Cochlear Implantation in Brown–Vialetto–Van-Laere syndrome

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

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Bibliographic Details
Published in:Journal of laryngology and otology 2011-03, Vol.125 (3), p.314-317
Main Authors: Sinnathuray, A R, Watson, D R, Fruhstorfer, B, Olarte, J R, Toner, J G
Format: Article
Language:English
Subjects:
Adult
Age
Auditory Threshold - physiology
Biological and medical sciences
Bulbar Palsy, Progressive - pathology
Bulbar Palsy, Progressive - physiopathology
Bulbar Palsy, Progressive - surgery
Cochlear Implantation
Cochlear Implants
Disease Progression
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Evoked Potentials, Auditory
Female
Hearing Aids
Hearing loss
Hearing Loss, Sensorineural - pathology
Hearing Loss, Sensorineural - physiopathology
Hearing Loss, Sensorineural - surgery
Humans
Lipreading
Male
Malformations of the eye
Medical sciences
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Non tumoral diseases
Ophthalmology
Ostomy
Otorhinolaryngology. Stomatology
Patients
Reading comprehension
Siblings
Speech Discrimination Tests
Speech Perception
Treatment Outcome
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Summary:To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome. 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. Cochlear implantation. Post-implantation speech discrimination scores. 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. These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.
ISSN:0022-2151
1748-5460
DOI:10.1017/S0022215110001982