The Medical Consumer's Advocate

Cholesteatoma

 

The writer reports that her young son has had two operations for cholesteatoma; she states that the doctor "botched" the operation, and he is now completely deaf in the operated ear. She is seeking more information on cholesteatoma.

 

A: Did you son’s surgeon tell you that he botched the operation, or are you just assuming so, because your son’s hearing is worse now? Quite possibly, the surgeon botched nothing more than the explanation of surgery. I’ll get back to this in a moment.

Cholesteatoma is best defined as "skin growing in a place where skin normally does not grow." The middle ear (the cavity behind the ear drum) is the usual location for cholesteatoma, but I have heard of cholesteatomas arising within the facial sinuses and other parts of the body (usually as a result of previous penetrating trauma, such as a stab wound, which presumably forced a collection of skin cells into the sinus or body cavity.)

Middle ear cholesteatoma can arise in a number of ways. For example, some people have a condition called Eustachian tube dysfunction, in which the Eustachian tubes fail to appropriately ventilate the middle ear space. If the middle ear is poorly ventilated, the air pressure behind the ear drum can become lower than the "outside" air pressure, and if this pressure difference is large and persistent, a portion of the ear drum can be "sucked inward," forming a retraction pocket.

The outer part of the ear drum is skin. Skin constantly replaces itself; the outer layers of skin consist of old, dead skin cells. In the case of the ear drum, these dead cells flake off and eventually find their way out of the ear canal. If a person has a retraction pocket, these dead cells may become trapped within the pocket. This occurs if the pocket is very deep. Skin debris can accumulate and fill the pocket. The skin lining the pocket does not automatically "shut down," but continues to produce more dead layers of skin. A ball of skin debris forms and continues to grow, much as a "rubber band ball" grows larger as its owner adds more and more rubber bands.

As the ball of skin debris grows, the skin lining the retraction pocket changes, becoming less and less like normal ear drum skin. It begins exuding enzymes that destroy the tissue around it. This allows the ball to grow larger. Unchecked, growth of the ball can damage the middle ear bones, the inner ear, and the facial nerve. This can result in deafness (which, in the case of inner ear damage, can be permanent), dizziness (due to inner ear damage), and paralysis of the muscles of facial expression. The ball can continue to grow, eroding through the skull base. This can lead to meningitis, brain abscess, and death.

You may have guessed already that the ball of skin debris (along with its lining of abnormal skin) is a cholesteatoma. Since cholesteatomas are potentially deadly (and are, at the very least, extremely destructive), complete removal of the cholesteatoma is the ear surgeon’s first priority. Restoration of hearing is the second priority.

Did the surgeon botch the operation? Maybe not. Many ear surgeons intentionally stage the operation. In other words, there is a first stage (in which the cholesteatoma is removed) and a second stage (in which the hearing is restored.) Typically, the doctor will allow 6 months to pass between the two stages. More often than not, hearing is poorer after the first operation than it was preoperatively.

The operation is staged because repair of the hearing mechanism at the first operation can put the inner ear at risk of bacterial infection. This is because the cholesteatoma often harbors some very nasty organisms. If the patient were to get a bacterial inner ear infection, his hearing would be lost permanently. Another reason to stage the operation is that cholesteatomas may recur; recurrence could ruin the repair altogether. At the second stage, the surgeon will look for recurrent cholesteatoma and eradicate it.

Your son’s hearing loss may be due to the fact that he needs the second stage procedure. On the other hand, if his hearing loss is due to inner ear damage, then I am afraid the hearing loss is permanent. I would still not assume that the surgeon botched the operation, since cholesteatomas are certainly capable of causing inner ear damage.

 

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