Please tell me about a disease called cholesteatoma and deafness. My son had surgery for cholesteatoma. The doctor "botched" it and my son is now totally deaf in his left ear. Is there any way to restore his hearing? He is only 12 years old.

C.N.

Did your 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 the surgery. I'll get back to this in a moment.

The term cholesteatoma is best defined as "skin growing in a place where skin normally does not grow." The middle ear (the cavity behind the eardrum) is the usual location for cholesteatoma, but I have heard of cholesteatomas arising within the facial sinus cavities and other parts of the body (usually as a result of previous penetrating trauma, such as a stab wound).

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 eardrum can become lower than that outside the eardrum. If this pressure difference is large and present for a long time, a portion of the eardrum can be sucked inward, forming a something called a "retraction pocket."

The outer part of the eardrum is skin, which constantly replaces itself as dead cells flake off the surface. If a person has a very deep retraction pocket, dead cells may become trapped within it. Skin debris can accumulate and fill the pocket. The skin lining the pocket continues to produce more dead layers of skin. A ball of skin debris forms and continues to grow, much as a ball of rubber bands grows larger as you add more and more rubber bands.

As the ball grows, the skin lining the pocket changes and 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 erode through the skull base. This can lead to meningitis (infection of the tissue covering the brain), 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 plan a first stage of operation (cholesteatoma removal) and a second stage (hearing restoration). Typically, the doctor allows six months to pass between the two stages. More often than not, hearing is poorer after the first operation than before surgery.

The surgery is done in stages 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 very nasty organisms. If the patient were to get a bacterial inner-ear infection, hearing would be lost permanently. Another reason to stage the operation is that a cholesteatoma may recur, and a recurrence could ruin the repair. At the second surgery 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 loss is permanent. I would still not assume that the surgeon botched the operation, since cholesteatomas are certainly capable of causing inner-ear damage on their own.