What is a Cholesteatoma?

Cholesteatoma: Causes, Symptoms, and Potential Complications

A cholesteatoma is an abnormal growth of skin inside the ear. It can act like a small benign tumor, but is not made of any cancerous cells. In fact, the content of a cholesteatoma is mainly keratin, which is found in skin.

The skin can grow abnormally due to trauma, infections, surgery, and even during fetal development. The skin and keratin grow slowly over months to years and can begin to expand aggressively to the point where hearing becomes affected.

There are enzymes within the cholesteatoma which slowly erode the overlying bone and hearing structures of the ear. This erosion leads to conductive hearing loss, chronic infection, ear drainage, tinnitus, vertigo, taste disturbance, and even facial weakness in advanced cases..

Very rarely, untreated cholesteatoma can erode the bony wall which separates the ear from the brain. This can result in drainage of brain fluid (CSF leak) and brain herniation. It is very important to have cholesteatoma surgery by a trained otologist (ear surgeon) before any of these complications arise.

Who is most at risk to develop a Cholesteatoma?

Chronic Eustachian tube dysfunction (ETD) is the leading cause of cholesteatoma. The Eustachian tube connects the middle ear to the back of the nose, and is responsible for equalizing air pressure in the ear and also draining the ear.

ETD causes blockage of the Eustachian tube which leads to increased negative pressure in the middle ear. This negative pressure results in retraction of some parts of the eardrum over time. The retracted skin of the eardrum can then grow to become a cholesteatoma.

Patients with ETD also have a history of chronic allergies, recurrent sinus infections, or enlarged adenoids. Other risk factors include chronic ear infections, history of ear tubes (myringotomy tubes, or PE tubes), and prior ear surgery.

How are Cholesteatomas diagnosed?

Cholesteatomas can be diagnosed by an experienced ENT, or fellowship trained otologist (ear surgeon). Your ear specialist will first need to take a detailed history and examination under a microscope. You will also need a comprehensive hearing test, as well as a CT scan. An MRI is not needed unless there is concern for brain involvement or CSF leak.

How are Cholesteatomas treated?

There is no medical treatment for cholesteatoma, except for surgery. It is important to treat any underlying ear infection prior to cholesteatoma surgery. Your  ear surgeon will discuss an operation called a tympanoplasty and mastoidectomy. The exact techniques of these surgeries vary based on cholesteatoma size and surgeon experience. Some surgeons choose a minimally invasive technique using endoscopes. It is important for the patient to discuss the possible different techniques with their surgeon.

During a tympanoplasty and mastoidectomy operation,  the otologist will create an incision behind the ear to clear the mastoid bone and access the middle ear space. The cholesteatoma usually arises in the middle ear and can spread to the mastoid. Depending on the extent of the disease, the surgeon may choose a “canal wall up” mastoidectomy procedure versus a more extensive “canal wall down” technique. It is important to talk to your surgeon about these techniques and which will be performed.

It is also very important to discuss the high possibility of a 2nd surgery which is done 6 -12 months after the first operation. This is called a “2nd look procedure”. There is technology now which allows some surgeons to monitor the recurrence of cholesteatoma through clinical and imaging techniques. An MRI may be performed 6-12 months after surgery which has a high sensitivity for detecting recurrent cholesteatoma. This technology has helped decrease the number of 2nd look procedures at our institute.