This article is about cholesteatoma, a rare condition experienced by patients and seen by General Practitioners, Otolaryngologists, and Audiologists. This resource is open to everyone. Patients, Medical Students and Medical Professionals may find this useful. Adjacent boxes provide additional information for Medical Professionals.

 

What is Cholesteatoma?

Cholesteatoma is an abnormal skin growth behind the eardrum in the middle ear. It is not cancer however it causes damage to the surrounding tissue. Cholesteatoma is usually a complication of chronic (long-term) middle ear infections. A rare congenital form (present from birth) can occur in the middle ear or elsewhere nearby in the bones of the skull.

 

Who gets Cholesteatoma?

Cholesteatoma can occur in children or adults. Most people with cholesteatoma have problems with repeated middle ear infections before they develop cholesteatoma. Most people who have had ear infections will not develop cholesteatoma.

 

What are the causes?

Most people with cholesteatoma have poor Eustachian tube function. The Eustachian tube connects the middle ear with the nose to allow the ears to be equalised (popping the ears). In a healthy person air travels from the nose , through the Eustachian tube, into the middle ear. If you have difficulty popping the ears, negative pressure can develop in the middle ear causing the eardrum to become sucked inwards (retracted). Sometimes the whole eardrum is sucked inwards. Sometimes a small part of the eardrum is sucked inwards, forming a small pocket which is called a retraction pocket.

The skin over the whole body constantly sheds old skin cells – the eardrum is the same. In a healthy person old skin cells from the eardrum move out of the ear in the ear wax. If there is a retraction pocket in the eardrum, the cells may become trapped in the pocket. This can form a lump of old skin cells that slowly grows over time.

 

What are the symptoms?

Most people with cholesteatoma will have fluid leakage (discharge) from the ear. The discharge may be continuous or may come and go. It is often smelly. Most people will also notice a slow loss of hearing in their ear. Some people may have discomfort or a feeling of pressure in the ear. It is rare to have pain because of cholesteatoma. Other symptoms can include ringing in the ears (tinnitus), dizziness, or balance upset. Weakness of the muscles of the face can sometimes occur with advanced cholesteatoma, although this is rare.

Examination of the ear may show discharge in the ear canal. Often a retraction pocket is visible in the eardrum, although it can be very small. Yellow, white or brown coloured material may be visible sitting on the eardrum.

 

How is it diagnosed?

Cholesteatoma is suspected in people with chronic ear discharge and hearing loss. Patients should be seen by an Otolaryngologist (ENT Surgeon) and hearing tests should be performed. The ear will be cleaned and a microscope used to view the eardrum looking for a retraction pocket, material collecting on the eardrum, or other ear abnormalities.

If your Doctor thinks you may have cholesteatoma imaging of the ear and skull with a CT scan will usually be performed. Imaging helps your Doctor see the size of the cholesteatoma and its relationship to nearby important structures.

Cholesteatoma is diagnosed by suspicion from the patient’s symptoms, how the eardrum looks when it is examined with a microscope, and how the deeper parts of the ear look on scans. Most cholesteatoma is treated with surgery. Findings during surgery confirm the diagnosis.

 

What are the potential complications?

Complications of cholesteatoma happen due to damage to surrounding tissues caused by the growing lump.

Complications include:

  • Ossicular erosion – the three small bones in the middle ear form a tiny chain. Sound waves pass from the ear drum, through the chain, to the inner ear. Cholesteatoma can erode one or more of these bones, causing a gap in the chain. This reduces the transmission of sound waves to the inner ear, leading to hearing loss.
  • Semicircular canal dehiscence – the semi-circular canals form part of the balance organ in the inner ear. The lump can cause damage to one of the semi-circular canals, causing dizziness and balance upset.
  • Tegmen tympani dehiscence – the tegmen tympani is a thin plate of bone which separates the inner ear from the brain. Cholesteatoma can cause damage to this bone, creating an opening between the ear and the brain. If this occurs, infection can spread more easily from the ear to the brain. People with tegmen dehiscence are at risk of brain infections such as brain abscess or meningitis, which can be life threatening.
  • Facial paralysis – the facial nerve gives movement to the facial muscles that allow us to smile, frown, and raise the eyebrows. The facial nerve passes through bone very close to the middle ear. Cholesteatoma can damage the bone protecting the facial nerve to cause pressure and injury to the nerve.
  • Infection – cholesteatoma can become infected with bacteria. Different infections can occur including infection of the middle ear, mastoid bone, abscess in the surrounding tissues, or rarely brain abscess or meningitis.

 

What are the treatment options?

Surgery is almost always needed to remove cholesteatoma. The type of surgery that your Doctor will recommend depends on the size and location of the cholesteatoma. Sometimes surgery is only needed to the middle ear, however often the mastoid bone behind the ear must be surgically opened with a drill to remove all of the disease.

The main aim of surgery is to remove the cholesteatoma. The second aim is to preserve or improve hearing. Sometimes both can be done at once, however sometimes a second procedure many months later may be needed to restore hearing. In some cases it is difficult to restore hearing, and a hearing aid may be recommended instead. Surgery may also worsen hearing.

Some patients may choose not to have surgery. In this case the cholesteatoma will probably grow slowly over time. Complications can occur. Treatment without surgery is usually only recommended for older patients who are not fit for long surgery and anaesthesia.

For further details on cholesteatoma surgery see our pages on tympanoplasty and mastoidectomy.

 

 

Can you prevent Cholesteatoma?

Many people with cholesteatoma do not know they are developing a cholesteatoma until it has formed. However a Doctor, Ear Nurse, or other medical professional may diagnose a retraction pocket of the eardrum by examining the ear. Not all retraction pockets will  develop into a cholesteatoma, however some will. If you have a retraction pocket, sometimes medical treatments or surgery will be recommended to try to stop it from worsening, and to reduce the risk of a cholesteatoma forming.

For patients who have had a cholesteatoma in one ear, there is an increased risk of a cholesteatoma forming in the other ear. These patients should have their other ear checked by an Otolaryngologist at the time of diagnosis. If any symptoms develop, the ear should be checked again, and cholesteatoma treated early to prevent hearing loss in both ears.

 

What is the prognosis?

Most patients with cholesteatoma can be successfully treated with surgery. Cholesteatoma can come back, sometimes years after treatment. The chance of cholesteatoma coming back depends on the size of the initial cholesteatoma, as well as the type of surgery performed to remove the cholesteatoma.  Cholesteatoma can recur in up to 70% of patients treated for cholesteatoma. In general, patients should be checked by a medical professional for years after cholesteatoma removal. If cholesteatoma comes back it can be treated with more surgery. Cholesteatoma is not a tumour and it does not spread to other parts of the body.

 

Further reading:

See our pages on mastoidectomy and tympanoplasty for more information about the surgical treatment of cholesteatoma.

 

References:

Kerckhoffs KG, Kommer MB, van Strien TH, Visscher SJ, Bruijnzeel H, Smit AL, Grolman W. The disease recurrence rate after the canal wall up or canal wall down technique in adults. Laryngoscope. 2016 Apr;126(4):980-7. doi: 10.1002/lary.25591. Epub 2015 Sep 25. PMID: 26404516.
Tomlin J, Chang D, McCutcheon B, Harris J: Surgical Technique and Recurrence in Cholesteatoma: A Meta-Analysis. Audiol Neurotol 2013;18:135-142. doi: 10.1159/000346140

 

Author + Affiliation:
Dr Rachael Bentall, Department of Otolaryngology, Waikato Hospital
Reviewed by….. Dr Shueh Lim, Otolaryngologist, Waikato Hospital

 

Date of Publication +/- Review:

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