bilateral choanal atresia and a CT scan is required to confirm the diagnosis. A pyriform aperture width of 11mm or less associated with a single central incisor, in a term baby is diagnostic.4,5 Early management is to secure the neonatal airway. Bony overgrowth of the pyriform aperture is removed via a sublabial surgical approach. The use of nasal stents is common in the postoperative period. Not all neonates require surgery. Some may be managed conservatively with a simple nasal airway. 3. Sinusitis and its complications Rhinosinusitis is very common in children, and often more extensive than seen in adults. It also has the tendency to resolve spontaneously as the child grows and develops. Acute rhinosinusitis commonly follows an URTI and can have severe complications with spread of infection locally into the orbit with threat to vision and also intracranial spread with a mortality rate quoted as high as 2030%.3 A child presenting with periorbital cellulitis requires an urgent ENT and ophthalmological examination. The presence of proptosis, chemosis, restriction of eye movement, red colour desaturation and reduced visual acuity should alert the clinican to the urgency of the situation. Nasal decongestion and broad spectrum antibiotic therapy should be commenced immediately. It is difficult to clinically identify whether cellulitis is pre- or postseptal. An urgent CT scan with intravenous contrast of the paranasal sinuses, orbits and brain is the investigation of choice. ENT & audiology news | www.entandaudiologynews.com 15 feature Urgent drainage of the affected sinuses should be performed and orbital infection may be drained either by an endoscopic and / or external approach. For those children with intracranial complications, close cooperation with neurosurgical team is necessary. Early surgical drainage of the involved sinuses is necessary and should ideally be performed at the time of the neurosurgical drainage procedure. 4. Nasal foreign bodies This group of patients constitutes the largest component of paediatric nasal emergencies. Typically the history includes nasal obstruction, foetor and unilateral rhinorrhoea. The important factor is the nature of the foreign body. Ideally the foreign body should be removed in the outpatient setting at the time of presentation however some children require a general anaesthetic. Controversy exists regarding the true risk of aspiration of nasal foreign bodies if they are not removed immediately. Review of the literature reveals very little evidence to support this aspiration risk.6 Inert and nontraumatic nasal foreign bodies are managed on a semielective basis however foreign bodies such as the ‘button’ battery are urgent. The button battery rapidly causes tissue destruction when placed in a damp environment such as the nose, and results in extensive soft tissue destruction which in turn leads to nasal deformity. Examination of both nostrils is advised after foreign body removal to ensure no other foreign matter is present. 5. Epistaxis This a very common problem in childhood but it is often self limiting and of short duration. Epistaxis is most commonly seen in children between the age of three and eight years. Clinicians should have a high index of suspicion for any child that presents with excessive bleeding that fails to settle conservatively. Epistaxis may be the first sign of underlying systemic disease or tumours such as an angiofibroma. The management of paediatric epistaxis depends on the age of the child. Adequate resuscitation is the first line of treatment for epistaxis. Unlike adults however, it is rarely required in children. Digital pressure should be applied to control the bleeding. Topical application of cotton wool soaked in local anaesthetic to the nose is often not tolerated by younger children. Silver nitrate topical nasal cautery may be used. Soft and malleable (resorbable) nasal packs such as Nasopore may also be considered. In children known to have a bleeding diathesis, cautery and nasal packing is best avoided wherever possible and haematology 16 Figure 1: Axial CT scans demonstrating bilateral bony and membranous choanal atresia. Figure 2: Axial CT scans illustrating Pyriform Aperture Stenosis and a midline central incisor. Figure 3. Sublabial surgical approach to widen the nasal pyriform aperture. Figure 4: Axial CT scan (without contrast) showing subperiosteal collection within the right orbit and complete opacification of the right ethmoidal air cells. advice regarding administration of appropriate clotting factors should be sought. 6. Nasal trauma and septal haematoma Paediatric nasal trauma is a common presentation to the Accident and Emergency department but not all cases are then referred to ENT. In the face of any paediatric nasal trauma it is important for the nasal septum to be properly examined for any signs of a septal haematoma. Failure to identify such an injury may result in cartilage necrosis, abscess formation and later a saddle deformity. The external structure of the nose in young children is predominantly cartilaginous and the nasal bones are soft. Fracture of the nasal bones is therefore very rare particularly in children under the age of six years and displacement is a more common finding. Manipulation is usually all that is required for such displacements. A septal haematoma (both unilateral or ENT & audiology news | www.entandaudiologynews.com bilateral) requires urgent evacuation if the cartilage is to be saved and abscess formation is to be avoided. Broad spectrum antibiotic therapy is advised for five to seven days. n References 1. Devgan BK, Harkins WB. Congenital choanal atresia. Twenty years experience. Int Surg 1977;62(8):397-9. 2. Froehlich P, Ayari-Khalfallah S: Management of Choanal Atresia. In: Pediatric ENT. Edited by Graham JM, Scadding GK, Bull PD. New York: Springer; 2007;291-4. 3. Samadi DS, Shah UK, Handler SD. Choanal atresia: a twenty year review of medical co morbidities and surgical outcomes. Laryngoscope 2003;113(2):254-8. 4. Belden CJ, Mancusso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology 1999;213(2):495-501. 5. Rollins N, Booth T, Biavati M. Case 40: congenital pyriform aperture stenosis. Radiology 2001;221(2):392-4. 6. Qureshi AA, Lowe DA, McKiernan DC. The origin of bronchial foreign bodies: a retrospective study and literature review. Eur Arch Otorhinolaryngol 2009;266(10):1645-8. Neck Abscesses in Children Louise Melia, MBChB, MRCS, ST5 ENT, Royal Hospital for Sick Children, Yorkhill, Glasgow, G3 8SJ, UK. C ervical lymphadenopathy in children is extremely common, and neck infections account for a significant proportion of acute paediatric otolaryngology admissions and therefore resource allocation. Although a high proportion of acute lymphadenitis resolve with antibiotic therapy, a significant number will progress to a suppurative collection requiring surgical incision and drainage, as well as intravenous antibiotics and prolonged hospital stay. Superficial lymph node abscess Haytham Kubba, Consultant Paediatric Otolaryngologist, Royal Hospital for Sick Children, Yorkhill, Glasgow, G3 8SJ, UK. Correspondence E: louisemelia07@ gmail.com Declaration of Competing Interests None declared. Acute lymphadenitis is common in children, and usually presents as a swollen lymph node or nodes after an upper respiratory tract infection (URTI). Superficial lymph node abscesses tend to present with a triad of neck swelling, neck pain and pyrexia. In addition, patients may have poor oral intake. Children may have signs or symptoms of sepsis, and may be dehydrated, and this needs addressed immediately.1 Lymph node abscesses are usually of bacterial aetiology, with infections potentially arising from the oropharynx or anterior nares. The most frequently isolated organism is Staphlococcus aureus.2,3 Treatment for superficial neck abscesses is surgical and medical. Small abscesses of less than 3ml volume can be treated with intravenous antibiotics only. Initial surgical incision and drainage with cultures is reserved for patients presenting with typical rapid onset neck mass and symptoms consistent with abscess such as fever and increased white blood cell count.4 The main issue for the surgeon is to distinguish those children who require incision and drainage from those who can be managed conservatively. Clinical assessment for fluctuance, and ultrasound for liquefaction are the usual means for doing this, although both are fallible. Peritonsillar abscess This can be difficult to diagnose. In a frightened, toxic child it may not be possible to examine the oropharynx due to trismus and poor co-operation. An experienced ultrasonographer may get surprisingly good views of the tonsil to allow diagnosis. Some cases will improve with intravenous antibiotics, but most will need to proceed to surgery with a presumptive diagnosis only. A senior anaesthetist and a gas induction are mandatory. The trismus will usually relax once the child is anaesthetised. At this point, a tonsillectomy is probably the most reasonable course of action if quinsy is confirmed. Floor of mouth Infection in the floor of mouth is usually secondary to a dental source, and can present with swelling in the submandibular, submental or submasseteric regions (Figures 1a and 1b). Life threatening airway compromise can develop rapidly and intubation or tracheostomy may be needed. Management should include drainage of the abscess as well as treatment of the primary infective source, therefore a dentist or maxillofacial surgeon should be involved. Antibiotics must provide anaerobic cover. Deep neck space infections Figure 1a: Floor of mouth infection. Figure 1b: Intraoperative image of floor of mouth infection. Deep neck infections spread along the fascial planes and spaces of the head and neck region. Despite the widespread use of antibiotics for the early treatment of cervical infections and worldwide ENT & audiology news | www.entandaudiologynews.com 17 feature Figure 2: CT scan of large parapharyngeal abscess. improvements in dental care and oral hygiene, deep neck infections remain relatively frequent. Life threatening complications such as airway damage, jugular vein thrombosis, mediastinitis, pericarditis, pneumonia, and arterial erosion may develop because of delays in diagnosis and treatment.5 Abscesses in the retropharyngeal space often present as airway emergencies in infants. They develop from suppuration in retropharyngeal lymph nodes. The presentation is similar to epiglottitis with pyrexia, drooling and soft stridor. Drainage can be achieved transorally, but an experienced anaesthetist is essential to secure the airway. Infection in the parapharyngeal space can be due to dental infection or tonsillitis as well as the usual URTI. Presentation can be insidious with little external swelling in the neck and only ‘soft’ signs such as low grade pyrexia, malaise and torticollis. CT scanning is helpful in confirming the diagnosis and planning the surgical approach (Figure 2).6 An external approach allows a drain to be placed to prevent reaccumulation. In some cases, depending on the position of the abscess in relation to the great vessels and oropharyngeal mucosa, it may be possible to drain intraorally and avoid a scar, but the risk of reaccumulation is higher. Atypical mycobacterial infection This is surprisingly common and often misdiagnosed. The organisms (M. bovis, M. avium intracellulare, and so on) are widely present in the soil and in the limescale deposits in your shower head. They enter via oral ingestion and pass to the regional lymph nodes, typically parotid and submandibular nodes. The child presents with a neck lump, which develops violet-red skin discolouration, with eventual skin breakdown (Figure 3a). A sinus discharges for months before healing with a puckered scar (Figure 3b). This whole process can take up to three years to complete. 18 Figure 3a: Typical appearance of atypical mycobacterial infection. The diagnosis is clinical on the basis of the characteristic skin appearance in a child who remains systemically well and apyrexial and whose history is longer than would be expected for a bacterial abscess (usually a few weeks). Aspiration is controversial: while it may provide a sample for Ziehl-Neelsen staining, this comes at the cost of potentially causing skin breakdown and a discharging sinus, compromising the ultimate cosmetic result. Incision and drainage should clearly be avoided for the same reason. Culture takes six weeks and is only positive in a minority of cases. A chest x-ray is mandatory, but tuberculin testing is only necessary for children at high risk of TB. Some children will resolve spontaneously in a short time, or respond to prolonged treatment (3-9 months) with antibiotics. Single-agent regimens (clarithromycin, ciprofloxacin) and multi-agent regimens have been used (clarithromycin plus ethambutol, isoniazid or rifabutin). How many children will settle with antibiotics is currently unknown. For the remainder, surgery should be considered if it will shorten the duration of the disease and if the cosmetic result is likely to be better than that resulting from natural resolution. If antibiotics are to be used, follow-up should be early and frequent to identify those who are not resolving: in these children surgery should be done early to prevent skin loss and optimise the cosmetic outcome. Surgical excision is the treatment of choice if the skin is at risk of breakdown or if the disease progresses despite antibiotic therapy. Surgery is difficult due to the large mass of matted nodes and overlying collarstud abscesses in the subcutaneous plane. A block dissection of lymph nodes will suffice for limited disease but a partial parotidectomy and supraomohyoid neck dissection are not uncommonly required for extensive disease. Discoloured skin can usually be saved if it is in a cosmetically obvious place Figure 3b: Burnt-out atypical mycobacterial infection allowed to run its course without treatment over 2.5 years – early surgery is recommended to avoid this sort of cosmeticallyobvious scarring. (such as the cheek) by curetting the subcutaneous abscess from the deep surface of the dermis. The skin colour will eventually return to normal. In such cases, we would suggest clarithromycin for three months postoperatively, but there is currently no evidence to support this. In cases where the skin has already broken down, skin must either be excised or the disease left alone to run its course. Conclusion Neck abscesses in children can present in a variety of forms. Clinical assessment should be used in conjunction with ultrasound and CT scanning to determine those children that require surgical management with incision and drainage. It is vital that the airway remains safe throughout, and that an experienced anaesthetist is involved in cases where there is any concern. Dentists or maxillofacial surgeons need to be involved if there is any suspicion of a primary dental source. n References 1. Butler KM, Baker CJ. Cervical lymphadenitis. Paediatric Infectious Diseases. Philadelphia: WB Sauders Company; 1992:220-30. 2. Simo R, Hartley C, Rapado F, Zarod AP, Sanyal D, Rothera MP. Microbiology and antibiotic treatment of head and neck abscesses in children. Clin Otolaryngol Allied Sci 1998;23(2):164-8. 3. Inman JC, Rowe M, Ghostine M, Fleck T. Pediatric neck abscesses: changing organisms and empiric therapies. Laryngoscope 2008;118(12):2111-4. 4. Dodds B, Maniglia AJ. Peritonsillar and neck abscesses in the pediatric age group. Laryngoscope 1988;98(9):956-9. 5. Marioni G, Staffieri A, Parisi S, Marchese-Ragona R, Zuccon A, Staffieri C, Sari M, Speranzoni C, de Filippis C, Rinaldi R. Rational diagnostic and therapeutic management of deep neck infections: analysis of 233 consecutive cases. Ann Otol Rhinol Laryngol 2010;119(3):181-7. 6. Wetmore RF, Mahboubi S, Soyupak SK. Computed tomography in the evaluation of pediatric neck infections. Otolaryngol Head Neck Surg 1998;119(6):624-7. ENT & audiology news | www.entandaudiologynews.com Congenital Midline Nasal Masses Compared Mary-Louise Montague, MBChB (Hons), FRCS ORLHNS, Consultant Otolaryngologist, Honorary Senior Clinical Lecturer, University of Edinburgh, Edinburgh, UK. Correspondence Department of ENT Surgery, The Royal Hospital for Sick Children, 9 Sciennes Road, Edinburgh, EH9 1LF, UK. E: mary-louise.montague @luht.scot.nhs.uk Declaration of Competing Interests None declared. N asal dermoid cysts, encephaloceles and gliomas are the most frequently seen congenital midline nasal masses. Overall congenital midline nasal masses are rare. Although rare they are clinically important because of their potential for connection to the central nervous system. Their potential to cause disfig- urement, destruction of the cranial base and meningitis should not be underestimated. This article compares the embryological development, clinical presentation, optimal imaging, complications and surgical management of nasal dermoid cysts, encephaloceles and gliomas. Embryology Congenital midline nasal masses are believed to share similar embryogenetic origins. A failure of the normal separation of the different germ cell layers is considered to be causative. Consequently, all patients with congenital midline nasal masses must be considered as potentially having an intracranial extension. During formation of the skull base and nose the mesenchymal structures are formed from several centres which eventually fuse and ossify. Before fusion there are recognised spaces between these elements – the fonticulus frontalis, prenasal space, and foramen cecum (Table 1). They are important in the development of congenital midline nasal masses. During development dura projects through the foramen cecum and attaches to skin. This connection is normally lost when dura separates from nasal skin and retracts through the foramen cecum. If skin maintains an attachment to the underlying fibrous tissue, nasal capsule, or dura, epithelial elements may be pulled into the prenasal space with or without dural connection, this predisposing to dermoid cyst formation.1 The hypothesis for the formation of gliomas is similar to that of nasal dermoids. They are thought to develop from extracranial rests of glial tissue persisting after abnormal closure of the fonticulus frontalis. A second theory hypothesises that they may represent encephaloceles that have lost their cerebrospinal fluid (CSF) connection. Encephaloceles are thought to develop as a result of abnormal closure of the fonticulus frontalis resulting in herniation of meninges with or without brain tissue. Clinical presentation Of the three anomalies, nasal dermoid cysts are the most common accounting for approximately 60% of all congenital midline nasal lesions.2 The mode of presentation and other characteristics of these lesions are compared in Table 2. A positive Furstenberg test refers to expansion of a lesion with crying, Valsalva manoeuvre, or compression of the ipsilateral jugular veins. Optimal imaging All suspected congenital abnormalities of the nose require radiological evaluation, the objective being to confirm the clinical diagnosis, delineate any intracranial involvement and detect associated abnormalities. It is crucial not to biopsy or excise any intra- or extranasal mass in infants and children prior to complete work-up, Table 1. Potential spaces important in the development of congenital midline nasal masses. ● ● ● Fonticulus frontalis – space between the frontal and nasal bones which eventually fuses with the foramen cecum to create a separation between intracranial and extracranial structures Prenasal space – space between the nasal bones and the nasal capsule (the precursor of the septum and nasal cartilages) Foramen cecum – formed by articulation of a small notch at lower end of the frontal crest of the frontal bone with the ethmoid ENT & audiology news | www.entandaudiologynews.com 19 feature Table 2. Nasal dermoid cysts, encephaloceles and gliomas compared. Nasal Dermoid Cyst Nasal Encephalocele Nasal Glioma Sex predilection Occur equally between sexes More frequent in males Occur equally between sexes Presentation Slow growing midline mass, midline sinus or combination of these Midline mass Firm mass Nasal obstruction Slow growing firm, non pulsatile unilateral nasal Hypertelorism Polyp – reddish coloured sometimes with telangiectasias Hair protruding from sinus tract/cyst pathognomonic Site Broad nose Intermittent discharge of sebaceous material CSF Rhinorrhoea Nasal obstruction Inflammation/abscess Recurrent meningitis CSF Rhinorrhoea Anywhere from glabella to philtrum of lip. Lower 1/3 of nasal bridge most common Root of nose or inferior to nasal bones 60% Extranasal – near root of nose 30% Intranasal – typically medial to middle turbinate 10% Combined a c Transillumination No Yes Compression May be fluctuant -ve Furstenberg test Soft and compressible +ve Furstenberg test Non compressible -ve Furstenberg test Contents Skin and dermal elements including hair follicles, sweat glands and sebaceous glands Meninges with or without brain tissue Benign masses of glial tissue containing large aggregates of astrocytes and fibrous connective tissue enveloping blood vessels Intracranial extension Up to 20-45% All 15%-20% Frequency of associated abnormalites 40% associated with craniofacial malformations 30-40% Generally isolated anomalies b d Figure 1: CT and MRI demonstrating a nasal dermoid cyst with intracranial extension. (a) Axial CT showing separation of the nasal bones. (b) Widening of the foramen cecum on coronal CT. (c & d) T1 and T2-weighted sagittal MR views respectively showing multiple cysts extending intracranially with attachment to dura. 20 No including imaging, due to the risk of CSF leakage and meningitis if there is an intracranial connection. The complimentary roles of high resolution computed tomography (HRCT) and multiplanar MRI in congenital midline nasal masses are much quoted in the literature.3 But MRI is undoubtedly the best imaging modality with the distinct advantages of more precise soft tissue imaging, direct sagittal imaging planes and the possibility to generate multiple tissue contrasts. Many infants and children will require general anaesthesia for adequate MRI evaluation but this is justified in the knowledge that surgical planning is optimised. Common CT findings in nasal dermoid cysts are bifid nasal bones, a thickened or bifid nasal septum, and a circular translucency under the nasal bones. The presence of a widened foramen cecum or a bifid crista galli on CT suggests intracranial extension of the dermoid. Non visualisation of an intracranial mass on CT does not exclude the possibility of intracranial extension. False positives may also be seen with CT and may be accounted for by anatomic variants and incomplete ossification of the crista galli and foramen cecum (14% of children younger than one year of age). Nasal dermoids appear strikingly hyperintense on T1-weighted MRI due to their fat content (Figure 1). ENT & audiology news | www.entandaudiologynews.com feature Figure 2: A midline nasal dermoid cyst presenting with local infection and abscess formation extending superolaterally. The dorsal midline pit or sinus is apparent. Figure 3: Intranasal glioma obstructing the right nasal cavity of a neonate. This had no intracranial attachment and was removed endoscopically with a microdebrider. A nasopharyngeal airway was required preoperatively. Nasal gliomas are usually isodense on CT. Calcifications are reported rarely and cystic changes occasionally within them. On MRI nasal gliomas are usually hyperintense on T2-weighted images with variable intensity on T1-weighted images. Because of its high soft-tissue resolution MRI is particularly useful in imaging encephaloceles. MRI demonstrates the pathway of intracranial herniation and the presence of associated anomalies more clearly than CT. MRI will also differentiate encephalocele from glioma if an adjacent communicating CSF space is present. The lack of ionising radiation associated with MRI is especially important in children who may on occasion require follow-up imaging. causing remodelling and deformity of the nasal bones as well as progressive facial disfigurement. Obstruction of the nasal passage and nasolacrimal duct can occur causing respiratory distress in the neonate and epiphora on the affected side respectively (Figure 3). Complications Prompt diagnosis and management is essential in light of the potential for complications. Dermoid cysts Failure to recognise and treat this condition promptly may lead to progressive enlargement of the cyst and bony and cartilaginous distortion of the nose. There is also potential for primary or recurrent infection that may progress to loss or scarring of overlying skin, meningitis, brain abscess, cavernous sinus thrombosis, or periorbital cellulitis (Figure 2). Encephaloceles and gliomas Untreated these may be complicated by CSF rhinorrhoea, meningitis and intracranial abscess. Both may also expand gradually Surgical management Treatment of congenital midline nasal masses requires early, well-planned, careful, complete surgical resection. Nasal dermoid cysts must be removed intact as even a small epithelial remnant can lead to recurrence. The external rhinoplasty approach has largely replaced midline vertical and lateral rhinotomy incisions.4 It provides excellent exposure of the nasal dorsum and allows sinus tracts to be followed and removed in their entirety by providing an adequate angle of approach to and visualisation of the anterior skull base. More control can be exerted during osteotomies and the aesthetic outcome is vastly superior compared to a midline dorsal incision. Cartilage grafts are occasionally required for dorsal augmentation. For dermoids with substantial intracranial components a combined procedure with a neurosurgeon may be required. A neurosurgical approach combined with either an external rhinoplasty approach or lateral rhinotomy is generally recommended for encephaloceles and gliomas with intracranial extension. A number of cases of successful endoscopic microdebrider excision of small gliomas with and without evidence of intracranial extension have now also been reported. n ENT & audiology news | www.entandaudiologynews.com It is crucial not to biopsy or excise any intra- or extranasal mass in infants or children prior to complete work-up, including imaging, due to the risk of CSF leakage and meningitis if there is an intracranial connection References 1. Charrier JB, Rouillon I, Roger G, Denoyelle F, Josset P, Garabedian EN. Craniofacial dermoids: an embryological theory unifying nasal dermoid sinus cysts. Cleft Palate Craniofac J 2005;42(1):51-7. 2. Brown K, Brown OE. Congenital Malformations of the nose. In: Pediatric Otolaryngology Head & Neck Surgery, 3rd edition. Edited by Cummings CW. St Louis: Mosby; 1998:92-8. 3. Barkovich AJ, Vandermarck P, Edwards MS, Cogen PH. Congenital nasal masses: CT and MR imaging features in 16 cases. Am J Neuroradiol 1991;12(1):105-16. 4. Bilkay U, Gundogan H, Ozek C, Tokat C, Gurler T, Songur E, Cagdas A. Nasal dermoid sinus cysts and the role of open rhinoplasty. Ann Plast Surg 2001;47(1):8-14 5. Agirdir B, Derin A, Ozbilim G, Ozçaglar H. Endoscopic management of intranasal glioma. J Pediatric Surg 2004;39(10):1571-3. 21 Virus Infection of the Paediatric Airway and Chronic Disease Effects Adam J Donne, PhD, FRCS(ORL-HNS), Consultant Paediatric Otolaryngologist and Honorary Senior Lecturer. W ithin the realms of Paediatric ENT arguably the most important airway infecting viruses are the types that result in bronchiolitis and Recurrent Respiratory Papillomatosis (RRP). Both Respiratory Syncytial Virus (RSV) and Human Papillomavirus (HPV) can result in chronic conditions. This article reviews some of the evidence published on the effect of these viruses upon the paediatric airway. This article does not intend to explore all viral aetiologies of bronchiolitis but rather indicate the relative complexity of this condition. Chronic airway disease implies a group of airway diseases, typically chronic obstrucMichael P Rothera, Royal Manchester Children's Hospital, Oxford Road, Manchester, M13 9WL, UK. Correspondence Adam J Donne, PhD, FRCS(ORL-HNS), Consultant Paediatric Otolaryngologist and Honorary Senior Lecturer, Alder Hey Children’s NHS Foundation Trust, Alder Hey, Eaton Road, Liverpool, L12 2AP, UK. E: ajdonne@ doctors.org.uk Declaration of Competing Interests AJD has spoken /lecturered at events sponsored by Sanof Pasteur MSD. 22 tive pulmonary airway disease. RSV may indeed result in chronic airway disease however this article focuses on the longer term viral effects. HPV infections can result in a disease of chronic duration Recurrent Respiratory Papillomatosis. RSV who have a predisposed risk. A 20 year prospective study reports that croup before two years of age generates an increased risk of asthma until adulthood independently of development of atopy.3 Viruses are not the only risk for bronchiolitis as passive smoking is a significant risk.4 RSV usually causes simple coryzal symptoms but it can result in bronchiolitis and pneumonia. RSV infection is common however, premature babies are more at risk as they have weaker immunity and a possibility of chronic lung disease. Chronic lung disease of prematurity is also termed bronchopulmonary dysplasia. It is thought to result from damage to lung tissue especially in the very premature hence low birth weight babies requiring mechanical ventilation. RSV is associated with an increased rate of asthma. Term babies who have RSV as infants are ten times more likely to have bronchodilators by eight to 10 years of age.1 This has been confirmed in other studies that identified asthma was present in 30% of children previously hospitalised for RSV compared to 3% in age and gender-matched controls.2 Controversy exists as to whether RSV causes asthma or simply highlights the children Croup (laryngotracheobronchitis) is characterised by a barking cough, stridor and hoarseness. Numerous definitions appear to exist in the published literature but they are based upon the clinical picture. Croup is caused by a viral infection resulting in swelling of the subglottis and on a chest radiograph would classically give a church steeple outline in the subglottis (Figure 1). On endoscopy the subglottic inflammation is visible (Figure 2). Croup is most commonly caused by parainfluenza virus type 1 and 2, other causes include RSV, Figure 1: Image of church steeple sign on chest radiograph is characteristic of croup. Figure 2: Endoscopic image demonstrating laryngeal inflammation and reduced subglottic airway. Croup ENT & audiology news | www.entandaudiologynews.com feature coronavirus, adenovirus, metapneumovirus, etc.5 The severity of croup can be classified by the Westley score into mild, moderate or severe;6 though this is primarily of research value. Recurrent croup is poorly defined. Originally, it was thought that recurrent croup did not have a viral aetiology however RT-PCR studies indicate that the viral aetiologies are similar. Up to 6% of children under four years suffer from recurrent croup.7 The causes of recurrent croup include: Subglottic stenosis,8 Asthma 17%9 and GERD 60%.9 However, inhaled steroids and GERD therapy result in an improvement in 77%.9 The rate of subglottic stenosis in reports on recurrent croup does vary and may reflect referral practices. Recurrent Respiratory Papillomatosis RRP is caused by HPV, predominantly types 6 and 11. This condition affects children and adults and typically has a more locally aggressive nature in children but very rarely undergoes malignant transformation. The likelihood of transformation has resulted in the terminology low risk (6 and 11) and high-risk (types 16 and 18) of malignant change. RRP is extremely difficult to treat due to the recurrent nature and the varied activity over the course of the disease. Many fundamental questions about RRP remain unanswered, perhaps the most important being why so many people are infected (albeit transiently) by HPV but so few develop disease. There is geographical variation in HPV prevalence from cervical smear study data, however the prevalence varies according to age and there in now a recognised U-shaped age-dependent distribution curve for prevalence.10 The prevalence for the UK is approximately 520% depending upon age group. Why does HPV manifest as RRP? This may be due to a problem with host immunity or HPV factors / cofactors which are currently undefined. Detection of HPV has improved over the last 20 years. Originally techniques were in situ hybridisation methods but PCR techniques are more sensitive however even these reply upon searching for a specific DNA sequence. This explains why additional HPV types have only recently been identified in RRP tissue. Could it be that different HPV have different prognostic potential? Many now believe that HPV 11 causes more aggressive disease than HPV6 though age of onset is more important than either 6 or 11.11 There are 19 HPV6 variants and 10 HPV11 variants Figure 3: Endoscopic image of Recurrent Respiratory Papillomatosis. and no commercially available test attempts to identify these.12 It is highly probable that the variants are important and differ in activity. Indeed in vitro studies go some way to prove this to be the case for some variants.13 Clinical management The endoscopic appearance of RRP is characteristic (Figure 3). There is difficulty in defining how frequently to perform MLTB surgery and papillomatous tissue clearance. Obviously the first surgery depends upon the extent of disease and potential for airway compromise at presentation. The second surgery should probably be around four to six weeks later. This will allow an assessment of the aggressiveness of the condition, though each patient’s condition does vary throughout its course. There are numerous methods for papillomatous clearance but cold techniques allow less collateral thermal damage hence better voice quality outcomes than laser techniques14 and the more laser procedures the worse the voice quality.15 This, plus the addition of more controllable techniques such as small laryngeal microdebriders, means that lasers seem to be used less frequently now even though they do offer a very precise area to be targeted. Coblation is a relatively new method which allows covalent bonds within tissue to be broken down due to generation of a flux of sodium ion yet the temperature generated is not greater than 60°C. Irrespective of the method used best practice should include a tissue biopsy at every surgery as the naked eye cannot differentiate between papillomatosis and early carcinoma. This is the safest way to ensure that an emerging carcinoma is not missed. Adjuvant therapy There have been many adjuvant therapies used, most with no direct scientific foundation for their use in RRP. The most contemporary is cidofovir which is a nucleoside analogue which, on entering the cell ENT & audiology news | www.entandaudiologynews.com undergoes two stage phosphorylation process. At this point the molecule resembles a nucleotide and can then be incorporated into expanding DNA. Scientific studies have shown that cidofovir has effects against HPV 16 (high risk)16 but no study has demonstrated such an effect with HPV6 or 11 (low risk).17 It was reasonable to assume that cidofovir may have been effective and the patient is more complex than a simple cell model. However, evidence does show that cidofovir in altered human cells that contain an HPV 6 gene (E6) results in changes in gene expression and results in an environment in which malignancy may develop.18 The cells that did not contain HPV did not demonstrate the same gene expression. So the risk of malignancy is cidofovir plus HPV. Rodent studies have shown cidofovir to be carcinogenic though this was not found to be the case in primates.19 There are no absolutely convincing reports of carcinoma following cidofovir exposure though it may be that the effects have not been fully appreciated. For example smoking is thought to be very carcinogenic yet only causes cancer in 10-15%.20 Radiation causes thyroid cancer but anything from three to 60 years post exposure.21 It could be that cidofovir has a low potency with a long duration before malignant effect is seen. The in vitro work demonstrated that at high enough doses cidofovir killed all cells. However, at lower (sub-lethal) doses a genetic environment was created in which malignant transformation might be possible.18 HPV exists not only at the point of the papillomas but also other areas of the neighbouring airway tissue and trachea.22 There must be a lowering gradient of drug the further away from the cidofovir injection site. Indeed, it is conceivable that HPV infected (but otherwise normal in appearance) trachea might develop a malignancy if exposed to this low dose. Indeed, such a report does exist.23 At the very least when taking consent for cidofovir administration to control RRP a risk of ‘unquantifiable malignant potential’ should be identified to the patient. Summarising the outcomes of cidofovir treatments from numerous published articles it does appear that cidofovir has some positive effect24 but it is not a cure and the administration regime should be carefully controlled. In vitro work has indicated that sub-lethal doses appear to stimulate growth of cells containing HPV genes and this observation has been made clinically with too long an interval between cidofovir administrations.25 23 feature trials and initial post-marketing surveillance conducted in several continents show both vaccines to be safe.”27 Current research Figure 4: Gardasil vaccine. Alpha-interferon has historically been used and summarising the results would indicate that the numbers undergoing some element of resolution is less than with cidofovir. Interferon works by stimulating the immune response against virus infections. The side-effects of alpha-interferon were frequently nausea and vomiting with a potential for hepatic derangement. The usage of an adjuvant should only be considered when there have been at least four interventions per year to control the airway (RRP task force guideline). Prevention is better than cure and it seems appropriate to offer a prophylactic vaccine if possible. Unfortunately the current UK HPV vaccination programme administers Cervarix which is effective against high risk HPV whereas Gardasil is also effective against HPV 6 and 11 (Figure 4). From the perspective of RRP it seems more appropriate to offer Gardasil. There have not been studies to evaluate the effect of Gardasil directly upon the rate of RRP due to numerous problems, not least the relative rarity of the condition. Genital warts (condylomata accuminata) are caused by the same HPV types 6 / 11 and it has been shown that Gardasil did reduce the development of genital warts.26 The implication is that Gardasil would do the same for RRP. Given the potential concerns of cidofovir safety it is reassuring that the WHO reports that, “Data from clinical Dr Farrel Buchinsky in Pittsburg, USA is working to identify the genetic susceptibility links to the development of RRP. He requires blood and tissue samples from patients for this major NIHR funded study. Ethical approval has been granted for the first UK site and involvement is encouraged to make this important study a success. For information contact Adam Donne at the email address provided above. Conclusion Viral infections are common and potentially important in childhood. They may have long-term effects. Increasing numbers of viruses are being found to result in bronchiolitis and the balance of evidence would suggest this is important in the development of asthma. HPV is remarkably common also yet predominantly transient. The factors that make HPV result in disease are not understood. There is no antiviral agent which effectively targets HPV 6 / 11. Vaccines and further research provide hope for the future. ■ References 1 Noble V, Murray M, Webb MS, Alexander J, Swarbrick AS, Milner AD. Respiratory status and allergy nine to 10 years after acute bronchiolitis. Arch Dis Child 1997;76(4):315-9. 2 Sigurs N, Bjarnason R, Sigurbergsson F, Kjellman B. Respiratory syncytial virus bronchiolitis in infancy is an important risk factor for asthma and allergy at age 7. Am J Respir Crit Care Med 2000;161(5):1501-7. 3 Piippo-Savolainen E, Remes S, Kannisto S, Korhonen K, Korppi M. Asthma and lung function 20 years after wheezing in infancy: results from a prospective follow-up study. Arch Pediatr Adolesc Med 2004;158(11):1070-6. 4 Jones LL, Hashim A, McKeever T, Cook DG, Britton J, Leonardi-Bee J. Parental and household smoking and the increased risk of bronchitis, bronchiolitis and other lower respiratory infections in infancy: systematic review and meta-analysis. Respir Res 2011;12:5. 5. Wall SR, Wat D, Spiller OB, Gelder CM, Kotecha S, Doull IJ. The viral aetiology of croup and recurrent croup. Arch Dis Child 2009;94(5):359-60. 6 Westley CR, Cotton EK, Brooks JG. Nebulized racemic epinephrine by IPPB for the treatment of croup: a double-blind study. Am J Dis Child 1978;132(5):484-7. 7 Hide DW, Guyer BM. Recurrent croup. Arch Dis Child 1985;60(6):585-6. 8 Kwong K, Hoa M, Coticchia JM. Recurrent croup presentation, diagnosis, and management. Am J Otolaryngol 2007;28(6):401-7. 9. Arslan Z, Cipe FE, Ozmen S, Kondolot M, Piskin IE, Yöney A. Evaluation of allergic sensitization and gastroesophageal reflux disease in children with recurrent croup. Pediatr Int 2009;51(5):661-5. 10. de Sanjosé S, Diaz M, Castellsagué X, Clifford G, Bruni L, Muñoz N, Bosch FX. Worldwide prevalence and genotype distribution of cervical human papillomavirus DNA in women with normal cytology: a meta-analysis. Lancet Infect Dis 2007;7(7):453-9. 24 11. Buchinsky FJ, Donfack J, Derkay CS, Choi SS, Conley SF, Myer CM 3rd, McClay JE, Campisi P, Wiatrak BJ, Sobol SE, Schweinfurth JM, Tsuji DH, Hu FZ, Rockette HE, Ehrlich GD, Post JC. Age of child, more than HPV type, is associated with clinical course in recurrent respiratory papillomatosis. PLoS ONE 2008;3(5):e2263. 12. Donne AJ, Hampson L, Homer JJ, Hampson IN. The role of HPV type in Recurrent Respiratory Papillomatosis. Int J Pediatr Otorhinolaryngol 2009;74(1):7-14. 13. Heinzel PA, Chan SY, Ho L, O'Connor M, Balaram P, Campo MS, Fujinaga K, Kiviat N, Kuypers J, Pfister H, Steinberg BM, Tay SK, Villa LL, Bernard HU. Variation of human papillomavirus type 6 (HPV-6) and HPV11 genomes sampled throughout the world. J Clin Microbiol 1995;33(7):1746-54. 14. Holler T, Allegro J, Chadha NK, Hawkes M, Harrison RV, Forte V, Campisi P. Voice outcomes following repeated surgical resection of laryngeal papillomata in children. Otolaryngol Head Neck Surg 2009;141(4):522-6. 15 Crockett DM, McCabe BF, Shive CJ. Complications of laser surgery for recurrent respiratory papillomatosis. Ann Otol Rhinol Laryngol 1987;96(6):639-44. 16. Abdulkarim B, Sabri S, Deutsch E, Chagraoui H, Maggiorella L, Thierry J, Eschwege F, Vainchenker W, Chouaïb S, Bourhis J. Antiviral agent Cidofovir restores p53 function and enhances the radiosensitivity in HPV-associated cancers. Oncogene 2002;21(15):2334-46. 17 Donne AJ, Hampson L, He XT, Rothera MP, Homer JJ, Hampson IN. Effects of cidofovir on a novel cellbased test system for recurrent respiratory papillomatosis. Head Neck 2007;29(8):741-50. 18. Donne AJ, Hampson L, He XT, Day PJ, Salway F, Rothera MP, Homer JJ, Hampson IN. Potential risk factors associated with the use of cidofovir to treat benign human papillomavirus-related disease. Antivir Ther 2009;14(7):939-52. 19 Inglis AF Jr. Cidofovir and the black box warning. Ann Otol Rhinol Laryngol 2005;114(11):834-5. 20 Dubey S, Powell CA. Update in lung cancer 2007. Am J Respir Crit Care Med 2008;177(9):941-6. 21 Seaberg RM, Eski S, Freeman JL. Influence of previous radiation exposure on pathologic features and clinical outcome in patients with thyroid cancer. Arch Otolaryngol Head Neck Surg 2009;135(4):355-9. 22. Smith EM, Pignatari SS, Gray SD, Haugen TH, Turek LP. Human papillomavirus infection in papillomas and nondiseased respiratory sites of patients with recurrent respiratory papillomatosis using the polymerase chain reaction. Arch Otolaryngol Head Neck Surg 1993;119(5):554-7. 23 Lott DG, Krakovitz PR. Squamous cell carcinoma associated with intralesional injection of cidofovir for recurrent respiratory papillomatosis. Laryngoscope 2009;119(3):567-70. 24 Donne AJ, Rothera MP, Homer JJ. Scientific and clinical aspects of the use of cidofovir in recurrent respiratory papillomatosis. Int J Pediatr Otorhinolaryngol 2008;72(7):939-44. 25. Albright JT, Pransky SM. Update on the use of cidofovir for managing recurrent respiratory papillomatosis. ENT News (Mar/Apr) 2003;12(1);71-3. 26. Muñoz N, Kjaer SK, Sigurdsson K, Iversen OE, Hernandez-Avila M, Wheeler CM, Perez G, Brown DR, Koutsky LA, Tay EH, Garcia PJ, Ault KA, Garland SM, Leodolter S, Olsson SE, Tang GW, Ferris DG, Paavonen J, Steben M, Bosch FX, Dillner J, Huh WK, Joura EA, Kurman RJ, Majewski S, Myers ER, Villa LL, Taddeo FJ, Roberts C, Tadesse A, Bryan JT, Lupinacci LC, Giacoletti KE, Sings HL, James MK, Hesley TM, Barr E, Haupt RM. Impact of human papillomavirus (HPV)-6/11/16/18 vaccine on all HPV-associated genital diseases in young women. J Natl Cancer Inst 2010;102(5):325-39. 27. World Health Organization, Human Papillomavirus (HPV) [www.who.int/nuvi/hpv/en/index.html] ENT & audiology news | www.entandaudiologynews.com The Rehabilitation of the Deaf Child Konstance Tzifa, MPhil, FRCS(ORL-HNS), DLO, Consultant ENT Surgeon, Birmingham Children’s Hospital, Birmingham, UK. R ehabilitation usually refers to restoration of a skill that is lost. In paediatric deafness the ability to hear may have not been there in the first place, therefore the term ‘habilitation’ is more appropriate because the skill needs to be taught. ‘Aural habilitation’ for children with congenital or acquired deafness depends on many factors relevant to the onset and type of deafness, and also on the mode of communication. Kate Hanvey, Specialist Speech and Language Therapist, The Midlands Hearing Implant Programme – Children’s Service, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK. Correspondence E: Konstance.tzifa@ bch.nhs.uk E: Kate.hanvey@ bch.nhs.uk Declaration of Competing Interests None declared. The importance of a critical learning period for language development is well accepted. This learning must occur very early in life to avoid greater social, educational and psychological disadvantages. Delays in diagnosis of deafness can be detrimental for the child’s language development. Therefore, the focus for clinicians is early diagnosis and intervention. For most children with normal hearing, listening and spoken language develops in line with other developmental milestones. This is not the case for most children who have a severe to profound hearing loss. Where listening skills are impaired, the development of spoken language becomes a challenge. Communication through visual language is limited by the skills of the parents and primary carers. Therefore the following are essential. 1. Early identification of hearing impairment in infants and children 2. Determine aetiology and progression of hearing loss 3. Provision of amplification, sound philosophy 4. Early educational options Infants can be fitted with hearing aids as soon as the hearing loss is confirmed, even prior to age three months. Binaural amplification and education facilitates the acquisition of speech and language by the child with profound hearing loss.1 The use of binaural hearing aids for amplification in children is well accepted.2 Monitoring of the child wearing hearing aids and hearing aid assessment is essential because changes in hearing performance are commonly seen in children using hearing aids. There are many causes for changes in hearing performance including malfunctioning hearing aids, additional acquired cause of hearing loss and progression of the existing hearing loss. For children with profound hearing loss, where hearing aids offer little or no benefit, a cochlear implant is the device of choice. Cochlear implant candidates should receive them as early in life as possible. In the UK, most centres aim for around 10 to 12 months of age. Since the first two implantations by Graeme Clarke3 and his group at the University of Melbourne in Australia, experience of cochlear implantation and rehabilitation for children has increased and proved cochlear implantation to be a remarkable achievement in habilitation of deafness and speech and language development. How the brain learns to interpret signals from the cochlear implant as meaningful is an amazing process. Hearing aids amplify sound to the cochlea. Cochlear implants replace the cochlea function through electrical stimulation of the hearing nerve. Hearing aids rely on existing hair cells. Implants restore the sensation of hearing, whereas hearing aids exploit existing hearing. Fitting of optimal amplification is just the beginning of the parental journey in helping their hearing impaired child develop competent and effective communication. The task that is faced by parents cannot be underestimated. To illustrate this, in a recent presentation, Carol Flexer4 shared some startling research conducted with normally hearing children. By the age of four years, children in ‘professional’ families have heard 46 million words spoken whereas children from ‘welfare’ families have heard 13 million words. This puts children from welfare families at such a disadvantage, the gap cannot be closed.5 For a congenitally profoundly deaf child awaiting cochlear implant fitting, a conservative assumption is that they will have already lost 12 months of listening experience and once fitted, their hearing is not comparable to their normal-hearing peers. Yet with carefully structured guidance and support, it is possible for some hearing impaired individuals to close the gap. One may assume that if optimal amplification is fitted ‘early enough’, then therapy and / or early educational intervention may not be required. However the following issues remain:6 ENT & audiology news | www.entandaudiologynews.com 25 feature 1. Reduced ‘listening time’ during the day during times when amplification cannot be worn (for example sleeping, bathing and so on), by comparison with 24/7 listening in normal hearing 2. Reduced opportunities for incidental learning through overhearing (90% of learning and knowledge in children is incidental), due to background noise and distance listening 3. Learning to listen begins after a period of sensory deprivation and therefore the early building blocks of communicative interactions that come through hearing, have not been established. Of course, all children and their families are different. There are several factors that affect outcomes of cochlear implantation and also play an important role in rehabilitation and education. A significant proportion of children who are born with a profound hearing loss will have additional needs.7 These sometimes impede learning and are often related to the primary aetiology. Awareness of the cause of deafness and any additional impairment is important for counselling as this may impact on the communication options chosen by parents or recommended by professionals. There may be individual family circumstances that make it difficult to access or take on board the support offered. In particular, in the early stages following diagnosis, parents may need time to grieve, to adjust and consider the options on behalf of their child. Therefore there is no unique intervention option that suits all, circumstances change over time and children and their families require a highly individualised programme which has been tailored to their needs.8 Ninety to 95% of children with hearing impairment are born into families where both parents have normal hearing.9,10 Many parents from this group would like their children to learn to listen and talk as their primary means of communication, to attend a mainstream school and to have the same opportunities available to them as their normal-hearing peers. Although not without its challenges, this is now a realistic desire for many, particularly for children who do not have significant additional needs above and beyond their hearing impairment. Auditory-Verbal Therapy and / or Auditory-Oral programmes can support parents who have chosen this route. It should be noted that these approaches are not the same, and sources of further information can be found at the end of this article. A high quality service should 26 provide support to parents and families to guide, coach, engage and empower them with confidence, skill and knowledge to develop their child’s auditory brain for listening and talking. Total Communication is a philosophy that includes a wide range of communication strategies using hearing, vision and touch. It appears to be most commonly interpreted to mean that individuals are using some sign language in addition to spoken language. Unfortunately, the result can be an impoverished rather than enhanced communicative exchange whereby the child does not receive a good language model in either modality.11 However a total communication approach does have a place. Some parents find it particularly useful to help establish basic meaningful communication in the absence of appropriate amplification (for example whilst waiting for a cochlear implant in toddlers) and some also like to use a total communication approach as a way of keeping options open. In addition, some children require a degree of sign language to augment their spoken communication and for these individuals sign support may be required throughout their educational career. Choosing a total communication route as a long-term option implies that all family members and other key carers will learn to sign to a competent level in order to provide enriched models of language. Sign Bilingualism aims to develop competent and age appropriate language using a formal sign language system (such as British Sign Language). Functional understanding and use of written and spoken language is also encouraged. Within the UK, Sign Bilingual programmes are usually delivered in some Special Schools for the Deaf and are therefore not usually available until a child is of Nursery age (around three or four years old). Parents who have little or no experience of deafness and sign language should be encouraged to think about the long-term implications of choosing this option for their child, as it is absolutely essential that all family members and other significant carers become fluent in the Sign Language used in the school. Learning sign language to a fluent level requires significant financial investment and takes several years. Without this, their children may become isolated from the rest of the family and may grow up being unable to communicate effectively within the family unit. Regardless of the amplification device and communication modality, the aim of an early intervention programme should be to prevent significant language delay and educational underachievement, and habilitate through a normal developmental sequence, rather than to rehabilitate disordered communication that has developed over time as a consequence of hearing loss. Not all roads lead to the same destination. Choices made when the child is 12 months old may not fit with the parents’ long-term hopes, dreams and wishes for their child. Parents should therefore be encouraged to obtain information from a range of sources in order to come to an informed decision about the choices they make on behalf of their child. n Further information National Deaf Children’s Society, Communicating with your deaf child [http://www.ndcs.org.uk/family_support/ communication_m/communicating_with_your_ deaf_child/] Carol Flexer homepage [http://www.carolflexer.com/] Auditory Verbal Lounge [http://auditoryverballounge.co.uk/] Auditory Verbal [http://www.auditoryverbal.org.uk/] Deaf Education Through Listening and Talking [http://www.deafeducation.org.uk/] References 1. McConnel F, Liff S. Symposium on sensorineural hearing loss in children: early detection and intervention. The rationale for early identification and intervention. Otolaryngol Clin North Am 1975;8(1):77-87. 2. Downs MP. Amplification in the habilitation of the young deaf child. In Early management of hearing loss. Edited by Mencher GT, Gerber SE. New York, NY: Grune & Stratton; 1981;199-224. 3. Clark G, Tong Y, Black R, Foster I, Patrick J, Dewhurst D. Multiple electrode cochlear implant. J Laryngol Otol 1983;91:41-4. 4. Flexer C. Cochlear implantation and neuroplasticity: linking auditory exposure and practice. Presented at the British Cochlear Implant Group annual conference, Nottingham, UK; 2011. 5. Hart B, Risley TR. The social world of children: learning to talk. Baltimore: Brookes Publishing Company; 1999. 6. Tyszkiewicz E, Stokes J. Paediatric habilitation. In Cochlear Implants A Practical Guide, Second edition. Cooper HR, Craddock LC. London: Whurr Publishers; 2006. 7. Condon M-C. Unique challenges, children with multiple handicaps. In Pediatric Amplification, Omaha. Edited by Feigin JA, Stemachowicz PG. Boys Town National Research Hospital; 1991. 8. Wyatt JR, Niparko JK. Evaluation of the benefit of the multichannel cochlear implant in children in relation to its cost. In: Cochlear Implant Rehabilitation in Children and Adults. Edited by Allum DJ. San Diego, CA: Singular: 1996;20-30. 9. Mitchell RE, Karchmer MA. Chasing the mythical ten percent: Parental hearing status of deaf and hard of hearing students in the United States. Sign Language Studies 2004;4(2):138-63. 10. NDCS factsheet. Statistics on childhood deafness in the UK; 2003. 11. Wilcox S. American deaf culture. Silver Spring, MD: Linstok Press; 1989. ENT & audiology news | www.entandaudiologynews.com Serious Complications of Acute Otitis Media: mastoiditis and intracranial sepsis Mr William PL Hellier, MBChB, FRCS(ORL-HNS), Consultant ENT Surgeon. Correspondence Department of ENT, Southampton University Hospital and Royal Hampshire County Hospital, Hampshire, SO16 6YD, UK. E: [email protected] Declaration of Competing Interests None declared. A cute Otitis media (AOM) is one of the most common diseases diagnosed in the paediatric population. Rates of AOM vary between countries, but approximately 30-60% of children will have an episode in their first year of life, and up to 70-80% by the time they are three years old. The greatest risk of AOM occurs in the second six months of life.1 In the USA it is the most common indication for antibiotic therapy. Mastoiditis Most cases of AOM will involve infection of both the middle ear cleft and mastoid air cells with purulent exudate. However in certain serious episodes, possibly due either to mastoid obstruction because of mucosal oedema leading to mucopus under pressure, or possible bacterial virulence, bacteria may penetrate the middle ear or mastoid mucosa. This leads to an infective osteitis or acute mastoiditis, often with involvement of the periosteum either by direct spread or via venous channels. If the infiltrative and destructive infective process continues, progression to coalescent mastoiditis may occur with breakdown of the normal mastoid architecture, associated, or followed by spread beyond the mastoid either intra or extra cranially. Serious complications of AOM are unusual but are of great interest to the otolaryngologist, as they will need management under the care of the ENT surgeon. Serious complications are classically divided into extracranial / intratemporal complications and Intracranial complications. These are detailed in Table 1. Mastoiditis is the most common of the serious complications of AOM. The classic presentation is that of a child with a history of AOM who develops a reddened, tender posterior auricular sulcus often with a degree of effacement, and protrusion of the auricle. This occurs due to the underlying osteitis and periosteitis. If left untreated this will progress and pus will collect under the periosteum forming a subperiosteal abcess or infection may spread further, potentially intracranially. There have been a number of studies that have examined the incidence of mastoiditis in different populations. In Norway the incidence from 1999-2005 of mastoiditis was found to be 4.3-7.1 / 100,000 in the two to 16 year old age group, but 13.5-16.8 in the under two year old population.2 In the UK a Table 1. Complications of Acute Otitis Media Intratemporal Complications Acute Mastoiditis Facial paralysis Labyrinthitis Sensorineural hearing loss The significance of mastoiditis is that it is the ‘staging post’ to intracranial complications Petrositis / Gradenigo’s Syndrome Extratemporal Complications Subperiosteal mastoid abscess Bezold’s abscess Intracranial Complication Extradural abscess Subdural abscess Cerebral / cerebellar abscess Sigmoid sinus thrombosis Otitic hydrocephalus Meningitis ENT & audiology news | www.entandaudiologynews.com 27 feature Figure1: Mastoiditis. CT scan showing opacification of the right middle ear and mastoid, post-auricular swelling with abscess formation. recent study has estimated the incidence as 0.15 / 1,000 child-years.3 Other studies have quoted incidences of between 1.1 and 12 / 100,000. Most have found higher rates in the under two years of age group.4 This is unsurprising as this group is when this incidence of AOM is at its greatest. Certainly this reflects the experience from my own unit where the vast majority of AOM complications have been in this age group. The incidence of mastoiditis after AOM has also been studied and, in the UK this was found to be approximately 2.4 cases of mastoiditis per 10,000 cases of AOM (0.024%), but this was higher in children not treated with antibiotics (0.038%).3 Historically Rudberg in 19545 reported mastoiditis occurring in 17 % of cases of AOM. Palva in 1959 described the incidence of mastoiditis after AOM in Finland as 0.3%.6 Palva reported again in 19857 showing an incidence of mastoiditis of 4 / 100,000, which he described as a marked reduction, and which he attributed to the increased use of antibiotics. However there may have been other factors such as a change in living standards over this time, which may have also influenced the rate of AOM. There has been some debate over the recent years whether there has been an increase in the incidence of mastoiditis, as there has been a growing trend for not prescribing antibiotics for AOM. Van Buchem in 1985 published a paper in the BMJ examining this subject,8 finding only a small effect of antibiotic use on the rate of mastoidtitis. However children under two were excluded from the study, and this is the most common age group to suffer from complications of AOM. Other recent studies have shown a reduced incidence of mastoiditis in countries where antibiotic prescribing is higher (rates in Holland where antibiotic use is low are 3.8 / 100,000, and 1.2-2.0 / 100,000 in 28 countries with high rates of prescribing).9 There have been a number of papers that have suggested no change in mastoiditis rates (including a recent publication from the UK comparing 1991-1998 and 19902006),3 but a few suggesting a rise in cases over the last few years.10,11 The picture is therefore a little mixed. This issue is further confused however when one examines the literature, as in most series approximately 4060% of children admitted with a complication of AOM had already been started on antibiotics by their primary care physician for the preceding AOM. Although 50% of children have had no prior treatment, it would seem a high proportion of cases of complications have occurred despite oral antibiotic therapy. This could be related to the virulence of the bacteria involved, the incorrect antibiotic or poor patient compliance. Bacteriology Reviews of the bacteriology of mastoiditis show that in a high proportion of cases, 40-50%, no growth will be found on microbiological swab analysis.4,12-14 This is possibly due to the previous administration of antibiotics. Of those cases with positive microbiology, in most series Streptococcus pneumoniae is the most frequent bacteria isolated. Strep. pyogenes, Staphylococcus aureus and Haemophilus influenzae are the next most common, with Pseudomonas aeruginosa and a mixture of anaerobes and other bacteria found infrequently. Antibiotic therapy for mastoiditis or a complication of AOM must therefore be targeted at these organisms. Intracranial complications The most concerning sequelae of AOM are undoubtedly those of intracranial septic complications, where there may be high morbidity and potentially mortality. The significance of mastoiditis is that this is the ‘staging post’ to intracranial complications. In all series intracranial sepsis occurs in nearly all cases as a combination with or after mastoiditis (the only exception is meningitis which may occur without signs of mastoiditis). This is the reason mastoiditis must be treated seriously and with urgency. Intracranial complications occur due to direct spread of infection from the mastoid ostetitis, but also via a septic thrombophlebitis of the local venous system or mastoid emissary veins. Inflammation of the neighbouring middle or posterior fossa dura may occur with localised abscess formation. Thrombophlebitis from the inflamed dura may lead to intracerebral or intracerebellar abscess collections. The walls of the sigmoid sinus are formed by dural folds, infection or inflammation of which leads to localised mural thrombus formation that may progressively reduce or totally obstruct venous flow. Propagation of this sigmoid sinus thrombus (SST) may extend into the internal jugular vein, the transverse sinus, or other venous sinuses. This thrombus may become infected leading to frank pus, or cause the obstruction of the cerebral venous drainage system leading to hydrocephalus (otitic hydrocephalus). In the preantibiotic era the mortality of sigmoid sinus thrombosis and intracranial abscess was almost 100%, however in modern times this mortality has fallen sharply but death still occurs. The incidence of intracranial complications after mastoiditis varies between modern series but is in the order of 4-20%.12,13 There seems to be reasonably even distribution between extra and intradural abscesses and infective SST. However there may well be a combination of these complications. Management The management of a child with mastoiditis has changed over the years. In the preantibiotic era surgery was the mainstay of treatment with mastoidectomy with myringotomy. In the antibiotic era there has been a marked change in management. A number of recent series have shown that the percentage of children with mastoiditis who need to undergo mastoidectomy has fallen to 1634.5%.15 The majority of children with mastoiditis, especially if early in its course, will resolve with intravenous antibiotics +/myringotomy. Some studies16,17 have shown that myringotomy does not always need to be performed but should be considered if the tympanic membrane is still intact to allow middle ear drainage, and also to collect microbiological samples. If a subperiosteal mastoid abscess is present at admission or develops subsequently, this will need drainage.15 Usually this will be performed by formal surgical incision, but some series have described management with simple needle aspiration in addition to myringotomy and antibiotics.17,18 Careful observation of a child with mastoiditis is needed because if the condition does not improve or deteriorates, further intervention with cortical mastoidectomy may be needed. This will probably be required if the child has developed an intracranial or severe extracranial complication. However there are a number of children where the decision to perform a mastoidectomy is more finely balanced. If there is a subperiosteal abscess, the child is systemically ENT & audiology news | www.entandaudiologynews.com feature Figure 2: Sigmoid Sinus Thrombosis. Axial CT scan with contrast showing enhancement of the right sigmoid sinus walls due to inflammation with no luminal flow due to thrombus. This is often called the Delta Sign. Figure 3: Cerebellar Abscess. Axial MRI scan showing opacification of the left middle ear and mastoid with a large cerebellar abscess. Figure 4: Sigmoid Sinus thrombosis. MRA showing no flow in the left sigmoid sinus (arrow). unwell or there is failure of resolution over 2448 hours, many otologists would argue that cortical mastoidectomy, with drainage of the abscess, should be carried out. However some series have managed such patients conservatively, and in the modern day era of subspecialisation not all otolaryngologists may be entirely comfortable exploring the mastoid of a 14 month old child. There have been a number of studies that have looked at features of mastoiditis that may predict for the need for mastoidectomy (high white cell count and CRP seem to be predictive), but none have shown any absolute indications, and there still remains a degree of clinical judgement. There is also no information that suggests whether a complete cortical mastoidectomy or a purely drainage procedure is most appropriate. Some children with AOM and mastoiditis will have established intracranial sepsis when they present to secondary care, others will develop this during the course of their disease. The classic symptoms of intracranial complications are an unwell child, with high fever (with spiking pyrexias, the so called ‘picket fence’ chart), often drowsy or lethargic and possibly with neurological signs. Children presenting in this way, or whose mastoiditis is not resolving need radiological investigation to assess the temporal bone and intracranial compartment. In the era of broad spectrum antibiotics, it must be remembered that not all children with an intracranial complication will present with classic signs however, and a high index of suspicion, and a low threshold for radiological investigations is important. CT scanning with contrast will give good bony information, and can show intracranial sepsis. MRI scanning gives far more brain and dural detail and will show peri-dural infection, cerebritis or cerebral abscess. Adding MRA gives more information about the sigmoid sinus,19,20 especially showing thrombus formation or cerebral sinus occlusion (Figures 2, 3, 4). Treatment of the intracranial complication will depend upon its nature but may need involvement of the neurosurgeons or paediatric neurologists. Extradural abscesses may be drained by complete cortical mastoidectomy with drilling down of the posterior or middle fossa plate to expose the extradural pus and exteriorise this into the cortical cavity. Subdural abscesses may be drained in a similar way but with needle aspiration through the exposed dura in combination with the neurosurgeons. Intracerebral or cerebellar abscesses are often drained by the neurosurgery team via a separate burr hole. A common finding in these cases is generalised dural inflammation with granulation tissue. All intracranial complication will need a prolonged course of intravenous antibiotics. Sigmoid sinus thrombosis (SST) presents a condition which is not usually managed by the neurosurgeons, as it is more often seen in children with clotting derangements and presents usually to the paediatric neurologists where it is managed medically, typically with anticoagulation. The treatment of SST secondary to AOM is different however and has slowly changed over the years. In the pre- and early antibiotic era thrombus extension and septic emboli were not uncommon, and internal jugular vein ligation (IJVL) with sigmoid venotomy, thrombus removal and packing were performed regularly. More modern series have shown that IJVL is now rarely needed, and surgical treatment has become more conservative, although there is still discussion over the optimum management.19-24 Cortical mastoidectomy can be indicated and it is important to fully expose the sigmoid sinus walls and some of the posterior fossa dura. This allows drainage of any perisinus collections or granulation tissue.21 Many papers have suggested that placing a needle into the sinus gives information about whether there is any blood flow, but this may be unnecessary as MRA now gives excellent preoperative information regarding blood flow and sinus patency. A number of series have indicated that in the presence of thrombus, the sinus should be opened and a thrombectomy performed.22-24 Recent studies however have shown that this may not be needed, and if the thrombus is noninfected, the sinus can be left intact and recannalisation may occur in a number of cases.19-21 If there is infection of the intrasinus thrombus then the sinus must be opened and the pus drained. My own experience from a number of cases of SST is that with thrombus alone the dural walls of the sigmoid sinus were thickened with granulations and could be left in situ, but in three of the cases where the thrombus had become infected the dural walls had already thinned and the area of the sinus was filled with purulent material and gas which was easily opened and drained. If a SST has occurred there is debate in the literature whether anti-coagulation is needed.20,21,24 A number of earlier case series routinely anticoagulated such children. However there have been reports of haemorrhagic complications,25 and recently there have been a number of series where anticoagulation has not been instituted, without any progression of the thrombus or septic emboli. 20-22 The need for anticoagulation needs consideration and ENT & audiology news | www.entandaudiologynews.com 29 feature discussion with the paediatric neurology team, but the indication is certainly not absolute. n References 1. Teele DW, Klein JO, Rosner B. Epidemiology of otitis media during the first seven years of life in children in greater Boston: A prospective, cohort study. J Infect Disease 1989;160(1):83-94. 2. Kvaerner KJ, Bentdal Y, Karevold G. Acute mastoiditis in Norway: No evidence for an increase. Int J Paed Otorhinolaryngol 2007;71(10):1579-83. 3. Thompson PL, Gilbert RE, Long PF, Saxena S, Sharland M, Wong ICK. Effect of antibiotics for acute otitis media on mastoiditis in children: A retrospective cohort study using the United Kingdom genral practice research database. Pediatrics 2009;123(2):424-30. 9. Van Zuijlen DA, Schilder AGM, Van Balen FAM, Hoes AW. National differences in incidence of acute mastoiditis: relationship to prescribing patterns of antibiotics for acute otitis media? Pediatr Infect Dis J 2001;20(2):140-4. 10. Finnbogadottir AJ, Petersen H, Laxdal P, Gudbrandsson F, Gudnason P, Haraldsson A. An increasing incidence of mastoiditis in children in Iceland. Scand J Infect Dis 2009;41(2):95-8. 11. Benito MB, Gorricho BP. Acute mastoiditis: Increase in the incidence and complications. Int J Paed Otorhinolaryngol 2007;71(7):1007-11. 12. Dhooge IJM, Albers FWJ, Van Cauwenberge PB. Intratemporal and intracranial complications of acute suppurative otitis media in children: renewed interest. Int J Paed Otorhinolaryngol 1999;49 Suppl 1:S109-14. 17. Taylor MF, Berkowitz RG. Indications for mastoidectomy in acute mastoiditis in children. Ann Otol, Rhinol, Laryngol 2004;113(1):69-72. 18. Lahav J, Handzel O, Yehuda MP. Postauricular needle aspiration of subperiosteal abscess in acute mastoiditis. Ann Otol Rhinol Laryngol 2005;114(4):323-7. 19. Bales BB, Sobol S, Wetmore R, Elden LM. Lateral sinus thrombosis as a complication of otitis media: 10-year experience at the children’s hospital of Philadelphia. Paediatrics 2009;123(2):709-13. 20. Chistensen N, Wayman J, Spencer J. Lateral sinus thrombosis: A review of seven cases and proposal of a management algorithm. Int J Paed Otorhinolaryngol 2009;73(4):581-4. 21. Wong I, Kozak FK, Poskitt K, Ludemann JP, Harriman M. Pediatric lateral sinus thrombosis: Retrospective case series and literature review. J Otolaryngol 2005;34(2):79-85. 4. Leskinen K, Jero J. Complications of acute otits media in children in southern Finland. Int J Paediatr Otorhinolaryngol 2004;68(3):317-24. 13. Luntz M, Brodsky A, Nusem S, Kronenberg J Luntz M, Brodsky A, Nusem S, Kronenberg J, Keren G, Migirov L, Cohen D, Zohar S, Shapira A, Ophir D, Fishman G, Rosen G, Kisilevsky V, Magamse I, Zaaroura S, Joachims HZ, Goldenberg D. Acute mastoiditis – the antibiotic era: a multicenter study. Int J Paed Otorhinolaryngol 2001;57(1):1-9. 5. Rudberg RD. Acute otitis media: comparative therapeutic results of sulphonamide and penicillin administered in various forms. Acta Otolaryngol Suppl 1954;113:1-79. 14. Quesnel S, Mguyen M, Pierrot S, Contencin P, Manach Y, Couloinger V. Acute mastoiditis in children: a retrospective study of 188 patients. Int J Paed Otorhinolaryngol 2010;74(12):1388-92. 23. Manolidis S, Kutz JW Jr. Diagnosis and management of lateral sinus thrombosis. Otol Neurotol 2005;26(5):1045-51. 6. Palva T, Pulkkinen K. Mastoiditis. J Laryngol Otol 1959;73:573-88. 15. Tamir S, Shwartz Y, Peleg U, Shaul C, Perez R, Sichel JY. Shifting trends: mastoiditis from a surgical to a medical disease. Am J Otolaryngol 2010;31(6):467-71. 24. Bradley DT, Hashisaki GT, Mason JC. Otogenic sigmoid sinus thrombosis: What is the role of anticoagulation? Laryngoscope 2002;112(10):1726-9. 16. Geva A, Oestreicher-Kedem Y, Fishman G, Landsberg R, DeRowe A. Conservative management of acute mastoiditis in children. Int J Paed Otorhinolaryngol 2008;72(5):629-34. 25. Shah UK, Jubelirer TF, Fish JD, Elden LM. A caution regarding the use of low-molecular weight heparin in pediatric otogenic lateral sinus thrombosis. Int J Paed Otorhinolaryngol 2007;71(2):347-51. 7. Palva T, Virtanen J, Makinen J. Acute and latent mastoiditis in children. J Laryngol Otol 1985;99(2):127-36. 8. Van Buchem FL, Peeters MF, Van ‘t Hof MA. Acute otitis media: a new treatment strategy. BMJ 1985;290(6474):1033-7. 22. Lee HL, Choi JC, Park K, Choung Y. Managements for lateral sinus thrombosis: does it need ligation of internal jugular vein or anticoagulants? Eur Arch Otorhinolaryngol 2009;266(1):51-8. www.entandaudiologynews.com Why not list your event in our online diary? It’s free Click and complete the simple interactive PDF form. It couldn’t be easier! 30 ENT & audiology news | www.entandaudiologynews.com Reassembling the Auditory World in Children with Cochlear Implants Blake C Papsin, MD. I t has been over twenty years since cochlear implants (CI) were first approved for use in children with sensorineural hearing loss (SNHL) and at the Hospital for Sick Children in Toronto, we recently implanted our 1,000th CI. While our under- standing of CI in children has evolved dramatically, we still cannot fully predict or explain their benefits and limitations for any one child. Clearly there are many research questions left to answer. Improved surgical techniques have minimised complications, Sharon L Cushing, MD. allowing us to reduce the age of implantation and CI is now routinely performed bilaterally on infants with safe, reliable and incredibly sophisticated devices.1 Likewise criteria for implantation have evolved and we now provide CI to individuals not originally considered to be candidates. Implanted children are developing linguistic skills on par with their peers, albeit with the aid of significant intervention from auditory verbal therapists. The outcome is magnificent when we measure speech perception in quiet or even with controlled background noise. By contrast, important questions regarding auditory plasticity and how children with early onset SNHL function in the real world Karen A Gordon, PhD. Correspondence Blake C Papsin, MD, Department of Otolaryngology – Head and Neck Surgery, Hospital for Sick Children, 555 University Avenue, Elm Wing 6103-C, Toronto, ON, Canada, M5G 1X8. E: blake.papsin@ utoronto.ca Declaration of Competing Interests None declared. remain. Whilst we were once thrilled with any improvements in hearing following CI, our present objectives are no less than to restore ‘normal’ auditory function. This brief communication will not act as a review of the current literature, but rather takes advantage of this rare opportunity to speculate on what we think is fundamental to CI outcomes in children based on questions we are presently asking in our laboratories. The auditory system, stimulated by either normal hearing or auditory prostheses, does not act in isolation. There is ample evidence that the auditory pathways can be modulated by other sensory and arousal mechanisms.2 As humans, we need to take in multiple, diverse inputs, focus on those which are important at that time, and decide how to integrate and respond to this information. The perception of the signal is as important as the context in which it resides. For example, a siren in most cases elicits a basic instinct to run for the exits. However, when a siren is heard in Bob Dylan’s Rainy Day Women No. 12 & 35 (which opens his 1966 album Blonde on Blonde), our reaction is completely different. Clearly the context in which sound is presented is key to understanding this input and deciding how to react to it. In the lab, we start to see the limitation of CI when we ask recipients to reassemble auditory information by judging the emotion in a spoken sentence. We found that these children are less accurate and Figure 1: Plain radiograph demonstrating bilateral cochlear implants with the electrode array within the cochlea (white arrowhead). respond more slowly than their normal hearing peers when doing this task.3 Increased reaction times strongly suggest that additional processing time is required to reconstruct the auditory environment and react to it. Along the same line, we have been studying musical perception in children with CI and have found that their ability lags far behind their normal hearing ENT & audiology news | www.entandaudiologynews.com 31 feature The human brain rapidly assembles and processes multi-sensory information for both survival and enjoyment. Assembly of the auditory environment in children with significant hearing loss is best achieved by early cochlear implantation, ideally performed bilaterally, to encourage auditory development which is as close to normal as possible Figure 2: Histologic section of the cochlea with cochlear implant electrode array in situ in the basal turn (white arrow). Photo courtesy of Cochlear Limited. peers. These deficits reflect the poor temporal information provided by the CI.4 Despite these limitations, however, children with CI are still able to distinguish between happy and sad music and that they love to listen to music.3 Again, we see that they compensate for limited input and enjoy using what they have. Measuring progress It is important to note that our ‘standard’ battery of speech perception tests provides a narrow view of auditory perception. Such measures do not elucidate how children use their CI in daily life as they develop socially, scholastically, preparing for employment and 32 adulthood, nor the complex neural network involved in hearing. Although the fundaments of auditory perception can be examined in the laboratory by asking listeners to identify the properties of simple sounds, more complex and ‘real world’ auditory processing is often left unexamined. The use of complex stimuli and listening tasks can help define the limits of hearing in children using CI and allow us to study how these children might compensate to meet these listening challenges. It follows then that the real test of CI effectiveness should be how well the sensory input it provides steers the contextual reassembly of the auditory environment. It is reasonable to assume that hearing is compromised when auditory input is limited or degraded. Yet, the auditory pathways appear remarkably adept at using whatever information it receives. To our initial surprise, even children with significant anatomic malformations of the cochleae perceive reasonably well in test environments.5 This speaks to the complexity and redundancy that must exist in the auditory system which, when fully functional, is able to perceive subtle cues in speech such as innuendo and sarcasm. Efforts to 1) increase the amount of information made available to the auditory system, 2) improve the ability to extract information from the ENT & audiology news | www.entandaudiologynews.com feature environment and / or 3) promotes normal development of the auditory system, would enhance the ability to reconstruct the auditory environment for CI users. These three principles should provide the motivation for improved implant technology and novel methods to test CI outcomes in the future. Reorganisation of the hearing network Is it naïve to think that one or even two CIs could provide sufficient information for children to reassemble the auditory environment and make important decisions about how to interact or react? To answer this, we need to understand how the underlying mechanisms of auditory perception develop. It is clear that the normal interplay between different brain areas is altered in congenital deafness, resulting in abnormal reorganisation.6 Although we would like the early restoration of audition through CI to eliminate any changes away from normal, we realise that reorganisation likely begins with the onset of deafness and is already underway even when we provide CIs in early infancy.7 Given that the CI cannot fully mimic the normal hearing ear, we do not know whether CI use can halt or reverse the effects of deafness in early development. It is important to remember that the CI may not be treating the same deafness or the same effects of deafness in all children. We have recently shown a high degree of heterogeniety in cortical responses in children at initial CI use. Interestingly, responses look more uniform in children with biallelic GJB-2 mutations. 8 This suggests that the etiology of the hearing loss may define the state of the auditory pathways at the time of CI and perhaps even predict how the auditory system will respond to CI stimulation. This issue could become more important as more discrete and focused stimulation of the auditory nerve is provided through advances in technologies. Binaural input In addition to the possible limitations of CI stimulation on auditory development, we must acknowledge the implications of unilateral auditory deprivation through the provision of a single sided CI. We are finding that the auditory cortex becomes reorganised after long periods of unilateral CI use (no contralateral hearing aid). This reorganisation persists even when a second CI is provided and the bilateral implants are used for three to four years.9 Our recent data shows that the auditory brainstem is able to detect interaural level and timing cues after sequential bilateral implantation (long inter-implant delay), but that binaural input is not normally processed in the cortex. Perhaps this is why these children are unable to perceive changes in interaural timing cues in a behavioural lateralisation task.10 Even with these abnormalities, we see that bilateral implantation has been beneficial, providing an improvement in spatial hearing for sound localisation and detecting speech in noise.11 Although these skills are far from normal in this group, the benefits attest to the remarkable ability of the child to integrate and use whatever information is available to make sense of the world around them. Auditory support of non-auditory tasks The auditory environment is often used to support non-auditory tasks. Normal binaural auditory processing allows us to construct a spatial organisation of sounds in our environment and directs our visual attention to the sound source. These instinctive reactions allow us to navigate safely through traffic or locate a friend in a crowd and it is these fundamental hearing skills which we should strive to restore for children with SNHL and CI. Up until recently, most individuals received a single CI which precludes binaural processing, challenging sound localisation. The advent of bilateral, and particularly simultaneous bilateral CI, allows us to re-approximate, at a minimum, the physiology of binaural sound input with the aim of restoring binaural processing and localisation and our ongoing studies suggest that we are making progress. Another example of how auditory information supports non-auditory tasks, is the statistically significant improvement in balance function (on the BruininksOseretsky Test of Motor Proficiency 2 balance subset) observed when the CI is used during the balance task.12 These data provide a strong case for improved balance function using the reconstructed auditory environment as an additional source of sensory input. Conclusion and future directions Of late, our thinking is less about the implant and more about plasticity, and current efforts are focused on improving the child’s ability to correctly reassemble all the inputs that contribute to their ENT & audiology news | www.entandaudiologynews.com auditory environment. Can implant recipients detect emotion in music and speech? Can they understand sarcasm? Can they perceive innuendo? These critical aspects of the auditory environment allow the listener more than just survival; they comprise the ‘fine points’ of listening and allow maximal enjoyment of the auditory world. Can the implant recipient reassemble the auditory environment accurately and quickly enough to allow the full use of short-term memory and how does that affect the ability to learn? Should different strategies be employed in educating children with CI? The technology, though magnificent, has been reasonably static and the real gains in our opinion have been in understanding how the human uses this limited information so completely in an attempt to accurately reassemble the auditory world that surrounds them. n References 1. James AL, Papsin BC. Cochlear implant surgery at 12 months of age or younger. Laryngoscope 2004;114(12):2191-5. 2. Shimojo S, Shams L. Sensory modalities are not separate modalities: plasticity and interactions. Curr Opin Neurobiol 2001;11(4):505-9. 3. Hopyan T, Gordon KA, Papsin BC. Identifying emotions in music through electrical hearing in deaf children using cochlear implants. Cochlear Implant Int 2011;12(1):21-6. 4. Rubinstein JT. How cochlear implants encode speech. Curr Opin Otolaryngol Head Neck Surg 2004;12(5):444-8. 5. Papsin BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. Laryngoscope 2005;115(1 Pt 2 Suppl 106):1-26. 6. Rapin I. Consequences of congenital hearing loss – a longterm view. J Otolaryngol 1978;7(6):473-83. 7. Gordon KA, Valero J, Jewell SF, Ahn J, Papsin BC. Auditory development in the absence of hearing in infancy. Neuroreport 2010;21(3):163-7. 8. Gordon KA, Tanaka S, Wong DD, Stockley T, Ramsden JD, Brown T, Jewell S, Papsin BC. Multiple effects of childhood deafness on cortical activity in children receiving bilateral cochlear implants simultaneously. Clin Neurophysiol 2011;122(4):823-33. 9. Wong D, Gordon KA. Hemispheric Lateralization of Evoked Potentials in Bilaterally Implanted Cochlear Implant Users. In: 32nd Annual Midwinter Meeting of the Association for Research in Otolaryngology. Baltimore, MA; 2009. 10. Salloum CA, Valero J, Wong DD, Papsin BC, van Hoesel R, Gordon KA. Lateralization of interimplant timing and level differences in children who use bilateral cochlear implants. Ear Hear 2010;31(4):441-56. 11. Litovsky RY, Johnstone PM, Godar S, Agrawal S, Parkinson A, Peters R, Lake J. Bilateral cochlear implants in children: localization acuity measured with minimum audible angle. Ear Hear 2006;27(1):43-59. 12. Cushing SL, Chia R, James AL, Papsin BC, Gordon KA. A test of static and dynamic balance function in children with cochlear implants: the vestibular olympics. Arch Otolaryngol Head Neck Surg 2008;134(1):34-8. 33 The Future of Paediatric ENT Surgery Gavin Morrison, MA, FRCS, Consultant Paediatric Otolaryngologist. Correspondence Evelina Children’s Hospital, Guy’s & St Thomas’s NHS Foundation Trust, London, SE1 7EH, UK. E: gajm@ gavinmorrison.com Declaration of Competing Interests None declared. increasing numbers of young children requiring surgery are being transferred to regional tertiary centres for both emergency and elective surgery, without this transfer being planned, managed or resourced 34 I wish to discuss the future provision of ENT services within the UK. To determine where we are going, it should be helpful to look at where we have come from and at recent trends. Many factors will influence how paediatric ENT services are delivered across the UK. These include changes in medical education and surgical training, a reduction in experience resultant from years of compliance to the European Working Time Directive (EWTD), healthcare politics, driven by central government, and perhaps most importantly, what is happening to the allied specialties with whom we work so closely. Specifically, we will be driven by the changes we have seen in anaesthetic practice, over the past decade or more. Look back in anger The way we live now Since 1948, the NHS has tried to address healthcare inequalities. The model of our NHS ‘free at the point of delivery’ but centrally funded out of taxation and thus politically directed makes it vulnerable to the constant shifting of party politics. During my training and consultant years, I have witnessed endless initiatives, frameworks, plans and guidelines for the provision of health delivery and medical education. The 1983 ‘Griffiths Report’, the 1991 The Patients’ Charter and ‘Designed to Care’ – the White Paper of 1997 all had their merits. Through The NHS Plan (2000) we witnessed competition and the internal marketplace, fundholder GPs came and went, yet the Wanless Report of 2002 further increased the power and influence of primary care. More personally we have all been influenced by Action on ENT, MMC, The New Consultant Contract, PMETB, PBR, Patient Choice / Choose and Book, NICE and more. The current political will is going to see GP consortia handling ‘real’ budgets to buy care (from ‘any willing provider’) on behalf of their local communities; the abolition of all primary care trusts and of strategic health authorities, and the creation of a new NHS Commissioning Board. A similar American Model showed mixed success over a 20 year period and only a small proportion of these groups have survived.1 We are about to boldly go where no man has gone before with the ‘starship’ of the Health and Social Care Bill to guide us.2 Are we in a position to influence the future of Children’s ENT healthcare? We should be, as only the paediatric ENT surgical body itself has the understanding and experience to see what is really needed, and what is practicable. Above all, the need should be patient focused. The 1989 NCEPOD Report3 highlighted that surgeons and anaesthetists should not undertake occasional paediatric practice. The concept of a ‘minimum case load’ was introduced, as was the increasing centralisation of paediatric services. In 1989, over 80% of consultant anaesthetists had anaesthetised one or more infants of less than six months of age, within that year. Nine years later, that number had fallen to 40%. Evidence from Hospital Episode Statistical data (HES) confirms the perception that children’s surgery has shifted from District General Hospitals to specialist centres. In 2005, The Royal College of Surgeons Children’s Surgical Forum reported on trends in children’s surgery, in England.4 Marker operations for the study included grommets, tonsillectomy and adenoidectomy. Over the 10 year period from 1994 to 2004, the percentage of procedures undertaken in DGHs dropped from almost 80% to just over 60%, with the specialist centres showing the inverse of these percentages. The evidence is strong that there has been a significant shift in children’s ENT surgery from DGHs into specialist centres, and a reduction in overall number of procedures. Trends in general ENT & audiology news | www.entandaudiologynews.com feature paediatric surgery will also influence paediatric ENT. In 2006, a joint statement on general paediatric surgery provision in DGHs was made on behalf of the relevant colleges.5 It confirmed that increasing numbers of young children requiring surgery are being transferred to regional tertiary centres for both emergency and elective surgery, without this transfer being planned, managed or resourced. A future was foreseen in which almost all children under five years and most under eight, presenting with paediatric surgical emergencies, would be transferred from a DGH to a specialist centre for treatment. The development of paediatric surgical regional networks between groups of hospitals was envisaged. A three centre model for paediatric surgery, comprising small DGHs, intermediate centres which were large DGHs and some university hospitals, and thirdly, specialist or tertiary centres was proposed. This same model could be applied with benefit to paediatric ENT services. A recent review of children’s surgery6 highlighted minimum ages for emergency or urgent surgery to be under five or even under eight years of age. If these ages applied to paediatric ENT surgery many children would be disadvantaged as they could not receive ENT procedures locally and the specialist centres do not have sufficient resources to treat more three to eight year olds. Let us now consider how anaesthetic services are being driven in the UK. The Royal College of Anaesthetists (RCoA) in their publication ‘Raising the Standard: a Compendium of Audit Recipes’7 confirmed that avoidable deaths were identified in the under-10 age group. They proposed that a target for best practice should be that a consultant anaesthetist or equivalent be expected to hold core competencies in the management of simple elective procedures in fit children who are ASA 1 or 2 down to at least five years of age. The more up to date RCoA publication of April 2010, ‘Guidance on the Provision of Paediatric Anaesthesia Services’,8 broadly suggests that children with significant acute or chronic medical problems, those undergoing more complex procedures, neonates and small infants should be referred to specialist units or tertiary paediatric centres. Nevertheless, DGHs should have arrangements for treating simple surgical emergencies and should be able to resuscitate and stabilise seriously ill children of all ages, prior to their transfer. All consultant anaesthetists with a CCT or equivalent should be competent to provide care for common Table 1: ASA Physical Health Classification • ASA 1 is a normal healthy patient in all respects • ASA 2 has no functional limitations, but may have a well controlled disease in one body system, or mild obesity. These are the patients who can be treated in the DGH setting with an age limit to be agreed • ASA 3 patients have some functional limitation and controlled disease in more than one body system or in one major system • ASA 4 has poorly controlled severe disease with possible risk of death • ASA 5 is not expected to survive 24 hours without surgery and has imminent risk of death or multi-organ failure • ASA 6 is declared brain dead for organ donorship surgical conditions, both elective and emergency, for children aged three years and older. Day care surgery was considered appropriate for children for non-complex surgery in the healthy child with no comorbidities. In 2008, when President of BAPO, Peter Robb discussed and reported on the provision of ENT services highlighting that ENT training differs from general surgical training.9 Paediatric subspecialist work is embedded into both the syllabus and the final intercollegiate examination in ENT. Thus our consultants across the UK have experience and the expertise to manage younger children. In December 2010 The RCS of England published a survey10 in which of 305 DGHs, less than half were actually able to provide an emergency paediatric surgical service. Many of the Trusts faced problems sustaining anaesthetic services for children and 33% of hospitals were unable to anaesthetise children under the age of three. The London Specialised Children’s Services Review, commissioned by the NHS11 is suggesting organisation and coordination of currently fragmented services across London, where 28 hospitals provide children’s services, yet the number treated per annum ranged from 14 to 2,795! The key recommendations for change are to ENT & audiology news | www.entandaudiologynews.com focus a wide range of specialised children’s services in fewer hospitals, and to establish networks that co-ordinate specialised services in a defined area. The 8,000,000 population would support only two networks. Similarly, the ongoing ‘Safe and Sustainable NHS Review’ is dictating on the future of children’s congenital heart services and neurosurgery, in England.12 The result will be fewer tertiary specialised services, each with the potential for the best of outcomes. Back to the future I believe that the same principles currently being applied in other paediatric surgical specialties will, and should be, taken up by paediatric otolaryngology. The model of regional networks is essential but will in fact be driven by changes in anaesthetic practice. We are a strong sub-specialty group with the highest levels of training. Both our current close attention to agreed guidelines for Tonsillitis and OME, and our representation of the specialty at the international paediatric ENT meetings demonstrates that we have much to be proud of. Nevertheless, co-ordinated and resourced planning should enhance our delivery of safe care. I envisage a model in which paediatric ENT care continues to be delivered across the range of NHS settings. Primary care and community based ENT delivery could have an important place for certain outpatient services. Secondary care provision in hospitals should continue to provide the majority of routine paediatric ENT surgery in the over three year olds if possible. However, the routine paediatric ENT procedures will probably only be undertaken in secondary care DGHs where the children are ASA 1 or 2 and over the agreed age. If the anaesthetists’ current training guidelines are adopted, then children from three years and over should be accepted. If the trends in general paediatric surgery wield undue influence, then the cut-off age could become five (or even eight). This would disadvantage many; children would have to travel great distances for their ENT surgery and a much greater increase in the resources at the bigger centres would be required. The model of smaller (intermediate secondary care) and larger DGHs or university hospitals, networking together, is the right one. Those larger centres perhaps with maternity services and higher dependency critical care, will certainly be able to routinely undertake the slightly more complex and younger child. The tertiary centres will inevitably continue to receive more and more of the specialist problems, 35 feature the paediatric ENT emergencies and small children. Thus any young child with a potentially life threatening airway problem, and indeed many of the more complicated septic conditions such as neck abscesses, mastoiditis and complicated sinusitis, will not meet the criteria of being ASA 1 or 2, and all those under three will need to be retrieved or transferred to the tertiary centres. The ASA (American Society of Anaesthesiologists) classification system,13 together with a younger age cut off point, does provide a simple and practical means of determining healthcare policy. All children considered ASA 3 or above are likely to require transfer to tertiary or specialist centres. I envisage that we need in the region of a dozen tertiary centres across the UK. Currently, we have at least nine very strong providers of tertiary paediatric ENT care. The few major centres that are weaker should be actively developed with new recruitment and funding. These tertiary centres should all undertake the full range of paediatric ENT surgery and are also largely well positioned to encompass paediatric cochlear implantation. A smaller subset of these regional tertiary units, which might be termed quaternary, should provide for paediatric oncology, neurosurgery and the most demanding of the airway problems such as complex low tracheal work and other rarer congenital disease. I believe this model would be successful, but there are numerous problems, including training issues, ENT on-call rotas and who should undertake the paediatric ENT surgery in the DGH setting. To achieve a critical load, the ENT surgeon should perform an operating list at least once a fortnight and ideally, weekly. The other non-paediatric ENT operators should not undertake occasional surgery on children, but the whole department will need to be updated in emergency stabilisation and resuscitation of paediatric life-threatening complications, for on-call to be manageable. CME and revalidation could valuably include mandatory annual update training in these areas, run by the regional tertiary centres. Within the expanded tertiary centres, the emergency workload will be much greater than our current representation of 30%. To accommodate this really requires a significant expansion in consultants and facilities and would allow the emergence of stand alone paediatric ENT on call rotas. Training of all ENT surgeons in the UK already robustly includes paediatric otolaryngology. It 36 could be enhanced if all trainees are obliged to spend six months in a tertiary unit and if post-CCT fellowships were to be undertaken by all who intend to undertake DGH paediatric ENT surgery as the local lead clinician. There is a need for organised planning of these resources. If the principles of the pathways for care are agreed, then appropriate central funding of highly specialised services, together with the primary care commissioners, should allow a successful and financially viable integrated networked service. The difference between ‘high volume, low tariff’, ENT procedures and ‘low volume, high tariff ones’, needs to be recognised and the tariffs adjusted to ensure the viability of hospital services. The specialist tertiary centres will be undertaking remarkably little in the way of routine care and the low volume airway cases as well as emergency care in the under threes will need to attract much larger tariffs or the units will become unsustainable. I personally believe that there is no place for paediatric ENT services in the treatment centres (CATS/ICATS/SPMS). To meet the exacting criteria of the healthcare commission and to have facilities for safe resuscitation and emergency management and transfer, would be very costly. Current tariffs make it non-viable and would see both educational and financial destabilisation of NHS secondary care. Similarly the private sector is unlikely to wish to compete for NHS surgery on cost. The trends we have seen for a reduction in the overall numbers of tonsils and grommet surgery, across the UK, will almost inevitably continue because of financial impecunity. Whilst academic debate may still rage about the value of these operations, the prospect for the next two public spending reviews looks tight for the NHS up to perhaps 2017, and increased healthcare rationing in some form seems inevitable. This future as I have envisaged it is realistic. It would provide a structure for a safe and enhanced delivery of care to children across the whole spectrum of ENT conditions. It would allow for closely controlled audit, but has not addressed the need for paediatric ENT research in the UK. Currently, no structure exists for the choice to become a primary researcher in paediatric ENT as a career. I would love to see a future in which this deficit is addressed. That would truly enhance paediatric ENT care into the far future – the final frontier. n References 1. O’Dowd A. GP consortiums will need first class management support. BMJ 2011;342:d337 2. Health & Social Care Bill, 2010-2011 [http://www.dh.gov.uk/en/Publicationsandstatistics /Legislation/Actsandbills/index.htm] 3. National confidential enquiry into patient outcome and death, 1989. [http://www.ncepod.org.uk/1989.htm] 4. Trends in Children’s Surgery 1994-2005: Evidence From Hospital Episode Statistics Data. [http://www.bipsolutions.com/docstore/pdf/ 16070.pdf] 5. Joint statement on General Paediatric Surgery on behalf of the Association of Paediatric Anaesthetists, the Association of Surgeons of GB and Ireland, the British Association of Paediatric Surgeons, the Royal College of Paediatrics and Child Health and the Senate of Surgery for GB and Ireland, 2006. [http://www.rcseng.ac.uk/service_delivery/ children2019s-surgical-forum/ the-csf-e-newsletters/documents/ Aug%2006%20Joint%20statement%20GPS.pdf] 6. Pye JK. Survey of General Paediatric Surgery Provision in England, Wales and Northern Ireland, on behalf of the Association of Surgeons of Great Britain and Ireland. Ann R Coll Surg Engl 2008;90(3):193-7. 7. ‘Raising the Standard: a Compendium of Audit Recipes’ The Royal College of Anaesthetists [http://www.rcoa.ac.uk/index.asp?PageID=125] 8. Guidance on the Provision of Paediatric Anaesthesia Services, The Royal College of Anaesthetists 2010 [http://www.rcoa.ac.uk/docs/GPAS-Paeds.pdf] 9. Robb,P. Provision of Children’s ENT Services, 2008 [http://www.rcseng.ac.uk/service_delivery/ children2019s-surgical-forum/ the-csf-e-newsletters/documents/PJR%20Newsletter %20Childrens%20ENT%20March%2008.pdf] 10. Royal College of Surgeons of England, General paediatric surgery service provision Survey summary, 2010 [http://www.rcseng.ac.uk/publications/docs/ general-paediatric-surgery-service-provision-survey] 11. The London Specialised Children’s Services Review, 2011 NHS Commissioned. [http://www.londonhp.nhs.uk/ wp-content/uploads/2011/03/ FINAL-London-specialised-childrens-services -review-summary-guidance.pdf] 12. National Paediatric Surgery Reviews: Safe and Sustainable 2011 [http://www.specialisedservices.nhs.uk/ safeandsustainable] 13. American Society of Anaesthesiologists Physical Status Classification System [http://www.asahq.org/For-Members/ Clinical-Information/ASA-PhysicalStatus-Classification-System.aspx] ENT & audiology news | www.entandaudiologynews.com audiology matters Paediatric Audiology Guest edited by Josephine Marriage Post Newborn Hearing Screening ABR: quality assurance and the role of peer review Helping Families Accept Technology Auditory Verbal Therapy Provision in the UK Unilateral Hearing Loss in Early Childhood: what are we doing about it? Amplification Options for Mild Bilateral Hearing Loss & Unilateral Hearing Loss in Children: a literature overview Paediatric Tool Development: developing a rationale for empowering children with hearing loss In this issue... Paediatric Audiology Josephine Marriage, PhD, UCL Ear Institute, 332 Grays Inn Road, London, WC1X 8EE, UK. E: [email protected] am delighted to have been asked to edit this section on Paediatric Audiology for ENT & audiology news. The implementation of newborn hearing screening programmes (NHSP) around the world has reduced the age of diagnosis of hearing loss for children and improved potential outcomes for speech and language for these children. The fact that the UK was the first country to introduce universal coverage of newborn screening is testament to the innovative nature of its audiology base, but paediatric audiology is now at a cross roads in the UK. Screening is only the first step of the complex process of defining extent and type of hearing loss and expanding family choices for intervention. Guy Lightfoot gives a candid reflection of the pitfalls and challenges of evaluating hearing levels on which options for hearing aid amplification are based and the need for continuous training and peer-review to develop clinical skills in recording and interpreting ABR traces. While nationally-based policies and regulation are possible within an integrated healthcare system, the lessons for constantly evolving effective practice are equally important for all those engaged in assessing ABRs in infants. The benefits offered by early hearing aid fitting to provide neural stimulation from the first months of life need to be matched by exposure to meaningful, socially-relevant auditory communication. The use of hearing aids in infancy relies primarily on parents, who may be struggling to consolidate the life changes inherent with the arrival of a new baby with the devastating identification of hearing loss. Jane Madell has outlined her excellent thoughts on helping to support the use of technology by parents and other family members at this crucial time. As non-verbal responses of an infant with hearing loss are not the same as those of a typically hearing infant there is a need to support the auditory-based communication dynamic. It is an equally critical component in realising the benefits of early identification of hearing loss. Without this support David Luterman’s comment that “Screening efforts have far out stripped our habilitation efforts, leaving parents with diagnosis but without support” has a ring of truth. Parents are entitled to consider all options in finding a technique or strategy that addresses their own goals and wishes for their child. If the onus of responsibility for use of amplification is conferred on the parents, they are equally central to communication choices. Elizabeth Tyszkiewicz provides an overview of the Auditory Verbal Therapy (AVT) approach which, although widely available in other countries with early screening programmes, until recently has only been available through non-government providers in the UK. The adoption of AVT at Manchester and Birmingham Cochlear implant programmes marks an important stage in expanding choices for auditory-oral communication in the UK. One of the incidental challenges that has materialised from NHSP is the identification of unilateral hearing loss, a proportion I 38 of which will develop into bilateral hearing loss. Here, Priya Singh provides an up-to-date and comprehensive review of the known impact of unilateral hearing loss (UHL) in childhood, reviewing the impact of UHL on susceptibility to speech delay, educational underachievement and social disadvantage. This perspective is far removed from the traditional advice proffered that ‘one ear is enough’. Cherilee Rutherford then goes on to describe the developing range of amplification technologies appropriate for children with UHL emphasising that careful assessment and management consideration for each individual case is required in the same way as for bilateral hearing loss. It is intriguing to note that this collection of articles has been provided by seven people who were trained at universities in four of the five continents of the world. A global resource now exists for paediatric audiology, a necessary requirement if the challenges and complexities in this specialised area of audiology are to be faced head-on. Amplification and implantable devices are more complex, and both the human interface between technology and fundamental communication needs and the range of assessment techniques available requires constant up-skilling and new patterns of training. It is deeply unsettling therefore to report that the training of graduate audiologists in the UK is regressing back to a diagnostic science curriculum, to be combined with vision and diagnostic neurosciences delivered by means of a three-year training, in-service programme. The current economic climate and Government cuts have reduced resources within the NHS UK healthcare system, the universities and social support for deaf people to the point that recent advances in hearing healthcare are in real peril. The apparent solution to these challenges – a fundamental regression of audiology primarily to that of a diagnostic healthcare science – will not only cost more to deliver but (in terms of the actual spend required to produce graduates) it will likely generate a large pool of poorly-trained healthcare workers, possessing only low-level, generic qualifications and little currency within the international team of paediatric audiology professionals. Audiologists are autonomous practitioners for long-term management of hearing and balance impairments. These needs are not, and never will be, met solely by the fitting of a hearing aid, as will become apparent to the increasing proportion of the population with hearing loss. There is no possibility that generically-trained ‘neurosensory’ diagnosticians will work in vision science one week, diagnostic neuroscience the next and audiology the following week, and still be able to fulfil the needs of infant, teenage, adult and elderly hearing impaired people. As with many politicallycontrived schemes, this programme for the modernisation of scientific careers will be soon be found to be unfit for purpose but, for the coming decade of children born with hearing loss and their families, the future looks opaque indeed. n ENT & audiology news | www.entandaudiologynews.com Post Newborn Hearing Screening ABR: quality assurance and the role of peer review Guy Lightfoot he English Newborn Hearing Screening Programme (NHSP) began in 2001 and implementation was completed across England in March 2006. The NHSP Programme Centre then embarked upon a Quality Assurance programme to assess performance against the agreed Quality Standards.1 The Quality Assurance programme encompassed early audiological assessment, habilitation and early intervention as well as screening activity. This QA process began to identify problems with the quality of ABR assessment; some audiology departments’ standards of performing and interpreting ABR tests were so poor that there were serious risks of misdiagnosis. Indeed in some cases the ABR service had to be suspended and was only reinstated when further training, supervision and ongoing quality assurance had been implemented. These errors are usually an example of unconscious incompetence – people thinking that they know what they are doing, when they do not. This tends to arise when staff do not understand the underlying concepts of the task (were T Graham Sutton Sally Wood Correspondence English Newborn Hearing Screening Programme Clinical Group E: [email protected] Declaration of Competing Interests GL has acted as an expert witness for the plaintiff in legal cases involving misdiagnosis associated with poor ABR practice. In all cases to date, the defendant has settled out of court. never properly trained), do not update their knowledge (CPD), or do not invite external scrutiny of their work (peer review). This is often linked to managerial pressure on staff to accept new responsibilities without the necessary specialist training or support. Another contributory factor can be how often tests are performed. Too small a catchment population and / or too great a number of staff doing the work may result in an individual performing tests too infrequently to develop or maintain the required degree of competence. To address this urgent training need a series of ABR refresher days was initiated to disseminate the important principles needed to ensure good ABR practice, together with individual training visits and mentoring of some departments. These refresher days continue and are a good means for testers to check their understanding of the latest guidance and to benchmark their own standards. Figures 1 and 2 give a ‘before and after’ example of ABR waveforms from one department that had a ‘serious incident’ for a peer review system to work effectively Figure 1 practitioners must feel confident and comfortable in submitting their own results for review Figure 2 ENT & audiology news | www.entandaudiologynews.com 39 feature One important weakness of any regional peer review group is that it can be only as good as its best contributor and subsequently had NHSP specialist training and mentoring. Both cases were discharged but the child shown in Figure 1 was actually profoundly deaf. Aficionados of ABR will be able to identify the specific errors made in this example. Such cases are rare but do continue to occur. Figure 2 is an example of the same department’s current work, with unequivocal 4kHz clear responses down to discharge level. Like many other departments, this centre also routinely tests at 1kHz although this exceeds the NHSP requirement for discharge. It helps keep skills sharp for those cases that require multifrequency assessment prior to amplification. NHSP guidance for conducting and interpreting ABR tests has been updated2,3 based on certain important requirements designed to minimise the scope for serious misdiagnosis. One significant development included in these recent documents is the move away from clicks toward 4kHz tone pips as the primary stimulus upon which discharge is based. This was a move that NHSP resisted for some time because frequency-specific ABR responses are smaller than click responses and this presents a more challenging task, with greater scope for interpretive error. We did not want to compound existing errors of procedure and interpretation by making the task more difficult. However, this change has now been introduced because the skills of the majority of ABR staff have improved to the required level. An ABR discussion forum was added to the NHSP web site so that interesting and difficult cases could be submitted, ideas shared and expert opinion given. So what about peer review? A pilot scheme in which all ABR test results were reviewed at a regional (Strategic Health Authority) level has been undertaken, after first training local ABR staff to review tests to standards described in current NHSP guidance. An infrastructure including the anonymous distribution of tests to reviewers and central support with a team of national ‘expert’ reviewers was necessary. This approach did demonstrably improve practice but was clearly too expensive and unwieldy to roll out as a national model. The feedback provided by the reviewers to testers was constrained by 40 the reviewing spreadsheet used and was sometimes perceived as stark, impersonal and negative with therefore limited effectiveness in terms of a catalyst for change. A full report on this pilot is in preparation. More attractive and manageable is the establishment of regional peer review groups. Some areas have successfully used a regional peer review model for some time. There are a number of models that could be used, ranging from the independent review of every case by local peers, to the occasional group meeting over coffee to discuss ‘difficult cases’. The former is labour intensive and in practice simply doesn’t happen; the latter is probably not very effective in raising and maintaining standards. In our opinion for a peer review system to work effectively practitioners must feel confident and comfortable in submitting their own results for review. There must be systematic selection of cases for review otherwise there is a risk that only the best results will be submitted for review. There needs to be a systematic approach to the review (we have a new spreadsheet that can be used to structure the review) and individuals must be prepared to openly critique the work of other (sometimes more senior) clinicians, and we sometimes observe an understandable reluctance to do this. Other features of a peer review model include: ● rotation of reviewers with sufficient cover for holiday and sickness ● a quick (typically 48-72 hours) review turnaround time for specific cases to allow the feedback to be used in the management of that case ● use of a standard review format plus phone discussions between reviewer and tester when helpful ● regular meetings between reviewers to check and benchmark their own practice ● access to NHSP experts for disagreements, queries and periodic moderation ● annual report to include an audit and review of arrangements and results of an external moderation One important weakness of any regional peer review group is that it can be only as good as its best contributor and errors could continue to occur if poor methods or standards are considered acceptable by all participants. For this reason, all peer review groups should have both an initial training element and periodic independent external moderation. We would also like to strongly suggest there needs to be a system of formal assessment and accreditation of competence of individuals before they start routinely performing newborn ABRs. This is equally important for those performing VRA and other behavioural tests on young children. We hope that the professional bodies, DH and HEIs will move rapidly towards such a scheme. Getting the right balance between conflicting practical and technical requirements is difficult. NHSP are currently helping establish a number of regional peer review groups and will continue to learn what works and what doesn’t. Closing remarks To use a motoring analogy, the UK still has an ‘MOT’ test to check that cars are roadworthy. Many countries have abandoned this idea, arguing that it is actually more cost effective to allow accidents to happen than to pay for the infrastructure around the MOT. In pure financial terms that is probably correct. That is also what most countries have, by default, with their current postscreening ABR test services: no systematic check of performance, with occasional misdiagnoses leading to substantial financial settlements. NHSP believe that our profession can and should do better than that; we also think it is important to consider the cost to the child of a missed opportunity to optimally develop language. QA and peer review may be somewhat inconvenient and have financial implications but they are the right thing to do. This is what marks us out as professionals. n References 1. NHSP Quality Standards. Newborn Hearing Screening Programme (England) [http://hearing.screening.nhs.uk/standardsandprotocols] 2. Guidance for Auditory Brainstem Response testing in babies. Version 1.1 April 2010. Newborn Hearing Screening Programme (England) [http://hearing.screening.nhs.uk/ audiologicalassessment] 3. Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme. Version 2.5 March 2011 Newborn Hearing Screening Programme (England) [http://hearing.screening.nhs.uk/ audiologicalassessment] ENT & audiology news | www.entandaudiologynews.com Helping Families Accept Technology Jane R Madell, PhD, CCC A/SLP, ABA, LSLS Cert AVT, Director, Paediatric Audiology Consulting, Correspondence 450 West 23rd Street New York, NY 10011 E: Jane@ JaneMadell.com W ith the technology that is currently available, almost every child should be able to hear sufficiently to use audition to develop listening and spoken language. However, children will only be able to use audition to develop spoken language if they wear the technology which means that parents have to under- stand and accept the need for full time use of technology. Why then, do parents have difficulty accepting technology? Declaration of Competing Interests None declared. It is not really about the technology. Today’s parents are comfortable with technology – they use cell phones, computers and so on. But hearing aids are different. Accepting hearing aids means accepting the hearing loss. To succeed in getting kids to use hearing aids full time, families must accept the hearing loss. For each family, we need to know what is interfering with the family accepting the hearing loss. Why can some families move ahead through their sadness and fit hearing aids and start therapy, while other families seem paralysed and cannot move ahead. While there is a little time to spare, we need to get hearing aids on relatively quickly to help children become auditory learners and develop an auditory brain. Our ability to assist parents in accepting hearing loss is closely tied to how we, as clinicians, feel about hearing loss. Although I feel sad for the family when I diagnose a hearing loss, I am very optimistic about what is possible for a child identified with hearing loss in 2011. I know technology available today will enable virtually every child to hear and learn, especially when paired with auditory based early intervention services. Part of counselling families is passing on optimism about the future of children with hearing loss. If we are not optimistic, we do families a disservice. The first reason is, because we will not provide them with the hope to move on. Secondly, we may not push as hard as we might to be sure ENT & audiology news | www.entandaudiologynews.com 41 feature Children will only be able to use audition to develop spoken language if they wear the technology which means that parents have to buy into the need for full time use of technology kids are hearing as well as they can, accepting ‘good enough’ in place of ‘excellent’. We can become more optimistic by getting to know families who have kids who are successful, by talking to and listening to kids who have had the advantage of current technology, by meeting today’s young adults, who did not have the advantage of current technology, and are still successful. The more optimistic we are, the more optimistic we can help families become. therapists in understanding performance and planning remediation. Keeping the technology on the head Parents need to believe that technology will help Parents need to understand that the ears are the pathway to the brain, and that audition is critical for auditory brain development which results in both spoken language and academic learning. They need to understand that children learn language by overhearing. Unless they are hearing soft speech all day long, they are going to miss out on incidental learning. They need to know that auditory access leads to reading. Parents are anxious that their children should be able to fulfill their potential and be successful. Helping them understand the relationship between hearing, language, reading and learning is critical in helping them understand the importance of wearing technology on a full time basis.1 The technology needs to be working optimally The most encouraging thing for a parent is seeing the child respond to sound. On the other hand, if a child does not seem to be responding, it is difficult for parents to fight with children to keep the equipment on. When a parent first sees a child alert, turn to sound, or search for a sound, they feel hopeful – “maybe this will be okay,” and they can work on being sure the technology is kept on. How do we know if technology is working optimally? It is not guesswork. We need to test. With tiny ones, we start off with an ABR, but we need to move to behavioural testing quickly. ABR cannot 42 be used to monitor hearing aid performance but behavioural testing can. Behavioural observation audiometry (BOA) can be used to test infants under six months of age,2,3 VRA and Conditioned Play can be used for older children. Obtaining aided thresholds and detection levels for the Ling sounds with hearing aids will let us, and parents, know if the hearing aid is providing sufficient gain throughout the frequency range. Moeller et al. (2010)4 have reported that 40% of children are not receiving sufficient gain from their hearing aids. Real Ear testing tells us what is reaching the ear drum, but not what is reaching the auditory brain. While Real Ear testing is critical, it is not sufficient. Aided thresholds can be accurately obtained by starting below threshold and presenting short duration warble tones or noise bands. This should not set off compression and should provide reliable results. If the child is not hearing softly enough, hearing aid settings can be adjusted to provide sufficient hearing to receive all auditory information. Once a child is old enough, speech perception testing should be added to the test protocol at normal and soft conversational levels in quiet and in competing noise.5 This information is useful both for parents and for Parents sometimes have difficulty keeping technology on. It is important to know why in order to make the appropriate suggestions. Children sense their parent’s discomfort and may remove hearing aids for that reason. Parents need to understand that they must give a very clear message to their children indicating that the hearing aids may not be removed. If the child takes it off, it has to go right back on. Children should be using the hearing aids full time after two weeks. Parents need to understand that it will be a difficult two weeks but once accomplished, the child is on his / her way. Keeping the child entertained helps. Children are less likely to take technology off while they are being read to and more likely to remove it when alone in the back seat of the car (FM can help in that situation). A bonnet covering the ears and tied under the chin will help in keeping hearing aids on during the early weeks. Being optimistic about what is possible for children with hearing loss, will help parents be optimistic. Our job is to keep them from giving up. n References 1. Robertson L. Literacy and Deafness: Listening and Spoken Language. Plural Publishing; San Diego: 2009. 2. Madell JR. Using Behavioral Observation Audiometry to Evaluate Hearing in Infants from Birth to 6 Months. In Pediatric Audiology: Diagnosis, Technology, and Management. Edited by Madell JR, Flexer C. New York; Thieme: 2008. 3. Madell J. Behavioral evaluation of hearing in babies: Yes, you can! AudiologyOnline Recorded Course 12919, eLearning library; 2009. [http://www.audiologyonline.com] 4. Moeller PM. Outcomes of children with Hearing Loss. ASHA Conference, Boston; 2010. 5. Madell JR. Evaluation of Speech Perception in Infants and Children. In Pediatric Audiology: Diagnosis, Technology, and Management. Edited by Madell JR, Flexer C. New York; Thieme: 2008. ENT & audiology news | www.entandaudiologynews.com Auditory Verbal Therapy Provision in the UK Elizabeth Tyszkiewicz, Auditory Verbal Therapist. Correspondence Children's Service, Midlands Hearing Implant Programme, Optegra Building, Aston University, Birmingham, B4 7ET, UK. E: Elizabeth.Tyszkiewicz @bch.nhs.uk A uditory verbal therapy, is “an individual, auditory, developmental programme, implemented by the child’s family in close collaboration with a therapist, with the goal of achieving age appropriate spoken language ability, and full social partici- pation throughout childhood and beyond.” (AVUK, 2003). It has particularly urgent rele- vance now, as NHSP and early amplification fitting presents support services with what Carol Flexer, Distinguished Professor Emeritus, University of Akron and Northeast Ohio AuD Consortium (NOAC), calls ‘a new paradigm’: “Rapid infant brain growth requires prompt intervention, typically including amplification Declaration of Competing Interests None declared. and a program to promote auditory skill development. In the absence of sound, the brain reorganises itself to receive input from other senses, primarily vision: …this reduces auditory neural capacity.” (Carol Flexer, 2011). The parent is at the centre of each AVT session (photo by kind permission of Auditory Verbal UK). AVT is labour intensive, requiring whole-family commitment and active participation Auditory Verbal Therapy (AVT) practice developed in North America, and is well established in Australia. It increasingly informs mainstream thinking about service provision for children with early-identified hearing impairment in UK and Europe, as well as many other countries around the world. AVTs are licensed practitioners in Audiology, Speech and Language Therapy or Education, who have undergone a three year post-qualification training and mentoring programme. The knowledge base for this programme of study encompasses a range of domains, including parent guidance, education and support, child development, spoken language communication, auditory functioning, hearing and hearing technology, strategies for listening and spoken language development. A key element of the practice is the provision of regular, individual hour-long parent and child sessions, until the child is shown to have attained age-appropriate spoken language ability, or makes the transition to another, more appropriate programme. The approach is founded on the principle that language is learned by all children from the main carers, usually the parents. The therapist provides an intensive, individual coaching programme, through which they gradually gain confidence and expertise to become the main ‘agent of change’ for the listening and spoken language development of their child with impaired hearing. Families who research options when they find out their child has impaired hearing often identify AVT as relevant to them, but have difficulty in finding a suitably qualified ENT & audiology news | www.entandaudiologynews.com 43 feature therapist. Until recently, the limited resources available in the UK were all in the independent sector, either as private practitioners, or within voluntary sector organisations. Now, two of the Cochlear Implant programmes in the UK offer follow-up from Certified AVTs – others employ therapists who have an interest, or have undergone training in AVT. There is considerable interest from both specialist educators, and speech language therapy services, as they are increasingly asked to provide for the children identified via the NHSP. However, there is still no therapy offered in NHS departments which fit non-implantable hearing technology, and which cater for the majority of children with permanent hearing impairment in the UK. In Auditory Verbal Therapy, an informed choice to ‘opt in’ is involved, from the family’s and the therapist’s point of view. You don’t find yourself in an AVT programme by default, or by accident. Especially in the early years, AVT is labour intensive, requiring whole-family commitment and active participation. It sets clear short and long-term goals for the child, and constantly generates new diagnostic information which must be incorporated into day to day living. The child’s family must regularly attend AVT sessions, and also, inform and enlist the support of the wider team, working to ensure the child has access to mainstream education. People often imagine there is ‘homework’ for the parents. In fact, it is more a matter of learning to interact with the child at all times in a way that stimulates the development of the ‘auditory brain’ through spoken language embedded in play, problem solving, and the most apparently mundane activities of daily living. It takes far more energy than the already considerable demands of bringing up young children. For the therapist, the programme means hour-long, packed and very detailed sessions, tailored to the learning needs of the specific family and child. Although good therapy is often mistaken by observers for a relaxed and playful time, each session contains careful planning, goal-setting, and targeted activities. Regular individual assessment and reporting are crucial, and there is hands-on coaching for parents throughout the session. The emphasis is strongly on audition, with a consequent need to monitor / maintain technology, feeding back immediately to audiologist or ENT specialist when hearing is not optimal. Each session yields diagnostic information which must be turned into action to be implemented. The therapist must also inform, 44 and enlist the support of the team around each child in the school / local area. At the early stages, the therapist, working against the adults’ natural belief that information must be made visually easy for the child, has to make changes in the learning environment and ‘kickstart’ the neural connections between sound and meaning. It is crucial that the family feels successful, and sees progress as a result of their effort. Sometimes the steps are very small, and the therapist has a crucial role in helping them to observe changes in their child’s learning. All the above are in conflict with trends in public sector service delivery, which are away from individually tailored care, and towards generic ‘advisory’ programmes, or group-based interventions. You cannot ‘grow’ an early developing auditory brain in a group. If true spoken language competence is to emerge, the child needs an optimised version of the opportunities given to the typically hearing newborn: individual, highly audible, and exactly tailored to move the learning on to the next developmental stage. Where there is no hearing difficulty, parents provide all this without a second thought. In the presence of hearing impairment, and using hearing technology, AVTs are there to map the route, support the process, and measure the outcome at regular intervals to ensure learning is on track. Within an NHS service such as the Midlands Hearing Implant Programme Children’s Service in Birmingham, the caseload is extremely diverse, in terms of social circumstances, hearing history, and the presence of complicating factors in addition to hearing impairment. While families make an informed choice to have a cochlear implant for their child, they may not wish, or be able, to opt into an intensive AVT follow-up programme. Other family pressures or social needs may come first, or, indeed, they may have other goals for their child than those defined by AVT, which aims for fluent spoken communication and mainstream schooling. Flexibility and adaptability are also key features of AVT practice in the course of the diagnostic assessment. Hearing impairment doesn’t mean there are no other complications which interfere with learning. It may be necessary for the AVT practitioner to become the ‘hearing adviser’ on a multidisciplinary team which addresses the child’s predominant needs. A great strength of AVT is its rigorous use of ongoing diagnostic observation and formal assessment in the domains of audition, speech, language, cognition and social interaction. There is very little ‘watchful waiting’ in AVT practice. The clear understanding of each child’s latent auditory potential, and of the typical developmental trajectory, leads to active investigation of the reasons why milestones are not reached in a timely fashion. Sadly, there is inequity of access to services, not only because certified therapists are still rare (totalling approximately ten in the UK) but because some people now believe that AVT is ‘only for children with cochlear implants’, as this is the group that most readily gains access to AVT services in the public sector. Ideally all children with impaired hearing would be offered the strong diagnostic benefits of AVT to optimise use of hearing technology, inform management, and identify complicating factors. If paediatric hearing aid departments had a staff AVT, a missing piece of the NHSP puzzle might be slotted into place: intervention would begin on the day of amplification fitting, and dovetail smoothly with follow-up from the local multidisciplinary team. The need for quality intervention to ensure that children with impaired hearing fulfil their true listening and speaking potential has colossal training implications. The ‘paradigm change’ in the last few years means that not enough people know how to harness the tremendous human potential of the next generation of young children with impaired hearing, or what it is reasonable to expect from them and from their families. On the other hand, there is a groundswell of interest and initiative which bodes well for the future: some trainee audiologists receive a grounding in the theory and practice of Auditory Verbal Therapy, special interest groups (SIGs) meet regularly to learn and network, short courses and training days are offered in the public and voluntary sectors, web based resources are easily accessible (see references below), and a Post-Graduate Diploma course is offered at Aston University in Birmingham. n References Dornan D, Hickson L, Murdoch B, Houston T, Constantinescu G. Is Auditory-Verbal Therapy Effective for Children with Hearing Loss? The Volta Review (Fall) 2010;110(3):361-87. Goldberg DM, Flexer C. Auditory-verbal graduates: outcome survey of clinical efficacy. J Am Acad Audiol 2001;12(8):406-14. Hogan S, Stokes J, White C, Tyszkiewicz E, Woolgar A. An evaluation of Auditory Verbal therapy using the rate of early language development as an outcome measure. Deafness Educ Int 2008;10(3):143-67. Alexander Graham Bell Association for the Deaf and Hard of Hearing [www.agbell.org] Auditory Verbal UK [www.avuk.org] Equal Voice for Deaf Children [www.evdcweb.org] ENT & audiology news | www.entandaudiologynews.com Unilateral Hearing Loss in Early Childhood: what are we doing about it? Priya Singh, AuD, Senior Clinical Lecturer, Correspondence UCL Ear Institute, 332 Grays Inn Road, London, WC1X 8EE, UK. E: [email protected] T he impact of unilateral hearing loss (UHL) on children was recognised and reported by Bess, Tharpe and colleagues in 1986. Since then, evidence has continued to emerge to change the popularly held misconception that UHL is a benign condition that requires little more than identification. The introduction of newborn hearing screening programs (NHSP) has allowed cases of UHL to be identified Declaration of Competing Interests None declared. earlier in life than was previously the case. However, there are no recommendations for specified early intervention for these children. Management therefore varies from one centre to the next, from no intervention to amplification with or without support services. The purpose of this review is to remind ourselves of what we know, what we don’t know and to ensure that we make best use of the early identification to start management as soon as possible. Benefits of binaural hearing Research has enlightened thinking on the benefits of binaural hearing in all listening environments. Studies on binaural listening and amplification have shown favourable effects from binaural summation on speech understanding and ease of listening. Studies on the head shadow effect have demonstrated that children with UHL had poorer speech discrimination scores than normal hearing peers regardless of which ear the signal and noise were directed at (that is, monaural direct or indirect). This has implications for the child in a classroom, or kindergarten, from poor signal to noise ratios, even when the child has preferential seating arrangements. It is known that children with UHL require a higher signal to noise ratio than adults when listening to speech in noise. When assessing the impact of the squelch effect, it is necessary to look specifically at noise effects in children as they are less efficient in suppressing background over the primary signal in order to improve speech perception. When the negative impact of background noise is coupled with reverberation and poor classroom acoustics as is the commonest context for learning speech, language or academic skills, it is clear that there are good theoretical reasons for there being a functional negative impact from UHL in these areas of childhood learning. Howard et al. (2010) measured listening effort using a dual task paradigm in a typical classroom and found that while children were able to maintain performance on the primary task, the higher listening effort required greater cognitive resources, and that performance decreased on the secondary task. Tharpe’s studies on dual task paradigm (2002) found that even children with normal hearing had difficulties with these tasks. Impact of UHL on speech and language development, and educational achievement The literature on speech and language development shows mixed outcomes, with some studies reporting delay in speech and language development in children with UHL and others not. A number of studies showing disadvantage experienced by this population are given in Table 1 below. The majority of the studies were on school aged children, and it is important to note that in most it is not known whether the hearing losses were congenital or acquired or whether pre- or postlingually acquired, which may impact on the findings. Further, these early studies were conducted before the introduction of newborn hearing screening, implying later identification rather than late onset of the hearing loss. children with UHL require a higher signal to noise ratio than adults when listening to speech in noise ENT & audiology news | www.entandaudiologynews.com 45 feature Table 1. Studies looking at speech and language development in UHL vs normal hearing children. Study and Year Severity of UHL Number of subjects Speech / Language Problems Klee & Davis-Dansky (1986) >= 40dB 25 Few differences on 6 standardised language subtests (6-13 years) Borg et al. (2002) >= 20dB 58 Delayed language development Kiese-Himmel (2002) >= 30dB 31 Normal first words but delayed acquisition of 2-word phrases Colorado HIP (2002) Varied 15 Significant or borderline language delay in 33% Colorado Study II (2003) 30-120dBHL 155 8% received speech and language therapy, and in total 53% had other interventions Table 2. Impact of UHL on education and academic development. Study and year Severity of UHL Number Educational Findings Bess and Tharpe (1986) >= 45dB HL 60 51% satisfactory, 35% repeated a grade, 13% needed assistance Bovo et al. (1988) Profound 115 22% failed a grade, 12% needed assistance Oyler et al. (1988) >= 25dB HL 54 27% failed a grade, 41% needed assistance Watier-Launey et al. (1998)) >= 20dBHL 175 40.4% repeated a grade compared to 16% in general population Hallmo et al. (1986) >= 20dB HL 56 School results & linguistic development normal (NB: no testable or repeatable measures used, no comparison group) One of the challenges in evaluating the literature with respect to UHL in children is the lack of a universally accepted definition Table 2 above summarises the findings of studies regarding the impact on educational and academic outcomes for children with UHL. Many of these studies show that a significant proportion of the children with UHL repeated a grade, with an additional number requiring additional educational support. The failure rate at school in the Bess and Tharpe (1986) study found that children with UHL were 10 times greater than the failure rate of the general school population, which was only 3.5%. Waber-Launay et al. (1998) had a larger number of children in their study and had a similar finding, with 40% of the children with UHL failing a grade compared to 16% of the normal hearing population. It is important to note that in the majority of the studies listed in Tables 1 and 2, the findings were similar irrespective of the degree of UHL, thus suggesting that even mild unilateral loss can have a functional impact on achievement and should not be ignored. This may be shown to relate to reduced neural inhibition within the brainstem pathways, thereby changing the input to the ‘normal hearing’ ear, in future physiological research. 46 Behaviour at school and quality of life considerations In addition to educational achievement, behaviour at school is another factor for children with UHL. Brookhauser (1991), in a five year study of 391 children with UHL, found that 59% had academic and behavioural problems. Behavioural issues in school were also noted by Bess and Tharpe (1984) and Stein (1983) who reported that 24% of the group of 19 had behavioural problems in spite of adequate grades. These issues could be related to frustration and fatigue created by the concentration level necessary for UHL children in poor listening conditions, resulting in a lack of co-operation and perceived inattention at school. If we are to be truly comprehensive in the assessment and management of children with UHL, then their mental well-being must also be considered. Studies on children with bilateral hearing loss demonstrated lower scores on tests which assess quality of life (QoL) than normal hearing individuals. The negative emotional and social impact of UHL in adults has been documented. At present there is limited data available regarding the QoL in children with UHL, which requires further investigation and consideration. Co-exisiting intrinsic and extrinsic factors UHL has not been fully explored with respect to other co-existing factors like cognitive deficits, developmental disabilities, and demographic factors such as socioeconomic status, level of language stimulation at home, child care quality and common childhood diseases like otitis media with effusion (OME). This co-existence may create a negative synergy, and these children are at greater risk for speech and language delays during the critical period of development, which then impacts on educational progress. Challenges in evaluating the evidence base One of the challenges in evaluating the literature with respect to UHL in children is the lack of a universally accepted definition. Bess et al. (1998) provided a definition which was adopted by the CDC in 2005 but there are a number of other definitions used in research for example Niskar et al. ENT & audiology news | www.entandaudiologynews.com feature (1998) and BAAP (2009). These differences probably contribute to the high variability in the prevalence figures for children with UHL (Ross et al., 2010). From an estimation of 1 to 3 per 1,000 newborns and 1 to 5% of school aged children having UHL, Davis (2005) reports a prevalence of 0.7/1,000 identified with UHL in the NHSP. It must be noted however that there is the possibility of underestimation of true prevalence figures not solely due to definitions, but also the types of losses included in these figures, poor follow-up rates, acquired aetiologies and newborn screening programs not being specifically designed to identify UHL. It is important to recognise the importance of new skills in applying appropriate masking in follow-up testing with both objective and behavioural testing. In addition, late identification has led to causes of the hearing loss not being fully investigated, thus making it difficult to know whether the losses are congenital or acquired and, more importantly, which losses are likely to deteriorate or progress to bilateral hearing loss. Clinical strategy for children with UHL With newborn hearing screening programmes (NHSP) identifying UHL in early life and in greater numbers than was the case a decade ago, audiology teams are in a better position to intervene early on. Full evaluation of the aetiology of UHL ensures that risk of progression to bilateral HL is identified, and can be addressed in the treatment planning. This allows parents to be part of the decision-making and management for their children to minimise speech / language and education delays. There needs to be ongoing audiological assessment to monitor progressive and fluctuating losses and to identify cases that may become bilateral as soon as possible, usually associated with enlarged vestibular aqueduct syndrome (EVAS). It seems that early neural stimulation of the auditory system through both ears applies just as much to UHL as to bilateral HL. Full and effective masking to define true hearing levels in the impaired ear, are critical in deciding on the potential benefit of amplification, which has no benefit on a profoundly deaf ear. Management options, including amplification, acoustic modifications and FM systems, must all be considered and trialled where appropriate. Amplification trials should not be based merely on degree of loss and speech perception scores, but quality of life as well. Appropriately high expectations can play a very important role and as such the intermittent use of amplification may have to be considered if there is the potential for improving academic and QoL outcomes for these children in certain situations. In addition, functional auditory assessments for both the child and the parents can contribute valuable information for rehabilitation, together with speech / language assessment and monitoring. Even if we are uncertain of the benefits of the intervention strategy, we are obligated to consider them and evaluate outcomes before they are discounted, ensuring that informed choices are being made for the child with the family. A study conducted by the NHSP team in the UK (Bears study) recognised that the impact of diagnosis of UHL in early life could be just as devastating for families as for those with bilateral hearing loss, which lead into a structured intervention plan. Borg E, Risberg A, McAllister B, Undemar BM, Edquist G, Reinholdson AC, Wiking-Johnsson A, WillstedtSvensson U. Language development in hearing-impaired children. Establishment of a reference material for a 'Language test for hearing impaired children. Int J Pediatr Otorhinolaryngol 2002;65(1):15-26. Conclusion McKay S. To aid or not to aid: children with unilateral hearing loss; 2002. [http://www.audiologyonline.com/ articles/pf_article_detail.asp?article_id=357] The negative impact of UHL on children’s speech and language development and academic outcomes is now well recognised. With the NHSP detecting cases of UHL in early life, there is a clear need to define the population, to identify risk factors for speech, language, educational and social delay in UHL and to establish consistency, timely and effective management for each case aiming to reduce the gap between detection and appropriate intervention. The management plan starts at diagnosis and continues into school and beyond. We have an obligation to contribute to policy across audiology teams and to be the advocates for these children and families, improving their QoL and levelling the playing field for equal opportunities and choices into adulthood. The current evidence base for UHL is clear; UHL is not benign and therefore should not be treated as such. Our approach to management of UHL in children may be expressed by a quotation by Michaelangelo: “The greater danger for most of us lies not in setting our aim too high and falling short; but in setting our aim too low, and achieving our mark.”n Further reading Davis A, Devoe S, Robertson H. UK Trial of Early amplification in children with unilateral or mild hearing impairments. National workshop on mild and unilateral hearing loss, Colorado, CDC and Marion Downs Centre; 2005. Watier-Launcey C, Soin C, Manceau A, Ployet MJ. Necessity of auditory and academic supervision in patients with unilateral hearing disorders: retrospective study of 175 children. Ann Otolaryngol Chir Cervicofac 1998;115(3):149-55. Ross DS, Visser SN, Holstrum WJ, Qin T, Kenneson A. Highly variable population-based prevalence rates of unilateral hearing loss after the application of common sense definitions. Ear Hear 2010;31(1):126-33. ENT & audiology news | www.entandaudiologynews.com Fitzpatrick EM, Durieux-Smith A, Whittingham J. Clinical practice for children with mild bilateral and unilateral hearing loss. Ear Hear 2010;31(3):392-400. Bess FH, Tharpe AM. Case history data on unilaterally hearing-impaired children. Ear Hear 1986;7(1): 20-6. Lieu JE, Tye-Murray N, Karzon RK, Piccirillo JF. Unilateral hearing loss is associated with worse speech-language scores in children. Pediatrics 2010;125(6):1348-55. Kiese-Himmel C. Unilateral sensorineural hearing impairment in childhood: analysis of 31 consecutive cases. Int J Audiol 2002;41(1): 57-63. Lieu JE. Speech-Language and educational consequences of unilateral hearing loss in children. Arch Otolaryngol Head Neck Surg 2004;130(5):524-30. Lieu JE. Children with unilateral hearing loss. Semin Hear 2010;31(4):275-88. Kenworthy OT, Klee T, Tharpe AM. Speech recognition ability of children with unilateral sensorineural hearing loss as a function of amplification, speech stimuli and listening condition. Ear Hear 1990;11(4):264-70. Brookhauser PE, Worthington DW, Kelly WJ. Unilateral hearing loss in children. Laryngoscope 1991;101(12, part 1):1264-72. Oyler RF, A. Oyler AL, Matkin ND. Unilateral hearing loss: demographics and educational impact. Language, Speech and Hearing Services in Schools 1988;19:201-10. Bovo R, Martini A, Agnoletto M, Beghi A, Carmignoto D, Milani M, Zangaglia AM. Auditory and academic performance of children with unilateral hearing loss. Scand Audiol Suppl 1988;30:71-4. Borton SA, Mauze E, Lieu JE. Quality of life in children with unilateral hearing loss: a pilot study. Am J Audiol 2010;19(1):61-72. Stein DM. Psychosocial characteristics of school-age children with unilateral hearing losses. J Acad Rehabil Audiol 1983;16:12-22. Klee TM, Davis-Dansky E. A comparison of unilaterally hearing-impaired children and normal hearing children on a battery of standardized language tests. Ear Hear 1986;7(1):27-37. Tharpe AM. Minimal hearing loss in children: the facts and the fiction. Sound foundation through early amplification, Chicago; 2007. Tharpe AM. Unilateral hearing loss in children: a mountain or a molehill? Hear J 2007;60(7):10-17. Tharpe AM. Unilateral and mild bilateral hearing loss in children: past and current perspectives. Trends Amplif 2008;12(1):7-15. Bess FH, Tharpe AM. Unilateral hearing impairment in children. Pediatrics 1984;74:206-16. Holstrum WJ, Gaffney M, Gravel JS, Oyler RF, Ross S. Early intervention for children with unilateral and mild bilateral degrees of hearing loss. Trends Amplif 2008;12(1):35-41. Yoshinaga-Itano C. Johnson CD, Carpenter K, Brown AS. Outcomes of children with mild bilateral hearing loss and unilateral hearing loss. Semin Hear 2008;29(2):196-210. 47 Amplification Options for Mild Bilateral & Unilateral Hearing Loss in Children: a literature overview Cherilee Rutherford, AuD, Lecturer and Director of the MSc Advanced Audiology & Audiology Short Courses. Correspondence The Ear Institute, University College London, 332 Gray’s Inn Road, London, WC1X 8EE, UK. E: cherilee.rutherford@ ucl.ac.uk Declaration of Competing Interests None declared. T he past few years has shown a growing interest from professionals in the diagnosis and management of children with minimal hearing loss, including mild bilateral (MBHL) and unilateral hearing loss (UHL). In addition to moderate and more severe losses, MBHL and UHL are sometimes identified through universal newborn hearing screening programs, but more often these type of hearing losses are identified later during the school age years. The impact of moderate and severeprofound hearing loss on language, communication and academic development is well documented and subsequent management in terms of amplification, parental guidance, and early communication intervention is well established. The management involved with MBHL and UHL is less clear and many questions remain unanswered. In 2005 the Centre for Disease Control and the Marion Downs Hearing Centre hosted a national workshop on mild and unilateral hearing loss to consolidate current knowledge regarding prevalence, screening, diagnosis, amplification, outcomes, and early intervention. This review will attempt to (1) highlight the differences between MBHL, UHL, and single-sided deafness (SDD) and, (2) summarise the amplification options as part of the holistic treatment approach for children with MBHL or UHL. Definitions of MBHL, UHL & SDD Historically there has been no consensus on the definitions of MBHL and UHL and terms like ‘slight’, ‘mild’, and ‘minimal’ were used to describe a variety of permanent, sensorineural, conductive, and unilateral hearing losses ranging from 16-45dB HL. For the purpose of this review the definitions of MBHL and UHL will be used as proposed by the 2005 CDC National Workshop: MBHL: “Calculated or predicted average pure tone air conduction (PTA) thresholds for both ears at 500Hz, 1kHz, 2kHz between 20 and 40dB HL or PTA thresholds greater than 25dB HL at two or more frequencies above 2kHz (that is, 3, 4, 6, 8kHz)”. UHL: “Calculated or predicted average PTA threshold in the low frequencies (500Hz, 1kHz, and 2kHz) of any level greater than 20dB HL, or, in the high frequencies, PTA thresholds greater than 25dB HL at two or more frequencies above 2,000Hz in the affected ear. PTA average thresholds in the good ear should be less than or equal to 15dB HL”. Ross et al. (2008)1 described terms such as ‘mild’, ‘slight’, ‘minimal’ as confusing and indicated that the terminology could be misleading as to how serious the effects of these types of hearing losses potentially could be. Single-sided deafness (SDD): Sometimes the terms UHL and SDD are used interchangeably and again these can be confusing. Although there is currently no clinical consensus, the more typical reference to SDD is in the context of permanent, severe-to-profound sensorineural hearing loss in the poor ear, with normal hearing in the other ear.2,3 Hearing aids Hearing aid fitting to children with MBHL presents parents and clinicians with quite a few dilemmas. Does the benefit of amplification outweigh the cost associated with ...terms such as ‘mild’, ‘slight’, ‘minimal’ as confusing and indicated that the terminology could be misleading as to how serious the effects of these types of hearing losses potentially could be 48 ENT & audiology news | www.entandaudiologynews.com feature it? Should children be fitted monaurally or binaurally? What type of ear mould coupling is preferred and which dynamic features are proven to deliver the best outcomes? Would non-amplification be detrimental to their development and when is the best time to provide hearing aids? More importantly, what are the benefits of amplification for this group? Davis, Reeve & Hind (2001)4 suggested that benefit assessments for amplification should include acceptance of amplification, use of amplification, development of speech, language and communication, quality of life for the child, and quality of family life. These are questions that clinicians are faced with in everyday practice and unfortunately the evidence base is currently not yet there to support decision making. While the clinical community is awaiting the results of research efforts in this field, clinicians are encouraged to follow existing guidelines (American Academy of Audiology Paediatric Amplification Protocol, 2003; JCIH 2007 Position Statement)5,6 until more evidence becomes available. McKay, Gravel, & Tharpe (2008)7 suggested that the lack of acoustic stimulation could lead to auditory deprivation. Children who have been wearing amplification for longer were found to have better speech recognition abilities. They propose that, in a conservative effort to reduce poor speech recognition in the future, hearing aids should be considered and offered to children with MBHL or UHL. Following current best practice guidelines, this means that a binaural fitting would be recommended for MBHL patients. According to the American Academy of Audiology Pediatric Amplification Protocol (2003),5 children with MBHL should be considered for amplification and / or FM systems. Decisions about trialling hearing aids and / or FM systems should be made with the family and child’s preferences in mind, and considering the child’s educational and communicative development. When the decision has been made to fit hearing aids, standard good practice guidelines should be followed, including the use of probe microphone measures and existing prescriptive methods such as the Desired Sensation Level (DSL i/o). Several input levels should be measured to prevent over-amplification in this population. Probe microphone measures should also be performed at every appointment to accommodate the child’s growing ear canal that could influence gain settings on the instrument. RECD’s should be used when possible. When a child rejects the use of hearing aids, it may be that some acoustic cues have been reduced by the fitting of a full shell earmould. Open fittings should be considered to maintain the natural quality of the child’s own voice perception, although an external vent may be more suitable for infants with small ear canals. McKay, Gravel, & Tharpe (2008)7 provided these practical guidelines on hearing aid features and settings for children with MBHL: 1. Hearing instrument features need to be considered for each patient individually. 2. Feedback cancellation may not be necessary due to limited gain settings, but it may be required in certain conditions such as when the child is wearing a hat or lying down. 3. Other features such as multiple memories and directional microphones should be considered in light of the child’s ability to manipulate the controls and choosing the correct situation to make the adjustment in. The use of automatic microphone switching could be beneficial. Alternatively, the hearing aid could be set to directional mode during instruction time. Tharpe, Eiten & Abbot Gabbard (2008)8 suggested further that: 1. Linear amplification with low threshold compression could be appropriate on the basis that children with MBHL have a larger dynamic range of hearing so WDRC is not needed. 2. Omni-directional microphone setting should be used for infants / toddlers who are mostly in the home environment perhaps with auto switching for schoolage children with the option to manually override. 3. Clinicians should carefully consider the internal noise floor in certain hearing aids that may interfere with speech perception. The issue of binaural interference (poorer bilateral performance with asymmetric auditory input) may be a problem in children with UHL who have been provided with a hearing aid on the poor ear and therefore should be monitored closely by the clinician. FM systems FM is a type of wireless technology that helps with speech understanding in noise. The person speaking typically wears a transmitter microphone and speech signals are then sent directly to the listener via harmless radio waves to an FM receiver which the user wears as part of a small behind-the-ear hearing aid. It is well known that children with HI need a much better signal to noise ratio (SNR) than normally hearing (NH) children ENT & audiology news | www.entandaudiologynews.com for optimum speech understanding in background noise. FM systems provide the best improvement in SNR (perhaps as much as 12-18dB) over directional hearing aid microphones (Hawkins, 1984).9 As a general guiding principle, Hawkins recommended that HI children in mainstream education should have the best sound quality from amplification and SNR as reverberation times and SNR in typical classrooms are often outside recommended optimal levels. There is a common perception among professionals that FM use is reserved for more severe-profound hearing losses and that the high cost of personal FM systems often discourage patients from making use of it. Perceptions like these need to be challenged by evidence about the real world benefits of FM use in children with milder losses. A combination of speech perception scores, functional measures, and survey data could be used to demonstrate the efficacy of such an approach to amplification. When should an FM system be offered to a child with MBHL? Tharpe, Eiten & Abbot Gabbard (2008)8 suggested that candidacy be considered when a child performs more poorly than normative data in speech in noise tasks, struggles academically, shows listening fatigue, or struggles to listen in noisy situations. This decision needs to be made while taking the parents’, teacher’s and, importantly, the child’s views into account. A monaural FM fitting configuration is recommended, leaving the other ear unoccluded and free for surround listening when the speaker is not using a microphone.8 Hawkins (1984)9 recommended that the teacher’s transmitter microphone should be set to directional mode as the standard configuration (this would lead to an additional 3dB advantage). McKay et al. (2008)7 pointed out that sound level FM / infrared devices could potentially benefit all children in a classroom (HI and NH), as well as the teacher (less vocal strain), but it might not be sufficient for the HI child who may still require an ear level FM system in addition. It is recommended that this be provided on a trial basis and that an assessment tool such as the SIFTER is used to monitor functional listening. Tharpe & Bess (1999)10 outlined some of the advantages of soundfield FM: (1) The child is not being isolated and labelled as hearing impaired; (2) Reasonable cost – ear level FM systems are more expensive than soundfield systems; (3) Normal hearing children could also benefit from SNR improvements. For infants and toddlers there is currently no evidence to suggest benefit from FM systems, but theoretically they should benefit from an improved SNR. Guidance 49 feature should be provided on when to use FM with infants & toddlers, for example in the stroller, in the car, in day care or preschool during instruction time.7 CROS aids, Bone anchored solutions and Transcranial amplification CROS aids may be considered as an option for older children with SDD as they are more able than younger children to monitor their environment, change their head position, and change settings on the device to accommodate changes in the listening environment.3 Limited outcome data exist for the use of bone anchored devices in children. The two studies found in the literature had typically small sample sizes, and reported outcomes for mostly teenage children. The data, however small, does seem to indicate some benefit for users post-fitting with increases in speech performance in noise and listening abilities.11,12 Transcranial devices like Transear™ and Transcranial CROS work on the principle of providing stimulation to the better cochlea through placement on / in the poorer ear using a BTE, ITE or CIC device. Again, these technologies are showing some promise but currently have very limited outcome data to support an evidence-based recommendation to parents of children with SDD. The interested reader may refer to www.transear.com and Valente & Oeding (2010)13 for a more detailed review on these devices. Cochlear implants Some preliminary data for cochlear implants as a treatment option for SDD are starting to emerge,14-16 but these are based focus groups of parents indicated they want to know as soon as possible of any HI, regardless of the degree of loss 50 on adult patients and predictions about the applicability to the paediatric population may be premature. In the UK, this approach is not currently supported by the NICE (National Institute of Clinical Excellence) guidelines, which governs decision making regarding implantation, but it is an interesting concept to watch for the future. No discussion of treatment options for hearing impairment however can be complete by simply referring to technology alone, and must always be accompanied by early intervention considerations, parental counselling and other rehabilitative efforts to ensure optimal outcomes for the HI child and his / her family. Early intervention Davis, Reeve & Hind (2001)4 noted that in focus groups, parents indicated they want to know as soon as possible of any HI, regardless of the degree of loss. This could have important implications for screening practices and equipment / algorithms to detect mild losses and more international surveys should be done to add data to this issue highlighted by parents. These authors also suggested such children be monitored for progressive HL and that genetic counselling may also have to be part of the management process. Davis et al. (2001)4 suggested management of mild HL in four areas: (1) Family support, including education; (2) Ongoing assessment and review, mainly audiological monitoring of hearing loss; (3) Communication including the encouragement of early communication, speech and language development; (4) Management choices such as amplification and FM device usage. McKay, Gravel & Tharpe (2008)7 recommended that if parents preferred to follow a ‘wait & see’ approach before fitting amplification, this time should be used to educate them on assessing auditory behaviours, managing the acoustic environment for optimal communication, and to stimulate and model good communication. Referral to early intervention services should be made and speech & language development should be proactively monitored. Parental counselling A list of recommended activities and practices for informational counselling is attached in Appendix A. A downloadable reference document for parents on functional auditory measures is also available in Appendix B. Another excellent resource for parents has been developed by McKay (2010),3 entitled Infants and Toddlers with UHL – A Parent’s Guide, and is well worth a read. Tharpe, Eiten & Abbot Gabbard (2008)8 noted that children typically have large RECDs and therefore gain predictions may only show a few decibels. The counselling implication is therefore a discussion on the cost effectiveness of wearing hearing aids. Perhaps counselling the family on effective communication strategies should take greater prominence. Hearing aids as a treatment option can then again be considered later on. Part of the counselling discussion should also incorporate awareness training of hearing conservation and noise protection, and the benefits of binaural hearing (localisation, binaural squelch, binaural summation, head shadow effect). n Appendix A Suggested Early Intervention Strategies for children and families with MBHL or UHL.17 ● As a minimum, family members must receive information about MBHL or UHL. Provide information to help them observe their child, provide ideas for early intervention activities, and help them monitor their child for signs of progressive hearing loss or bilateral hearing loss. ● Audiologists must provide information regarding functional auditory skills, provide hearing aids and / or FM systems on a trial basis, help the family ● ● ENT & audiology news | www.entandaudiologynews.com with use of amplification and developing communication skills in more challenging acoustic environments. Create or modify the acoustic environment to suit learning: reduce background noise, distance from the teacher / speaker, reduce reverberation, use carpets / drapes, cover feet of chairs with felt, ensure good lighting, keep the child’s interest by adjusting activities, help family read infant cues to stimulate reciprocal communicative behaviour. Put parents of children with MBHL / UHL in touch with each other to share experiences. feature ● ● ● ● Provide informational support to the teacher / caregivers regarding the effects of MBHL / UHL. Provide visual information in addition to auditory information to aid understanding. Write instructions down. Help child to see speaker’s lips so they can benefit from lipreading. Make eye contact and obtain child’s attention before speaking to him / her. Speak a little bit louder. Maintain face-to-face contact with family / caregivers (not just ‘phone calls / emails) to ensure the importance of the situation in perceived. Appendix B Incorporating functional auditory measures into paediatric practice (Tharpe & Flynn) http://www.oticonusa.com/eprise/ main/SiteGen/Uploads/Public/ Downloads_Oticon/Pediatrics/ Inc_Functional_Measures_Guide.pdf References 1. Ross DS, Holstrum WJ, Gaffney M, Green D, Oyler RF, Gravel JS. Hearing screening and diagnostic evaluation of children with unilateral and mild bilateral hearing loss. Trends Amplif 2008;12(1):27-34. 2. Lieu JEC. Unilateral hearing loss in children. Semin Hear 2010;31(4):275-89. 3. McKay S. Audiological management of children with single-sided deafness. Semin Hear 2010;31(4):290-312. 4. Davis A, Reeve K, Hind SB. Children with mild and unilateral hearing loss. In A Sound Foundation Through Early Amplification – Proceedings of the Second International Conference; 2001:179-186. 5. American Academy of Audiology. Pediatric Amplification Protocol; 2003. [http://www.audiology.org/resources/documentlibrary/Documents/peda mp.pdf] 6. Joint Committee on Infant Hearing (JCIH) Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervemtion Programs. [http://www.soundbeginnings.org/download/JCIH_Executive_Summary.p df] Now you can Subscribe Securely online. Subscribe to ENT& audiology news for 1 year (6 issues) 7. McKay S, Gravel JS, Tharpe AM. Amplification considerations for children with minimal or mild bilateral and unilateral hearing loss. Trends Amplif 2008;12(1):43-54. 8. Tharpe AM, Eiten L, Abbot-Gabbard S. Hearing technology. Semin Hear 2008;29(2):169-77. 9. Hawkins DB. Comparisons of speech recognition in noise by mildly tomoderately hearing impaired children using hearing aids and FM systems. J Speech Hear Disord 1984;49(4):409-18. 10. Tharpe AM, Bess FH. Minimal progressive and fluctuating hearing losses in children. Characteristics, identification & management. Pediatr Clin North Am 1999;46(1):65-78. 11. Christensen L, Dornhoffer JL. Bone anchored hearing aids for unilateral hearing loss in teenagers. Otol Neurotol 2008;29(8):1120-2. and receive 2 issues FREE 12. Christensen L, Richter GT, Dornhoffer JL. Update on bone anchored hearing aids in pediatric patients with profound unilateral sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2010;136(2):175-7. 13. Valente M, Oeding K. Transcranial contralateral routing of the signal as a fitting option for patients with single-sided deafness. Semin Hear 2010;31(4):366-77. 14. Anrdt S, Aschendorff A, Laszig R, Beck R, Schild C, Kroeger S, Ihorst G, Wesarg T. Comparison of Pseudobinaural Hearing to Real Binaural Hearing Rehabilitation After Cochlear Implantation in Patients With Unilateral Deafness and Tinnitus. Otol Neurotol 2011;32(1):39-47. 15. Baguley DM. Cochlear Implants in Single-Sided Deafness and Tinnitus. Semin Hear 2010;31(4):410-3. 16. Buechner A, Brendel M, Lesinki-schiedat A, Wenzel G, Frohne-Buechner C, Jaeger B, Lenarz T. Cochlear Implantation in Unilateral Deaf Subjects Associated With Ipsilateral Tinnitus. Otol Neurotol 2010;31(9):1381-5. 17. National Workshop on Mild and Unilateral Hearing Loss: Workshop Proceedings. Breckenridge (CO). Centres for Disease Control and Prevention (2005). scan the QR code above for your instant link ENT & audiology news | www.entandaudiologynews.com 51 Tool Development: developing a rationale for empowering children with hearing loss Melanie Gregory, Staff Audiologist. Correspondence Ida Institute, Egebaekvej 98, DK-2850, Naerum, Denmark. E: [email protected] A s an educational institute, we design and create processes and tools to enhance communication between the patient and hearing care professional and to facilitate a patient-centred approach to rehabilitation. In this article we outline the Ida institute’s approach to innovation and tool development for professionals working with children with hearing loss and their families and describe a proto- Declaration of Competing Interests None declared. The Ida Institute is a non-profit making educational organisation, funded by the Oticon Foundation, whose mission is to ‘foster a better understanding of the human dynamics associated with hearing loss’. W: www.idainstitute.com type of a new tool that we are developing to empower older children with hearing loss within their own rehabilitation process. In January 2011 the Ida Institute held a paediatric focus group in Skodsborg, Denmark. The purpose of this group was to address the following question: “What are the unique challenges facing professionals who work with children with hearing loss and their families? How can existing Ida Institute tools be adapted or improved to address these challenges?” A co-creative process to develop new tools Real life knowledge and experience of the field is a key element for the innovation process as it is the starting point for developing a shared understanding of the dilemmas experienced by the participants 52 The focus group was designed as a cocreative laboratory, which means we bring professionals together from around the world to engage with each other and to bring their expertise, knowledge, ideas and everyday clinical experience to the innovation process. The process has three interdependent stages: 1. To develop a shared understanding of the topic 2. To explore the challenges experienced by hearing care professionals who work within the area of paediatric audiology 3. To create ideas that can be transformed into a tangible form for tools that address these dilemmas. The aim is to identify a specific clinical dilemma or challenge and to develop ideas into a practical tool that can be used in a clinical situation. The tool could be a board game, a metaphor, a new process, a service on the internet… the possibilities are limitless. Developing the rationale for the paediatric tool Understanding the challenges experienced by paediatric audiologists Eileen Rall, PhD described the holistic role of the paediatric audiologists when she said: “Audiologists not only help enhance communication by improving access to sound through technology, but also have a role to play in promoting the healthy development of the child, as hearing can impact on the social, emotional and cognitive development of the child. Part of our role can be seen as helping parents to understand the importance of healthy attachment within the family system, supporting parents through their feelings associated with grief and helping parents to understand the impact of hearing loss on communication.” “Some of the challenges facing paediatric audiologists are: limited training opportunities for student audiologists within the field of paediatrics, quality assurance is frequently focused on protocols, rather than the real life experience of the patient and an ‘expert’ model does not always lead to a satisfying experience of audiological services for parents or their children.” Josephine Marriage, Skodsborg, 2011. Real life knowledge and experience of the field is a key element for the innovation process as it is the starting point for developing a shared understanding of the dilemmas experienced by the participants. Real life case examples were assigned before the focus group, allowing group discussions in a closed web forum and during group sessions in the focus group. Three specific challenges emerged: 1. The management of hearing loss in paediatrics carries a unique responsibility The impact of hearing loss goes beyond the ear and can impact on other areas of development with a profound impact on the family system. Therefore there is a need ENT & audiology news | www.entandaudiologynews.com feature Figure 1: Collage of ideas. Figure 2: A tool draft presented during role play. to not only support the family in management of the hearing loss, but to understand how the family is integral to the overall development of the child. Often the technology or tools used in paediatric audiology are adapted from adult technologies, with little consultation of professionals at an early stage in the development process and therefore these technologies do not fully address the needs of the paediatric population. 2. Effective communication with children and families is critical The first appointment with a family is the beginning of a long journey and participants felt it was imperative to start the journey well together. It is difficult to get a complete picture of daily life for a child from within the clinical setting. Participants wanted to be able to set the scene for positive, proactive interaction despite significant appointment time constraints. Their goal was to enable the child to express his / her own needs and to participate in decision making. 3. Managing hearing loss is a dynamic process There are challenges in understanding the changing circumstances of the family and different cultural backgrounds, alongside the current needs of children with hearing loss through different developmental stages. They wanted to be better equipped to see the whole child within his or her own personal context. the dilemmas was developed. Transforming ideas into tools Participants presented their new tool prototype using role play, which was filmed so that the rest of the group could review and evaluate each tool prototype. The tool drafts that were presented displayed some common themes and patterns. Some of the patterns included the use of pictures to represent and describe the individual daily routines of children with figures of family members and friends and objects that could be used by the child. Reflections from the tool draft presentations We interviewed each group after the role play presentation so that they could describe what happened when they used the tool. These are some of the reflections about the new tool drafts from participants: “By using open-ended questions and the situations, we became more child-focused. The appointment is more focused on the child’s own routine and daily activities.” “The audiologist spent more time listening” “There was a balanced three-way dialogue between the audiologist, child and parent” “The child had a voice” “The parent, child and audiologist problem solve together” Figure 3: The My World Tool prototype. Exploring the challenges and possible new solutions During this phase live theatre and role play were used based on clinical cases to further explore the dilemmas and to enact some clinical scenarios to explore the use of new or different approaches to those they might usually employ. Through reflection on ethnographic videos and discussion of case examples, a deeper understanding of ENT & audiology news | www.entandaudiologynews.com “It felt safe and empowering to manipulate the figures and objects” “I can concretely show what happens to me in daily life” A new tool prototype Through a combination of the design elements, a new clinical tool emerged and was subsequently developed into an applicable prototype named ‘My World’. It is a booklet with schematic room drawings in which the child manipulates items relevant to different scenarios and shows the challenges or positive events linked to the situations of his / her real world experience. The My World framework allows the child to express himself / herself in pictures of daily events, including challenges and successes. My World is still in the development phase, and will be completed in cooperation with audiologists in the UK, Australia and the USA. Conclusion From the point of view of the participants, the rationale for developing the tool in this format was to facilitate a child-centred dialogue reflecting the child’s own experience and so focus on important hearing and communication issues for the child at that time. The tool prototypes aimed to engage the child as an active participant giving him or her an equal voice with the parent and the audiologist. The tool gave a window for the parent and audiologist to understand the child’s experience of daily life. Each situation described by the child became an opportunity to understand the communication, social interaction and emotional response to that situation for the child. In addition, the description provided the possibility to review effective communication strategies and to problemsolve together. n 53 features European Society of Paediatric Otolaryngology May/June 2009 Paediatric Audiology at Alder Hey Children’s NHS Foundation Trust Sept/Oct 2009 Imaging the Paediatric Airway Jan/Feb 2010 Bone Anchored Hearing Aids in Children Mar/Apr 2010 Epidemiology of Paediatric Sleep Disordered Breathing Jul/Aug 2010 The European Society of Pediatric Otolaryngology Mr John Graham, Consultant Otolaryngologist. Correspondence Mr John Graham, Royal National Throat Nose and Ear Hospital, Gray's Inn Road, London WC1X 8DA, UK. E: john.graham10@ virgin.net Declaration of Competing Interests None declared. Acknowledgements I am very grateful to Carel Verwoerd and Chris Raine for input to this article and to Chris for the photograph of the ESPO Board. Evolution of ESPO 1968-2010 ESPO was born in 1968, when Carlo Gatti Manacini, Renato Fior and Guilio Pestelozza, three ENT surgeons in the north of Italy, admired the existence of paediatric ENT as a separate subspecialty in Poland and other Eastern Bloc nations and decided to put their own primary interest in paediatric otolaryngology on a more formal footing. They organised a meeting of likeminded paediatric otolaryngologists in Sirmione, home of the Latin poet Catullus, on Lake Garda. The outcome of this meeting was that the European Working Group in Pediatric Otorhinolaryngology was formed. This fairly informal group grew and recruited members from other European countries, including the United Kingdom, where Robert Pracy in Liverpool and John Evans at Great Ormond Street were already established in the field of paediatric ENT. The First International Congress of Pediatric ENT was organised by the working group, again in Sirmione, in April ESPO fosters strong links between British otolaryngologists and our colleagues on the continent and between specialists in ‘Western’ Europe and our colleagues in the previous Eastern Bloc, who have contributed greatly to ESPO throughout its life and are now well placed to take a leading role in its future 1977 and attracted 400 delegates. John Evans volunteered to host the next meeting in Bath, UK in 1981 and many of us who had recently been appointed to consultant jobs in the UK checked in, greedy for advice from experts like Sylvan Stool, Robin Cotton, John Evans and others on how to do a safe tracheotomy in a neonate weighing 1,200 grams and how to correct the laryngeal stenosis produced as a complication of the new lifesaving method of intubating and ventilating premature infants; as well as the new methods of objective hearing tests in infants. Cochlear implants were still largely in the future, but we were aware of their potential. Subsequently the main rôle of the European Working group was to hold four, then two yearly meetings, to introduce colleagues working in this expanding field to each other and encourage and present research, and to expose younger surgeons to teaching from experienced colleagues. The attendance at these meetings grew from the original 400 in Sirmione, and a more formal organisation, ESPO, was founded during the meeting hosted in Rotterdam, the Netherlands by Carel and Jetty Verwoerd in 1994. At subsequent congresses in Helsinki (1998), Oxford (2002) and Paris (2006) as many as 1,200 delegates registered. Prof Robert Ruben, from New York, attended the first Sirmione meeting in 1977 and two years later founded the International Journal of Pediatric Otorhinolaryngology; Bob Ruben has remained a strong supporter of ESPO and always encourages authors to publish the papers they have read at ESPO meetings. Structure and functions of ESPO ESPO is run by its board, consisting of past (me), present (Noel Garabedian) and future (Javier Cervera) presidents, a General Secretary (Carel Verwoerd), the Treasurer (Chris Raine, who else?) and the chair of the Members committee (Jetty VerwoerdVerhoef). ESPO Council has representatives from 23 European countries. Peter Robb ENT & audiology news | www.entandaudiologynews.com 55 feature and David Albert are the current UK reps. The national societies of paediatric ENT in Europe: Dutch-Flemish, Hungarian, Italian, Polish, Spanish, Greek, French and British (BAPO) are all associated with ESPO. The next president after Javier will be Anne Schilder from Utrecht, who will organise the 2012 congress in Amsterdam, the Netherlands. We also have liaison delegates from the US (ASPO), South America (IAPO) and India (APOI). The Japanese society (JSPO) has also contributed to meetings. Noel Garabedian is our current liaison link with ASPO. Carel Verwoerd, from Rotterdam, took over the job of General Secretary of ESPO in 2001 from the founding General Secretary, Renato Fior and acts as a point of liaison with other organisations. Javier Cervera, from Madrid will be the next President and he and Manuel Manrique will host the next meeting in Pamplona in Spain: June 5th-8th, 2010 (please note this in your diary NOW). We visited Pamplona and found it an excellent city to spend a few days; friendly and small enough to allow exploration on foot. Good range of hotels and excellent food and drink. The new conference centre seems the right size for an ESPO meeting. The St Firmin festival, when bulls are let loose in the streets of Pamplona, is held a month after the congress, in July, perhaps just as well. The congress website can be found at: www.espopamplona2010.com. What of the current role of ESPO? I’m glad to say that ESPO does not have anything as tiresome as a Mission Statement, however it does have clear objectives, which are set out in the website: to disseminate knowledge of ENT disorders in children and their treatment, ● to encourage co-operation across national boundaries and establish links between organisations involved with the care of children with ENT disorders, ● to promote clinical and basic research. Although ESPO does not have funds to pay for research, it can put researchers in touch with others in similar fields in other countries, ● to hold regular scientific congresses, ● to promote training programmes and help define standards for professional practice in Paediatric ENT. The ESPO website www.espo.eu.com has more details of the Society’s basic structure and functions, and the membership form. ● Why join ESPO? • ESPO members normally join because they have a clear interest in paediatric ENT. Many are members of their national paediatric otolaryngology societies. In fact the UK has the highest number of members of any country. This is partly because of the success of BAPO, partly due to effective recruiting by Chris Raine and partly because of: • £££ / €€€ You get a discount on the registration fee for all ESPO meetings. You also get the International Journal of Pediatric Otorhinolaryngology at the significantly reduced subscription of €120 a year. • The membership subscription is absurdly low, at €25 for one year, ---50 for two years. The application form is on the ESPO website. • You can attend, at a reduced rate (see The ESPO Board. (L-R): John Graham, Jetty Verwoerd-Verhoef, Chris Raine, Noel Garabedian (President), Carel Verwoerd, Javier Cervera. previous column) an ESPO congress every two years, meet old friends and update yourself from experts in the rapidly advancing fields of paediatric ENT. What’s the use of attending conferences? Some hospital managers certainly doubt the value of ‘jaunts for doctors’. One of our own dear managers provided the view that she didn’t “see the point of people being paid to have a holiday”, before disappearing out of contact on a series of totally essential management away days… My view is that medical conferences remain extremely valuable, even when they take us away from the important business of removing myriad tonsils to boost hospital income from the UK’s primary care trusts. Web searches, books (even Pediatric ENT: edited by Graham, Scadding and Bull; now in paperback – order your copy today!), emails and telecongresses cannot really replace live presentations that include time for indepth questions and discussion and total immersion in a topic, with unlimited time for sharing problems and setting up lifelong professional links that a three day meeting provides. Our own paediatric ENT association, BAPO, was founded as a spin-off from the Ghent ESPO meeting in 1990 when Peter Bull and I locked all the British delegates into a lecture room, only letting them out after we had all agreed to found our own society; the final details being sorted out during lively discussions with David Proops in the bar of the crosschannel ferry going home. Conclusion ESPO is a lively organisation with excellent links across all European countries. In particular it fosters strong links between British otolaryngologists and our colleagues on the continent and between specialists in ‘Western’ Europe and our colleagues in the previous Eastern Bloc, who have contributed greatly to ESPO throughout its life and are now well placed to take a leading role in its future. ESPO is also highly respected and well placed to influence training and the organisation of services in paediatric ENT in Europe. Join now! n FURTHER INFORMATION Website: www.espo.eu.com www.espopamplona2010.com 56 ENT & audiology news | www.entandaudiologynews.com feature Paediatric Audiology at Alder Hey Children's NHS Foundation Trust Apart from the medical staff, the department is backed up by non-medical audiologists. The full spectrum of audiological services is offered here. Since I have been in this department, I have been involved in testing and managing the hearing of all age groups of children including those who have severe learning disabilities, syndromes, and neurological difficulties. As a Staff Grade in a busy teaching hospital, I have made a significant contribution to providing a comprehensive range of out-patient services for the children. My experience has allowed me to contribute to the development and improvement of the Audiology Service in the region and I have set up a peripheral clinic at May Logan in South Sefton which I run independently. I have one clinic per month for fast-track assessment of children hearing after meningitis and I am the key person to see the children referred to audiology for targeted follow-up from Newborn Hearing Screening. I also share one clinic per week with an ENT Consultant to assess the children who have hearing problems and need surgical intervention. In addition, I have made a considerable contribution to the teaching programme of the medical and audiological students. n Dr Ezeddin El Tabal, Staff Grade in Paediatric Audiology, Alder Hey Children's Hospital, Merseyside, UK. E: [email protected] reprints available > > > > Peer reviewed ABPI compliant Translations available Multiple country coordination For more information and quotes, contact [email protected] matters ENT & audiology news | www.entandaudiologynews.com 57 Imaging the Paediatric Airway Laura Coleman, MRCP, Specialist Registrar in Radiology M ultidisciplinary co-operation in the assessment of the paediatric airway is essential to the safe management of infants and children with respiratory compromise. This article is not intended to be a comprehensive atlas of airway anomalies but seeks to illustrate some of the situations where high quality imaging is central to diagnosis and management. General considerations in radiology Helen Williams, FRCR, Consultant Paediatric Radiologist Kate Parkes, Radiology Clinical Systems Manager Michael Kuo, FRCS, Children’s Ear, Nose and Throat Surgeon Correspondence Michael Kuo, Children’s Ear, Nose and Throat Surgeon, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK. E: [email protected] Declaration of Competing Interests None declared. 58 Many aspects of current imaging, such as Computed Tomography (CT) and Magnetic Resonance Imaging (MRI), require a degree of patient co-operation and control in order to obtain adequate images. However, with a young child, especially those with developmental delay, this may not be possible. Therefore sedation and general anaesthesia have to be considered. Sedation can produce excellent results, avoiding invasive procedures, but requires careful monitoring at all times (respiratory rate, oxygen saturation). It poses the risk of destabilising the airway, increasing the risk of aspiration and causing respiratory compromise. Therefore only suitable candidates must be chosen and resuscitation equipment needs to be close by. Multidetector spiral CT is often used in paediatric imaging due to its faster acquisition times, reduction in ionising radiation exposure and ability to produce better 3D image reconstructions. Faster scanning helps to reduce movement artefact which is of particular benefit with a paediatric population who can't necessarily follow instructions and co-operate with, for example, breath-holding. If there is any risk of destabilising the airway in a child, especially one with known or suspected airway abnormality, then general anaesthesia is by far the safest option. It is important to remember that neonates and young infants are obligate nasal breathers, so any form of nasal airway obstruction can lead to acute respiratory compromise. Airway control aided by general anaesthesia is best considered on a planned basis rather than as an emergency procedure in the CT or MRI suite. It should be carried out and closely supervised by a fully trained paediatric anaesthetist. Care must be taken to use MRI compatible equipment when necessary. Foetal imaging Rapid improvements in ultrasound technology, particularly in improved image resolution, have facilitated the diagnosis of more complex disorders . Of particular relevance to the airway are the diagnoses of neck / thoracic masses causing compression of the airway and disease entities such as laryngeal atresia, both needing immediate intervention at birth. Apart from ultrasound visualisation of mass lesions, polyhydramnios picked up on foetal ultrasound may be an indirect indicator of possible foetal airway abnormalities, prompting further investigation. Where a mass lesion is detected in the neck, foetal MRI can be used to further define the mass, contributing to the differential diagnosis as well as defining its relationship to the airway (Figure 1). This aids the obstetrician and the paediatric airway surgeon in counselling the prospective parents particularly with respect to airway Figure 1: Sagittal T2 weighted foetal MRI image showing mass in the anterior neck. ENT & audiology news | www.entandaudiologynews.com feature Figure 2A: Axial CT scan showing congenital piriform aperture stenosis. Figure 2B: Coronal CT scan of same patient showing single incisor associated with congenital piriform aperture stenosis. management during delivery and the appropriateness of an ex-utero intrapartum therapy (ExIT) procedure. Neonates are obligate nasal breathers for the first few months of life and significant nasal obstruction results in respiratory distress, apnoea and cyanosis. Less acutely, it may manifest itself as difficulty feeding and a failure to thrive. The paediatric otolaryngologist may be asked to review a child in whom it has been impossible to pass a nasogastric tube down one nostril to assess for unilateral choanal atresia. The availability of fine neonatal endoscopes (1.8mm) allows endoscopic examination, but CT remains the modality of choice for defining bony anatomy and is able to highlight the absence of nasal bones as well as the presence and thickness of bony atresia plates. Three-dimensional CT imaging can be used for planning nasal construction / reconstruction surgery in those paediatric patients who are developmentally normal or near normal. Multiplanar CT or MRI is used to assess the intracranial contents in order to aid prognosis. MRI provides more detailed neuroanatomy and is able to confirm the presence or absence of the olfactory nerves. Congenital anterior piriform aperture stenosis / central midline incisor syndrome arises from overgrowth of the nasal process of the maxilla, narrowing the nasal piriform aperture and is an uncommon cause of upper airway obstruction in the neonate. Clinically, there is a narrowing of the anterior choana. CT demonstrates a narrowed anterior nasal cavity: piriform aperture <8mm diameter including nasal septum and single anterior nare width <2mm (Figures 2A and 2B). Features which may also be present include a hypoplastic triangular-shaped palate, bony overgrowth of the maxillary nasal process, incisor abnormalities – a single central midline incisor or partial central incisor fusion. It is important to image the intracranial contents of these patients due to the risk of other midline abnormalities. An MRI head scan in these patients will identify the presence or absence of associated intracranial midline abnormalities including holoprosencephaly, posterior pituitary ectopia, medial deviation of the cavernous carotids and Chiari I malformation. Posterior choanal stenosis / atresia is caused by defective development of the oronasal membrane at the level of the Figure 3A: Bilateral mixed bony-membranous choanal atresia. Figure 3B: These images show coronal / axial images of unilateral left bony choanal atresia. Nasal airway pathology of infancy ENT & audiology news | www.entandaudiologynews.com choanae. Computed posterior Tomography is the imaging modality of choice in determining whether there is choanal atresia or just stenosis, whether it is unilateral or bilateral and for surgical preparation, whether an atresia is membranous, bony or mixed. Sedation is contraindicated in infants with bilateral disease and airway intubation under general anaesthesia is essential. The patient needs to have nasal suction and instillation of vasocontrictive nasal drops pre-scan. High resolution axial scans with multiplanar reconstruction are obtained. Axial and coronal views help to demonstrate the presence or absence of a complete bony bridge. Computed Tomography features of posterior choanal stenosis / atresia include a narrowed or funnel shaped posterior nasal cavity, medial deviation of the posterior maxilla, lateral deviation and thickening or splitting of the posterior vomer, fusion or bridging of the vomer to the maxilla, complete or partial obstruction of the posterior choanae by a bony bridge or soft tissue membrane (choana <3.5mm diameter), airfluid level within the obstructed nasal cavity, elevation of the ipsilateral hard palate and hypoplasia of the inferior turbinates. Observation of these features prepares the surgeon for the operative approach. Where choanal atresia is diagnosed, high resolution CT imaging of the petrous bone should be performed at the same time, if possible, especially in infants with bilateral disease due to the high incidence of CHARGE syndrome in this population (coloboma, heart defects, atresia choanae, retarded growth / development, genital hypoplasia, ear anomalies)(Figures 3A and 3B). Radiology and the assessment of stridor in children The majority of children presenting with stridor will require direct visualisation of 59 feature Figure 4: Plain radiograph showing the ‘steeple’ sign in acute laryngotracheobronchitis. Figure 5: Inspiratory and expiratory chest radiographs showing air-trapping in the left lung on expiratory film. the airway with either flexible or rigid endoscopy. However, radiology provides a useful adjunct to this and can give invaluable additional information during both diagnosis and follow-up which aids the appropriate management of the patient. the glottic and subglottic regions, dilatation of the piriform sinuses and ballooning of the pharynx (Figure 4). Plain films of the neck can show radioopaque inhaled foreign bodies. When there is clinical suspicion of an inhaled foreign body, inspiratory and expiratory chest radiographs should be obtained and may help to assess for indirect signs of a foreign body in the airway such as airtrapping or lung collapse / consolidation (Figure 5). Plain radiography Frontal (anteroposterior) and lateral views of the neck help to demonstrate airway calibre and outline important anatomical landmarks. However, they can be misleading as small variations in patient position can dramatically alter the appearances on the film and radiographs are not performed as a routine in clinical practice. The high-kV ‘Cincinnati’ view is not used as frequently today as in the past due to the improvement in image quality obtained with modern-day computed radiography. Plain radiographs are helpful in demonstrating adenoidal hypertrophy and the effect on the nasopharyngeal airway in obstructive sleep apnoea. The upper limit of normal adenoid size is 12mm AP diameter. Enlarged palatine tonsils may also be seen. Plain film imaging of the child with acute stridor has fallen out of favour because it is considered dangerous, especially out of hours, to take a child with a marginal airway away from the resuscitation room and into an environment which the child may find intimidiating. The diagnosis of the child with acute stridor is primarily a clinical one. Acute epiglottitis in children has largely been eradicated in this country courtesy of the HIB vaccination programme but it remains a cause of stridor and odynophagia in adults. Acute epiglottitis is primarily a clinical diagnosis and requires prompt treatment. Plain radiography can aid the clinical diagnosis of acute laryngo-tracheo-bronchitis / ‘croup’ demonstrating the characteristic ‘steeple’, ‘pencil tip’ or ‘wine-bottle’ sign caused by narrowing of the subglottic airway on frontal views. Additional features include loss of soft tissue distinction in the glottic region on lateral films, ill-defined haziness at the soft tissue – air interfaces between 60 movements of the vocal cords can be assessed during deep inspiration and vibration movements during phonation. The resting position of the vocal folds can also be seen (Figure 6). Ultrasonography can also contribute to the ‘work-up’ of babies with a laryngeal web, subglottic haemangioma and congenital subglottic stenosis, but endoscopy remains the principal tool for investigation of these conditions. Fluoroscopy Ultrasound of the neck Ultrasound of the neck is useful in assessing cystic and solid masses which may be causing extrinsic compression on the airway. It is also of particular use in imaging the larynx in children, which can be inadequately assessed using CT or MRI due to poor soft tissue contrast. It provides a non-invasive and dynamic mode of imaging without radiation exposure. More recently, laryngeal ultrasound has been used to monitor infants with vocal fold paralysis. Abduction-adduction Contrast swallow is a useful dynamic study but it involves ionising radiation. It can aid in the diagnosis of aspiration, laryngeal cleft, tracheomalacia and tracheo-oesophageal fistula. While pH studies are the investigation of choice for the definitive diagnosis of gastrooesophageal reflux, contrast swallow examinations can demonstrate reflux into the oesophagus if it occurs during the study although in itself, the contrast swallow is a poor test for detecting reflux. Extrinsic compression from vascular Figure 6: Laryngeal ultrasound of a child with a right vocal fold paralysis. The right vocal fold is abducted. ENT & audiology news | www.entandaudiologynews.com feature Figure 8: Three-dimensional reconstruction of CT thorax demonstrating tracheal stenosis. All children with significant stridor should undergo cross sectional imaging with CT or MRI under general Figure 7: Lateral contrast swallow view showing indentation in oesophagus created by an aberrant subclavian artery. Figure 9: Three-dimensional MIP (maximum intensity projection) post-contrast MRI volume image of the thorax showing double aortic arch. anaesthesia structures can also be appreciated although further imaging, usually with MRI, is required (Figure 7). Cross-sectional imaging Children with significant stridor requiring cross-sectional imaging with CT or MRI should be imaged under general anaesthesia. As an adjunct to airway endoscopy, CT is particularly useful where a tight stenosis prevents safe endoscopic examination distal to the stenosis. In these situations, multidetector CT, especially with 3D reconstruction can define both the luminal diameter as well as the length of the stenosis. Computed Tomography is also beneficial in the further assessment of extra-luminal and thoracic lesions and tumour staging. It is fast and readily accessible but has its limitations due to ionising radiation and requirement for iv contrast which may be contraindicated in certain patients. In the future, spiral CT under controlled ventilation may be used to acquire virtual endoscopy images of the airway. This may help to replace the more invasive diagnostic methods but the additional ionising radiation exposure needs to be considered. At present, this remains a research tool in development (Figure 8). MRI is able to easily demonstrate anatomy in the neck and mediastinum in any plane without exposure to ionising radiation or necessarily to contrast media. However, image quality is affected by movement, such as breathing or pulsation artefact from vascular structures so alterations in technique such as respiratory triggered scans are often required. MRI provides detailed imaging of soft tissue structures and vascular anatomy. It has now become accepted as the single most valuable modality for assessing airway compression, accurately and non-invasively demonstrating the level, severity and cause of airway compression (Figure 9). Summary High quality imaging is vital to complement endoscopy in the assessment of the infant with a compromised airway. Safe imaging in the presence of airway compromise requires expertise from radiographers and radiologists but also good communication between the airway surgeon and the radiologist to ensure that the optimal modality is employed to give the necessary clinical information with minimal risk. n FURTHER READING King SJ, Boothroyd AE: Pediatric ENT Radiology. Berlin, Germany: Springer-Verlag; 2003. Johnson K, Williams H, Foster K, Miller C. Paediatric Radiology. Oxford Specialist Handbook in Paediatrics. Oxford, UK: Oxford University Press; 2009. ENT & audiology news | www.entandaudiologynews.com 61 Bone Anchored Hearing Aids in Children Patrick Sheehan, BCh, MPhil, FRCS(ORL-HNS), Consultant Paediatric Otolaryngologist. oday, the Bone Anchored Hearing Aid (BAHA) is a well described and accepted form of T auditory rehabilitation. In the adult population, the technique is a relatively uncomplicated, and is usually performed as a one-stage procedure under local anaesthesia with few complications. Ann-Louise McDermott, FDS, RCS, PhD, FRCS(ORL-HNS), Consultant Paediatric Otolaryngologist. Correspondence Mr Patrick Sheehan, Consultant Paediatric Otolaryngologist, Royal Manchester Children’s Hospital, Oxford Road, Manchester, M13 9WL, UK. E: Patrick.Sheehan@ manchester.ac.uk Declaration of Competing Interests Patrick Sheehan has been reimbursed by Cochlear Ltd, the manufacturer of Baha, for attending several conferences. In the paediatric population, however, it is not so simple. Firstly, general anaesthesia is preferred and two-staged surgery is generally necessary. As many children who need a BAHA often have a significant medical history and / or are syndromic, the procedure may carry more risk. Secondly, there are issues of soft tissue and skull thickness, which vary greatly in young children and may significantly affect the surgical procedure itself as well as any subsequent complication rates. The aims of the BAHA in the paediatric population is to restore hearing, maximise auditory pathways and improve speech and language development as soon as possible in order for the child to achieve his / her full potential. Indications In the early days of BAHA surgery, the indications were predominantly for chronic suppurative otitis media. Now BAHA is the hearing aid of choice for hearing loss associated with congenital ear deformities. The recent role of BAHA in unilateral hearing loss has further expanded the indications. Indication for paediatric BAHA are: 1. 2. 3. 4. 5. 6. 7. 8. 9. Congenital conductive hearing loss, Congenital aural atresia, Congenital microtia, Chronic suppurative otitis media, Persistent otitis media with effusion, Chronic otitis externa, Unilateral profound hearing loss, Failure with conventional aids, Trauma to external ear. Congenital conductive hearing loss is usually associated with syndromal children such as Treacher Collins syndrome, Goldenhaar’s syndrome and Oto-renal syndrome. Children with these syndromes have benefited from BAHA's. The use of BAHA in adults with single sided deafness (SSD) has initially been very positive giving the patient a perceived improvement in directionality of sound particularly in noise situations or background noise. Small studies on older children with SSD have supported the 62 adult results,1 however, studies on children with unilateral congenital ears showed that many of these children have excellent directional hearing and objective studies on speech in noise showed no significant improvement after BAHA fitting.2 Overall, there is no convincing evidence for early intervention in those children with unilateral congenital ears.3 However it is good clinical practice to involve the child’s carers in the decision process when considering aiding the unilaterally deafened child. The child A large proportion of children with congenital ear abnormalities have associated syndromes and complex medical conditions. This may in turn make the surgical process more difficult. These children require two general anaesthetics and many of them may pose intubation difficulties. These children should ideally be treated in specialised childrens’ centres where there are dedicated multidisciplinary teams.4 BAHA has been shown to be very successful in children with Down syndrome and also in children with severe learning difficulties who find conventional hearing aids difficult to tolerate (see article on page 55). The Baha® Softband Currently, the Baha® Softband is the most appropriate means to provide aural rehabilitation to very young children. The use of the BAHA on a test band is now the gold standard for audiological preoperative assessment in children. In those children with conductive hearing loss in whom a BAHA is being considered for aural rehabilitation a four week trial of a Baha® Softband will allow the child to experience the hearing benefits of BAHA. This trial leads to better informed decision making between child, parents and audiologists. Timing of surgery Another reason to use the Baha® Softband in younger children is the higher rate of fixture loss. Fixture failure rates of children below the age of five years and those 5-10 years are 40 and 8% ENT & audiology news | www.entandaudiologynews.com feature Table 1: Overview of paediatric bone anchored hearing aid surgical outcomes. Year 2009 2008 2008 2007 Number of children 182 93 39 40 2007 2005 2002 2001 71 41 51 19 Age years (mean) 6.8 9.3 3.3 3.2 7.6 9.6 8.4 7.6 11.2 Skin complications % 19 9.4 44 NA NA 37 2.4 3.8 16 Revision soft tissue. Surgery per patient 7.7 10.9 14.5 7.5 Fixture loss % 14 16.3 40 17.5 25.9 17.1 NA 10.5 25 9.1 21.5 5.3 Adapted with permission from a table in De Wolf et al. 2008.6 respectively. Those children over the age of 10 years have 1% fixture loss.4 The child’s activities, medical history and social settings must all be considered. There is no fixed age for BAHA surgery but the older the child, the thicker the calvarial bone, the longer the fixture that can be placed and the better the longterm results are. The use of the Baha® Softband until age four years is preferred. There are studies reporting successful implantation in under three year olds but other factors such as prolonged osseointegration times are practised.5 The site of fixture placement should be in bone that is of sufficient thickness at the time of surgery, to support a fixture of 3mm or more. Thus to obtain the best results, surgery is recommended after the age of four years. One or two stage surgery Two-stage surgery is standard practice for children. This allows for better osseointegration period in the water-rich / mineral deprived bone of children and also allows for ease of surgery and the osseointegration period requires no aftercare / maintenance for child or carer. Surgical technique Various soft tissue reduction techniques have been described in children. The surgical principles are to use either a free or pedicled split skin graft to produce an area of skin surrounding the abutment that is hairless and tightly bound to the periosteum, encroaching on neither the abutment, nor the sound processor. The introduction of the dermatome to produce suitably thin skin around the abutment has shown to give reproducible results. The linear incision technique recently described by the Nijmegen team6 which does not require a skin graft but just undermines the surrounding skin is reported to have fewer complications. The ideal time for soft tissue reduction and the skin graft is following the planned period of osseointegration. At this second stage of BAHA surgery, the fixtures are uncovered after all the soft tissue work is completed and the abutment is attached to the osseointegrated fixture. The child can attached the BAHA processor about one week after the second stage surgery. The position of the BAHA is important in the child with congenital atresia if autologous reconstruction is to be considered at a later stage. More posterior placement of the fixture is recommended to avoid compromising the healthy tissues at the site of the future reconstructive surgery.7 Another point of interest is the use of a ‘sleeper’ fixture. This is usually an ipsilateral fixture close to the primary fixture. All children under the age of 10 years should ideally have a sleeper fixture due to the increased rates of fixture failure and trauma. Recent suggestions have been to place the sleeper fixture on the contra lateral side in potential bilateral cases, thus reducing the overall costs and allowing the option of bilateral BAHA use.8 Fixture size It has been suggested that 2mm of cortical bone thickness is necessary for implantation for BAHA. There is no consensus of the size of fixture that is used in children. Very often the dura is exposed when using the guide drill. Some surgeons described the placement of a 3mm fixture flush with the outer bone table (dura depressed by the fixture) then a longer period of osseointegration time before Stage II. Other surgeons routinely use a 4mm fixture even if calvarial bone is very thin. No countersink is created and the fixture is either left proud of the outer bone table and bone dust augmentation used or a very gentle displacement of the dura is carried out by placing the fixture at low torques of 20Nm2 and in some cases by hand without the use of the handpiece. Most centres practise a three to four ENT & audiology news | www.entandaudiologynews.com month period between surgeries to allow for osseointegration unless there were difficulties encountered at the first procedure such as inadequate bone thickness in which cases the intervening time is increased to six months. Complications It is well established that BAHA surgery in children is associated with higher complication rates.4-6 These will include: Failure of osseointegration There is a significantly higher rate of fixture failure particularly in the very young.6 Most authors agree that the majority of fixture failures occur in the first year following surgery.4,6 Soft tissue complications Increased soft tissue complications are well recognised in children4,6 and are a significant problem for centres dealing with paediatric BAHA. Children rely greatly on their carers for the maintenance and hygiene of the soft tissues around the abutment. Many of these children have a significant medical condition, some of which are associated with underlying behavioural and developmental delay. This would make maintenance of their BAHA more challenging. Furthermore, as the child grows the fixture and abutment may become buried by new cortical bone and for some children there is marked hypertrophy of the skin surrounding the abutment requiring skin reduction. Therefore the aftercare of children with BAHA may be longterm. Table 1 illustrates an overall summary of reported surgical outcomes from Paediatric BAHA centres. Trauma Finally, trauma is a challenge to any paediatric BAHA team: Damage to the device and / or the abutment appears to be not 63 feature uncommon. Clinicians involved in paediatric BAHA provision must be aware that unusual and unexpected complications can occur long after the surgery is complete and this highlights the importance of a multidisciplinary team in the longterm management of these children. Quality of life A great deal of research has been undertaken by the majority of paediatric BAHA centres into establishing whether the provision of a BAHA improves the quality of life of the child.3,9 The overwhelming consensus from all published literature is that there is a significant improvement in quality of life reported by the children / carers despite the associated increased morbidity in the younger age groups. The measure of success of a BAHA in a child should be reflected by the number of hours / days per week that the child wears his / her BAHA. The recent literature clearly reflects success rates of 96% and above in all the larger paediatric centre.4,9 Conclusion References The bone anchored hearing aid in children is an effective means of aural rehabilitation in children with conductive hearing loss who otherwise cannot benefit from conventional hearing aids. The main indication remains in those children with chronic ear disease that require hearing amplification. Their role in the child with congenital atresia is widely supported by many units around the world. The BAHA system has been shown to improve the overall quality of life of the child. Although, it is a well tolerated and relatively ‘simple’ procedure it is not without risk. A multidisciplinary team approach combined with careful patient selection, the counselling of the child and carer and an understanding of the problems that present in children should result in a successful outcome for the child. Although various surgical techniques are described the predominately important consideration is attention to detail in the surgery. This management approach consistently gives a good audiological and quality of life outcome as has been reported in the literature. n 1. Christensen L, Dornhoffer JL. Bone anchored hearing aids for unilateral hearing loss in teenagers. Otol Neurotol 2008;29:1120-2. 2. Kunst SJ, Leijendeckers JM, Mylanus EA. BAHA system application for unilateral congenital conductive hearing impairment: audiometric results. Otol Neurotol 2008;29:2-7. 3. Snik AFM, Leijendeckers J, Hol MKS. The bone anchored hearing aid for children: recent developments. Int J Audiol 2008;47:554-9. 4. McDermott A-L, Williams J, Kuo MJ, Reid AP, Proops DW. The Birmingham Paediatric Bone Anchored Hearing programme: a 15 year experience. Otol Neurotol 2009;30:173-83. 5. Davids T. Bone anchored hearing aids in infants and children younger than 5 years. Arch Otolaryngol Head Neck Surg 2007;133:50-5. 6. de Wolf MJ, Hol MK, Huygen PL, Mylanus EA, Cremers CW. Nijmegen results with application of a bone anchored hearing aid in children: simplified surgical technique. Ann Otol Rhinol-Laryngol 2008;117:805-14. 7. Zeitoum H, De R, Thompson SD, Proops DW. Osseointegrated implants in the management of childhood ear abnormalities. J Laryngol Otol 2002;116:87-91. 8. Bernstein JM, Sheehan PZ. An approach to bilateral bone anchored hearing aid surgery in children: contra lateral placement of sleeper fixture. J Laryngol Otol 2009;123:555-7. 9. McDermott A-L, Williams J, Kuo MJ. Quality of life in children fitted with a bone anchored hearing aid. Otol Neurotol 2009;30:344-9. For a quick link to www.entandaudiologynews.com scan this QR code with your mobile device. 64 ENT & audiology news | www.entandaudiologynews.com Epidemiology of Paediatric Sleep Disordered Breathing Michelle Wyatt, MA, FRCS(ORL-HNS), Consultant Paediatric ENT Surgeon. Correspondence Michelle Wyatt, MA, FRCS(ORL-HNS), Consultant Paediatric ENT Surgeon, Great Ormond Street Hospital, London, WC1N 3JH, UK. E: [email protected] Declaration of Competing Interests None declared. he concept of sleep-disordered breathing (SDB) in the paediatric population was introduced by Guilleminault in 1976 to reflect the difference between the well-recognised concept of obstructive sleep apnoea seen in adults and the broader spectrum of different issues seen in children. SDB encompasses primary snoring, upper airways resistance syndrome (UARS), obstructive hypoventilation (OH) and obstructive sleep apnoea (OSA) on a continuum of worsening levels of respiratory disturbance. Classically primary snoring is noise generated by the vibration of the soft palate during sleep. There is no associated alteration in sleep quality. In UARS there is an increase in negative intrathoracic pressure during inspiration with resulting arousals and sleep fragmentation. The American Thoracic Society defines OSA as a ‘disorder of breathing during sleep characterised by prolonged upper airway obstruction and / or intermittent complete obstruction (obstructive apnoea) that disrupts normal ventilation during sleep and normal sleep patterns’.1 Obstructive hypoventilation is seen by this organisation as part of OSA. Interestingly the latest International Classification of Sleep Disorders2 uses respiratory related arousals (seen in UARS) in the diagnosis of OSA and so UARS itself may also soon fall under the OSA terminology umbrella. The prevalence of OSA could therefore be underestimated as those with UARS and OH have been excluded from the majority of research studies to date. Another difficulty in reporting the true epidemiology of paediatric SDB is the fact that different studies use different methods of data collection and different diagnostic criteria to define the problem. Generally the two measures most frequently used in assessment are the Apnoea Hypopnoea Index (AHI) and the Respiratory Disturbance Index (RDI). The former refers to the number of obstructive apnoeas and hypopnoeas per hour of sleep and the latter includes these two plus the number of respiratory related arousals per hour of sleep. An AHI or RDI of between 1-5 suggests a child has OSA. T General prevalence of SDB Primary snoring in the UK has a quoted prevalence of 12.1%.3 A meta-analysis of 41 studies from around the world showed a prevalence of 7.45%.4 For OSA, figures of between 0.7 and 1.8% are quoted for Europe3,5 and 1.2% for the USA.6 Specific epidemiological factors Gender It has generally been felt that gender did not affect the prevalence of SDB. However, a number of studies have shown that male sex is associated with an increased incidence of SDB, with rates of between 50-100% quoted.4 These authors suggest that failure by others to detect a difference related to small sample sizes. The gender difference was initially felt to be more notable when older children were studied. It was therefore proposed that this variance was due to the hormonal and physiological changes seen at puberty. However, further analysis shows that differences are still present in cohorts reviewed before hormonal changes occur and so other factors must also be involved. Ethnic origin SDB is known to be higher in children of AfroCaribbean origin. One study in American adolescents showed it to be twice as likely,7 and another looking at eight to eleven year olds showed it to be four to six times more likely.8 This may reflect craniofacial differences and / or upper airway lymphoid tissue size variation. Age There is varying evidence regarding effect of age on the prevalence of SDB with some studies showing no differences in SDB as the child grows. However, an Australian review of 985 children did show a significant decrease in snoring prevalence between the ages of four and twelve9 and others have shown that problems become more common again in the older teenager.10 More severe OSA and a higher incidence of central apnoea has been detected in those less than three years of age, leading to the concerns regarding careful postop monitoring in this group.11 Of note over 40% of neonates are known to have obstructive or mixed apnoeas, which are more frequent between the ages of two to seven weeks than from eight to 28 weeks.12 SDB was three to five times more likely in children of eight to eleven years who were born prematurely.8 ENT & audiology news | www.entandaudiologynews.com 65 feature Obstructing adenoids (left) viewed through a flexible endoscope. Large tonsils (right) classical of the upper airway lymphoid hyperplasia particularly seen in children of Afro-Caribbean origin. Obesity The impact of increasing levels of obesity on the numbers of those with significant SDB is followed with great interest. Again difficulties with accurate and consistent data collection generate their own issues. The use of Body Mass Index (BMI), for example, can only be in the context of age and sex specific growth charts. Increased weight has been shown in a number of studies to be a risk factor for OSA.13 One study has shown that heavier babies at two to four months old have more issues with snoring.14 A review of adolescents showed a 3.5 fold increase in the risk of OSA syndrome for each standard deviation increase in BMI Z-score.15 (The Zscore has been introduced to monitor changes in patients with a BMI above the 99th percentile or below the 1st percentile.) In obese children, less adenotonsillar enlargement is required to affect sleep quality to the same degree compared with non-obese individuals.16 For the overweight teenager pharyngeal adipose tissue and decreased respiratory capacity are also felt to be important issues. Obese children are more likely to have persistent OSA and poor quality of life scores after adenotonsillectomy.17 OSA in pre-school children is often due to large tonsils and adenoids. Note mouth breathing is a common feature. 66 Craniofacial anomalies Children with craniofacial anomalies have an increased incidence of SDB. Reasons for this are multifactorial with issues occurring at a number of levels including the nose, post nasal space, tongue base, pharynx or mandible. Abnormal polysomnography (PSG) with evidence of OSAS was obtained in 57% of children with Down syndrome with a figure of 80% seen if those with an elevated arousal index were also included.18 The contributing factors to this are pharyngeal hypotonia, midfacial / mandibular hypoplasia and macroglossia / glossoptosis. Adenotonsillectomy therefore does not have the same high incidence of effective intervention as it does in the general population. Two studies show persistent issues post surgery in around two thirds of individuals.19,20 A review of 95 children with achondroplasia showed clinical evidence of obstructive sleep apnoea in 38%, with adenotonsillectomy being an effective intervention. However, 18% of these children who had Ts and As required further therapy.21 Contributing factors which lead to OSA have been shown on radiological studies and include significant midface OSA in Down Syndrome occurs for a number of reasons, including macroglossia (illustrated here). A two-year-old boy with Apert syndrome with associated mid face hypoplasia. His severe obstructive sleep apnoea is successfully managed with a nasopharyngeal airway. Baby with Pierre Robin Sequence illustrating a small mandible. Image provided courtesy of the Cleft Lip and Palate Association (CLAPA): www.clapa.com narrowing, retrusion of the chin and increased lower facial height.22 A small jaw, as seen in Pierre Robin Sequence, is also associated with SDB. Thirty one of 52 children (60%) in a Mexican study were shown to have OSAS.23 Treatments aimed at bypassing the obstruction at the mandibular level have good outcomes. Neuromuscular disorders These children are particularly vulnerable to SDB and its complications. OSAS is more likely as, in addition to the more usual causes such as adenotonsillar hypertrophy, there is often low muscle tone in the tongue and pharynx resulting in significant upper airway obstruction. The neuromuscular condition can affect the diaphragm and other respiratory muscles. This results in sleep related hypoventilation, which along with OSAS, can lead to an increased severity of hypoxaemia during sleep compared to those with either condition alone. There is also an increased incidence of central apnoea with reduced central neural chemoresponsiveness.24 Particular care is required for these individuals if surgery is planned. Summary Although there is considerable evidence as to which population groups are affected by SDB, there are limited data as to how the natural history progresses in an individual. Conclusions in the review by Lumeng and Chervin4 suggest that: 1. Children with abnormal PSG are likely to continue with problems for several years if untreated, 2. A significant number of primary snorers will see spontaneous resolution in a few years and 3. A small number of children without problems will commence primary snoring as they get older. The detrimental impact of SDB on the general health of the paediatric population ENT & audiology news | www.entandaudiologynews.com feature is becoming increasingly obvious as time goes on. An understanding of those most at risk allows focus when resources are limited. However, consideration should always be given to the potential diagnosis, as evidence suggests that even primary snoring can have neurobehavioural consequences and obstructive issues can exist in the absence of obvious snoring. n References 1. American Thoracic Society. Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med 1996;153:866-78. 2. American Academy of Sleep Medicine. International classification of sleep disorders:diagnostic and coding manual. Second edition. Westchester, IL: American Academy of Sleep Medicine; 2005. 3. Ali NJ, Pitson DJ, Stradling JR. Snoring, sleep disturbance and behaviour in 4-5 year olds. Arch Dis Child 1993;68:360-6. 4. Lumeng JC and Chung RD. Epidemiology of Pediatric Obstructive Sleep Apnea. Proc Am Thorac Soc 2008;5:242-52. 5. Brunetti L, Rana S, Lospalluti ML, Pietrafesa A, Francavilla R, Fanelli M, Armenio L. Prevalence of OSA syndrome in a cohort of 1207 children of Southern Italy. Chest 2001;120:1930-5. 6. Bixler EO, Vgontzas AN, Lin H-M, Liao D, Calhoun S, Vela-Bueno A et al. Sleep Disordered Breathing in children in a general population sample: prevalence and risk factors. Sleep 2009;32:731-6. 7. Johnson EO, Roth T. An epidemiologic study of sleep disordered breathing symptoms among adolescents. Sleep 2006;29:1135-42. 8. Rosen CL, Larkin EK, Kirchner HL, Emancipator JL, Bivins SF, Surovec SA, Martin RJ, Redline S. Prevalence and risk factors for sleep-disordered breathing in 8-11 year old children: association with race and prematurity. J Pediatr 2003;142:377-82. 9. Zhang G, Spickett J, Rumchev K, Lee AH, Stick S. Snoring in primary school children and domestic environment: a Perth school based study. Respir Res 2004;5:19. 10. Cordo GM, Forastiere F, Agabiti N, Pistelli R, Dell’Orco V, Perucci CA, Valente S. Snoring in 9 to 15 year old children: risk factors and clinical relevance. Pediatrics 2001;108:1149-54. 11. Don DM, Geller KA, Koempel JA, Ward SD. Age specific differences in pediatric OSA. Int J Pediatr Otorhinolaryngol 2009;73:1025-8. 12. Kato I, Franco P, Groswasser J, Kelmanson I, Togari H, Kahn A. Frequency of obstructive and mixed sleep apneas in 1023 infants. Sleep 2000;23:487-92. 13. Shine NP, Coates HL, Lannigan FJ. Obstructive sleep apnoea, morbid obesity and adenotonsillar surgery: a review of the literature. Int J Pediatric Otorhinolaryngol 2005;69:1475-82. 14. Kelmanson IA. Snoring, noisy breathing in sleep and daytime behaviour in 2-4 month old infabts. Eur J Pediatric 2000;159:734-9. 15. Kohler MJ, Thormaehlen S, Kennedy JD, Pamula Y, van den Heuvel CJ, Lushington K, Martin AJ. Differences in the association between obesity and OSA among children and adolescents. J Clin Sleep Med 2009;5:506-11. 16. Dayyat E, Kheirandish-Gozal L, Sans Capdevila O, Maarafeya MM, Gozal D. OSA in children:relative contributions of body mass index and adenotonsillar hypertrophy. Chest 2009;136:137-44. 17. Mitchell RB Boss EF. Pediatric OSA in obese and normal weight children: impact of adenotonsillectomy on quality of life and behaviour. Dev Neuropsychol 2009;34:650-61. 18. Shott SR, Amin R, Chini B, Heubi C, Hotze S, Akers R. Obstructive sleep apnea: should all children with Down syndrome be tested? Arch Otolaryngol Head Neck Surg 2006;132:432-6. 19. Merrell JA, Schott SR. OSAS in Down syndrome: T&A versus T&A plus lateral pharyngoplasty. Int J Pediatr Otorhinolaryngol 2007;71:1197-203. 20. Shete MM, Stocks RM, Sebelik ME, Schoumacher RA. Effects of adenotonsillectomy on polysomnography patterns in Down syndrome children with obstructive sleep apnea: a comparative study with children without Down syndrome. Int J Pediatr Otorhinolaryngol 2010;74:241-4. 21. Sisk EA, Heatley DG, Borowski BJ, Leverson GE, Pauli RM. Obstructive sleep apnea in children with achondroplasia: surgical and anesthetic considerations. Otolaryngol Head Neck Surg 1999;120:248-54. 22. Sleep disordered breathing in children with achondroplasia. Part 2 Relationship with craniofacial and airway morphology. Int J Pediatr Otorhinolaryngol 2006;70:453-61. 23. Bravo G, Ysuna A, Arrieta J, Pamplona MC. Videonasopharyngoscopy is useful for identifying children with Pierre Robin sequence and severe obstructive sleep apnoea. Int J Pediatr Otorhinolaryngol 2005;69:27-33. 24. Alves RS, Resende MB, Skomro RP, Souza FJ, Reed UC. Sleep and neuromuscular disorders in children. Sleep Med Rev 2009;13:133-48. Writing for ENT & audiology news If you have attended a meeting or conference recently, and would like to write for our Newsround section, please get in touch! Meeting reports should be 250-300 words long, with the main message of ‘what you missed if you didn’t attend’ focusing on: • • • • Get in touch Who were the key speakers? What were the highlights? Were there any controversies? Who won prizes? Please email [email protected] for conference report requirements and issue deadlines. ENT & audiology news | www.entandaudiologynews.com 67 audiology matters A Clinical Service for Children and Adults with Suspected APD Jan/Feb 2009 Managing Children with Auditory Processing Disorders in the Educational Environment Jan/Feb 2009 The Challenges Ahead in Paediatric Audiology Mar/Apr 2009 New Developments in Hearing Aids for Children and Adults May/June 2009 Cochlear Implantation in Early Childhood: what is happening in the longterm? Jul/Aug 2009 New Frontiers: auditory brainstem results in adults and children Jul/Aug 2009 An Approach to the Dysmorphic Child with Deafness Sept/Oct 2010 A Clinical Service For Children and Adults With Suspected APD Dr Doris-Eva Bamiou, MD, MSc(distinction) PhD. DoH HEFCE Senior Lecturer (UCL Ear Institute). Consultant in Audiovestibular Medicine. H earing impairment may have a profound effect upon an individual’s ability to function in his personal, social, and occupational life. In the last five to six decades, clinicians have become increasingly aware that hearing difficulties can be caused by disordered processing of the sound signal within the brain across all age ranges. The following article will highlight key theoretical considerations and clinical issues regarding the diagnosis and management of patients with suspected disordered auditory processing. Dr Tony Sirimanna, MB, BS, FRCS, DLO(UK), MS, MSc, FRCP, Consultant Audiological Physician. Correspondence Dr Doris-Eva Bamiou Consultant in Audiovestibular Medicine, National Hospital for Neurology and Neurosurgery & Great Ormond Street Hospital for Children. Email: d.bamiou@ ucl.ac.uk Declaration of Competing Interests Doris-Eva Bamiou is the elected Chair of the Steering Committee of the Auditory Processing Disorders Interest Group of the British Society of Audiology. Dr Bamiou runs clinics for adults with APD at the National Hospital for Neurology and Neurosurgery and for children with APD at Great Ormond Street Hospital. What is the current scientific theoretical background that may inform setting up a clinical service for auditory processing disorders (APD)? Recent advances in auditory neuroscience have enhanced our understanding of how the brain extracts biologically relevant meaning from the pressure and frequency waves representing sounds in the cochlea. Auditory processing is thought to be subserved by an initial ‘decomposition’ of the auditory signal in the cochlea, accurate transmission of the information contained in the initial activity patterns and further analysis (which includes summation, subtraction or correlation) of the transmitted signal by the ‘central processor’ in the brain. At the level of the auditory cortex, sounds are represented as ‘auditory objects’ rather than in terms of their acoustic features (Nelken 2008).1 Functional organisation of the auditory cortex spans a continuum, from basic sensory processing in primary cortical areas, to polymodal integration in non-primary (or association) areas, to behavioural modulation in the limbic system and multisensory processing in multisensory subregions (Winer et al., 2006).2 Neural representation of sound is influenced by task-specific demands, expectations, and higher order top down effects on multiple levels of the auditory pathway. For example, auditory attention may modulate cochlear activity as reflected on otoacoustic emission recordings in a frequencyspecific manner, while attentive expectation (such as when listening to a known melody with silent gaps) helps to shape cortical responses (as the auditory cortex will be active even during the silent gaps in the melody) (reviewed in Fritz et al., 2007).3 The common representation between auditory function and known cognitive, emotional and visual centres may help explain how other non-auditory factors influence and modulate the perception of sounds. Audiologists have long been aware that a normal functioning ear does not guarantee normal hearing and conversely, that patientor parent- (in the case of a child) reported auditory difficulties such as difficulties with speech in background noise may not always arise because of problems within what is traditionally regarded as the auditory domain. Our current understanding of the intricate process of audition has not as yet entirely translated into the clinic in the form of gold standard tests for auditory processing, but may help inform our clinical practice and decision making. What drove the development of the APD service? The development of the paediatric APD service at Great Ormond Street Hospital (GOSH) and of the adult APD service at the National Hospital for Neurology and Neurosurgery (NHNN) was driven by realisation of the need and, in recent years, by a rapidly increasing patient as well as professional demand. Children with suspected APD may experience language or learning difficulties, may be perceived as unruly, due to his / her poor listening skills, or may be perceived as not fulfilling their potential. Adults with APD (and this includes a significant proportion of the aging population) may similarly experience difficulties in their personal, social and professional environments, and may be perceived as unsociable or uncooperative. Are there controversies surrounding APD? A great many, including what exactly constitutes an APD, with some professionals ENT & audiology news | www.entandaudiologynews.com 69 feature being still unconvinced that it exists as a separate clinical entity, poor understanding of the boundaries and overlap between APD and language or other developmental disorders, and lack of uniform accepted guidelines regarding testing and management of APD. Auditory processing disorders are not formally recognised in current editions of any of the formal classification manuals, such as the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV). It is beyond the scope of this paper to argue for or against its existence – both authors use the term APD as referring to ‘disordered auditory processing’ and distinguish APD from language processing disorder that extends from the point at which speech sounds are categorised into psycho-linguistically distinct percepts. What is the diagnostic test protocol at GOSH and NHNN, on what premises was it chosen and how is the diagnosis of APD made? The clinical presentation of APD in both children and adults may include difficulties with localisation and distinction / discrimination of sounds. Understanding speech in background noise and fast / distorted speech, auditory memory or musical perception may be impaired, to name but a few patient- and parent-reported symptoms, and there may be additional social, emotional, language or educational sequelae. This clinical presentation is characteristic but non specific to APD, in that other higher order disorders (for example of language, attention, cognition and so on) may also be implicated. The APD Interest group of the British Society of Audiology4 thus recommends that the assessment for APD should be multidisciplinary and requires a test battery approach. The assessment should include: Detailed audiometry to define hearing thresholds and to assess cochlear and auditory nerve function ● Specific central auditory tests, both non-speech and speech, which should assess >2 different aspects of auditory processing ● Tests of language, cognition and memory (conducted by the appropriate certified professionals) to assess the effect of higher order factors on the patient’s presentation. It is essential to ensure that the child's (or adult’s) attention is optimum at all times during behavioural testing. At GOSH, paediatric patient referrals are only accepted if they have had a recent speech and language and cognitive psychology assessment, especially if there are concerns about language or cognitive function, while adult patients at the NHNN are referred for cognitive neuropsychometry and speech and language assessment after they have been seen in the clinic. The audiological test battery in both settings has been informed by the BSA recommendations, by previous recommendations of the American Speech Language and Hearing Association (ASHA 1996, 2007)5,6, as well as by what tests are available for clinical practice with ageappropriate normative data. This battery includes: a. baseline audiometry (including tympanometry, acoustic reflexes, otoacoustic emissions and suppression, speech discrimination in quiet and auditory brainstem evoked responses) b. central auditory tests (obtained from Audiology Illustrated7 or from Auditec8) Dichotic digits (dichotic listening) Frequency and duration pattern tests (temporal sequencing) Gaps in noise and / or random gap detection tests (temporal resolution) ● Children with suspected APD may experience language or learning difficulties, may be perceived as unruly, due to their poor listening skills, or may be perceived as not fulfilling their potential 70 Masking level difference (Binaural integration) Low redundancy speech tests such as filtered words or words presented in a background of babble (from the SCAN C or A by Keith from Tartan Products9, in the paediatric setting, and a homedeveloped test delivered via the Matlab platform at the NHNN) c. Selected cases may have additional tests, such as a formal assessment of musical skills (Montreal battery for the evaluation of amusia by Peretz et al. 200310 for adults, or Gordon’s musical aptitude profile for children from GIA Publications11), electrophysiology such as mismatch negativity or other auditory event related potentials and imaging as needed. The Newcastle Auditory test Battery (NAB, Griffiths et al., 2001)12 and the IMAP test battery (see Melanie Ferguson’s article in same issue) may also be applied on patients for research purposes. Diagnosis of APD is made upon the findings of abnormal results in at least two tests, one of which is non-speech, and by exclusion of other potential confounders as per the basic audiology, speech and language and cognitive assessments. How can APD patients be managed? Diagnosis of APD helps to explain the patient’s difficulties and to provide the adult or child sufferer and their families with advice that may help. This diagnosis will also help provide some guidance for the child’s teachers as to how to provide optimum support that will help the child to achieve his / her best. Intervention for children and adults with APD is based on the patient’s test deficits and complaints, and implemented by a range of professionals. This may include (Bamiou et al., 2006)13: a. Modifying the educational environment, including provision of personal or soundfield FM systems (that is, wireless devices that receive, amplify and transmit the sound to the listener’s ear), speaker / teacher based adaptations and environmental modifications in order to reduce ambient noise and reverberation (that is, multiple reflections of sounds within a room) as per current UK building regulations for schools b. Auditory training (AT). This aims to improve auditory processing by tapping into the brain’s potential for plasticity, but it may also enhance or facilitate other higher order / top-down processes ENT & audiology news | www.entandaudiologynews.com feature such as auditory attention or memory. Auditory training can be implemented by means of commercial computerised game type programmes, or conducted in the form of ‘games’ or exercises and can be aimed at improving specific deficits detected on testing for example interhemispheric exercises, phonemic discrimination c. Compensatory linguistic and other strategies may also be beneficial. However, although there is some initial evidence that such interventions help both children and adults with APD, there is a great need for robust randomised controlled trials to assess whether a specific type of intervention is more beneficial than other types of non-specific input. What are the priorities for the future? There is a pressing need for reliable diagnostic tools and for uniform diagnostic criteria for APD in order to facilitate research, which would translate into evidence-based clinical practice (Bamiou and Luxon, 2008).14 This has been acknowledged by several multiprofessional consensus conferences, both in the UK and abroad (for example BSA 2007, ASHA 2005). In the UK, the British Society of Audiology established a multidisciplinary APD Interest Group in October 2003 with an elected steering committee (of which the first author is current chair and the second author is past chair), which aims to define guidelines and identify research needs via a multidisciplinary forum (please see the website on how to become involved if interested15). The clinician who deals with the assessment of suspected APD cases is faced with challenges and scientific uncertainties. However, this relatively new field is rapidly expanding, as basic scientific findings are being translated into clinical practice, and clinical questions and ambiguities are identified and addressed by scientific studies. n References 1. Nelken I. Processing of complex sounds in the auditory system. Curr Opin Neurobiol 2008 (Epub ahead of print) 2. Winer JA. Decoding the auditory corticofugal systems. Hear Res 2006;212:1-8. 3. Fritz JB, Elhilali M, David SV Shamma SA. Auditory attention —focusing the searchlight on sound. Curr Opin Neurobiol 2007;17:437-55. 4. Auditory Processing Disorder (APD) Steering Committee British Society of Audiology. Interim Position Statement on APD [http://www.thebsa.org.uk/apd/BSA_APD_Position_statement_Final_ Draft_Feb_2007.doc] 5. American Speech and Language Hearing Association. Central auditory processing: current status of research and implications for clinical practice. Am J Audiol 1996;5:41-54. 6. American Speech-Language & Hearing Association (ASHA), 2005. (Central) Auditory Processing Disorders. http://www.asha.org/members/deskref-journals/deskref/default 7. Audiology Illustrated [http://www.audiologyillustrated.org/] 8. Auditec [http://www.auditec.com/] 9. Tartan Products [http://www.capdtest.com/default.cfm] 10. Peretz I, Champod S, Hyde K. The Montreal Battery of Evaluation of Amusia. Ann NY Acad Sci 2003;999:58-75. 11. GIA Publications [http://www.giamusic.com/products/P-musicaptitudeprofile.cfm] 12. Griffiths TD, Dean JL, Woods W, Rees A, Green GGR. The Newcastle Auditory Battery (NAB). A temporal and spatial test battery for use on adult naive subjects. Hear Res 2001;154:165-9. 13. Bamiou DE, Campbell N, Sirimanna TS. Management of Auditory Processing Disorders. Audiological Medicine 2006;4:46-56. 14. Bamiou DE, Luxon LM. Auditory processing disorders – an editorial. BMJ 2008;337:a2080. Forward Features for 2012 January | February SPECIAL FOCUS The Voice AUDIOLOGY MATTERS New Perspectives in Ménière’s Disease March | April SPECIAL FOCUS Implantable Devices AUDIOLOGY MATTERS Ototoxicity May | June SPECIAL FOCUS Sinus/Allergy AUDIOLOGY MATTERS Hyperacusis July | August SPECIAL FOCUS Endoscopic Ear Surgery and Tele-health AUDIOLOGY MATTERS Tele-audiology September | October SPECIAL FOCUS The Thyroid and Parathyroid AUDIOLOGY MATTERS Diagnostics November | December SPECIAL FOCUS Laryngeal Cancer AUDIOLOGY MATTERS World Audiology Topics are subject to change. 15. British Society of Audiology [http://www.thebsa.org.uk/] ENT & audiology news | www.entandaudiologynews.com 71 Auditory Processing Disorders in the Educational Environment Pauline Grant, Advisory Teacher of the Deaf, London Borough of Harrow. ncreasing numbers of children are being identified with auditory processing diffi- I culties and there is currently a degree of uncertainty about how they can be best Correspondence Pauline Grant, Teachers’ Centre, Tudor Road, Harrow, HA3 5PQ, UK. Email: pauline.grant @harrow.gov.uk Declaration of Competing Interests None declared. supported in school – and indeed, who should be responsible for providing it. In my local authority of Harrow, Greater London, teachers of the deaf have taken the lead and offer training and support to schools. We also acknowledge the importance of working closely with our colleagues in speech and language therapy and educational psychology and we are working together to produce an information leaflet which will explain the condition, offer general teaching and learning strategies and explain our different support roles. This leaflet will go out to all Harrow schools and perhaps to other professionals – such as GPs. We hope that this will reduce the levels of confusion around referrals, intervention and who is responsible for what. At the moment it is all rather muddled, with schools learning that a child may have a problem from a number of sources. Raising awareness through training is essential and recent local sessions have been very well attended. In October, I offered a Special Educational Needs Joint Initiative for Training (SENJIT) half day on this topic, held at the Institute of Education in London, which attracted almost a hundred delegates representing twenty-five local authorities. Intervention and training in schools It is important that teachers and support staff understand the difference between hearing and listening (auditory processing). Katz, Stecker & Henderson1 defined auditory processing as: “What we do with what we hear – the ability of the brain (the central nervous system) to process incoming auditory signals.” Teachers often say to me, “I know he can hear me, so it isn’t as if he isn’t listening!” Figure 1: How do schools find out? 72 ENT & audiology news | www.entandaudiologynews.com feature Hearing is passive, but listening is an active behaviour which takes much longer to develop. Carol Flexer, PhD, states that in normal listening development, it is not until between the ages of eleven and fifteen years that higher level listening skills, such as listening in background noise and filling in the gaps of missed auditory information, develop. In other words, children’s listening skills are different to adults. I am indebted to a young man I was asked to see around fourteen years ago. He was thirteen and his teachers thought he had a hearing loss. He didn’t, but he clearly had real trouble listening in conflicting background noise. He sparked my interest in auditory processing disorders (APD) and I still use his definition of his own auditory processing problems: “Miss – I’m fine in here with you, it’s quiet. But in the classroom, sometimes it’s like I’m listening to scribble.” For children with APD, hearing is not the problem; but listening is, and some listening situations will always cause difficulty. The educational implications may be devastating. In a mainstream school, listening is the primary method by which children absorb new information – it is the cornerstone of learning. Yet schools can be very noisy places – particularly in the early years, when we expect children to lay the foundations of reading (phonics) and to learn to listen to the teacher and each other – often in conflicting background noise greater than 80dBA. Prof Bridget Shield (South Bank University) and Prof Julie Dockrell (Institute of Education, London) are researching the effects of noise conditions on academic performance in London primary schools. They have found that performance in verbal and speed tasks is significantly reduced in ‘babble’ conditions. Table 1 illustrates the difficulties in a typical classroom caused by a diminishing signal over distance, competing against background noise. Children in the middle or back rows will miss important information spoken by the teacher. Schools have a responsibility to provide a good acoustic learning environment, but, unfortunately, this is not always the case. Although the basic acoustic requirements for a school are met, they are not wholly satisfactory, even in new builds. In many schools, children are taught in mobile classrooms or classrooms with high ceilings, hard surfaces and lots of glass, resulting in high levels of reverberation – a cacophony of sound bouncing around the room creating a stressful environment for teacher and pupil. It is no wonder that many children either ‘switch off’ because listening is too tiring, or else behave poorly because they miss important information and fall behind. Teachers of the Deaf have always known the importance of good classroom acoustics for children with a hearing loss – if it is too noisy, they cannot learn to listen effectively through hearing aids or cochlear implants. But a good listening environment is essential for all children – not just those with APD or deafness. The National Deaf Children`s Society has produced a useful handbook – ‘The Acoustic Toolkit’ which provides an overview for non specialists on the school listening environment – as well as more technical advice aimed at teachers of the deaf, educational audiologists and acoustical engineers.2 Our responsibility as educators is, first and foremost, to acknowledge the problem. Once students realise that his / her difficulties are understood and that there are strategies that will help them minimise the effects of APD, they become more secure learners. It is not until between the ages of eleven and fifteen years that higher level listening skills, such as listening in background noise and filling in the gaps of missed auditory information, develop What does educational intervention need to address? Dr Dilys Treharne (Department of Communication Sciences at Sheffield University) describes the following areas: ● Separating a target sound from background noise ● Auditory closure ● Memory: sequential and processing ● Recognising sounds ● Sequencing verbal concepts ● Rhythm / pattern perception and prosody ● Processing at speed ● Integrating speech with movement So, for example, children who have difficulty separating a target sound from conflicting background noise, will find it hard to function in classrooms that have poor acoustics, or have a persistent buzz from overhead projectors or computers. Environmental noises from outside the classroom – grass cutting or traffic – may be so distracting that it is impossible to focus on what the teacher is saying. Similarly, during group work, it may be impossible to take part in or understand a discussion against the general babble. Students can be helped to develop this skill by being asked to follow directions in different listening environments. Increasing levels of background noise can be introduced – for example low level music or radio talk shows. Young children might be asked to follow directions to complete a picture, older students will be asked to show his / her comprehension of a piece of text read to them while background noise is present. Without this skill, note taking in high school or college will be impossible. In Harrow, we advise schools on activities to address particular areas of difficulty that can be carried out by a teaching assistant for a short period each day. We also provide parents with some games and activities that can be done at home. The activities should be enjoyable and not cause additional anxiety – there must be no sense that the child Table 1: Illustrates the difficulties in a typical classroom caused by a diminishing signal over distance, competing against background noise. Average classroom noise – 50 – 70 dBA Adults need a +6dB advantage. Young children need +16dB Distance from teacher 6 feet 12 feet 24 feet Teacher’s voice 65dB 59dB 53dB Noise 60dB 60dB 60dB Signal to noise +5dB -1dB -7dB Dr Tony Sirimanna – Great Ormond Street Hospital, UK. ENT & audiology news | www.entandaudiologynews.com 73 feature has ‘failed’. Many schools will already have resources to develop listening skills which are perfectly suitable and can be adapted to meet individual needs. It is also helpful if the child is ‘cued in’ to the lesson (what the lesson will be about and the sequence of the tasks to be set). Teachers often put this on the board, which is excellent practice. Many children also find it helpful to be given a glossary of terms and vocabulary for each subject so that they are prepared in advance for unfamiliar words; their meaning, their sound, how they look on the lips and what they look like on the page. This will give them confidence to participate in the lesson and not feel that they are in a constant state of ‘catch up’. General class management advice is offered: ● ● ● ● ● Preferential seating Encouraging ‘good listening, good looking’ Checking understanding Written information to consolidate verbal instructions Reducing background noise. Rooms with poor acoustics can be improved by carpets, wall hangings and displays, cork notice boards, soft reading areas with bean bags and making sure that rubber tips are in place on chairs and tables. ● Making sure that lip reading conditions are favourable. Once strategies are in place and there is a high level of awareness of APD in the school, it may be appropriate for the child to try an assisted listening device. This is a discreet ear level fm receiver that is fitted to the child’s ear – usually the right. The teacher wears a transmitter and microphone so that his / her voice is transmitted directly to the child’s ear and the effects of background noise are minimised. It is fairly costly and there is some debate about who should fund it, but some parents have purchased it and it may also be possible to have one on loan to try. Some schools opt to fit classroom sound field systems. Again the teacher wears a microphone and fm transmitter, which transmits to four speakers in each corner of the room. These can improve listening conditions for all the children, but they only work optimally if the room acoustics are fairly good to start. If the acoustics are poor, then the voice coming out of the speakers simply adds to the general noise in the room. In summary, strategies and listening exercises, together with sensitive handling of the effects of APD will certainly help, but the message needs to be sent loud and clear to education authorities that schools must ensure that they provide a good listening and learning environment. Without it, children will not develop good listening skills, they will miss information, fall behind and risk educational failure. The issue of poor acoustics in many classrooms – particularly in old buildings or ‘temporary’ mobile classrooms – needs to be addressed as a matter of urgency. Not just for those with APD, but for all children. To quote John Erdreich, PhD: “We would never teach reading in a classroom without lights. Why then do we teach in acoustical darkness? Speaking to a class, especially of younger children, in a room with poor acoustics, is akin to turning out the light.” n References 1. Introduction to central auditory processing. In Central Auditory Processing: a transdisciplinary view Edited by: J Katz, NA Stecker, D Henderson. Mosby Year Book Inc; St Louis, USA: 1992. 2. National Deaf Children’s Society [www.ndcs.org]. Would YOU like to be a Journal Reviewer for ENT & audiology news? If you would like to join our panel of experts and review the latest ENT and audiology research, we want to hear from you! Please email your CV to our Editorial Co-ordinator, Joe Crossland, and outline the areas in which you would like to review, or the specific journals you are interested in reviewing. We are also interested to hear from our readers if there are: • • any areas / journals not covered at present any journals deserving more reviews per issue We look forward to hearing from you! [email protected] www.entandaudiologynews.com 74 ENT & audiology news | www.entandaudiologynews.com The Challenges Ahead in Paediatric Audiology Jane R Madell, PhD, CCC A/SLP, ABA, LSLS, Cert AVT Director, Hearing and Learning Centre and Co-Director, Cochlear Implant Centre. here are a number of challenges facing paediatric audiology in the next few T years. Some revolve around changes in the way audiologists are being trained, and others around reimbursement for services. When the decision was made to move to the AuD as the basic degree for audiologists in the USA, many looked Correspondence Jane R Madell, The Ear Institute at The New York Eye and Ear Infirmary, New York City, NY, USA. E: [email protected] Declaration of Competing Interests None declared. forward to what another year of training would provide in increased skills for audiologists. However, the move away from graduate programs which offered degrees in both audiology and speech-language pathology significantly changed the philosophy of many audiology training programs. In many graduate programs, audiologists are getting extensive training in the technical aspects of the profession and less in the habilitation / rehabilitation aspects. As a result, many audiologists are having problems meeting the needs of providing services to infants and children with hearing loss. Working with babies Most audiologists are comfortable using auditory brainstem response (ABR) and auditory steady state response (ASSR) testing to diagnose hearing loss in infants but are not comfortable obtaining behavioural testing for infants. While electrophysiologic testing is useful, it is not a direct measure of hearing. If a hearing loss is identified using ABR, and the need for hearing aids is determined, the audiologist sets hearing aids using real ear measures. While the information provided with real ear measures tells us how much sound is being presented to the ear, it does not tell us what the child is actually hearing. The audiologist has used a protocol that is not a direct measure of hearing (ABR) and an indirect measure of how much sound a hearing aid is providing (real ear measurements) to provide critical information about whether we are providing sufficient Figure 1: Test results comparing BOA, VRA, and conditioned play audiometry for a child at eight weeks, eight months, and 2.5 years. ENT & audiology news | www.entandaudiologynews.com 75 feature Figure 2: BOA evaluation of an eight week old infant identifying a moderate hearing loss and aided thresholds for each ear. auditory access for a child to use audition to develop speech and language. We cannot ask infants to report what they hear so it is essential that audiologists have an accurate way of determining what the child is actually hearing. We cannot use ABR to monitor performance with hearing aids. If we are going to effectively fit infants with advanced technology, we need to be able to measure how well they are hearing with it. Without behavioural testing, it is not possible to determine if the infant is receiving sufficient benefit from the hearing aids. Appropriately fit technology may be the most important thing we can do for the children we work with – ‘what we hear is what we say’. For children over six months of age many audiologists are comfortable using visual reinforcement audiometry to monitor both hearing levels and gain obtained from amplification. But if we are to meet our goals of fitting hearing aids appropriately before six months of age, it will be necessary for audiologists to gain competence testing infants using behavioural observation audiometry (BOA). As cochlear implants (CI) are being fitted at younger ages, with many infants being implanted at six to eight months, we absolutely must know that the child has received a sufficient hearing aid trial to be able to comfortably report that hearing aids are not providing sufficient benefit and that the child is a candidate for a CI. With BOA we can determine if a child is receiving sufficient gain and hearing at sufficiently soft levels. BOA can be accurately obtained by observing changes in 76 sucking.1-3 Figure 1 demonstrates test results obtained for a child using BOA at eight weeks, visual reinforcement audiometry (VRA) at eight months, and conditioned play audiometry at 2.5 years. The results demonstrate that BOA is a reliable test protocol. Figure 2 demonstrates test results for an eight week old who had hearing loss identified by ABR, confirmed using BOA, and benefitted from hearing aids measured using BOA. By using this protocol, the audiologist and the family can feel comfortable that the child is hearing at sufficiently soft levels to enable him / her to develop speech and language skills. Developing the ability to accurately monitor hearing in infants using behavioural techniques can significantly improve the services available to infants. Auditory habilitation / rehabilitation As audiology focuses more on the technical aspects of the profession, fewer audiologists are interested and involved in auditory habilitation / rehabilitation. Auditory habilitation is frequently allocated only a portion of a course in graduate school. Much of audiology training takes place in hospitals where medical audiology is the primary service. Exposure to selection and dispensing of hearing aids and cochlear implants is frequently limited. When audiologists are involved in hearing aid dispensing, patients are frequently fitted and sent off to adjust to the hearing aids or cochlear implants on their own. Children may be referred for speech-language therapy but in many cases, the speechlanguage pathologists are not trained in auditory habilitation. It is assumed that wearing the hearing aids or cochlear implants will be enough to provide auditory access. No matter how good the technology is, children (and adults) with hearing loss are listening through a damaged auditory system. We should not expect that they will develop maximum auditory skills without specific training. They need to be specifically taught to learn to listen and to use auditory information to make fine distinctions between similar sounding phonemes (for example s / sh, f / th). Children who are taught to use audition have been shown to have significantly better speech and language skills than children taught using other methods.4-6 Audiologists need to know more about development of auditory skills so they can monitor the progress of their young patients and either provide appropriate therapy or know when to refer them on to good auditory therapists. For this to happen, auditory habilitation / rehabilitation will need to become a more significant part of the graduate school curriculum and internships in this area will need to be expanded. Staffing in paediatric audiology Staffing in paediatric centres is becoming a concern. There are not a sufficient number of clinicians available to work in the area of paediatric audiology and reimbursement for paediatric audiology ENT & audiology news | www.entandaudiologynews.com feature editorial board Appropriately fitted technology may be the Editor most important thing we can do for the children we work Mr Raymond Clarke, BSc, DCH, FRCS, FRCS (Orl), ENT Consultant, Royal Liverpool University Hospital and Royal Liverpool Children’s Hospital, Liverpool, UK. Email: [email protected] Section Editors and Specialist Advisors Section Editors – Audiology Matters with - what we hear is what we say Dr David Baguley, PhD, MBA, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Cambridge, CB2 2QQ, UK. Email: [email protected] Amanda Casey, Director of Audiology Programmes, Aston University, Birmingham, UK Email: [email protected] services does not necessarily cover expenses of running paediatric centres. Many people graduating with AuD degrees in the USA report that they have spent a significant amount of money getting educated and want to work in a situation in which they can feel comfortable about their earning capacity. This is not likely to happen in a paediatric audiology program and so they are choosing to work in ENT offices or in private practices dispensing hearing aids. Paediatric centres have a difficult time bringing in sufficient money to meet their expenses so salaries are often less that they are in some other practices. This problem is a difficult one to resolve. Audiology services for children take more time and require more staff. Reimbursement for an audiological evaluation is the same for a short test for an adult and a long test requiring two clinicians for a child. It is possible that in some practices where both adults and children are seen this works out, but it does not work out in paediatric centres where all patients require additional time and additional staff. For children identified with hearing loss and their families, having services provided in a specialty centre is often the best option. It is essential that audiologists be reasonably reimbursed for the time and effort involved in providing audiology services to children. Currently, these centres count on fundraising to cover the expenses not covered by insurance reimbursement. In difficult financial times, fundraising may not be adequate. If the problem of providing adequate reimbursement is not resolved, we risk having even fewer centres able to provide quality services for children. The negative impact of this for children with hearing loss is obvious. Paediatric audiology continues to be a most exciting clinical area. We must continue to help it grow. n References 1. Madell J. Behavioral Evaluation of Hearing in Infants and Children. Thieme Medical, New York, USA: 1998. 2. Madell J. Video – Behavioral Evaluation of Hearing in Infants and Children. Thieme Medical, New York, USA: 1998. Dr Gareth Smith, MSc, AuD, Deputy Head Audiology Service Southend University Hospital, Southend, Essex, UK. Email: [email protected] – Book Reviews Mr M Shahed Quraishi FRCS, FRCS(ORL, H&N), Consultant Otolaryngologist, Thyroid & Parathyroid surgeon, Clinical Director for Head & Neck and Special Surgery, Hon Senior Lecturer in Surgical Oncology, University of Sheffield, Doncaster Royal Infirmary, Doncaster, South Yorkshire, UK. Email: [email protected] – Journal Reviews Mr Liam Flood, FRCS, Consultant ENT Surgeon, Middlesbrough, UK. Email: [email protected] – Newsround Dr Sunil Narayan Dutt, MS, PhD, FRCS, DNB, DLO, DORL, Senior Consultant in Otolaryngology and Head & Neck Surgery, Bangalore, India. Email: [email protected] – Trainee Matters Nazia Munir, FRCS, DOHNS, ENT Specialist Registrar, Liverpool, UK. Email: [email protected] Jonathan Bernstein, FRCS, Clinical Research Fellow, Manchester Royal Infirmary and Christie Hospital, UK. Email: jonathan.bernstein@ doctors.org.uk Specialist Advisors – Audiology Prof Mark E Lutman, Prof of Audiology, Southampton, UK. Email: [email protected] – How I Do It Mr Kim Ah-See, MD, FRCS(ORL), Consultant Otolaryngologist, Head and Neck Surgeon, Aberdeen, UK. Email: [email protected] – Internet Review Mr Tunde A Odutoye, FRCSEd, Consultant Head and Neck Surgeon, London, UK. Email: [email protected] Mr David Pothier, TWJ Fellow in Neurotology, University Health Network, Canada. Email: [email protected] – Hearing Industry Karen Finch, RHAD, FSHAA, FRSA, Managing Director, The Hearing Care Centre Ltd, Ipswich, Suffolk, UK. Email: karenfinch@ hearingcarecentre.co.uk – Speech and Communication Mr Paul Carding, PhD, Head of Speech and Voice Department, Newcastle-upon-Tyne, UK. Email: [email protected] New Editor for ENT & audiology news NEWS We are delighted to announce that we have appointed a new Editor and two new Features Editors! An announcement in the magazine will follow in the January/February 2012 issue, but for a sneak peek at our new line-up, visit our website. www.entandaudiologynews.com 3. Madell J, Flexer C. Pediatric Audiology: Diagnosis, Technology and Management. Thieme; New York, USA: 2008. 4. Moog JS, Geers AE. Epilogue: major findings, conclusions, and implications for deaf education. Ear Hear 2003;24:1215-55. 5. Nicholas JG, Geers AE. Effects of early auditory experience on the spoken language of deaf childen at 3 years of age. Ear Hear 2006;27:286-98. 6. Ying E. Speech / language / auditory management of infants and children with hearing loss. In: Pediatric Audiology: Diagnosis, Technology and Management. Thieme; New York, USA: 2008. ENT & audiology news | www.entandaudiologynews.com 77 New Developments in Hearing Aids for Children and Adults Josephine Marriage, PhD, UCL Ear Institute Correspondence Josephine Marriage, UCL Ear Institute, Gray's Inn Road, London, UK. WC1X 8EE E: [email protected] Declaration of Competing Interests None declared. C hanges in hearing aid technology over the past decade have been steady. Hearing research has informed the development of new strategies in hearing aid design to try and overcome the reduced detection and discrimination of sounds by the impaired cochlea. The recognition that basilar membrane compression is compromised in cochlear hearing loss emphasised the importance of compression in amplification. Thus the first compression hearing aids were analogue, for example the kamp, evolving to digitally programmable, and then to fully digital devices. Since digital technology has become the norm, there has been a period of steady improvements in function, represented by increasing the frequency bandwidth for speech amplification, directional microphone technology and noise reduction. These have been positive advances, but not really groundbreaking in terms of changing outcomes and acceptance of hearing aids for either children or adults. Another interesting example of new applications of technology for improving life quality is the development of hearing aids with background music to help reduce tinnitus awareness and situation anxiety 78 However the past couple of years have seen a huge number of new initiatives and developments, with inspired new concepts in amplification. Some of this new creativity has been helped by having common themes with mobile phone technology. These include improvements in miniaturisation and an increased expectation for ease of listening in adverse listening conditions. While it is true that no hearing aid can overcome the distortion that is due to the impaired cochlear hearing mechanism, there is a new creativity in using technology to compensate for historical limitations of hearing aid amplification. As long as hearing aids have been around audiologists have grappled to find the compromise between the occlusion effect arising from a closely fitting earmould, set against the increased susceptibility to acoustic feedback given by a more open fitting. Now with the use of signal processing to control feedback, for example using signal cancellation and inversion strategies, a new generation of open fitting hearing aids are available. Initially these were constrained to use with high frequency hearing losses, but now can be fitted to low, mid and high losses, with some devices successfully being used with severe extents of hearing loss. Open fittings can be done at a single appointment, there is no occlusion effect, the aids are lightweight and come in funky shapes, colours and finishes. They can even curl around inside the concha with a very discrete fitting, or reflect the wearer’s self image as part of a personal or fashion statement. Many of these fittings are equally appropriate for children with specific audiometric configurations, as for adults with acquired high frequency loss. Another hugely innovative development is the synchronisation of binaural hearing aids, using wireless technology to maintain communication for the adaptive function of two hearing aid devices. This gives rise to a more stable representation of the spatial scene for the listener from two synchronised hearing aids. This gives perceptual improvements in speech clarity for the listener, increasing the potential for higher-level auditory processes for localisation and segregation. Wearers report better perception of sound source movement, for example being able to hear an oncoming bicycle from the tyre noise, and being able to judge its distance. Perhaps the most difficult nut to crack has been in developing technologies for improving understanding of speech in noise.1 The irony is that most people first decide to think about a hearing aid because they can’t follow speech in a group of people. They usually can follow one to one conversation. They get a hearing aid that improves their ease of listening in one to one situations but is of little benefit in noisy or group settings. Many noise reduction approaches have been explored and continue to be limited in benefit, partly due to the cochlear processing mechanism. However a different approach has been to improve ENT & audiology news | www.entandaudiologynews.com feature The be by Resound. The water resistant Naida, by Phonak. The Dual and Streamer from Oticon. transmission of signals from a distance, through having integrated receivers for radio aids, wifi and, recently, bluetooth technology. These systems will undoubtedly have new applications that are unfulfilled at the moment but represent potential for the future. A new approach for increasing speech transmission beyond the traditional hearing aid amplification bandwidth is the use of frequency compression. The search for a method of transposing inaudible high frequency speech information into the lower residual hearing range has been pursued for decades (frequency transposition). Recently algorithms have been produced that show clear research evidence for improvements in speech recognition.2 This frequency compression approach is not only beneficial for people with restricted hearing in the high frequencies, but also for flat audiometric configurations. The high frequency cues that fall outside the normal amplification bandwidth therefore have benefits for perception of voiceless fricatives including the allimportant / s / in spoken English.3 The increased speech clarity improves, among other things, the ability to hear the regional accent of the speaker with all the connotations that accent has for relating to the person behind the speech. The effect on localisation skills has not yet been evaluated. This particular innovation has applications for severe and profoundly impaired adults and children, and may be an alternative consideration for some people who were potentially candidates for cochlear implantation. Cochlear implants (CI) have been very effective in developing new ways of extracting a finite number of features of speech to give the extraordinary performance that can be achieved now, when the CI intervention is timely and individualised. Imagine the celebration there will be for an equivalent device that can restore visual perception, as CI have been able to supplement auditory perception. The constraints of the impaired natural cochlea are still the constraints of hearing aid technology. As there is better understanding of the function of the inner and outer hair cells in the cochlea, coupled to creative use of acoustic signal processing, we may expect to see more radical ways of sculpting amplification around the specific impairment of function rather than the profile of the audiogram (for example, Moore, 2004).4 As technology provides new and better solutions for hearing impaired people, ENT & audiology news | www.entandaudiologynews.com more people will use hearing devices and the stigma that was traditionally associated with hearing aids will fade. (Remember when everyone had contact lenses, and now it seems the majority of middle-aged adults wear fashion-statement glasses). Another interesting example of new applications of technology for improving life quality is the development of hearing aids with background music to help reduce tinnitus awareness and situation-anxiety that can exacerbate hyperacusis, or aversion to specific sounds. While these innovations in effective hearing aid technology can provide new hearing opportunities, the challenge for all of us is to use hearing aid technology as part of the wider context of rehabilitation of a hearing impaired individual within his / her own community. This article does not aim to cover all the new innovations in technology that are breaking for hearing aid products at present, it aims to give some examples of the excellent and focused commitment to hearing aid production by the hearing aid manufacturers and their research and development (R&D) teams. One device that has been on the wish list for children and parents for many years is a waterproof hearing aid, that can be used for swimming, water play and beach holidays. An analogue version has been available for many years, but with a linear amplification output and it is no longer available in the UK. Clearly the waterproofing has been shown to be effective, it just needs applying to the better quality modern digital devices that we are blessed with today. Come on you hearing aid manufacturers, see if you can’t sort this out for the next generation of children. They will be able to express their own thanks to you. n References 1. Turner CW, Henry B. Benefits of amplification for speech recognition in background noise. J Acoust Soc Am 2002;112:1675-80. 2. Bagatto MP, Scollie SD, Glista DA, Pasa V, Seewald R. Case study outcomes of hearing impaired listeners using non-linear frequency compression technology. Audiology Online 2008. [http://www.audiologyonline.com/articles/article_detail.asp?article_id =1990] 3. Stelmachowicz PG, Pittman AL, Hoover BM, Lewis DE and Moeller MP. The importance of high-frequency audability in the speech and language development of children with hearing loss. Arch Otolarygol Head Neck Surg 2004;130:556-62. 4. Moore BCJ. Dead regions in the cochlea: conceptual foundations, diagnosis and clinical applications. Ear Hear 2004;25:98-116. 79 Cochlear Implantation in Early Childhood: what is happening in the long term? Sue Archbold, MPhil, The Ear Foundation, Nottingham, UK. Correspondence Sue Archbold, The Ear Foundation, Nottingham, UK. E: Sue@ earfoundation.org.uk Declaration of Competing Interests The Ear Foundation receives educational grants from Advanced Bionics, Cochlear Europe, and MED-EL UK. Note This article is based on a presentation given at the Royal Society of Medicine, February 5th 2009, as the invited Graham Fraser Memorial Lecture. O ver the last twenty years cochlear implantation in children has developed from being considered experimental and highly controversial to being the accepted management for profoundly deaf children. In most developed countries the majority of deaf children now receive implants, and developing countries are making great strides in implant provision too. Over 80,000 children have implants worldwide, and are being implanted increasingly early in life, following progress in screening programmes and the earlier diagnosis of deafness. More recently, bilateral implantation has been increasingly considered as the appropriate way forward for children. As experience has grown, and technology developed, expectations have changed far beyond what was envisaged possible in the early history of childhood implantation. Early expectations in children When implantation began in children in the 1980s, it was largely carried out on those who had been deafened, typically by meningitis, and those who gained no benefit at all from conventional hearing aids. Early expectations were that cochlear implantation would provide environmental sound awareness and cues for lipreading. Outcomes have far exceeded these expectations, providing deaf children with the ability to understand conversation without lipreading, and to acquire new spoken language, thus encouraging implantation in those who were born deaf and in those with complex needs. What do we know of the long-term outcomes in these groups? What issues remain? Outcomes from implantation in children Outcomes in children were initially measured in terms of speech perception and production; little information was initially gathered about the effects of implantation on the development of language, on education and on psycho-social functioning1 and these areas were largely under-researched. However, the effect of implantation in early childhood on these areas takes years to emerge, and is complex to investigate, with many confounding variables. Changing technology and candidacy sets challenges for researchers.2 How to manage the effect of profound deafness from early childhood on the development of language has long been the subject of controversy; whether to use sign language, signed support or spoken language for example. The effect of cochlear implantation on the development of spoken 80 language far exceeded expectations: so much so that two researchers of deaf education who had been conservative about implantation, commented: “Spoken language development of deaf children may be more possible today than ever before. …we are now presented with the opportunity to learn from earlier mistakes and misunderstandings and to synthesise the best ideas from the past with the technological, programming and social advances of today. …….we may finally be able to fulfil the promise of effective support for speech and spoken language with hearing loss.” Marschark and Spencer, 2006, p17.3 Increasingly, implantation in the first year of life is enabling the development of early communication skills and spoken language to those levels expected of hearing children4,5; outcomes not foreseen ten years ago. The effect of this progress on levels of educational attainments, particularly literacy, are now being reported in the long term. Educational outcomes Traditionally, reading has been a challenge for deaf children, and studies show that little has changed since the seminal study of Conrad (1979)6 showing deaf children leaving school with the reading age of an eight year old. A number of studies demonstrate improvements in reading progress for children with implants. For example, Geers7 found over half of children tested aged 8 / 9 years of age, scored within the average range for hearing children (n=181). Vermuelen8 found the reading comprehension of children with implants was significantly better than those without (n=50). Archbold et al.9 found that ENT & audiology news | www.entandaudiologynews.com feature age at implant was a significant factor: those implanted below 42 months were reading at age-appropriate level five years later. Few studies have looked at a broader range of educational outcomes, but Stacey et al.10 in a study of 2,853 children, including 468 with implants, found improvements in educational attainments when the group with cochlear implants were compared with those with hearing aids. Thoutenhoofd,11 exploring comprehensive Scottish data, showed that children with implants educationally outperformed those with hearing aids, particularly in Maths. What about the effect of cochlear implantation on educational decisions of communication mode and educational placement, which have been taken as measures of success? We know that more children with implants go to mainstream schools than to special schools, when compared with groups with hearing aids12 and that children with implants are more likely to use spoken language than to use sign language.13 Watson et al.14 found children implanted young were likely to move to using spoken language, whether or not sign support had been used prior to implantation. She went on to show15 that from responses by 142 parents, 120 indicated a change in communication choice after implantation, with 113 towards spoken language and seven towards signed communication. A further in-depth study by Wheeler et al.16 showed that parents viewed communication as a journey – while the goal after implantation was spoken language most were pragmatic about communication, recognising that some sign or gestural support can be useful at different times. A study of 29 young people aged 13-19 years in the UK by Wheeler et al.16 revealed that 76% considered the cochlear implant helped understanding in the classroom compared with 7% who felt that signed support was useful. All viewed their implants positively, and all but two wore them all the time. Other long-term studies17,18 showed benefits over ten years after implantation in terms of high levels of usage, and reported benefits in spoken language and education. The reality? While outcomes from implantation have far exceeded expectations, there are a few reality checks to be made. Research in outcomes from deaf children is made complex by the lack of heterogeneity in the group, and research into those with implants has an increased number of variables to account for; type of device, the effectiveness of programming for example. Marschark et al.19 commented on the need to control variables such as age at implantation, language and reading skills before implantation, and consistency of implant use. Thouthenhoofd et al.1 made similar comments: the variability in outcomes from implantation is known to be large.2 Comprehensive studies into long-term outcomes from implantation are rare: to date Beadle et al.17 and Uziel et al.18 are unusual in their completeness and length of follow-up. Others report on skewed populations and omit to report on those lost to follow-up. In the study of Stacey et al.10 those with implants were younger as a group and from more affluent families, known factors for success. In Holland, Damen et al.20 found that children with implants did less well than hearing children in mainstream schools, as did Mukari et al.21 In the report of Thoutenhoofd11 those with implants were doing better than those with hearing aids, but not as well as their hearing peers. Similarly, the Vermuelen8 study reported that those with implants were still delayed compared with hearing peers. In the large US Geers et al.7 study, when they retested the group at aged 15 / 16 years of age, some had made little progress in reading skills in the intervening years. They concluded: “early cochlear implantation had a longterm positive impact on auditory and verbal development, but did not result in ageappropriate reading levels in high school for the majority of students.” Geers et al., 200822 At high school level, linguistic demands are high and children with implants may find this challenging, particularly in poor acoustic conditions and demanding learning situations. Increasing numbers of reports reveal difficulties in the more subtle linguistic skills, which are important at this stage. A study asking the young people themselves what they found in secondary schools revealed the challenges they meet in the more demanding conditions they face there. For example, two mainstream students with excellent speech perception scores: “I need one person at a time, just one person at a time not all the same time, who, over my head, I lose control. It is quite hard to work… other people talk too fast, then walk off” Mainstream student (14) “Group work is the biggest pain you could ever possibly imagine. Small group is all right but big group or class discussions they are the worst.” Mainstream student, (14) (Report to RNID, 2009)23 The changing educational needs of those with implants in high school or secondary school do not appear to be being met. Continuing to provide similar support to ENT & audiology news | www.entandaudiologynews.com that previously provided for profoundly deaf children is not appropriate. What are the long-term needs after implantation? In a European study carried out by Archbold & Wheeler (submitted) of the long-term needs of children with implants, training for local professionals and long-term management were by far the most common issues to be raised by parents and professionals. They commented that early support was not continuing, that new teachers need training in managing the technology and how to maximise its use, particularly in mainstream schools. They also had concerns about support into adulthood for children. Comments included: “Need to realise that CI is not a quick ‘fix’ and the children still need support in whatever educational provision they are in whether signing or oral, mainstream or special” “Most day to day support is from parents and teachers – they need to be well informed” “Our son had implant at brilliant centre, and was sent to school with little knowledge… we have been fighting for an education which will help him to develop his CI use best” (Archbold & Wheeler, submitted) A study of parents in the UK revealed similar comments. One parent when asked about the follow-up care after implantation commented: “It is dreadful in my experience. It is incomprehensible that my daughter could receive £60K worth of technology and no clear plan about how to enable her to make best use of it. Complete waste of resources with lots of people 'involved' in her care but doing very little of any use other than endless assessment. Thank goodness for the voluntary sector. CI centre care great at technical support” (Report to NDCS, 2009)24 Comment The long-term outcomes from early implantation in children have transformed the opportunities for developing spoken language and for its consequent effects on education in ways which were unforeseen ten years ago. Evidence about linguistic and educational outcomes inevitably takes years to accrue, and in the meantime technology and candidacy has moved on. The research evidence produced here is based largely on populations which were older at time of implantation than is currently the case, who were using older technology, had less residual hearing and typically were wearing 81 feature Our latest addition only one implant, rather than two. There is every likelihood that future outcomes will be even more encouraging: those involved in cochlear implantation are required to collect rigorous data, collaborating with those in other associated fields such as cognition and linguistics, ensuring that the data collected is robust, inclusive, and long-term. However, this will only be possible when long-term management is secure with an informed community-based infrastructure to support it. n References 1. Thoutenhoofd E, Archbold SM, Gregory S, Lutman ME, Nikolopoulos TM, Sach TM: Paediatric Cochlear Implantation. England: Whurr; 2005. 2. Kirk KI, Choi S. Clinical investigations of cochlear implant performance. In Cochlear implants: principles and practice (2nd edition). Edited by: Niparko JK. Philadelphia; Lippincott Williams & Wilkins. 2009:191-222. 3. Marschark M, Spencer P. Historical and Theoretical Perspectives. In Advances in Spoken Language in Deaf and Hard of Hearing Children. Edited by: Marschark M, Spencer P. New York: Oxford University Press; 2006:3-21. 4. Dettman SJ, Pinder D, Briggs RJ, Dowell RC, Leigh JR. Communication development in children who receive the cochlear implant younger than 12 months: risks versus benefits. Ear Hear 2007;28:11S-18S. 5. Tait ME, Nikolopoulos TP, Lutman ME. Age at implantation and development of vocal and auditory preverbal skills in implanted deaf children. Int J Pediatr Otorhinolaryngol 2007;71:603-10. 6. Conrad R. The Deaf School Child. London: Harper Row, 1979. 7. Geers AE. Predictors of reading skill development in children with early cochlear implantation. Ear Hear 2003;24:59S-68S. 8. Vermeulen AM, Van Bon W, Schreuder R., Knoors H, Snik A. Reading comprehension of deaf children with cochlear implants. J Deaf Stud Deaf Educ 2007;12:283-302. 9. Archbold S, Harris M, Nikolopoulos TP, O’Donoghue G, White A, Lloyd Richmond HL. Reading abilities after cochlear implantation: the effect of age at implantation on outcomes at five and seven years after implantation. Int J Pediatr Otorhinolaryngol 2008;72:1471-8. 10. Stacey P, Fortnum H, Barton G, Summerfield A. Hearing-impaired children in the United Kingdom, I: Auditory performance, communication skills, educational achievements, quality of life, and cochlear implantation. Ear Hear 2006;27:161-86. 11. Thoutenhoofd E. Cochlear implanted pupils in Scottish schools: 4-year school attainment data 2000-2004. J Deaf Stud Deaf Educ 2006;11:171-88. 12. Archbold SM, Nikolopoulos TP, Lutman ME, O’Donoghue GM. The educational settings of profoundly deaf children with cochlear implants compared with age-matched peers with hearing aids: implications for management. Int J Audiol 2002;41:157-61. 13. Archbold SM, Nikolopoulos TP, Tait M, O’Donoghue GM, Lutman ME, Gregory S. Approach to Communication, Speech Perception and Intelligibility after Paediatric Cochlear Implantation. Br J Audiol 2000;34:257-64. 14. Watson LM, Archbold SM, Nikolopoulos TP. Children’s communication mode five years after cochlear implantation: changes over time according to age at implant. Cochlear Implants Int 2006;7: 77-91. 15. Watson LM, Archbold S, Hardie T, Wheeler A. Parents’ views on changing communication after cochlear implantation. J Deaf Stud Deaf Educ 2007;13:104-16. 16. Wheeler A, Archbold S, Gregory S, Skipp A. Cochlear implants: the young peoples’ perspective. J Deaf Stud Deaf Educ 2007;12:303-16. 17. Beadle EAR, McKinley DJ, Nikolopoulos TP, Brough J, O’Donoghue GM, Archbold SM. Long-term functional outcomes and academic-occupational status in implanted children after 10 to 14 years of cochlear implant use. Otol Neurotol 2005;26:1152-60. 18. Uziel AS, Sillon M, Artieres F, Pison JP, Daures JP, Mondain M. Ten-year follow-up of a consecutive series of children with multichannel cochlear implants. Otol Neurotol 2007;28:615-28. 19. Marschark M, Rhoten C, Fabich M. Effects of cochlear implants on children’s reading and academic achievement. J Deaf Stud Deaf Educ 2007; 12:269-82. 20. Damen GW, Langereis MC, Snik AF, Chute PM, Mylanus EA. (2007). Classroom performance and language development of CI students placed in mainstream elementary school. Otol Neurotol 2007;28:463-72. 21. Mukari S, Ling L, Ghani H. Educational performance of pediatric cochlear implant recipients in mainstream classes. Int J Pediatr Otorhinolaryngol 2007;71:231-40. 22. Geers A, Tobey E, Moog J, Brenner C. Long-term outcomes of cochlear implantation in the preschool years: from elementary grades to high school. Int J Audiol 2008;47 Suppl 2:S21-30. 2008; 23. Wheeler A, Archbold S, Gregory S. Supporting Children with Cochlear Implants in Secondary School. Report to RNID; 2009. 24. NDCS report: Children with cochlear implants: what do they need and what do they get? 2009. 82 Now online. This is just the start... 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It only takes one click to find what you’re looking for. www.entandaudiologynews.com ENT & audiology news | www.entandaudiologynews.com New Frontiers: auditory brainstem implant results in adults and children Martin O’Driscoll, MSc, Consultant Clinical Scientist (Audiology) Correspondence Martin O’Driscoll, Head of Audiology, Manchester Cochlear Implant Programme, Ellen Wilkinson Building, University of Manchester, Manchester, M13 9PL, UK. E: martin.odriscoll@ manchester.ac.uk Declaration of Competing Interests None declared. Acknowledgements Many thanks to Lise Henderson, Deborah Mawman, Adam Walker and Prof Richard Ramsden. Background Cochlear implantation is a remarkably effective treatment for many people with severe to profound sensorineural hearing loss. For a successful outcome, the cochlear implant (CI) requires a cochlea structure and an intact auditory (VIII) nerve to allow the electrical signal to be transmitted to the central auditory system. For some causes of hearing loss, the auditory nerve or the cochlea is compromised to such an extent that a CI is not a viable option. The auditory brainstem implant (ABI) has been developed from CI technology and is indicated for totally deaf people who have anatomical abnormalities of the cochlea or dysfunction of the auditory nerve. About 1,000 people have received an ABI worldwide, with the majority suffering from neurofibromatosis type 2 (NF2). NF2 is a genetic condition that affects about one in 40,000 people and is characterised by the presence of bilateral vestibular schwannomas. The presence of these tumours, or the surgery to remove them, usually results in total deafness. Other more recent indications for ABI are for children with cochlear or auditory nerve agenesis or hypoplasia (absent or malformed cochleas or VIII nerves) or for children and adults for whom cochlear implant surgery is complicated by the presence of total cochlea ossification following meningitis.1,2 The Nucleus ABI consists of an electrode array which is paddle-shaped with 21 small disc electrodes (Figure 1). The array is inserted on to the surface of the cochlear Figure 1: The Nucleus ABI internal implant. (Image courtesy of Cochlear Ltd). nucleus of the brainstem in the lateral recess of the fourth ventricle. The device stimulates the auditory pathway at a higher level than the damaged cochlea or VIII nerve. During surgical placement of the ABI, electrodes on the array are stimulated and an electrically evoked auditory brainstem response (EABR) is recorded to aid the surgeon in the optimal placement of the device on the cochlear nucleus. The EABR recorded via ABI stimulation usually consists of anything from one to four peaks (Figure 2). In comparison to a standard acoustic ABR we do not see waves I or II as these are generated within the auditory pathway before the cochlear nucleus. Post-operatively, the ABI is set up in a similar manner to a CI with individual electrodes stimulated to establish threshold (T) and comfortably loud (C) levels of response which are then used to form a MAP or Programme within a conventional speech processor. There are, however, notable differences between the programming of a CI and ABI: Stimulation of the ABI electrodes may produce undesirable side-effects by activating neural tissue that is not auditory. Patients report a variety of non-auditory sensations from dizziness to sensations in muscle groups down the body ipsi-lateral to the side of the implant.1,2 Patient feedback is important in establishing the non-auditory sensations, and electrodes that produce these side-effects are deactivated and not used in the take home MAP. The CI takes advantage of the well defined tonotopic map within the cochlea in which stimulation of electrodes positioned in the basal end of the cochlea will elicit a higher pitch sensation than those positioned in the apical end. In contrast, the tonotopic map within the cochlear nucleus runs parallel and obliquely through the nucleus3,4 and the ABI positioned on the surface does not stimulate neural structures in such a clear, tonotopically ordered way. To optimise the clinical fitting of the ABI, the clinician must rely on the patient to report the relative pitch perceptions produced when each electrode is stimulated in order to create a patient specific tonotopic map. Speech perception outcomes with an ABI are relatively poor compared to those ENT & audiology news | www.entandaudiologynews.com 83 feature Figure 2: Example of an intra-operative, three peak EABR. Figure 3: Environmental sound discrimination with the ABI in adults and older children (n = 23). Boxplots indicating median value with inter-quartile range and the upper and lower range of measures. reported in multichannel CI users.5 Possible reasons for this may be due to the presence of non-auditory side-effects limiting the overall number of channels that can deliver useful frequency information coupled with poor frequency specificity delivered by a restricted tonotopic range. In addition, the debilitating disease process of NF2 may take its toll. Colletti and his colleagues from Verona have described outcomes of ABI in non-tumour patients compared with the traditional tumour (NF2) patients.6 The nontumour patients had post-meningitic cochlea ossification, advanced otosclerosis or post-traumatic avulsion of both cochlear nerves. Some of these subjects achieved good speech discrimination without lipreading and they compared well with the best CI results. The presence of the tumour in NF2 was believed to have damaged 84 specialised cells in the cochlear nucleus important for speech perception leading to relatively poor outcomes in the NF2 patients. Coletti was also the first to report the use of ABI for children born deaf with cochlear nerve aplasia or hypoplasia.7,8 In a series of five children aged between 14 months and four years of age, the ABI was reported to give between eight and 21 electrodes that produced an auditory sensation. The longer term outcome and potential of ABI in these children is not yet reported. Manchester ABI experience: candidacy and device fitting Older children and adults Prof Richard Ramsden performed the first CI in Manchester just over 20 years ago and under his direction the implant programme has grown to support over 1,000 adults and children. The first ABI was performed in 1994 and now Manchester is the largest centre for ABI in the UK supporting 50 older children and adults with the Nucleus ABI system. All but two of these patients have NF2, one has biltateral vestibular schwannoma not related to NF2 and another has otosclerosis with a previous removal of a CI following infection. The patients range in age from 12 years to 73 years with a mean age of 30 years. Nine patients had their implant inserted during removal of their first vestibular schwannoma whilst retaining useful hearing in their contra-lateral ear. Typically, these patients do not use the ABI until the hearing has deteriorated or the tumour removed from the contra-lateral ear. Four of these subjects are now using their ABI. Programming the ABI and fitting the external speech processor occurs when the patient is well enough and about eight weeks post tumour removal. Initial fitting takes place within the hospital clinic. ECG monitoring is performed due to the small risk that heart rhythm could be disturbed by stimulation of the vagus nerve by an ABI electrode. In practice, this has never been observed within our series of patients. The levels of electrical stimulation required to produce auditory sensations through an ABI are higher than that typically found in patients with a CI and wider stimulus pulse widths and greater amplitudes of electrical current are usually seen. Depending on the stimulus levels required, either a Freedom behind-the-ear or SPRINT body worn processor is used. The T and C levels are measured and the patient asked to report any non-auditory sensation. The electrodes with auditory sensations are placed in pitch ranked order which is determined behaviourally using a midpoint comparison procedure.4 Once pitch ranked, the electrodes producing auditory sensations are used in the patient’s take home fitting. Further appointments for fine tuning and intensive auditory rehabilitation take place over a nine month period with annual appointments thereafter. Young children The incidence of cochlear nerve aplasia or hypoplasia is not certain, however, most paediatric cochlear implant teams will have been referred children who have absent cochlear nerves. The only hearing option for these children is an ABI. With the experience gained from both a large paediatric CI and relatively large ABI programme and after careful consideration of the ethical issues involved, the team in Manchester decided to ENT & audiology news | www.entandaudiologynews.com feature the rest. They are, however, patients that report clear and consistent pitch variation across the electrode array. Figure 4: CUNY sentences discrimination in three conditions: lipreading (LR) alone, LR and ABI together and ABI alone in adults and older children (n = 23). Boxplots indicating median value with inter-quartile range and the upper and lower range of measures, except for outliers more than 1.5 box lengths from the box edge. offer ABI to three congenitally deaf children with cochlear nerve aplasia or hypoplasia. The children’s families were carefully counselled regarding the risks of surgery and the uncertainty about whether the ABI could provide sufficient information for the development of speech and language. The children, all boys, received their implants in late 2006 when they were aged between three years seven months and four years nine months. The initial behavioural activation of the ABI for these children was performed with ECG monitoring in a hospital setting by clinicians experienced in programming speech processors for children with cochlear implants. Children were encouraged to stand and they were closely observed to note any body sway, induced nystagmus or signs of discomfort. If a clear behavioural reaction was observed, such as smiling, stilling, looking up or pointing to the ear, a conditioned response using play audiometry was attempted. Where possible, the stimulus level was reduced to find the T level. From experience gained in setting speech processors for children with cochlear implants, the C level was set at or just below the first stimulus level that provoked a clear behavioural response. During this initial programming, bipolar electrode combinations that were found to produce EABR intra-operatively were stimulated. Any electrodes that produced clear non-auditory responses were not used in the final take home fitting for the child. Electrodes were pitch ordered using the theoretical physiological distribution of pitch within the cochlea nucleus with low pitch relating to the proximal end of the electrode array and high pitch to the distal end of the array. Following the initial device fitting, further appointments for fine tuning and habilitation using an audi- tory verbal approach were offered on a regular basis. Outcomes Adults and older children Six patients (12%) experienced non auditory sensations on all electrodes on the array and were not able to use the ABI. On average, the number of electrodes eliciting auditory sensations was 11 (range: 0 to 21). The most common non-auditory sensation was dizziness which was reported by 53% of patients for at least one electrode. Four patients chose to not use their device due to perceived poor sound quality and lack of benefit and five patients continue to have some useful hearing in their contralateral ear. Audiological results are available for 23 patients. Figure 3 shows the results from a standardised, recorded test of 20 environmental sounds in the auditory only condition. Results are displayed over time up to 21 months post activation of the implant and then the best percentage score obtained for patients at any time after 36 months of implant use. Figure 4 shows results from standardised, recorded CUNY sentences in three conditions: lipreading (LR) alone, LR and ABI together and ABI alone. Again, results are presented over time with the result after 36 months representing the best score obtained. It may be seen from both Figures 3 and 4 that most progress with the ABI occurs within the first nine months and that the ABI acts as an aid to lipreading and gives an awareness of environmental sounds. Of interest, however, is that some patients are able to achieve significant open set discrimination with the ABI (note outliers in the 36 m +, CUNY sentences, ABI only). These patients have NF2 and it is not entirely clear why they score so much higher than ENT & audiology news | www.entandaudiologynews.com Younger children The children have had their ABI for just over two years. Parents report that the children use their ABIs on a full time basis and will ask for their speech processors. Parents are able to recognise if there is a fault with equipment through the changes in their child’s responses to sound, but only two of the three children can reliably report when their equipment is faulty. Over time there have been improvements in each child’s auditory awareness but there are marked differences in the auditory abilities of the children. Whilst all three are able to detect the Ling sounds (oo, m, ah, ee, sh, s) and, therefore, have access to all the components of spoken English, their ability to discriminate and imitate these speech sounds varies significantly. The Meaningful Auditory Integration Scale (MAIS) has been administered at regular intervals to monitor the progression in auditory development. The MAIS gathers information from families regarding their child’s early auditory responses through a 10 item questionnaire. Parents are asked to rate the frequency of a range of auditory behaviours from Never = 0 to Always = 4. The maximum score that can be achieved is 40. The scores are outlined in the Table 1. In addition, Child 1 has scored 82% on the Iowa speech perception test at two years post-implant. All of the children use sign language as their main mode of communication and they are educated either in schools for the deaf or hearing impaired units attached to mainstream schools. Child 1 recognises a wide range of environmental sounds and responds consistently to his name. He is able to understand a number of spoken everyday phrases without lip patterns, he uses his voice consistently in conjunction with sign and has a small spoken vocabulary that would be recognisable to listeners familiar with the speech of hearing impaired children. Child 2 responds consistently to a range of environmental sounds and his name. He understands speech only with the support of lip patterns, he uses his voice more consistently in conjunction with sign. The majority of his speech attempts are unlikely to be recognisable without the support of sign language. Child 3 responds consistently to a limited number of environmental sounds (dogs barking, phone ringing). His parents report 85 feature Table 1. MAIS scores over a 12 month period for 3 children with an ABI. 4 months 6 months 12 months Child 1 17 32 40 Child 2 12 19 20 Child 3 15 17 Not available he will turn to voice in low levels of background noise but he does not understand any spoken language without the support of sign. He has become more vocal since receiving his ABI but has no recognisable spoken words. Conclusion Clearly there is only a small number of children with an ABI and we are in the early stages of establishing whether an ABI provides sufficient auditory information to allow the acquisition of meaningful speech and language. The three children presented here may be considered quite old when they had their implant and the outcomes for them are typical of those seen in children who are implanted late with a CI. To achieve the maximal potential with an ABI we will probably need to capture the benefits of auditory plasticity and provide the implant at a younger age. This will, of course, present even greater challenges in the programming of the device. To this end we have been investigating the use of evoked potentials, both brainstem and cortical, to assist in the setting up of the device. To offer the ABI to even younger children will call upon the highest levels of skill and experience from the implant team. Apart from the notable exceptions, the results of the ABI for older children and adults, mostly with NF2, are poor compared with typical results from CI users. It is important to emphasise, however, that from the recipients’ point of view, the ABI is the only hearing option available and the benefits achieved with lipreading and awareness of environmental sounds are regarded as a worthwhile connection to the hearing world. n References 1. Colletti V, Carner M, Miorelli V, Guida M, Colletti L, Fiorino F. Auditory brainstern implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg 2005;133:126-38. 2. Nevison B, Laszig R, Sollmann WP, Lenarz T, Sterkers O, Ramsden R, Fraysse B, Manrique M, Rask-Andersen H, Garcia-Ibanez E, Colletti V, von Wallenberg E. Results from a European clinical investigation of the Nucleus (R) multichannel auditory brainstem implant. Ear Hear 2002;23:17083. 3. Illing RB. Maturation and plasticity of the central auditory system. Acta Oto-Laryngologica 2004;124:6-10. 4. Long CJ, Nimmo-Smith I, Baguley DM, O'Driscoll M, Ramsden R, Otto SR, Axon PR, Carlyon RP. Optimizing the clinical fit of auditory brain stem implants. Ear Hear 2005;26:251-62. 5. Di Nardo W, Fetoni A, Buldrini S, Di Girolamo S. Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation. European Archives Of Oto-Rhino-Laryngology 2001;258:5-8. 6. Colletti V, Shannon RV. Open set speech perception with auditory brainstem implant? Laryngoscope 2005;115:1974-8. 7. Colletti V, Fiorino F, Carner M, Miorelli V, Guida M, Colletti L. Perceptual outcomes in children with auditory brainstem implants. International Congress Series 2004;425-28. 8. Colletti V, Carner M, Fiorino F, Sacchetto L, Miorelli V, Orsi A, Cilurzo F, Pacini L. Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia. Otol Neurotol 2002;23:682-93. product guide 2012 / 2013 The ENT & audiology news product guide is an annual supplement accompanying the May/June issue of ENT & audiology news and distributed worldwide to our readership. It’s an informative listing of products available within ENT, audiology and related fields and is listed by product type for easy reference. Take advantage of an early booking price freeze at just £96+VAT per listing Book before 31st Dec to avoid the price increase and pay 2011 rates Alternatively increase your product awareness with a full page display advert from £1,522+VAT Deadline 5th April 2012 Contact: ENT & audiology news, Tel: +44 (0)131 478 8401, Email: [email protected] 86 ENT & audiology news | www.entandaudiologynews.com An Approach to the Dysmorphic Child with Deafness Melissa M Lees, MBBS, DCH, MRCP, FRACP, MSc, MD, Consultant in Clinical Genetics Correspondence Melissa M Lees, Great Ormond Street Hospital NHS Trust, Great Ormond Street Hospital, London, WC1N 1EH, UK. E: Melissa.lees@ gosh.nhs.uk A child with a hearing loss may have a number of unusual clinical features, which may help to identify the aetiology of the hearing loss. These features may form part of a recognisable pattern of anomalies, known as a syndrome. There are over 200 syndromes with deafness as a feature listed on the Winter-Baraitser Dysmorphology Database (WBDD). Alternatively, the features may not be recognised as part of a known condition, and may be the result of an underlying chromosome imbalance, congenital infection, teratogenic effects or other unspecified cause. down a Dysmorphology is the study of physical characteristics in order to diagnose an underlying disorder. Reaching a diagnosis gives the family and health professional an insight into the natural history of that condition, information regarding appropriate monitoring, management and the inheritance pattern, in addition to access to patient or parent support groups. While technology in the genetic laboratories is increasingly improving our ability to make a precise syndromic diagnosis, the diagnosis is usually clinically made or at least suspected, with expensive genetic tests supporting the clinical diagnosis only where indicated. Although the clinician may recognise that the child or individual may appear ‘different’ from other family members, characterising the features can be difficult. This article aims to help guide the clinician through the somewhat daunting task of assessing the dysmorphic child with deafness. particular History Declaration of Competing Interests None declared. Whilst the physical signs might lead you diagnostic pathway, the findings must be taken in conjunction with the history of prenatal, prenatal and postnatal factors As with the majority of disciplines within medicine, the evaluation of a child with deafness and dysmorphic features starts with a thorough history. A detailed family history should include three generations where possible, with specific questions to determine whether relatives may be similarly affected or have more subtle signs of a related condition. For example in Stickler syndrome where the proband may have a sensorineural hearing loss, the mother may have had a cleft palate repair. It is helpful to document any consanguinity, as rare recessive conditions are more common in the offspring of a consanguineous union. Information regarding the pregnancy should be sought, specifically asking about drugs, medications and alcohol (teratogenic effects), maternal diabetes or epilepsy, fevers and rash (possible congenital infection), or pv loss (vascular compromise in hemifacial microsomia). Information about the delivery including mode, gestation and size of the baby are relevant. Perinatal birth asphyxia, prematurity and sepsis (meningitis, ototoxicity of aminoglycosides) may all contribute to hearing loss in a child. Developmental delay is commonly found in children with dysmorphic features and a careful developmental history should be taken to try and establish whether developmental delay is present, and if so whether this is global, affecting all areas of development, or more specific such as with speech or motor development. Poor head control and delayed walking may be early signs of vestibular dysfunction, and therefore very relevant in a child with a hearing loss. Depending on the age of the child, the progress of the individual at school is important, noting any additional support received within the educational environment. Information on the social integration and behaviour is helpful. Significant medical events such as an admission to hospital for meningitis are clearly fundamental to a thorough history. Physical examination Facial gestalt Sometimes a child may present with easily and quickly recognisable features, such as the facial features seen in trisomy 21, where the diagnosis can be made by the assessment of the overall picture of the face. This is also known as the ‘facial gestalt’. More commonly, however, although there may be unusual features present, the clinician will need to work through the features individually, before being able to put the various findings together to consider if a common link can be found. ENT & audiology news | www.entandaudiologynews.com 87 feature Head and neck The head should be measured and the shape evaluated, by looking from all angles, including from above. An unusual head shape may suggest premature fusion of the cranial sutures (craniosynostosis syndromes include Apert, Crouzon, Saethre-Chotzen syndromes). Microcephaly is seen in many conditions including congenital infections. missing teeth, which may suggest an ectodermal dysplasia. Is there gum hypertrophy? Is there a cleft palate (Treacher Collins, some craniosynostosis conditions). Neck It is important to look at the neck for any remnants of a branchial sinus (branchio-otorenal syndrome) or thyroid enlargement (Pendred syndrome). Face Does the face appear symmetrical (hemifacial microsomia)? Can the face be described as long, round, or triangular in shape? Is there undervelopment of part of the face such as hypolastic zygomatic arches in Treacher Collins syndrome, or mid-face hypoplasia (Stickler syndrome)? The shape of the forehead is important when considering certain conditions for example skeletal dysplasias. Ears Do the ears appear normally formed and positioned? Are they simple in shape or particularly prominent or cupped? Are the lobes unusual? Note the presence or otherwise of preauricular pits or tags, post-auricular skin defects (branchio-oculo-facial syndrome), and ear creases (Beckwith Wiedemann syndrome). Eyes When looking at the eyes the following should be considered: do the eyes appear close together or widely spaced, are the globes prominent or do the eyes appear small, are epicanthic folds present, do the palpebral fissures slant up or down, do the eyebrows meet or are they sparse, are there colobomas of the iris or eyelid, are the eyes the same colour? An example of the importance of the eyes is in the diagnosis of the type of Waardenburg syndrome. White forelocks and heterchromia are common to both type 1 and 2, but synophrys and telecanthus (lateral displacement of the inner canthi) are seen in type 1. Nose Is the nasal bridge wide or flat, or high? Is the tip of the nose broad or upturned, or bifid? Looking at the philtrum – is this long, smooth and featureless, or are the philtral pillars wellpronounced? Growth Evaluation of growth is an important part of the assessment of any child with dysmorphic features. If short, is this disproportionate with short limbs? Skin The skin provides many clues towards underlying aetiology of a complex disorder. Areas of hypopigmentation may be seen in Waardenburg syndrome, café au lait patches in neurofibromatosis, and multiple lentigines in Leopard syndrome. Digits The hands again provide many diagnostic clues. Palmar creases may be single (trisomy 21) or deep (mosaic trisomy 8). The digits may be short (skeletal dysplasias) or long (22q11 deletion). Fifth finger clinodactyly is present in trisomy 21, Silver-Russell syndrome. Fingers or toes may be webbed and the pattern of the webbing may be important for example 1/2 toe webbing in triploidy, 4/5 syndactyly in oculo-dental-digital syndrome. Are the joints hyperextensible, seen in Stickler syndrome, or are there joint contractures (facio-audio-symphalangism). Further evaluation Clinical photographs are an extremely useful tool in the assessment of a dysmorphic child, as these may be further assessed outside the clinic setting, shared with other colleagues, presented at a conference or to a web-based discussion board for a wider diagnostic opinion, and comparison to published cases of overlapping conditions. Many facial features are part of a continuous trait such as distance between eyes, Assessment pathway Three generational pedigree Mouth Pre- peri- and postnatal history Is the mouth wide (macroglossia may be part of branchial arch defects)? Is there evidence of a cleft lip repair? Are the lips thin (fetal alcohol syndrome) or full and everted (Coffin Lowry sydrome), or is the cupids bow prominent? Are the teeth normally formed or are there small, underdeveloped, unusually shaped or Developmental history 88 Physical examination including growth parameters Clinical photographs Books, literature and web-based resources broadness of forehead, and so on, and comment on these may be subjective. However there are standard values for many measurable features, which can then be plotted on a chart to determine the significance, although in a busy clinical this may not be practical. Whilst the physical signs might lead you down a particular diagnostic pathway, the findings must be taken in conjunction with the history of prenatal, perinatal and postnatal factors and supported by appropriate investigations. In some cases, where a facial gestalt has been recognised, a clinical diagnosis can be made which may or may not need confirmation by a laboratory test. In many cases further assessments are necessary to gain sufficient information to make a diagnosis. For example an ophthalmic assessment may identify vital clues in the diagnostic search (retinopthy in congenital infections, high myopia in Stickler syndrome, coloboma in CHARGE), a renal ultrasound scan in a patient and sometimes other family members may confirm a clinically suspected diagnosis of branchio-oto-renal syndrome, or an echocardiogram where a clinical diagnosis of Noonan syndrome is being considered. Interpretation of features When faced with a child with complex difficulties, in spite of an excellent and detailed history and documentation of the physical features, no diagnosis may have come to light. This may be because the collection of signs and symptoms do not fall within a recognised pattern, and may represent a subtle chromosome imbalance or a ‘new’ syndrome. Alternatively the features may be part of a recognised pattern but one which is not familiar to the clinician, and in this situation the information needs to be further evaluated using additional resources. There are a number of excellent reference books, where the chapters are divided according to the main features of the group of conditions, such as external ear malformations. The physical differences identified can also be entered onto a searchable database such as the WBDD. The various ‘handles’ may lead to a number of suggested diagnoses, which match to some extent the features identified, which can then be evaluated individually on their own merit as to whether the patient may or may not have the condition in question. Some features may be considered strong ‘handles’, where the particular feature is only present in a few conditions for example heterochromia iridis, where others are more non-specific for example single palmar creases, and therefore less diagnostically helpful. A number of key features can be ENT & audiology news | www.entandaudiologynews.com feature searched on, making any ‘strong handle’ a mandatory feature. Using such a tool, a number of diagnostic suggestions are made, which the clinician can consider in turn, deciding whether it remains a diagnostic possibility. If a specific syndrome is being considered, greater evaluation of the literature may be necessary, which may help to clarify whether this is indeed the correct diagnosis or whether additional investigations may help to confirm or refute the diagnosis. Unfortunately this is not currently freely available but may be available within your institution. It is important to be aware that making a diagnosis prematurely with insufficient evidence is unwise as it may be incorrect and removing a diagnostic label once applied can be very difficult and distressing for the patient and their family. Genetic testing Any child with deafness with additional features such as dysmorphic features and / or developmental delay should have a karyotype performed. Some centres are now offering array CGH (comparative genome hybridisation) as the first line investigation for chromosomal copy number variation, which identifies smaller areas of chromosome imbalance than was previously available through conventional G-banding techniques. Where a clinical diagnosis is suspected and the molecular basis known it may be possible to confirm the diagnosis by DNA analysis. Each individual case needs to be assessed as to whether the additional information would be beneficial to the health professional and family, as the costs involved are often significant. If the condition is rare there may be a research group working on that disorder which would be happy to receive a DNA sample. When blood is being taken, it is often helpful to store a DNA sample in case additional DNA tests are warranted at a later stage. Role of the clinical geneticist It is often both interesting and challenging for the ENT surgeon and audiologist to try and pinpoint the precise cause of the hearing loss in a child with complex difficulties or dysmorphic features. Often, however, in spite of following the above and obtaining a detailed family history and careful physical examination, no diagnosis has been made. Referral to a clinical geneticist either to confirm the clinical suspicion or to help evaluate the clinical picture further may be appropriate. The geneticist will also be able to suggest appropriate additional investigations, and use their specific expertise in the assessment of a dysmorphic child to help make a diagnosis, in addition to providing the parents with genetic counselling. n Further reading Winter-Baraitser Dysmorphology Database (WBDD), London Medical Databases [www.lmdatabases.com] Syndromes of the head and neck, 4th edition. Edited by: Gorlin RJ, Cohen MM Jr, Hennekam RCM. New York: Oxford University Press; 2001. Hereditary Hearing Loss and Its Syndromes. Edited by: Gorlin RJ, Toriello HV, Cohen MM Jr. Oxford University Press; 1995. Oxford Handbook of Genetics. Bradley-Smith G, Hope S, Firth HV, Hurst J. Oxford University Press; 2009. Website resources Online Mendelian Inheritance in Man (OMIM) provides a catalogue of human single gene disorders [www.ncbi.nlm.nih.gov/omim] GeneTests·GeneClinics provides excellent reviews of a broad range of genetic diseases and syndromes [www.geneclinics.org] National Organization for Rare Diseases (NORD) provides information and links to support groups [www.rarediseases.org] WAIT. . . there’s more! take a look at our website: entandaudiologynews.com a wealth of resources at your fingertips – and all the content is FREE to access JOURNAL REVIEWS Now categorised and online for your convenience, archived from Oct/Nov 2010. Each listing has a link to either the original source or to PubMed. 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By distributing ENT & audiology news our partners can: ● Supply a useful, informative, unbiased and educational resource free of charge and enjoy the goodwill from the profession ● Have direct access to potential clients who get in touch to receive a regular copy of the magazine ● Share their own news by including their own promotional literature with the magazine, direct clients to relevant advertisements in the magazine, or use delivery as an opportunity to arrange a meeting ● Share information on what is happening in their region and act as an ambassador for local ENT and audiology professionals Finally, with a reason to communicate with doctors every two months, our Distribution Partners get a sprinkling of that indefinable magic that has made ENT & audiology news a worldwide success for two decades. When a market is proving ‘hard to crack’, becoming a Distribution Partner of the magazine can change the situation overnight. New partners welcome We particularly welcome enquiries from companies that might wish to become new Distribution Partners, or suggestions from readers about companies who may be suitable candidates for this in their own countries. Distributing such a high profile specialised magazine enabled Jordan Hearing Aids to do much more within its outreach initiatives in an environment where access to specialists' news and trends is challenging. We thank ENT & audiology news for their pivotal role in keeping the ENT care community in touch with the global news. Raed Billeh, General Manager, Jordan Hearing Aids In some countries different companies work together, sharing the readership between them and liaising closely to avoid duplication. In these instances the readers can be split geographically or by professional interest for instance, with one company delivering to otolaryngologists and another to audiologists or hearing healthcare practitioners. Costs of being a Distribution Partner As our Distribution Partners help us to reach the widest possible readership, we do all we can to keep the costs of this service to a minimum, finding the cheapest print and transport costs every time. Magazines can be sent in batches of fifty. The printing cost of fifty magazines is approximately £70 Sterling (GBP), while transport costs vary from country to country. For a quote, please contact Ms Nova McMillan at: [email protected]. A big THANK YOU from the Publisher This feature is an expression of appreciation of all our Distribution Partners, past and present, for their generosity – and sometimes their ingenuity – in ensuring the international circulation of the magazine. If you already avail of this service, maybe you would like to let them know that you appreciate it too. If you do not get the magazine from one of our Distribution Partners, check out the list on page 15 and apply to join a scheme. Or if none exists for your country contact Ms Nova McMillan, Distribution Co-ordinator, to organise a quote, or Mr Doug Elder for a personal subscription. With every issue we include a cover letter detailing our products which are advertised inside, and giving some information on the other ENT products we supply. We are sure the distribution of the magazine has been a considerable help in achieving our aim of becoming the first port of call for Irish ENT surgeons when they are in the market for any new equipment or instruments. Tekno Surgical, Republic of Ireland We choose to distribute the magazine because we want to take on our shoulders the responsibility of community services for people in Kuwait and Oman. One of the most important ways is to increase awareness and knowledge of the medical field. The magazine is one of the best informative and accredited sources and so we decided to sponsor distribution to serve all our ENTs and audiologists. Hassans Optician Co., a distributor since 2003 ENT & audiology news is distributed to physicians and audiologists across Israel with great success. We have been receiving excellent feedback regarding the magazine’s range of topics. The request for new subscriptions continues to grow and for this reason we intend to continue and support its circulation around Israel. Ursula Bayer, MED-EL’s Marketing Project Manager reads the latest issue. ENT & audiology news is highly valued in our team as it gives us an outstanding overview about current developments both in scientific research and in the hearing device industry. The ‘News Update’ section regularly informs us about product news of hearing device manufacturers, the ‘Newsround’ section gives a perfect insight into hearing events all over the world. Steiner Hearing Instruments, Israel ENT & audiology news | www.entandaudiologynews.com MED-EL, Austria 91 distribution Through delivery of the magazine we not only enhance our professional reputation amongst customers and hospital doctors, but we also support development of China’s domestic audiology, to cultivate new audiologists, open their minds and eyes to new areas and the latest technology. Starkey China, a distributor since 2001 ENT & audiology news will help us to grow our businesses. Readers are very positive about the contents of the magazine. There are so many articles and so much information on ENT. The magazine is a very good tool to develop relationships with ENT doctors, audiologists & dispensers. Global otorhinolaryngology information can be easily understood and is found useful – we distribute to ENT Professors at the University Hospital in Osaka. As a result of distributing ENT & audiology news, our sales of hearing aids and otorhinolaryngology inspection machines have gone well. Toshin Jitsugyo Co. Ltd, Japan, a distributor for 10 years Widex India, a distributor since 2001 Our readers declare they are very satisfied with the range of topics which are presented in the publication. It is a well known magazine in medical society and for that reason we intend to support its circulation in Greece. We continually strive to bring new and innovative products to the ENT surgeons. Lanoy Medical has issued complimentary issues to ENT practitioners in South Africa and Namibia for over a decade. The surgeons are kept informed of all that is happening in the field of ENT surgery, and distributing ENT & audiology news keeps us in continual contact with our client base. Lanoy Medical, South Africa Uni-Pharma S.A., Greece a distributor since 2003 Twenty years on Now in its twentieth year, it may come as a surprise for readers to hear that when ‘ENT News’ first launched, we only expected to circulate the magazine to the UK’s ENT profession. Demand quickly arose however from the audiology and hearing healthcare professions, as well as from associated specialists such as oral and maxillofacial surgeons, voice scientists and head and neck cancer nurses. The cross-over between ENT and the related specialties quickly became apparent, and the magazine began to cater to this wider market, ultimately resulting in the magazine’s name change to ENT & audiology news. Growth of the international circulation Within months of the magazine’s launch we began to receive subscription requests by ‘phone, fax and post from specialists based overseas. Equally surprised and delighted by these requests, we soon learned that doctors from overseas would come across the magazine at meetings, or when visiting colleagues in the UK. Its mixture of events, reviews and news of the latest industry developments was a hit with doctors at home and abroad. One Italian doctor, who ‘phoned to arrange a subscription for his friend as a birthday gift, explained that the short reviews and attractive layout 92 made the magazine accessible and easy to read for readers whose first language was not English. The birth of the Sponsored Distribution scheme As processing new subscriptions began to take up more and more resources, a time-saving solution was needed to meet the international demand for the magazine. Many of the early subscription requests came from the Netherlands and from constructive discussions, EnterMed BV became our first official sponsored distributor. This was quickly followed by Widex who have for many years distributed ENT & audiology news far and wide. The resulting contribution to the education of, and communication with ENTs, audiologists and related specialists from Egypt to Hong Kong and from Brazil to Libya has been invaluable, and has also ensured the goodwill of the profession towards those that distribute the magazine around the globe. In the following months companies throughout Europe, The Middle East, Australia and New Zealand came on board to replicate the scheme. The cumulative result today is that ENT & audiology news has the largest international circulation of any magazine in its field. n ENT & audiology news | www.entandaudiologynews.com international circulation ENT & audiology news now averages 18,500 readers worldwide thanks, in part, to the following companies who sponsor distribution of the magazine to the profession outside the UK. Individual readers based in the countries listed below may apply for a complimentary copy of the magazine from one of our Distribution Partners. Log onto www.entandaudiologynews.com for direct links to the companies listed below. Australia HAC Acoustic Technologies, Libya South Africa Arthrocare (Australasia) Pty Ltd, TF3 Kokesh Tower, 18 Kodambakkam High Road, Chennai, India, 600034, Email: [email protected] Widex Libya, Lanoy Medical, PO Box 10, Jaafer Ben Abitaleb St, Menshia 7, Tripoli, Libya. Tel: +218 927310296. PO Box 3342, Halfway House, 1685, Johannesburgh. Tel: +27 (0)11 466 1820, Fax: +27 (0)11 466 1825, Email: [email protected] Building 3, Unit 3, 49 Frenchs Forest Road, Frenchs Forest, NSW 2086, Australia. Tel: +61 2 8422 1100, Fax: +61 2 8422 1102, Email: [email protected] Austria Med-El Medical Electronics, Furstenweg 77a, A-6020, Innsbruck, Austria. Tel: +43 (0)512 28 88 89 0, Fax: +43 (0)512 29 33 81. Bahrain Delmon Optical and Audiological Centre, PO Box 5201, Manama, Bahrain. Tel: +973 17 294171, Fax: +973 17 294476. Brazil Indonesia Melawai Hearing Aid, Jalan Melawai Raya 192, Jakarta Selatan, Indonesia. Tel: +62 21 530 6956 or +62 23 532 1111, Fax: +62 21 548 0677. Web: www.widex.co.id or www.melawai.com Iran Fann Azarakhsh Co Ltd, Malta No6, 2nd Floor, 15 Ave, Gandi St, Tehran, Iran. Tel: +98 21 88 77 12 83, Fax: +98 21 88 77 8473, Email: [email protected] Ireland Rua 24 de Maio 77-2, Amdarconj 202, Villa Buarque, Sao Paulo Cep 01041-907, Brazil. 10 Fonthill Business Park, Dublin 22, Ireland. Tel: +353 1 675 4800, Email: [email protected] Starkey (Suzhou) Hearing Technology Co, Ltd. C2,128 Hongye Road, Suzhou Industrial Park, Jiangsu Province, 215006. Email: [email protected] Tekno Surgical Ltd, Israel Steiner Hermina Hearing Instruments Ltd, Croatia 10 Abbas St, Haifa 31094, Israel. Tel: +972 485 215 14, Email: [email protected] Bontech Research Co doo Italy A.B.Simica 48, 21000 Split, Croatia. Tel: +385 21 371 505 Email: [email protected] Web: www.bontech.hr Seda S.p.a. Cyprus Euthymiades Audiology Centre Ltd, Euthymiades Building, 8, Methonis, 1070 Nicosia, Cyprus, Tel: +357 22 377177, Fax: +357 22 377161, Email: [email protected] Web: www.euthymiades.com.cy Denmark Widex A/S -Via Tolstoj, 7, 20090 Trezzano sul Naviglio (MI), Italy. Tel: +39 02 48 42 41, Fax: +39 02 48 42 42 90, Web: www.seda-spa.it Japan Starkey Japan Co, Ltd. 5-2-20 Nakamachidai, Tsuzuki-ku, Yokohama, Kanagawa 224-0041. Tel: +81 45 942 6593, Fax: +81 45 942 7158, Email: [email protected] Web: www.starkey-japan.co.jp/ Toshin Jitsugyo Co Ltd, Nymoellevej 6, DK-3540 Lynge, Denmark. Tel: +45 44 35 56 00, Fax: +45 44 35 56 01. 2-4-7 Nishihonmachi, Nishi-Ku, Osaka, 5500005, Japan. Egypt Azuma Hearing Aid Center Co, Ltd, Widex Egypt, 25 El-Khalifa El-Maamon St, 2nd floor, Flat No 11, Roxy, Cairo, Egypt. Tel: +202 2918421, Fax: +202 6905758. Greece Uni-Pharma SA, 14 KLM National Road, 145 64 Kifissia, Athens, Greece. Tel: +30 210 8072512 or +30 210 8072534, Fax: +30 210 8078907, Email: [email protected] Hong Kong Widex Hong Kong Hearing & Speech Centre Ltd, Room 1101-2, Hang Shing Bldg, 363 Nathan Road, Yaumatei, Kowloon, Hong Kong. Tel: +852 2771 0501, Fax: +852 2388 4627, Email: [email protected] India Widex India Private Limited, DLF Infocity, Tower 'D', 2nd Floor, Chandigarh Technology Park, Chandigarh,-160 101 (INDIA), Tel: + 91 172 5000201, Fax: + 91 172 5042075, Email: [email protected] Top Hearing Centre LOT SB6 to SB9 Lower Ground Floor, Bangunan Cahaya Suria, Jalan Tun Tan Siew Sin (Jalan Silang), 50050 Kuala Lumpur, Malaysia. Tel: +60 3 207 80204, Fax: +60 3 207 23632, Malaysia: Email: [email protected] or [email protected] Web: www.widex.com.my Centro Auditivo Widex-Brasitom Ltda, China Malaysia 2-2-18Sakai-Cho, Niihama, Ehime 7920812, Japan. Tel: 0897 35 2277. Beacon Healthcare Ltd, ‘Sea Breeze’, Qawra Coast Road, Qawra SPB 05, Malta. Tel: +356 576 171, Fax: +356 419 300, Email: [email protected] New Zealand Widex New Zealand Ltd, PO Box 46-323, Herne Bay, Auckland, New Zealand. Tel: +64 9 360 3412, Fax: +64 9 360 3426, Email: [email protected] Nigeria Otana Hearing and Edu-Health Services, Otana House, 69 Wawuna Street (off Obi Wali Road), Rumugibo, PO Box 8867, Port Harcourt, Nigeria. Tel: +234 803 339 7678, Fax: +234 803 505 6602, Email: [email protected] Sri Lanka D S Jayasinghe Opticians (Pvt) Ltd, 41 Symonds Road, Colombo 10, Sri Lanka. Tel/Fax: +94 1 449780 or +94 1 446518, Email: [email protected] Sudan Sudanese Hearing Centre, Altayar Gameel Street Cross al said Abdel Rahman Square 6 Home 3, PO Box Albarari Khartoum, Albarari. Tel: +249 11 79 76 78, Fax: +249 11 79 67 67, Email: [email protected] Taiwan Melody Medical Instruments Corp, 2F-1, No191, Section 4, Chung-Hsiao East Road, Taipei, Taiwan 106. Tel: +886 (2) 2741-9117 x216, Fax: +886 (2) 2741-9139, Email: [email protected] Pakistan Thailand Rehabilitation Centre for Hearing Impaired, D MED Hearing Center Co, Ltd. Ghafoor Plaza, 289 Ferozepur Road, Lahore, Pakistan. Tel: +92 42 588 3773, Fax: +92 42 585 5585, Email: [email protected] Web: www.widex.com/pk Phillippines Ledesma Audiological Center, Lower Level Makati Cinema Square, Pasong Tamo Street, Makati City, Phillippines. Tel: +63 2 818 8564, Fax: +63 2 811 1255, Email: [email protected] Web: www.ledesma.com.ph Portugal Casa Sonotone, 326,326/3-4 Sukhothai Rd. Suanchitladda, Dusit, Bangkok 10300. Tel: +66 (0)2668 1300 3, Fax: +66(0)2668 1304. Email: [email protected] Web: www.widex.co.th or www.dmedhearing.com Turkey Starkey Turkey – Golden Star Ltd, Halaskargazi Cad, Teyyareci, M Alibey Sk. No1/2 80260 Sisli, Istanbul, Turkey. Tel: +90 212 230 4532 or +90 212 232 1755 or +90 212 232 3287, Fax: +90 212 230 4502. PO Box 2208, Amman 11181 – Jordan, 4th Circle, Ibn Khaldoun St, Building 77. Tel: +962 6 461 1331 Praça da Batalha 92-1, 4000 Porto, Portugal. Tel: +351 223 3930 60, Fax: +351 223 323 091. Acustica Medica Rua Conde De Arnoso No 5-2 Piso, 1700-112, Lisbon. Kenya Romania Widex Emirates Hearing Care, Beam Hearing Centre, Sonorom SRL, Adalyn flats – 4B, Ngong’ Roads, PO Box 72073, Nairobi, Kenya. Tel: +254 2 722 861, Email: [email protected] Str. Unitatii 130, Bloc B4, Ap. 106, Sector 3, Bucuresti 74355, Romania. Tel: +40 1 337 0942/335 7562, Fax: +40 1 337 0942, Email: [email protected] PO Box 26402, Sharjah, United Arab Emirates. Tel: +971 6 5744734, Fax: +971 6 5744735, Email: [email protected] Saudi Arabia Vietnam Jordan Jordan Hearing Aids Man Co, Korea Starkey Korea, 656-766 Sungsoo, 1-Ka 2-Dong, Sungdong-Ku, Seoul, 133-112, Korea. Tel: +82 2 465 0999, Fax: +82 2 465 1012, Email: [email protected] Web: www.starkey.co.kr Al-Madina Hearing Aids Centre, Kuwait & Oman Slovenia Hassan’s Optician Co, Neuroth sluš ni aparati d.o.o. PO Box 1139, Safat 13012, Kuwait. Tel: +965 242 1818, Fax: +965 242 1815. Tbilisijska 59, 1000 Ljubljana, Slovenia, www.neuroth.si Old Airport Road, PO Box 6812, Riyadh 11452, Saudi Arabia. Tel: +966 1 478 8809, Email: [email protected] ENT & audiology news | www.entandaudiologynews.com United Arab Emirates Quang Duc Hearing Services Co, Ltd. 384 (new no.1056) CMT 8, Ward 4, Tan Binh District, Ho Chi Minh City, Vietnam Tel: +84 8 3844 6415 / 3948 5919 Fax: +84 8 3811 9668 Email: [email protected] Web: www.qdhs.vn 93 contributors Graham Sutton Dr Ezeddin El Tabal Michelle Wyatt Blake C Papsin Jane R Madell Laura Coleman Dr Doris-Eva Bamiou Mary-Louise Montague Sharon L Cushing Elizabeth Tyszkiewicz Helen Williams Dr Tony Sirimanna Mr Joe Grainger Adam J Donne Karen A Gordon Kate Parkes Pauline Grant Michael Kuo Michael P Rothera Gavin Morrison Cherilee Rutherford Jane R Madell Ann-Louise McDermott Konstance Tzifa Josephine Marriage Melanie Gregory Patrick Sheehan Sue Archbold Mr Tom Beech Kate Hanvey Guy Lightfoot Mr John Graham Ann-Louise McDermott Martin O’Driscoll Melissa M Lees Mr Iain Bruce Louise Melia Miss Sujata De Haytham Kubba Fiona B MacGregor Mr William PL Hellier Priya Singh
