Objective: To analyze clinical presentation, modes of detection, growth pattern, operative findings, and results of surgery in children 3 years old or older who had extensive congenital cholesteatoma (keratoma).
Design: Survey, case series.
Setting: Two academically affiliated medical centers: a children's hospital and an eye, ear, and throat hospital, both located in major metropolitan cities.
Patients: Twenty-five children selected according to specified criteria.
Intervention: Tympanomastoid surgery, ie, canal wall up and canal wall down, some with ossicular reconstructive surgery.
Main outcome measure: Audiologic assessment (speech reception threshold) and recurrence (recidivism) of cholesteatoma.
Results: Incidence of recidivism, 52%. Hearing maintained within the range of normal to mild hearing impairment postoperatively in 91% of the patients for whom complete data are available.
Conclusions: Congenital cholesteatoma may grow for years without causing signs or symptoms and, having grown without early detection, can extend to involve the epitympanum and mastoid antrum, cause ossicular erosion, and even extend to the middle cranial fossa. To adequately remove a congenital cholesteatoma that has gone undetected for many years, exposure of the anterior epitympanum is often necessary and removal of both the body of the incus and the head of the malleus often is required. Since congenital cholesteatoma usually develops in a child with a well-pneumatized mastoid that would create a large mastoid bowl if exteriorized, the otologic surgeon is likely to hesitate in using the canal wall down mastoidectomy technique. Alternatives to the canal wall down mastoidectomy technique, which can achieve reasonably good hearing results and avoid creation of a large mastoid bowl, include planned two-stage canal wall up surgery or canal preservation with primary reconstruction and close follow-up with otomicroscopy and serial computed tomographic scans.