Congenital cholesteatoma: classification, management, and outcome.
OBJECTIVE
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.


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


METHODS
Four tertiary care children's hospitals.


METHODS
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.
Nelson
Marc
M
Section of Pediatric Otolaryngology, The Cleveland Clinic Foundation, 9500 Euclid Ave, Desk A71, Cleveland, OH 44195, USA.
Roger
Gilles
G
Koltai
Peter J
PJ
Garabedian
Erea-Noel
EN
Triglia
Jean-Michel
JM
Roman
Stephane
S
Castellon
Roberto J
RJ
Hammel
Jeffrey P
JP
D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000145:classification* / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D003638:Deafness / Q000209:etiology; D004427:Ear Diseases / Q000145:classification* / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012008:Recurrence; D016896:Treatment Outcome