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Cochlear Implantation in Brown–Vialetto–Van-Laere syndrome

Published online by Cambridge University Press:  19 October 2010

A R Sinnathuray ,
D R Watson ,
B Fruhstorfer ,
J R Olarte  and
J G Toner
A R Sinnathuray*
Affiliation:
Northern Ireland Regional Cochlear Implant Centre, Belfast City Hospital, Northern Ireland, UK
D R Watson
Affiliation:
Department of Mental Health, Queen's University Belfast, Belfast City Hospital, Northern Ireland, UK
B Fruhstorfer
Affiliation:
Northern Ireland Regional Cochlear Implant Centre, Belfast City Hospital, Northern Ireland, UK
J R Olarte
Affiliation:
Northern Ireland Regional Cochlear Implant Centre, Belfast City Hospital, Northern Ireland, UK
J G Toner
Affiliation:
Northern Ireland Regional Cochlear Implant Centre, Belfast City Hospital, Northern Ireland, UK
*
Address for correspondence: Mr A R Sinnathuray, 30 The Boulevard, Wellington Square, off Annadale Avenue, Belfast BT7 3LN, Northern Ireland, UK Fax: 02890263549 E-mail: rajsinn@aol.com
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Abstract

Objective:

To report outcomes for the first known cochlear implantation procedures in two patients with Brown–Vialetto–Van-Laere syndrome.

Patients:

Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown–Vialetto–Van-Laere syndrome. The female patient presented with a milder form of the syndrome.

Intervention:

Cochlear implantation.

Main outcome measure:

Post-implantation speech discrimination scores.

Results:

Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford–Kowal–Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford–Kowal–Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise.

Conclusion:

These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

Keywords

Cochlear ImplantationBrown–Vialetto–Van Laere SyndromeProgressive Bulbar PalsySensorineural Hearing LossAuditory Evoked Potentials
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 125 , Issue 3 , March 2011 , pp. 314 - 317
Copyright
Copyright © JLO (1984) Limited 2010

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