RESUMO
OBJECTIVE: We present an extremely rare case of congenital cholesteatoma in identical twins. METHOD: Case report of congenital cholesteatoma in identical twins, and a literature review, are presented. RESULTS: Both cases presented to the ENT out-patient clinic, but with different clinical pictures. Both were managed surgically. CONCLUSION: Congenital cholesteatoma presents as a whitish mass in the middle ear, with an intact tympanic membrane. It is a rare entity comprising between 3.7 and 24 per cent of total cholesteatoma cases. The key to its diagnosis is the absence of previous ear infection, ear surgery and local trauma. To our best knowledge, this paper represents the first report in the world literature of congenital cholesteatoma in identical twins.
