Autosomal Dominant Nonsyndromic Hearing Impairment: an Overview

Abstract

Fifteen percent of cases of prelingual non-syndromic hereditary hearing impairment have an autosomal dominant mode of inheritance. In addition, postlingual non-syndromic progressive hearing impairment is often inherited in an autosomal dominant fashion. Similar to autosomal recessive non-syndromic hearing impairment, autosomal dominant non-syndromic hearing impairment is extremely genetically heterogeneous. To date, 41 chromosomal regions have been shown to contain a gene that is involved in an autosomal dominant type of non-syndromic hearing impairment, the so-called DFNA loci. Fifteen of the corresponding genes have been identified, including channel and gap junction proteins, unconventional myosins, transcription factors, extracellular matrix proteins and genes with an unknown function. These gene identifications are increasing our knowledge of the molecular basis of hearing, and might lead to new therapeutic measures for hearing impairment in the future.