Management of congenital ear abnormalities.

Congenital malformation of the ear has a complex origin and the possibility of hereditary, viral and toxic etiologies. The authors require audiometric evaluation, using the objective brain stem evoked response in young children, and polytomography of the middle and inner ears in all patients with atretic external auditory canals. Unilaterally atretic ear canals can be repaired electively at the discretion of the family and the patient, while in bilateral cases reconstruction is ideally done at approximately age four years. Regular follow-up is recommended to be certain that no cholesteatoma develops medial to an atretic external ear canal. The authors propose a working classification for congenital malformation of the ear. In the author's series of 35 operations for the repair of congenital ear abnormalities, 55% were improved to adequate levels of hearing without the use of a hearing aid. Eight exemplary cases and comments are presented.