A cholesteatoma is an abnormal skin growth that can develop in the middle ear. It usually begins as a collection of dead skin cells and develops into a cyst-like pocket behind the eardrum. It can significantly impair a person’s hearing and balance, as well as the function of their facial muscles.
Cholesteatomas are not common, with acquired forms (those not present at birth) having an annual incidence of 9–12.6 out of 100,000 adults and 3–15 out of 100,000 children. These growths are more common in boys than in girls, and their rate is highest among Caucasian people.
Continue reading to learn about the causes, symptoms, and treatment of a cholesteatoma.
According to the BMJ Best Practice, a cholesteatoma is “the presence of keratinizing squamous epithelium within the middle ear.” In other words, it is a tough growth of accumulating skin cells and keratin that can develop anywhere from near or behind the eardrum to the bone behind the ear.
Due to the position of a cholesteatoma, if it increases in size, it can destroy the delicate bones in the middle ear and possibly extend into the inner ear. This expansion could result in problems with hearing, balance, and the function of the facial muscles.
There are two types of cholesteatoma: congenital, which means present from birth, and acquired. The latter type can affect both children and adults.
The majority of cholesteatoma cases are acquired. These growths can form because of the following:
- repeated ear infections
- skull or facial bone birth abnormalities
- an injury to the eardrum
- negative pressure in the Eustachian tube (the tube connecting the middle ear to the back of the nose and throat), which makes the eardrum pull inward and allows keratin and dead skin cells to accumulate in the middle ear
- dead skin cells and earwax moving into the middle ear through a hole in the eardrum
Studies show that only 2–4% of cholesteatomas in children between the ages of 4 and 6 years are present at birth. Children with a cleft palate have a much higher risk of developing a cholesteatoma than other children.
The symptoms of a cholesteatoma are usually fairly mild initially. They typically become more severe as the mass grows larger and causes more problems.
The symptoms of a cholesteatoma may include:
Without treatment, a cholesteatoma will grow larger and may cause a variety of complications.
As dead skin cells accumulate in the ear, they provide an ideal environment for bacteria and fungi to thrive. As a result, the cholesteatoma is prone to infection and inflammation.
Over time, if the cholesteatoma continues to grow, it may erode the adjacent bone. This erosion can damage the eardrum, the bones inside the middle ear, the inner ear, the bones near the brain, and the nerves near the inner ear and face. The damage may result in hearing loss and facial weakness.
An untreated cholesteatoma can lead to the following complications:
- permanent hearing loss
- erosion of hearing bones
- chronic ear infections
- paralysis of facial muscles
- ongoing problems with balance
- brain abscess
An untreated cholesteatoma can be life threatening in the long term.
To diagnose a cholesteatoma, a doctor will take a complete medical history and conduct a physical exam, often using an otoscope to examine the ear. An otoscope is a combination of a magnifying glass and a flashlight.
If the doctor suspects a cholesteatoma, they will refer the person to a specialist in ear, nose, and throat (ENT) medicine for further examination.
ENT specialists may order further tests, such as a CT scan, to determine whether a cholesteatoma is present, where it is, and whether it is growing.
They may also perform an audiogram to provide more information about the state of the person’s hearing.
If a cholesteatoma is small and contained, and the individual can handle the procedure, regular cleaning in a doctor’s office may be sufficient treatment.
However, the vast majority of people with a cholesteatoma undergo surgery. Cholesteatomas do not go away naturally, tending to continue to grow and cause complications. Therefore, surgery is typically the best way to remove a cholesteatoma and prevent any complications.
Before surgery, doctors will try to clear up any infections affecting the area. They may prescribe antibiotic therapy to treat the tissue surrounding the growth to reduce infectious inflammation.
The surgery is typically an outpatient procedure, which means that the person does not need to stay in the hospital afterward. However, people with a very large cholesteatoma or a serious infection may require hospitalization.
Cholesteatoma surgery removes the growth in the ear, addresses any infection, and helps return the ear to a healthy, stable, and soundly functioning condition. The specific operations that the surgeon performs will depend on the location of the cholesteatoma and how much repair work is necessary.
In addition to removing the growth, surgery may be necessary to:
- restore the eardrum
- rebuild the hearing bones
- remove bone defects from behind the ear
Before undergoing the reconstruction of the hearing bones, some people will need a second surgery to completely remove all of the cholesteatoma. If necessary, a surgeon may schedule a second surgery 6–12 months after the first.
After surgery, people need to protect the area by:
- keeping their ears dry, which means not washing the hair for about a week and plugging the ears after that
- avoiding strenuous activities for a few weeks
- avoiding swimming and air travel for a few weeks
- being conscientious about their follow-up appointments and aftercare to make sure that the cholesteatoma does not return
It is not possible to prevent congenital cases of cholesteatoma. However, it is worth parents and caregivers being aware of the condition, so that children can quickly receive diagnosis and treatment.
For acquired cholesteatoma, properly treating ear infections is the best prevention. However, cholesteatomas may still develop.
Some researchers suggest that placing tympanostomy tubes in the ears can prevent the growth of a cholesteatoma. These tubes can aerate the middle ear, allow fluid drainage, and reduce the risk of negative pressure in the Eustachian tubes.
The American Academy of Otolaryngology — Head and Neck Surgery recommend that people see a doctor as soon as they develop any symptoms of a cholesteatoma.
It is important to treat a cholesteatoma as early as possible to prevent any complications.
The outlook for people with a cholesteatoma is generally positive. Although surgery is almost always necessary to remove this benign mass before complications occur, the procedure is usually successful.
Follow-ups are important after surgery because cholesteatomas can regrow after years.
Complications are rare if a surgeon removes the cholesteatoma at an early stage. However, if the cholesteatoma was particularly large at the point of removal, problems such as hearing loss and imbalance may remain after surgery.
Without treatment, a severe cholesteatoma can be fatal.
Cholesteatomas are growths that develop in the middle ear.
Symptoms may be mild at the start, but they can include recurring ear infections.
If a person does not receive treatment, a cholesteatoma can cause a number of complications, such as hearing loss and imbalance, and it may even be fatal.
Typically, surgery to remove cholesteatoma is the best treatment.