Cholesteatoma

What is it?

Cholesteatoma is a disease of the ear also known as epidermoid tumor, epithelial cyst, or simply put, skin in the wrong place. In adults, cholesteatoma is usually secondary to longstanding ear disease related to poor ventilation (eustachian tube dysfunction), and repeated ear infections. Sometimes it is a consequence of a perforated eardrum or a ventilating tube insertion. Congenital growth of cholesteatoma is unusual in an adult.

How does it grow?

The growth of a cholesteatoma usually begins as a “retraction pocket” or a skin inversion from a hole in the ear drum (invagination). This pocket has the appearance of an entrapped cyst that grows slowly like a benign tumor, yet it is not defined as such. In its early stage, a cholesteatoma envelops the little hearing bones in the ear (ossicles) with gradual erosion leading to hearing loss. As the disease expands, it begins to invade other structures in the ear that would produce the various complications associated with this condition.

How does it present?

Cholesteatoma is slow growing and often goes unnoticed for several years before it is diagnosed. The common presenting features are:

• Hearing loss: this is mostly a mechanical loss (conductive) but not infrequently also a nerve loss (sensorineural) if the inner ear is involved
• Heaviness and fullness in the ear
• Tinnitus: a noise in the ear such a ringing, buzzing, etc.

In advanced disease, more serious features are found, such as:

• Dizziness (vertigo) from inner ear invasion of the semi-circular canal that controls one’s rotational balance function.
• Facial paralysis: This occurs when the facial nerve is invaded resulting in drooping of the face on one side.
• Mastoiditis: This is a serious bacterial infection of the bone surrounding the ear that requires urgent surgical treatment
• Meningitis

When mastoiditis occurs, the brain, its lining and the brain fluid could become infected resulting in a neurologic emergency.