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.
D000293:Adolescent; D000328:Adult; D001309:Auditory Threshold; D005260:Female; D005808:Genes, Recessive; D008040:Genetic Linkage; D006311:Hearing Disorders / Q000235:genetics*; D006801:Humans; D007621:Karyotyping; D008297:Male; D010375:Pedigree; D012730:Sex Chromosomes