Congenital stapedial fixation associated with Beckwith--Wiedemann syndrome: two cases of a woman and her brother.

Beckwith-Wiedemann syndrome (BWS) is characterized by the triad of exophthalmos, macroglossia, and gigantism (EMG syndrome) presented at birth. Two patients with BWS, a woman and her younger brother, who presented with conductive deafness caused by middle ear anomaly are reported. Stapedial fixations were confirmed by tympanotomy, and satisfactory hearing improvements were obtained by small fenestra stapedectomy. Hearing loss associated with BWS has not previously been reported. Characteristic sulci on the auricular lobe of BWS are thought to be caused by malposition of the antitragus, which originates from the second branchial arch. Stapedial fixation might be due to maldevelopment of the stapedial lamina, which also originates from the second branchial arch. There could be a causal relationship between stapedial fixation and BWS.