A rare case of stapes anomaly with ossified stapedius tendon in a patient with Turner syndrome

Congenital anomaly of ossicles in the middle ear is rare. We present a patient with Turner syndrome who presented with hearing loss. Pure-tone audiometry revealed bilateral moderate-to-severe hearing loss with conductive type at the lower and mixed type at the higher frequencies. There was a bone threshold dip at 2 kHz on both sides. The patient was diagnosed as having bilateral otosclerosis and underwent left middle ear surgery. Intraoperative findings showed an ossified stapedius tendon and a fixed monopod stapes with obliterated obturator foramen. The stapedius tendon was divided causing the footplate of the stapes to be mobile. This resulted in improved hearing with the closure of air-bone gap by around 40-50 dB. Stapes anomaly and ossified stapedius tendon causing stapes fixation may results in hearing loss with Carhart's notch on pure-tone audiometry and misdiagnosed as otosclerosis. Surgery in which the ossified stapedius tendon is divided may help improve the patient's hearing.