Cholesteatoma: Causes, Symptoms, and Diagnosis

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Cholesteatoma is a growth of excess skin or a skin cyst (epithelial cyst) that contains desquamated keratin and grows in the middle ear and mastoid (Thio, Ahmed, & Bickerton, 2005). A cholesteatoma can grow and spread, destroying the ossicles, tympanic membrane and other parts of the ear. They appear on the pars flaccida and pars tensa sections of the tympanic membrane. A cholesteatoma can occur when a part of a perforated tympanic membrane is pushed back into the middle ear space, debris and skin cells can build up forming a growth. It can obstruct tympanic membrane movement and movement of the ossicles. As the layers grow, the amount of hearing loss can increase. A cholesteatoma can be congenital (present at birth) or be acquired as a result of another disease. They can also be formed as a result of a surgery, trauma, chronic ear infection, chronic otitis media, or tympanic membrane perforation. It can develop beyond the tympanic membrane and cause intracranial and extracranial complications. Due to this patients can experience permanent hearing loss as a result of an infection of the inner ear as well as other serious health concerns. These include dizziness, facial nerve weakness and infections of the skull (Hall, 2013). Patients may present chronically discharging ear, hearing loss, dizziness, otalgia (ear pain), and perforations (marginal or attic).
Congenital cholesteatoma
Congenital cholesteatoma is relatively rare. It presents as a white mass behind an intact tympanic membrane in patients with no previous history of ottorhea, and no history of previous ear surgery (Isaacson, 2007). With this form of cholesteatoma eustacian tube dysfunction does not occur in the same manner as it does in acquired forms. Symptoms specific to the congenital form have been known to range from asymptomatic to conductive hearing loss, labyrinthitis, facial palsy, sensorineural hearing loss, and intracranial complications (Park, Park, Chang, Jung, & Yeo, 2009).
In the journal article, A Staging System for Congenital Cholesteatoma, the researchers aimed to develop a staging system for congenital cholesteatoma in order to determine an association between stage and residual disease. They defined four stages: Stage 1, single quadrant affected; stage 2, multiple quadrants affected, but not affecting the ossicles or mastoid extension; stage 3, ossicles are affected but not the mastoid extension; stage 4, mastoid disease. The creation of this staging system helps in standardizing the reporting of congenital cholesteatoma (Potsic, Samadi, Marsh, & Wetmore, 2002). Patients with higher stages of the disease have been shown to have worse postoperative hearing and a higher chance of having a cholesteatoma present during a second look follow up visit.

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