What is Cholesteatoma?

You might not have heard of it before, as cholesteatoma is a relatively uncommon disease, but it can have incredibly serious repercussions if it is left untreated. Cholesteatoma refers to a collection of abnormal cells that gather deep inside the ear and form a skin-lined cyst. This sac can be filled with air, fluid or dead skin cells. The cyst begins near the eardrum, expanding towards the middle and inner ear. Whilst it is not a cancerous growth, it does grow aggressively. Eventually it will eat into the bone and wrap around the incus, a hearing bone. This can result in hearing loss and other symptoms that greatly diminish a sufferer’s quality of life.

What causes cholesteatoma?

Normally, dead skin cells are naturally passed through the ear when earwax is formed. However the ear drum can be damaged either from an injury, infection or as a complication after undergoing surgery. When this happens, the ear drum can collapse. This creates a pocket that will collect dead skin cells instead of letting them pass through the ear canal. It is more common in men than in women, and in extremely rare cases you can be born with cholesteatoma.

How do I know if I have it?

Cholesteatoma has a few typical symptoms that you can look out for if you are worried you might have it. You are likely to experience gradual loss of hearing in the affected ear. This could be accompanied by slight discomfort, pain or a feeling of fullness in the ear. As the cyst forms so close to the eardrum, it may also cause pulsating tinnitus which is like having a rhythmic beat disrupting your hearing. A telltale sign is if you experience watery, often pungent, discharge from the ear. This will either continually flow or come and go and can be quite embarrassing.

Getting a diagnosis

If you have any of the symptoms above, it’s time to visit your GP. Your doctor will examine the inside of your ear with an otoscope. This magnifies and illuminates the ear canal so that they can get a better idea of what is damaging your hearing. If they think it is a case of cholesteatoma, you will likely be referred to an ear, nose and throat specialist. An ENT can order tests to establish how much it has grown and how much of the ear has been damaged. From there, you can discuss your treatment options.

What happens if cholesteatoma is untreated?

Cholesteatoma is an aggressive disease. Leaving it untreated can lead to other associated ear conditions. The cyst retains bacteria, causing ear infections that might not be treatable with a course of antibiotics. In rare cases it can lead to labyrinthitis, which is an inner ear infection that causes persistent dizziness and a complete loss of hearing. As cholesteatoma erodes the bone, this could allow infections to spread outside of the middle ear which has serious complications as well. If the infection spreads to the space around the brain, this can cause meningitis or a brain abscess. A bony abscess, known as mastoiditis, can form as a result of untreated cholesteatoma. Equally, if left, it can damage facial nerves, which can be identified by weakness or paralysis of one side of the face. Whilst these are all rare, it is best to seek treatment as soon as possible as a preventative measure.

Treating cholesteatoma

Unfortunately, surgery is the only treatment option, as cholesteatoma needs complete removal to prevent it growing back. The chances of regrowth are increased in children but can still occur in adults. The surgery is in 2 parts and the type of procedure will depend on the stage of the disease. Often these decisions are made on the day of the surgery, as the surgeon will need to check is the disease has progressed since your last consultation.

As with any surgery, there are some associated risks. Operating on cholesteatoma is an incredibly delicate procedure that is performed under a microscope. You will be placed under general anaesthetic and the surgery usually lasts between 2 and 3 hours. Usually a patient experiences hearing loss before surgery and the procedure will try to improve this where possible, but scar tissue and inflammation may hinder the overall effect. There is also a small risk to the facial nerve. In most cases, surgery is extremely effective and you can even go home the same day it takes place.

In most cases, patients are treated with a single-stage procedure. This removes the disease completely and then tries to reconstruct as much of the ear as possible. If there is scarring or inflammation reconstruction might not be possible, so a second-stage operation might take place once this has had time to heal.

The first part of the surgery is referred to as a mastoidectomy. Where the disease has grown into a mastoid, this is removed by opening the bone behind the ear. In an open procedure, they remove all of the bony walls to create an open cavity. This decreases the chance of the cholesteatoma coming back, but will mean having to wear earplugs when swimming and a yearly visit to your doctor to remove earwax. If the canal wall can be preserved, they conduct a closed procedure operation and form a closed cavity. This will not require the same aftercare of an open procedure but it does increase the risk of recurrence in cases where the disease is hidden behind the bone, possibly leading to a second operation.

After this, you move into the second stage of surgery, the tympanoplasty. This is when they repair the eardrum and hearing mechanism to reconstruct the ear. They repair this with either a graft of cartilage or fascia which comes from the lining of the muscle behind your ear. This can close holes in the eardrum permanently. Where small hearing bones have been damaged by the disease, these will be repaired where possible with natural bone or cartilage. If this is not possible, synthetic prosthetics can be made that consist of bone material.

After surgery

It is important to see your doctor regularly for check ups after your surgery because of the possibility of recurrence. They can monitor your progress and answer any questions or concerns you might have. They can also let you know if a second operation is required.