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Meniere Disease

(Meniere's Disease; Endolymphatic Hydrops)

By

Mickie Hamiter

, MD, New York Presbyterian Columbia

Reviewed/Revised May 2023
View PATIENT EDUCATION

Meniere disease is an inner ear disorder that causes vertigo, nausea, fluctuating sensorineural hearing loss, and tinnitus. There is no reliable diagnostic test. Vertigo and nausea are treated symptomatically with anticholinergics or benzodiazepines during acute attacks. Diuretics and a low-salt diet, the first line of treatment, often decrease the frequency and severity of episodes. For severe or refractory cases, the vestibular system can be ablated with topical gentamicin or surgery.

In Meniere disease, pressure and volume changes in the labyrinthine endolymph affect inner ear function. The etiology of endolymphatic fluid buildup is unknown. Risk factors include a family history of Meniere disease, preexisting autoimmune disorders, allergies, trauma to the head or ear, and, very rarely, syphilis. Peak incidence is between ages 20 and 50.

Meniere syndrome refers to the triad of vertigo, tinnitus, and hearing loss not caused by endolymphatic fluid buildup (eg, congenital anomalies).

Symptoms and Signs of Meniere Disease

Patients with Meniere disease have sudden attacks of vertigo Dizziness and Vertigo Dizziness is an imprecise term patients often use to describe various related sensations, including Faintness (a feeling of impending syncope) Light-headedness Feeling of imbalance or unsteadiness... read more Dizziness and Vertigo that usually last for 20 minutes to 12 hours; rarely, the attacks last up to 24 hours. Usually, nausea and vomiting are also present Other symptoms include diaphoresis, diarrhea, and gait unsteadiness.

Hearing impairment, typically affecting low frequencies, may follow the attacks. Before and during an episode, most patients sense fullness or pressure and hyperacusis (sensitivity to loud sounds) in the affected ear. In most patients, only one ear is affected.

During the early stages, symptoms remit between episodes; symptom-free periods may last > 1 year. As the disease progresses, however, hearing impairment fluctuates but eventually persists and gradually worsens, and tinnitus may be constant, even between attacks.

Diagnosis of Meniere Disease

  • Clinical evaluation

  • Audiogram and gadolinium-enhanced MRI to rule out other causes

The diagnosis of Meniere disease is made clinically. The simultaneous combination of fluctuating low-frequency sensorineural hearing loss, episodic vertigo, ipsilateral fluctuating aural fullness, and tinnitus is characteristic. Similar symptoms can result from vestibular migraine, vestibular neuronitis Vestibular Neuronitis Vestibular neuronitis causes a self-limited episode of vertigo, presumably due to inflammation of the vestibular division of the 8th cranial nerve; some vestibular dysfunction may persist. Sometimes... read more , viral labyrinthitis, a cerebellopontine angle tumor (eg, vestibular schwannoma Vestibular Schwannoma A vestibular schwannoma (acoustic neuroma) is a Schwann cell–derived tumor of the 8th cranial nerve. Symptoms include unilateral hearing loss. Diagnosis is based on audiology and confirmed by... read more ), or a brain stem stroke. Although bilateral Meniere disease can occur, bilateral symptoms increase the likelihood of an alternate diagnosis (eg, vestibular migraine). Vestibular migraine (also known as migrainous vertigo) is characterized by episodes of vertigo in patients who have a history of migraines Migraine Migraine is an episodic primary headache disorder. Symptoms typically last 4 to 72 hours and may be severe. Pain is often unilateral, throbbing, worse with exertion, and accompanied by symptoms... read more or with other features of migraines, such as headache, photophobia and phonophobia, or visual aura; there is no loss of hearing.

Between attacks, the examination may be entirely normal. But during an acute attack, the patient has nystagmus Nystagmus Nystagmus is a rhythmic movement of the eyes that can have various causes. Vestibular disorders can result in nystagmus because the vestibular system and the oculomotor nuclei are interconnected... read more and falls to the affected side. Also, in long-standing or refractory cases with associated labyrinthine hypofunction, the Fukuda stepping test (marching in place with eyes closed, previously known as the Unterberger test) causes the patient to turn toward the affected ear, consistent with a unilateral labyrinthine lesion.

The Halmagyi head thrust maneuver, or head impulse test, is another technique that is used to check for unilateral labyrinthine dysfunction. In the Halmagyi maneuver, the examiner has the patient visually fixate on a target straight ahead (eg, the examiner's nose). Then while observing the patient's eyes, the examiner rapidly rotates the patient's head 15 to 30° to one sides.

When the head is rotated to one side, vestibular function on that side is normal if the patient's eyes remain fixated on the target. When vestibular function is impaired, the vestibulo-ocular reflex is absent and the patient's eyes do not remain fixated on the target but instead transiently follow the head rotation and then quickly and voluntarily return back to the target (called delayed catch-up saccades).

Patients with symptoms suggesting Meniere disease should have an and an MRI (with gadolinium enhancement) of the central nervous system with attention to the internal auditory canals to exclude other causes. Audiogram typically shows a low-frequency sensorineural hearing loss in the affected ear that fluctuates between tests. The Rinne test Physical examination Worldwide, about half a billion people (almost 8% of the world's population) have hearing loss ( 1). More than 10% of people in the US have some degree of hearing loss that compromises their... read more Physical examination and the Weber test Physical examination Worldwide, about half a billion people (almost 8% of the world's population) have hearing loss ( 1). More than 10% of people in the US have some degree of hearing loss that compromises their... read more Physical examination may indicate sensorineural hearing loss.

Treatment of Meniere Disease

  • Symptom relief with antiemetics, antihistamines, or benzodiazepines

  • Diuretics and low-salt diet

  • Rarely vestibular ablation by medications or surgery

Meniere disease tends to be self-limited. Treatment of an acute attack is aimed at symptom relief and done in a staged fashion; the least invasive measures are done first, and then if the measures are ineffective, ablative procedures are sometimes done.

Anticholinergic antiemetics (eg, prochlorperazine 25 mg rectally or 10 mg orally every 6 to 8 hours; promethazine 25 mg rectally or 25 mg orally every 6 to 8 hours) can minimize vagal-mediated gastrointestinal symptoms; ondansetron is a second-line antiemetic. Antihistamines (eg, diphenhydramine, meclizine, or cyclizine; dosage is the same for these drugs: 50 mg orally every 6 hours) or benzodiazepines (eg, diazepam 5 mg orally every 6 to 8 hours) are used to sedate the vestibular system. Neither antihistamines nor benzodiazepines are effective as prophylactic treatment. Some physicians also use an oral corticosteroid burst (eg, prednisone 60 mg orally once a day for 1 week, tapered over another week) or intratympanic dexamethasone injections for an acute episode. Traditional migraine preventive medications (eg, tricyclic antidepressants, serotonin-norepinephrine reuptake inhibitors [SNRIs]) are also beneficial in some patients with Meniere disease.

A low-salt (< 1.5 g/day) diet, avoidance of alcohol and caffeine, and a diuretic (eg, hydrochlorothiazide 25 mg orally once a day or acetazolamide 250 mg orally 2 times a day) may help prevent or reduce the incidence of vertigo attacks and are commonly used first steps. However, there are no well-designed studies that clearly prove the efficacy of these measures for Meniere disease.

Because there is strong overlap between Meniere disease and migraine disorders, a trial of traditional migraine preventive medications (eg, nortriptyline, venlafaxine) may also be considered for refractory or recurrent episodes.

Although more invasive, endolymphatic sac decompression relieves vertigo in most patients, spares vestibular function, and poses minimal risk of hearing loss. Thus this procedure is still classified as a vestibular-sparing treatment.

When vestibular-sparing treatments are ineffective, an ablative procedure is considered. Intratympanic gentamicin (chemical labyrinthectomy—typically 0.5 mL of a 40 mg/mL concentration) is injected through the tympanic membrane. Follow-up with serial audiometry is recommended to check for hearing loss. The injection can be repeated in 4 weeks if vertigo persists without hearing loss.

Ablative surgery is reserved for patients with frequent, severely debilitating episodes who are unresponsive to less invasive modalities. Vestibular neurectomy (an intracranial procedure) relieves vertigo in about 95% of patients and usually preserves hearing. A surgical labyrinthectomy is done only if preexisting hearing loss is profound.

Unfortunately, there is no known way to prevent the natural progression of hearing loss. Most patients sustain moderate to severe sensorineural hearing loss in the affected ear within 10 to 15 years.

Key Points

  • Meniere disease typically causes vertigo with nausea and vomiting, unilateral tinnitus, and chronic, progressive hearing loss.

  • Testing is with audiogram, and MRI is done to rule out other disorders.

  • Antiemetics and antihistamines can help relieve symptoms; some clinicians also use oral or transtympanic corticosteroids or migraine preventive medications (eg, tricyclic antidepressants or SNRIs).

  • More invasive treatments for refractory cases include endolymphatic sac decompression, intratympanic gentamicin, and vestibular neurectomy.

  • Diuretics, a low-salt diet, and avoidance of alcohol and caffeine help prevent attacks.

Drugs Mentioned In This Article

Drug Name Select Trade
Garamycin, Genoptic, Genoptic SOP, Gentacidin, Gentafair, Gentak , Gentasol, Ocu-Mycin
Compazine, Compazine Rectal, Compazine Solution, Compazine Syrup, Compro
Anergan-50, Pentazine , Phenadoz , Phenergan, Phenergan Fortis, Prometh Plain, Promethegan
Zofran, Zofran in Dextrose, Zofran ODT, Zofran Solution, Zuplenz
Aid to Sleep, Alka-Seltzer Plus Allergy, Aller-G-Time , Altaryl, Banophen , Benadryl, Benadryl Allergy, Benadryl Allergy Children's , Benadryl Allergy Dye Free, Benadryl Allergy Kapgel, Benadryl Allergy Quick Dissolve, Benadryl Allergy Ultratab, Benadryl Children's Allergy, Benadryl Children's Allergy Fastmelt, Benadryl Children's Perfect Measure, Benadryl Itch Stopping, Ben-Tann , Children's Allergy, Compoz Nighttime Sleep Aid, Diphedryl , DIPHEN, Diphen AF , Diphenhist, DiphenMax , Dytan, ElixSure Allergy, Genahist , Geri-Dryl, Hydramine, Itch Relief , M-Dryl, Nighttime Sleep Aid, Nytol, PediaCare Children's Allergy, PediaCare Nighttime Cough, PediaClear Children's Cough, PHARBEDRYL, Q-Dryl, Quenalin , Siladryl Allergy, Silphen , Simply Sleep , Sleep Tabs, Sleepinal, Sominex, Sominex Maximum Strength, Theraflu Multi-Symptom Strip, Triaminic Allergy Thin Strip, Triaminic Cough and Runny Nose Strip, Tusstat, Unisom, Uni-Tann, Valu-Dryl , Vanamine PD, Vicks Qlearquil Nighttime Allergy Relief, Vicks ZzzQuil Nightime Sleep-Aid
Antivert, Bonine, Dramamine Less Drowsy, Dramamine-N, Medivert, Meni-D , Travel Sickness
Diastat, Dizac, Valium, VALTOCO
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
AK-Dex, Baycadron, Dalalone, Dalalone D.P, Dalalone L.A, Decadron, Decadron-LA, Dexabliss, Dexacort PH Turbinaire, Dexacort Respihaler, DexPak Jr TaperPak, DexPak TaperPak, Dextenza, DEXYCU, DoubleDex, Dxevo, Hemady, HiDex, Maxidex, Ocu-Dex , Ozurdex, ReadySharp Dexamethasone, Simplist Dexamethasone, Solurex, TaperDex, ZCORT, Zema-Pak, ZoDex, ZonaCort 11 Day, ZonaCort 7 Day
Levophed
Cafcit, NoDoz, Stay Awake, Vivarin
Esidrix, Ezide, HydroDIURIL, Microzide, Oretic, Zide
Diamox, Diamox Sequels
Aventyl, Pamelor
Effexor, Effexor XR, Venlafaxine
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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