To determine the locations of congenital cholesteatomas and to elucidate the relationship between ossicular anomalies and congenital cholesteatomas, congenital cholesteatomas from our patients and those reported in the Japanese literature were investigated.
We analyzed 40 ears with congenital cholesteatomas (38 patients) experienced by us during the past 9 years. Our criteria of congenital cholesteatoma were as follows: 1. The tympanic membrane manifests neither retraction, perforation, nor granulation. 2. There is no continuity between the tympanic membrane and the matrix of a cholesteatoma. Twenty patients with past histories of otitis media were included. Thirty-three patients (87%) were males and 2 had bilateral congenital cholesteatomas. The ages of the patients ranged from 2 to 46 years (mean: 10.7). A survey of the Japanese literature revealed 55 additional cases of congenital cholesteatoma reported by others.
Among this total of 95 congenital cholesteatomas in Japanese patients, 14 occupied the anterosuperior quadrant (ASQ) of the mesotympanum, 33 were located in the posterosuperior quadrant (PSQ) of the mesotympanum, and the remaining 44 including those in 4 ears with multicentric congenital cholesteatomas, were too extensive to identify the origin. This observation of the predominantly PSQ location of small congenital cholesteatomas is contrary to that reported in the United States, in which congenital cholesteatomas were usually found in the ASQ.
Another finding was the frequent association (17 ears) of presumed congenital anomalies of the ossicular chain (absence of the long process of the incus and/or the superstructure of the stapes) with a small congenital cholesteatoma in the PSQ and attic. It is also noteworthy that the association of ossicular anomalies was more common in membraneous cholesteatomas, the socalled open-type congenital cholesteatomas (13 ears), than in cystic cholesteatomas (4 ears). The present study revealed that small open-type congenital cholesteatomas occupying the PSQ and attic were frequently associated with ossicular anomalies.
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