A cochlear implant device is normally inserted into the scala tympani via a posterior tympanotomy and cochleostomy. There has been no previous report of displacement of the array into the vestibular part of the labyrinth. The authors present and discuss the audiological and electrophysiological measurements of a case in which part of the array herniated through into the vestibule.
The lateral semicircular canal has been described to be the most commonly affected part of the inner ear in humans, and flouncer provides a mouse model for genetic and developmental analysis of such defects.
Waardenburg syndrome presents with dystopia canthorum, pigmentary abnormalities of hair, iris and skin (often a white forelock and heterochromia iridis) and sensorineural deafness. The authors review the electrophysiological and psychophysical findings of implanted children with Waardenburg syndrome at the Sydney Cochlear Implant Centre. Twenty children with Waardenburg syndrome received cochlear implants between 1985 and 2001. Electrical auditory brainstem response (EABR) was performed in all of these patients intra-operatively as part of the routine investigations. Only 13 of these patients were assessed one year or more post-operatively by means of the Melbourne Categories (0-7). Four patients (20%) were found to have abnormal EABR recordings. The mode of Melbourne Categories in this group (n = 3) was 1 at one year post-operation. The other 16 patients were found to have normal EABR and the mode of Melbourne Categories in this group (n = 10) was 7. A poor outcome after cochlear implantation was associated with abnormal EABR recordings (a 'true' auditory neuropathy) and was found in a significant proportion of patients with Waardenburg syndrome.
The study results indicate developmental problems of the first and second branchial arches and otocyst as a result of a single gene mutation. Similar defects are found in humans, and hush puppy provides a mouse model for investigation of such defects.
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