Congenital cholesteatoma.

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.