Congenital stapes fixation, one of the causes of congenial conductive heaing loss, may occur alone or in combination with other syndromes and rarely is accompanied by anomalies of branchial arch or branchial cleft. It must be differentiated from otosclerosis, since both present normal tympanic membrane and profound conductive hearing loss. Hearing loss in congenital stapes fixation can be restored by stapedectomy. Authors report a case of stapes fixation associated with symphalangism and a case of bilateral stapes fixation which underwent stapedectomy with favorable postoperative results.