Stapes fixation associated with symphalangia.

We describe a 10-year-old boy and his 9-year-old sister, both of whom had bilateral hearing loss associated with ankylosis of the proximal interphalangeal joints (symphalangia). They had no other abnormal findings except hearing loss and ankylosis of the joints of some fingers and toes on systemic examination. The cause of conductive hearing loss in both cases was bony fusion between the stapes and the bone of the oval window niche. There were no other anatomical abnormalities in the middle or the external ear. The patients' hearing improved markedly after stapes surgery. Histopathologic examination of the stapes revealed an abnormal zone of ossification near the anterior annular ligament and calcification in the annular ligament, which seemed to be the cause of the stapes fixation.