For prelingual hearing loss, epidemiological data show that 1 neonate in 1, 000 is born with severe to profound hearing loss and in half that number the loss is inherited. Hereditary hearing loss is classified into syndromic (30%) and non-syndromic (70%) deafness. Nonsyndromic deafness is further classified by mode of inheritance (DFNA, dominant (30%); DFNB, recessive (66%); DFN, X-linked (4%)), with the loci being numbered in the order of discovery by Human Genome Organization. Deafness caused by maternal inheritance by mitochondrial DNA has been also demonstrated. To date, 41 autosomal dominant, 30 autosomal recessive, and 8 X-linked nonsyndromic sensorineural hearing impairment loci have been mapped and 27 genes have been cloned (Hereditary Hearing Loss Homepage, http://dnalab-www.uia.ac.be/dnalab/hhh/). The common deafness genes as well as deafness genes which can cause variable phenotype, such as syndromic or nonsyndromic deafness, or dominant or recessive inheritance, are reviewed. And mutant mice with inner ear disorders, which represent the most likely candidates for homology with syndromic and non-syndromic losses of hearing in humans are briefly discussed.
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