Congenital cholesteatoma in the mastoid cavity

Congenital cholesteatoma may arise from various sites within the temporal bone including the petrous apex, the cerebellopontine angle, the mastoid, the middle ear and the external auditory canal. Of these, the mastoid cavity is the least frequent site of origin. Herein, we report an extremely rare case of congenital cholesteatoma of mastoid origin with extension into the epitympanum and presentation of conductive hearing loss. Computed tomography (CT) displayed anatomic anomalies including ossicular erosion, exposure of the dura, and dehiscence and exposure of the facial nerve. This patient was successfully treated with canal wall up mastoidectomy with ossicular reconstruction. The hearing threshold showed significant improvement, and there has been no recurrence during 18 month-follow-up. Copyright 2005