INTRODUCTION: Congenital cholesteatoma (CC) of the middle ear is a rare entity that may be undiagnosed for years. The lesion can grow undetected until it produces symptoms such as reduced hearing or otalgia.
METHOD: Case report.
RESULTS: We report two cases of young ladies with CC who presented with different otological symptoms. The first case complained of recurrent unilateral ear pain while the second case presented with unilateral reduced hearing. Examination of both cases revealed a whitish mass seen behind an intact tympanic membrane. Both cases underwent surgery and histopathological examinations; findings were consistent with cholesteatoma.
CONCLUSION: There are varieties of clinical presentations of CC and the diagnosis is based on clinical findings.
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