Meniere’s Disease

Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people will have single attacks of dizziness separated by long periods of time. Others may experience many attacks closer together over a number of days. Some people with Ménière’s disease have vertigo so extreme that they lose their balance and fall. These episodes are called “drop attacks.”

The current diagnostic criteria defined by the Barany society by Lopez-Escamez et al. can help differentiate between a probable and a definite Meniere’s disease.

Patients with a definite Meniere disease according to the Barany Society have:

1. Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours
2. Audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on in at least one instance prior, during, or after one of the episodes of vertigo
3. Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear
4. Not better accounted for by any other vestibular diagnosis?

Probable Meniere disease can include the following clinical? findings:

1. Two or more episodes of dizziness or vertigo, each lasting 20 minutes to 24 hours
2. Fluctuating aural symptoms (fullness, hearing, or tinnitus) in the affected ear
3. The condition is better explained by another vestibular diagnosis[2]

What causes the symptoms of Ménière’s disease?

The symptoms of Ménière’s disease are caused by the buildup of fluid in the compartments of the inner ear, called the labyrinth. The labyrinth contains the organs of balance (the semicircular canals and otolithic organs) and of hearing (the cochlea). It has two sections: the bony labyrinth and the membranous labyrinth. The membranous labyrinth is filled with a fluid called endolymph that, 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.

Signs and symptoms of Meniere’s disease include:

• Recurring episodes of vertigo. You have a spinning sensation that starts and stops spontaneously. Episodes of vertigo occur without warning and usually last 20 minutes to several hours, but not more than 24 hours. Severe vertigo can cause nausea?.
• Hearing loss. Hearing loss in Meniere’s disease may come and go, particularly early on. Eventually, most people have some permanent hearing loss.
• Ringing in the ear (tinnitus). Tinnitus is the perception of a ringing, buzzing, roaring, whistling or hissing sound in your ear.
• Feeling of fullness in the ear. People with Meniere’s disease often feel pressure in an affected ear (aural fullness).

In Ménière’s disease, the endolymph buildup in the labyrinth interferes with the normal balance and hearing signals between the inner ear and the brain. This abnormality? causes vertigo and other symptoms of Ménière’s disease.

• feel dizziness with a spinning sensation (vertigo)
• feel unsteady on your feet
• feel sick (nausea) or be sick (vomit)
• hear ringing, roaring or buzzing inside your ear
• have a sudden drop in hearing

Why do people get Ménière’s disease?

Many theories exist about what happens to cause Ménière’s disease, but no definite answers are available. Some researchers think that Ménière’s disease is the result of constrictions in blood vessels similar to those that cause migraine headaches. Others think Ménière’s disease could be a consequence of viral? infections, allergies, or autoimmune reactions. Because Ménière’s disease appears to run in families, it could also be the result of genetic variations that cause abnormalities in the volume or regulation of endolymph fluid.

How does a doctor diagnose Ménière’s disease?

Ménière’s disease is most often diagnosed and treated by an otolaryngologist (commonly called an ear, nose, and throat? doctor, or ENT). However, there is no definitive test or single symptom that a doctor can use to make the diagnosis. Diagnosis is based upon your medical history? and the presence of:

• Two or more episodes of vertigo lasting at least 20 minutes each
• Tinnitus
• Temporary hearing loss
• A feeling of fullness in the ear

Some doctors will perform a hearing test to establish the extent of hearing loss caused by Ménière’s disease. To rule out other diseases, a doctor also might request magnetic resonance imaging (MRI) or computed tomography (CT) scans of the brain.

How is Ménière’s disease treated?

Different treatment options for Meniere disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with non-invasive approaches with the fewest possible side effects and proceed to more invasive steps.

1. Sodium restriction diet: Low-level evidence suggests that restricting sodium intake may help to prevent Meniere attacks.[rx]
2. Diet – People with MD are often advised to reduce their sodium intake. Reducing salt intake, however, has not been well studied.^[rx]Based on the assumption that MD is similar in nature to a migraine, some advise eliminating “migraine triggers” such as caffeine, but the evidence for this is weak.^[rx] There is no high-quality evidence that changing diet by restricting salt, caffeine or alcohol improves symptoms.^[rx]
3. Physical therapy – While the use of physical therapy early after the onset of MD is probably not useful due to the fluctuating disease course, physical therapy to help to retrain of the balance system appears to be useful to reduce both subjective and objective deficits in balance over the longer term.^[rx][rx]
4. Counseling – psychological distress caused by vertigo and hearing loss may worsen the condition in some people.^[rx] Counseling may be useful to manage the distress,^[rx] as may education and relaxation techniques.^[rx]

Ménière’s disease does not have a cure yet, but your doctor might recommend some of the treatments below to help you cope with the condition.

• Medications. The most disabling symptom of an attack of Ménière’s disease is dizziness. Prescription drugs such as meclizine, diazepam, glycopyrrolate, and lorazepam can help relieve dizziness and shorten the attack.
• Betahistine: Substantial disagreement in the medical community about the use of betahistine exists. A Cochrane review found low-level evidence to support the use of betahistine with substantial variability between studies.[rx] Medical therapy in many medical centers often starts with betahistine orally. Intratympanic steroid injections may reduce the number of vertigo attacks in patients with Meniere disease.[rx]
• Diuretics: This medication reduces the amount of fluid in your body. Reducing fluid overall may bring down inner ear fluid levels.
• Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. Side effects are sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.[rx]
• Salt restriction and diuretics. Limiting dietary salt and taking diuretics (water pills) help some people control dizziness by reducing the amount of fluid the body retains, which may help lower fluid volume and pressure in the inner ear.
• Other dietary and behavioral changes. Some people claim that caffeine, chocolate, and alcohol make their symptoms worse and either avoid or limit them in their diet. Not smoking also may help lessen the symptoms.
• Cognitive therapy. Cognitive therapy is a type of talk therapy that helps people focus on how they interpret and react to life experiences. Some people find that cognitive therapy helps them cope better with the unexpected nature of attacks and reduces their anxiety about future attacks.
• Injections. Injecting the antibiotic gentamicin into the middle ear helps control vertigo but significantly raises the risk of hearing loss because gentamicin can damage the microscopic hair cells in the inner ear that help us hear. Some doctors inject a corticosteroid instead, which often helps reduce dizziness and has no risk of hearing loss.
• Pressure pulse treatment. The U.S. Food and Drug Administration (FDA) recently approved a device for Ménière’s disease that fits into the outer ear and delivers intermittent air pressure pulses to the middle ear. The air pressure pulses appear to act on endolymph fluid to prevent dizziness.
• Surgery. A nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to complete hearing loss in the affected side.[rx]Surgery may be recommended when all other treatments have failed to relieve dizziness. Some surgical procedures are performed on the endolymphatic sac to decompress it. Another possible surgery is to cut the vestibular nerve, although this occurs less frequently.
• Alternative medicine. Although scientists have studied the use of some alternative medical therapies in Ménière’s disease treatment, there is still no evidence to show the effectiveness of such therapies as acupuncture or acupressure, tai chi, or herbal supplements such as ginkgo Biloba, niacin, or ginger root. Be sure to tell your doctor if you are using alternative therapies since they sometimes can impact the effectiveness or safety of conventional medicines.

Long-term medication use

Your doctor may prescribe a medication to reduce fluid retention (diuretic) and suggest that you limit your salt intake. For some people, this combination helps control the severity and frequency of Meniere’s disease symptoms.

Noninvasive therapies and procedures

Some people with Meniere’s disease may benefit from other noninvasive therapies and procedures, such as:

• Rehabilitation. If you have balance problems between episodes of vertigo, vestibular rehabilitation therapy might improve your balance.
• Hearing aid. A hearing aid in the ear affected by Meniere’s disease might improve your hearing. Your doctor can refer you to an audiologist to discuss what hearing aid options would be best for you.
• Positive pressure therapy. For vertigo that’s hard to treat, this therapy involves applying pressure to the middle ear to lessen fluid buildup. A device called a Meniett pulse generator applies pulses of pressure to the ear canal through a ventilation tube. You do the treatment at home, usually three times a day for five minutes at a time.Positive pressure therapy has shown improvement in symptoms of vertigo, tinnitus and aural pressure in some studies, but not in others. Its long-term effectiveness hasn’t been determined yet.

If the conservative treatments listed above aren’t successful, your doctor might recommend some of these more-aggressive treatments.

Middle ear injections

Medications injected into the middle ear, and then absorbed into the inner ear, may improve vertigo symptoms. This treatment is done in the doctor’s office. Injections available include:

• Gentamicin, an antibiotic that’s toxic to your inner ear, reduces the balancing function of your ear, and your other ear assumes responsibility for balance. There is a risk, however, of further hearing loss.
• Steroids, such as dexamethasone, also may help control vertigo attacks in some people. Although dexamethasone may be slightly less effective than gentamicin, it’s less likely than gentamicin to cause further hearing loss.

Surgery

If vertigo attacks associated with Meniere’s disease are severe and debilitating and other treatments don’t help, surgery might be an option. Procedures include:

• Endolymphatic sac procedure. The endolymphatic sac plays a role in regulating inner ear fluid levels. During the procedure, the endolymphatic sac is decompressed, which can alleviate excess fluid levels. In some cases, this procedure is coupled with the placement of a shunt, a tube that drains excess fluid from your inner ear.
• Labyrinthectomy. With this procedure, the surgeon removes the balance portion of the inner ear, thereby removing both balance and hearing function from the affected ear. This procedure is performed only if you already have near-total or total hearing loss in your affected ear.
• Vestibular nerve section. This procedure involves cutting the nerve that connects balance and movement sensors in your inner ear to the brain (vestibular nerve). This procedure usually corrects problems with vertigo while attempting to preserve hearing in the affected ear. It requires general anesthesia and an overnight hospital stay.

What is the outlook for someone with Ménière’s disease?

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.

What research about Ménière’s disease is being done?

Insights into the biological mechanisms in the inner ear that cause Ménière’s disease will guide scientists as they develop preventive strategies and more effective treatment. The NIDCD is supporting scientific research across the country that is:

• Determining the most effective dose of gentamicin with the least amount of risk for hearing loss.
• Developing an in-ear device that uses a programmable microfluid pump (the size of a computer chip) to precisely deliver vertigo-relieving drugs to the inner ear.
• Studying the relationship between endolymph volume and inner ear function to determine how much endolymph is “too much.” Researchers are hoping to develop methods for manipulating inner ear fluids and treatments that could lower endolymph volume and reduce or eliminate dizziness.

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