Congenital cholesteatoma

Current Opinion in Otolaryngology & Head and Neck Surgery
Ken Kazahaya, William P Potsic

Abstract

Congenital cholesteatomas have historically been considered a rare disorder. However, a review of the literature reveals an incidence ranging from 4 to 24%, and these values are probably underestimated. This article summarizes the general diagnostic criteria of congenital cholesteatomas, their usual presenting symptoms and findings, perioperative studies, a proposed staging system to assist with predicting postoperative outcomes, general surgical procedures, and recidivism rates. Congenital cholesteatomas have been defined as evidencing no prior history of otorrhea, tympanic perforation, or previous otologic procedures; a normal pars flaccida and tensa; and a pearly white mass medial to the tympanic membrane. Their cause remains controversial; however, the epithelial rest theory is most commonly accepted. The incidence of this disorder seems to be on the rise; however, this may be a result of increased awareness and reporting. The most common sites of presentation on physical examination are the anterior-superior and posterior-superior quadrants of the tympanic membrane. Conductive hearing loss is the most common presenting symptom. There is a lack of uniformity of reporting and classifying congenital cholesteatomas. Staging sy...Continue Reading

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