Ménière’s disease is a disorder of the inner ear that causes severe dizziness (vertigo), ringing in the ears (tinnitus), hearing loss, and a feeling of fullness or congestion in the ear. Ménière’s disease usually affects only one ear.
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:
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Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours
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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
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Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear
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Not better accounted for by any other vestibular diagnosis
Probable Meniere disease can include the following clinical findings:
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Two or more episodes of dizziness or vertigo, each lasting 20 minutes to 24 hours
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Fluctuating aural symptoms (fullness, hearing, or tinnitus) in the affected ear
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The condition is better explained by another vestibular diagnosis[2]
What causes the symptoms of Ménière’s disease?
The labyrinth in relation to the ear
The labyrinth is composed of the semicircular canals, the otolithic organs (i.e., utricle and saccule), and the cochlea. Inside their walls (bony labyrinth) are thin, pliable tubes and sacs (membranous labyrinth) filled with endolymph.
Studies of the temporal bone revealed endolymphatic accumulation in the cochlea and the vestibular organ in patients with Meniere disease. Current research links endolymphatic hydrops to a hearing loss of >40dB. Vertigo may or may not be associated.[rx] Therefore endolymphatic hydrops is not entirely specific for Meniere disease and can be found in cases of idiopathic sensorineural hearing loss.
1) Migraine: Migraine occurs more often in patients diagnosed with Meniere disease, although there might be an overlap between basilar migraine wrongly diagnosed as Meniere disease.[rx]
2) Autoimmune diseases: Several autoimmune diseases are associated with Meniere disease, namely rheumatoid arthritis, systemic lupus erythematosus, and ankylosing spondylitis.[rx]
3) Genetic component: Meniere disease is a polygenic disorder. Ten percent of cases of patients of European descent have familial Meniere disease. MD may show autosomal dominant or autosomal recessive inheritance but may be sporadic.
Symptoms
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
At the emergency room or in the general practice the physician will differentiate between the vertigo of central, peripheral, and cardiovascular causes. Red flags for a central origin of vertigo, according to Harcourt et al., are neurological symptoms or signs, acute deafness, a new type or onset of headache, or vertical/torsional/rotatory nystagmus.[rx]
If Meniere disease is suspected, the patient should be questioned about the character of vertigo, hearing loss, and earlier episodes. A full otologic history is part of the clinical investigation.
If Meniere disease is suspected, one should perform a full otologic examination, facial nerve testing, and assessment of nystagmus with Frenzel goggles, Rinne, and Weber tests.
Rinne and Weber: Will show sensorineural hearing loss in acute Meniere disease or advanced disease.
Frenzel goggles: May show horizontal nystagmus with a fast-beating component away from the affected vestibular organ in the acute setting.
Head impulse testing (HIT): In contrast to other peripheral vestibular disorders, this test has a low sensitivity in Meniere disease.[rx]
Audiometric evaluation is mandatory in all patients with Meniere disease. Fluctuating low-frequency unilateral sensorineural hearing loss is characteristic of the disease. The hearing loss can progress to all frequencies. Tinnitus is common and ipsilateral.[rx]
Hearing assessment
A hearing test (audiometry) assesses how well you detect sounds at different pitches and volumes and how well you distinguish between similar-sounding words. People with Meniere’s disease typically have problems hearing low frequencies or combined high and low frequencies with normal hearing in the midrange frequencies.
Balance assessment
Between episodes of vertigo, the sense of balance returns to normal for most people with Meniere’s disease. But you might have some ongoing balance problems.
Tests that assess function of the inner ear include:
- Videonystagmography (VNG). This test evaluates balance function by assessing eye movement. Balance-related sensors in the inner ear are linked to muscles that control eye movement. This connection enables you to move your head while keeping your eyes focused on a point.
- Rotary-chair testing. Like a VNG, this measures inner ear function based on eye movement. You sit in a computer-controlled rotating chair, which stimulates your inner ear.
- Vestibular-evoked myogenic potentials (VEMP) testing. This test shows promise for not only diagnosing, but also monitoring Meniere’s disease. It shows characteristic changes in the affected ears of people with Meniere’s disease.
- Posturography. This computerized test reveals which part of the balance system — vision, inner ear function, or sensations from the skin, muscles, tendons and joints — you rely on the most and which parts may cause problems. While wearing a safety harness, you stand in bare feet on a platform and keep your balance under various conditions.
- Video head impulse test (vHIT). This newer test uses video to measure eye reactions to the abrupt movement. While you focus on a point, your head is turned quickly and unpredictably. If your eyes move off the target when your head is turned, you have an abnormal reflex.
- Electrocochleography (ECoG). This test looks at the inner ear in response to sounds. It might help to determine if there is an abnormal buildup of fluid in the inner ear, but isn’t specific to Meniere’s disease.
- Audiometric exam. This will find hearing loss in the affected ear. It might include a test to gauge your ability to tell the difference between words like “fit” and “sit.” That’s called speech discrimination.
- Electronystagmogram. This evaluates your balance. You will be placed in a darkened room and have your eye movements measured as cool and warm air blows through your ear canal.
- Electrocochleography. This measures fluid pressure in your inner ear.
- Video head impulse test (VHIT). This uses video images to see how well you can focus and how your eyes respond to sudden movement.
- Auditory brainstem response test (ABR). With this test, you wear headphones, and a computer measures your brain waves as you respond to different sounds. It’s typically only used for people who can’t have other types of hearing tests (like babies) or who can’t have imaging tests.
- Additional imaging tests. Your doctor also might recommend an MRI or CT scan to rule out the possibility that something other than Meniere’s is causing your symptoms.
All patients with one-sided hearing loss should undergo magnetic resonance imaging (MRI) to rule out retrocochlear pathology. In some countries a BERA (brainstem-evoked response audiometry) is sufficient. There is no need to perform imaging in the acute setting but may be done within a few weeks after the onset of symptoms. High-resolution MRI imaging may directly show endolymphatic hydrops in the affected organs. More research is underway to show if this is of clinical use.[rx][rx]
Vestibular (caloric) function testing may show a significantly under-functioning affected organ in 42% to 74% and a full loss of function in 6% to 11%.[rx]
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.
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Sodium restriction diet: Low-level evidence suggests that restricting sodium intake may help to prevent Meniere attacks.[rx]
- 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]
- 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]
- 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.
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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.
Prevention
Medicines
A GP may recommend a medicine called betahistine to help reduce the frequency and severity of attacks of Ménière’s disease.
Betahistine is thought to reduce the pressure of the fluid in your inner ear, relieving symptoms of hearing loss, tinnitus and vertigo.
Foods to avoid
There’s not much proof that changes to your diet can help.
But some people claim their symptoms improve by:
- eating a low-salt diet
- avoiding alcohol
- avoiding caffeine
- stopping smoking
Driving and other risks
Because you cannot predict when your next attack might happen, you may need to change how you do things to avoid putting yourself or others in danger.
Consider the risks before doing activities such as:
- driving
- swimming
- climbing ladders or scaffolding
- operating heavy machinery
You may also need to make sure someone’s with you most of the time in case you need help during an attack.
Driving
You should not drive when you feel dizzy or if you feel an attack of vertigo coming on.
You must inform the Driver and Vehicle Licensing Agency (DVLA) if you’re prone to sudden attacks of vertigo without any warning signs.
It’s likely that you will not be allowed to continue driving until you have control of your symptoms.
Flying
Most people with Ménière’s disease have no difficulty with flying.
These tips may help any anxiety you feel about flying, which may reduce the risk of an attack:
- get an aisle seat if you’re worried about vertigo – you’ll be away from the window and will have quicker access to the toilets
- sit away from the plane’s engines if noise and vibration are an issue
- drink water regularly, to stay hydrated, and avoid alcohol
- ask if the airline can offer food for a special diet that suit your needs
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.
References
