What Is Ménière’s Disease?

Ménière’s disease is a chronic inner ear disorder affecting balance and hearing.

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French doctor Prosper Ménière first identified and described the symptoms of the medical condition that now bears his name in 1861. These symptoms include fluctuating hearing loss, episodic vertigo, a sensation of ear fullness, and tinnitus. 

Many Disorders Have Similar Characteristics

In its 2020 clinical practice guideline, the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) uses this definition:

“Ménière’s disease is a disorder of the inner ear that includes episodes of vertigo with possible hearing loss, ringing or buzzing in the ear, or ear pressure.

“Ménière’s disease is defined by spontaneous vertigo attacks, each lasting 20 minutes to 12 hours, with low- to mid-frequency sensorineural hearing loss in the affected ear  before, during, or after one of the episodes of vertigo. The clinical practice guideline defines vertigo as the feeling of spinning or moving when one is not moving. This is different from dizziness, which can mean feeling lightheaded or feeling like passing out.”





Most Cases Are in One Ear and in Adults Ages 40 to 50

Most cases of Ménière’s disease are unilateral (affecting only one ear), but some will be affected in both ears. Ménière’s disease often starts as unilateral and progresses to bilateral. Around 15 percent of people with Ménière’s will experience bilateral hearing loss.

It's estimated that there are 600,000–750,000 cases of Ménière’s disease in the United States, with 45,000 to 60,000 new cases diagnosed annually. Estimates of the worldwide incidence of Ménière’s disease vary significantly, from 3.5 to 513 per 100,000. 

According to AAO-HNS, “Ménière’s disease is almost exclusively reported in adults, with less than 3 percent of cases estimated to occur in children younger than 18 years old. The disease is most prevalent between ages 40 to 60 years, with peak onset in the 40s and 50s.

“In many patients, the most detrimental decline in hearing and balance function occurs within the first decade of diagnosis, yet patients continue to have longstanding deficits that make Ménière’s disease a chronic disease. It is important to evaluate and document the hearing in both ears as a subset of patients will eventually experience Ménière’s disease bilaterally.”

 

Causes of Ménière’s Disease

Even though the exact causes of Ménière’s disease are not clearly known, symptoms are believed to arise from the buildup of fluid in the chambers of the inner ear.

According to the National Institute on Deafness and Other Communication Disorders (NIDCD), the disorder is physically caused by a buildup of a fluid called endolymph in the compartments of the inner ear, called the labyrinth. The labyrinth contains the organs of balance (the semicircular canals and otolithic organs) and hearing (the cochlea).

The labyrinth has two sections: the bony labyrinth and the membranous labyrinth, the second of which is filled with endolymph which, in the balance organs, stimulates receptors as the body moves. The receptors then send signals to the brain about the body’s position and movement. In the cochlea, fluid is compressed in response to sound vibrations, which stimulates sensory cells that send signals to the brain.

This excess fluid–beyond the normal amount of fluid that exists in the cochlea–affects both balance and hearing. The cause of the fluid buildup is not yet completely understood. Some researchers believe it is related to the same blood vessel constrictions that lead to migraine headaches; others say it might be due to an autoimmune condition, a viral infection, an allergic reaction, or head trauma. Ménière’s disease appears to have a hereditary component, so there may be a gene mutation connected to the regulation of endolymph fluid.

 
 

Symptoms and Diagnosis of Ménière’s Disease

The National Institute on Deafness and Other Communication Disorders (NIDCD) and the Mayo Clinic list four main symptoms of Ménière’s disease:

  • Recurring episodes of vertigo: This is experiencing a spinning sensation that starts and stops spontaneously. Episodes of vertigo occur without warning and can last several minutes to several hours. Some may experience drop attacks, where the vertigo is so severe and sudden that the patient falls to the floor.

  • Hearing loss: Hearing loss in Ménière's disease usually fluctuates but can become permanent.

  • Tinnitus: Hearing a ringing, buzzing, roaring, whistling, or hissing sound.

  • Ear fullness: This is feeling pressure or fullness in the ear, usually in one ear.

Since Ménière's disease can have similar symptoms as other illnesses, your healthcare provider will look into your medical history, hearing and/or balance assessments, and, according to the Mayo Clinic, the presence of the following conditions to diagnose the disease: 

  • Two or more vertigo attacks, each lasting 20 minutes to 12 hours, or up to 24 hours

  • Hearing loss validated by a hearing test

  • Tinnitus or a feeling of fullness or pressure in the ear

These signs can be accompanied by other additional symptoms, according to the Cleveland Clinic: headaches, abdominal pain, and nausea. Individual patients may report a range of other symptoms that could be connected with the experience of Ménière’s disease.

 

Treatment for Ménière’s Disease

The following is general information only. HHF does not offer medical advice. Please consult your hearing care professional with any specific questions about your auditory health and healthcare.

A cure for Ménière's disease does not yet exist, but lifestyle modifications and treatments can help patients. According to the NIDCD, “Scientists estimate that six out of 10 people either get better on their own or can control their vertigo with diet, drugs, or devices. However, a small group of people with Ménière’s disease will get relief only by undergoing surgery.”

Treatments include reducing the body’s retention of fluids through dietary changes (e.g., eliminating or reducing salt, caffeine, and/or alcohol). Medications such as antihistamines, anticholinergics, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid. Eliminating tobacco use and reducing stress levels may also help.

Hearing Health Foundation-funded scientists continue to investigate causes to find better treatments. Emerging Research Grants scientist Bryan K. Ward, M.D., reviewed surgical interventions in a paper published in Frontiers in Neurology in 2021.

  • Medical therapy is directed at treating the underlying disorder and controlling the symptoms. The primary method of treating the underlying hydrops (distension of the endolymphatic space) is to implement a low-sodium diet and diuretics to reduce fluid retention and, as a result, inner ear fluid pressure. A variety of vestibular suppressants, ranging from antihistamines to benzodiazepines, are utilized to control patients’ acute symptoms. Most patients are well controlled on medical therapy and require no surgical intervention.

  • One of the earliest interventions is intratympanic steroids. A steroid solution is placed directly into the middle ear, allowing for passive perfusion into the inner ear via the round window. Intratympanic steroids are often offered to patients with episodic vertigo, sensorineural hearing loss, and other classic symptoms of Ménière’s disease that do not respond well to medical management. Like any treatment for the condition, intratympanic steroids have variable effects among patients. They have been popular because of their low risk and easy administration in the doctor’s office. Many patients often prefer intratympanic steroids on a frequent basis as opposed to taking daily diuretics. Intratympanic steroids are associated with fewer side effects than systemic steroids.

  • The Meniett device was approved by the Food and Drug Administration in 1999 and requires a tympanostomy tube to be placed in the ear drum. A self-administered pressure device, the Meniett is inserted into the ear canal and applies intermittent, alternating air pressure pulses to the middle ear in order to reduce the fluid pressure that causes dizziness. While it showed early promise as a conservative treatment option, it is now used less and less frequently due to other treatments.

  • Endolymphatic sac surgery is done in the operating room under general anesthesia and is classified as a conservative procedure because it retains the function of the inner ear. By decompressing the endolymphatic sac, there is a chance to decompress the functional component of the inner ear, reducing symptoms. This procedure’s success rate is around 80% according to “Endolymphatic Sac Surgery for Ménière's Disease—Current Opinion and Literature Review,” published in International Archives of Otorhinolaryngology in 2017.

  • Another option is intratympanic gentamicin. Gentamicin is an antibiotic with a known side effect of hearing loss and a capacity to destroy the vestibular system. It is delivered specifically to the middle ear with the intent to reduce the function of the vestibular system in that ear. It is titrated so that hearing can be preserved while vestibular function is reduced or eliminated.

  • A vestibular nerve section requires surgically opening the skull. The balance portion of the 8th (auditory-vestibular) nerve is cut, sparing the hearing portion. It is now rarely performed due to the advent of transtympanic steroids and gentamicin therapy.

  • Labyrinthectomy is the surgical removal of the inner ear and is definitive in eliminating vestibular (inner ear) function. It remains the gold standard for treating Ménière’s disease patients with poor hearing but is generally only used after other treatment options have failed.

  • Hearing aids: Even though hearing aids do not cure Ménière’s disease, they can sometimes help to alleviate the hearing loss, one of the hallmark features of the disease. Consult with your hearing care provider to learn if hearing aids would be effective in managing your Ménière’s disease.

According to the NIDCD, other treatments include reducing the body’s retention of fluids through dietary changes (e.g., eliminating or reducing salt, caffeine, and/or alcohol). Medications such as antihistamines, anticholinergics, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid. Eliminating tobacco use and reducing stress levels may also help.

HHF offers general information only and does not offer medical advice. Please consult your hearing care professional with any specific questions about your auditory health and healthcare.

 

How Balance Works

The inner ear is a tiny but notable body part; not only is it important to hearing, but it is also where the balance organs and nerves are located.

The basic components of the inner ear include semicircular canals, the cochlea, the utricle, the saccule, and the vestibulocochlear nerve. The cochlea and one half of the vestibulocochlear nerve (the cochlear nerve) are responsible for hearing. The remaining semicircular canals, utricle, saccule, and vestibular nerve are responsible for balance.

There are three semicircular canals that contain fluid to activate sensory hair cells, which are arranged at 90-degree angles and detect different kinds of movement:

  • Up and down

  • Side to side

  • Tilting

The utricle connects the semicircular canals to the saccule, which also detect motion. They are located in the vestibule inside of the labyrinth, which is the bony outer wall of the inner ear. All of this is the vestibular system.

But it is not only the vestibular system that assists with balance. Vision and sensory receptors (muscles, joints, skin, etc.) all transmit messages to the brain that work together to regulate balance.

Emerging Research Grants scientist and Hearing Restoration Project member Jennifer Stone, Ph.D., provides an overview of the vestibular system in this Hearing Health Hour webinar and Hearing Health magazine article.

 

Our Research on Ménière’s Disease

Thanks to the generosity of our donors, Hearing Health Foundation (HHF) funds groundbreaking research to advance our scientific understanding of the often debilitating inner ear and balance disorder Ménière's disease through the Emerging Research Grants (ERG) program.

For an overview of Ménière’s disease and its definition, evaluation, and interventions, please click on the video recording of the webinar above featuring ERG scientist Wafaa Kaf, M.D., Ph.D., and including answers to additional questions.

Reviewed in June 2023 by Rohima Badri, Ph.D.

 

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