HEARING-LOSS AND THE MAYER-ROKITANSKY-KUSTER-HAUSER SYNDROME

The hearing of 51 female patients with the Mayer-Rokitansky-Kuster-Hau ser syndrome was examined using otoscopy and standard audiometry. A un ilateral or bilateral hearing loss of more than 15 dB Fletcher index w as found in 13 of 51 (25.5%). Four of these 13 patients had a hearing loss of less than 20 dB in the worst ear. The remainder had a hearing loss of at least 30 dB in the worst ear. Five of the 13 patients had p ure conductive hearing loss; in four of these five, a congenital origi n was accepted. Two of the 13 had mixed hearing loss that was a residu al symptom from previous otitis media; six had sensorineural hearing l oss. A congenital cause was found in one of these six, based on the fa ct that she had been deaf and dumb since birth. In one other patient, noise-related deafness was likely (i.e., an acquired cause). In the ot her four cases in this group, the cause was unknown. The results of th is study show that hearing loss is a characteristic associated with th e Mayer-Rokitansky-Kuster-Hauser syndrome.