Dr. Mark Levenson removes a congenital cholesteatoma.
Some cholesteatomas which are very small can be removed through the ear canal. Most cholesteatomas require that an incision be made behind the ear to expose the tumor adequately. The cholesteatoma is completely removed microscopically.
Hearing reconstruction is often delayed because it is necessary to rebuild the bones of hearing at a future date.
In more extensive cholesteatomas, the tumor may have eroded through the bony wall which separates the middle ear from the mastoid. This may require a more radical operation, removing the wall separating the middle ear from the mastoid.
In less extensive cholesteatomas, especially when infection is well controlled before surgery, an intact canal wall operation is the favored procedure. This operation preserves the wall between the middle ear and mastoid. The principal advantages of the intact canal wall operation are a more normal canal and ear drum, and a greater possibility of hearing restoration. In addition, most patients with the intact canal wall operation can allow water in the ear.
With an intact canal wall, regrowth of cholesteatoma may be completely unnoticed until it becomes very large. Thus, many ear surgeons will delay rebuilding the bones of hearing for a year after an intact canal wall operation for cholesteatoma. The ear drum is opened at the second operation and the bones of hearing are then reconstructed. If a regrowth of cholesteatoma is found, the tumor is again removed and reconstruction may be delayed for another 6 months or a year. Repeat CAT scans may also be performed in some cases to avoid further surgery.
Even with careful microscopic surgical removal of cholesteatoma, 10% to 20% of cholesteatomas can recur. In children, some ear surgeons report up to 50% recurrence rates with the intact canal wall procedure. Thus, careful follow-up visits must be planned, in order to identify regrowth early on.
The majority of cholesteatomas especially in adults, are acquired. They are a result of severe infections from perforations of the ear drum or are the result of chronic middle ear problems.
Also, the ear drum can be progressively pulled down and retracted into the middle ear space. The skin of the ear drum then grows inside the middle ear and mastoid to form a cholesteatoma. Less commonly, cholesteatomas may form congenitally.
These congenital cholesteatomas grow from birth behind the ear drum and, as they enlarge, may cause no symptoms until they become quite large. Congential cholesteatomas are often discovered by the pediatrician at the time of a routine visit.
They appear as a white ball behind the ear drum. The child is then referred to an ear specialist and the diagnosis can be confirmed by examining the ear drum with the surgical microscope. A CAT scan is also useful in confirming a congenital cholesteatoma, as well as a hearing test.
Congenital cholesteatomas, when small, can often be removed without separating the bones of hearing. Larger congential cholesteatomas may require extensive surgery including an open cavity operation. Large congenital cholesteatomas can be very deep in the inner ear. These usually present extensive hearing loss and facial weakness on the side of the cholesteatoma. Fortunately, these are quite rare.
As acquired or congential cholesteatomas increase in size, they can also affect the nerve that moves the face, the facial nerve. This nerve extends from the brain to the face by going through the inner ear, the middle ear, exiting near the forward tip of the mastoid bone, rising up to the front of the ear, and finally branching into the upper and lower face. Infected cholesteatomas may erode the bone covering this nerve. Pressure or irritation by the cholesteatoma on the facial nerve may then result in facial weakness or actual paralysis of the face on the side of the involved ear. In this case, ear surgery may be necessary on an emergency basis to prevent permanent facial paralysis.
Microsurgical removal of cholesteatoma is readily accomplished once infection of the ear has been cleared up. In many cases, infection can take six weeks or more to clear.
In congential cholesteatomas, the ear drum is intact and there is no infection present, so the surgery can be performed more quickly. The ear surgeon will generally discuss the removal of these kinds of cholesteatomas and will indicate that surgery is being performed first to remove the tumor completely from the ear and mastoid.
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