The overall incidence is 7 per 100,000 children per year in children in Iowa,  but incidence varies both worldwide and with the population studied. In Scotland, an annual incidence of 13 per 100,000 of the population was noted for the period 1966 to 1986.  In Israel, the annual incidence of surgical treatment for cholesteatoma was estimated at 66 per 100,000.  A study from Denmark reports an annual incidence of surgical treatment for congenital cholesteatoma of 0.12 per 100,000.  Incidence may be affected by medical practice. Although in Scotland and Ireland no change has been noted in the incidence of cholesteatoma related to use of grommets or aural ventilation tubes in patients with chronic otitis media,   other countries such as Finland and Israel have shown a fall in the prevalence of the disease with these treatments.  
There are fewer data on prevalence, but in Jerusalem it has been quoted as high as 70 per 100,000 children.  In Australian Aboriginal children, a prevalence of 50 per 100,000 in a study of 7362 ears has been reported. 
Both males and females are affected, with a ratio of 3:2. Cholesteatoma in children has been found to affect the eustachian tube, anterior mesotympanum, retrolabyrinthine cells, and mastoid tip more than in adults. Clinical and histological evidence suggest that cholesteatoma in children tends to be more aggressive.