Meniere Disease

What is Meniere Disease

Meniere disease is an inner ear disorder. It causes attacks of a spinning sensation (vertigo), dizziness, and ringing in the ear (tinnitus). It also causes hearing loss and a feeling of fullness or pressure in the ear. This is a lifelong condition, and it may get worse over time.

You may have drop attacks or severe dizziness that makes you fall. A drop attack is when you suddenly fall without losing consciousness and you quickly recover after a few seconds or minutes.

9 Interesting Facts of Meniere Disease

1. Meniere disease is an inner ear disorder characterized by sudden and recurring vertigo, fluctuating hearing loss, tinnitus, and a sensation of pressure or fullness in 1 or both ears 
   • Required for diagnosis: pressure in the ears (ie, aural fullness) and/or tinnitus combined with 2 or more spontaneous episodes of vertigo lasting more than 20 minutes 
2. Diagnosis is primarily clinical, based on patient history and supported by audiometry tests 
3. Diuretics are typically used to prevent attacks (reduce frequency or severity) 
4. Benzodiazepines or antihistamines act as vestibular suppressants to abort attacks; these medications may be used to relieve nausea 
5. Primary interventions aimed at reducing frequency and severity of attacks include dietary adjustments (eg, low-salt diet) and diuretics 
6. Vestibular physical therapy and use of hearing aids may be indicated for patients who experience residual effects
7. In patients whose disease is difficult to treat or when all other treatment options have failed, consider surgical procedures (eg, endolymphatic sac decompression, vestibular neurectomy, labyrinthectomy) 
8. Prognosis is good when a combination of lifestyle changes and pharmacologic intervention is employed 
9. More than 85% of patients improve without major surgeries 

What are the causes of Meniere Disease?

This condition is caused by having too much of the fluid that is in your inner ear (endolymph). When fluid builds up in your inner ear, it affects the nerves that control balance and hearing. The reason for the fluid buildup is not known. Possible causes include:

• Allergies.
• An abnormal reaction of the body’s defense system (autoimmune disease).
• Viral infection of the inner ear.
• Head injury.

What increases the risk of Meniere Disease?

You are more likely to develop this condition if:

• You are older than age 40.
• You have a family history of Meniere disease.
• You have a history of autoimmune disease.
• You have a history of migraine headaches.

What are the symptoms of Meniere Disease?

Symptoms of Meniere Disease can come and go and may last for up to 4 hours at a time. Symptoms usually start in one ear. They may become more frequent and eventually involve both ears. Symptoms can include:

• Fullness and pressure in your ear.
• Roaring or ringing in your ear.
• Vertigo and loss of balance.
• Dizziness.
• Decreased hearing.
• Nausea and vomiting.

How is this diagnosed?

Meniere Disease is diagnosed based on:

• A physical exam.
• Tests , such as:
  • A hearing test (audiogram).
  • An electronystagmogram. This tests your balance nerve (vestibular nerve).
  • Imaging studies of your inner ear, such as CT scan or MRI.
  • Other balance tests, such as rotational or balance platform tests.

How is this treated?

There is no cure for Meniere Disease, but treatment can help to manage your symptoms. Treatment may include:

• A low-salt diet. Limiting salt may help to reduce fluid in the body and relieve symptoms.
• Oral or injected medicines to reduce or control:
  • Vertigo.
  • Nausea.
  • Fluid retention.
  • Dizziness.
• Use of an air pressure pulse generator. This is a machine that sends small pressure pulses into your ear canal.
• Hearing aids.
• Inner ear surgery. This is rare.

When you have symptoms, it can be helpful to lie down on a flat surface and focus your eyes on one object that does not move. Try to stay in that position until your symptoms go away.

Follow these instructions at home:

Eating and drinking

• Eat the same amount of food at the same time every day, including snacks.
• Do not skip meals.
• Avoid caffeine.
• Drink enough fluids to keep your urine clear or pale yellow.
• Limit alcoholic drinks to one drink a day for non-pregnant women and 2 drinks a day for men. One drink equals 12 oz of beer, 5 oz of wine, or 1½ oz of hard liquor.
• Limit the salt (sodium) in your diet as told by your health care provider. Check ingredients and nutrition facts on packaged foods and beverages.
• Do not eat foods that contain monosodium glutamate (MSG).

General instructions

• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Take over-the-counter and prescription medicines only as told by your health care provider.
• Find ways to reduce or avoid stress. If you need help with this, ask your health care provider.
• Do not drive if you have vertigo or dizziness.

Contact a health care provider if:

• You have symptoms that last longer than 4 hours.
• You have new or worse symptoms.

Get help right away if:

• You have been vomiting for 24 hours.
• You cannot keep fluids down.
• You have chest pain or trouble breathing.

• Meniere disease is an inner ear disorder characterized by sudden and recurring vertigo, fluctuating hearing loss, tinnitus, and a sensation of pressure or fullness in 1 or both ears 
  • Required for diagnosis: 
    • Pressure in the ears (ie, aural fullness) and/or tinnitus
    • 2 or more spontaneous episodes of vertigo lasting more than 20 minutes 

Classification

• 2 main diagnostic categories 
  • Probable Meniere disease 
    • 2 or more reported attacks of vertigo or dizziness that can last from 20 minutes to 24 hours
    • Fluctuating aural symptoms in affected ear, which may include diminished hearing, tinnitus, or fullness
    • Exclusion of all other causes
  • Definite Meniere disease 
    • Consists of criteria for probable Meniere disease plus low- to medium-frequency sensorineural hearing loss before, during, or after vertigo episode
• Hearing loss staging (used to evaluate treatment in definite Meniere disease) 
  • Stage 1: 4-tone average of less than or equal to 25 dB
  • Stage 2: 4-tone average of 26 to 40 dB
  • Stage 3: 4-tone average of 41 to 70 dB
  • Stage 4: 4-tone average of greater than 70 dB

Clinical Presentation

History

• Spontaneous rotational peripheral vertigo that lasts more than 20 minutes, but it can last up to 24 hours 
  • Dizziness or unsteadiness are symptoms, but they are not specific to Meniere disease and thus are not diagnostic
  • May be triggered by excessive consumption of sodium or caffeine, atmospheric pressure changes, or low-frequency noises
    • Triggered vertigo typically occurs later in disease process
  • Typically associated with disequilibrium that can last for days and can be accompanied by severe nausea, vomiting, and retching 
• Fluctuating tinnitus or fullness is associated with first vertigo episode 
• Unilateral (60%-80%) or bilateral (20%-40%) hearing loss 
  • During first few years of disease, hearing loss tends to fluctuate
  • Repeated attacks may lead to permanent and profound hearing loss associated with diplacusis 
    • Once hearing loss is permanent, tinnitus may become persistent
  • Hearing loss does not necessarily occur at the same time as vertigo and may precede vertigo by months to years or occur several weeks or months later 

Physical examination

• Low- to medium-frequency sensorineural hearing loss as determined by audiometry 
  • Defined by a larger (by at least 30 dB) increase in pure-tone threshold in affected ear compared with contralateral ear at each of 2 frequencies (below 2000 Hz)
  • In bilateral hearing loss, an absolute threshold of 35 dB or more at each of 2 frequencies (below 2000 Hz) is used
• Horizontal or horizontal-rotatory nystagmus accompanies a definitive Meniere disease episode (100% of cases) 
• Aside from nystagmus, hearing loss, and an unsteady gait, neurologic examination is normal

Causes and Risk Factors

Causes 

• Has been associated with an increase in pressure in the semicircular canals due to an accumulation of endolymph, but no cause for this fluid imbalance has been identified definitively
  • Autoimmune and viral mechanisms have been suggested
• Thought by some researchers to be associated with vascular regulation analogous to (but different from) migraine

Risk factors and/or associations 

Age

• Most often observed in adults aged 40 to 60 years 
  • Symptoms are rarely diagnosed in those older than 60 years

Sex

• Evidence suggests there is a slight female preponderance (1.3:1) 

Genetics

• 5% to 13% of patients have family history of the disease 
  • Consider Meniere disease in patients for whom there appears to be a strong familial history 
  • Mutations in DTNA and FAM136A genes have been reported in cases of familial Meniere disease 

Ethnicity/race

• White people of northern European descent are often more affected than African and black populations 

Other risk factors/associations

• Head or ear trauma
  • Can cause damage to the peripheral vestibular system 
• Strong association with seasonal allergies 
• Strong association with circulating immune complexes 
• There is a higher prevalence of Meniere disease among individuals with severe obesity (odds ratio of 1.7) 
• Autoimmune disorders
  • Can be present in 15% to 30% of patients with a coexisting systemic autoimmune disorder 
    • Patients with Meniere disease display an elevated prevalence of rheumatoid arthritis, systemic lupus erythematosus, and ankylosing spondylitis 
    • Approximately 50% of patients with autoimmune inner ear disease present with vestibular disorders 
• Metabolic disorders considered to be risk factors associated with Meniere disease: 
  • Dyslipidemia
  • Hypertension
  • Sleep apnea
  • Diabetes
  • Atherosclerosis

Diagnostic Procedures

Primary diagnostic tools 

• Base diagnosis on patient history and physical examination; the following symptoms are necessary for definitive diagnosis: 
  • At least 2 spontaneous episodes of vertigo lasting 20 minutes or more
  • Low- to medium-frequency sensorineural hearing loss occurring before, during, or after an episode of vertigo
  • Fluctuating aural symptoms including hearing loss, tinnitus, or fullness in the affected ear
  • Exclusion of other vestibular diseases
• Document and calibrate hearing loss with audiometry 
• Use electrocochleography to support and improve the certainty of diagnosis 
• Because diagnosis is primarily clinical, rule out other causes of vertigo 
  • Recommended tests include CBC, sedimentation rate, thyroid function, lipid profile, fasting glucose level, hemoglobin A1C level, and serologic test for syphilis

Functional testing

• Audiometry 
  • Shows upsloping low-frequency sensorineural hearing loss
    • Increase in the threshold for bone-conducted sound of at least 30 dB (35 dB if bilateral) at each of 2 contiguous frequencies below 2000 Hz 
  • Serial studies show characteristic fluctuation over time
• Electrocochleography 
  • Can help enhance diagnosis
  • Needle electrode is placed on the tympanic membrane or in ear canal to measure cochlear microphonics, summating potentials, and action potentials
  • Measures electrical potential of the eighth cranial nerve in response to auditory stimuli
    • Based on summation potential to action potential ratio, the following are indicative of Meniere disease:
      • Greater than 0.3 for trans–tympanic membrane electrode 
      • Greater than 0.4 for tympanic membrane electrode 
      • Greater than 0.5 for ear canal electrode 

Differential Diagnosis

Most common

• Cerebellar or brainstem stroke and transient ischemic attack
• Benign positional vertigo
• Viral labyrinthitis
• Bacterial labyrinthitis
• Otosyphilis
• Vestibular neuritis, neuronitis, and benign recurrent vertigo
• Migraine associated vertigo
• Multiple Sclerosis
• Acoustic neuroma 
• Toxicity from aminoglycosides, alcohol, or quinine – These drugs and chemical agents can become toxic, producing symptoms that resemble Meniere disease Can cause subjective tinnitus Differentiated by history of exposure to the ototoxic substance.
• Trauma – Damage and injury may cause temporary or permanent hearing loss and/or changes to vestibular systemLabyrinthine concussion due to blunt force trauma or changes in atmospheric pressure Barotrauma due to extreme changes in atmospheric pressure. Blast trauma due to ear slap or explosion near ear Penetrating trauma to the inner ear Symptoms may be similar to Meniere disease (eg, tinnitus, vertigo, unsteady gait) Differentiated by determining nature and history of head trauma or injury

Treatment Goals

• There is no cure; primary goal is to treat symptoms
  • Reduce or eliminate attacks 
  • Stabilize and improve hearing

Admission criteria

• Emergency department visit or inpatient admission is occasionally necessary to administer IV fluids and IV antiemetic agents for patients with intractable vomiting, which can last several days to a week 

Recommendations for specialist referral

• Specialized testing (eg, vestibular evoked potentials for difficult-to-diagnose patients) should be performed by an otologist, neurologist, and/or otoneurologist
• Refer to audiologist for hearing evaluation and hearing aid prescription
• Consult with otorhinolaryngologist regarding administration of intratympanic medications or selective vestibular neurectomy in patients with intractable vertigo
• Refer to physiotherapist for fall prevention and vestibular rehabilitation

Treatment Options

There are no proven, demonstrable, effective treatments to arrest progression of hearing loss and vertigo

• First line treatment is low-salt diet 
• Some experts also recommend avoidance of dietary components known to trigger migraine (eg, chocolate, red wine, monosodium glutamate) 
• Drug therapy may be used
  • Diuretics are typically used to prevent attacks (reduce frequency or severity); however, conclusive evidence of efficacy is lacking 
  • Benzodiazepines or antihistamines act as vestibular suppressants to abort attacks; these medications may be used to relieve nausea 
    • A small study showed diazepam and meclizine to be equally effective 
  • Betahistine may reduce the number of attacks and vertigo intensity, and it may provide symptomatic improvement. Use remains controversial due to low quality of evidence 
    • Currently not available in the United States
  • Oral steroids given as a tapering dose may help reduce severity of acute attacks in some patients, but evidence is unclear 
• Intratympanic injection of drugs, in consultation with an otorhinolaryngologist, is used for patients who have intractable vertigo with sudden hearing loss 
  • Patients whose Meniere disease does not respond to first line treatment are offered corticosteroids 
    • May diminish vertigo as well as frequency and severity of attacks 
  • Gentamicin effectively controls vertigo but may increase hearing loss 
• Surgery typically is done after all other treatment options have been tried and have failed to produce a response 
  • Meniere disease: endolymphatic sac surgery (nondestructive) 
  • Meniere disease with intractable vertigo with residual hearing: vestibular nerve resection (neurectomy)
  • Meniere disease with intractable vertigo with no residual hearing: transmastoid labyrinthectomy
• Supportive devices (eg, hearing aids) are indicated for patients with hearing impairment 
• Vestibular physical therapy is recommended to help patient with central compensation for unilateral hearing loss

Drug therapy

• Thiazide diuretics 
  • Hydrochlorothiazide and triamterene
    • Triamterene, Hydrochlorothiazide Oral capsule; Adults: 25 mg hydrochlorothiazide combined with 37.5 mg triamterene PO once daily.
• Benzodiazepines 
  • Diazepam
    • Diazepam Oral tablet; Adults: 2 to 5 mg PO 3 times daily as needed for nausea.
• Antihistamines 
  • Meclizine 
    • Meclizine Hydrochloride Chewable tablet; Adults: 25 to 100 mg/day PO given in divided doses, depending on clinical response. Start at the lowest dosage for geriatric patients as elderly patients are more sensitive to anticholinergic effects.
• Betahistine 
  • Currently not commercially available in the United States
  • Betahistine dihydrochloride Oral tablet; Adults: 8 to 16 mg PO 3 times a day or 24 mg PO twice daily. 

Nondrug and supportive care

Low-salt diet 

• Sodium intake should be lower than 2000 mg
• Thought to help lower endolymphatic pressure

Reduce stress and avoid trigger foods and drinks such as chocolate, red wine, monosodium glutamate, and others identified by patient 

Hearing aids 

• Conventional hearing aids: indicated for patients who have residual hearing loss
• Contralateral routing of sound hearing aid: indicated for patients who have total hearing loss in 1 ear
  • Transmitter collects sounds from weaker ear and transmits them to a receiver on stronger ear
  • Helps patient better understand speech and localize sound
• Osseointegrated mastoid implant plus bone-conduction processor: indicated to reduce hearing impairment in unilaterally deaf patients

Vestibular physical therapy 

• At-home exercises to improve gaze and postural stability
• Patient education
• Self-empowerment
• Vestibular adaptation or habituation

Procedures 

Intratympanic drug injections 

General explanation

• Drug is injected posteroinferiorly into inferior middle ear space (approximately 0.4 mL)
• Delivers a high dose of drug to the inner ear while avoiding systemic effects
• Can be done in outpatient setting using otologic operating microscope

Indication

• Disease was unresponsive to first line treatment

Contraindications

• Active middle ear disease
• Ear anatomy inconsistent with drug uptake

Complications

• 1% risk of perforating tympanic membrane and producing middle ear inflammation

Endolymphatic sac surgery 

General explanation

• Surgical procedure used to decompress the endolymphatic sac by resecting a portion of the mastoid bone surrounding it
• Alternatively, a shunt may be placed into the endolymphatic sac, allowing excess fluid to drain
• Results are similar between decompression and shunting surgeries
• Controversial in terms of efficacy in reducing frequency of vertigo attacks

Indication

• Failure of nonsurgical options
• Recommended for patients with Meniere disease who are experiencing intractable vertigo

Contraindications

• Active mastoiditis
• Active middle ear disease

Complications

• Occlusion or migration of shunt

Vestibular neurectomy 

General explanation

• Destructive procedure in which the vestibular branch of the vestibulocochlear nerve is severed to stop vestibular stimuli from transmitting from ear to brain

Indication

• Failure of nonsurgical options
• Unilateral, but not total, hearing loss

Complications

• Facial nerve injury
• Temporary cerebrospinal fluid leak

Labyrinthectomy 

General explanation

• Labyrinth and cochlea are removed to prevent vestibular signals from reaching the brain

Indication

• Failure of nonsurgical options
• Patients experiencing unilateral hearing loss, labyrinthine vertigo, and tinnitus

Contraindications

• Absolute contraindications 
  • If the involved ear is the only hearing ear
  • Contralateral vestibular dysfunction
• Relative contraindications 
  • Any condition that prohibits central compensation for vestibular input
  • Central nervous system disease, physiologic old age, and generally poor medical condition

Complications

• Facial nerve injury
• Cerebrospinal fluid leak

Complications

• Can cause progressive deafness and tinnitus 
• Labyrinthectomy and, in some cases, intratympanic gentamicin, will cause irreversible hearing loss 

Prognosis

• More than 85% of patients improve without major surgery (eg, labyrinthectomy) 
  • Combining changes in lifestyle and medical intervention (eg, pharmacological treatment) is more effective than a single therapy

Summary

• Meniere disease is an inner ear disorder. It causes attacks of a spinning sensation (vertigo), dizziness, and ringing in the ear (tinnitus). It also causes hearing loss and a feeling of fullness or pressure in the ear.
• Symptoms of this condition can come and go and may last for up to 4 hours at a time.
• When you have symptoms, it can be helpful to lie down on a flat surface and focus your eyes on one object that does not move. Try to stay in that position until your symptoms go away.

Sources

Thompson TL et al: Vertigo: a review of common peripheral and central vestibular disorders. Ochsner J. 9(1):20-6, 2009 Reference