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BME Information Day 16th March 2013 (Glasgow) Information on: Cochlear Implants And Auditory Rehabilitation post surgery by Dr Mary Shanks Role of Genetic Counselling for Hearing Impaired in Ethnic Minority Communities by Dr Juan Mora Role of Community Speech And Language Therapy in Hearing Impaired Children by Fiona Jarvis The key speakers on the day: Dr Juan Mora (Consultant Audio Vestibular Physician) Dr Mary Shanks (Consultant ENT Surgeon, Crosshouse), Fiona Jarvis (Community Speech and Language Therapist), Janette Joyce (Speech and Language Therapist, Advanced Rehabilitation Advisor, Crosshouse) Email response from Lise Henderson (Co-ordinator Paediatric Cochlear Implant) Questions asked by parents and some of the responses given by the speakers in their own words. 1. Because of brain plasticity changes during early childhood, children who have not acquired a first language in the early years might never be completely fluent in any language. If they miss this critical period for exposure to a natural language, their subsequent development of the cognitive activities that rely on a solid first language might be underdeveloped, such as literacy, memory organization, and number manipulation. Is this true? My son was diagnosed only when I came into this country at the age of 9. Fiona Jarvis (SLT): “If not diagnosed until age 9, suggests he was capable of disguising his difficulty. It also suggests that his hearing loss is not profound in which case, in quieter environments, he may have been able to access some speech/aspects of speech. Brain plasticity/neuroplasticity continues throughout an individual’s lifetime but is most in evidence in the first few years of life In hearing infants, as a result of repeated sensory stimulation and experiences of the world, the numbers of synapses or connections in the brain grow until there is about twice the number found in the adult brain. Then something called ‘synaptic pruning’ starts to happen and the weaker synaptic contacts are eliminated while stronger connections are kept and strengthened. The connections which have been activated most often by a baby’s repeated experiences, are preserved while ineffective, unused or weak connections are pruned. The optimal time for meaningful auditory neural connections to form is during the first 3 years of life. If the brain is not stimulated by sound it will reorganise itself to maximise processing through other senses – primarily vision. After about 3½, the brain has considerably less flexibility to develop effective skills to process auditory information which is why HI children identified late have a much harder time learning to listen and speak proficiently. There is a lot of documented evidence of the potential impact of delayed first language acquisition, but few authors offer suggestions of positive actions to progress the child’s abilities and to prevent potential gaps in language and cognition. Schick et al (2002) suggest some ‘positive actions’. Areas to focus on include : - pretend play/role play - narrative – experiencing books and stories and progressing to story telling - talking about past events - not limiting linguistic experiences – involve child in discussions, invite opinions, feelings/emotions relating to life experiences Your child is an individual and advice should be tailored to his individual needs. For general ideas on accelerating language development, speak to your local specialist speech and language therapist (specify that you want advice from a specialist in Hearing Impairment). “ Theory of Mind : Language Cognition in Deaf Children (B.Schick, J.de Villiers, P.de Villiers, B. Hoffmeister) Dec.03, 2002 in ASHA 2. I have read about long lasting batteries requiring little electric current will allow for a completely implantable cochlear device in the future. Are we any closer to getting such a device for our children? Will children who have already been implanted be able to have such a device fitted? Dr Mary Shanks: “There has been research and development into TICIs (totally implantable cochlear implants) for many years. There are still problems to be solved with battery and microphone technology as well as the need to avoid repeat surgery. At present upgrading external processors allows children to benefit from the latest technology without repeat surgery. If a TICI was to become available in future it is likely that current cochlear implant users could be upgraded but they would need a further operation to replace their existing implant.” 3. I was told my child was born without auditory nerves...is this rare. Will my child ever be able to use any listening device to hear? Dr Juan Mora: “The absence of auditory nerves is very rare, particularly when it is in both ears. There are devices like brainstem implants or other simpler devices which use vibration but they will help with hearing to a limited extent.” Janette Joyce (SLT Crosshouse): “I have located the info I was seeking last summer for one of the Scottish SLT's who had a little boy on her caseload with no auditory nerves. He was referred to our CI programme initially but as we can only offer cochlear implants at this time I believe he was referred on to the Manchester CI programme. This appears to be the only CI centre in the UK offering Auditory Brainstem Implants (ABI) to children. Both Manchester and Cambridge have experience with adults who have received ABI. The experiences I am hearing about at the moment is similar to the early days of cochlear implant when there were no guarantees about the benefits. Unlike now when we see the great results on a regular daily basis. ABI surgery is much more daunting at this time and will only usually be undertaken when there really is no other option. This is an extremely difficult decision for any parent to make so the collection of relevant information and the opportunity to talk to others who have been through the experience will be invaluable. Dr Colletti at the University of Verona in Italy is the main specialist in this field and I understand that at least one child from the UK has travelled to Italy for surgery. I am copying the e-mail I received from Lise Henderson, the Specialist SLT and Auditory Verbal Trained therapist at Manchester CI programme. If the family who asked the question would like to speak to her I am sure she would be happy to offer some advice based on her experiences. The outcomes are not terribly positive at this stage with only one out of six children achieving the outcome of being able to understand spoken communication without lip reading. But this was last summer and there may be more news by this time.” Email response from Lise Henderson (Co-ordinator Paediatric Cochlear Implant) “In Manchester we have done 60 adult and 6 paediatric ABIs (Auditory Brainstem Implants) the outcome is much poorer for ABI patients than CI and there is some data to suggest that only 5% obtain significant benefit. There are a small number of patients who are able to understand speech without lip reading but many adults find it an aid to lip reading. However adults who get an ABI will have had NF2 so their brainstem has been damaged by a tumour so you may anticipate a poorer outcome.” “With the children we have only 1 who demonstrates an ability to understand speech without lip reading the others generally are aware of speech and like to wear their implants but have no open set recognition. Due to the difficulty in placing the electrode there is often non auditory stimulation so 2 children do not obtain any auditory sensation from their implant they only have non auditory sensations such as eye twitches /coughing/pain in their ear etc. “ “ABI in children is not recommended under the age of 18 months and currently Manchester is the only UK centre doing paediatric ABI and Manchester and Cambridge the only place doing adult ABIs. I believe London is looking into setting up a paediatric Programme as well” “Hope that helps a little feel free to get in touch if you need any further info.” Kind Regards Lise 4. CI’s given to children with complex and multiple disabilities, such as those with visual, motor, or learning disabilities in addition to deafness it has been argued that there will be benefits in relation to perception of environmental sounds. Is this true? Dr Juan Mora “Visual, motor and learning disabilities are certainly NOT a contraindication to undergo assessment for cochlear implants. However significant developmental delays will influence whether the child will benefit from cochlear implants and therefore will have to be considered very carefully to decide is the child can be helped by cochlear implants. “ Fiona Jarvis (SLT): “The research suggests that benefits may be limited to perception and some recognition of environmental sounds. However, we need to remember that every child is an individual and consider a number of factors including: - child’s age - cognitive level - the support available from the family Parents considering cochlear implantation for their child, need to be provided with comprehensive and honest information in order to make an informed decision. Case example - implanted July 2011, aged 3, This child was referred for assessment for cochlear implantation at age 2. She is blind and deaf – the most profoundly dual sensory impaired child I have encountered. Research suggests that for deafblind children, the best outcome is awareness/recognition of environmental sounds and maybe recognition of a few words after a lot of input. This child is not, however, typical of deafblind children. She is bright, inquisitive, creative and determined. The parents were invited to discuss their concerns and given clear information about the procedures and the potential outcomes. For their child, if she made no gains post-implant, she remained, as before, deafblind. No exaggerations were made re the outcomes but staff expressed the positive potential in such an able child. The parents decided to go forward with implantation and currently their daughter is showing understanding of familiar phrases in both her first language and in English. She is interested in words and will copy some but also uses a growing number of words independently and appropriately.” 5. Implantation is taking place at increasingly earlier ages, with evidence that normally hearing infants make use of audition before birth, both for recognition of mother’s voice and also in relation to identification of the syllable structure of the mother’s language. Is there scope for such antenatal testing and techniques for antenatal implantation in the future? Dr Mary Shanks: “Antenatal surgery is very hazardous to the unborn child and unlikely to be considered for non- life-threatening conditions such as deafness, even if antenatal hearing testing was available. Children implanted around the time of their first birthday make excellent progress and their spoken language skills quickly catch up with normal hearing children so it is not necessary to expose younger children to the additional risks associated with general anaesthesia in very young babies. Many deaf children have residual low frequency hearing which allows them to hear environmental and some speech sound in the womb”. 6. Neurotrophic treatments-I have read about these: Neurotrophic factors which support survival and stimulate axonal growth of spiral ganglion neurons within the cochlea may provide enhanced outcomes for cochlear implants or as a means of regenerating a fully functional auditory system (Miller et al., 2007). Research in this area is likely to provide alternatives to current surgical interventions. Please could you explain this better? Dr Mary Shanks: “Neurotrophic or ‘nerve re-growth’ treatment is being developed but it will be many years until this type of treatment is available. Electrode design is being constantly improved to minimise damage to the spiral ganglion cells. In the near future it will also be possible to deliver drugs directly to the cochlea by coating implant electrodes or having drug delivery channels within the electrode. Steroids delivered in this way would reduce inflammation and scar tissue in the cochlea and help preserve spiral ganglion cells following surgery. In the future neurotropic factors could be delivered in this way.” 7. Approximately 5% of recessive deafness is in south Asians than other populations. Does this mean that if people don’t have consanguineous marriages they can prevent recessive deafness? Dr Juan Mora “Recessive hearing loss can appear in the children of both consanguineous and non-consanguineous marriages. However in general Recessive medical conditions are less common in non-consanguineous marriages. “ 8. False positives in universal neonatal screening for permanent childhood hearing impairment-What is this? Dr Juan Mora “Some newborns do not pass the hearing screening test although they may have normal hearing. However then they will undergo a thorough assessment to find out about their hearing which is very reliable.” 9. Please could you explain auditory neuropathy in layman’s terms? Would you consider a child with auditory neuropathy for an implant? Dr Mary Shanks: “The term auditory neuropathy includes a number of different conditions. It means that there is a problem with the nerve of hearing. Some of these conditions may be improved by cochlear implantation but some are not. If there is a problem with the hair cells in the cochlea transmitting sounds to the auditory nerve (hair cell dysynchrony) this can sometimes improved by cochlear implantation as the electrode stimulates the nerve directly. Other types of auditory neuropathy might also benefit but every child needs to be assessed individually”. Janette Joyce (SLT Crosshouse): “Cochlear implantation should certainly be considered for someone with auditory neuropathy and that normal speech can be an expected outcome. As with all CI surgery the level of ability is not guaranteed and is dependent on many other factors. In layman's terms an auditory neuropathy means that there is a fault somewhere along the auditory pathway but it can be difficult to tell exactly where. This is why the outcomes for a child with this diagnosis can be very variable” 10. In ear hearing aids-How effective are they? Dr Juan Mora “They are effective and have advantages and disadvantages compared with behind the ear hearing aids. In general they are not recommended for young children. They are not adequate for all degrees of hearing loss, they are lost more easily than behind the ear aids and are more difficult to replace if they have a technical problem, which may lead to o lack of consistency wearing the aids.” 11. Is there any way of diagnosing deafness during pregnancy? Dr Juan Mora “Not really unless you are referring to genetic studies during pregnancy which could identify mutations which may cause to hearing loss.” 12. Will a child with auditory neuropathy spectrum develop normal speech? Fiona Jarvis (SLT): “As with any group, the outcome varies from individual to individual. Australian study in International Journal of Audiology 2009; 48(6): 313-20. Rand, G., Barker EJ. Aim – to assess the receptive language and speech production of school aged children with Auditory Neuropathy/dys-synchrony type hearing loss. . Three groups were included in the research: Group 1 10 ch with AN and Cochlear Implant (CI) in one/both ears Group 2 10 implanted children from the general population of CI recipients Group 3 10 with AN who were long-term hearing aid users Outcome – generally the same for each of the groups, with similar levels of receptive language and speech production, except for 1/10 from Group 1 who gained little perceptual benefit from CI and showed severely delayed spoken language. If considering cochlear implantation, this may suggest that it would be better to give a single implant and see if the child benefits rather than automatically proceeding with bilateral implants. Research into auditory neuropathy spectrum disorder is continuing. With our current level of knowledge, outcomes can be unpredictable. A child with AN may or may not benefit from hearing aids/cochlear implant(s). Additional evidence suggests increased difficulty listening against background noise. Speech and language outcomes will be affected by the child’s level of hearing loss and on their ability to make sense of sound with some developing good speech and language and others struggling to process what they hear. Some may benefit from the use of sign language.” 13. Is there a link between certain syndromes and ethnicity? My child has Alport syndrome. Dr Juan Mora “Some mutations are more common in certain populations but I think Alport syndrome would not be a good example of those.” 14. If my child gets an implant am I changing his/her chance of future developments. Dr Mary Shanks: “Improvements in external processors which improve understanding of speech, and more recently music, are already available to current implant users. It will also possible to replace the internal receiver and electrode during a child’s lifetime, perhaps two or three times. Future developments which involve regrowing hair cells will not be possible if an ear is implanted. However unless this treatment was carried out during the critical period of language development, from birth to four years old, it would not to be effective. It is likely to be more than ten years until there is any alternative to cochlear implantation for the treatment of profound hearing loss”. 15. My child wears an implant and has tinnitus. Any tips on tinnitus? Janette Joyce (SLT, Cross House): “Child with tinnitus:1. Check programming of CI and let scientists know that tinnitus is a problem. 2. If only one CI consider a hearing aid in contra lateral ear if that is where the tinnitus is a problem. 3. If tinnitus is only a problem when CI is not worn then considers wearing the equipment during quiet periods and at bedtime. 4. As suggested by another parent - try changing position of bed at night time. 5. I would also add that it may be necessary to discuss sounds with the child. It could be that the problem may be down to lack of knowledge of the natural sounds around us.” Dr Mary Shanks “Tinnitus is often improved by cochlear implantation and when the processor is switched on the tinnitus is less prominent. If tinnitus is severe at night it may be advisable to wear the processor in bed”. Some Research Articles read by parents: http://news.bbc.co.uk/1/hi/health/7582640.stm http://www.bbc.co.uk/news/health-21320859 http://news.bbc.co.uk/1/hi/health/5102486.stm http://news.bbc.co.uk/1/hi/health/6755897.stm http://www.bbc.co.uk/news/health-20960418 http://www.bbc.co.uk/news/health-19570024