Three of four sibs were affected by a nonprogressive neural hearing loss and atypical atopic dermatitis. History, physical and neurological examinations revealed no causes other than familial for the hearing loss. Audiometric examinations suggested the hearing loss was cochlear in origin. A review of the literature describing types of familial hearing loss associated with skin disease showed no similar report. It is suggested that these three children have a type of recessive familial hearing loss differing from others in its association with atypical atopic dermatitis.