Branchio-oto-renal dysplasia (BOR dysplasia) is an autosomal dominant disorder characterized by ear malformation, hearing loss, cervical fistulae and renal abnormalities. In this report we presented three cases of children with BOR dysplasia accompanied by mild or severe deafness. In one case exploratory myringotomy revealed an adhesion of the malleus neck to the scutum. Mobilization of the adhesive region did not improve the hearing loss. In mother of this case a sensorineural hearing loss in both ears deteriorated gradually in several years. In another case tympanoplasty was carried out in both ears for middle ear cholesteatoma. However, hearing loss did not improve postoperatively. The audiological findings indicated the malformation and the vulnerability of the inner ear. So audiological management over a long period of time should be needed and the indications of surgical treatment should be considered carefully.
