title: The risk of cholesteatoma in congenital aural stenosis.
abstract: Congenital aural stenosis, as compared to congenital aural atresia, carries a much greater risk of cholesteatoma. In a review of over 600 patients with major congenital ear malformations, 50 patients (54 ears) were found to have congenital aural stenosis. Ninety-one percent of the ears in patients 12 years and older with a stenosis of 2 mm or less presented with cholesteatoma. Based on this review, the following opinions are given: 1. A bony ear canal opening of 2 mm or less puts the patient at risk of cholesteatoma formation; 2. the cholesteatoma is slow-growing; and 3. undiagnosed cholesteatoma will cause extensive damage to the ear by early adulthood. We recommend surgery for patients with stenosis of the external ear canal measuring 2 mm or less. The appropriate time for surgery is late childhood or early adolescence, before irreversible damage has occurred.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Health Science Center, Houston 77030.
authors.forename: R R
authors.initials: RR
authors.lastname: Cole
authors.forename: R A
authors.initials: RA
authors.lastname: Jahrsdoerfer
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000000981:diagnostic imaging / Q000209:etiology*; D004424:Ear Canal / Q000002:abnormalities* / Q000000981:diagnostic imaging; D004427:Ear Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D011859:Radiography; D012307:Risk Factors