X-linked recessive inheritance of sensorineural hearing loss expressed during adolescence

Eight males in 4 generations with hearing impairment were observed in a kindred; family transmission suggested X-linked recessive inheritance. In previously reported cases of X-linked sensorineural hearing loss, hearing impairment was usually severe to profound and was either present at birth or manifested by 5 years of age. In the present cases, rapid onset of a bilateral sensorineural hearing loss occurred during adolescence and did not generally progress beyond moderate impairment. Significant deterioration in seech production did not usually result.