Nearly 2 out of 3 people living with myasthenia gravis (MG) also have a co-occurring health condition, known as a comorbidity. One example is Ménière’s disease — a condition of the inner ear that can cause problems with hearing and balance. Although Ménière’s disease is rare, people with some autoimmune diseases may have a higher risk of developing it.

Continue reading to learn more about Ménière’s disease and if it might be linked to myasthenia gravis.

What Is Ménière’s Disease?

Ménière’s disease affects the inner ear and causes problems with hearing and balance. Researchers don’t know what causes this condition, but they believe that a buildup of endolymph (fluid in the inner ear) plays a role in triggering symptoms.

The inner ear helps you hear by turning sound waves into electrical signals that can be transmitted to the brain. Additionally, the inner ear contains your vestibular system — the organ system that helps you maintain balance. When there’s too much endolymph, the extra pressure on the inner ear can cause symptoms, including:

• Vertigo (a spinning or dizzy feeling)
• Tinnitus (ringing or buzzing in the ears)
• Hearing loss
• A feeling of pressure or fullness in the ear

The symptoms of Ménière’s disease usually affect just one ear and can last anywhere from 20 minutes to 24 hours. Without treatment, symptoms may get worse over time. Some people develop permanent hearing loss and chronic (long-term) balance problems.

Ménière’s disease is rare, affecting an estimated 615,000 people in the United States. It’s unknown how many people with myasthenia gravis also have Ménière’s disease.

Are People With Myasthenia Gravis at Risk of Ménière’s Disease?

No studies have looked into the prevalence (commonness) of Ménière’s disease among people with myasthenia gravis. However, researchers have found that Ménière’s disease happens more often in people with autoimmune conditions, such as:

• Lupus — Affects the skin, joints, and other organs
• Rheumatoid arthritis (RA) — Causes joint pain and swelling
• Ankylosing spondylitis — Mainly affects the spine, causing stiffness and pain

Some studies also show that people with Ménière’s disease are three to eight times more likely than the general population to have an autoimmune condition. A 2023 study found links between Ménière’s disease and autoimmune conditions including thyroid disorder, vitiligo (a skin condition that causes loss of pigment), and psoriasis (a skin disease that causes discolored, scaly patches).

Risk Factors for Ménière’s Disease and Myasthenia Gravis

Ménière’s disease and myasthenia gravis share some risk factors, which means they may affect some of the same people. Both conditions most commonly affect women, starting around age 40, according to Cleveland Clinic. Additionally, having another autoimmune condition, such as lupus or an autoimmune thyroid disease, is a risk factor for both Ménière’s disease and MG.

Myasthenia Gravis Symptoms May Look Like Ménière’s Disease

Hearing loss isn’t a typical symptom of myasthenia gravis, but MG can look different from person to person and may cause hearing loss in some people.

Myasthenia gravis occurs when an overactive immune system makes antibodies (proteins that normally fight infection) that attack the neuromuscular junction — the space where nerves and muscles communicate. Most often, the immune system targets acetylcholine receptors (AChRs), which muscles need to work properly. When AChRs are attacked, it’s harder for nerves and muscles to communicate, leading to muscle weakness.

In some people with MG, the AChR antibodies also affect hair cells in the inner ear. Normally, hair cells help translate sound into an electrical signal that travels through the auditory (hearing) nerve to the brain. When they’re attacked by AChR antibodies, hair cells don’t work properly, leading to hearing problems. This type of hearing loss is called sensorineural hearing loss — it’s caused by problems with the hair cells and auditory nerve.

Hearing usually isn’t among the first MG symptoms, but some people may notice changes in how well they hear over time. One study found hearing loss in 30 percent of people with myasthenia gravis, but most people don’t notice this symptom without testing.

Vertigo and tinnitus are other possible MG symptoms. However, dizziness in MG is most often related to muscle weakness, not problems with the vestibular system.

Ménière’s Disease May Look Like an Autoimmune Ear Disease

The symptoms of Ménière’s disease are very similar to those of autoimmune inner ear disease (AIED). AIED happens when the immune response mistakenly attacks the inner ear.

In about 70 percent to 85 percent with AIED, the ear is the only part of the body affected. However, AIED can also occur in people with other autoimmune conditions.

Both AIED and Ménière’s disease can cause sensorineural hearing loss, typically affecting one ear. Tinnitus, ear pressure, and dizziness are also common AIED symptoms. In the early stages, AIED and Ménière’s disease can be difficult to tell apart. AIED often progresses over weeks to months, whereas Ménière’s disease usually worsens slowly. It can take eight to 10 years for Ménière’s disease to progress to permanent hearing loss.

How Is Ménière’s Disease Diagnosed?

If you begin having inner ear symptoms, your healthcare provider may refer you to an otolaryngologist — an ear, nose, and throat specialist (ENT) for a diagnosis.

There isn’t one specific test to confirm Ménière’s disease. Instead, doctors use several tests to rule out AIED and other causes of inner ear problems. These tests may include:

• Hearing test — Identifies hearing loss
• Vestibular testing — Evaluates balance and how your eyes and inner ear work together
• MRI of the brain — Detects tumors or other causes of vertigo or hearing problems
• Blood tests — Reveal inflammation or issues with how your organs are working

Managing Inner Ear Problems With Myasthenia Gravis

Getting an accurate diagnosis is the first step in finding the right treatment for inner ear symptoms. Different causes of inner ear problems require different treatments. It’s important to talk to your healthcare team right away if you notice any new symptoms that affect your balance and hearing. Early diagnosis and treatment may help prevent more serious complications.

Treatment of Ménière’s Disease

There’s no cure for Méneire’s disease, but several treatments can help reduce symptoms. Many treatments focus on regulating fluid levels in the body. A low-salt diet may help prevent fluid retention that can trigger symptoms. Diuretics (water pills) can help remove extra fluid from the body. The drug betahistine can help reduce pressure in the inner ear by improving blood flow.

If symptoms don’t improve, a corticosteroid injection through the eardrum may help reduce episodes of vertigo. Gentamicin injections may help control severe vertigo, and surgery can relieve inner ear pressure or alter the vestibular organs.

Treatment of Autoimmune Inner Ear Disease

For inner ear symptoms caused by an autoimmune disease such as AIED, treatment usually focuses on reducing inflammation. Corticosteroids (a type immunosuppressant medication) are often used to reduce swelling in the inner ear.

If AIED is treated early with corticosteroids, hearing loss may be reversible. However, about 30 percent of people with AIED don’t respond to steroids. If steroids stop working, other treatment options may be considered, though research is limited. These treatments include:

• Azathioprine (Azasan, Imuran)
• Cyclophosphamide (Cytoxan)
• Methotrexate (Trexall, Xatmep)
• Mycophenolate mofetil (CellCept, Myfortic)
• Rituximab (Rituxan, Truxima)

Your healthcare team can help you weigh the risks and benefits of these options and decide on the next steps based on your individual needs.

Find Your Team

On MGteam, the social network for people living with myasthenia gravis and their loved ones, more than 2,000 members come together to ask questions, give advice, and share their stories with others who understand life with myasthenia gravis.

Are you living with Ménière’s disease and MG? Have you noticed any hearing changes since your MG diagnosis? Share your experience in the comments below, or start a conversation by posting on your Activities page.