The natural history of congenital cholesteatoma.

OBJECTIVES

To describe the natural history of congenital cholesteatoma (CC) and to determine whether such a description provides clues about the origins and end points of these lesions.

DESIGN

A retrospective qualitative analysis of intraoperative illustrations of 34 consecutive patients with 35 CCs (1 bilateral).

SETTING

Two tertiary care children's hospitals.

PATIENTS: Thirty-four children with CC, mean age, 5.6 years (range, 2-13 years).

RESULTS

Congenital cholesteatoma originates generally, but not universally, in the anterior superior quadrant. The progression of growth is toward the posterior superior quadrant and attic and then into the mastoid. Contact with the ossicular chain generally results in loss of ossicular continuity and in conductive hearing loss.

CONCLUSIONS

Congenital cholesteatoma appears to have a predictable trajectory of growth, starting as a small pearl in the middle ear, eventually growing to involve the ossicles and mastoid, and causing varying degrees of destruction and functional impairment. The clinical picture of a young child with otorrhea, conductive hearing loss, tympanic membrane perforation in a nontraditional location, and a mastoid filled with cholesteatoma may represent the end point in the natural history of CC, despite the fact that this type of lesion is outside the accepted definition of CC.