Congenital cholesteatoma is a rare ear disorder. The most common presentation is a pearly and white mass, visible with an intact tympanic membrane in individuals with no previous history of ear discharge, ear surgery or perforation of tympanic membrane. Based on a careful overview of literature, authors of this article present: the most probable theories of the cause of development of congenital cholesteatoma, diagnostic criteria of congenital cholesteatoma, its most common clinical symptoms, preoperative studies, methods of surgical treatments and goals of postoperative proceedings. Furthermore, authors present a comparison of histological, molecular and clinical features of congenital and acquired cholesteatomas.
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