WHAT IS A CHOLESTEATOMA?
Cholesteatoma is a growth of skin cells occurring behind the ear drum that causes damage to the ear drum itself, the middle ear bones and in some instances the inner ear, the facial nerve, the barrier between the brain and the ear, and even the blood vessels supplying brain structures. Cholesteatoma can lead to more serious problems including chronic ear infections, permanent hearing loss and dizziness. More rarely, it can lead to inability to move the facial muscles (facial nerve weakness / paralysis) and serious infections involving the brain including meningitis and brain abscesses. Cholesteatoma can be a silent and relentless process for years before symptoms advance. The tissue is destructive to normal structures but is not malignant in nature.
WHAT CAUSES CHOLESTEATOMAS?
There are two types of cholesteatomas: acquired cholesteatoma and congenital cholesteatoma. The most common type of cholesteatoma is acquired, meaning that the cholesteatoma grows over time in a previously healthy ear. In these cases, the problem starts with a hole in the ear drum or a retraction in the ear drum. Ear drum perforations can result from ear infections, trauma, or a long term difficulty in being able to equalize pressure in the middle ear (or “pop” the ears) when changing altitudes or when suffering from congestion. After the hole is created, the opportunity exists for healthy skin cells to move from the ear canal and begin growing behind the eardrum.
Some individuals have congenital cholesteatomas, meaning that they are born with the cholesteatoma growth already existing behind an intact ear drum. These cholesteatomas frequently go unnoticed for a long time as they may be difficult to see behind the ear drum. It is thought that congenital cholesteatomas arise from cells that get “misdirected” during fetal development. Instead of forming cells that normally occur in the middle ear, they form skin cells instead.
ISSUES CAUSED BY CHOLESTEATOMAS
As live skin cells continue to grow, the old cells slough off, or exfoliate. Exfoliation is a normal process for skin cells. On the body’s surface, the dead cells fall off and new skin cells replace them. However, behind the ear drum there is no place for the dead skin cells to go, and the dead skin cells that can’t leave the body become a big problem as the cholesteatoma forms a growing mass of dead and live skin cells behind the ear drum. As it grows, the cholesteatoma damages many structures in its path including the ear drum, middle ear bones and even bone overlying the brain, facial nerve and inner ear. Dead skin cells also provide a rich environment for bacteria and fungus to grow, leading to chronic infections.
Cholesteatomas can grow for long periods of time without any visible outward symptoms. When symptoms do occur, they commonly include hearing loss, intermittent or continuous drainage from the ear, build-up of dried crusts in the ear, acute and chronic infections, and pain. In extreme cases, symptoms such as facial weakness / paralysis, dizziness, complete deafness and neurological symptoms occur.
Cholesteatomas are typically diagnosed by a direct examination of the ear using a high powered microscope. Ear surgeons (Otologists) can examine the ear with a microscope and see the edge of the cholesteatoma from the ear canal. Specialized studies, including CT and MRI scans, may be used to examine more extensive cholesteatomas when determining a plan of treatment, but are not necessary to diagnose the problem in most cases.
Cholesteatoma treatment, at a minimum requires surgery to remove the growth. Antibiotics can decrease associated infections, but there is no medication that will cure cholesteatomas. Surgery results in a safe, healthy ear, free of disease, and can also give a secondary benefit of restoring the hearing lost due to the cholesteatoma. The procedure most commonly used to remove a cholesteatoma growth is called a tympanomastoidectomy. This outpatient surgery is completed in 1.5-2 hours with the patient going home the same day. For patients with moderate to large cholesteatomas, a two-stage series of surgeries is recommended. The first procedure is completed to remove the cholesteatoma growth and clean up any associated chronic infections. The second surgery, completed 6-12 months after the first, is a re-examination to make certain that no skin cells have re-grown. During the second surgery, damaged middle ear bones (ossicles) can be repaired, or if too damaged to repair, they can be replaced with titanium prosthetics known as PORPs or TORPs.
Cholesteatoma is difficult to diagnose without a comprehensive medical examination and specialty care from an Otologist, who is an otolaryngologist (ENT) who has undergone two additional years of training and specializes in hearing only. With appropriate medical treatment and therapy, individuals with cholesteatomas can fully function in a hearing world.
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