Cochlear implants for DFNA17 deafness

Abstract

BACKGROUND: Nonsyndromic autosomal-dominant, adult-onset sensorineural hearing loss resulting from DFNA17 was described in a single American kindred in 1997, and the causative gene was subsequently identified as MYH9. OBJECTIVE: The objective of this study was to report clinical and genetic analyses of an Australian family with nonsyndromic adult-onset sensorineural hearing loss. METHODS: The clinical presentation of the family was detailed and identification of the causative gene was conducted by SNP genotyping and direct sequencing. RESULTS: Sequence analysis of the MYH9 gene revealed the same missense mutation as in the original DFNA17 family. We are not aware of a link between the two ki..