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.”

Robert Lebovics, MD, has a famous observation in his practice: “It is amazing what you can see when you just take a look.” As an ear, nose, and throat physician who has specialized in treating autoimmune disorders that impact the airway for more than three decades, he likes to think of himself as the eyes and ears of his patients and other medical specialties—looking at places inside the nose, mouth, and windpipe where they cannot see.

Robert S. Lebovics, MD, FACS, Site Chair of Otolaryngology-Head and Neck Surgery and Co-Director of the Airway Center at Mount Sinai West

Autoimmune disorders occur when the body’s immune system mistakenly attacks healthy tissue and can cause significant damage to the upper airway.

Disorders like Sjogren’s syndrome, Systemic Lupus Erythematosus, and asthma, as well as rare conditions like granulomatosis with polyangiitis (GPA or Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), relapsing polychondritis, and sarcoidosis, can cause inflammation to develop within the airway that restricts both blood and air flow. Patients may experience symptoms like coughing, voice changes, facial pain, loss of smell, and difficulty breathing, swallowing, or hearing.

“The airway is ultimately a pipe system,” says Robert Lebovics, MD, Site Chair of Otolaryngology-Head and Neck Surgery and Co-Director of the Airway Center at Mount Sinai West. “If a pipe in your house is blocked it can’t drain or supply water. In the case of autoimmune disease, inflammation forms in the inner lining of these tissues and may cause it to swell, scar, or bleed. The ability of air to move is diminished.”

Typically, patients are diagnosed with an autoimmune disorder by a rheumatologist or immunologist. If the disease is known to affect the airway, they are often referred to an ear, nose, and throat physician. In general, autoimmune diseases are more common in women than men.

Dr. Lebovics uses specialized instruments like a bronchoscope — a thin tube with a camera that is inserted through the mouth and down the throat—to visualize the airway, voice box, windpipe, upper bronchi, and related structures. Even if patients are not experiencing symptoms like difficulty breathing or painful swallowing, it is vital to get a baseline breathing evaluation.

“The best way to deal with a problem is to prevent it,” says Dr. Lebovics. “I do a lot of surveillance. Sometimes patients do not have any damage yet, and other times they come to me and I see they have been breathing through a three millimeter airway that is significantly scarred from Wegener’s or relapsing polychondritis.”

Treatment for Autoimmune and Airway Disorders

Autoimmune and airway diseases are rarely curable and require lifelong monitoring and management. Many of these conditions have a high incidence of relapse and may be life-threatening if left untreated.

Fortunately, there are many therapies that can help. Depending on the severity of disease, patients may need medications like steroids and immunosuppressants, pulmonary rehabilitation, or surgical interventions. Dr. Lebovics works closely with rheumatologists and pulmonologists to develop a personalized treatment plan for every patient. For example, many individuals with granulomatosis with polyangiitis or Wegener’s have associated ear conditions. “These conditions can be multisystem disorders,” says Dr. Lebovics. “It is critical for physicians to work together and create long-term monitoring relationships with their patients.”

COVID-19 Impact on the Airway

Now, more than ever, it is important for anyone experiencing a breathing problem in the nose, mouth, or chest to seek medical attention. If someone becomes infected with COVID-19, it may cause damage to the lungs and lining of the airway. When someone is gasping for air, they often call 911 or visit the closest emergency room. But those who experience slower changes, like finding it progressively harder to climb the stairs or making noises when they breathe, often do not seek treatment fast enough.

“On a simple level, breathing is not underrated, and your physicians need to help you move air,” says Dr. Lebovics. “At Mount Sinai, we have a niche practice that specializes in treating rare disorders of the airway and autoimmune system, and a track record of success in partnering with patients living with chronic disease to improve their quality of life.”