Abstract

 Cholesteatoma is a rare condition affecting 9-12.6 adults and 3-15 children per 100,000 per annum [1–4], with a more aggressive presentation in the paediatric population [5].
 Intermittent otorrhea (ear discharge) is the presenting complaint in over half of cholesteatoma patients [6, 7]. The peak incidence of cholesteatoma is 5-15 years of age [8] which overlaps significantly with a period of high incidence in otitis media [9] and externa [10], diseases that often present the same way as cholesteatoma. This results in diagnosis that may take several years.
 Left untreated, cholesteatoma can cause significant lasting damage in the form of deafness, vertigo, facial paralysis, meningitis, and brain abscesses which may prove fatal [11].
 Current treatment options are limited to surgical excision with the aim to establish a safe and manageable ear, while maintaining hearing is secondary. Improving surgical instrumentation has allowed a better success rate, however, revision surgeries remain a mainstay of practice. In practical terms, this means that those affected by bilateral disease often undergo surgery 4 or more times [12]. This represents a significant burden for patients.
 The decision about the exact surgical approach (canal wall up vs canal wall down) is a careful balancing act of safety versus functionality, and the pros and cons must be weighed in light of available evidence and the skill of the surgeon [13].
Highlights
Cholesteatoma is a rare disease with potentially life-threatening complicationsCholesteatoma is the non-cancerous locally invasive growth of keratinising squamous epithelium in the middle ear and mastoid air cells, where such tissue is not normally found[14]
While NICE does reference cholesteatoma as a differential diagnosis for a subset of conditions that have a significant overlap of symptoms, such as chronic middle ear infection, it does not mention it in others, like otitis media with or without effusion
Complete visualisation of the tympanic membrane, with particular attention to the attic and posterosuperior quadrant, is recommended for all patients with recurrent ear disease[27,57], as early detection of cholesteatoma is associated with better outcomes[17]
Summary
Cholesteatoma is a rare condition affecting 9-12.6 adults and 3-15 children per 100,000 per annum[1,2,3,4], with a more aggressive presentation in the paediatric population[5]. The peak incidence of cholesteatoma is 5-15 years of age[8] which overlaps significantly with a period of high incidence in otitis media[9] and externa 10, diseases that often present the same way as cholesteatoma. This results in diagnosis that may take several years. Improving surgical instrumentation has allowed a better success rate, revision surgeries remain a mainstay of practice. In practical terms, this means that those affected by bilateral disease often undergo surgery 4 or more times[12]. The decision about the exact surgical approach (canal wall up vs canal wall down) is a careful balancing act of safety versus functionality, and the pros and cons must be weighed in light of available evidence and the skill of the surgeon[13]
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