Bilateral congenital cholesteatoma: A case report and review of the literature

Congenital cholesteatoma (CC) occurs less commonly than acquired cholesteatoma (AC), and bilateral CC (BCC) is even more rare with only 38 such cases having been reported in the past 42 years. Because of the rarity of this condition, providers confronted with cases of BCC may find it difficult to treat while balancing complete removal of disease, optimal hearing outcomes, and minimized surgical burden in the pediatric patient. This review alerts physicians that BCC occurs, highlights past presentations and management strategies, describes the considerations in treatment and offers an algorithm helpful in the management of BCC.

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