The question mark ear: an unappreciated major anomaly of the auricle.

Eight and possibly nine cases of a major congenital ear anomaly that has been termed the question mark ear have been presented in the course of the last two decades. The consistency of the appearance of the anomaly and the demonstration that it has in several instances been familial makes it clear that a chance event is not involved, but rather that a consistent anomaly is being described. Appropriate repair requires reduction in size and prominence of the upper ear as well as correction of the waist between the upper ear and the lobule. This anomaly should take its place with the other major congenital defects of the external ear.