Abstract

Congenital Cholesteatomas have historically been considered a rare disorder, an incidence ranging from 4 to 24%. The most common sites of presentation on physical examination are Anterior superior and posterior superior quadrants of the tympanic membrane; conductive hearing loss is the most common presenting symptom. There is a lack of uniformity of reporting and classifying congenital cholesteatomas. Recidivism of the lesion appears more commonly with posterior superior congenital cholesteatomas. Treatment of Congenital Cholesteatoma is still surgical. We are here presenting a case of isolated congenital cholesteatoma in Mastoid process treated with modified radical mastoidectomy and tympanoplasty, patient followed up to one year no recurrent was found, hearing also improved, heightened awareness and early diagnosis of congenital cholesteatoma is imperative, early treatment decreases the extent of the disease and reduces the risk of recidivism and complications.

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