Cholesteatoma is a rare condition that a cyst-like growth develops in the ear. It may be a congenital problem (birth defect), but usually occurs as a complication of chronic ear infection (long). The most common symptoms are loss of hearing and a foul-smelling discharge from the ear. It is a malignant (cancer) condition, but it is important because it can lead to serious complications such as permanent deafness and serious illnesses such as meningitis.

On this page

• What is the ear and how we hear?
• What is a cholesteatoma?
• What causes a cholesteatoma?
• How common is cholesteatoma?
• What are the first symptoms of a cholesteatoma?
• What are the possible complications and why is it important?
• How is it diagnosed cholesteatoma?
• Do I need any tests?
• What is the treatment for cholesteatoma?
• Is any of the measures taken?
• What is the prognosis (outlook)?
• More help and advice
• References

What is the ear and how we hear?

The ear is divided into three parts – the outer (external) ear, the middle ear and the inner ear. The middle ear behind the eardrum (tympanic membrane) is filled with air. Air comes from the back of the nose of a thin channel called the Eustachian tube. In the middle ear are three tiny bones (ossicles) – Malleus's, anvil and stirrup (also known as the hammer, anvil and stirrup). The inner ear contains the semicircular canals and cochlea.

Sound waves enter the outer (external) ear and hit the eardrum. The sound waves cause the eardrum to vibrate. The sound vibrations from the eardrum pass the ossicles. The ossicles then transmit the vibrations to the cochlea in the inner ear. The cochlea converts the vibrations to sound signals that are sent by the nerves of the ear to the brain, allowing us to hear.

The semicircular canals of the inner ear contains a fluid that moves as you move into different positions. The fluid motion is detected by tiny hairs in the semicircular canals that send messages to the brain via the auditory nerve to help maintain balance and posture.

What is a cholesteatoma?

Cholesteatoma is the name given to a set of skin cells in the ear forming a mass (lump). It is a pearl white oily mass that can be seen by examining the ear.

What causes a cholesteatoma?

The cause is not fully understood. It is believed that cells in the skin of the ear canal wall are trapped in the middle ear (which normally contains no skin cells). Skin cells, including those lining the ear canal, normally multiply regularly to replace those who have died. Normally, these cells flake off the skin. The dead cells are trapped and accumulate too. This accumulation of dead skin cells over time is what forms the cholesteatoma.

There are two types of cholesteatoma

• Congenital Cholesteatoma behind the eardrum grows from birth. It is believed that some skin cells develop in the wrong place at the hearing and develop a cholesteatoma.
• Acquired cholesteatoma develops later, usually in adults. This is often the result of an ear infection chronic (long-term) or recurring. Infection causes a blockage of the Eustachian tube. This is the tube that connects the middle ear to the back of the nose and throat. The blockage creates a negative (suction) pressure draws into the eardrum. This can result in a small pocket formed, usually at the top of the tympanic membrane. In this pocket some skin cells collect, get caught, but multiply to form cholesteatoma. The pocket is too deep to allow dead skin cells to escape, so that gradually expands cholesteatoma.

How common is cholesteatoma?

Cholesteatoma is rare. The true incidence (occurrence) rate is not known. About 1 in 1,000 people with hearing referred to ear, nose and throat (ENT) clinics have cholesteatoma. It has also been suggested that there is about 1 case per 10,000 population. The majority of cases are of acquired type.

What are the first symptoms of a cholesteatoma?

Cholesteatoma is usually unilateral (one ear). The most common initial symptoms are a smelly discharge from the ear, and hearing loss. You are also likely to have had previous problems with ear infections. Other symptoms that may occur include tinnitus (ringing in the ear) and headache.

What are the possible complications and why is it important?

If untreated, a cholesteatoma gradually grow and expand. As it grows, it can erode (a) and destroy everything in its path.

Therefore, the possible complications that can occur over time are:

• The damage and eventual destruction of the ossicles (small bones of the ear). If they are damaged, permanent hearing loss can occur.
• Damage to the mastoid bone. This is the knot can feel thick bone behind the ear. The mastoid bone is normally filled with air bags (a bit like a honeycomb). Cholesteatoma can grow in the mastoid bone causing infection and destruction.
• Damage to the cochlea and other inner ear structures. This can cause permanent deafness on that side, and / or dizziness and balance problems.
• Damage to the nerves that travel near the face. This can cause paralysis (weakness) of some of the facial muscles.
• Cholesteatoma is often infected and the infection can spread to other body parts nearby. In rare cases a cholesteatoma can erode through the skull near the ear and brain. As a result of the spread of infection, conditions such as meningitis and brain abscesses may develop. These conditions can cause death.

Cholesteatoma not malignant (cancer) and does not extend to distant parts of the body.

How is it diagnosed cholesteatoma?

The ENT doctor may suspect or cholesteatoma based on typical symptoms. When examining the ear with an otoscope (torch), cholesteatoma can be seen. Often there is a perforation (hole) in the eardrum also.

Do I need any tests?

Audiometry (hearing tests) may show deafness or hearing loss is usually done in a hospital clinic. Swabs of the ear discharge may also be taken. The discharge often contains a bacterium (germ) called Pseudomonas is responsible for the smell. CT scans may be needed to see the extent of damage caused by cholesteatoma and to plan treatment.

What is the treatment for cholesteatoma?

Surgery is usually recommended to remove the cholesteatoma and prevent further damage. You may be given antibiotics to treat any infection and the ear dry, before surgery.

Surgery

Surgery removes all diseased areas, including cholesteatoma itself.

Tympanomastoidectomy This is the elimination of the tympanic membrane and the entirety or part of the mastoid bone of the skull, depending on the extent of cholesteatoma. It is often the procedure is performed and a large cavity (hole) is created in the region of the inner ear that requires regular cleaning and dewaxing (removing the wax) in the ENT clinic. This procedure is the most successful in the removal of all cholesteatoma. Complications of surgery can sometimes occur. These include deafness, dizziness and facial nerve damage, leading to paralysis of the facial muscles on the affected side.

Tympanoplasty This is another operation which can be performed in the eardrum. It is less invasive (closed) with a better technique cosmetic (visual) appearance after surgery. However, it is associated with increased risk of recurrent cholesteatoma, and an additional operation is required to check up one year later.

Nonsurgical treatment

If you are not fit enough to undergo general anesthesia or unwilling to undergo surgery, you may cholesteatoma treated in the best way possible with the help of "aural toilet. This involves flushing the ear canal and sucking waste (bits) using a microscope. This has to be done regularly and ultimately not prevent cholesteatoma growth.

Is any of the measures taken?

If you have had cholesteatoma, which will be followed up for life in an ENT clinic. You will need to have their ears cleaned regularly in clinic to remove wax and dirt that has accumulated. The specialist will need to ensure that the cholesteatoma has not returned. If the hearing starts the download again, additional surgery may be required. MRI is increasingly used to replace the need for further surgery checkup.

What is the prognosis (outlook)?

This depends on how much damage has been caused by the cholesteatoma at the time it is detected and treated. It is also affected by the fact that complications such as meningitis or deafness occurred. Surgery was performed before and attend regular monitoring, the greater the likelihood of a good result.

More help and advice

Deafness Research UK

330-332 Gray Inn Road, London, WC1X 8EE
Tel: 0808 808 2222 Text: 020 7915 1412 Web: www.deafnessresearch.org.uk
Provides an information line for all medical aspects of deafness and hearing loss.