Vestibular disease

Cholesteatoma

This information is intended as a general introduction to this topic. As everyone is affected differently by balance and dizziness problems, you should speak to your doctor for individual advice.

For reasons of better readability, the generic masculine is used and the simultaneous use of the language forms male, female and diverse (m/f/d) is dispensed with. Unless otherwise indicated, the personal designations used in this patient information refer to all genders.

What is a cholesteatoma?

A cholesteatoma is a benign (non-cancerous) growth in the middle ear behind the eardrum (tympanum). Simply put, it's skin in the wrong place. People with a cholesteatoma usually have a foul-smelling discharge from the ear. A cholesteatoma can cause conductive hearing loss. In advanced disease, it can spread to the inner ear and cause dizziness. Most cholesteatomas require surgery. Cholesteatomas are sometimes called keratomas.

Cholesteatomas grow slowly. In the early stages, a cholesteatoma can become so large that it encloses and damages the three tiny ear bones, causing conductive hearing loss. Hearing loss can also occur if the cholesteatoma blocks the opening of the Eustachian tube, causing fluid to build up in the space behind the eardrum (middle ear effusion).

Cholesteatomas are very rare, cannot be inherited and usually only one ear is affected. Each year, about 3 in 100,000 children and about 9 in 100,000 adults are diagnosed with cholesteatomas. Men are slightly more likely to be affected by cholesteatomas.

The word cholesteatoma comes from the Greek and means “fat” and “tumor”. However, the term is misleading; a cholesteatoma does not contain fat and is not a tumor.

Summary

What causes a cholesteatoma?

The cause of a cholesteatoma depends on its type:

  • A congenital cholesteatoma occurs when small pieces of skin become lodged behind the eardrum in the middle ear before birth. It is the least common form of cholesteatoma.
  • A primary acquired cholesteatoma results from a malfunction of the Eustachian tube. It is the most common type of cholesteatoma. When functioning normally, the Eustachian tube passes air from the back of the nose into the middle ear to equalize the pressure in the ear. Sometimes one of the Eustachian tubes does not work properly. This can be caused by allergies, sinusitis, or middle ear infections (otitis media). Reduced pressure in the ear can cause the eardrum to collapse and stretch (retract) inward. When this happens, a pocket-like cyst filled with dead skin cells can form.
  • A secondary acquired cholesteatoma occurs when skin from the outer part of the eardrum grows through a hole in the eardrum. This usually happens after a chronic middle ear infection, an injury, or as a result of a minor surgical procedure to insert ventilation tubes in the ear. Timely and thorough treatment of recurrent middle ear infections can help prevent this type of cholesteatoma.

What are possible risk factors?

Science does not fully understand the risk factors for the development of a cholesteatoma. Known risk factors include chronic middle ear infections and a dysfunction of the Eustachian tube. Studies suggest that a cleft palate or osteoporosis may also play a role.

Cholesteatomas grow slowly. There may be no symptoms for the first few years. The characteristic symptom of an acquired cholesteatoma is a foul-smelling discharge from the ear (otorrhea).

  • Other common early symptoms include:

    • Gradual conductive hearing loss in the affected ear (initially in secondary acquired cholesteatomas and later in primary acquired cholesteatomas).
    • A feeling of pressure in the ear (aural fullness).
    • Pain or numbness in or around the ear.

    See your doctor if you have any or all of these symptoms.

If left untreated, a cholesteatoma can spread to the inner ear over time. Complications at this stage may include:

  • Persistent ear discharge.
  • Vertigo caused by damage to one of the semicircular canals.
  • tinnitus.
  • A failure of the facial nerves, which leads to facial paralysis.

In very rare cases, a cholesteatoma can gradually destroy the temporal bone and attack the brain. This is a life-threatening complication. Urgent surgery is required if any of the following situations occur:

  • Mastoiditis - bacterial infection of the mastoid bone (large bony area at the base of the skull behind the ear that is connected to the middle ear).
  • Brain abscess - a collection of pus and other material in the brain.
  • Meningitis - an infection of the membranes that cover the brain and spinal cord.

The symptoms of congenital cholesteatoma are slightly different. Usually, the eardrum looks normal and is intact. Children often do not have a history of purulent ear infections, previous ear surgery, or a perforated eardrum. The most common symptom is hearing loss.

Diagnosis of a cholesteatoma

  • A cholesteatoma can be diagnosed by a doctor who specializes in vertigo, such as an ENT doctor. The doctor will take a thorough medical history, perform a neurological exam, and various tests to assess the function of your vestibular system. They will also examine your ear with an otoscope (an instrument that can look inside the ear). It can be difficult to confirm a cholesteatoma by looking inside your ear. When pus collects in the ear, a cholesteatoma is often difficult to detect.

You will probably have some of the following diagnostic tests:

  • An audiogram to determine the extent of conductive hearing loss.
  • Tympanogram to determine the condition of the eardrum and middle ear.
  • CT (computed tomography) scan to examine the inner ear and mastoid bone (the bone behind the temporal bone, just behind the inner ear). CT scans can detect very small defects in the bones.
  • VEMP test - usually the affected side does not respond to this test. It is rarely performed when there is no doubt that the patient has a cholesteatoma.
  • MRI (magnetic resonance imaging) in advanced cases when the doctor suspects that the cholesteatoma has spread into the skull.

A congenital cholesteatoma is usually not diagnosed until the child is 2 to 3 years old and hearing loss is detected.

Early diagnosis is important to achieve the best outcomes for all types of cholesteatomas.

Treatment of Cholesteatomas

Therapy option

A small cholesteatoma with few symptoms can be treated with regular, repeated cleaning (debridement) by a doctor. You may be given antibiotic ear drops. This treatment may work for older people and those at "anesthesia risk."

 

 

 

 

 

Therapy option

There are several types of surgery to treat a cholesteatoma. The procedure the surgeon chooses depends primarily on how far the cholesteatoma has spread and the severity of the hearing loss.

Cholesteatomas can grow aggressively and cause serious and even life-threatening complications. For this reason, the benefits of surgery almost always outweigh the risks. Discuss potential risks with your surgeon before surgery.

You will receive written instructions for the period after the operation and a follow-up appointment with your surgeon.

Some cholesteatomas recur after surgery.

What happens next?

What you can expect in the future.

Cholesteatomas usually continue to grow if they are not removed. Surgery is usually successful, but you will likely need to see an ENT doctor for regular ear cleanings for the rest of your life.

If the cholesteatoma recurs, further surgery may be required. You will need to see your ENT doctor regularly to keep an eye on the situation and further surgery (revision) may be required.

In some cases, hearing improves after surgery. In other cases, complete removal of the tumor requires removal of the affected ossicles as well. This can cause hearing loss. And if the cholesteatoma begins to grow again, hearing loss can worsen.

It remains to be hoped that research will develop non-surgical therapies to treat cholesteatomas.

 

In order to keep this patient information as short as possible, we have not included a detailed list of references. However, this can be requested at any time at info@ivrt.de.

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