A New Era in the Genetics of Deafness

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Abstract
There has been tremendous progress in research on the genetic basis of deafness. Ten different genes for nonsyndromic (uncomplicated) deafness have been identified since May 1997.1 It has always been assumed that single-gene defects were responsible for the condition in approximately half of the 1 in 1000 children who are born with a serious hearing impairment, but many of the newly identified genes causing deafness involve progressive loss of hearing that begins in adulthood, suggesting that single genes may also play an important part in hearing loss in the population as a whole. Sixteen percent of adults have clinically relevant . . .