Abstract
Objective: We report a rare case of family clustering of cholesteatoma. Method: Case reports of three first-degree relatives in a family affected with cholesteatoma and a review of world literature on hereditary causes for cholesteatoma are presented. Results: The family consists of parents and two siblings, of whom the father and both siblings (a daughter and a son) were surgically treated for cholesteatoma. All cholesteatomas in this family cluster were acquired. Conclusion: Family clustering of acquired cholesteatoma is rare. This report indicates the interplay of hereditary factors along with environmental factors in the genesis of cholesteatoma.
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