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


METHODS
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.


METHODS
Cochlear implantation.


METHODS
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.


CONCLUSIONS
These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.
Sinnathuray
A R
AR
Northern Ireland Regional Cochlear Implant Centre, Belfast City Hospital, Belfast, Northern Ireland, UK. rajsinn@aol.com
Watson
D R
DR
Fruhstorfer
B
B
Olarte
J R
JR
Toner
J G
JG
D000328:Adult; D001309:Auditory Threshold / Q000502:physiology; D010244:Bulbar Palsy, Progressive / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D019929:Cochlear Implantation; D003054:Cochlear Implants; D018450:Disease Progression; D005072:Evoked Potentials, Auditory; D005260:Female; D006310:Hearing Aids; D006319:Hearing Loss, Sensorineural / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D035781:Siblings; D013063:Speech Discrimination Tests; D013067:Speech Perception; D016896:Treatment Outcome