Meniere’s Disease – Causes, Symptoms, Diagnosis, Treatment

• Stages of Meniere’s Disease
• Causes of Meniere’s Disease
• Symptoms of Meniere’s Disease
• Diagnosis of Meniere’s Disease
• Treatment of Meniere’s Disease

Meniere’s disease is a disease of the inner ear, characterized by the clinical triad of recurrent vertigo, fluctuating sensorineural hearing loss, and tinnitus.[rx] The relapsing nature of the disease may significantly affect the patients’ quality of life, especially during periods of acute symptomatology.[rx,rx] Vertigo mainly influences the physical dimension, while tinnitus and hearing loss influence the psychosocial dimension of patients’ lives.[rx]

Meniere disease is a disorder of the inner ear characterized by hearing loss, tinnitus, and vertigo. In most cases, it is slowly progressive and has a significant impact on the social functioning of the individual affected.[rx]

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

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

• Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours
• 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
• Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear
• Not better accounted for by any other vestibular diagnosis

Probable Meniere disease can include the following clinical findings

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

Stages of Meniere’s Disease

Meniere’s disease commonly affects people in various stages, with symptoms developing over time.

• Early stage – During this time, a person will experience sudden and often out-of-the-blue episodes of vertigo that last anywhere from 20 minutes to an entire day. An person’s ear may feel blocked or full, and they may have some hearing loss, which typically goes away after the episode fades. It is also common to feel the effects of tinnitus.
• Middle stage – Symptoms of vertigo tend to become less severe during this stage, while hearing loss and tinnitus will increase in severity. Many people will also experience long-term remission (the disease goes away) that can last several months.
• Late stage – During the late stages of Meniere’s disease, patients will not suffer from vertigo as often, and some people will be relieved from it forever. However, tinnitus and hearing loss will likely get progressively worse, and people will likely experience unsteady balance regularly. Most people at this stage feel unstable in dark conditions, for example.

Causes of Meniere’s Disease

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.

• The exact etiology of Meniere disease remains unclear – Different theories exist, but genetic and environmental factors play a role. The relation to common comorbidities remains elusive.
• 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]
• Autoimmune Diseases – Several autoimmune diseases are associated with Meniere disease namely rheumatoid arthritis, systemic lupus erythematosus and ankylosing spondylitis.[rx]

Potential causes or triggers of Meniere’s disease include:

• Head injury
• Infection to the inner or middle ear
• Allergies
• Alcohol use
• Stress
• Side effects of certain medications
• Smoking
• Stress or anxiety
• Fatigue
• Family history of the disease
• Respiratory infection
• Recent viral illness
• Abnormal immune response
• Migraines

Symptoms of Meniere’s Disease

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).

A person with Ménière’s disease may experience any or all of these symptoms

• Vertigo and dizziness, often so severe that it is temporarily disabling. There may be a sense that the room is spinning, twisting, or rocking. Balance can be severely affected. The sensation can last from a few minutes to several hours. After vertigo goes away, a sense of imbalance can remain for hours or days.
• Nausea and vomiting during an episode of vertigo.
• A feeling of pressure or fullness in the affected ear.
• Ringing, buzzing, or other noises in the affected ear (tinnitus). This ringing is often low-pitched and may distort normal sounds.
• Hearing loss that comes and goes but gets progressively worse over time. Low-pitched hearing often is affected earlier in the disease.

Paying attention to these warning symptoms can allow a person to move to a safe or more comfortable situation before an attack.

• balance disturbance
• dizziness, lightheadedness
• headache, increased ear pressure
• hearing loss or tinnitus increase
• sound sensitivity
• vague feeling of uneasiness

During an attack of early-stage Ménière’s disease, symptoms include:

• spontaneous, violent vertigo
• fluctuating hearing loss
• ear fullness (aural fullness) and/or tinnitus

In addition to the above main symptoms, attacks can also include:

• anxiety, fear
• diarrhea
• blurry vision or eye jerking
• nausea and vomiting
• cold sweat, palpitations or rapid pulse
• trembling

Following the attack, a period of extreme fatigue or exhaustion often occurs, prompting the need for hours of sleep.

The periods between attacks are symptom free for some people and symptomatic for others. Many symptoms have been reported after and between attacks:

• anger, anxiety, fear, worry
• appetite change
• clumsiness
• concentration difficulty, distractibility, tendency to grope for words
• diarrhea
• fatigue, malaise, sleepiness
• headache, heavy head sensation
• lightheadedness (faintness)
• loss of self-confidence and self-reliance
• nausea, queasiness, motion sickness
• neck ache or stiff neck
• palpitations or rapid pulse, cold sweat
• sound distortion and sensitivity
• unsteadiness (sudden falls, staggering or stumbling, difficulty turning or walking in poorly lit areas, tendency to look down or to grope for stable handholds)
• vision difficulties (problems with blurring, bouncing, depth perception, glare intensification, focusing, watching movement; difficulty looking through lenses such as binoculars or cameras)
• vomiting

Diagnosis of Meniere’s Disease

History and Physical

In the emergency room or in the general practice the physician will differentiate between the vertigo of central, peripheral, and cardiovascular cause. Red flags for a central origin of vertigo, according to Harcourt et al., are neurological symptoms or signs, acute deafness, 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]

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 for Meniere’s disease.

Tests that may be used to aid in diagnosis include

• A hearing test, also called audiometry — This simple test can tell whether you are experiencing hearing problems, how much hearing you have lost, and what type of hearing problems you have. People with Ménière’s disease have a particular type of damage to nerves important for normal hearing, which may make it difficult to tell the difference between similar-sounding words such as “boat” and “moat.”
• Computed tomography (CT) – or magnetic resonance imaging (MRI), scans that allow physicians to see the brain, middle ear, and other structures inside the head — These scans can check for tumors and other problems that can cause symptoms that are similar to Ménière’s.
• Electronystagmography or rotational testing — These tests use the nerve connection between the ears and the eyes to examine your body’s balance system. In a darkened room, electrodes are placed near the eyes. Then, the ear canal is stimulated with water, air or changes in position. The electrodes measure how the inner ear responds. In Ménière’s disease, your doctor can spot typical changes caused by the buildup of fluid in the inner ear.

Evaluation

• 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]
• 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 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]
• ECochG – has also been widely used in the diagnosis of Meniere’s disease. During the EcoChG, a needle electrode is placed either through the tympanic membrane on the promontory, or on the tympanic membrane, or simply in the ear canal.
• 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.
• Vestibular-evoked myogenic potential (VEMP) – This measures your reaction to sudden, loud noises.
• 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.

The components measured are

• a) cochlear microphonics,
• b) summating potentials (SP), and
• c) action potentials (AP). The cochlear microphonics and the summating potentials reflect the cochlear bioelectric activity, while the action potentials reflect the activity of distal afferent fibers of the 8th nerve. In ECoChG, we determine the amplitude of the SP and the AP from a common baseline.

Treatment of Meniere’s Disease

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.

• Sodium restriction diet – Low-level evidence suggests that restricting sodium intake may help to prevent Meniere attacks.[rx]
• 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]
• Intratympanic gentamycin injections – Gentamycin has strong ablative properties towards vestibular cells. Side effects are a sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.[rx]
• Vestibular nerve section or labyrinthectomy – Nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to a complete hearing loss on the affected side.[rx]
• Anti-vertigo medications – such as meclizine or betahistine, to relieve or prevent vertigo and dizziness
• Anti-nausea medications – such as prochlorperazine, to relieve nausea and vomiting
• Diuretics, such as hydrochlorothiazide (HydroDIURIL) – reduce the amount of fluid that builds in the inner ear.
• Meclizine, chewable – Dose ranges from 12.5 twice/day to 50 mg three times/day. This medication is over the counter. No prescription is necessary.
• Lorazepam (Ativan) 0.5 mg – The usual dose is twice/day or both at the same time at the onset. This medication is effective even if it is not swallowed (i.e. you can just suck on it). Tiredness is expected.
• Promethazine (Phenergan) – orally (12.5) or rectal suppository (25 mg). The usual dose is once every 12 hours as needed for vomiting.
• Prochlorperazine (Compazine, orally or suppository) – Usual dose is 5-10 mg every 12 hours as needed for vomiting.
• Ondansetron (orally or sublingual) – The usual dose is 8mg q 12 hrs for vomiting. This medication formerly was very expensive, but now it can be obtained at reasonable prices at places such as Costco. Although Ondansetron isn’t as strong as Phenergan or Compazine and doesn’t always work, it also doesn’t have many side effects either. One can certainly work and drive after taking nearly any dose of ondansetron. The same cannot always be said for meclizine, lorazepam, and clonazepam, Phenergan, or Compazine.
• Dexamethasone (Decadron) – 4 mg orally for 4-7 days. Or a Medrol dose packs this convenient, rapid, but not very effective treatment is gradually being replaced by steroid injections through the eardrum. It is usually an “add-on at the time of a physician’s visit for persistent symptoms. Being hyper is the most common side effect. Often people feel that they don’t need sleep and do a lot of cleaning.
• Diet – People with MD are often advised to reduce their salt intake.^[rx][rx] 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” like caffeine. However, 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]
• Vestibular rehabilitation therapy – VRT is an exercise program that retrains your brain to use other senses, such as your vision, to help with your balance.
• Positive pressure therapy (Meniett device) – This approach uses a device to apply pressure to your ear canal through a tube. This improves how fluid moves through your ear. You can do these treatments at home.
• Counseling – The 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]

MEDICATIONS USED BETWEEN ATTACKS (also see flowchart below)

Diuretics — those in common use all tend to be a combination of a thiazide (that is potassium decreasing) and a sodium channel blocker (e.g. triamterene or amiloride). The combinations have the advantage that they may not require potassium supplementation.

• Triamterine/HCTZ (Dyazide or Maxide).
• Moduretic(amiloride/HCTZ)
• Acetazolamide

Diuretics that do not contain sulfa (Ponka, 2006)

• Amiloride
• Ethacrynic acid
• Spironolactone
• Triamterene

Note that we have four chemical groups of diuretics here sodium channel blockers (triamterene, amiloride), loop diuretics (ethacrynic acid), carbonic anhydrase inhibitors (acetazolamide), and aldosterone antagonists (spironolactone).

When there is a sulfa allergy, one may try amiloride by itself or ethacrynic acid. Loop diuretics such as Edecrin should be used in low doses and with caution because they are ototoxic. Note that the diuretics listed are mainly ones that increase serum potassium. If it is true that the positive effect in Meniere’s of diuretics is to increase aldosterone as has been suggested by several Japanese authors, spironolactone, as well as eplerenone, would be bad choices as they are aldosterone antagonists.

Vestibular Suppressants

Benzodiazepines – these drugs have fallen out of favor because of their addictive properties.

• Clonazepam(Klonopin) 0.5 mg twice a day or as needed
• lorazepam (Ativan) 0.5mg twice a day or as needed
• diazepam (Valium) 2 mg twice a day or as needed

Antihistamines

• meclizine (Antivert, Bonine, Dramamine non-drowsy) 12.5 mg to 25 mg as needed up to 3-4 times/day
• diphenhydramine (Benadryl)

Note that antihistamines that do not cross into the brain are not used because they don’t work — i.e. loratadine, cetirizine, fexofenadine.

Calcium Channel Blockers – these drugs are rarely used as well. They are more commonly used for migraines.

• Verapamil  120-240 mg. Sustained-release should be used. Watch out for drug interactions.
• Nimodipine
• Cinnarizine (not available in the USA)
• Flunarizine (not available in the USA)

Steroids (commonly for severe bouts) – commonly used, the evidence is not strong for efficacy

• Dexamethasone
• Prednisone
• Methylprednisolone (usually in a self-tapering “dose pack”).

Immune suppressants (rarely used, see AIED)

• Methotrexate (very rarely)
• Steroids (see above)
• Enbrel (injectable drug), Humira (injectable)

Agents that are controversial

• Serc (betahistine) – commonly used, maybe placebo, but often worth trying. The usual dose is 16 mg twice/day but more can be used too.
• Antifungals such as Mycostatin (Nystatin) – Evidence is weak, and no rationale. (Leong et al, 2014)
• Histamine injections – (irrational treatment as histamine is broken down rapidly in the body).
• Homeopathic treatments – such as VertigoHeel. As is the case with all homeopathic treatments, VertigoHeel is a placebo.
• Antiviral therapy – (such as acyclovir, no evidence for effectiveness)
• Intratympanic dexamethasone or other steroids – (becoming more common, reasonable evidence for temporary effectiveness, no rationale for a long term effect)

What can be done to reduce the frequency and severity of Meniere’s disease attacks (i.e. prevention)?

The purpose of treatment between attacks is to prevent or reduce the number of episodes and to decrease the chances of further hearing loss and damage to the vestibular system. Permanent tinnitus (ringing in the ears), constant imbalance, or a progressive hearing loss may be the consequence of long-term Meniere’s disease. Hearing aids may be necessary.

Standard medical treatments

• The hydrops diet regimen – will probably be recommended. This is an important part of treatment for virtually all patients with Meniere’s disease. STRICT adherence to this dietary regimen will result in stabilization in most patients.
• Between attacks, diuretic medication – may be prescribed to help regulate the fluid pressure in the inner ear, thereby reducing the severity and frequency of the Meniere’s episodes. Dyazide (a combination of triamterene and hydrochlorothiazide) is the most common medication for this purpose, and others are listed above.
• Vestibular suppressants such as Antivert (meclizine) – or Clonazepam, and anti-emetics (e.g. Phenergan or ondansetron) are used on an as-needed basis.

Not so standard treatments

• Verapamil (typical dose: 120 SR) – sometimes reduces the frequency of attacks. Nimodipine and Flunarizine have also been used. These medications are all calcium channel blockers. The evidence is not as good that these medications work. Because Menieres and Migraine are very often combined, this medication’s main role may be to treat the migraine associated vertigo that can be confused with or accompany Meniere’s disease. This medication is especially logical if the dizzy attacks are associated with headaches. There have been recent reports that other migraine medications are useful in patients who have failed diuretic treatment.
• Some physicians prescribe Histamine injections – Most physicians in the USA consider this treatment to be ineffective.
• Prednisone or other steroids (e.g. Decadron) – are occasionally helpful in short bursts. We would most often use these when considering a destructive treatment.
• Medications – that do not have much of a track record that can be tried under the supervision of your doctor. There also some unusual medications which are either considered “alternative” or which are available only outside the US which might be worth considering.

What the author recommends in his practice in Chicago for medical prevention of Meniere’s. These drugs are administered to most of his patients, generally in the following sequence:

• Low sodium (2000 mg) diet (1-month trial)
• A salt-wasting diuretic such as dyazide (1-month trial)
• Betahistine (2-week trial, often combined with verapamil)
• Verapamil 120 SR (one-month trial dyazide is stopped) – Verapamil is essentially a migraine prevention medication.
• These are combined with symptomatic drugs such as meclizine, benzodiazepines, and antiemetics, to be taken during attacks.

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.