Congenital Stapes Fixation Accompanied by Symphalangism

Five Patients in Two Families

Abstract

Five patients in two different families were treated surgically for congenital stapes fixation. The footplate of the stapes was found to be completely fixed to the oval window in each case. Ankylosis was present in the proximal interphalangeal joints of both hands. This type of symphalangism was probably due to a maldevelopment of ossification and a lack of formation of joint apertures through the differentiating regions of cartilage. These processes were presumed to occur at about the eighth week of gestation. In such patients, stapes surgery is indicated and at the same time, all the joints in the body should be examined.