STAPEDIAL ANKYLOSIS IN THE MAYER-ROKITANSKY-KUSTER-HAUSER SYNDROME

We describe a 21-year-old woman with congenital unilateral conductive hearing loss and an atypical form of the Mayer-Rokitansky-Kuster-Hause r syndrome. To our knowledge, this is the first reported case of this syndrome in which surgery for congenital stapedial ankylosis was succe ssful. Besides aplasia of the vagina and uterus, the patient also had various other anomalies, such as the Klippel-Feil syndrome, Sprengel's deformity , and congenital stapedial ankylosis. Congenital hearing lo ss is an associated characteristic of the Mayer-Rokitansky-Kuster-Haus er syndrome (10% to 20% of cases), particularly in the atypical form. Against the background of the favorable results of surgery for isolate d unilateral congenital stapedial ankylosis and other unilateral conge nital anomalies of the middle ear that have been described in the lite rature and the significant advantages of bilateral hearing, we used st apedectomy to successfully treat this case of Mayer-Rokitansky-Kuster- Hauser syndrome with unilateral congenital stapedial ankylosis.