Congenital intratympanic membrane cholesteatoma (ITMC) is a rare type of congenital cholesteatoma located within the tympanic membrane. This lesion tends to increase in size over time. The development of ITMC can cause several complications such as hearing impairment, dizziness, facial palsy, and intracranial complications, similar to any other cholesteatoma. The treatment of congenital cholesteatoma requires the removal of the lesion through surgery, because disease progression induces bony destruction of the nearby tissue. Most patients presenting with this cholesteatoma type are also treated with primary surgical removal. However, we recently experienced a case of an ITMC that showed a natural transition to an external auditory canal cholesteatoma.
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