Meniere's disease is a disorder of the inner ear that affects balance and hearing. The most common symptoms include vertigo (dizziness), hearing loss, tinnitus (ringing in the ear), and a feeling of fullness in the ear. In the early stages, Meniere's disease is usually unilateral (occurring on one side). There is no predominance of right or left ear. Meniere's disease is also called idiopathic endolymphatic hydrops. The cause of Meniere's disease is unknown. It was first described by a French physician, Dr. Prosper Meniere, in 1861.

There have been many hypotheses regarding the underlying causes of Meniere's disease since its first description in 1861, including:

• Genetic predisposition - Approximately 5%-20% of individuals diagnosed with Meniere's disease report that a family member has or had Meniere's disease or similar symptoms. In addition, chromosomal studies appear to be making progress in identifying certain genetic markers in individuals diagnosed with Meniere's disease.

• Allergies - Individuals with Meniere's disease report higher rates of allergies than people not affected with Meniere's, and some studies have shown higher rates of serum immune markers in individuals with Meniere's disease than those without. In addition, studies have shown that individuals with allergies and Meniere's disease report an improvement in the severity and duration of their Meniere's attacks when treated with immunotherapy and/or dietary adjustments.

• Migraine headaches - There appears to be an association between Meniere's disease and migraine headaches. Reports in one study indicated that the prevalence of migraine in individuals with Meniere's disease was 56%, compared to 10% among the general population; and that 45% of individuals with Meniere's disease always experienced at least one symptom of migraine headaches (e.g., aura, or an aversion to light) during Meniere's attacks.

• Otosclerosis - Some individuals with otosclerosis (a rare condition of formation of spongy bone around the stapes) have symptoms of Meniere's disease. It is thought that otosclerotic formations may cause malformation in the endolymphatic sac or may change the chemical properties of the perilymphatic and endolymphatic fluid.

Although the exact underlying cause of Meniere's disease is not clear, it has been associated with an excess of endolymphatic fluid within the cochlear duct and vestibular system - a condition known as -endylymphatic hydrops-. It is also not clear whether endolymphatic hydrops is the cause or the result of Meniere's disease, since there are known cases of individuals with endolymphatic hydrops who otherwise have no symptoms of Meniere's disease.

While Meniere's disease is the second most common cause of vertigo in the U.S., it is listed in the National Organization of Rare Diseases (NORD) database as a rare disease. Information regarding the precise incidence and prevalence of Meniere's disease is scarce, but some estimates range from 15 to 150 cases per 100,000 people.

Additional important statistics about Meniere's disease include:

• Meniere's disease most often affects adults between the ages of 40 and 60.
• Approximately 40,000 new cases of Meniere's disease are diagnosed annually in the U.S.
• Approximately 3% of patients diagnosed with Meniere's disease are children.
• Male-to-female ratio ranges from equal to a slight preponderance of females over males. Some women report improvement of symptoms following pregnancy.
• Although most cases of Meniere's disease are usually unilateral (occurring on one side only), up to 10% of individuals have bilateral Meniere's disease affecting both ears at the time of diagnosis. Over the course of time, approximately 50% of individuals also develop symptoms in the opposite ear.

There is no consistent pattern for the progression of Meniere's disease. It may or may not be progressive. For many people, vertigo attacks occur for the first two to three years, during which vertigo can usually be controlled by medication. In approximately one out of four individuals, progression of vertigo continues despite medical treatment and reaches a point where the vertigo is refractory (not responsive) to treatment and is incapacitating. At that point, individuals may be considered candidates for surgery.

The diagnosis of Meniere's disease is based on:

• History and physical examination
• Laboratory testing
• Audiometric (hearing) studies
• Balance tests
• Imaging studies

The diagnosis of Meniere's disease can be challenging because there are many other conditions that can present with similar clinical symptoms. Some of the conditions that need to be considered in the differential diagnosis of Meniere's disease include:

• Benign paroxysmal positional vertigo (BPPV) - Episodes of short-lasting vertigo that are usually brought on by movement and are not associated with hearing loss or tinnitus.
• Acoustic neuroma - A slow-growing tumor of the auditory nerve that can cause symptoms similar to Meniere's disease.
• Vestibular neuritis - A viral inflammation of the vestibular nerve that causes vertigo.
• Labyrinthitis - Inflammation of the labyrinth that causes dizziness, imbalance, and temporary hearing loss.
• Acute hearing loss - Hearing loss that could be idiopathic (for no observable reason).
• Migraine headaches - Severe headaches that can cause dizziness.

Although currently there is no cure for Meniere's disease, there are many treatments available to manage individual symptoms, especially vertigo, the single symptom that people report as being the most disruptive in their daily lives. The goals of treatment are to relieve the symptoms, to prevent or minimize vertigo attacks, and to help people suffering from vertigo achieve as little disruption of their daily activities as possible. Psychological support is beneficial as it helps individuals with Meniere's disease learn to better cope with symptoms and their impact on quality of life.

In general, the treatment options for Meniere's disease include:

• Restriction of dietary salt intake
• Medications to prevent and treat vertigo
• Intratympanic injections of steroids or gentamicin
• Surgical therapy for patients with unremitting attacks of vertigo that cannot be controlled with medications.

The prognosis of Meniere's disease varies significantly from patient to patient. There is no cure for the condition, only symptom management. Meniere's disease usually affects only one ear and estimates vary widely regarding the number of people who develop bilateral Meniere's disease. Surgery is performed in up to 10% of patients with Meniere's disease, typically those who do not respond to more conservative treatments and who continue to experience disabling episodes of vertigo.

Knowledge is Critical when Dealing with a Life-Altering Condition such as Meniere's Disease

If you or a loved one has been diagnosed with Meniere's disease, it's critical to learn everything you possibly can about this condition so that you can make informed decisions about your treatment. That's why we created the Medifocus Guidebook on Meniere's Disease, a comprehensive 144 page patient Guidebook that contains vital information about Meniere's disease that you won't find anywhere in a single source.

The Medifocus Guidebook on Meniere's Disease starts out with a detailed overview of the condition and quickly imparts fundamentally important information about Meniere's disease, including:

• The theories regarding the underlying causes of Meniere's disease.
• The triggers that may precipitate an acute attack of Meniere's disease in some patients.
• How Meniere's disease is diagnosed based on factors such as signs/symptoms, patient history, physical examination, and audiometric (hearing) studies.

• Specific balance tests that are often used to confirm the presence of vertigo associated with Meniere's disease. These balance tests may include:

  • Electronystagmogram
  • Sharpened Romberg test
  • Dix-Hallpike maneuver
  • Rotational test

• The stages in the progression of Meniere's disease from initial signs/symptoms to severe, incapacitating vertigo attacks and complete hearing loss, if left untreated

Understanding the Standard Treatments...and the Treatment Options

Because currently there is no known cure for Meniere's disease, the goal of treatment is to control and relieve the symptoms - particularly vertigo - that patient's with Meniere's disease report as the single, most bothersome symptom. Controlling the symptoms of Meniere's disease is important because it enables patients to continue to perform routine activities of daily living and maintain a reasonably good quality of life. Understanding the treatments - and the treatment options - is critical for successfully achieving both control of symptoms and preserving quality of life. As you read through the section of the Guidebook that focuses on the treatments for Meniere's disease, you will specifically learn about:

• The medications that are commonly prescribed for the management of acute vertigo attacks in patients with Meniere's disease.
• A new implantable device, called the Meniett device, that delivers intermittent pulses of positive air pressure to the inner ear and has been shown to significantly reduce the severity of vertigo in people with Meniere's disease.

• The surgical treatment options that are available for patients with Meniere's disease who experience severe, unremitting episodes of vertigo that cannot be controlled with more conservative treatments. These surgical treatments include:

  • Endolymphatic sac surgery
  • Labyrinthectomy
  • Vestibular neurectomy

• The risks, benefits, and complications, associated with the various surgical techniques that are used for the treatment of Meniere's disease.

• The role of cochlear implants and vestibular rehabilitation in the management of people with Meniere's disease.
• Nutritional and lifestyle modifications that can help you better control the symptoms of Meniere's disease.
• The role of complementary and alternative therapies in the management of Meniere's disease.
• Important questions to ask your doctor about Meniere's disease.

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Updated: January 18, 2023
144 Pages