Cholesteatoma: What You Need to Know
When Skin Grows Within the Middle Ear
Medically reviewed by William Truswell, MDMedically reviewed by William Truswell, MD
Cholesteatoma is an abnormal skin growth within the middle ear. This serious ear condition can be present at birth or develop later in life, often as a result of multiple ear infections.
While cholesteatomas are noncancerous (benign), they can eat away at and damage the delicate bones within the middle ear and cause permanent hearing loss. Left untreated, another potential, albeit rare, complication of a cholesteatoma is an infection that can spread through the ear to the brain.
This article will review cholesteatomas' causes, symptoms, diagnosis, and treatment. It will also explore how this condition can be prevented and its general prognosis (outcome).
Jacob Wackerhausen / Getty Images
What Is the Middle Ear?
The middle ear is an air-filled space between the eardrum (tympanic membrane) and the inner ear, a fluid-filled structure that transmits sound and balance signals to the brain.
What Causes a Cholesteatoma?
The precise cause of a cholesteatoma is not fully known.
Experts suspect that acquired cholesteatomas most commonly occur as a result of a blocked eustachian tube.
Eustachian tubes link the middle ear to the sinuses. They help drain fluid from the middle ear and balance the pressure on either side of the eardrum.
Eustachian tubes can become blocked if:
Excess fluid collects within the middle ear from, for example, a common cold or allergies
Prolonged exposure to pressure differences between the outside environment and the middle ear, such as from air travel or scuba diving
With a blocked eustachian tube, the eardrum may retract or be sucked towards the inside of the ear.
Inflammation behind the eardrum can then trigger skin cells to grow rapidly. These cells eventually mature and die, shedding themselves with keratin (a protein that helps form the outer skin layer) into a mass, enlarging over time to form a cholesteatoma.
An acquired cholesteatoma can also form if there is inflammation within the outer lining of the eardrum and the eardrum has a hole or tear (perforation).
In such cases, rapidly growing cells may travel through the eardrum, embedding themselves within the middle ear.
This type of cholesteatoma is often caused by repeat infections in the middle ear—what's known as chronic otitis media.
Rarely, cholesteatomas are congenital, meaning they are present at birth. Congenital cholesteatomas arise from tissue that becomes trapped within the middle ear during development.
Cholesteatoma in Children
Cholesteatoma is a rare condition, and its symptoms and treatment are similar in children and adults.
Cholesteatoma is slightly more common in adults than children, affecting around 3 out of 100,000 children and 9 out of 100,000 adults.
Conversely, the recurrence rate of cholesteatoma (when the growth returns after being surgically removed) is higher in children than in adults.
What Are the Symptoms of a Cholesteatoma?
If cholesteatoma symptoms are present, they include:
Gradual hearing loss on the affected side
Watery ear drainage, often with a foul odor, from the affected ear: The ear drainage (otorrhea) may come and go or be persistent.
Hearing loss from a cholesteatoma is generally attributed to the wearing away and eventual destruction of the middle ear's tiny, fragile bones called ossicles.
Bone destruction occurs from several different processes, including pressure from the aggressively growing mass of skin cells and bone-destroying enzymes produced by the cholesteatoma.
What Are the Potential Complications?
As the cholesteatoma grows and spreads into the inner ear, symptoms reflecting damage or inflammation of the inner ear structures can develop.
These inner ear symptoms include:
Since cholesteatoma retains bacteria, it can spread and infect the mastoid bone (mastoiditis). The mastoid bone is honeycombed-shaped and behind the ear. It is part of the temporal bone, located at the sides/base of the skull.
Potentially life-threatening, although rare, infections can then travel into the brain via the temporal bone.
These infections include:
A cerebral (brain) abscess is a collection of pus within the brain. Symptoms may include headache, fever, weakness, vomiting, confusion, and neck stiffness.
Meningitis is inflammation of the tissues that line the brain and spinal cord. Symptoms may include headache, fever, neck stiffness, confusion, and photophobia (sensitivity to light).
Other severe complications of a cholesteatoma include:
Facial nerve paralysis causes facial drooping on the affected side. This complication can result from direct compression of the nerve or toxic substances produced by the cholesteatoma that damage the nerve.
Cerebral sinus thrombosis is a blood clot in a vein in the brain. Symptoms can include headache, seizures, blurry vision, and fainting.
How Is a Cholesteatoma Diagnosed?
A cholesteatoma can appear as a white lump or cyst behind the eardrum during a routine ear exam performed by a healthcare provider using an otoscope.
A person with suspected cholesteatoma should be urgently referred to an otolaryngologist, also known as an ear, nose, and throat (ENT) specialist, for a more thorough evaluation.
If worrisome symptoms (e.g., vertigo or headache) are present, a person should go to the emergency room.
An ENT can confirm the presence of a cholesteatoma. Removing debris, wax, and drainage within the ear canal allows them to better see the tympanic membrane and any nearby mass/growth.
Next, a high-resolution computed tomography (CT) scan of the temporal bone is performed to assess the extent of the cholesteatoma. Magnetic resonance imaging (MRI) may also be performed if complications are suspected.
After a cholesteatoma is visualized, a person will undergo an audiogram to assess hearing loss, including its type and severity.
How Is a Cholesteatoma Treated?
The treatment of a cholesteatoma is surgical removal under general anesthesia. The exact operation performed depends on the size and extent of the growth but generally involves mastoidectomy and tympanoplasty:
Mastoidectomy involves removing diseased cells within the mastoid bone's hollow spaces.
Tympanoplasty involves repairing the eardrum and rebuilding the sound-conducting mechanism of the middle ear to restore or salvage hearing.
Ossicular reconstruction—repair of the small bones in the middle ear—may also be performed.
Ways to Prevent Cholesteatomas
Congenital cholesteatomas cannot be prevented, while acquired cholesteatomas may possibly be prevented by doing the following:
Do not clean your ear canal with a cotton tip applicator/swab, which can damage and tear the eardrum open.
If your eardrum is perforated, avoid swimming or getting your ear canal wet during bathing.
Undergo surgical correction of a perforated eardrum if it does not heal promptly on its own.
See an ENT specialist for recurrent ear infections, hearing loss, or new/recurrent ear drainage.
Long-Term Outlook for People With a Cholesteatoma
For most people, surgical removal of the cholesteatoma is successful.
However, cholesteatomas can grow back (recur). Another surgery may or may not be needed, depending on the size and other factors.
Research suggests that cholesteatoma recurrence rates are as high as 30% in adults and 70% in children. The type of cholesteatoma—acquired vs. congenital—does not appear to affect the likelihood of recurrence.
Due to the risk of recurrence, people must follow closely with their ENT after surgery.
Lastly, while surgical removal of a cholesteatoma is necessary, it does not always improve hearing. Sometimes, a second surgery is needed to help restore hearing loss.
Summary
Cholesteatoma is an expanding skin growth within the middle ear that can develop in children or adults. Although rare, it can be present at birth.
Symptoms of a cholesteatoma include hearing loss and persistent or recurrent ear drainage. Left untreated, complications such as dizziness, loss of balance, and, rarely, brain infections or clots can develop.
An otolaryngologist (ENT specialist) diagnoses cholesteatomas using an otoscope. Imaging tests are performed to evaluate the extent of the skin growth and for complications.
Treatment involves surgical removal. Sometimes, a second surgery is needed if the cholesteatoma recurs or to help restore hearing loss.
If you have a cholesteatoma or have had one removed, speak with an ENT specialist. Early treatment and close follow-up are key to managing this serious ear condition.
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