Cholesteatoma-an immune disease

Cholesteatoma is abnormal epithelial tissue in the middle ear cleft and an important cause of chronically discharging ears and hearing loss. Its proximity to important middle ear structures and the brain can lead to significant complications. Cholesteatoma presents both as congenital and acquired types and can occur both in children and adults. The mainstay of treating cholesteatoma is a destructive surgery with attendant morbidity. Despite radical surgery cholesteatomas can recur, sometimes repeatedly, causing a significant impact on quality of life. The role of inflammation in cholesteatoma is an area of keen interest. However, immune processes are highly complex, and while various individual components of the process have been studied in cholesteatoma, we are far from a cohesive understanding of the role of immune cells and inflammation pathways in the pathogenesis of cholesteatoma. Better understanding of its immune-pathogenesis would enable identification of disease-modifying therapies and novel pharmacological targets. In this review, we provide a detailed overview of the existent knowledge and recent developments on immune cells and inflammatory pathways implicated in cholesteatoma pathogenesis, progression, and damaging bone eroding properties. As cholesteatoma surgery is inherently destructive with poor hearing results, there is a definite necessity for new treatments to reduce the surgical burden and enable functional preservation in the management of this condition. We focus on the current and future areas of research that could lead to new therapies targeting inflammation and bring about a step change in the management of this aggressive ear condition.