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The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco
Ferrara
Program
The XIV International Symposium
in Audiological Medicine
Index
p. 3 Welcome
p. 4 Important Addresses
September 18-21, 2008
Sala S. Francesco
Ferrara
p. 5 Committees
p. 6 Program
p.19 Abstracts
p.20 Invited Presentations
p.31 Papers
p.53 Posters
p.62 General Information
p.64 Social Events
Program
Welcome
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September 18-21, 2008
Sala S. Francesco, Ferrara
My Dearest Friends and Colleagues,
Please allow me to welcome you to the 14th edition of the International
Symposium in Audiological Medicine for the International
Association of Physicians in Audiology.
I am very grateful and honored that Ferrara was proposed as the host for
this meeting and, I hope, with your collaboration and contributions, that
the meeting will be a huge success.
Our field makes daily significant advances and the focus of this meeting is
to open channels of dialogue to discuss the hearing and balance evaluation and disorders, training
and research to further progress in our field and Quality assessment to continue to improve progress
already achieved.
Ferrara, being the ‘City of Bicycles’ means that the bicycle is ‘de rigueur’ and what better means
to discover this splendid Renaissance City and visit its famous architectural landmarks such as the
Estense Castle, the Certosa Cemetery, the Cathedral, the historical town centre and the Jewish
Ghetto. The city has managed to maintain its all important green spaces within the urban stretch
such as the Jewish Cemetery (which immediately brings to mind the famous works of Giorgio
Bassani). The origins of Ferrara are Etruscan-Roman, and was governed by the Countess Matilde di
Canossa, it was later surrendered to the Azzolino d’Este, who in 1146 married the last remaining
descendent of the Adelardi family. Ferrara remained under the rule of the Este Family until 1598
and then returned once more under the rule of the church. Ferrara thrived under the Este Family
rule, numerous palaces were constructed, the city walls were further fortified and its perimeters
lengthened. Important literary protagonists such as Tasso and Ariosto wrote works for the Este
Family, painters famous throughout Europe such as Cosmè Tura, Rogier van der Weyden and
Francesco del Cossa decorated the many new palaces that the dukes regularly commissioned. Today
Ferrara is a lively city and home to an historical and important University which was founded in
1391 by Alberto D’Este. The University can count Nicolò Copernico, Giovanni Pico della Mirandola
and Paracelso amongst its most successful graduates. The city is also renowned for its contribution
to the arts; with painters such as Boldini, De Pisis naming Ferrara as their birthplace and yielding
the first Metaphysic paintings by De Chirico. Ferrara has also played an important role in Cinema,
producing noted artists such as Antonioni and Vancini. It can boast a musical vocation and hosts
a yearly celebrated concert season. I conclude hoping that this meeting will give us all a positive
opportunity to discuss and contrast our relative experiences. I want to thank you in advance for your
attendance and valuable time.
Alessandro Martini
Department of Audiology
University of Ferrara, Italy
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3
The XIV International Symposium
in Audiological Medicine
Important addresses
Scientific Secretariat
Dipartimento di Discipline Medico-Chirurgiche
della Comunicazione e del Comportamento
Cattedra e U.O. di Audiologia
Università degli Studi di Ferrara
Corso Giovecca, 203 - 44100 Ferrara
Tel. 0532 237451 - Fax 0532 236887
e-mail: [email protected]
Organizing Secretariat
Piazza del Sole e della Pace, 5
35031 Abano Terme (Padova)
Tel. 049.8601818
Fax 049.8602389
e-mail: [email protected]
www.meetandwork.it
Committees
September 18-21, 2008
Sala S. Francesco, Ferrara
Chairman
Alessandro Martini
Department of Audiology
University of Ferrara
Organizing Committee
Edoardo Arslan
Maurizio Barbara
Umberto Barillari
Ettore Cassandro
Antonio Cesarani
Giancarlo Cianfrone
Luigi D’Angelo
Mario Fabiani
Elio Marciano
Paolo Pagnini
Antonio Pirodda
Oskar Schindler
Francesco Ursino
Congress Venue
Sala S. Francesco
Congress Website
www.iapacongress2008.it
4
5
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
P
rogram
Thursday 18th September 2008
➛ 15.00❙Registration
19.00
➛ 16.00❙PRE-MEETING COURSE
19.00 Vestibular Evaluation and Rehabilitation
Chairpersons: P. Pagnini, M. Sliwinska-Kowalska
Moderators: G. Guidetti and M. Toupet
Recent concepts in vestibular evaluation
and compensation mechanisms
M. Toupet (France)
➛08.30❙ROUND TABLE
10.30 Genetics of Vestibular Disorders
Chairpersons: J.J. Barajas de Prat, A. Martini
Moderator: L.M. Luxon
Mouse models with genetic vestibular dysfunction
K. Steel (UK)
The genetics of vestibular disorders
M. Bitner (UK)
Inherited neurological disorders with vestibular involvement
D. Kullman (UK)
Temporal bone imaging in genetic vestibular disorders
T. Beale (UK)
➛10.30❙IAPA group photo
Cognitive processes in vestibular adaptation
G. Guidetti (Italy)
➛ 11.00❙PAPERS – Session I
13.00 Genetics and Vestibular Disorders
Moderators: K. Steel, E. Marciano
Short and long term effects of the vibratory activation
of the proprioceptors and the vestibular system
V. Pettorossi (Italy)
❙01.
EXPRESSION OF CONNEXIN 26, 31, 36, 43 AND 30 (CX26, 31, 36, 43, 30)
IN THE HUMAN COCHLEA
Liu W, Boström M and Rask-Andersen H (Sweden)
The individual rehabilitative program
A. Cesarani (Italy)
Novel vestibular rehabilitation
L.M. Luxon (UK)
Physical Therapy of BPPV
P. Vannucchi (Italy)
Discussion
➛ 19.00❙Welcome Reception
6
Friday 19th September 2008
❙02.
ADDUCIN 1 MUTATION AS A POSSIBLE MECHANISM LEADING
TO ENDOLYMPHATIC HYDROPS
Teggi R, Manunta P, Lanzani C, Zagato L, Bussi M (Italy)
❙03.
GENETIC EVALUATION IN THE CONGENITAL HEARING LOSS:
THE CAMPANIA NEWBORN HEARING SCREENING PROGRAMME EXPERIENCE
Lilli G, Riccardi P, Errichiello M, Toscano F, Barrier F, Continisio P, Continisio GI, Auletta G, Landolfi E, Munaretto A, Pisacane, Chinetti I, Iossa S, Franzé A, Marciano E (Italy)
❙04.
A BIOBANK AS A TOOL TO IMPROVE KNOWLEDGE OF GENETIC HEARING LOSS
Alemanno MS, Ortore RP, Palladino T, D’Ecclesia A, Zadro C, Gasparini P, Zelante L,
Melchionda S, Vigliaroli L, Carella M (Italy)
7
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
❙05.
AUDIOLOGICAL PHENOTYPE OF PATIENTS SHOWING CONNEXIN 26/30
RELATED DEAFNESS IN A COHORT INCLUDING 628 PATIENTS
Cama E, Santarelli R, Melchionda S, Palladino T, Carella M, Toffolatti L, Incognito A,
Zelante L, Arslan E (Italy)
❙06.
“AUDIOLOGICAL PROFILES” AND GJB2 MUTATIONS: FERRARA EXPERIENCE
Berto A, Castiglione A, Pellati D, Gualandi F, Martini A (Italy)
❙07.
LARGE VESTIBULAR AQUEDUCT SYNDROME: AUDIOLOGICAL, RADIOLOGICAL,
CLINICAL AND GENETIC FEATURES
Berrettini S, Forli F (Italy)
❙08.
EFFECT OF NOISE ON THE VESTIBULAR LABYRINTH: FUNCTIONAL AND
IMMUNOHISTOCHEMICAL ANALYSES IN THE EXPERIMENTAL MODEL
Fetoni AR, Picciotti P, Troiani D, Paludetti G (Italy)
❙09.
SUPERIOR SEMICIRCULAR CANAL DEHISCENCE SYNDROME:
A POSSIBLE EXPLANATION FOR THE VARIABILITY OF SYMPTOMS?
Brandolini C, Castellucci A, Pirodda A, Modugno GC (Italy)
❙10.
IMAGING EVALUATION IN OTOSCLEROSIS: SINGLE PHOTON EMISSION
COMPUTED TOMOGRAHY VERSUS COMPUTED TOMOGRAHY
Berrettini S, Ravecca F, Volterrani D, Neri E, Forli F (Italy)
❙11.
LYMPHOCYTES IMMUNPHENOTYPING IN CONTRALATERAL TYPE DELAYED ENDOLYMPHATIC HYDROPS
Quaranta N, Vacca A, Candreva T, Capuzzimati L, Quaranta A (Italy)
❙12.
HEMOSTATIC ALTERATIONS IN PATIENTS WITH ACUTE PERIPHERAL VERTIGO
Ursino F, Nacci A, Fattori B (Italy)
➛13.00❙Lunch break
➛14.00❙IAPA General Assembly
8
➛15.00❙PAPERS – Session II
17.00 Vestibular and Hearing Disorders
Moderators: E. Raglan, L. D’Angelo
❙13.
SUBJECTIVE VISUAL VERTICAL AND HORIZONTAL: REPEATABILITY
AND RELATIONSHIP WITH THE PRESET ANGLE
Pagarkar W, Bamiou D, Ridout D, Luxon L (UK)
❙14.
REHABILITATION THERAPY OF PERIPHERAL VESTIBULAR DISORDERS:
OUR EXPERIENCE
Livi W, Cioni V (Italy)
❙15.
SELF-THERAPY IN THE TREATMENT OF BENIGN PAROXYSMAL POSITIONAL VERTIGO CAUSED BY CUPOLOLITHIASIS IN THE POSTERIOR
SEMICIRCULAR CANAL: A NEW PROPOSAL
Pagliari AV, Forner P, Bonelli A, Blotta P (Italy)
❙16.
SPEECH AUDIOMETRY WITH VERBAL TASK AND MOTOR RESPONSES:
A NEW METHOD IN THE DIAGNOSIS OF AGE-RELATED HEARING LOSS
Cesarani A, Di Berardino F, Barozzi S (Italy)
❙17.
PSYCHOLOGICAL PROFILE AND SOCIAL BEHAVIOR OF WORKING ADULTS
AFFECTED BY MILD TO MODERATE HEARING LOSS
Monzani D, Genovese E, Marrara A, Forghieri M, Galeazzi G, Martini A (Italy)
❙18.
SENSORINEURAL HEARING LOSS IN SUPERFICIAL SIDEROSIS OF THE CENTRAL NERVOUS SYSTEM: UNUSUAL AUDIOLOGICAL FINDINGS
Conti G, Marchese MR, Martina BM , Colosimo C (Italy)
❙19.
OVARIAN STEROIDS INFLUENCE THE GENDER DIFFERENCES
IN AUDITORY FUNCTION
Al-Mana D, Ceranic B, Djahanbakhch O, Luxon LM (UK)
❙20.
EFFECTS OF CLASSROOM NOISE AND REVERBERATION ON THE SPEECH
PERCEPTION OF BILINGUAL CHILDREN LEARNING IN THEIR SECOND LANGUAGE
Bovo R (Italy)
9
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
❙21.
COCHLEAR POTENTIALS RECORDED BY TRANSTYMPANIC
ELECTROCOCHLEOGRAPHY (ECOCHG) IN PATIENTS
WITH AUDITORY NEUROPATHY
Santarelli R, Starr A, Michalewski HJ, Arslan E (Italy)
❙22.
NEAR – INFRARED SPECTROSCOPY ( NIRS) OF THE AUDITORY CORTEX DURING PASSIVE LISTENING: PRELIMINARY DATA
Grasso DL, Bembic S, Bava M, Clarici A (Italy)
❙23.
SPEECH PERCEPTION TEST IN EARLY IDENTIFICATION OF APD
Palma S, Mennucci E, Guarnaccia MC, Genovese E (Italy)
❙24.
INNER EAR DYSFUNCTION: THE ROLE OF NON-AUDIOLOGICAL FACTORS
Pirodda A, Brandolini C, Raimondi MC, Modugno GC, Degli Esposti D, Borghi C (Italy)
➛ 17.00❙ROUND TABLE
19.00 Evaluation and Rehabilitation
of Central Auditory Processing Disorders
Chairpersons: E. Arslan, P. Berruecos
Moderators: O. Schindler and A. am Zehnhoff-Dinnesen
Introduction
O. Schindler (Italy)
The neural basis of central auditory processing
A. Schindler (Italy)
The relationship between central auditory processing
and other neuropsychological functions
C. Semenza (Italy)
The diagnosis of APD
C.M. Schmidt (Germany)
The assessment of CAPD
E. Genovese (Italy)
The rehabilitation of CAPD
A. am Zehnhoff-Dinnesen (Germany)
Discussion
Saturday 20th September 2008
➛08.30❙PAPERS – Session III
10.50 Cochlear Implants and Hearing Disorders
Moderators: M. Fabiani, R. Illing
❙25.
MUSICAL INTERVALS DISCRIMINATION IN IMPLANTED PATIENTS
Tani A, Patelli I, Danesi G (Italy)
❙26.
CLINICAL CASE: COCHLEAR IMPLANT IN A PATIENT
WITH FAMILIAL TRANSTHYRETIN AMYLOIDOSIS AND SUDDEN HEARING
LOSS WITHOUT VIII NEUROPATHY
Negri M, Guarnaccia MC, Benincasa P, Galli S (Italy)
❙27.
HABILITATION ISSUES FOR COCHLEAR IMPLANTS PEDIATRIC RECIPIENTS FROM BILINGUAL FAMILIES
Nassif N Barezzani MG, Botta N, Zanetti D (Italy)
❙28.
ABNORMALITIES OF WHITE MATTER AND OUTCOMES
OF COCHLEAR IMPLANTATION
Zanetti D, Nassif N, Barezzani MG, Giordano L, Pinelli L (Italy)
❙29.
EAR AND BRAIN NEURORADIOLOGICAL FINDINGS AND OUTCOMES
IN COCHLEAR-IMPLANT CANDIDATE CHILDREN
Busi M, Trevisi P, Destro E, Calzolari* F, Martini A (Italy)
❙30.
ASSESSMENT OF SPEECH INTELLIGIBILITY IN NOISE IN COCHLEAR IMPLANT
USERS USING FM SYSTEMS
Barajas de Prat JJ, Zenker Castro F, Mora Espino R
and Fernandez Belda R (Canary Islands, Spain)
❙31.
ASSESSMENT OF BENEFIT AFTER BILATERAL COCHLEAR IMPLANTATION
Obrycka A, Lorens A, Piotrowska A, Zgoda M, Skarżyński H (Poland)
❙32.
HEARING AND QUALITY OF LIFE OF NF2 PATIENTS WITH ABI
Konradsson KS, Nyberg G, Svedberg A, Olsson G-B Kinnefors A
and Rask-Andersen H (Sweden)
➛ 2 0.30❙Conference Dinner
Palazzo San Crispino, Ferrara
10
11
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
❙33.
TRANSMEATAL LOW LEVEL LASER THERAPY FOR CHRONIC TINNITUS
WITH COCHLEAR DYSFUNCTION
Bellini C, Teggi R, Piccioni L O, Palonta F, Bussi M (Italy)
❙34.
NON PULSATILE OBJECTIVE TINNITUS IN CHILDREN
Spyridakou C , Rajput K, Veness J, Sirimanna T (UK)
❙35.
THE INFLUENCE OF THE HEARING AID MICROPHONES POSITIONING
ON THE LOCALIZATION CUES. RESOUND BE: A CASE STUDY
Picinali L, Mancuso A, Vercellesi G, Pulga M (Italy)
❙36.
SENSORINEURAL HEARING LOSS AND SEVERE NEONATAL RESPIRATORY FAILURE
Morando C, Gamba PG, Midrio P, Sgrò A, Filippone M, Magnano San Lio AG, Orzan E (Italy)
❙37.
THE UNIVERSAL NEWBORN HEARING SCREENING PROGRAM AT FERRARA, ITALY
Hatzopoulos S, Ciorba A, Petruccelli J*, Camurri L, Rossi M,
Malagutti M and Martini A (Italy, *USA)
❙38.
GENETIC TESTING - THE PREDICTING ROLE IN GENETIC COUNSELING
AND INSTRUCTION FOR DEAF COUPLES
Bing H, Pu D, Guo-jian W, Dong-yang K, Xin Z, Yong-yi Y, Dong-yi H (China)
❙39.
FROM NHS TO EARLY DIAGNOSIS: OAE, ABR AND ASSR EXPERIENCES IN MEXICO
Berruecos P. and Martínez R (Mexico)
➛10.50❙ROUND TABLE
12.50 Aetiology of Age Related Hearing Impairment
Chairpersons: A. Cesarani, U. Rosenhall
Moderator: D. Stephens
Brief Historical
D. Stephens (UK)
Genetic aspects of ARHI
L. Van Laer (Belgium)
Medical and environmental aspects
U. Rosenhall (Sweden)
Interaction between noise and other factors
I. Pyykko (Finland)
Discussion
12
➛13.00❙LUNCH SESSION
14.30 Information Management
for Newborn Hearing Screening
Moderators: A. Parving, E. Marciano
in cooperation with OZ Systems
How IT(information technology) works in Newborn Hearing Screening
Pool K (OZ Systems, USA)
eSP™ - Information Management for Newborn Hearing
Screening - from the birthing facility to audiologists
and other care providers
Daussat L (OZ Systems, USA)
Child centric integrated electronic health records
Daussat L (OZ Systems, USA)
Live Demonstration of eSP™
Daussat L (OZ Systems, USA)
Question and Answer – Implementing Newborn Hearing
Screening programs
Daussat L and Pool K (OZ Systems, USA)
➛14.30❙POSTERS DISCUSSION
16.00 Moderators: S. Berrettini, N. Quaranta
❙P1.
PREDICTORS OF HEARING SCREENING FAILURE FOR TARGETED NEWBORN HEARING SCREENING IN A DEVELOPING COUNTRY
Olusanya BO, Luxon LM (UK)
❙P2.
TEOAE+AABR: reduction of refers in neonatal hearing screening in Liguria Region (“Progetto STERN-LIGURIA”)
Zavattoni V (Italy)
❙P3.
EARLY DETECTION OF HEARING IMPAIRMENT IN NEWBORNS.
ITALIAN UNIVERSAL NEWBORN HEARING SCREENING PROGRAM
Bubbico L (Italy)
❙P4.
THE NEWBORN WITH OTHOLOGICAL MALFORMATIONS:
A COMPLEX DIAGNOSTIC APPROACH
Garani MC, Caldarelli V, Trevisi P, Ballardini E, Busi M, Garani G (Italy)
13
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
❙P5.
SENSORINEURAL HEARING LOSS (SNHL) IN A COMPOUND HETEROZYGOUS
FOR 35DELG AND V153I
Chiarella G, Passafaro G*, Cassandro C, Mangone G*, Misasi S*, Perrotti N*, Cassandro E (Italy)
❙P6.
USHER SYNDROME: GENOTYPE PHENOTYPE CORRELATION
Rangesh N1, Saihan Z2, Haralambous E3, Le-Quesne P3, Cohen M4, Bitner-Glindzciz M 3, Webster A2, Luxon L1 (UK)
❙P7.
EVALUATION OF VESTIBULAR FUNCTION IN CHILDREN WITH OTITIS MEDIA
WITH EFFUSION ACCORDING TO HEARING LOSS
Pazdro-Zastawny K, Pospiech L, Gawron W, Kochman M (Poland)
❙P8.
EVALUATION OF EUSTACHIAN TUBE FUNCTION IN CHILDREN AFTER SURGICAL
OPERATION OF CLEFT PALATE
Zalesska-Kręcicka M, Szleląg J, Kochman M, Pazdro-Zastawny K (Poland)
❙P9.
STATE OF HEARING AT CHILDREN WITH CLEFT PALATE
Dobrota Davidovic N Jovanovic Simic N (Serbia)
❙P10.
DIFFERENTIAL DIAGNOSIS BETWEEN LOGOPATHY AND AUDIOPATHY
IN HEARING IMPAIRED CHILDREN
Jovanovic Simic N1 Dobrota Davidovic N2 (Serbia)
❙P11.
AUDITORY ABILITIES IN SCHOOL-AGE CHILDREN
Anastasio ART, Toscano RDGP (Brazil)
❙P12.
NEUROPSYCHOLOGICAL CORRELATES OF PROFOUND DEAFNESS
IN ASSOCIATION TO MULTIHANDICAP IN A GROUP OF CHILDREN TREATED
WITH COCHLEAR IMPLANT
Gubernale M, Trevisi P, Genovese E., Martini A (Italy)
❙P13.
AGE RELATED HEARING THRESHOLDS IN AN EXTENDED FREQUENCY RANGE
Jilek M, Syka (Czech Republic)
❙P14.
AHEAD III - ASSESSMENT OF HEARING IN THE ELDERLY:
AGING AND DEGENERATION - INTEGRATION THROUGH
IMMEDIATE INTERVENTION
1Grandori F, 2Wolf-Dieter Baumgartner, 3Chryssoula Thodi, 4Arne Ernst, 5Thomas Janssen,
6Sophia Kramer, 7Mariola Sliwinska-Kowalska, 8Stefan Stenfelt, 9Rudolf Probst,
10Adrian Davis (1Italy, 2Austria, 3Cyprus, 4,5Germany, 6The Netherlands, 7Poland,
8Sweden, 9Switzerland, 10UK)
❙P15.
VARIATIONAL ANATOMY OF THE HUMAN COCHLEA
IMPLICATIONS FOR COCHLEARIMPLANTATION
Erixon E, Rask-Andersen H (Sweden)
❙P16.
ASSESMENT OF SALICYLATE AND NOISE INDUCED TINNITUS IN RATS USING
A NOVEL BEHAVIORAL PARADIGM
Ralli M, Lobarinas E, Paludetti G, Salvi R (Italy)
❙P17.
PROTECTIVE EFFECTS OF GINKGO BILOBA EXTRACT
AGAINST CISPLATIN OTOTOXICITY
Simoni E, Donnarumma T, Astolfi L, Ciorba A, Martini A (Italy)
➛16.00❙ROUND TABLE
18.00 Global Development of Audiological Medicine
Chairperson: A. Pirodda, K. Konradsson
Moderator: D. Lucas
Audiological medicine and hearing health awareness in Mexico:
the threshold of change
P. Berruecos (Mexico)
Another Olympian challenge: Audiological medicine
and health in the east
A. Smith (UK)
Third world and third sector.
Is audiological medicine important for this population?
I. Mackenzie (UK)
Audiological medicine in Europe.
Are we all practising the same speciality?
E. Raglan (UK)
14
15
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
How might training and education enable
the global development of audiological medicine?
K. Harrop-Griffiths (UK)
Synchrotron radiation-based micro computed tomography
to visualize the cochlea- Three-dimensional Sub-cellular
resolution with a non-destructive method
R. Glueckert (Austria)
Discussion
Sensory and Signal transduction in the inner ear
F. Mammano (Italy)
➛18.30❙Conference Conclusion
❙Copernico Prize Giving Ceremony
Aula Magna of the University of Ferrara
Calcium imaging in cochlear slices
J.-L. Puel (France)
Imaging and Synapses
M. Eybalin (France)
The Human Cochlea - Can we regenerate
its neurosensory elements?
H. Rask-Andersen (Sweden)
Magnetic resonance imaging for studying nanoparticles
into the cochlea
I. Pyykko (Finland)
➛12.30❙Lunch break
Sunday 21st September 2008
Joint Symposium with IEB
Imaging the Plasticity of the Central Auditory System
on the Cellular and Molecular level
R. Illing (Germany)
➛09.00❙Auditory Imaging from the Cell to the Cortex
18.00 Chairpersons: J. Ashmore, A. Serra
Moderators: R. Pujol, A. Martini
A “Realistic voyage through the imaging along
the auditory pathways”
F. Calzolari (Italy)
Introduction and Welcome
A. Martini (Italy)
Functional MRI for hearing and language
F. Di Salle (Italy-The Netherlands)
Imaging of the cochlea: state of the art
R. Pujol (France)
Brain imaging and recovery of speech comprehension
in cochlear implanted deaf subjects
P. Barone (France)
Three-dimensional confocal microscopy
of the mammalian cochlea
G. MacDonald (USA)
Visualization of multifunctional nanoparticles at light
and electron microscopic level in in-vitro systems
A. Schrott-Fischer (Austria)
16
Chairpersons: G. Paludetti, J.-L. Puel
Moderators: R. Pujol, A. Martini
Hearing Music Images
G. Haus, A. Mancuso (Italy)
Discussion
➛18.00❙Conclusion
17
I 5 benefici
di
1.
UDITO NATURALE
2.
CORRETTA PERCEZIONE DELLO SPAZIO
3.
ELEVATA QUALITÀ SONORA COMPLESSIVA
4.
AGEVOLE ACCESSO AL MONDO DELLA COMUNICAZIONE
5.
ESTETICA E TECNOLOGIA DEL 21°SECOLO
L’udito acquista profondità e naturalezza.
Il mondo sonoro torna ad essere intenso ed espressivo.
Il beneficio è percepibile da subito.
Riconoscimento della provenienza dei suoni.
Avanti/Dietro, Destra/Sinistra. Riconoscimento della distanza
della sorgente sonora. Udito stereofonico.
Abstracts
Ideale per Utlizzatori esperti. Gestione ottimale di tutte le situazioni
di ascolto: comprensione della voce, riduzione del rumore,
segnali di avvertimento. Fedeltà di riproduzione e massima flessibilità.
Collegamento, senza fili, attraverso lo Streamer,
al telefono cellulare, al computer, all’Ipod, alla televisione
e a qualunque dispositivo audio dotato di Bluetooth®.
Molto piccolo. Design d’avanguardia.
Soluzione RITE. Colori tecnologici.
Bello da vedere e invisibile quando indossato.
OTICON ITALIA S.r.l.
Via Panciatichi, 94 - int. 10/20 - 50127 Firenze, Italia
Tel. 055 3260411 - Fax 055 3260424 - www.oticon.com
19
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
I
nvited presentations
Rehabilitation of central auditory processing
Antoinette am Zehnhoff-Dinnesen
Department of Phoniatrics and Pediatric Audiology, Muenster, Germany
Therapeutic intervention possibilities in the rehabilitation of central auditory processing disorders include auditory
training of specific deficits, for example in temporal discrimination or phoneme discrimination. Auditory learning
enhances top-down and bottom-up processing. Transfer mechanisms were observed in several studies. During
auditory training changes in the central auditory system occur which can be evidenced by altered auditoryevoked potentials. Computer-assisted training programs can successfully be applied.
Compensatory strategies and procedures to improve the signal noise ratio (for example in the class room) are
additionally presented.
The benefit is discussed of wireless communication systems (FM systems) and special devices as the Edulink
system.
The presented intervention possibilities can be combined. They should be initiated as early as possible to derive
maximal benefit from the plasticity of the central auditory system in childhood.
Brain imaging and recovery of speech comprehension in cochlear implanted
deaf subjects
Pascal Barone1 and Olivier Deguine1,2
1. Cerveau et Cognition, CNRS UMR5549, Toulouse , France
2. ORL, CHU Purpan, Toulouse, France
A cochlear implant allows post-lingual adult deaf patients to understand speech through long-term adaptative
processes to build coherent percepts from the coarse information delivered by the implant. Because the success
of rehabilitations relies on the functional plasticity in the auditory system, it is of crucial importance to understand
the reorganization of the cortical network involved in speech comprehension that occurs during deafness and
following the progressive recovery. As a consequence of a long period of deafness and because of the crude
information provided by the implant, CI users have developed compensatory strategies for speech comprehension
favoring speech-reading and visuo-auditory interactions1,2. Because of the neuro-prosthesis, only PET scan
(Positron emission tomography) neuro-imaging studies can be performed to visualize brain activity based on
regional changes in cerebral blood flow. Neuro-imaging studies have reported that CI patients present different
levels of activation in cortical areas involved in semantic and/or phonological speech processing. Further we will
present recent evidences that the of cross-modal compensation observed at the behavioral level is accompanied
by plastic changes in the visual and auditory network involved in visual and visuo-auditory speech integration3.
1 Rouger J, Lagleyre S, Deneve S, Fraysse B, Deguine O. Barone P: Evidence that cochlear implanted deaf
patients are better multisensory integrators. Proceedings of the National Academy of Sciences USA. 104 (2007):
7295-7300.
20
2 Rouger J, Fraysse B, Deguine O., Barone P: McGurk effects in cochlear implanted deaf subjects. Brain Research
1188 (2008) 87-99.
3 Lagleyre S, Rouger J, Demonet JF, Fraysse B, Deguine O, Barone P. Audiovisual integration of speech in
postlingually deaf cochlear-implant patients: a behavioral and PET study. Society for Neuroscience, 2007 San
Diego (USA). Program No. 836-12
AUDIOLOGICAL MEDICINE AND HEARING HEALTH AWARENESS IN MEXICO:
THE TRESHOLD OF CHANGE
Pedro Berruecos
Head, Audiology and Phoniatrics Dept., General Hospital of Mexico & Faculty of Medicine, National University of
Mexico, Mexico. Dr. Balmis 148, Col. Doctores. Mexico City, 06726, Mexico
Interest on hearing, voice and language has been present in occidental cultures for centuries but it was just
after the Second World War when the hybrid word (Greek and Latin roots) “Audiology”, was coined in 1945
by Canfield, an Otologist, and Carhart, a Speech Pathologist, professionals from different areas actually mixed,
among others, in our discipline. At that time, the outcomes of the surgery of deafness techniques, intensified the
interest in the ear physiology and pathology and the invention of the vacuum tube, allowed the development of
diagnostic and amplification devices. Once again, medical and technological advances traveled together along
with the multidisciplinary contribution of other fields. In 1951, only 6 years after the emergence of the word
“Audiology”, our specialty born in Mexico, The Mexican Audiology and Phoniatrics Centre and the National
Institute of Audiology established in 1951 and 1953, included Phoniatrics, based on the fact that voice/language
production is the main physiologic consequence of hearing. The first postgraduate courses defined medical
specialty that studies the normality and the pathology of hearing, voice and speech/language processes. Its
objective is very clear: to establish, develop or re-establish hearing and linguistic communication when it is
absent, disturbed or interrupted. The Mexican Society of Audiology and Phoniatrics was founded (1977) with 21
specialists and today gathers more than 700 medical (Full members) and non-medical (Associate members)
professionals. The Board of Audiology and Phoniatrics (1983), recognized by the National Academy of Medicine
(1988), the highest academic medical authority in Mexico in charge of the Medical Boards regulation, certified
until now the suitability of around 350 Physicians. In 1990, the Faculty of Medicine (National University of
Mexico) guarantee the Audiology/Phoniatrics well developed specialization postgraduate 4-year program.
After a long period of persevering insistence and hard lobbying job, hearing health is now considered in the
National Health Program of the Federal Government. We know the demographic and epidemiological data and
consequently, the potential demand for services; health providers, structure of services and equipment and
material resources needed to suggest the assignment of financial resources; how many Physicians in Audiology
needs our country; how to modify, if necessary, the actual program of the medical residency; how to make a
useful follow up of the whole system; target groups to give them the essential priority and finally, how to spread
the information on health education and promotion in our field. The ways to face hearing and communicative
disorders are different from one country or one region to another, but we consider that the role of Physicians in
Audiology is basic to integrate the multidisciplinary team needed in this field, because people with hearing and
communication disorders deserve a responsible person to coordinate the team.
One of the actual main steps is centered in the National NHS program and in the National Training Program of
human resources for the NHS. We are in the threshold of change and confident that soon we will be able to inform
21
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
the advances of our programs and how we are trying to avoid or dramatically reduce the ear and hearing damage,
dysfunction, impairment and handicap, which are the objectives of the three prevention levels in our field.
IMMUNOCYTOCHEMICAL IMAGING OF ORGAN OF CORTI SYNAPSES
Michel Eybalin
Inserm U583, Institut des Neurosciences, Hôpital Saint Eloi, 34091 Montpellier, France
Different immunocytochemical approaches in routine use in our laboratory aimed at characterizing synapses in
the organ of Corti will be presented.
The immunoreactivity of neurotransmitters or synapse-associated proteins (e.g. receptors, synthesizing enzymes,
transporters, synaptic vesicle- or ribbon-associated proteins) is used as synapse labels. Localizing these labels
at the light microscopic level involves the use of immunofluorescence associated to confocal microscopy and,
sometimes to deconvolution, 3D-reconstruction or running a colocalization algorithm. At the electron microscopic
level, an immunoperoxydase technique, using DAB or the more punctiform VIP as chromogens, could be used if
the label is rather homogeneously distributed in the synaptic terminal. In the case of much localized distribution,
the use of the more refined post-embedding immunogold technique appears necessary.
Representative results will be shown including the immunocytochemical localization of the synaptic ribbon protein
RIBEYE, the ribbon-anchoring protein Bassoon and AMPA and NMDA receptor subunits at the inner hair cell
synapses. Examples of immunocytochemical colocalization at lateral efferent synapses will also be shown.
Synchrotron radiation-based micro computed tomography to visualize
the cochlea- Three-dimensional Sub-cellular resolution with a nondestructive method
Rudolf Glueckert
Medical University Innsbruck, ENT Clinic, ENT Research Laboratory for Inner Ear Biology
Anichstr. 35, A-6020 Innsbruck, Austria
Synchrotron radiation-based micro computed tomography contributes to the increasing demand for uncovering
non-destructively the microscopic morphology of bony tissues and their interface regions with
implants using isotropic spatial resolution in three-dimensional space.
Gross morphologic evaluation of the bony and membranous makes this technique ideally suited to visualize
pathologic aberrations with excellent resolution.
It further enables intensitybased segmentation of metallic implants within bone and thereby to quantitatively
study the bone morphology around different kinds of middle and inner ear implants.
This knowledge permits improving medical interventions and optimising the implant’s design with respect to
surface modification, mechanical properties, and shape.
Vestibular adaptation
Giorgio Guidetti
Department of Audiology, Vestibology and Vestibular Rehabilitation
Azienda Unità Sanitaria Locale di Modena, Italy
22
Equilibrium is an essential function and cannot be entrusted to a single organ or apparatus, but requires an entire
system, a set of communicating structures and processes.
Vestibular, visual and proprioceptive peripheral inputs are processed by CNS with a certain degree of
preferentiality but in non-pathological conditions vestibular afferences have absolute predominance. Comparison
between information obtained by integrating the various peripheral inputs and previously stored experience
enables rapid adaptation not only to the ongoing reality but also to the expected forthcoming reality. Adaptation
mechanisms are active also in normal conditions, because they are essential in case of modifications in ordinary
life conditions.
Acute lesions affecting peripheral receptors or nerves cause serious signs and symptoms (nystagmus, and
related rotatory vertigo; loss of head, ataxia and body tilt; spatial disorientation; altered self-motion perception)
because they produce a sudden change in information in the balance system. In the days and weeks after the
acute vestibular deficit the balance system needs an immediate reorganization. The main mechanism are the
sensory substitution (or compensation) and the new strategies and the relearning processes (or adaptation).The
sensory substitution is based on the neuronal plasticity. The adaptation is principally based on the mechanisms of
habituation, storage, learning and modulation. Daily repetition of abnormal conditions causes habituation, usually
involving a reduction of the gain of the concerned reflexes. New strategies involve the activation of learning and
biological memory processes of non-declarative procedural memory. Procedural learning is activated through
practice, it is slow and incremental and it requires immediate and consistent feedback. The experience of the first
seven days are the most important to facilitate the functional recovery. The vestibular adaptation is not constant
in the time: it depends on many individual factors. It is usually satisfactory but, a vestibular cicatrix remains for
ever (ocular tilt reaction, substitutional saccade during quick head mouvement, deviation of locomotor trajectory
toward the lesioned side with the eyes closed, spatial learning and memory deficits, abnormal levels of anxiety
and depression), also in patients no longer suffering from vestibular symptoms.
Some drugs oppose the adaptation (alcohol, barbiturates, phenothiazine, ACTH antagonists, antihistaminic,
cholinemimetic, cholinesterase inhibitors, adrenergic agents, GABA agonists and antagonists, atropine,
scopolamine, alfa-adrenolytics) while other drugs improve the physiological processess of vestibular adaptation
(caffeine, amphetamine, ACTH, piracetam)
Inherited paroxysmal disorders affecting balance and cerebellar
function
D.M. Kullmann
UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
Two main forms of dominantly inherited episodic ataxia have been genetically elucidated. Episodic Ataxia type
1 (EA1) is caused by mutations of KCNA1, which encodes the potassium channel subunit Kv1.1. Episodic Ataxia
type 2 (EA2) is caused by mutations of CACNA1A, which encodes theα1A calcium channel subunit. Both these
ion channels are widely expressed in the brain, spinal cord and peripheral nervous system, but appear to play
especially important roles in cerebellar function. They are also expressed in other parts of the auditory and
vestibular pathways. Although much is known about their biophysical properties, and how they might affect
neuronal signalling in isolation, definitive explanations for the paroxysms of cerebellar in-coordination are lacking.
Several other forms of episodic and progressive ataxia are also recognised, some of which also involve disorders
of neuronal excitability.
23
The Hearing Implant Company
September 18-21, 2008
Sala S. Francesco, Ferrara
Three-dimensional confocal microscopy of the mammalian cochlea
Glen MacDonald
University of Washington, Department of Otolaryngology-Head and Neck Surgery, V.M. Bloedel Hearing Research
Center - Seattle, WA 98195-7923 USA
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The mammalian inner ear is a challenge to light microscopy from multiple tissue types, each with a unique
refractive index, overlapping in a spiraling structure. Additionally, the large open chambers of the scala tympani,
scala media and scala vestibuli take on the refractive index of embedding or mounting media. These multiple,
overlapping refractive indices produce spherical aberration and light scattering that cause loss of signal and
resolution
Traditional methods for high-resolution microscopy reduce spherical aberration by microdissection and sectioning
to physically remove refracting structures. Such approaches impose limitations of labor and tissue distortion to
produce 2-dimensional images. Additional processing is required to create 3-dimensional representations by
serial reconstruction from sections.
We have developed a means to obtain 3-dimensional images by confocal microscopy, with minimal dissection
from inner ears that have been fixed, decalcified and fluorescently labeled. The labeled specimen is dehydrated
and then infiltrated with a mixture of 5 parts methyl salicylate and 3 parts benzyl benzoate for imaging by
confocal microscopy.
It is possible to obtain optical volumes through an intact, fluorescently labeled cochlea up to 900 µm in thickness,
limited by the working distance of the objective lens rather than by spherical aberration. This procedure creates
a homogeneous refractive index closely matching the highest refractive index in the inner ear to greatly reduce
spherical aberration, even in the presence of tissue structures with high refractive index.
We can now acquire optical volumes from the cochlea over a range of spatial resolutions, without sectioning, with
most fluorescent labels. Optical volumes collected by this method promise to be useful for applications such as
tracing innervation patterns in the organ of Corti, counting sensory cells or other structures over large regions of
the sensory epithelium and characterization of the inner ear in animal models of human deafness disorders.
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ESTABLISHING A TRAINING MODEL FOR ARHI
Ilmari Pyykkö, Esko Toppila, Lido Ao, Kati Iltanen, Martti Juhola
Department of Otolaryngology and Computer Science, Tampere University, Tampere, Finland
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The aim was to create a computer-based modelling for risk factors in ARHI that is user friendly, is able to classify
the risk factors, profile the hearing loss caused by the genetic factors and perform decision making and interact
with persons searching for etiology of ARHI.
In establishing a basic training model for ARHI we investigated 1548 subjects (mean age 74,0 years (range 45
to 99 years) entering to the hearing centre of a tertiary referral hospital for evaluation of hearing aid. Their mean
hearing threshold for the right and left ear at speech area (500, 1000, and 2000 Hz) were 50,1 dB HL and 50,0
dB HL respectively, 685 had significant environmental noise exposure.
We used machine learning methods of Top Down Induction of Decision Trees (TDIDT, Qiuinlan See5 classification
system) and self constructed data mining system based on nearest neighbour selection. The subjects were
dichotomized to those with hearing loss as speech area (500, 1000 and 2000 Hz) 24 dB or less from ISO median
value and those exceeding 24 dB HL. In learning we used statistical and logical operations to squeeze the data
25
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
originally consisting of about 250 questions 96 questions (attributes) that were used in the analysis.
The basic model for ARHI is composed from decision tree with 11 branches. The Decision tree could identify leaves
with minimum of 25 persons by making wrong decisions in 37 %. This error is mainly due to misclassification of
subjects with prominent hearing loss and large size of the leaves. By reducing the leaves to contain 5 persons
the accuracy improved.
The data indicates that the most important predictors for ARHI and their relative importance (percentages in
parenthesis) were: Raynaud’s phenomenon (98%), blood relatives (hereditary factors, 87%), smoking history
(86%), exposure to hand held weapon noise (51%), sensitivity to sunburns (43%), a history of transient ischemic
attack (39%), use of blood pressure medications (32%), use of analgesics (14%) and gender (11%). This model
will be tested for the ARHI database consisting of cases from different EU countries by comparing two different
machine learning methods.
Medical and environmental aspects
Ulf Rosenhall
Department of Audiology, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden
Age-related hearing impairment (ARHI) is a multifactorial process involving numerous, interactive causative
factors. Some factors are intrinsic, caused by biological processes and health factors. Others are extrinsic,
caused by environmental influence. Noise exposure, which is discussed in another lecture of the Round Table,
is one of the most important factors within this domain. However, there is a multitude of other factors that are
of importance for the hearing in old age. These factors are accumulated during the course of many decades,
and therefore they become manifest late in life. The state of health and the presence of different diseases
influence the hearing function in old age. Examples of medical disorders that have been related to poor hearing
are cardiovascular disease, like hypertension and hyperlipidaemia / hypercholesterolaemia, diabetes mellitus,
some infectious diseases, and middle ear infections. The general health situation is also of importance for the
state of hearing, the presence of many medical conditions, irrespective of which, and polypharmacy, can be
related to poor hearing in old age. A healthy lifestyle is of great importance to maintain good health, including
the aural health. One factor that has been studied is smoking, and there is evidence that smoking is correlated
with hearing impairment. Another possible ototraumatic factor is alcoholism. There are also factors that might
protect against ARHI, like physical fitness and healthy dietary habits. The socio-economic factor, expressed by
the social class affiliation and the level of education, is also related to hearing function. Extrinsic, environmental
factors have been correlated to poor auditory function. Such factors are ototoxic drugs, organic solvents, heavy
metals, and head trauma. In the present communication factors of importance to the hearing capacity in old age
are discussed. This discussion includes a literature review and attempts to evaluate the level of evidence for the
factors discussed.
The diagnosis of auditory processing disorder
Claus-Michael Schmidt
Department of Phoniatrics and Pediatric Audiology, Muenster University Hospital
Kardinal-von-Galen-Ring 10, 48149 Muenster - Germany
Auditory processing includes sound localization and lateralization, auditory discrimination, auditory pattern,
26
recognition, temporal aspects (integration, discrimination, ordering, masking), auditory performance in competing
acoustic signals and auditory performance with degraded acoustic signals. (Central) Auditory Processing Disorder
((C)APD) refers to difficulties in the perceptual processing of auditory information as demonstrated by poor
performance in one or more of the above skills (ASHA 2005, ASHA 1996, Bellis 2003; Chermak & Musiek 1997).
APD is diagnosed on the basis of difficulty in identifying or discriminating sounds despite normal peripheral
hearing. Difficulty in understanding speech in noise is the most common manifestation (Dawes 2008). The APD
consensus statement published by the German Society for Phoniatry and Paedaudiology (Nickisch et al. 2007)
gives a wide definition of this neurocognitive construct, including isolated problems of auditory processing and
perception (bottom-up), combined with other deficits such as ADHD or learning disorders or as a symptom of
these deficits (top-down). According to a scientific controversy concerning definition, association with learning
problems and causation, no gold standard is established for diagnosis of APD. Nevertheless, the number of children
with suspected APD introduced to our department is growing. Mostly visiting primary school classes, they present
with problems in hearing, especially in noisy environment, auditory short term memory, speech and language
development, reading and writing abilities, attenuation, concentration, emotion, behavior and learning. Frequently,
school career is at risk. The “Muenster APD diagnostic protocol” includes audiological history and examination,
periphereal and central audiologic tests, speech and language diagnosis and a psychological investigation. Our
aim is to point out a deficit-based profile of abilities. The most frequent subtypes and symptom constellations
such as „auditory processing disorder“ (poor speech-in-noise discrimination), „auditory perception disorder”
(phonological short term memory deficits, SLI and developmental dyslexia) and „symptomatic APD” (secondary
to attention deficit disorders or learning disability) are described. Consequences for further diagnostics and
therapy are illustrated.
Visualization of multifunctional nanoparticles at light and electron
microscopic level in in-vitro systems
Antoinette Schrott-Fischer A
Department of Otolaryngology, Medical University of Innsbruck, Innsbruck, Austria
Multifunctional, highly penetrating nanoparticles may be future delivery vehicles to carry and release drugs
precisely to targeted tissue sites and selected cells in the inner ear. Basic science not only needs to evaluate
toxicity and bioefficacy of such new drugs but also need to visualise submicroscopic particles with light
microscopic techniques. Pros and Cons of different labelling systems and confirmation of localization at electron
microscopic level will be presented and discussed.
The relationship between central auditory processing and other
neuropsychological functions
C. Semenza (Italy)
Department of Neurosciences, University of Padova, Italy
L’incapacità nel percepire e riconoscere i suoni linguistici differisce in modo sottile dalla incapacità nel percepire
e riconoscere i suoni in generale. La percezione linguistica (Liberman et al.,1967): 1) è “categoriale”, 2) è
controllata dall’emisfero sinistro.
I disturbi della comprensione uditiva linguistica nell’afasia consistono della incapacità a riconoscere i suoni
27
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
linguistici e della incapacità a riconoscere il significato dei suoni linguistici.
I suoni raggiungono l’ area acustica primaria (giro temporale trasverso, o giro di Heschl) bilateralmente. Il
vantaggio dell’emisfero sx inizia quando il parlante riconosce il suono come appartenente a una lingua conosciuta.
La sequenza di elaborazione è: analisi acustica > analisi fonologica > analisi lessicale.
Secondo la “teoria motoria” della percezione linguistica (Lieberman & Whalen, 2000), il segnale acustico sarebbe
confrontato con la rappresentazione del movimento articolatorio necessario per produrlo. I fonemi verrebbero
riconosciuti in base a tale confronto.
Dall’area acustica primaria l’informazione andrebbe nell’emisfero sx e seguirebbe due vie:
-via ventrale (cosa?), verso aree adiacenti nel lobo temporale: per comprensione di parole già note
-via dorsale (come/dove?), verso il lobo parietale e frontale: per segmentazione fonemica e messa in memoria
a breve termine di parole nuove
La comprensione esplicita dei fonemi non appare necessaria alla comprensione linguistica.
Alcuni pazienti possono discriminare /da/ e /ta/ ma non comprendono le parole udite
(danno via ventrale). Altri pazienti comprendono le parole udite ma non sono capaci di discriminazione fonemica
(danno via dorsale). In questo caso non possono ripetere le non parole.
L’accesso alla comprensione della parola (via ventrale), avviene tramite l’accesso a un deposito,
il lessico fonologico di entrata che è il vocabolario contenente tutte le parole conosciute.
Un deficit di comprensione linguistica può essere a vari livelli: 1) elaborazione uditivo/fonologica, 2) elaborazione
fonologica, 3) lessicale/semantico, sintattico.
I disturbi del riconoscimento e dell’elaborazione uditiva che seguono a danno corticale includono una serie di
condizioni che interessano sia i suoni non linguistici che i suoni linguistici.
1) sordita’ corticale 2) agnosia acustica 3) amusia recettiva 4) turbe comprensione nell’afasia 5) anomia
acustica.
La diagnosi differenziale deve tenere conto, di fattori come: stato dell’udito periferico, grado di attenzione,
capacità di risposta (non inficiata da turbe prassiche o di indicazione/localizzazione), capacità percettive centrali
(non agnosia), capacità cognitive generali e funzioni esecutive, stato della memoria semantica (il disturbo può
eccedere l’afasia).
Si distingue la sordita’ verbale pura (afasia subcorticale sensoriale) provocata da raro danno ad afferenze ad
area di Wernicke da aree acustiche primarie. l paziente non comprende, pur udendoli, i suoni linguistici. Gli altri
aspetti del linguaggio sono intatti. La sordità verbale compare anche nell’afasia di Wernicke. Per danno all’area
di Wernicke: la sordità verbale si somma ad altri sintomi.
Nell’afasia di Wernicke si sommano, alle turbe fonologiche (sordità verbale), turbe semantiche.
Nell’afasia transcorticale sensoriale ci sono esclusivamente turbe semantiche. In ambo i casi l’eloquio è infarcito
di errori semantici e fonologici. In alcune condizioni afasiche la cattiva comprensione è determinata solo da un
deficit sintattico. L’anomia acustica è una rarissima condizione in cui il paziente è incapace di denominare gli
stimoli uditivi riconosciuti. Forse è associato inevitabilmente alla sordità verbale pura.
complex disorder with both environmental and genetic factors contributing to the sensory deficit as indicated
by several heritability studies. Heritability estimates ranged between 0.25 and 0.75 depending on the study
design. The contribution of various environmental factors has been relatively extensively studied. In contrast,
investigations to identify the genetic risk factors have only recently been initiated. So far, two genome wide
linkage studies have been performed, resulting in the localization of different susceptibility regions for ARHI,
although none of them reached genome wide significance levels. In addition, a number of association studies on
candidate susceptibility genes have been performed. Some of these failed to detect significant associations with
ARHI. Other studies succeeded in identifying genes that are associated with ARHI. A significant and replicated
association was found between ARHI and a polymorphism in N-acetyltransferase (NAT2), an enzyme involved
in the metabolism and detoxification of cytotoxic and carcinogenic compounds. ARHI was also associated with
several SNPs in a 13 kb region in KCNQ4, a potassium channel that is also involved in a monogenic type of
hearing loss. In addition, GRHL2, encoding a transcription factor probably involved in the maintenance of the inner
ear epithelium, has recently been identified as ARHI susceptibility gene. Also, acquired mitochondrial mutations
have been proposed as cause of ARHI in humans. These include both the “common” mitochondrial DNA ageing
deletion of 4977 bp and an accumulation of many different mitochondrial mutations. Despite of these first series
of studies, the impact of genetic factors on ARHI remains largely undeciphered. Therefore, the first whole genome
association studies are currently being performed.
GENETIC ASPECTS OF AGE RELATED HEARING IMPAIRMENT
Lut Van Laer, Guy Van Camp
Department of Medical Genetics, University of Antwerp, Antwerp, Belgium
Age-Related Hearing Impairment (ARHI) is the most prevalent sensory impairment in the elderly. ARHI is a
28
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The XIV International Symposium
in Audiological Medicine
Papers
September 18-21, 2008
Sala S. Francesco, Ferrara
Friday 19th September
➛ 11.00 - 13.00 SESSION I
GENETICS AND VESTIBULAR DISORDERS
Moderators: K. Steel, E. Marciano
01
Expression of Connexin 26, 31, 36, 43 and 30 (Cx26, 31, 36, 43, 30) in the human cochlea
Liu W, Boström M and Rask-Andersen H
Dept. of Otolaryngology, Uppsala University Hospital, Uppsala, Sweden
Objective
Mutations in GJB2 and GJB6, the genes encoding the gap junction proteins Connexin 26 and 30 (the major isoforms found in the mammalian
cochlea) are associated with approximately half of all cases of human autosomal nonsyndromic prelingual deafness in Caucasian populations.
The pathogenesis of deafness due to GJ Connexin mutations remains obscure partly because histological and molecular examinations carried
out in the human ear are infrequent. Here we analysed for the first time the expression and distribution of CX26 and CX30 in fresh human
cochlea. Other connexin proteins, i.e.Cx31,36,43,etc., were also analysed.
Material and method
Human cochleae were taken out at occasional surgery. Immunohistochemistry including confocal microscopy were carried out in decalcified
specimens.
Result
The Cx26 and Cx30 proteins are widely expressed in the human cochlea. In the lateral wall there was strong antibody co-labeling for CX26
and CX30 which support the existence of channels comprising heteromeric CX26/CX30 connexons. In the organ of Corti there was little
co-labeling suggestive of a homomeric construction. CX30 and CX26 (to a minor grade) were also expressed independently in spiral ganglion
perikarya and associated nerve terminals together with CX31 and CX36; a gap junction protein specific to neurons. Cx43 exists in the satellite
cells of SG neurons.
Conclusion
Gap-junction-based electrical synapses are not known to occur in mammalian auditory system other than in bats suggesting a role for fast
electrical nerve transmission useful for echo-location. Their intriguing role in the processing of human auditory nerve signaling is to be
studied further.
02
Adducin 1 mutation as a possible mechanism leading to endolymphatic hydrops
Teggi R1, Manunta P2, Lanzani C2, Zagato L2, Bussi M1
1 ENT Division, University “Vita-Salute” San Raffaele, Milano, Italy
2 Division of Nephrology, Dialysis and Hypertension, University “Vita-Salute” San Raffaele, Milano, Italy
Objective
Ménière’s Disease (MD) is an inner ear disorder characterized by recurrent episodic vertigo, fluctuating hearing loss, aural fullness and
tinnitus. Raised endolymphatic pressure (hydrops) is commonly accepted as a causal condition. Approximately 90% of cases of MD are
sporadic whereas the remaining 10% of cases are linked to genetic factors. The ionic composition of endolymph may depend on the activity
of Na-K ATPase transporters. Adducin is a heterodimeric cytoskeleton protein consisting of three subunits (alpha, beta, and gamma) coded by
three different genes (ADD1, ADD2, and ADD3). ADD1 Gly460Trp polymorphism is associated with salt-sensitive hypertension and increased
Na-K pump activity in transfected cells. In order to verify the role of adducin in the development of MD,
Methods
We genotyped 28 patients affected by definite MD according to American Academy of Otolaryngology – Head and Neck Surgery Foundation
(AAO-HNSF) criteria. Results were compared with those from two different control populations (normotensive control group from San
Raffaele Hospital; Belgian general population group).
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The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
Results
We have not found any significant difference in the distribution of ADD2 C1797T and ADD3 IVS11+386A/G polymorphism genotypes. On the
other hand, the frequency of ADD1 Trp allele is significantly increased in MD patients compared to controls. In Belgian normal population
Trp carrier was present in 32% of subjects, while in control normotensive group in 30% of cases; on the opposite 54% of MD subjects
presented GlyTrp or TrpTrp genotype (respectively χ2= 5.29, p=0.0013 compared with Belgian population and χ2= 4.46, p=0.0034 compared
with control group)
Conclusions
Our data support the hypothesis that an increased Na-K ATPase activity may be one of the pathological mechanisms provoking hyperosmolarity
in endolymph which in turn may lead to hydrops; it may act as a co-factor, leading to increased possibilities of developing it.
03
Genetic evaluation in the congenital hearing loss: the Campania Newborn Hearing
Screening Programme experience
Lilli G, Riccardi P, Errichiello M,Toscano F, Barrier F,Continisio P, Continisio GI, Auletta G, Landolfi E, Munaretto A,
Pisacane , Chinetti I, Iossa S, Franzé A, Marciano E
Audiology Unit - Neuroscience Dept. and Pediatrics Dept. – University of Naples “Federico II”
Congenital permanent hearing disorders represent one of the most common disabilities in infants (incidence: ≥1:1000). They can effectively
influence both the speech and neurological development of the deaf children. The timing of the diagnosis has fundamental importance for
the outcomes of the rehabilitation of the newborn found hearing impaired. Since 2005 in a wide region of southern Italy, the Campania, has
been performed a newborn hearing screening programme (NHS). Almost 65.000 births per year are submitted to OAE tests in the most of
the regional delivery points. The Audiology Unit of the University of Naples “Federico II” leads the programme and collects the genetics data
of the children found deaf within this NHS programme. So far about 60% of congenital hearing loss has a genetic aetiology. The aim of this
study has been to analyze the DNAs of these young patients and the correlation with both the audiologic data and the obtained rehabilitation
outcomes. A molecular screening for genes GJB2 and GJB6 has been performed for the most of the children showing hearing loss. The
subjects found negative for these two genes mutations have been submitted to others genetic assessments. The presence of GJB2 mutations
is ascertained through direct sequencing of amplified PCR fragments of the coding region (exon 2) of GJB2 gene. The presence of the
deletions encompassing part of the GJB6 gene (del GJB6-D13S1830, del GJB6-D13S1854) is analyzed by PCR by tests for the combined
detection. Molecular screening analysis for GJB2 identified several mutations, meanwhile the analysis for the two deletions upstream the
GJB6 gene revealed no presence of these two alterations in all analyzed patients. The molecular screening confirms its important for an early
and accurate diagnosis and can be definitively important for the expectations for the very young deaf children rehabilitation.
04
A Biobank as a tool to improve knowledge of genetic hearing loss
Alemanno MS1, Ortore RP2, Palladino T1, D’Ecclesia A2, Zadro C3, Gasparini P3, Zelante L1, Melchionda S1, Vigliaroli
L2, Carella M1
1
Unità di Genetica Medica, IRCSS”Casa Sollievo della Sofferenza” San Giovanni Rotondo, Foggia-Italia
2
Dipartimento di Otorinolaringoiatria, IRCSS”Casa Sollievo della Sofferenza” San Giovanni Rotondo, Foggia-Italia
3
Dipartimento di Genetica Medica, IRCSS “Burlo Garofano” Trieste-Italia
Objectives
Hearing loss is a frequent disorder with an incidence ratio of 1:1000 and a correct diagnosis is very important because hearing is critical to
speech and learning abilities. This work aims to integrate molecular and clinical data in order to: i) identify new genes responsible of nonsyndromic hearing loss; ii) set up an epidemiological study of genes already involved in non-syndromic deafness. In order to reach those aims
we propose to collect a large cohort of patients via a biobank institution.
Methods
A specific recruitment form has been designed to record samples in the biological bank. All the informations concerning the audiological
phenotype of each patient have to be collected by an accurate clinical evaluation, familiar and personal anamnesis, excluding syndromic
deafness. After obtaining informed consent, peripheral blood will be obtained and DNA isolated from blood leucocytes according to standard
methods. DNA samples will be stored according to Italian rules, while personal data will be managed according to the instruction of the
enclosure B of the D.Lgs 196/03.
All the clinical data of the patients will be recorded into the established database and, to ensure confidentiality, each sample in the database
will be univocally identified by a code number.
Results
This form has been already validated by a pilot applicative study started in january 2008 in the Audiological division of Casa Sollievo della
32
Sofferenza Hospital. Our purpose is to divulgate it in the main national and international audiological centers, in order to recruit a large record
of patients.
Conclusion
The biobank is an excellent support for audiological and genetic research; it could be considered as a screening for candidate genes and to
indagate genotype-phenotype correlations. Sharing the audiological and genetic data within scientific community will reveal fundamental to
improve the knowledge of genetic hearing loss.
05
Audiological Phenotype of patients showing connexin 26/30 related deafness in a
cohort including 628 patients
Cama E1, Santarelli R1, Melchionda S2, Palladino T2, Carella M2, Toffolatti L3, Incognito A1, Zelante L2, Arslan E1
1
Servizio di Audiologia e Foniatria, Dipartimento Specialità Medico-Chirurgiche, University of Padua, 35128
Padova, Italy
2
Servizio di Genetica Medica, IRCCS-Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy
3
Unità Operativa di Anatomia Patologica, Istologia Patologica, Citodiagnostica e Citogenetica, Ospedale Generale
“Cà Foncello” P.za Ospedale, 1 31100 Treviso, Italy
Mutations in the GJB2 gene are found to account for approximately 50% of cases of autosomal recessive non-syndromic deafness. A 342-kb
deletion truncating the GJB6 gene has been associated with autosomal recessive non-syndromic deafness, mostly as digenic inheritance of
the Cx30 deletion/Cx26 mutation. The following retrospective study describes audiological features and genotypes of a large cohort of 628
hearing impaired patients who underwent genetic screening for the GJB2/GJB6 genes and received follow-up care at our centre between
January 2002 and July 2008. Mutations in GJB2/GJB6 genes were found in over than 30% of cases. Twenty-seven different genotypes
causing deafness in more than 25% of patients with either biallelic mutations or digenic inheritance GJB2/GJB6 were identified. The most
frequent mutations of the GJB2 were 35delG, L90P, M34T, delE120 and R184P. The 342-kb deletion del(GJB6-D13S1830) was also quite
frequent. In more than 14% of cases the cause of deafness was the homozygosis for the most common mutation among European people
the 35delG. Although more than 70% of patients with biallelic/digenic inheritance of the Cx30 deletion/Cx26 mutation exhibit a severe to
profound hearing loss, mild and moderate impairment were also found. Other clinical data such as course and degree of bilateral involvement
were analysed too.
06
“AUDIOLOGICAL PROFILES” AND GJB2 MUTATIONS: FERRARA EXPERIENCE
Berto A1, Castiglione A1, Pellati D1, Gualandi F2, Martini A1
1
UO di Audiologia, Dipartimento Discipline Medico-Chirurgiche della Comunicazione e del Comportamento –
Università di Ferrara
2
UO di Genetica Medica, Dipartimento Riproduzione e Accrescimento – Università di Ferrara
In many world populations, mutations in the connexin 26 gene (GJB2) are the most common cause of autosomal recessive nonsyndromic
hearing loss, account for nearly half of cases.
To date, approximately one hundred recessive mutations have been identified (The connexin homepage, 2008) and a significant difference
in the frequency and distribution of the mutations has been observed in different populations. The mutation 35delG accounts for up to 70%
of the pathologic alleles in European and American Caucasian populations.
The aim of this work was to describe audiological profiles in patients with GJB2 hearing impairment.
Five hundred and eighty four hearing impaired patients, seen at the Audiology department of Ferrara between January 2003 and July 2008,
were investigated: a retrospective analysis of audiological features and genotypes was carried out.
Mutations in GJB2 genes were found in 193 (33,04%) of the probands, 101 (17,3%) with biallelic mutations: 68 (67,3%) were homozygous,
33 (32,7%) compound heterozygous.
All categories of hearing loss (HL) severity were found: 29 (28,7%) cases with mild or moderate HL, 19 (18,8%) cases with severe HL, 53
(52,5%) cases with profound HL.
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The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
07
LARGE VESTIBULAR AQUEDUCT SYNDROME: AUDIOLOGICAL, RADIOLOGICAL, CLINICAL AND
GENETIC FEATURES
Berrettini S, Forli F
Ear Nose and Throath Unit, Neuroscience Department, University of Pisa, Italy
The aim of this study was to analyze the clinical, audiological, radiological and genetic features of a group of patients affected with large
vestibular aqueduct syndrome (LVAS).
17 patients affected with LVAS, diagnosed by means of high-resolution magnetic resonance imaging of the inner ear, with three-dimensional
reconstructions of the labyrinth and by high resolution spiral computed tomography of the temporal bone, executed only in the eldest patients,
have been submitted to a complete audiological evaluation, to a thyroid functional and ultrasonographic study and molecular study of Pds
gene.
The clinical presentation of LVAS was very variable in our group of patients. The enlarged vestibular aqueduct was bilateral in 15 cases and
unilateral in 2; it was the only malformation of the labyrinth in 12 patients, while it was associated to other inner ear anomalies in the other
5. The hearing loss was very variable in degree (from mild to profound), age at onset and progression. Moreover among the 17 patients,
10 resulted clinically affected by Pendred’s syndrome, 3 by distal renal tubular acidosis associated to large vestibular aqueduct, while in
three patients the large vestibular aqueduct was not syndromal. Finally we identified mutations in Pds gene in 5/10 patients with Pendred’s
syndrome.
Our data underscore the frequent role of the large vestibular aqueduct syndrome in the pathogenesis of sensorineural hearing loss and the
overall wide variability in its audiological features. It is also highlighted that LVAS is often part of some syndromal diseases, most of which
are Pendred’s syndrome, which is often misdiagnosed because of the varying degree of thyroid symptoms. This study also underscores the
possible role of hydro-electrolyte and acid-base endolymphatic fluid disorder s in the pathogenesis of LVAS.
08
Effect of noise on the vestibular labyrinth: functional and immunohistochemical
analyses in the experimental model
Fetoni ARa, Picciotti Pa, Troiani Db, Paludetti Ga
a
Institute of Otolaryngology and bInstitute of Human Physiology, cDepartment, Catholic University, Largo F. Vito,
00168 Roma, Italy
Loud noise causes damage to the cochlea with subsequent noise-induced hearing loss (NIHL); the effect of acoustic trauma on the
vestibular system has been given less attention and still remains a neglected aspect. Vestibular alterations after acoustic trauma have been
reported both in patients and animals. Up to now, the results of clinical studies remain controversial and neither a systematic functional
analysis of vestibular alterations distinguishing between the semicircular canal and the macular components nor a correlation between the
acoustic and the vestibular functions have been performed. This study is thus addressed to the analysis of the function of the semicircular
canals in the acute acoustic trauma by measuring auditory brainstem responses (ABR) and vestibulo-ocular reflex (VOR) gain and by the
immunohistochemical detection of the endothelial cell-specific mitogen, Vascular Endothelial Growth Factor (VEGF) and the free radicalinduced lipid peroxidation marker 4-hydroxy-2-noneal (4-HNE). Acoustic trauma, induced by a continuous pure tone of 6 kHz, at 120 dB SPL
for 40 minutes, yielded a hearing threshold shift of 60 dB SPL on day 1 after exposure in the frequency range from 6 to 20 kHz, it underwent
a partial recovery on day 7 and reached a value of about 20 dB SPL on day 21.A significant decrease of VOR gain was observed on day
1, recovery was detected at day 3 and completed at day 21. Both Western Blotting and Immunostaining showed increased cochlear VEGF
expression in noise-exposed animals. Before trauma no VEGF expression was found in the vestibular region, it progressively increased after
trauma. No specific staining for 4-HNE was found in both cochlear and vestibular regions before noise; 4-HNE expression increased in both
regions after trauma. Our data illustrate the functional vestibular impairment due to noise exposure and suggest a role for VEGF addressed
to successfully repair the damage in the vestibular end organs.
09
Superior Semicircular Canal Dehiscence Syndrome: a possible explanation for the
variability of symptoms ?
Brandolini C*, Castellucci A, Pirodda A*, Modugno GC
*Cattedra di Audiologia e Foniatria e Scuola di Specializzazione in Audiologia e Foniatria
Dipartimento di Scienze Chirurgiche Specialistiche ed Anestesiologiche, Università di Bologna
http://open.cineca.it/entunibo
34
It is well known that dehiscence of the superior semicircular canal (SSCD) can induce vestibular and/or cochlear symptoms on the basis
of the so-called “third mobile window” effect. Epidemiological and radiological studies, which document the relative frequency of this bone
defect, and the focalisation of the semeiological-diagnostic aspects emerging from the analysis of even fairly large case studies suggest that
SSCD should be considered worthy of independent classification. However, several factors are not yet well understood such as etiological
and pathophysiological aspects. One of the most important matters of discussion concerns the variability of symptoms, which could be due
either to morphologic or to functional (micro-mechanical) conditions. In order to yield a contribution to clarify this aspects, we performed
a correlation study between symptoms and HRCT morphological details concerning a series of 44 cases of SSCD. A logistic regression
analysis was performed to find out a possible statistically significant correlation. Results seem to indicate a correlation between tinnitus and
position of the otic capsule with respect to the cortical limits of the temporal bone and between vertigo and air-bone gap and the degree
of canal occlusion which could justify a change in micromechanics of labyrinthine fluids. These preliminary findings, which deserve further
confirmations, indicate the utility of more accurate morphological investigation which could be obtained by a volumetric analysis of the HRTC
data using specific rendering algorithms.
10
IMAGING EVALUATION IN OTOSCLEROSIS: SINGLE PHOTON EMISSION COMPUTED TOMOGRAHY
VERSUS COMPUTED TOMOGRAHY
Berrettini S1, Ravecca F1, Volterrani D2, Neri E3, Forli F1
1: Ear Nose and Throath Unit, Neuroscience Department, University of Pisa, Italy
2: Division of Nuclear Medicine, Oncology Department, Transplants & Advanced Technologies in Medicine,
University of Pisa, Italy
3: Division of Diagnostic and Interventional Radiology; Department of Oncology Transplants and Advanced
Tecnologies in Medicine, University of Pisa, Italy
The aim of our study was to demonstrate the utility of diphosphonate bone Single Photon Emission Computerized Tomography (SPECT) in
diagnosing otosclerosis and to correlate SPECT findings with age, gender and sensorineural hearing loss; we also evaluated High Resolution
Computerized Tomography (HR-CT) ability in detecting otospongiotic and otosclerotic foci and correlated HR-CT results with age and
sensorineural hearing loss.
73 subjects with surgically confirmed otosclerosis underwent SPECT and 45 of 73 patients also performed a HR-CT of the petrous bones.
In the patient sample examined in this study SPECT demonstrated a sensitivity of 95.2% and a specificity of about 96.7%. By correlating
SPECT findings with age, gender and sensorineural hearing loss, we have moreover revealed an inverse relationship between bone radioactivity
and age (i.e., greater disease activity in younger patients) and a direct relationship between bone radioactivity and severity of sensorineural
impairment in younger patients. In the 45 patients who underwent CT too, HR-CT sensitivity seemed to be lower than SPECT sensitivity (52%
versus 95.2%), without a significant correlation between HR-CT results and age; however, a significantly greater number of positive HR-CTs
was found in patients with worse sensorineural hearing loss.
In conclusion SPECT seems to be highly effective in differentiating normal from pathological petrous bone and also provides a quantitative
evaluation of the disease activity. HR-CT shows a lower sensitivity (about 58%) and provides only structural information.
11
Lymphocytes immunphenotyping in contralateral type delayed endolymphatic
hydrops
Quaranta N, Vacca A*, Candreva T, Capuzzimati L*, Quaranta A
Otolaryngology, Department of Ophtalmology and Otolaryngology, University of Bari, Bari, Italy
* Section of Internal Medicine, Department of Biomedical Sciences and Human Oncology, University of Bari,
Bari, Italy
Background
The clinical disorder of delayed endolymphatic hydrops (DEH) can be divided into ipsilateral and contralateral types. In the contralateral
type profound hearing loss in one ear precedes the onset of fluctuating hearing loss in the contralateral ear, with or without episodic vertigo.
The contralateral type is believed to have an autoimmune etiology. The aim of this study was to study in patients with contralateral DEH the
immunophenotype of circulating lymphocytes.
Study Design
The immunophenotype of circulating white cells was evaluated by direct immunofluoresence and multiparametric analysis by means of flow
citometry with monoclonal antibodies. In particular CD3, HLA-DR, CD4, CD8, CD16/CD56, CD19, CD5, CD20, TCRγ/δ, conjugated with FITC,
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The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
PE, PerCP (Becton Dickinson) were evaluated. Nine patients affected by contralateral type DEH were evaluated. Results were compared with
a group of patients affected by unilateral juvenile deafness and a normal controls.
Results
All patients presented an abnormal lymphocytes immunophenotype. In 7 patients there was an increase of the CD4/CD8 ratio. In 5 patients
there was an increase in activated T-cells (HLA-DR). An increase in CD4+ was present in 3 patients and a reduction in CD8+ in 4 cases.
An alteration of B cells was present in 2 cases and in 1 case there was an increase in NK cells. White cell immunophenotype was normal
in all controls
Conclusions
Patients with contralateral DEH may undergo an immuno-mediated attack against the opposite ear. The identification of the immune
mechanisms may open the way to immunosuppressive treatments in order to reduce the risk of bilateral hearing loss.
12
Hemostatic alterations in patients with acute peripheral vertigo
Ursino F, Nacci A, Fattori B
Chair of Audiology and Phoniatric – Department of Neuroscience University of Pisa – Pisa, Italy
The etiopathogenesis of acute unilateral peripheral vestibulopathy (APV) is still debated; vascular factors may play a role. We looked for
possible hemostasis alterations in a group of patients with APV of an unknown nature. We evaluated blood parameters known to be involved
in circulation disorders (total and HDL cholesterol, tryglycerides, apolipoprotein A and B, lipoprotein(a), homocysteine, folate, prothrombin
time, activated partial thromboplastin time, fibrinogen, D-dimer, antithrombin III, protein C, protein S and activated protein C resistance), in a
group of 50 APV patients (study group) and in 50 patients suffering from Menière’s disease (MD) (control group). Measurements were taken
both during the acute stage and after four to six weeks of pharmacological washout. The patients with APV in the acute phase compared
with the patients with MD exhibited increased plasma levels of fibrinogen, D-dimer, lipoprotein(a), high leukocyte count and low serum folate
concentration. During follow-up leukocyte count decreased in the APV patients whereas fibrinogen, D-dimer and folate were unchanged. Our
results lead us to postulate an involvement of the hemostatic system in APV.
Friday 19th September
➛ 15.00 - 17.00 SESSION II
VESTIBULAR AND HEARING DISORDERS
Moderators: E. Raglan, L. D’Angelo
13
SUBJECTIVE VISUAL VERTICAL AND HORIZONTAL: REPEATABILITY AND RELATIONSHIP WITH THE
PRESET ANGLE
Pagarkar W1, Bamiou D2, Ridout D3, Luxon L
1
Consultant in Audiovestibular Medicine - Royal Free Hampstead NHS Trust, Royal National Throat Nose & Ear
Hospital, Gray’s Inn Road, London WC1X 8DA, UK
2
Consultant in Audiovestibular Medicine and Honorary Senior Lecturer - UCL Institute of Child Health & National
Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK.
3
Senior Research Fellow, Centre for Paediatric Epidemiology and Biostatistics, UCL Institute of Child Health, 30
Guildford Street, London WC1N 1EH
Introduction
The subjective visual vertical [SVV] and horizontal [SVH] assess the function of the otoliths and semicircular canals. There is no standard
procedure for doing these tests with respect to the start position of the linear marker and studies of repeatability have not been reported.
Aims
This study investigated the repeatability of SVV & SVH and its relationship with the direction of deviation of the linear marker [preset angle]
in controls and patients with vestibular pathology.
Subjects & methods
Twelve replicate readings of SVV and SVH were obtained in seventeen controls and nineteen patients with unilateral peripheral vestibular
dysfunction -9 vestibular neuritis [VN] and 10 Menière’s disease [MD]. Repeatability indicator ‘R’ was calculated for three conditions:
36
baseline, after excluding first three readings and separately for clockwise and anticlockwise preset angles. SVV & SVH values were computed
separately for clockwise and counterclockwise direction of the preset angle and compared using the Random Effects Analysis of Variance.
Results
Repeatability of SVV and SVH was better in healthy subjects than in the VN and MD groups, was better for SVH than for SVV and improved
in all three subject groups if the preset angle orientation was constant at clockwise or anticlockwise. SVV depends on the direction of the
preset angle in all subject groups, more so in the VN and MD group as compared to controls [p<0.001]. The SVV is inclined towards the
direction of the preset angle [chi square P<0.001 in all three groups]. A weaker relationship is seen between SVH and the preset angle.
Conclusion
This study indicates that SVV & SVH values are biased by the direction of the preset angle, and the preset angle should be considered when
comparing SVV & SVH values between individuals in different test settings. Further research is needed to explore this effect with and without
visual roll illusion.
14
Rehabilitation tHerapy of peripheral vestibular disorders: our experience
Livi W, Cioni V
Dipartimento Organi di Senso, U.O Audiologia - Università degli studi di Siena
Peripheral vestibular disorders can determinate an imbalance between the two vestibular emisystems or an inappropriate stimulation of
the ampullar receptors (labirintolithiasis). The choice of rehabilitation is based on the identification of the pathological system, on the
temporal phase and on the predominant symptom. To rehabilitate patological oculomotors disorders is necessary to perform special exercises
to stimulate the mechanism of coordination eye-head. The rehabilitation of posture and gait is based on static and dinamic exercises,
gait on particular courses and the use of dynamic computerized posturography to facilitate the compensation mechanisms. The gait on
memorized courses is a technique able to develop spatial analysis; posturography uses softwers dedicated to postural static control ( through
images) and postural dinamic control (through visual aims). Rehabilitation techniques for VPPB includes “ habituation” (Five’s exercises)
and specific manouvres for CSP ( Semont’s exercise) and for CSL (Vannucchi, Gufoni or barbecue manouvres). Our study included 40
patients (age in range 40-65 years) suitable for vestibular rehabilitation, examined in the department of ORL in Siena from January 2006
to January 2008. Subjects were divided into four groups: 30 subjects with vestibular neuritis, 5 with Meniere’s disease, 4 with vestibular
aspecific disorders and a suspect of phobic postural vertigo, 1 with acoustic neuroma. Our rehabilitation included particular courses and
computerized balance training associated with a correct psichological approach; in fact, a strong emotional element emerged in all patients.
This study makes it clear that the rehabilitation is useful in vestibular neuritis and in aspecific disorders with psichiatric symptoms.
15
SELF-THERAPY IN THE TREATMENT OF BENIGN PAROXYSMAL POSITIONAL VERTIGO CAUSED BY
CUPOLOLITHIASIS IN THE POSTERIOR SEMICIRCULAR CANAL: A NEW PROPOSAL
Pagliari AV, Forner P, Bonelli A, Blotta P
Ospedale Maggiore di Crema, ENT Department
Benign paroxysmal positional vertigo in the posterior semicircular canal (BPPV) has long been treated with positional manoeuvres (expecially
Semont, Brandt-Daroff and Epley) with a very high success percentage. The Authors wanted to check whether Patents could be taught to
perform Semont’s therapeutic manoeuvre to make the treatment easier and to concurrently lighten the specialist’s work load.
30 Patients with documented acute BPPV were enrolled for this purpose; they were divided into two homogeneous groups and treated with
Semont’s manoeuvre for 3 weeks. The first group underwent liberatory manoeuvres performed in hospital by the Audiologist, while the second
group was taught how to perform the manoeuvre in order to do it at home.
The two groups’ results could be more or less overlapped ( 93,3% of healings), which confirms the possibility of proposing this solution to
Patients, whose physical conditions allow them to correctly perform the manoeuvres.
Considering the above, we can conclude that our experience was doubtless positive. The very high percentage of healing and the improvement
achieved in all individuals, who performed self-treatment at home enable us to declare that the latter’s efficacy overlaps or even exceeds
traditional treatment performed in specialist Outpatients Clinics.
From the Audiologist’s perspective, considering the high number of Patients suffering from BPPV, the proposal for self-treatment at home
involves a lesser work load, with subsequent positive repercussions on the Unit’s activity, which can thus channel human and professional
resources to other cases.
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The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
16
18
Speech audiometry is a good indicator of real hearing difficulties faced by the elderly and should be preferred to pure-tone audiometry.
However, it is often performed because it is compulsory and their results are not considered in clinical practice. Furthermore, as far as we
know, there are no reports concerning its role in establishing the ability to communicate and the dynamic interaction between hearing and
motor responses.
OBJECTIVE: aim of the study was to realize a new speech test able to allow a rapid evaluation of comprehension by assessing the ability to
process simultaneously presented auditory and visual information and to perform simple motor tasks with tridimensional and standardized
objects coming from familiar tools.
METHODS: 20 lists with 5 verbal tasks per list have been chosen from a pool of 960 combinations. Test has been balanced for the same
motor execution difficulty, colour types and spectral frequency pattern and recorded on a CD in different languages. Great elderly patients
(mean age 87,9 ± 6.4; 33 women and 2 males with Minimental State Examination: > 20) with age-related hearing loss were submitted to
audiological evaluation with speech audiometry with bisyllabic words and with the new test. Their motor, cognitive impairment and quality of
life have also been determined. Variance analysis has been performed (SPSS 13.0).
RESULTS: There were no significant difference in the intensity for each frequency of the used 20 lists. (p> 0.05) The new test was related
to speech audiometry with bisyllabic words (traditional) but gave significantly different results. (p<0.001; p=0.002). Both speech tests were
related to the motor performances (Tinetti scale).
CONCLUSION: Speech audiometry with verbal task and motor response resulted a quick and easy method to be performed in the elderly.
Further studies are needed to better define its clinical role on hearing id fitting.
Superficial siderosis of the central nervous system (SH-CNS) is a rare disorder due to chronic or recurrent subarachnoidal bleeding into the
cerebrospinal fluid that causes hemosiderin storage/deposition in leptomeningeal and subpial tissue, spinal cord and cranial nerves. Patients
affected by SH-CNS typically show progressive hearing loss, ataxia, pyramidal signs, and, less commonly, dementia. Since the eighth cranial
nerve is particularly susceptible to the deposition of hemosiderin it is not surprising that auditory system involvement in SH-CNS has most
often attributed to retrocochlear damage whereas only few reports suggested a cochlear site of lesion. We present the case of a 73 year-old
women affected by tetraparesis, as consequence of arachnoiditis, who was investigated for bilateral progressive hearing loss and dizziness.
Diagnosis of SH-CNS was already achieved on the basis of gadolinium enhanced MRI.. A severe bilateral asymmetric sensorineural hearing
loss (SNHL) was found at pure tone audiometry. Tympanometry was normal while stapedious reflexes were bilaterally absent. Transient
otoacoustic emissions (TOAEs) were absent, thus suggesting a cochlear site of lesion. Speech audiometry reflected the degree of hearing
loss but discrimination score was bilaterally good (100%). Auditory brainstem responses (ABR) showed an abnormal pattern and a bilateral
latency increase, as observed in retrocochlear involvement of the auditory pathway. In spite of ABR results, middle latency responses (MLR)
were normal. Audiological findings in our patient indicate that SNHL observed in SH-CNS may be due to coexisting cochlear and retrocochlear
involvement of the auditory periphery, as previously suggested by Takasaki K et al (2000). Leptomeningeal and subpial hemosiderin storage
in the ponto-cerebellar angle could easily explain dysfunction of the auditory nerve. In the other hand the cochlear damage was supposed as
a consequence of an altered internal cochlear environment caused by hypoxia or a change in perilymph composition. Nevertheless nowadays
no satisfactory explanation can be given for this unusual audiologic pattern.
SPEECH AUDIOMETRY WITH VERBAL TASK AND MOTOR RESPONSES: A NEW METHOD IN THE
DIAGNOSIS OF AGE-RELATED HEARING LOSS
Cesarani A, Di Berardino F, Barozzi S
Audiology Unit, I.R.C.C.S. Policlinico, Mangiagalli e Regina Elena. Department of Otolaryngology, University of
Milan, via Pace 9, Milan, Italy
17
PSYCHOLOGICAL PROFILE AND SOCIAL BEHAVIOR OF WORKING ADULTS AFFECTED BY MILD TO
MODERATE HEARING LOSS
Monzani D (1), Genovese E(1), Marrara A(1), Forghieri M (2), Galeazzi G (3), Martini A (4).
(1) Audiology Unit. Department of Neurosciences, Head-Neck and Rehabilitation. University Hospital of Modena,
Italy
(2) Department of Psychiatry. University Hospital of Modena, Italy
(3) East London NHS Foundation Trust, UK
(4) Department of Audiology. University of Ferrara, Italy
This study tested the global assumption that working adults with a mild to moderate sensorineural hearing loss experiment more negative
emotional reaction and socio-situational limitation than persons with no hearing problems and that a deterioration of health-related quality
of life on these specific domains would occur. Comparison between 73 hearing-impaired subjects and 96 controls, well-matched for sociodemographic variables, were performed on HHIA, MOS 36-Item Short Form Health Survey (SF-36) and SFQ questionnaires scores and
revealed that the former experience higher level of perceived hearing handicap and a deterioration of health-related quality of life while
investigating emotional and socio-situational domains than the latter (p < 0.005).
While investigating the psychological distress dimension of the hearing-impaired subjects by the mean of the Symptoms Check List (SCL-90),
it emerged that they are more prone to depression, anxiety, interpersonal sensitivity, and hostility than subjects with no hearing problem (p
< 0.05). It is argued that the sensory impairment with its associated disability discourage hearing impaired individuals to expose themselves
to socially challenging situations, producing isolation that leads to depression, irritability, feelings of inferiority or that the same psychological
symptoms, on the other hand, can compound and worsen the picture by influencing social behaviour of the affected persons. Further
perspective studies are need to address the question. Nevertheless, it is concluded that audiological service, despite time and costs involved,
should improve their diagnostic ability by exploring most areas of hearing-impaired subject’s concerns in order not to underestimate their
potentially reduced psychosocial well-being.
38
SENSORINEURAL HEARING LOSS IN SUPERFICIAL SIDEROSIS OF THE CENTRAL NERVOUS
SYSTEM: UNUSUAL AUDIOLOGICAL FINDINGS
Conti G. *, Marchese MR *, Martina BM *, Colosimo C **
*Istituto di Clinica Otorinolaringoiatrica
**Istituto di Diagnostica per Immagini
Policlinico “A.Gemelli” – Università Cattolica del Sacro Cuore – Rome, Italy
19
OVARIAN STEROIDS INFLUENCE THE GENDER DIFFERENCES IN AUDITORY FUNCTION
Al-Mana D1, 4, Ceranic B2, Djahanbakhch O3, Luxon LM1, 4
1
Department of Neuro-otology, The National Hospital for Neurology & Neurosurgery, London
2
Department of Audiology, St. George’s Hospital, London,
3
Newham University Hospital, Academic Department of Obstetrics & Gynaecology, Barts and The London Queen
Mary’s School of Medicine and Dentistry, London,
4
Department of Audiovestibular medicine, UCL Ear Institute, London
The aim of the study was to compare the auditory function between women during the normal ovarian cycle and men over a similar period
of time.
A group of women (n=16) underwent auditory testing and measurement of hormone levels (oestrogen and progesterone) four times during
their ovarian cycle, twice during the follicular and twice during the luteal phases. A group of men (n=13) had auditory tests once a week for
four consecutive weeks to correspond with the ovarian cycle measurements. The test procedures included:
• Transient evoked (TEOAE) and spontaneous (SOAE) otoacoustic emissions
• The medial olivocochlear (MOC) suppression
• Auditory brainstem evoked responses (ABR)
The TEOAE responses were significantly larger (p<0.001) in the women throughout the testing period, with a greater difference in TEOAE
responses observed between the follicular and luteal phase in the women compared with the men (p=0.006). The SOAE frequency shift
changed significantly in the women during the ovarian cycle, with the greatest frequency shift in the late follicular phase (p<0.001), but no
significant frequency shift was observed in the men. The MOC suppression was not different between the women and men, but the MOC
suppression significantly changed in the female group during the cycle which was lower in the late follicular and late luteal phase than in the
early follicular phase (p=0.03). The ABR wave latencies were significantly shorter in the women (p<0.001) with only wave V latency showing
a significant change during the ovarian cycle in the women.
The changes observed in OAEs, MOC suppression and ABR in women but not in men suggests that auditory system is sensitive to the
fluctuation of oestrogen and progesterone during the ovarian cycle, and that the hormonal effect could be one of the contributory factors to
the gender difference in auditory function.
39
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
20
KNJGDMLACLRPC
DMPPCQC?PAF?LB
QRSBGCQÉÊ
FOCUS ON RESEARCH AND EDUCATION IN AUDIOLOGY AND OTOLARYNGOLOGY
The CRS is a non-profit organization, established in 1971 by Amplifon founder, aimed at promoting basic and clinical
research and disseminating knowledge and advances in audiology and otolaryngology. The CRS institutional role is
carried out under the supervision of a Scientific Committee through a wide range of activities. They include:
EFFECTS OF CLASSROOM NOISE AND REVERBERATION ON THE SPEECH PERCEPTION OF
BILINGUAL CHILDREN LEARNING IN THEIR SECOND LANGUAGE
Bovo R
Audiology Department University of Ferrara, Ferrara - Italy
Understanding speech through noise is a skill that becomes adult-like at approximately the age of 15. Bilingual children learning in their
second language appear to be at a double jeopardy when listening under adverse conditions (noise, reverberation, background babble).
The aim of this study was to determine the effects of noise on speech perception skills in 15 typically developing immigrant children, (aged
6-10) learning through their second language and to compare the results with those relative to 15 monolingual, matched peers. The speechto-noise ratio (SNr) to obtain 50% word intelligibility had been measured by two different competitive speech tests and by using an adaptive
2 dB steps method. With ipsilateral energetic masking (narrow-band noise), the immigrant children demonstrated the necessity of a SNr of
16.2 dB (SD 7.9) with statistically significant differences in respect to monolingual children who instead needed a SNR of -1.1 dB (SD 3.9).
It’s reasonable to think that the immigrant children need a larger redundancy of acoustic features with respect to the monolingual children,
for a correct phonological analyses of the verbal message. On the other hand, the contralateral informational masking (continued discourse)
didn’t demonstrate a significant difference between the two groups. This excludes the role of central cognitive factors (i.e. memory, selective
attention) or semantic factors and is consistent with the teacher’s report that the immigrant children displayed a competency in the Italian
language (comprehension, production, reading and writing) that was normal for their age. Thus, the difference between the two groups would
be secondary to diverse phonological skills.
In conclusion, the recommended level of classroom noise and reverberation is based on studies that evaluated speech intelligibility in
normal monolingual children. Nevertheless, the number of immigrant children is probably increasing in developed countries and for them a
recommended 15 dB SNR may be still insufficient for adequate scholastic achievements.
21
™EDUCATIONAL COURSES
A rich programme of educational courses addressed to ENT specialists and residents, audiologists, audioprothesists,
speech therapists and other professionals in the field is implemented every year. These courses, usually held in
Italy, are organized in close co-operation with University ENT Departments and are accredited by the national
ECM Commission.
™INTERNATIONAL CONFERENCES
One or more international conferences are organized every year in co-operation with outstanding representatives
of the scientific community.
™RESEARCH PROJECTS
From 1995 to 2005 the CRS has been involved as contract holder on behalf of the European Commission in important
research projects on the use of Otoacustic Emissions for neonatal hearing screening (AHEAD I and II) and on the
study of genetic deafness (HEAR and GENDEAF).
™SCHOLARSHIPS AND AWARDS
Several Scholarships are granted every year to young trainees in Audiology/Otolaringology in order to support
training stages in different countries, attend international congresses, etc. The last International Prize was awarded
during the XVIII IFOS World Congress in Rome to Prof. Graeme Clark for “his pioneering research and development
of the first clinically successful cochlear implant”.
Cochlear potentials recorded by transtympanic electrocochleography (ECochG)
in patients with auditory neuropathy
Santarelli R1, Starr A2, Michalewski HJ2, Arslan E1
1
Service of Audiology and Phoniatrics, University of Padua, Italy
2
Department of Neurology, University of California, Irvine, USA
Auditory neuropathy (AN) is a recently identified disorder of auditory nerve characterized by prominent auditory temporal processing deficits.
Both auditory nerve and brainstem activities recorded as far-field potentials are typically undetectable whereas cochlear outer hair activities
(otoacoustic emissions and/or cochlear microphonics) are normal. We recorded cochlear receptor (summating potential [SP]; cochlear
microphonics [CMs]) and auditory nerve (compound action potential [CAP]) activities by transtympanic electrocochleography (ECochG) in
8 children and adults with AN. Stimuli were 0.1 ms clicks presented in free-field from 60 to 120 dB SPL. Measures were compared with the
ECochG results from 16 children who had normal thresholds of CAPs.
Receptor CMs were of normal or enhanced amplitude in AN. Neural CAPs and receptor SPs were separately identified in 5/16 AN ears. When
identified SPs were of normal amplitude. In the remaining ears the ECochG potentials were of negative polarity and of normal or prolonged
duration. Rapid stimulus rates were used in six of the AN subjects to help distinguish whether the generators of the negative potentials were
of neural or receptor origin by taking advantage of different amount of adaptation involving CAP and SP. Adaptation in controls resulted
in amplitude reduction of CAP twice that of SP without affecting the duration of ECochG potentials. In five AN ears with CAP, there was a
reduction in amplitude of CAP and SP as controls but with a significant decrease in response duration consistent with post-synaptic disorder
of proximal auditory nerve. In four ears without CAP and with normal duration potentials, adaptation was without effect consistent with
receptor generation. In seven AN ears without CAP and with prolonged negative potential, adaptation was accompanied by reduction of both
amplitude and duration of the negative potential to control values consistent with neural generation. We suggest that cochlear potential
measures may identify pre- and post-synaptic disorders of inner hair cells and auditory nerve in AN.
™BIBLIOGRAPHIC RESEARCH SERVICE
Thanks to its library, that currently subscribes to the main national and international Audiology and ENT journals,
the CRS offers to medical doctors and other healthcare operators a specialized bibliographic research service with
mailing of abstracts and whenever possible copies of papers. For a quick updating a selection of summaries of the
most relevant papers of the recent literature, called INDEX, is also published on line www.crsamplifon.com four
times a year.
WWW.CRSAMPLIFON.COM
41
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
22
NEAR – INFRARED SPECTROSCOPY (NIRS) OF THE AUDITORY CORTEX DURING PASSIVE
LISTENING: PRELIMINARY DATA
Grasso DL, Bembic S°, Bava M*, Clarici A°
E.N.T. Department - IRCCS “Burlo – Garofolo” Children Hospital, Trieste, Italy
°Reproduction Department – University of Trieste, Italy
* Hospital Information System - IRCCS “Burlo – Garofolo” Children Hospital, Trieste, Italy
Introduction
Optical Topography (OT) is a multichannel near-infrared spectroscopy (NIRS) system which allows to detect and localize cortical activation,
trough monitoring changes in cerebral blood flow (CBF) and cerebral blood volume (CBV). This spectroscopic method operates in the near
infrared region of the electromagnetic spectrum (Jobsis, 1977) and, by using optical fibers (or optodes) emitting and detecting infra-red light,
it can measure the localization of a brain regional activation by directly detecting changes in the concentration of oxy-haemoglobin (HbO2)
and deoxy-haemoglobin (Hb), thanks to their different specific spectra in the near-infr
called channel). Total haemoglobin (HbTot) concentration changes can be indirectly derived by summing concentration changes in HbO2 and
Hb. It is quite common to consider HbO2 concentration changes an esteem of CBF and HbTot concentration changes an esteem of CBV, while
there is more ambiguity in interpreting Hb variations (Meek, 2002).
Materials and Methods
NIRS data were acquired during the passive listening of the verbal stimuli list described above. Twenty normal subjects were tested in a dark
and silent room, sitting in front of a computer (Vectra VL, Hewlett Packard) connected to two loudspeakers (Arena™–530, Labtec®), one on
the left and the other on the right of it; this was the apparatus used for the passive listening task, whose verbal stimuli were presented at the
acoustic intensity of 60 Db SHL. Participants staid at a distance of 90 cm. from the loudspeakers, centrally positioned in respect to them, and
wore a blindfold over their eyes in order to limit stimulation to the auditory one as much as possible. The instructions given to the subjects
were “to listen in silence what it will be said to them”. After having correctly positioned the blindfold and the probes and checked for the
adequacy of the signal, the test began and ended 8 minutes and 10 seconds later (the total duration of the passive listening stimulation plus
10 seconds of silence that preceded the presentation of the first stimulus).
Results
We applied the FDR control for multiple statistical tests considering independently the two hemisphere, because of the lateralization in the
adult brain of language function, especially in right handed subjects. The channels whose t-tests resulted significant were all located in the
left hemisphere and were found in association to sentence stimulation and to bi-syllabic words stimulation (Table 2), while no statistical
significances were found with listening of vowel-consonant-vowel syllables and with no one of the stimuli associated to a competition.
The passive listening of sentences significantly activated the only channel 6 (t14 = 3.67; p = .003), located 3 cm. above T3 point of the 10-20
EEG system and anterior in respect to the bitragal line. The passive listening of bi-syllabic words significantly activated the channels 2 (t14 =
2.80; p < .014), 4 (t14 = 2.55; p < .023), 5 (t14 = 2.94; p < .011), 7 (t14 = 3.72; p < .002), 9 (t14 = 2.82; p < .014), 10 (t14 = 2.83; p < .013) and
11 (t14 = 3.46; p < .004). They are widely located in the monitored cortical area and posterior in respect to the bitragal line, with the exception
of channel 11.shows the time course of HbO2 and Hb signals of channels found active in this study during a passive listening task.
Conclusions
Data obtained from NIRS recording show that passive listening of different vocal material could elicit different area activation in the auditory
cortex.
23
SPEECH PERCEPTION TEST IN EARLY IDENTIFICATION OF APD
Palma S, Mennucci E, Guarnaccia MC, Genovese E
Audiologia- Clinica ORL - Università degli Studi di Modena - Via Del Pozzo 71 - 41100 Modena
Objective
This study aims to evaluate the speech perception performances in a group of children aged between 4- 6 years presententing an expressive
language disorder. All the children were admitted to the Audiology Depatment of Policlinico of Modena.
Method
The study was carried out in 30 children aged between 4 and 6 years who presented normal hearing evaluation, normal cognitive skills and
normal language communication abilities (grammar-morphologic and lexical aspects); in all of them a phonetic-phonological disorder was
diagnosed .
The perceptive-speech skills were tested by the Italian version of “Northwestern University-Children’s Perception of Speech” (NU-CHIPS,
Elliott & Katz, 1980) that is “Test di Identificazione di Parole Infantili” (T.I.P.I. 1); the test was administered in quiet and in different levels
of noise.
42
Results
All the children had normal otoscopic findings and normal middle ear function by impedance measurement; afterwards they had to pass a
hearing screening by pure tones 0,25, 0.5, 1.0, 2.0, 4.0, and 8.0 kHz presented at 20 dB HL to each ear separately. Starting from the results
we identified three different groups of children with different speech-perception skills:
1)normal abilities in quiet and in noise conditions
2)reduced abilities in both conditions
3)reduced abilities only in noise conditions
Conclusions
The study confirms the effectiveness of speech perception tests in the evaluation of the speech perception skills also in children with language
disorders; these test can help to identify the children that can reveal an Auditory processing Disorders (APD) in next years.
Moreover, the identification of the two groups (with and without speech perception disorders) can be used to check the state of rehabilitation
protocols.
24
Inner ear dysfunction: the role of non-audiological factors
Pirodda A, Brandolini C, Raimondi MC, Modugno GC*, Degli Esposti D°, Borghi C°
Cattedra di Audiologia e Foniatria e Scuola di Specializzazione in Audiologia e Foniatria, Dipartimento di Scienze
Chirurgiche Specialistiche e Anestesiologiche, Università di Bologna
*Cattedra di ORL e Scuola di Specializzazione in Otorinolaringoiatria, Dipartimento di Scienze Chirurgiche
Specialistiche e Anestesiologiche, Università di Bologna
° Cattedra di Medicina Interna, Dipartimento “D. Campanacci”, Università di Bologna
In the last decade our Group focused its interest on the possibile damage to inner ear by hemodynamic imbalances due to sharp lowering of
blood pressure values followed by an abnormal consequent response of the autonomic system, which could be responsible for a threaten to
the terminal type circulation which yields the blood supply to this organ. In fact, this hypothesis had a number of confirmations by studying
the possible association of sudden hearing loss or mild undiagnosed sensorineural hearing loss and hypotension (1,2), the role of the systemic
response (3), and the prevalence of tinnitus in subjects submitted to different antihypertensive therapy (4), on one hand, and in patients
affected by heart failure (5), on the other hand. More recently, a promising echocardiographic study was carried out in young healthy subjects
with tinnitus as compared to a control group, in order to draw a cardiovascular profile more easily prone to inner ear sufferance (6,7).
These observations led us to consider, more generally, the opportunity to plan a multidisciplinary approach in cases of inner ear disorders of
uncertain origin, including the search of all conditions which could act as a trigger to hemodynamic imbalance and autonomic dysregulation
(8). Among these, metabolic diseases as diabetes mellitus or central nervous system diseases are well known as factors able to influence
hearing, as well as chronic kidney diseases; however, the latter is less frequently considered by audiologists as a possible factor of autonomic
dysregulation by itself. Moreover, it has been recently proposed by us the possibility of including gastric disorders, which could in some cases
influence the autonomic activity and/or induce local changes, in a check list of affections to be excluded when investigating on all cases of
inner ear sufferance lacking a clear explanation (9). In our opinion, in such cases an approach as described could propose more appropriate
prophylactic and/or therapeutic options and contribute to minimize complaints deriving from an inner ear dysfunction.
References
1) Pirodda A, Saggese D, Ferri GG, Giausa G, Grippo MC, Gaddi A. The role of hypotension in the pathogenesis of sudden hearing loss.
Audiology 1997; 36: 98-108.
2) Pirodda A, Ferri GG, Modugno GC, Borghi C. Systemic hypotension and the development of acute sensorineural hearing loss in young
healthy subjects. Arch Otolaryngol Head Neck Surg 2001; 127: 1049-52.
3) Pirodda A, Ferri GG, Montana T, Riggio R, Innocenti G, Di Nino G. Hypotension as an isolated factor may not be sufficient to provoke hearing
impairment. J Laryngol Otol 2004; 118: 941-5.
4) Borghi C, Brandolini C, Prandin MG, Dormi A, Modugno GC, Pirodda A. Prevalence of Tinnitus in Patients with Hypertension and the Impact
of Different Antihypertensive Drugs on the Incidence of Tinnitus: A Prospective, Single-Blind, Observational Study. Curr Ther Res 2005; 66
(5): 420-32.
5) Degli Esposti D, Cosentino ER, Santi F, De Sanctis D, Dormi A, Bacchelli S, Enonguene JS, Brandolini C, Modugno GC, Prandin MG, Rinaldi
ER, Ambrosioni E, Pirodda A, Borghi C. Tinnitus and blood pressure values in heart failure patients. Proceedings of the 17th European Meeting
on Hypertension, Milan 2007,p S171.
6) Pirodda A, Degli Esposti D, Brandolini C, Modugno GC, Cosentino E, Borghi C. Could echocardiography yield a cardiovascular profile of the
tinnitus prone subject? Med Hypotheses 2008; 70: 252-4.
7) Raimondi MC, Brandolini C, Degli Esposti D, Borghi C, Pirodda A. Squilibri emodinamici ed acufeni. Atti del 95° Congresso Nazionale SIO
e Ch CF, Torino 2008.
8) Pirodda A, Brandolini C, Ferri GG, Modugno GC, Degli Esposti D, Borghi C. Inner ear dysfunction of uncertain origin: a multidisciplinary
approach could give something more. Med Hypotheses, accepted for publication.
9) Pirodda A, Brandolini C, Raimondi MC, Modugno GC. The possibile role of proton pump inhibitors on the homeostasis of the inner ear. Med
Hypotheses, accepted for publication.
43
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
Saturday 20th September
➛ 08.30 - 10.50 SESSION III
COCHLEAR IMPLANTS AND HEARING DISORDERS
Moderators: M. Fabiani, R. Illing
improvement in vowels and CVC identification and in bi-trisyllabic words and common phrases recognition both in quiet and in noise
conditions.
Conclusions
In very rare amyloidosis cases the deafness cannot be caused by VIII neuropathy or central auditory pathway involvement but probably by
microvascular inner ear dysfunction.
In these cases cochlear implantation offers improvement in communication skills, including the possibility of open-set speech
communication.
25
27
MUSICAL INTERVALS DISCRIMINATION IN IMPLANTED PATIENTS
Tani A*/**, Patelli I*, Danesi G***
* Centro di Audiofonologia – Ospedali Riuniti di Bergamo
** Studio di Acustica Psicofisiologica - Bergamo
*** USSC ORL e Microchirurgia Base Cranica – Ospedali Riuniti di Bergamo
Listening to the music is a particular task involving several structures, both in peripherial and central auditory system.
Hearing impaired patients affected by acquired loss and “acoustically experienced” bear evidence of difficulties, often weighty, when asked
to recognize musical sequencies like simple melodies; in addition, listening to complex sequencies (orchestral clips with or without use of
percussive instruments) can give rise to annoyance, maily due to the acoustic muddle perceived through hearing instruments (hearing aids
and cochlear implants).
On the other side, rhythm is often well perceived, mainly with cochlear implants (our first implanted patients, wearing a mono-channel
3M-House device, could easily dance!!).
In effects hearing instruments (aids/implants) are designed for a very specific task: restore a verbal discriminative function!
If we take a look at the physical peculiarities of speech, we can easily note that there is a great difference in comparison with the physical
peculiarities of music, commonly out of range for a clinical hearing instrument (aid/implant).
In these last years some manufacturers have shown a growing interest in music discrimination, and some modern hearing aids – evaluated
in a collateral investigation – exibit extraordinary acoustical features.
The present study aims to evaluate the ability of implanted patients in a particular task: discriminate between some of the simplest units of
melody, that’s to say the musical intervals.
The patients are wearers of an Advanced Bionics AURIA device, and the test is based on discrimination of intervals (0.5 – 1.0 – 1.5 – 2.0
tones) respect to the “unisono” (0 tones interval) in 3 frequency bands (low-mid-high).
Results, matched with those obtained in the same patients fitted with the new Advanced Bionics HARMONY device (sporting 120 virtual
channels) with no training, show that there is a good correlation between the number of stimulating channels and the discrimination rate.
26
CLINICAL CASE: COCHLEAR IMPLANT IN A PATIENT WITH FAMILIAL TRANSTHYRETIN
AMYLOIDOSIS AND SUDDEN HEARING LOSS WITHOUT VIII NEUROPATHY
Negri M, Guarnaccia MC, Benincasa P, Galli S
Unità Operativa Complessa ORL - Azienda USL Modena-Carpi
Objective
We report a clinical case of a patient with familial transthyretin amyloidosis and sudden hearing loss without VIII neuropathy.
The full spectrum of clinical manifestation of the pathology is still not known. The most common symptoms are slowly progressive sensorimotor
peripheral neuropathy and systemic disease as deposits in cerebral blood vessels, cerebral hemorrhage, liver disorder.
Acoustic root involvement has also been noted and generally it depends on abnormal deposits in organs and/or tissues.
Method
The study was carried out in a female of 48 year-old with diagnosis of amyloidosis who had a sudden bilateral hearing loss:in left side in
2005 and after 1 year in the other side. The patient was given a powered hearing aid for 2 months, and she was submitted to audiological,
neuropsychological and neuroradiological evaluation before surgery. Auditory threshold (aided and unaided), perceptive skills (in audiovisual
and only sound mode), ABR and electrocochleography, were studied preoperatively, and showed bilateral inner ear damage. Computer
Tomography demonstrated bilateral normal cochlea, MRI didn’t show temporal cortical degeneration. Because of the very lower speech
perception results with traditional hearing aids she underwent cochlear implantation in 2007.
Preoperative data was compared with the data obtained during the follow-up after the activation of the device.
Results
No postoperative complications were observed after surgery. At 12 months after cochlear implantation the results showed a very high
44
HABILITATION ISSUES FOR COCHLEAR IMPLANTS PEDIATRIC RECIPIENTS FROM BILINGUAL
FAMILIES
Nassif N Barezzani MG, Botta N, Zanetti D
Otorhinolaryngology Dept. University of Brescia Spedali Civili – Brescia,
Italy
Objectives
To evaluate the results of cochlear implantation (CI) in deaf.born children from bilingual families who received their primary habilitative
training in the Italian language.
Methods
Among 108 CI procedures performed since 2002, eleven out of 51 children were from foreign countries, where the diagnosis had been already
established: 4 Albania, 2 Arab states, 1 Bangladesh, 2 Pakistan, 2 Romania,. All underwent a CT scan of the TB and an MRI of the brain,
CX26-30 and mithocondrial DNA search, a Neurological examination with cognitive tests. The speech perception and production abilities were
checked with the TAU, MAIS, MUSS, LiP-R and Mac Arthur, delivered in Italian with the aid of an interpreter specifically trained to deal with
deaf individuals. Parents were also involved in developing pictorial and text material in their mother tongue with Italian translation. All tests
were repeated every 6 months after activation.
Results
In all bilingual children the speech perception scores were significantly lower than those of their age-matched Italian peers. Speech production
parameters started to improve later than average (mean 18 months), never reaching the level of the Italian implantees. In contrast, the
cognitive functions reached the average level of the CI pediatric population. Speech perception and production were positively influenced by
the degree of knowledge of the Italian language by parents, by their degree of alphabetization and their level of social integration. Conversely,
excessive parental expectations adversely affected them. Children fully immersed in an Italian environment outside their family showed better
speech comprehension than those placed in mother tongue communities. The worst results were observed in children returning to their
homelands for long periods during the habilitation period (e.g. summer vacations).
Conclusions
Growing in a bilingual environment is not a controindication to a CI, provided that at least one of the parents has good knowledge of the
acquired (second) language, that the family is well integrated in the society and willing to grow their child in the adopting country. Longer
habilitation periods are needed.
28
ABNORMALITIES OF WHITE MATTER AND OUTCOMES OF COCHLEAR IMPLANTATION
Zanetti D*, Nassif N*, Barezzani MG*, Giordano L°, Pinelli L^
*Otorhinolaryngology Dept., °Paediatric Neuropsychiatry, ^Neuroradiology Dept. - University of Brescia, Spedali
Civili – Brescia, Italy
Objectives
Aim of this study was to assess the results of cochlear implantation (CI) in deaf children with neuroradiological findings of leucoenkephalopathies
(LEP), which have been mainly correlated with prenatal CMV infection (Barbi et al 2003).
Methods
Among 108 consecutive CI procedures performed at our ORL Dept. during the last 5 years, 51 were children (mean age = 7,45 years). Seven
of them showed white matter abnormalities at MRI, typically hyperintense in T2, hypointense in T1 and without enhancement by contrast
medium.
The selection protocol included an assessment by the Pediatric Neuro-Psychiatrist, based on family psychological interview, cognitive
evaluation by Leiter scale and the Griffith’s test, and behavioural tests.
In case of abnormal cerebral MR findings, the Guthrie cards, archived for all newborns at the Regional Institute for Genetic and Metabolic
diseases, were retrieved for CMV-DNA search by polymerase chain reaction.
45
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
Results
In only 2 out of 6 children the DNA-CMV search was positive. None of them suffered neurological disturbances, nor metabolic anomalies. One
of the two showed also positivity for Cx 26.
The outcomes of the age-adjusted speech perception tests (TAU, MAIS, MUSS, LiP) in the 7 children were comparable to those of their agematched peers with no MR abnormalities. Disyllabic word recognition and comprehension scored between 70 and 100% at 24 months followup. All children except one greatly improved their cognitive performances. For the only child who did not achieve the expected improvement,
a differential diagnosis between CAPD and autistic tract is pending, and further diqagnostic assessment is scheduled.
Conclusions
The finding of white matter abnormalities at MRI is not a contraindication to a CI. These children can be expected to achieve similar results
to their implanted peers without such anomalies. A long term neurological follow-up with possible neuro-imaging is recommended for the
CMV-negative children.
is a useful way to establish a selection criterion for FM-user candidates and verify the benefits of the FM system. Recognition of words in
noisy and quiet conditions allows us to establish a highly-accurate selection criterion and identify those user of CIs and Hearing Aids that
can benefit from a FM system.
29
Objective
Numerous studies have shown that after bilateral cochlear implantation patient receives additional channels of information what makes
sound localization possible and improves speech understanding in noise conditions. The aim of this work is to show the results of bilaterally
implanted patients in the Institute of Physiology and Pathology of Hearing.
Methods
Patients aged from 9 months to 35 years implanted in simultaneous or sequential procedure with the same type of implant in both ears
are included in the study. Speech processor fitting was based on psychophysical and psychoacoustic methods adapted to patient’s age.
For benefits assessment AAST (Adaptive Auditory Speech Test) and open set monosyllabic words test was used in unilateral and bilateral
conditions.
Results
All patients regularly use both speech processors. We observe better attention to acoustic signals in children. We also note improvement in
speech understanding in complex sound environment.
Conclusions
Preliminary results of the group are very promising. It is necessary to continue a longitudinal study on this group of patients.
EAR AND BRAIN NEURORADIOLOGICAL FINDINGS AND OUTCOMES IN COCHLEAR-IMPLANT
CANDIDATE CHILDREN
Busi M, Trevisi P, Destro E, Calzolari* F, Martini A
Università degli Studi di Ferrara – Unità Operativa di Audiologia
* Neuroradiologia Ferrara
Purpose. Cochlear implants are electronic devices used in children with profound bilateral hearing impairment. Neuroradiological findings
should evaluate contraindications for cochlear implantation and factors that can modify surgical strategies or increase surgical risk. Aim of
this study was to discuss the results of cochlear implantation in deaf children whit ear and brain abnormalities and also the outcomes in
children whit added disabilities.
Materials and methods. Ear and brain neuroradiological findings of 271 cochlear-implanted children were retrospectively reviewed. In all cases
CT of the petrous bone and MRI of the inner ear and the posterior fossa and/or the whole brain were performed.
Results. Out of 271 patients, only 169 showed completely normal findings or unimportant abnormalities.
Among the ear abnormalities, we found 16 cases of bilateral labyrinthine ossification, 35 of inner ear malformations, 13 of large vestibular
aqueduct.
Concerning brain anomalies, 33 patients showed “dysmyelinating” areas or delayed myelination. Sixteen had post-meningo-encephalitic
lesions. Moreover, we found this anomalies: cortical dysplasia, callosal hypoplasia, cerebellar vermis hypoplasia, cerebellar hemispheres
hypoplasia and cerebellar tonsils ectopia. Finally we found 6 arachnoid cysts of the posterior fossa.
Conclusions. Although the frequency of cochlear implantation has increased remarkably over the paste decade, pre-operative imaging
protocols are yet not completely shared.
CT and MRI are complementary for an exhaustive evaluation of cochlear implant candidates. In pre-operative protocol we suggest to study not
only the ear and petrous mastoid structures, but also the brain. In fact whole brain MRI can demonstrate anomalies also in children without
neurological signs or symptoms and can explain the wide variation of performance across individual cochlear implant users.
30
ASSESSMENT OF SPEECH INTELLIGIBILITY IN NOISE IN COCHLEAR IMPLANT USERS USING FM
SYSTEMS
Barajas de Prat JJ (1), Zenker Castro F (2), Mora Espino R (3) and Fernandez Belda R (2)
(1) Universidad de La Laguna
(2) Clinica Barajas
(3) Fundación Canaria Dr. Barajas para la Investigacion y Prevencion de la Sordera
The use of Frequency-Modulated (FM) Systems with Cochlear Implants (CI) has steadily increased as the FM technology has become more
integrated with the CI speech processor. Although noise-reduction technology is incorporated into CI, this technology cannot compensate for
the reduction in the speech signal that occurs as the distance from the speaker is increased. The use of FM systems is most often addressed
in educational environments in which the noise level may interfere with learning new information. In the present study participated 16 subjects
with ages ranging from 5 to 14 years old, 7 of them with unilateral CI and 9 with Hearing-Aids. Speech intelligibility in noise was examined
in four acoustic conditions: with and without the FM system and with and without background noise. Testing was carried out in the hearingimpaired children’s classrooms. Significantly better speech recognition scores were obtained with the FM system than without FM system and
in quiet than in noisy condition. Frequently used words were more easily recognized than infrequently used words. A significant interaction
were found between the use of the FM system, the lexical frequency of the words and the presence of background noise. The main benefit
of the FM system is obtained for infrequently used words in noisy background conditions. The assessment of Speech Intelligibility in Noise
46
31
ASSESSMENT OF BENEFIT AFTER BILATERAL COCHLEAR IMPLANTATION
Obrycka A., Lorens A., Piotrowska A., Zgoda M., Skarżyński H.
Institute of Physiology and Pathology of Hearing, International Center of Hearing and Speech, Auditory Implants,
Perception and Rehabilitation Department, Kajetany near Wasaw, Poland
32
Hearing and Quality of Life of NF2 Patients with ABI
Konradsson KS*, Nyberg G**, Svedberg A*, Olsson G-B* Kinnefors A* and Rask-Andersen H*
Departments of Otorhinolaryngology* and Neurosurgery**, Uppsala University Hospital, Sweden
In Uppsala, Sweden 18 patients (7 females and 11 males), deafened by neurofibromatosis type 2 (NF2), have received an auditory brainstem
implant (ABI), since 1993. The patients’ age at the time of operation was 12 to 65 years (median age 30 years). All are from the Nordic
countries: Sweden, Norway and Iceland.
This study shows that only four of the eighteen implanted patients never use the ABI. All the nine active users feel that the ABI enhances their
quality of life and helps them to detect environmental sounds and to control their own voice. The six patients who always use the ABI have
achieved functional understanding of spoken language, some supplemented by lip reading. Few patients are able to communicate without
visual cues. In one case the ABI enables the patient to converse by the telephone.
33
TRANSMEATAL LOW LEVEL LASER THERAPY FOR CHRONIC TINNITUS WITH COCHLEAR
DYSFUNCTION
Bellini C, Teggi R, Piccioni L O, Palonta F, Bussi M
Department of Otorhinolaryngology, Vita-Salute University, San Raffaele Hospital, Milan, Italy
Objectives
Low Level Laser Therapy (LLLT) has recently been proposed as a therapeutic procedure for tinnitus. Its effectiveness is still under debate.
Methods
In order to establish the efficacy of LLLT, we performed a prospective double-blind study on 54 outpatients referring chronic tinnitus and
presenting sensorineural hearing loss from various causes. They were randomly divided into two groups, the first performing active laser
therapy (Group L), the second using a dummy device (Group C). LLLT was performed by patients at home with a 650 nm wavelength, 5 mW
soft laser for 20 minutes a day over a period of 3 months.
Results
THI has been considered the main outcome; no statistical difference has been detected between the 2 groups for THI total score (p=0.97),
functional (p=0.89), emotional (p=0.89) and catastrophic (p=089) subscales. Moreover, a VAS scale for self perceived loudness of tinnitus
showed no difference between groups (p=0.69). About psychoacoustic parameters, Minimum Masking Level showed no difference (p=0.42),
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The XIV International Symposium
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September 18-21, 2008
Sala S. Francesco, Ferrara
while Loudness expressed in Sensation Level exhibits lower values in Group L (p=0.0127). Group L subjects also presented a decreased rate
of hyperacusis (p= 0.02). No changes have been detected in audiometric threshold in both groups.
Conclusions
Soft laser therapy demonstrated efficacy in changing psychoacoustic parameters which often result in the incomplete therapeutic success
of Tinnitus Retraining Therapy (TRT). In our opinion, it may be worth trying soft laser therapy in association with “sound therapy” in those
patients presenting hyperacusis.
34
NON PULSATILE OBJECTIVE TINNITUS IN CHILDREN
Spyridakou C , Rajput K, Veness J, Sirimanna T
Department of Audiological medicine, Great Ormond Street Hospital for children - Great Ormond Street - London
WC1N 3JH, UK
Introduction
Objective tinnitus is the tinnitus that can be heard and/or recorded by an observer and is extremely rare(1). It can be categorized into pulsatile
and non pulsatile tinnitus. The prevalence of tinnitus in children has been reported to be from 3%-36%.(2) The objective non pulsatile tinnitus
is even rarer and review of the literature shows that only 11 paediatric cases have been reported in the last decade ( 1998-2008).
Objective
We present the cases of 4 children with objective non pulsatile tinnitus and we briefly review the medical literature with regards to the clinical
presentation, diagnosis, audiological findings and management of this rare symptom in children.
Material and Methods
4 children (age 10-11 years old) were referred to the department of Audiological Medicine with bilateral (3 children) and unilateral objective
tinnitus (1 child). All the children underwent full otolaryngological and neurological clinical examination, audiological investigations and
radiological investigations.
Results
All the children present with a longstanding history of tinnitus. 2 of the children were initially referred to the Auditory Processing Disorder
Clinic due to learning difficulties. In 2 of the children the clinical examination revealed palatal myoclonus which was the cause of their
objective tinnitus.In the other 2 the clinical examination was normal. All the children had normal hearing and the neurological and radiological
investigations did not reveal any pathologies related to their tinnitus. What is very interesting is that in addition to their tinnitus the children
present with learning difficulties (Auditory Processing Disorder, Dyslexia).
Conclusion
The objective tinnitus is rare and even rarer in children.The clinical presentation, diagnosis, investigations and management are discussed
along with the interesting finding of the Auditory processing deficit which may be related to the persistent longstanding tinnitus.
References
1.Golstein B,Shulman A.Tinnitus classification: medical audiological assessment . J.Laryngol. Otol.1981;4(suppl):33-8
2.Fritsch,M.H ,Wynne M.K,Matt B.H,et al.Objective tinnitus in children.Otol. & Neurotol. 22(5):644-649,Sept.2001
35
The influence of the hearing aid microphones positioning on the localization
cues. ReSound BE: a case study
*Picinali L, **Mancuso A, **Vercellesi G, ***Pulga M
*LIMSI-CNRS, Orsay, France, **DICO-LIM, Unimi, Milano, Italy
***GNReSound Italia, Montegrotto Terme, Italy
The auditory scene analysis (from now on, ASA) is the process by which the human auditory system organizes the sounds. Processes involved
in the ASA are often linked to the analysis of the four standard parameters of the sound signal: amplitude, frequency, duration and spectral
content, as well as to mnemonic elements. Nevertheless, other parameters play a really important role in ASA mechanisms: these parameters,
known as the localization cues, can be grouped into interaural differences and direction dependent filtering.
An example on how the localization cues can be used for the ASA, and in particular for the integration and the segregation of different sounds
in a complex auditory scene, can be done citing one of the most known binaural effect: the cocktail party effect. Speech can therefore been
segregated from noise, with a consequent increase of the speech intelligibility, thanks to the ASA based on the localization cues.
Using a standard BTE hearing aid, the device is placed behind the pinna of the prosthesized subject, therefore the microphones that send
the signals to the processing unit are located outside the outer ear system.This causes, of course, alterations in terms of the filtering effects
brought by the pinna to the signals that reach the outer ear, and is then translated in several problems during the sound sources localization
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The XIV International Symposium
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September 18-21, 2008
Sala S. Francesco, Ferrara
process.
If sound sources cannot be properly localized, then some of the ASA mechanisms will not work properly, with a consequent reduction in terms
of speech intelligibility in various real-life situations.
Recent studies with bilateral BTE hearing aids have shown that common adaptive directional microphone systems tend to distort localization
cues, leading to inappropriate and reduced spatial awareness for bilateral hearing aids users.
In the ReSound BE Hearing Aid, the microphone is located inside the pinna, just above the entrance of the ear canal: the localization cues,
and most of all the filtering effect brought by the pinna itself, are therefore preserved.
The aim of this study is to compare different hearing aids (BTE, CIC and BE), trying to establish how the positioning of the microphones
can influence the localization cues: HRTF (Head Related Transfer Functions) are measured from a Dummy Head, then from different devices
placed on the ears of the Dummy Head itself. The comparisons between these are then analyzed and discussed.
36
SENSORINEURAL HEARING LOSS AND SEVERE NEONATAL RESPIRATORY FAILURE
Morando C1, Gamba PG2, Midrio P2, Sgrò A2, Filippone M3, Magnano San Lio AG4, Orzan E4
1 Department of Pediatrics, University of Padova, Italy
2 Pediatric Surgery Department, University of Padova, Italy
3 Neonatal Intensive Care Unit, Department of Pediatrics, University of Padova, Italy
4 Pediatric Audiology Unit, University Hospital of Padova, Italy
Background
Congenital diaphragmatic hernia (CDH) survivors have increased morbidity, including sensorineural hearing loss (SNHL). The relationship
between CDH and SNHL has not been explained. Several risk factors may predispose the newborn admitted to the Neonatal Intensive Care
Unit (NICU) to the development of SNHL. Many studies analyzing patients treated for severe neonatal respiratory failure, have described an
higher incidence of SNHL in CDH patients than in their non CDH peers.
Objectives
Report the prevalence and the severity of SNHL, the presence of conductive hearing loss; the exposure to audiologic risk factors in CDH
survivors. Compare the incidence of SNHL between two populations with severe neonatal respiratory failure, exposed to NICU risk factors,
one affected by CDH and the other not.
Methods
Retrospective chart review to indentify audiologic risk factors (mechanical ventilation, high frequency oscillation, gentamycin and neuromuscular
blocking agents) and audiometric evaluation (speech audiometry, otoacoustic emission, immitance measures) of CDH survivors born between
1989 and 2007 and of children affected by primary pulmonary hypertension of neonates (PPHN) born between 2001 and 2007.
Results
Three of the 40 children affected by CDH have permanent SNHL for high frequencies of mild, moderate and profound severity respectively.
The child with profound SNHL is affected by Turner syndrome. Four of CDH survivors with normal cochlear function presented conductive
hearing loss at the time of evaluation. None of the 11 children affected by PPHN had SNHL.
Conclusions
In our study CDH survivors have lower prevalence of SNHL than previously reported. Cases identified are so few that we can neither find a
strong correlation between the exposure to risk factors and the development of SNHL nor affirm if it may be due to NICU risk factors or be
considered as a part of the “field defect” of CDH syndrome. Case-control study with larger populations is needed.
37
THE UNIVERSAL NEWBORN HEARING SCREENING PROGRAM AT FERRARA, ITALY.
FOCUS ON SCREENING COSTS AND SOFTWARE SOLUTIONS
Hatzopoulos S, Ciorba A, Petruccelli J*, Camurri L, Rossi M, Malagutti M and Martini A
Audiology Dept., University Hospital of Ferrara, ITALY
* Department of Mathematical Sciences, Worcester Polytechnic Institute, Worcester, MA 01609-2280
The present communication reports the advances of the UNHS-EHDI program at Ferrara in terms of procedures, screening devices and
software solutions for the administration of data. The latter refer to recordings from 6760 full-term newborns and 1150 NICU infants, tested
at the University Hospital of Ferrara, from January 2000 to December 2006. Numerous standard and automated OAE and ABR devices have
been employed in the development of the program and the experience accumulated will be described. Apart the standardized yield-statistics
on PASS and REFER rates additional information will be presented addressing three questions pertinent to hearing screening: (i) the costestimate of a UNHS program based on European economical and administration premises ; (ii) the development of a database-structure
for the evaluation of the UNHS / NHS performance and the individual patient tracking; (iii) the use of alternative technologies such as
50
CochleaScan and Steady State Auditory responses in the hearing intervention phase of the program.
38
Genetic testing —the predicting role in genetic counseling and instruction for
deaf couples
Bing H, Pu D, Guo-jian W, Dong-yang K, Xin Z, Yong-yi Y, Dong-yi H
Department of Otolaryngology-Head and Neck Surgery, Chinese PLA General Hospital, Beijing 100853, China
Background: The marriage mode of “deaf—deaf” is very common among Chinese deaf population. Most of them are eager to receive effective
genetic counseling to avoid having a deaf offspring. Recent advances within molecular genetics to identify deaf genes make it possible for
genetic-counseling services to offer genetic testing to deaf couples.
Objective: To analyze the molecular pathogenesis of deaf couples by genetic testing and provide genetic counseling based upon results of
genetic testing.
Methods: 19 deaf couples joined in this study. Genomic and mitochondrial DNA of each subject were extracted from whole blood. Genetic
testing of GJB2, SLC26A4 and mtDNA A1555G mutation were offered to each individuals.
Results: The recurrence risk of 3 deaf couples is 100%, as each pair were caused by same deaf gene(GJB2 or SLC26A4). In 2 deaf couples,
the husband from one couple carried homozygous GJB2 mutations and a single SLC26A4 mutation while his wife diagnosed with enlarged
vestibular aqueduct syndrome carried a single SLC26A4 mutation; and another couple was husband with mtDNA A1555G mutation while his
wife and first child were diagnosed with Warrdenburg’s symdrome. Therefore, offspring of the 2 deaf couples are confronting 50% recurrence
risk. In another 2 deaf couples, the wives were mtDNA A1555G mutation carriers but husbands with SLC26A4 compound mutations, the
instruction is that their offspring must strictly avoid the administration of amino glycoside antibiotics. Among the rest of 12 deaf couples,
1 couple were with different deaf gene mutations; 6 couples were ones with deaf gene mutations but the other ones without any positive
genetic testing results; and 5 couple all did not have positive results. The recurrence risk of these 12 couples’offspring is near to average
risk of whole nation.
Conclusion: Genetic testing plays an important predicting role in genetic counseling and instruction for deaf couples.
39
FROM NHS TO EARLY DIAGNOSIS: OAE, ABR AND ASSR EXPERIENCES IN MEXICO
Berruecos P, and Martínez R
Audiology and Phoniatrics Department, General Hospital of México
Mexico City, MEXICO
The present study was carried out to know the usefulness of a combinated DPOAE, ABR and ASSR protocol in the context of a NHS and
diagnosis program. It was performed in the Audiology and Phoniatrics Department of the General Hospital of Mexico (GHM) in coordination
with the Neonatology area of the Gynecology and Obstetrics Department. The GHM is the largest one in the country and receives the so-called
“open-population”, that is, people not included in the social security institutions benefits. With a mean of 15-20 births daily, 1 or 2 of those
newborns have an average of at least one high risk factor and are referred to the intermediate or to the NICU.
This protocol was also performed to specifically define, simultaneously, the ASSR role in the diagnostic process of deafness or profound HL in
neonates. DPOAE were performed in every normal newborn with a Pass/Fail criteria of 3dB S/N ratio. Those who fail underwent click ABR
and ASSR, as well as every newborn with high risk factors. The babies with abnormality in one of the tests received an appointment in two
months to complete the diagnostic process. Our testing procedure and outcome criteria was as follows: 80 and 30 dB clicks were used in
the ABR test. Abnormal results in latencies at 80 dB or a latency greater than 9.5 msec. with an intensity of 30 dB was considered abnormal.
Pure tones of 0.5, 1, 2 and 4 kHz were modulated in amplitude and frequency for ASSR tests. Results were considered abnormal when 2
or more frequencies were above 30 dB. In the September 2005-March 2008 period, we studied 13,494 neonates with DPOAEs. 503 normal
or HR babies from them, were referred for additional ABR and ASSR studies. Since testing newborns is not an easy task and many records
did not fill into the protocol criteria, 187 records were not included in the analysis. From the remaining 316 full-studied babies considered
for this study, 226 were normal neonates referred because they failed the first DPOAE examination, and 90 had at least one high risk factor
for deafness. Gestational age ranged from 30 to 42 weeks, 75% had less than 18 days of age, around 1/3, a weight under 2000 g. and more
than 50% had more than 2 RF. From the studied neonates, 9 % born without specific RF for HL, but they stayed in the NICU due to other
perinatal health problems. We diagnosed 9 babies with unilateral hearing loss, and 24 with bilateral hearing loss for a global rate of 2.44 /
1,000 or a rate of 1.77 / 1,000, considering only the bilateral profound HL cases.
As a consequence of our outcomes, the advantages and disadvantages of ASSR included in a comprehensive UNHS program are discussed.
In spite of the fact that our work have been discussed with the Canadian, American and Australian experts in this field, we have doubts and
so, we are still looking for answers. As a consequence, we present several suggestions, some of them directed to clinicians and other to
manufacturers of equipments, in order to have better results with the NHS programs, that must consider as its compulsory consequence, the
early diagnosis and intervention protocols.
51
Posters
September 18-21, 2008
Sala S. Francesco, Ferrara
Saturday 20th September
➛ 14.30 - 16.00 POSTERS DISCUSSION
Moderators: S. Berrettini, N. Quaranta
P1
PREDICTORS OF HEARING SCREENING FAILURE FOR TARGETED NEWBORN HEARING
SCREENING IN A DEVELOPING COUNTRY
Olusanya BO1, Luxon LM2
1
Maternal and Child Health Unit, College of Medicine, University of Lagos, Lagos, Nigeria; 2Academic Unit of
Audiological Medicine, Institute of Child Health, University College, London, UK
Objective
To identify predictors of hearing screening failure that can be used for targeted screening in developing countries where resources for
universal newborn hearing screening (UNHS) are limited.
Methods
A two-stage UNHS protocol consisting of transient-evoked otoacoustic emissions (TEOAE) and automated auditory brainstem response
(AABR) was implemented for newborns in a tertiary maternity hospital in Lagos, Nigeria over a period of 30 months from May 2005
following which antepartum, intrapartum and postpartum factors significantly associated (p<0.05) with screening failure were explored with
multivariable stepwise logistic regression to derive the adjusted odds ratio (OR) at 95% confidence intervals (CI).
Results
Of the total live births of 5,473 over the study period, 4,718 (86.2%) newborns were screened before hospital discharge out of which 202
(4.3%) did not complete the second stage-screening and were excluded from this analysis. Of the 4,516 who completed the protocol, 121
(2.7%) failed. Low birth-weight (<1,500g), mode of delivery, low Apgar scores at five minutes, admission for special care, ototoxic medication
and hyperbilirubinaemia requiring phototherapy were significantly associated with screen failure in the univariate analyses. However, only
admission into special care (OR: 2.34; CI: 1.46 - 3.78) as well emergency caesarean-section (OR: 3.67; CI: 1.13 - 11.93), spontaneous vertex
delivery (OR: 4.16; CI: 1.30 - 13.31) and assisted vertex (forceps and vacuum)/breech delivery (OR: 6.40; CI: 1.49 - 27.45) as compared to
elective caesarean section emerged as independent risk factors.
Conclusions
Admission into special care newborn unit and mode of delivery are predictive of hearing screening failure and may serve as risk factors for
targeted newborn hearing screening in poorly-resourced settings. In addition, cultural norms which discourage elective caesarean section in
this population portend significant risks of sensorineural hearing loss for surviving babies.
P2
TEOAE+AABR: reduction of refers in neonatal hearing screening in Liguria Region
(“Progetto STERN-LIGURIA”)
Zavattoni V(*)
(*) Psicologo-psicoterapeuta; Coordinatore del <<Centro Italiano di Studi e Ricerche sulla Comunicazione
Umana-STERN-LIGURIA>> A.I.R.H.-ONLUS (#)- Genova, Italy
(#)col sostegno della Compagnia di San Paolo-Torino, Italy
Progetto STERN-LIGURIA - U.O. NEO
-Prof. Sandro Trasino, Dott.ssa Alessandra Allodi, Neonatologia, Ospedale San Martino, Genova
-Dott. Stefano Macciò, Dott.ssa Lorella Mazzarello, Dott.ssa Rossella Franchini, Neonatologia,
Ospedale Villa
Scassi, Genova-Sampierdarena
-Dott. Enrico Giunta, Dott.ssa Renata Renda, Neonatologia, Ospedale Evangelico Internazionale,
Genova
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September 18-21, 2008
Sala S. Francesco, Ferrara
-Dott. Piero Zucchinetti, Dott.ssa Silvana Maccarrone, Neonatologia, Ospedale San Carlo, Genova-Voltri
-Dott.ssa Carla Beluschi, Dott. Alberto Morchio, Neonatologia, Ospedale di Lavagna, GE
-Prof. Stefano Parmigiani, Dott. Gianfranco Zaccagnini, Neonatologia, Ospedale San Bartolomeo Sarzana, SP
-Prof. Stefano Parmigiani, Dott.ssa Maria Franca Corona,Dr. Alessandro Gerali, Neonatologia, Ospedale Sant’Andrea,
La Spezia
-Dott.ssa Carla Navone, Dott.ssa Maria Cristina Poggi, Neonatologia, Ospedale Santa Corona, Pietra Ligure, SV
Progetto STERN-LIGURIA - U.O. ORL
-Prof. Angelo Salami, Dott. Renzo Mora, Università di Genova-Clinica Otorinolaringologica, Genova
-Prof. Stefano Nosengo, Dott. Alfonso Sorrentino, Chirurgia Cervicofacciale ed Otorinolaringoiatria Ospedale Santa
Corona, Pietra Ligure, SV
-Dott. Eugenio De Martini, Dott. Mattia De Ciccio, Otorinolaringoiatria, Ospedale Sant’Andrea, La Spezia
In 2002 AIRH-ONLUS started a program of “Screening, Treatment and Rehabilitation of Infants suffering from Hearing Disease” (Progetto
STERN-LIGURIA). The “Project STERN-LIGURIA” require two controls for each case. Each “ Neonatology Centre” is equipped with an
EchoScreen recording the TEOAE within the first 48-72 hours of life. Infants REFER make control between the 1st and 3rd week of life.
Confirmed REFER infants running by the 3rd months of life control of ABR to the ORL centre, and in case of hearing disease continuing
investigations. In case of ABR doubt or pathological doubt, a 2nd ABR exam has been run within the 7th month of life and infant starts
psychomotor checks.
One of the most important target of “Project STERN-LIGURIA” is control and support the anxiety induced by waitingthe results of audiological
screening using Psychotherapeutic Counselling. Besides the utilization of valutation scales for phonetic and linguistic learning from parents
is problematic and anxious for inadequacy of sur statistical data.
If anomaly of pattern ABR is confirmed, it proceeds with further ORL exams.The target is diagnosis and habilitation by the 6th month of
correct age.
Since 2007, in order to reduce the increasingly false positives, AIRH gave to 3 O.U. (total expected number of births per year 4000), a new
equipment that can make TEOE; DPOAE and AABR. The percentage of REFERS to the TEOAE, according to international literature, should be
<4%. The average percentage of REFERS TEOAE in STERN Project is 1,29%. The average value “no risk/risk hypacusics” is 0,65%.
The target of TEOAE+AABR sequence is to increasingly lower REFER percentage to reduce false positives.
From 2002 so far were screened more than 57,000 babies. Between 2002 and 2007 percentage of REFER to TEOAE (second test within the
15th day) was an average of 1,29%. The average of REFER to TEOAE+AABR sequence is 0,31%, confirming the new procedure audiologist
neonatal screening.
P3
Early detection of hearing impairment in newborns. Italian Universal Newborn
Hearing Screening Program
Bubbico L
Department of Biomedical Science Italian Institute of Social Medicine - Via P.S. Mancini 28 - 00196 Rome
Objective: This study analyses results from the 2006 Italian UNHS program Survey, to determine the implementation and coverage of
universal newborn hearing screening programme UNHS in Italy.
Methods: Data were collected during the period January-March, 2007 through a Screening Survey Questionnaire SSQ, that was sent to all
the public Birthing Hospitals/Centers active in Italy in 2006 (607) .
Results: Results revealed that UNHS in Italy coverage had a steep increase from 29.3% in 2003 (156,048 newborns screened) to 48.4% in
2006 (262,103 screened). UNHS screening penetration is different from one Region to the other. In 2006, UNHS was performed by 92 of 118
(77.9%) Birthing Hospital/Centers of the North-West area, by 45 of 84 (53.5%) hospitals of the North-East area, 32 of 110 (29.0%) hospitals
of the Center area, 71 of 190 (37.3%) hospitals of the Southern area, and 12 of 105 (11.4%) hospitals in the Island area.
The majority of UNHS programs were implemented in the two most economically developed areas, i.e., in the North-West area, (79.5%)
(108,200 out of 136.109 births), and in the North East area 57.2% (52,727 of 92,133 births), while still remain a limited diffusion in some
areas, typically in the Islands. 11.3 % (7,158 of 63,460 births).
Conclusion: Our results suggest a quick diffusion of newborn hearing screening programs in Italy and indicate that three conditions seem
to play a crucial role in the implementation of UNHS programs: the size (>800 births/year) and location (metropolitan urban areas ) of the
hospital, and the presence of an audiologist in the UNHS coordinating team.
54
P4
The newborn with othological malformations: a complex diagnostic approach
Garani MC, Caldarelli V, Trevisi P, Ballardini E, Busi M, Garani G
TIN e Neonatologia, Audiologia - Azienda Ospedaliera Universitaria S.Anna, Ferrara - Italy
The management of a newborn with congenital ear malformations is difficult for complexity of diagnostic and therapeutic problems and for
parental anxiety. The external ear anomalies (EEA) could be isolated or in association with others congenital malformations. Data of the IMER
register (Indagine Malformazioni Congenite Emilia Romagna) underlines, according with literature, that prevalence of EEA was found 6.15 x
10,000 newborn (isolated cases 3.45 x 10,000).
Our diagnostic protocol for newborn with EEA includes clinical, othological, audiological, genetic evaluations and imaging. In our opinion,
planning a complete and individualized diagnostic program according with different clinical conditions is very important.
For this reason the first step for neonatologist is to recognize any association with potentially life-threatening malformations which need
urgent medical or surgical management (bilateral choanal atresia, primary feature suspicious of CHARGE syndrome), then should focus
attention on other eventual organ anomalies to achieve diagnosis.
When clinical conditions of newborn are stable, the next step for physician is evaluating hypoacusia with audiological exams (i.e. A-ABR and
OEA)in a few days after birth. If bilateral hypoacusia is observed, neuroimaging must be performed as soon as possible to obtain a specific
diagnosis of anatomic malformations and also to choose the best therapeutical strategy.
Fortunately, we found more frequently mild or monolateral hypoacusia in which a different timing of management is possible: delaying
neuroimaging by allowing growth of inner and external ear to improve diagnostic accuracy.
At least, appropriate genetic tests can be performed as early as possible to obtain a specific diagnosis in complex cases. The latter situations
require anticipation of management including treatment because good hearing is essential for a child’s overall development, in order to avoid
severe language and social delays. Early intervention services greatly improve the child’s prognosis of achieving normal social, emotional
and language development.
P5
Sensorineural hearing loss (SNHL) in a compound heterozygous for 35delG and
V153I
Chiarella G, Passafaro G*, Cassandro C, Mangone G*, Misasi S*, Perrotti N*, Cassandro E
“Magna Graecia” University, Catanzaro, Dept of Experimental and Clinical Medicine “G. Salvatore”, Audiology and
Phoniatrics Unit, Regional Center for Cochlear Implants and ENT Diseases.
*Chair of General Pathology
About 16% of adults have hearing impairment of 25 dB or greater. The known causes of sensorineural hearing loss (SNHL) include genetic
(syndromic and nonsyndromic), viral, bacteriologic, traumatic, immunologic, and drug-related causes and other medical conditions but many
cases of SNHL still remain of unknown etiology, particularly if they are diagnosed in adult or old age. We present a report about a man (57
years old) with progressive bilateral SNHL with no audiological risk factor or other internal pathology, the family history was negative for
hearing loss. Audiometric tonal and vocal testing was completed with Auditory Brainstem Responses and imaging (magnetic resonance).
Genomic DNA was purified from a peripheral blood sample and amplified by polymerase chain reaction (PCR) in presence of primers designed
to amplify the entire coding region of the GJB2 gene (GI: 14753416). DNA fragments were partially purified and sequenced (Abi Prism 3100,
Applied Biosystems, Monza (MI), Italy) from both sides with the same primers used for the amplification.
The sequence of the GJB2 gene revealed the presence of two heterozygous mutations, the common deletion of guanine 35 (35delG) together
with the substitution of valine 153 for isoleucine (V153I).
V153I is generally considered a polymorphism not responsible for hearing loss although the exact role of the amino acid substitution is still
questionable.
The presence of a clear phenotype characterized by progressive sensoryneural hearing loss in a patient heterozygous for both 35delG and
V153I supports the idea that the substitution of valine 153 for isoleucine can be responsible of phenotype when associate with the common
recessive mutation 35delG.
55
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
P6
P8
Usher syndrome is an autosomal recessive condition characterised by sensorineural hearing loss, retinitis pigmentosa and variable vestibular
function. Three clinical forms designated type I, II and III and eleven genetic subtypes have been described, with considerable phenotypic
overlap.To understand more about this condition and establish genotypic-phenotypic correlation a national recruitment of Usher families was
undertaken in the UK.
220 individuals from 182 families were recruited. All subjects underwent a complete audiovestibular and ophthalmological protocol and
provided blood samples for genetic analysis.
Results
Detailed audiovestibular assessment was completed on 96 males and 106 females (N=202) with an age range of 3 to 68 years. Genetic
results are currently available on 115 individuals. USH2A was the largest group with 87 subjects of whom 38 had a frameshift mutation
2299delG. This mutation has been reported to produce variable vestibular function, but in our study all individuals had normal peripheral
vestibular function as demonstrated by rotation and caloric tests. Individuals with homozygous mutations tended to have slightly better gain
on rotation tests but this was not statistically significant
13 subjects were found to have mutations in MYO7A gene (USH1B); 12 of whom had absent peripheral vestibular function, One subject with
a heterozygous G1942X mutation had good vestibular function on both rotation and caloric tests. Hearing levels correlated well with the
vestibular picture with profound hearing loss in all those with USH1B genotype except the individual with retained vestibular function.
Usher type III is uncommon in our study population and only two families were found to carry mutations in that gene. A family diagnosed
clinically as Usher type II/III were subsequently found to be compound heterozygotes for mutations in the USH1C gene.
Conclusions
This study confirms the extreme heterogeneity of Usher syndrome. In common with other European studies there was a higher prevalence
of 2299delG mutation in our USH2A population. Based on currently available genetic results detailed vestibular evaluation may prove helpful
in directing molecular genetic analysis of mutations in USH2A.
Objective
In children with a congenital cleft palate the increased risk of development of conductive hearing loss is observed, which is also accompanied
by otitis media with effusion. It is also assumed that in children with a cleft palate tubal muscle function is abnormal.
Universal hearing screenings program enables to diagnose and treat children with hearing impairments, which enables undertaking early
rehabilitation. Treatment of older children who previously underwent surgical correction of palate is directed to the correction of malocclusion.
The aim of this study was to evaluate hearing in older children, including their eustachian tube function as a risk factor of the development
of hearing impairment.
Methods
The study material consisted of 38 children: 21 boys and 17 girls (mean age 10 years) with cleft lip/palate. All children had surgically repaired
cleft palate in 1st and 2nd year of life. Each patient received hearing screening test. The following procedures were performed: case history
taking, otoscopic examination and tympanometric screening and Eustachian tube function test.
Results
Abnormal otoscopic status was revealed in 46% of examined ears (76 ears);otitis media with effusion: 5 ears (6,8%). Eustachian tube
function test was in 65% cases incorrect.
Conclusions
The relative high prevalence of eustachian tube dysfunction was the risk factor of hearing disorders in children with palate/lip cleft. The test
of eustachian tube function was useful aid hearing screening for prediction hearing disorders in patient with cleft palate.
USHER SYNDROME: GENOTYPE PHENOTYPE CORRELATION
Rangesh N1, Saihan Z2, Haralambous E3, Le-Quesne P3, Cohen M4, Bitner-Glindzciz M 3, Webster A2, Luxon L1
1. Academic Unit of Audiological Medicine, UCL Institute of Child Health, London, UK
2. Moorfields Eye Hospital, Institute of Ophthalmology, London UK
3. Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London UK
4. Manchester University
P7
EVALUATION OF VESTIBULAR FUNCTION IN CHILDREN WITH OTITIS MEDIA WITH EFFUSION
ACCORDING TO HEARING LOSS
¹Pazdro-Zastawny K, ¹Pospiech L, ¹Gawron W, ¹Kochman M
¹Medical University Hospital ENT Department Wroclaw Poland
Objective: Otitis media with effusion (OME) is one of the most common clinical conditions in childhood characterized by an accumulation of
fluid in the middle ear without signs or symptoms of acute infection. OME occurs mainly as a sequela of Eustachian tube dysfunction. Fluid
accumulation may lead not only to sensorineural hearing loss and to compliance of the tympanic membrane but may also have impact on
inner ear. The purpose of this study was to investigate the condition of the vestibular system in children with OME using static posturography
and to examine whether a relation exists between balance control and hearing impairment.
Method: The study was performed on 53 children with OME, who were treated with drainage of the middle ear in the ENT department of
Wroclaw Medical University. All children underwent a complete otoneurologic examination. A detailed history case, with particular attention
to current disease, previous ENT diseases and presence of vertigo and/or disequilibrium was taken. Each subject was evaluated by physical
otolaryngological examination including microscopic examination of the eardrums. Audiologic function was performed by use of pure-tone
and impedancje audiometry.
Vestibular function was examined using sway posturography. Audiologic and vestibular assessment were performed before the drainage and
repeated one month postoperatively.
Results: Posturography revealed presence of elevated stabilogram parameters before and after drainage. An improvement of vestibular
function was recorded after drainage. Balance disturbances decreased in children with conductive hearing loss, in this subgroup hearing
improvement also has been shown. Vestibular disturbances in children with accompanied sensorineural hearing loss were still present just
as hearing loss.
Conclusions: The presence of fluid in the middle ear has negative impact on vestibular system function. Our study highlights the importance
of monitoring the condition of the vestibular system in all children with OME, especially in cases with sensorineural hearing loss.
56
Evaluation of eustachian tube function in children after surgical operation of
cleft palate
Zalesska-Kręcicka M¹ , Szleląg J², Kochman M¹, Pazdro-Zastawny K ¹
¹Medical University Hospital ENT Department Wroclaw Poland
²Medical University Clinic of Facial Developmental Malformation of Maxillofacial Orthopaedics and Orthodontics
Wroclaw Poland
P9
STATE OF HEARING AT CHILDREN WITH CLEFT PALATE
Dobrota Davidovic N1 Jovanovic Simic N2
1
Institute for Psyhopathological Disorders and Speech Pathology “Prof. dr Cvetko Brajovic”, Belgrade, Serbia
2
SLP, Faculty of Special Education and Rehabilitation, University of Belgrade, Serbia
Because of malformations in anatomical and morphological structures, children with cleft palate are high risk population for hearing
impairments.
Some of children with cleft palate also have hearing impairment too, and it make speech rehabilitation more complex. Research results
show that prevalence of hearing impairments at children with cleft palate, should not be neglected. By most authors prevalence is going
up to 23%.
In our research the sample was made of 30 children with hearing impairments and cleft palate. They was from 18 months to 3 years old, both
gender and average intelligence. All of them was patients of Institute for psyhopathological disorders and speech pathology “Prof. dr Cvetko
Brajovic”, Belgrade, and they was included in speech-language rehabilitation.
State of auditive perception was examine by AEP, and gained results we use as parameters . During the rehabilitation process we followed
and checked up this parameters periodically.
The results we got, confirm that children with cleft palate are under the high risk for hearing impairments. Degree and type of impairments
depend of many factors. In the beginning phase hearing impairment is conductive, but latter on it’s change in perceptive, in the case if we
don’t notice initial disturbances at plan of auditive potentials, or if child was impropriate cured.
Key words: hearing, hearing impairments, speech rehabilitation, children with cleft palate.
57
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
P10
DIFFERENTIAL DIAGNOSIS BETWEEN LOGOPHATY AND AUDIOPATHY IN HEARING IMPAIRED
CHILDREN
Jovanovic Simic N1 Dobrota Davidovic N2
1
Faculty of special education and rehabilitation, University of Belgrade, Serbia
2
Institute for psyhopathological disorders and speech pathology “Prof. dr Cvetko Brajovic”, Belgrade, Serbia
The subject of the research was distinction between logopathy and audiopathy in hearing impaired children.
Research was perform on population of 719 children, age from 7 to 17 years, in schools for deaf and hearing impaired children in Serbia. The
sample constituted 75 children of both gender with hearing loss between 40-70 dB, which according to international standards falls into the
middle and middle-severe hearing impairment, in the children who have remains of hearing which are repairable for audiological treatment,
and in which this logopathy occurred and could be rehabilitated.
The goal of the research was to establish when and in which cases are occurred audiopathy, distortion in pronunciation as consequence of
conductive type of hearing impairment, or when those are logopathy, as consequence of perceptive hearing impairment.
In this research were established the regularity which enable or unable audibility of some voices in conditions of first and second type of
handicap, as well as frequency of 9,49% cases with hearing loss between 40-70 dB, 9% with perceptive and 91% with conductive hearing
impairment.
In this research was established the correlation between the gender and logopathy of the otogenic origin, intelligence and articulation
disorders, school-age and voice pronunciation.
Key words: audibility, logopathy, auditive dysfunction, pronunciation, auditive perception, voice field, hard of hearing
P11
AUDITORY ABILITIES IN SCHOOL-AGE CHILDREN
Anastasio ART1, Toscano RDGP2
1
PhD in Science from the University of São Paulo. Professor at the Medicine School in Ribeirão Preto, University
of São Paulo, Brazil
2
Audiologist graduated at Medicine School in Ribeirão Preto, University of São Paulo, Brazil
This study aimed to verify the auditory abilities of Sound Detection (SD), Sound Localization (SL), Verbal Sequential Memory (VSM) and
Non-Verbal Sequential Memory (NVSM) in school children from 4 to 6 years old of a Brazilian private school. Sixty-one students had been
evaluated: GA (15 children of 4a 4a11m), GB (23 children of 5a 5a11m) and GC (23 children of 6a 6a11m). The Eye Blink Reflexes (EBR) was
searched. To evaluate the SD was used the pediatric audiometer. The stimulation “warble” was presented in the intensity of 20dBNA 20cm of
the external ear, in 500, 1000, 2000 and 4000 Hz. For analysis of the SD test, the adopted criterion was “pass-fail”, in which “pass” answers
in 20dBNA for all the frequencies and “fails”, absence answers in at least one of the frequencies. The application and analysis of the auditory
abilities of SL, VSM and NVSM had been based in Pereira (1993). To analyze the nonparametric data, Fisher’s test (p<0.05) was used. All the
evaluated children had presented presence of the EBR. In group GA, two children (13%) failed in the SD test and one (2%) failed in the LS. In
the test of VSM, six children (10%) failed, considering that 20% belonged to the GA. In the NVSM 13 children (21%) failed, from which that
26% belonged to the GC. Thirty-six percent of the children failed in at least one of the searched abilities. There is no statistical significant
difference between gender and groups. We conclude that the implantation of programs of inquiry and accompaniment of the auditory abilities
in preschool and school age can be applied with adjusted behaviors to eliminate or to minimize intercurrences that would modify the sociallinguistic development, a time that alterations in these abilities can indicate low school performance in long term.
P12
Neuropsychological correlates of profound deafness in association to
multihandicap in a group of children treated with cochlear implant
a
Gubernale M, aTrevisi P, bGenovese E, aMartini A
a
Università degli Studi di Ferrara, Dipartimento di Discipline medico-chirurgiche, della comunicazione e del
comportamento, UO di Audiologia - C.so Giovecca 203, 44100 Ferrara, Italy
b
Audiologia, Dip.to di Chirurgie specialistiche testa e collo, Università di Modena e Reggio Emilia, Via del Pozzo
71, 41100 Modena
Our clinical team has selected a group of 35 pediatric patients suffering from profound deafness in conjunction with a relatively wide range
58
of other diseases, whose cumulative effects could determine a pool of disabling effects on cognitive development.
The rationale of our contribute consist of trying to define the cognitive profile of the group, within a reasonably neuropsychological approach
to the question in order to some variables, some of them are the etiology of deafness, the grade and the characteristics of cognitive lack, the
neuroradiological aspects, the functional gain of audiological benefices and other behavioral aspects
The sample is composed from implanted patients with age varying from 2.1 to 14.5 age, mean 6.10, with clinic diagnosis indicating profound
sensoryneural deafness with a lot of different etiology linked to: pre-perinatal risk factors, such as positivity for TORCH in pregnancy, birth
foetal suffering, neonatal intensive therapy; infections after the birth; genetic syndromes, such as Waardenburg or Mondini, or other causes.
We have tested the group with the Leiter International Performance Scale Revised (LIPS-R), the LIPS-R subtest for Attention sustained
and Memory screener quotient and the VMI test to pull out the characteristics of cognition in profound deafness, evidencing the global
characteristics different from normal child and the characterization of the qualitative-quantitative relation to cognitive development. Moreover,
we issue the question about hypotethical association between these reports and the neural architecture suggested by RMN pre-implant.
We obtain some results such as: the behavioral response is quite normal or with mild attention deficit; the non-verbal QI is not less of 72
standard points; Attention and Memory are normal or mild insufficient in some case;Visual-motor integration is within normative range.
Finally, discussing of deafness caused from Cytomegalovirus infection is especially interesting, in order to clarify correlation between sites
of encephalic lesions and cognitive outcome.
P13
AGE RELATED HEARING THRESHOLDS IN AN EXTENDED FREQUENCY RANGE
Jilek M, Syka J
Department of Auditory Neuroscience, Institute of Experimental Medicine, Academy of Sciences of the Czech
Republic, v.v.i., Prague, Czech Republic
Objective
The hearing thresholds as a function of age are given by the ISO 7029 standard. The threshold shift with age is expressed as H=α(Y-18)2
(Y=age, α=coefficient given for men and women for each frequency). However, even the most recent version of this standard does not specify
“α” for frequencies over 8 kHz. The aim of our work was to assess the coefficient α for frequencies higher than 8 kHz.
Methods
We measured the hearing threshold levels of 102 (49 male, 53 female) otologically normal persons over a frequency range of 125 Hz -16 kHz
with a Madsen Orbiter audiometer and Sennheiser HDA 200 high frequency headphones.
Results
The measured data were plotted as the hearing level versus age and compared with the theoretical curves according to the ISO standard. The
fitting was found to be not precise for frequencies higher than 8 kHz. Better fitting was obtained with the linear approximation H=ß(Y–18).
On the basis of the calculated coefficients “ß”, the audiograms were normalized. The standard deviation of the mean of the averaged corrected
audiograms at 16 kHz equals 3.5 dB with square approximation and 2dB with linear approximation.
Conclusions
Because the ISO 7029 standard does not cover frequencies higher than 8 kHz, our results can be used in high frequency audiometry. Our
data show that for the highest audible frequencies, the square-law is not valid and better fitting may be obtained with a linear approximation.
Coefficients “ß” for linear approximation at frequencies of 10 kHz, 12.5 kHz and 16 kHz were calculated. The results can be used for the
determination of normal hearing thresholds depending on age.
P14
AHEAD III - Assessment of Hearing in the Elderly:
Aging and Degeneration - Integration through Immediate Intervention
1Grandori F, 2Wolf-Dieter Baumgartner, 3Chryssoula Thodi, 4Arne Ernst, 5 Thomas Janssen, 6 Sophia Kramer, 7
Mariola Sliwinska-Kowalska, 8 Stefan Stenfelt, 9 Rudolf Probst, 10 Adrian Davis
1 Istituto di Ingegneria Biomedica, Italy; 2 Medical University Vienna, ENT-Otology Department, Austria; 3 CH
& M Cyprus Audiology Center Interacoustics Ltd, Cyprus; 4 Hospital of the University of Berlin, Department of
Otolaryngology, Germany; 5 Technische Universitaet Muenchen, Falkultät für Medizin, Lehrstuhl für Hals-NasenOhrenheilkunde, Germany; 6 VU University Medical Center, Department of ENT/Audiology, Netherlands; 7 Medical
University of Lodz, Department of Environmental Otolaryngology, Clinic of Otolaryngology and Laryngological
Oncology, Poland (Presenting); 8 Linköpings Universitet, Faculty of Health Sciences, Division of Technical
Audiology, Sweden; 9 Universität Zürich, Klinik für Ohren-, Nasen-, Hals- und Gesichtschirurgie, Switzerland; 10
University of Manchester, MRC Hearing and Communication Group, United Kingdom;
59
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
Hearing loss is one of the most common chronic health conditions in the elderly population with important implications for patient quality of
life. The diminished ability to hear and to communicate is frustrating in and of itself, but the strong association of hearing loss with depression
and functional decline adds further to the burden on individuals who are hearing impaired. Hearing loss can limit communications skills:
not to hear means not to understand what is being said. Despite the prevalence and burden of hearing loss, hearing impairment is largely
underdiagnosed in older persons and undertreated. The reason for this is that one of the most conspicuous signs of a hearing loss is that it
cannot be seen! Actually, this is the reason why deafness does not receive the necessary attention. Too often, the public and still too many
health care professionals underestimate the dramatic effects of deafness. Novel strategies should be explored to make screening and early
intervention a feasible part of routine care. Project AHEAD III has been specifically designed too: Provide evidence of the effects of hearing
impairment in adults and particularly in the elderly. Analyse costs associated with the implementation of integrated large scale programmes
of hearing screening and intervention in the elderly. Provide quality standards and minimum requirements for screening methods and related
diagnostic techniques. Develop guidelines and recommendations on how to implement successful screening programmes to be tuned to the
local, social, and economical conditions of a country.
Acknowledgment
Supported by the 7th EC Framework Programme – Project acronym: AHEAD III, Grant agreement no. 200835; grant Co-ordinator – prof.
Ferdinando Grandori
P15
Variational anatomy of the human cochlea - implications for cochlear
implantation
Erixon E , Rask-Andersen H
Department of Otorhinolaryngology, Uppsala University Hospital, Uppsala, Sweden
Objective
To study variations in human cochlear anatomy with potential implications for cochlea implantation (CI) surgery.
A comprehension of anatomic variations of the human cochlea is essential for understanding the degree of surgical trauma induced by
inserting various electrode arrays in CI surgery. Variations in anatomy may also limit the potential for performing hearing preservation.
Methods
We studied 73 archival, non-selected, adult, corrosion casts of human inner ears. Anatomic reference points were constructed from
photographic reproductions taken at different angles and various dimensions were assessed using planimetry. Anatomic variants with
particular clinical/surgical interests were pinpointed.
Results
Results showed that human cochlea is individually shaped with varying dimensions (“fingerprint”). Various dimensions will be presented.
Total length of the human cochlea could differ up to 0.9 mm. The microanatomy of the spiral ganglion was analysed and related to place/
frequency map.
Conclusions
The human cochlea exhibits extensive anatomic variations. These variations will influence the location of various CI arrays and affect the
potential of hearing and preservation surgery. Our results may explain surgeons sometimes experienced difficulties to insert electrode array
entirely even in so-called “normal” cochleae.
pitch near 16kHz starting 21 days after exposure. Hearing function was tested in all animals: salicylate caused an average temporary threshold
shift of less than 10 dB and essentially no permanent shift; animals in the noise-trauma group showed a temporary and permanent threshold
shift at high frequencies (+/- 20dB,12-24Khz). In conclusion, the present study confirms the validity of the GPIAS protocol to rapidly assess
tinnitus in rats with no training, memory, learning or food deprivation necessary and the ability to identify the pitch of tinnitus. Data obtained
on salicylate and noise-induced tinnitus are consistent with those available in literature and contribute to clarify tinnitus induction and
assessment in the animal model with potential applications in future tinnitus research.
P17
PROTECTIVE EFFECTS OF GINKGO BILOBA EXTRACT AGAINST CISPLATIN OTOTOXICITY
Simoni E, Donnarumma T, Astolfi L, Ciorba A, Martini A
Audiology Department and Hearing Protection Plasticity and Regeneration Laboratory, University of Ferrara.,
Ferrara- Italy
Reactive oxygen species (ROS) are implicated in an increasing number of pathologies. Above all, oxidative stress is strongly implicated in
hearing loss due to the ototoxic action of cisplatin or aminoglycoside and noise damage. Cisplatin is a chemotherapy agent that is used as a
treatment for several types of cancer. Unfortunately it could injure several areas of the cochlea, including outer hair cells in the Organ of Corti,
the spiral ganglion and the stria vascularis. The focus of this research was verify the protective effects of a nutraceutical product compounds
with antioxidant activity (ACUVAL®, Scharper Healthcare, Italy) against cisplatin toxicity. The Gionkgo biloba extract (Ginkgoselect®) action
was evaluated by cytofluorimetry in a mouse inner ear cell line (OCk3). The Ginkgo biloba extract is know to have antioxidant proprieties
with a free radicals scavenging effect, and protecting cells against apoptosis. The data obtained in this study show that Ginkgo biloba extract
protects against cisplatin-induced ototoxicity. These results suggest the opportunity that the medical use of antioxidant agents could reduce
or prevent damage to the inner ear of patients exposed at cisplatin chemotherapy.
P16
ASSESMENT OF SALICYLATE AND NOISE INDUCED TINNITUS IN RATS USING A NOVEL
BEHAVIORAL PARADIGM
Ralli M (2), Lobarinas E (1), Paludetti G (2), Salvi R (1)
1: Center for Hearing and Deafness, Department of Communicative Disorders and Sciences, University at Buffalo,
Buffalo NY United States
2: Catholic University of Sacred Heart, Department of Otolaryngology, Rome, Italy
Sodium Salicylate has been shown to reliably induce short-term tinnitus in different animal models when administered at high doses. Noise
trauma has been instead reported to induce long-term tinnitus. The present study compared salicylate to noise induced tinnitus-like behavior
in rats using the novel gap pre-pulse inhibition of acoustic startle (GPIAS). Twelve rats were divided into 2 groups (n=6); one group was
injected intraperitoneally with salicylate (250 mg/kg), the other was exposed unilaterally to a 12kHz, 126dB narrow-band noise (BW=100Hz)
for 15 minutes. All 12 rats were subsequentially tested for tinnitus and hearing loss at 6, 12, 16, 20 and 24 kHz; tinnitus was assessed using
GPIAS, hearing function was measured with DPOAE, ABR and noise-burst pre-pulse inhibition of acoustic startle (NBPIAS). The results
revealed transient tinnitus-like behavior in all rats following salicylate injection, starting 2h after treatment and resolving spontaneously 24h
later; among rats exposed to unilateral noise trauma, 4 showed transient tinnitus during the first week and stable long-term tinnitus with a
60
61
The XIV International Symposium
in Audiological Medicine
September 18-21, 2008
Sala S. Francesco, Ferrara
G eneral Information
Certificate of Attendance
A certificate of attendance will be given to all registered participants.
Conference Venue
Sala S. Francesco
C/o Centro Culturale S. Francesco
(right by the S.Francesco Church)
Piazza S. Francesco, 7
44100 Ferrara (FE)
Tourist Program
A Tourist Desk will be available on site to provide information and sell excursions in Ferrara.
Exhibition Area
Ample space will be devoted to the Exhibition Area, featuring commercial displays of Pharmaceutical and Product Companies, Medical Publishers and Scientific Societies.
The exhibition will be open throughout Congress hours.
Insurance
Dates
September 18 – 21, 2008
Registration desk opening hours:
Thursday, September 18 from 15.00 to 19.00
Friday, September 19 from 8.00 to 19.00
Saturday, September 20 from 8.00 to 19.00
Sunday, September 21 from 8.00 to 18.30
Language
The official language of the Conference is English.
Translation Italian/English will be available.
Participants are advised to personally take out their own insurance policy for baggage, valuables and liability to third parties since the conference organisers decline any liability in this
regard.
Cancellation and refunds
Notice of cancellation of registration must be made by email (to [email protected])
or by fax (+39 049 860 2389) to the Conference Secretariat, Meet and Work srl.
For cancellations made by July 31, 2008 60% of registration fees received will be refunded.
The Conference Secretariat regrets that no refunds will be possible for cancellations received
after August 1, 2008.
All refunds will be processed after the conference.
Disclaimer
ECM
•Categoria “Medico Chirurgo”: 14 crediti
•Categoria “Tecnico Audiometrista”: 12 crediti
•Categoria “Tecnico Audioprotesista”: 10 crediti
•Categoria “Logopedista”: in fase di accreditamento
The Organisers cannot be considered responsible for the cancellation of the Conference or
parts of it. In case of total cancellation, conference participants will be reimbursed for the
registration fees actually paid. However, the Organisers are not liable for any other loss or
inconvenience caused as a result of such cancellation.
Access to the conference Site
Participants should wear the identification badge, collected at the Registration Desk, during all
conference sessions and events.
62
63
The XIV International Symposium
in Audiological Medicine
The Organisers gratefully acknowledge for their support to the
XIV International Symposium in Audiological Medicine:
Social Events
MAIN SPONSORS
❙Welcome Reception
at 19.00 on Thursday, September 18
in the Sala S. Francesco, conference venue
(open to all participants and accompanying persons)
❙Conference Dinner
at 20.30 on Friday, September 19
at Palazzo San Crispino in Ferrara
(via Contrari, 8).
Reservations will be possible on site
until 12.00 noon Friday 19 September,
provided places are still available.
Cost per person: 70 euro.
SPONSORS
❙The Copernico Prize Giving Ceremony
at 18.30 on Saturday, September 20,
in the Aula Magna of the University of Ferrara (via Savonarola 9).
(open to all participants and accompanying persons).
• Opening Concert
• Prize Giving Ceremony
• Cocktail Reception
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64
Piazza del Sole e della Pace 5
35031 Abano Terme (Padova)
Tel. 049.8601818
Fax 049.8602389
e-mail: [email protected]
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