This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal

Kenneth M. Grundfast, MD; Gurpreet S. Ahuja, MD; Simon C. Parisier, MD; Stephanie M. Culver, MD

Arch Otolaryngol Head Neck Surg. 1995;121(8):903-907.

Abstract
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.

(Arch Otolaryngol Head Neck Surg. 1995;121:903-907)

Author Affiliations
From the Children's National Medical Center, Washington, DC (Drs Grundfast and Ahuja); Manhattan Eye, Ear, and Throat Hospital, New York, NY (Dr Parisier); and University of Minnesota Hospital and Clinics, Minneapolis (Dr Culver).

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

A Staging System for Congenital Cholesteatoma
Potsic et al.
Arch Otolaryngol Head Neck Surg 2002;128:1009-1012.
ABSTRACT | FULL TEXT  

The Natural History of Congenital Cholesteatoma
Koltai et al.
Arch Otolaryngol Head Neck Surg 2002;128:804-809.
ABSTRACT | FULL TEXT  

Congenital Cholesteatoma: Classification, Management, and Outcome
Nelson et al.
Arch Otolaryngol Head Neck Surg 2002;128:810-814.
ABSTRACT | FULL TEXT