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Classification, Management, and Outcome

Marc Nelson, MD; Gilles Roger, MD; Peter J. Koltai, MD; Erea-Noel Garabedian, MD; Jean-Michel Triglia, MD; Stephane Roman, MD; Roberto J. Castellon, MD; Jeffrey P. Hammel, MS

Arch Otolaryngol Head Neck Surg. 2002;128:810-814.

Objectives  To assess whether a classification system for congenital cholesteatoma (CC) can be derived from analysis of a large clinical sample of cases and to assess whether such a classification system is a reliable guide for surgical intervention, reexploration, and hearing outcome.

Design  A retrospective review of clinical and surgical records of 119 patients with CC.

Setting  Four tertiary care children's hospitals.

Patients  One hundred nineteen children with CC (age range, 2-14 years).

Results  Congenital cholesteatomas in the anterior mesotympanum were treated successfully with exploratory tympanotomy. Congenital cholesteatomas involving the posterior superior quadrant and the attic usually had concurrent involvement of the incus and stapes and often required a canal wall up tympanomastoidectomy and a second look for its control. Congenital cholesteatoma involving the mastoid usually involved all of the ossicles, was inconsistently controlled with canal wall up tympanomastoidectomy, and had a poor prognosis for restoration of conductive hearing loss. The mean ± SD age of children with CC was 5.6 ± 2.8 years, while that of children with acquired cholesteatoma was 9.7 ± 3.3 years.

Conclusions  The sequence of spread of CC, involving 3 sites, suggests a natural classification system. The CC usually originates in the anterior superior quadrant, but does not consistently remain there, and may variably occupy the middle ear and mastoid and result in ossicular destruction and conductive hearing loss. The location of CC and the involvement of the ossicles is an accurate predictor of the type of surgery necessary for its control and for the success of hearing restoration.

From the Section of Pediatric Otolaryngology (Drs Nelson and Koltai) and Department of Biostatistics and Epidemiology (Mr Hammel), The Cleveland Clinic Foundation, Cleveland, Ohio; Children's Hospital Armand Trousseau, Paris (Drs Roger and Garabedian), and La Timone Children's Hospital, Marseille (Drs Triglia and Roman), France; and the Division of Otolaryngology, Albany Medical College, Albany, NY (Dr Castellon).

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RELATED ARTICLE

The Natural History of Congenital Cholesteatoma
Peter J. Koltai, Marc Nelson, Roberto J. Castellon, Erea-Noel Garabedian, Jean-Michel Triglia, Stephane Roman, and Gilles Roger
Arch Otolaryngol Head Neck Surg. 2002;128(7):804-809.
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The Natural History of Congenital Cholesteatoma
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Arch Otolaryngol Head Neck Surg 2002;128:804-809.
ABSTRACT | FULL TEXT