Family cluster of cholesteatoma.
OBJECTIVE
We report an extremely rare case of family clustering of cholesteatoma.


METHODS
Case reports and a review of the world literature concerning cholesteatoma and heredity are presented.


RESULTS
The family consists of parents and seven siblings of whom the mother and three sons have been surgically treated for cholesteatoma. All cholesteatomas in the family are acquired and all have a history of otitis media. Cholesteatomas occur with an incidence of 5/100,000 in Greenland, corresponding to two to three new cholesteatoma patients per year among the 57,000 inhabitants of Greenland. The family is very exceptional and interesting for further research concerning heredity in the pathogenesis of acquired cholesteatoma.


CONCLUSIONS
To our knowledge this is the first report in the world literature of family clustering of acquired cholesteatoma. This case indicates that hereditary factors interplay with other factors in the pathogenesis of cholesteatoma.
Homøe
P
P
Department of Otolaryngology, Head & Neck Surgery, H:S Rigshospitalet, University Hospital of Copenhagen, Denmark. phom@rh.dk
Rosborg
J
J
D000293:Adolescent; D000328:Adult; D018424:Cholesteatoma, Middle Ear / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D016000:Cluster Analysis; D004777:Environment; D005260:Female; D020022:Genetic Predisposition to Disease; D006116:Greenland; D006801:Humans; D008297:Male; D010375:Pedigree