Slide Presentation from the AHRQ 2007 Annual Conference

Genetics of Hearing Loss

Screen shot of the middle section of the Newborn Hearing Screening (NHS) Web page titled " Genetics of Hearing Loss"

Approximately sixty percent of prelingual hearing loss is genetic.

Several hundred genes are known to cause hereditary hearing loss and deafness. This hearing loss may be conductive, sensorineural, or a combination of both; syndromic or nonsyndromic; and prelingual (before language develops) or postlingual (after language develops).

Genetic forms of hearing loss must be distinguished from acquired (non-genetic) causes of hearing loss and are diagnosed by otologic, audiologic, and physical examination, family history, other tests such as CT examination of the temporal bone and molecular genetic testing. Molecular genetic tests are available for many types of syndromic and nonsyndromic hearing loss. The tests specific for these genes may be available only for research in some cases but many can be ordered as clinical tests.