Cholesteatoma is a skin-lined cyst that begins at the margin of the eardrum and invades the middle ear and mastoid (arrow). This photograph shows a typical cholesteatoma that has eaten into the bone, wrapped around the incus (hearing bone), and collected layers of dead skin. The cholesteatoma has grown to fill the mastoid, and is much larger than what is seen here.

Cholesteatoma grows aggressively. Because it retains bacteria, it is a commonly becomes infected. This infection may not go away until the cholesteatoma is removed.

Cholesteatoma has the capacity to eat away bone. Bone erosion can lead to hearing loss by destroying the small hearing bones (malleus, incus, stapes) that carry sound in the middle ear.

Bone erosion can also lead to more serious complications by allowing the spread of infection outside the middle ear. This infection can:

• Form a bony abscess (mastoiditis)
• Cause labyrinthitis — an inner ear infection causing dizziness or deafness
• Cause facial nerve paralysis
• Spread to the space around the brain, causing meningitis or a brain abscess

While these complications are rare, they are very serious and can be prevented by removing the cholesteatoma as soon as possible.

Treatment

Although surgery is rarely urgent, once a cholesteatoma is found, surgical treatment is the only choice. Surgery usually involves a mastoidectomy to remove the disease from the bone, and tympanoplasty to repair the eardrum. The exact type of operation is determined by the stage of the disease at the time of surgery.

• Mastoidectomy: When an infection or cholesteatoma has grown into the mastoid (the bone behind your ear), we will open the bone to remove the disease. Sometimes this requires removing all the bony walls within your ear to create an open cavity. This process is called an "open cavity," "canal wall down," or a "radical mastoidectomy." Sometimes the bony partitions can be preserved, resulting in a canal wall up”, or “closed cavity” mastoidectomy.
• Tympanoplasty: This refers to repair of the eardrum and hearing mechanism. The eardrum is repaired with a graft of cartilage or fascia (the lining of the muscle behind your ear). Usually this type of repair is successful in closing holes in the eardrum permanently. If the small bones involved in hearing are damaged, we will try to repair them with natural bone or cartilage, or synthetic prostheses made of bone mineral. We use natural bone whenever possible.

Cholesteatoma surgery, which is delicate surgery performed under a microscope, usually takes 2 to 3 hours, and patients may go home the same day. It is very important to remove the disease completely, or it may grow back. The rate of re-growth is higher in children than adults. Because of the aggressive nature of cholesteatoma, we ask patients come in regularly for careful follow-up. Sometimes a second operation will be necessary

Surgical Options for Cholesteatoma

At Mount Sinai, we prefer to use a single-stage approach whenever possible. Because of differences in the extent of the disease and differences in the anatomy of patients' ears, we usually decide on the exact details of the operation at the time of surgery.

• Single-stage procedure: In a single-stage operation, we remove the disease completely and reconstruct the ear in the best manner possible. If we take down the canal wall, we often reconstruct the bone to avoid creating a large postoperative cavity. If we find a lot of inflammation and scarring, however, we might not be able to reconstruct your ear. In this situation, we can attempt a second-stage operation after about a year.
• Open vs. closed procedures: An open cavity ("canal wall down" mastoidectomy) decreases the risk of the cholesteatoma returning. However, patients will need to see the doctor once or twice a year to remove wax, and, will need to wear earplug when swimming. A closed cavity ("canal wall up") heals more like a normal ear but carries the risk that recurrent disease might be hidden behind the bone. This situation demands regular follow-up, and sometimes a second operation.

Hearing Status After Cholesteatoma Surgery

Patients with cholesteatoma usually have hearing loss prior to surgery. We try to reconstruct the hearing whenever possible. This usually results in hearing improvement, but not always. Inflammation and scar tissue can sometimes prevent a good hearing result. A second-stage operation can be attempted in the future when the ear is stable and there is no more disease.

A few patients with cholesteatoma will start out with very good hearing. We always attempt to preserve the hearing bones, but this is not always possible, so a partial hearing loss may result.

There is a small risk to the facial nerve with any type of surgery. Fortunately, in experienced hands, the risk of facial weakness is extremely low.

What Are the Results of Surgery?

Surgery results in the complete removal of cholesteatoma in the majority of cases. Hearing loss can often be restored to some degree. If the disease prevents effective hearing restoration, a smaller operation in the future may be the best way to improve your hearing.

Surgery is generally safe. However, as with any operation, there are risks as well as benefits. The most common risks are:

• Recurrence of the cholesteatoma
• Hearing loss or failure to improve hearing
• The need for more than one operation

Even in the best of hands, cholesteatoma may recur, and additional surgery may be necessary in the future.

Recurrence of Cholesteatoma

Recurrent cholesteatoma can occur even in the most experienced surgeon's hands. This is because cholesteatoma is an aggressive disease. Recurrence comes in two forms: the first is when a tiny fragment of cholesteatoma lining is left behind ("residual cholesteatoma"), that re-forms a new ball of skin behind the eardrum. The second is when a new cholesteatoma forms in the middle ear or mastoid cavity (a true "recurrence").

Recurrence is usually detected in the office, which is why regular follow-up is so important. Occasionally it will cause a drop in hearing or drainage from the ear. If recurrence is detected early, we may be able to manage it in the office. If the cholesteatoma has reached a significant size, though, we will need to perform surgery (revision mastoidectomy). In our recent experience, revision surgery was needed in only 15% of the cases in which the original operation was performed at Mount Sinai. This rate is better than most centers, and much better than surgeons who routinely use a two-stage management philosophy.