Cochlear Implantation in Brown–Vialetto–Van-Laere syndrome

• Published in: Journal of laryngology and otology Vol. 125; no. 3; pp. 314 - 317
• Main Authors: Sinnathuray, A R, Watson, D R, Fruhstorfer, B, Olarte, J R, Toner, J G
• Format: Journal Article
• Language: English
• Published: Cambridge, UK Cambridge University Press 01.03.2011
• 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; Sydney New South Wales Australia; Australia; Cochlear Implantation; Sensorineural Hearing Loss; Brown–Vialetto–Van Laere Syndrome; Progressive Bulbar Palsy; Auditory Evoked Potentials; Brown-Vialetto-Van Laere Syndrome; Motor system disorder; Auditory disorder; ENT; Electrodiagnosis; ENT disease; Perception hearing loss; Paralysis; Oculomotor syndrome; Neurological disorder; Nervous system diseases; Brown syndrome; Internal ear disease; Implantation; Congenital disease; Progressive; Eye disease; Malformation; Auditory evoked potential; Ophthalmoplegia; Cochlea; Sensory hearing loss

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...