Signs and symptoms

The majority (98%) of patients with cholesteatoma have ear discharge or hearing loss or both in the affected ear.
Other more common conditions, such as otitis externa, may also present with these symptoms, but cholesteatoma is much more serious and should not be overlooked. If a patient presents to a doctor with ear discharge and hearing loss, the doctor should consider the patient to have cholesteatoma until the disease is definitely excluded.
Other less common symptoms (all less than 15%) of cholesteatoma may include: pain, balance disruption, tinnitus, ear ache, headaches and bleeding from the ear. There can also be facial nerve weakness. Balance symptoms in the presence of a cholesteatoma raises the possibility that the cholesteatoma is eroding the balance organs, which form part of the inner ear.
On initial inspection, an ear canal full of discharge may be all that is visible. Until the doctor has cleaned the ear and inspected the entire tympanic membrane, cholesteatoma cannot be either confirmed or excluded.
Once the debris is cleared, cholesteatoma can give rise to a number of appearances. If there is significant inflammation, the tympanic membrane may be partially obscured by an aural polyp. If there is less inflammation, the cholesteatoma may present the appearance of 'semolina' discharging from a defect in the tympanic membrane. The posterior and superior parts of the tympanic membrane are most commonly affected. If the cholesteatoma has been dry, the cholesteatoma may present the appearance of 'wax over the attic'. The attic is just above the ear drum.
The patient may commonly also have clinical signs of conductive hearing loss. Less frequently, there may be signs of imbalance or facial weakness.
If untreated, a cholesteatoma can eat into the three small bones located in the middle ear (the malleus, incus and stapes, collectively called ossicles), which can result in nerve deterioration, deafness, imbalance and vertigo. It can also affect and erode, through the enzymes it produces, the thin bone structure that isolates the top of the ear from the brain, as well as lay the covering of the brain open to infection with serious complications (rarely even death due to brain abscess and septicemia).
Both the acquired as well as the congenital types of the disease can affect the facial nerve that extends from the brain to the face and passes through the inner and middle ear and leaves at the anterior tip of the mastoid bone, and then rises to the front of the ear and extends into the upper and lower face

https://en.wikipedia.org/wiki/Cholesteatoma

ETymology: http://www.sciencedirect.com/science/article/pii/S1879729610000505
Cholesteatoma is a severe middle-ear pathology affecting both adults and children. It would seem to have been first described by De Verney in France in 1683, as what he called “steatoma” [1]. In 1829, the French anatomopathologist Cruveilhier described it as a pearl tumor of the temporal bone [2]. The term “cholesteatoma”, introduced in 1838 by the German physiologist Johannes Müller [3], is now well established, but in fact, etymologically faulty in as much as this benign tumor (“…oma”) contains neither cholesterol (“chole”) nor fat (“steat”). In 1855, Virchow categorized cholesteatoma under epidermoid carcinoma and atheroma, but only in 1861 did Von Troeltsch consider its epidermal origin. In the studies by Gruber, Wendt and Rokitansky (1855–1888), the physiopathology of cholesteatoma was described as a malpighian metaplasia of the middle-ear mucosa in response to chronic inflammation. At the end of the 19th century, Bezold and Habermann overturned this theory, showing that cholesteatoma was caused by migration of external auditory canal skin to the middle ear, induced by chronic inflammation [4]. At the present time, despite numerous investigations, the physiopathology of cholesteatoma has still not been elucidated.