Proximal Symphalangia and Stapes Ankylosis

• A father and his two sons had an autosomal-dominant syndrome of proximal symphalangia and conductive hearing loss. Exploratory tympanotomy revealed a congenitally fixed stapes and incus in the oldest boy. Surgical intervention resulted in normal hearing. To our knowledge, this is the first such histologic report of a stapes in this syndrome. ( Arch Otolaryngol 1985;111:765-767)