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Cholesteatomas are found in all age groups. Congenital cholesteatomas are usually found only in pediatric patients, whereas acquired cholesteatomas can be found in any individual with middle ear disease (1,2,3). Acquired cholesteatomas are divided into primary acquired and secondary acquired.
The average age for diagnosis of cholesteatoma is 9.7 years for acquired and 4.5 years for congenital (1). There exists a slight male preponderance for the disease. However, female patients are more likely to have bilateral disease and exhibit recurrence of disease in subsequent follow-up appointments (1). Overall, the incidence of acquired cholesteatoma is decreasing, secondary to the widely accepted and implemented early use of pressure equalization (PE) tubes for otitis media and other middle ear disease (1).
The prevalence of acquired cholesteatoma is approximately 15 cases per 100,000 children and 12 cases per 100,000 adults. By comparison, congenital cholesteatomas are rare only affecting approximately 0.12 per 100,000 children. Congenital cholesteatoma is becoming more commonly reported because recognition and screening of the disease has become more sensitive (1).
As described above, cholesteatoma can arise in various clinical situations. Cholesteatoma can arise as part of a constellation of symptoms in syndromic children (e.g., branchio-oto-renal syndrome). It can also stem from a disease process like otitis media with effusion. To date, there is no specific gene correlated with acquired cholesteatoma (4). Discussion of specific genetic contributions to syndromic processes associated with cholesteatoma is beyond the scope of this article.
As alluded above, acquired cholesteatoma is more prevalent in patients with long-standing middle ear disease. Chronic ETD causes negative pressure in the middle ear space leading to retraction of the tympanic membrane, accumulation of fluid in the middle ear space, predisposition for infection within accumulated fluid, and possible tympanic membrane perforation (1,2,3). Each of these disease states contributes in part to formation of an acquired cholesteatoma (either primary or secondary).
The primary treatment for ETD and hence cholesteatoma formation is primarily with PE tubes. Although perforation in the tympanic membrane is the primary mechanism of secondary acquired cholesteatoma, evidence supports placement of PE tubes for the treatment of ETD to prevent and potentially reverse the formation of retraction pockets in the tympanic membrane (1,2,3).