Cochlear implantation in Brown–Vialetto–Van- Laere syndrome

Sinnathuray, A R, Watson, D R, Fruhstorfer, B, Olarte, J R and Toner, J G (2011) Cochlear implantation in Brown–Vialetto–Van- Laere syndrome. Journal of Laryngology and Otology, 125 (3). pp. 314-317. ISSN 0022-2151

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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.

Item Type: Article
Schools and Departments: Brighton and Sussex Medical School > Neuroscience
Subjects: R Medicine > RC Internal medicine > RC0321 Neurosciences. Biological psychiatry. Neuropsychiatry
R Medicine > RF Otorhinolaryngology
Depositing User: Parisa Rafizadeh-Farahani
Date Deposited: 10 Aug 2016 13:09
Last Modified: 26 Sep 2017 12:44
URI: http://sro.sussex.ac.uk/id/eprint/62352

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