Epidemiology

The annual incidence of cholesteatoma is reported as 3 per 100,000 children and 9.2 per 100,000 adults.[4]​ This varies both worldwide and with the population studied. Approximately 70% to 96% of cholesteatomas are acquired.[1]​ Congenital cholesteatoma is much less common, with incidence estimated at 0.12 per 100,000.[6]

Incidence may be affected by medical practice. The use of grommets or aural ventilation tubes in patients with chronic otitis media was associated with a fall in the incidence of cholesteatoma surgery in Finland and Israel.[7][8] However, no change was noted in the incidence of disease in Scotland and Ireland, with these treatments.[9][10]

There are fewer data on prevalence, but in Jerusalem it has been quoted as high as 70 per 100,000 children.[11] In Australian Aboriginal children, a prevalence of 50 per 100,000 in a study of 7362 ears has been reported.[12] Prevalence has been shown to vary according to race; it is highest among white Americans, followed by Africans, whereas it is rarely observed in non-Indian Asians. Prevalence is higher in under-developed countries than in developed countries; however, no difference has been observed among different socio-economic groups.[1]​ One Swedish case-control study using nationwide register data found that the risk of cholesteatoma was strongly associated with a family history of the condition, suggesting a possible genetic pre-disposition; family history was nevertheless quite rare and could therefore only explain a limited number of all cases.[13]​​

Males slightly outnumber females, with a ratio of 1.4:1. Among children, a 72% preponderance of boys has been reported.[1]​

In the paediatric population, cholesteatomas account for 10% of chronic otitis media cases. The mean age of children with acquired cholesteatoma has been estimated at 9.7 years. Approximately 7% to 10% of affected children have simultaneous bilateral cholesteatomas or develop subsequent contralateral cholesteatomas during follow-up; both of these conditions are more common in girls. It has been proposed that children with craniofacial syndromes are predisposed to develop cholesteatoma; an estimated 0.9% to 5.9% of children with a cleft palate develop primary acquired cholesteatoma.[1]​ 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 suggests that cholesteatoma in children tends to be more aggressive.[14]

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