Deafness, Progressive, With Stapes Fixation

Thies et al. (1996) described 4 adult sisters with bilateral conductive hearing loss, starting between the ages of 8 and 24 years. Tympanotomy showed ossification of the stapedial tendon or a bony bar from the neck of the stapes to the pyramidal eminence, leading to stapes fixation. After surgical removal of the bony tendon, hearing became normal in 3 sisters; in 1 sister, who had an additional deformity of the head of the stapes in the right ear, normal hearing was not restored after surgery. The parents of these sisters and 4 other sibs had normal hearing. This family showed an apparently autosomal recessive form of hearing loss due to stapes fixation. A similar type of hearing loss was reported by Grant and Grant (1991).