Abstract

There are 12 types of hereditary hearing loss with no associated abnormalities: (1) dominant congenital severe deafness, (2) recessive congenital severe deafness, (3) sex-linked congenital deafness, (4) recessive early-onset deafness, (5) sex-linked early-onset deafness, (6) dominant low-frequency hearing loss, (7) dominant mid-frequency hearing loss, (8) dominant progressive hearing loss, (9) dominant unilateral deafness, (10) otosclerosis, (11) recessive congenital moderate hearing loss, and (12) sex-linked mixed hearing loss with fixation of stapedial footplate. The major features of these diseases are reviewed.

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