The risk of cholesteatoma in congenital aural stenosis

TLDR

Congenital aural stenosis carries a much greater risk of cholesteatoma than congenital aural atresia. The study recommends early surgery for patients with external ear canal stenosis ≤2 mm, ideally in late childhood or early adolescence before irreversible damage occurs. The authors reviewed more than 600 patients with major congenital ear malformations, identifying 50 patients (54 ears) with congenital aural stenosis. Among patients 12 years or older with a stenosis ≤2 mm, 91 % of ears had cholesteatoma, indicating that a bony canal opening ≤2 mm confers high risk of cholesteatoma formation.

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.