Malformations Associated With Pediatric Congenital Cholesteatomas.

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To describe malformations associated with pediatric congenital cholesteatomas of the middle ear.Retrospective study.Tertiary referral center.One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017.Demographic, clinical, and surgical data were collected from operative reports.We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation.CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p 

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