Michel's anomaly, type I microtia and microdontia.

Michel's anomaly is an extremely rare cause of congenital sensorineural hearing loss. We present a 2-1/2 year old white female with this inner ear defect associated with type I microtia and microdontia. Assessment of anatomic structures of the ear is critical in an infant with abnormal auditory brainstem responses to determine whether a structural lesion is present and the most appropriate rehabilitative approach available.