Primary cholesteatoma in the congenitally atretic ear

A developmental aberration significant enough to cause a congenital auricular atresia may well entrap ectodermal keratinous elements medial to an atresia plate, resulting in a primary cholesteatoma. We report such a case and discuss the proposed pathogenesis and pertinent embryology. A two-stage surgical procedure is offered as a reasonable management alternative when the cholesteatoma is well encapsulated.