Auditory responses in paediatric cochlear implant users with and without GJB2 deafness.
Published: 2006
Language: eng
Number of pages: 50
ISBN-13: 9780494161784
Description: In GJB2-related deafness, we hypothesize that because gap junction deficits are uniformly distributed along the cochlea, there will be a uniform pattern of cochlear damage as compared with subjects with non-GJB2 etiologies. Blood from 301 paediatric cochlear implant users was analyzed for mutations in GJB2. Thirty-nine children with biallelic GJB2 mutations and 58 without GJB2 mutations were evaluated. After cochlear implantation, neural activity at apical and basal ends of the implanted array was measured using electrically evoked compound action potentials of the auditory nerve and evoked stapedius reflexes. Children with GJB2 deafness had greater similarities between low and high frequency residual hearing and between neural activity evoked at apical and basal regions of the cochlea as compared with children with non-GJB2 deafness. The latter group demonstrated larger deficits in basal regions. Results suggest more uniform spiral ganglion survival along the cochlea in GJB2 deafness as compared with non-GJB2 deafness.
Notes: Source: Masters Abstracts International, Volume: 44-06, page: 2750.

Thesis (M.Sc.)--University of Toronto, 2006.

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