Korean J Otolaryngol-Head Neck Surg.  2007 Feb;50(2):169-173.

A Case of Congenital Cholesteatoma : Combined with Ossicular Anomaly

  • 1Department of Otolaryngology and Head and Neck Surgery, College of Medicine, Kyung-Hee University, Seoul, Korea. otorhino512@naver.com


Congenital middle ear cholesteatoma is presented as a pearl-like-material behind an intact tympanic membrane. Congenital cholesteatoma can be associated with other congenital anomalies, especially ossicular anomalies. The patient was a 10-year-old male with incidentally known hearing disturbance with intact tympanic membrane. The pure tone average through air conduction of his left ear was 55dB. During exploratory tympanotomy, congenital cholesteatoma was found in the middle ear cavity in a scattered pattern and fibrous stapes was located below the oval window with an intact stapedial footplate. We removed cholesteatoma matrix and performed ossiculoplasty with total ossicular replacement prosthesis (TORP). After 1 month, the postoperative pure tone average through air conduction of the left ear was 33dB. We report one case of congenital middle ear cholesteatoma with congenital ossicular anomaly with a review of the related literature.


Congenital; Cholesteatoma; Middle ear; Ear ossicles
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