Primary Cholesteatoma Within the Stenotic Ear Canal: Report of Two Cases, One With Extra-Temporal Complications

Abstract

Congenital aural stenosis can be complicated with primary cholesteatomas which may be overlooked until the occurrences of resultant complications or incidentally diagnosed during or before corrective surgeries. In this report, samples of both "complicated" and "occult" types of cholesteatomas associated with aural stenosis are presented with the aim of illustrating their clinical course and shedding light on their possible pathogenesis. We herewith report 2 patients with congenital aural stenosis who developed cholesteatoma related to the external ear canal (EEC) stenosis. One of the cases, had previously undergone craniotomy for intracranial complication of the disease, and also had postauricular fistula, whereas in the second patient it was silent and confined within the relatively enlarged stenotic ear canal in front of a rudimentary tympanic membrane. In the first case cholesteatoma was removed with mastoidectomy in the first phase and then atresioplasty was carried out with tympanoplasty. In both cases, a novel two-stage technique was used for EEC reconstruction with success. Congenital aural stenosis predisposes cholesteatoma most likely due to entrapping of the squamous epithelium within the stenotic EEC or tympanum, and these cases were more prone to the extra-tympanic complications of cholesteatoma than those with the normal EEC. In conclusion, otologists should hold a high grade of suspicion of cholesteatoma in a patient with stenotic EEC, and their presence should be ruled out prior to any definitive corrective surgery.