Unless you are experiencing hearing loss or an acute infection, you probably do not give much thought to your ear health. However, many conditions can affect this complex and very fragile part of the body that not only allows us to hear but also plays a major role in our sense of balance.
Cholesteatoma is a rare condition in which a benign skin growth develops in the middle ear, just behind the eardrum. Only nine out of every 100,000 adults in the United States are diagnosed with these skin growths each year. And, while cholesteatoma is not cancerous, it will continue to grow and may pose serious problems if not removed.
Enrique Perez, MD
“The middle ear is inside the body so it should never have any skin in it. When this condition occurs, skin has grown inward either due to a prior surgery, eardrum perforation, or from chronically reduced ventilation of the ear from conditions like eustachian tube dysfunction,” explains Enrique Perez, MD, MBA, Assistant Professor of Otolaryngology at Mount Sinai, who specializes in treating rare conditions like cholesteatoma. “The problem with this excess skin is that cholesteatomas behave destructively over time. If left untreated, they can cause infection, destroy the bones of hearing, and lead to serious issues like facial nerve paralysis or intracranial complications.”
Who is at risk of developing cholesteatoma? What are the signs and symptoms of the condition?
Patients who have a history of chronic ear infections or eardrum perforation are more likely to develop cholesteatoma. In the early stages of this condition, you may notice some mild pain, pressure, difficulty hearing, or drainage. As the cholesteatoma grows, your symptoms will get worse and may include dizziness, numbness, muscle weakness in one side of the face, or blood-tinged mucus draining from one ear.
Children can be born with a congenital form of cholesteatoma. “These kids often come to us with a history of progressive hearing loss without pain. The ear looks relatively normal, but upon close inspection we find a white structure, which is the trapped skin behind the eardrum,” explains Dr. Perez.
Even if your medical history does not put you at risk for a cholesteatoma diagnosis, make an appointment with your physician if you feel anything out of the ordinary, particularly in just one ear. A simple visit could save you from experiencing permanent damage at a later time.
How is this condition treated?
The only way to treat cholesteatoma is to remove the skin growth. Without surgery, the cyst will become larger and damage the surrounding bone and tissue. If it is left untreated, cholesteatoma can lead to permanent hearing loss, debilitating vertigo, and irreversible nerve damage.
“I tell my patients upfront that cholesteatoma is a surgical disease, and they will need an operation. There is no medication that can remove this growth,” says Dr. Perez.
Since the growths are difficult to remove fully—and the surrounding areas of nerve, bones, and tissues are extremely fragile—it is important to find a surgeon who has experience treating cholesteatoma. Once the skin is removed, there may be a hole in the eardrum or bones in the middle ear that requires reconstruction. There is also a risk that cholesteatoma will grow back if it is not removed properly.
What happens during the cholesteatoma removal procedure?
Most removal procedures are outpatient and, depending on the complexity of the cholesteatoma, the surgery can take anywhere between one to four hours.
At Mount Sinai, Dr. Perez and his surgical colleagues are trained in minimally invasive procedures and often use fully endoscopic approaches to treat cholesteatoma. Instead of making an incision in the back of the ear, these experts can access the growth through the natural opening in the ear canal. Operating rooms at Mount Sinai use the latest technology, including a 4K endoscopic camera system that provides more precise real-time images for surgeons during the procedure.
What is the prognosis for patients following cholesteatoma removal?
Cholesteatoma disease can be quite variable. In patients with recurrent cholesteatoma who have already undergone prior surgeries, it is possible that they will need to undergo a more extensive procedure like a tympanomastoidectomy—a procedure that repairs the middle ear in patients with chronic ear infections. In those cases, the prognosis for ending the infection is high but regaining prior hearing is not as good.
However, in a patient with an early stage of the disease that has not been operated on, the odds of achieving a good hearing outcome as well as successful removal of the cholesteatoma is quite high.
“People often underestimate a recurrent draining ear. It is not just a hassle but a problem you should take care of right away,” says Dr. Perez. “If you come in early, the procedure is more straightforward . By addressing the problem, we can improve a patient’s hearing and quality of life.”