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January 1995

Congenital Cholesteatoma

Author Affiliations

From the Chicago Otology Group, Hinsdale, Ill; and the Department of Otolaryngology—Head and Neck Surgery, Northwestern University Medical School, Chicago, Ill.

Arch Otolaryngol Head Neck Surg. 1995;121(1):19-22. doi:10.1001/archotol.1995.01890010007002

Objective:  To review the characteristics of congenital cholesteatomata.

Design:  Case series.

Setting:  Tertiary care (referral-based) private practice.

Patients and Other Participants:  Fourteen patients were included in the study. The diagnosis of congenital cholesteatoma was based on an intact tympanic membrane on physical examination; a history that excluded tympanic membrane perforation, otorrhea, or previous otologic procedure; and a documented cholesteatoma at the time of surgical removal.

Intervention:  Surgical procedures including tympanotomy, atticotomy, and tympanotomy with mastoidectomy were performed on all patients.

Main Outcome Measures:  Removal of cholesteatoma.

Results:  Four of the 14 patients had lesions isolated to the anterosuperior quadrant of the tympanum; the remainder had more extensive disease with notable posterior tympanic involvement. Three of the patients underwent surgery for recidivism; none were from isolated anterior lesions. One of these patients was referred at the time of recurrence, one had known residual cholesteatoma, and one had recurrence.

Conclusions:  Clinical presentation and surgical findings enable the differentiation of two separate sites of congenital cholesteatoma formation: the anterosuperior and posterior-superior regions of the tympanic cavity. Recidivism of the lesion appears more commonly with posterior-superior congenital cholesteatomas.(Arch Otolaryngol Head Neck Surg. 1995;121:19-22)