Congenital anomalies limited to the middle ear

Congenital anomalies of the middle ear are occasionally encountered during surgery for conductive hearing loss and are unexpected in patients with no other deformities. We reviewed 12 such patients operated on at The New York Eye and Ear Infirmary from 1985 through 1989. Nine of the patients (75%) had unilateral conductive hearing loss whereas three (25%) had bilateral symptoms. One had bilateral congenital middle ear anomalies. Three patients (25%) had anomalies limited to the malleus and scutum. Five patients (47%) had agenesis of the oval window. After reconstructive surgery, 72% of patients had hearing improvement ranging from 13 to 38 dB. The etiology of these anomalies is discussed and their evaluation and surgical indications are presented.