Ménière's disease
OVERVIEW
What is Meniere's disease?
Meniere's disease is an inner ear disorder primarily characterized by endolymphatic hydrops, with its exact etiology and pathogenesis remaining unclear [1].
Its symptoms mainly include sudden vertigo, tinnitus, hearing loss, and a sensation of ear fullness. Typically, symptoms initially affect only one ear but may later involve both ears. Episodes last from 20 minutes to several hours, with irregular intervals between attacks. Over time, the condition may progress to permanent hearing impairment, though complete deafness is rare [1].
Currently, there is no cure, and treatment focuses on symptom management. Clinical approaches primarily involve medication or surgery to alleviate symptoms [2].
Currently, there is no cure, and treatment focuses on symptom management.
Is Meniere's disease common?
Yes.
According to a 2020 report by the American Academy of Otolaryngology–Head and Neck Surgery, the prevalence of Meniere's disease is approximately 50–200 cases per 100,000 people, with the highest incidence among individuals aged 40–60 [3].
What is the origin of the name "Meniere's disease"?
The condition was first identified in the early 19th century by French physician Prosper Ménière, after whom it was later named [2].
Meniere's disease is also referred to as Ménière's disease or Ménière's syndrome—all transliterations of the same foreign term.
Which medical department should I visit for Meniere's disease?
Otolaryngology–Head and Neck Surgery or Neurology
Otolaryngology–Head and Neck Surgery is the primary choice, though patients with severe vertigo may also consult Neurology.
SYMPTOMS
What are the common manifestations of Ménière's disease?
The typical manifestations of Ménière's disease include recurrent episodes of vertigo, accompanied by fluctuating hearing loss, tinnitus (in one or both ears), and a sensation of ear fullness.
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Vertigo: The vertigo is rotational or swaying in nature, lasting from several minutes to several hours, but never exceeding 24 hours. Generally, vertigo is accompanied by nausea, vomiting, pallor, cold sweats, a slow pulse, and lowered blood pressure. These symptoms worsen with eye movement or head turning and improve when lying still with eyes closed. Typically, vertigo disappears during remission periods (the symptom-free intervals between episodes).
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Hearing loss: In the early stages, patients usually do not notice hearing loss, but after multiple episodes, they begin to experience noticeable fluctuating hearing loss (unstable hearing that varies in severity), typically in one ear. The degree of hearing loss worsens with each episode, meaning the more episodes occur, the greater the hearing decline. However, very few patients progress to complete deafness.
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Tinnitus: Tinnitus often appears before vertigo episodes. Initially, it manifests as a continuous low-pitched sound, like blowing wind or running water, which may later shift to a high-pitched ringing, whistling, or siren-like sound. Tinnitus worsens during vertigo attacks and may lessen during remission but does not disappear entirely.
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Ear fullness: During episodes, the affected ear or head may feel full, heavy, or under pressure, sometimes accompanied by a burning sensation around the ear[2].
How does Ménière's disease progress?
Ménière's disease can begin at any age, but symptoms typically appear between 40 and 60 years old, with women being more susceptible than men[3]. In the early stages, only one or two symptoms may occur, with vertigo being the primary symptom in two-thirds of patients.
After 5 to 15 years of disease progression, hearing loss becomes more stable and gradually worsens, while vertigo symptoms tend to diminish. Patients are often left with residual effects such as hearing imbalance, poorer hearing in the affected ear, and persistent tinnitus.
What are the complications of Ménière's disease?
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Sensorineural hearing loss: As episodes increase, hearing loss progressively worsens and may become irreversible permanent sensorineural hearing loss[4].
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Permanent tinnitus: After repeated episodes, tinnitus may become permanent and worsen during vertigo attacks.
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Diplacusis: This occurs when a patient perceives a single pure tone as two completely different sounds in pitch and timbre in each ear. Patients often find high-frequency loud sounds intolerably harsh[2].
Additional note: A "pure tone" refers to a sound with only one vibrational frequency, such as the sound produced by a tuning fork used in medical examinations. Pure tones are rare in everyday life and are mostly used in hearing tests.
What severe consequences can Ménière's disease cause?
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Hearing decline or even complete loss;
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Persistent tinnitus;
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Severe vertigo, potentially leading to unpredictable falls or accidents;
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Anxiety, depression, and reduced quality of life, significantly impacting work, study, and daily activities while imposing a substantial disease burden[5].
CAUSES
What is the cause of Ménière's disease?
The exact cause of Ménière's disease is unknown, but it may be related to endolymphatic hydrops caused by an imbalance in the production and absorption of endolymph. Currently accepted pathogenic mechanisms include mechanical obstruction of the endolymphatic duct and impaired endolymph absorption, immune response theory, inner ear ischemia theory, dysfunction of the endolymphatic sac theory, viral infection theory, and genetic theory [2].
What are the possible triggers of Ménière's disease?
Ménière's disease is generally believed to involve multiple factors. Its triggers include:
- Overexertion;
- Mental stress and emotional fluctuations;
- Sleep disorders;
- Weather or seasonal changes, barometric pressure changes, etc. [6].
These factors alone do not cause Ménière's disease but may trigger it in individuals with underlying conditions such as inner ear vascular ischemia, viral infections, or genetic predisposition.
Is Ménière's disease contagious?
No. Ménière's disease is not an infectious disease, and there is no evidence to suggest it is contagious.
Is Ménière's disease hereditary?
It has some hereditary tendency. Ménière's disease shows familial clustering, indicating a genetic component, but having a relative with the condition does not guarantee one will develop it. Genetic testing may identify susceptibility genes for the disease [7].
Who is more likely to develop Ménière's disease?
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Middle-aged adults: Ménière's disease can occur at any age, but it is more common in individuals aged 40–60, with a higher incidence in women. It is rare in children [3].
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Others: Individuals with a family history of the disease, inner ear vascular ischemia, or viral infections are also more susceptible than the general population [2].
DIAGNOSIS
How is Meniere's disease diagnosed?
According to domestic diagnostic criteria, the diagnosis of Meniere's disease requires meeting all the following standards [6]:
- ≥ 2 episodes of spontaneous vertigo, each lasting 20 minutes to 12 hours;
- At least one documented episode of fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear (confirmed by pure-tone audiometry, requiring hospital examination) before, during, or after vertigo attacks;
- Fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear;
- Exclusion of other vertigo-causing conditions, such as vestibular migraine, vestibular neuronitis, vestibular drug toxicity, sudden deafness, labyrinthitis, benign paroxysmal positional vertigo, etc.
What tests are needed to diagnose Meniere's disease?
Although symptoms like dizziness, tinnitus, and hearing loss can suggest Meniere's disease, these may not appear simultaneously or concurrently during the first episode. Additionally, some conditions may mimic Meniere's disease, so a series of tests are required for diagnosis [5].
- Otoscopy: Patients typically show normal tympanic membranes and good eustachian tube function [2].
- Audiological tests: Include pure-tone audiometry and speech recognition rate, usually measured with specialized equipment. Pure-tone audiometry is a key diagnostic criterion and helps determine the need for hearing rehabilitation, hearing aids, or cochlear implants. Speech recognition testing aids in assessing hearing levels and residual functional hearing [5].
- Vestibular function tests: Include spontaneous nystagmus, gaze nystagmus, optokinetic tracking, and smooth pursuit [5,6].
- Balance function tests: Include static/dynamic posturography, sensory organization testing, and gait evaluation [6].
- Tinnitus testing: Pitch and loudness matching.
- Dehydration test: Ingesting 1.2–1.5 g/kg glycerol mixed with saline or juice on an empty stomach, followed by hourly pure-tone audiometry for three hours [2].
- Imaging: Preferred method is MRI of the internal auditory canal or posterior fossa; gadolinium-enhanced labyrinthine MRI is recommended if available [5].
- Etiological tests: Include immunological, allergen, genetic, and endocrine function tests.
Why are so many tests required for Meniere's disease diagnosis?
Different tests help doctors confirm Meniere's disease or rule out similar conditions for targeted treatment.
- Otoscopy: Checks for middle ear or tympanic membrane issues.
- Hearing tests: Assess hearing loss patterns and monitor progression.
- Vestibular tests: Evaluate vestibular function and exclude other vestibular disorders.
- Balance tests: Identify sensory deficits and aid differential diagnosis.
- Tinnitus tests: Determine presence and severity of tinnitus.
- Dehydration test: Detects auditory changes by reducing endolymphatic fluid.
- Imaging: Rules out conditions like acoustic neuroma or otosclerosis.
- Etiological tests: Identify underlying causes.
What precautions should be taken during Meniere's disease diagnostic tests?
Not all tests are mandatory; doctors will guide patients based on their condition. Acute vertigo episodes may delay comprehensive testing, so most evaluations are performed during remission.
- Hearing tests: Subjective methods require patient cooperation and are unsuitable for non-organic deafness or cognitive impairment. Objective methods bypass patient response.
- Vestibular/balance tests: Avoid during vertigo attacks; defer until symptoms improve.
- MRI: Remove all metal objects (jewelry, implants). Sedation may be needed for uncooperative patients. Expect loud noise and prolonged duration (~15–30 minutes).
Are uncomfortable tests mandatory for Meniere's disease diagnosis?
Not all listed tests are required for every patient. Doctors prioritize necessary exams for accurate diagnosis and differentiation. Vestibular/balance tests may provoke vertigo or nausea but are essential if clinically indicated.
How is Meniere's disease staged by severity?
Staging is based on the pure-tone average (PTA) of 0.5, 1.0, and 2.0 kHz during the worst hearing period in the past six months:
- Stage 1: PTA ≤ 25 dBHL;
- Stage 2: PTA 26–40 dBHL;
- Stage 3: PTA 41–70 dBHL;
- Stage 4: PTA > 70 dBHL.
Staging guides treatment and prognosis. Bilateral cases require separate staging [2].
What conditions mimic Meniere's disease?
- Vestibular migraine: Symptoms overlap but typically affect pre-adolescent or menopausal women due to neurovascular dysfunction. Meniere’s peaks in middle age with slight female predominance and endolymphatic hydrops [8].
- Vestibular neuronitis: Post-viral onset with vertigo, nystagmus, and vomiting—no hearing loss or recurrence.
- Vestibular drug toxicity: Gradual vertigo from ototoxic drugs (e.g., streptomycin), non-episodic with compensation over time.
- Sudden deafness: Acute hearing loss ± vertigo, but non-fluctuating and non-recurrent.
- Benign paroxysmal positional vertigo (BPPV): Brief vertigo triggered by head movement, no auditory symptoms.
- Labyrinthitis: Severe vertigo, vomiting, deafness, and ear pain, often with otitis media history.
- Acoustic neuroma: MRI reveals cerebellopontine angle tumors [2].
TREATMENT
What are the treatment options for Ménière's disease?
Since the cause and pathogenesis of Ménière's disease remain unclear, there is currently no cure for this condition.
Current treatments primarily include medications to regulate autonomic nerve function, improve inner ear microcirculation, and relieve endolymphatic hydrops, as well as middle ear pressure therapy and surgical interventions[2].
What medications are used to treat Ménière's disease?
In the early stages of Ménière's disease, drug treatments fall into two categories: symptomatic medications and middle ear drug delivery. Clinicians may prescribe one or a combination of the following drugs based on the patient's condition.
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Symptomatic medications
- Vestibular suppressants: Such as diazepam and diphenhydramine, which effectively control acute vertigo attacks. These are used only during acute episodes and generally for no more than three days[2,6].
- Vasodilators or calcium channel blockers: Such as flunarizine, nimodipine, and betahistine[2], which can alleviate symptoms like dizziness and tinnitus.
- Diuretics: Such as mannitol, 50% glucose injection, hydrochlorothiazide, and furosemide, which may improve vertigo. Doctors often prescribe these, but treatment efficacy varies by individual[2,3].
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Middle ear drug delivery: Involves injecting medications into the tympanic cavity for patients unresponsive to other treatments. Possible side effects include bleeding and tympanic membrane perforation.
- Corticosteroids: Such as methylprednisolone and dexamethasone, which can improve hearing and vertigo, promote rapid symptom relief, and enhance prognosis.
- Aminoglycosides: Such as gentamicin. Low-dose injections can effectively relieve vertigo, outperforming dexamethasone. However, gentamicin is ototoxic—its mechanism relies on damaging vestibular receptors to stop vertigo, but it may worsen or cause hearing loss. Thus, it is only suitable for patients with severe vertigo and pre-existing hearing damage, not those with intact hearing[9].
Note: Treatment duration varies by patient, depending on disease progression, symptom severity, and remission intervals. The goal is symptom relief, with discontinuation considered once the condition is controlled.
What is middle ear pressure therapy for Ménière's disease?
Middle ear pressure therapy uses the Meniett device to deliver low-pressure pulses intermittently into the middle ear via a tympanostomy tube, providing short- or long-term relief for vertigo and tinnitus[2].
This treatment is performed in hospitals (typically tertiary hospitals). Each session lasts about 5 minutes, with the course determined by symptom severity.
For mild cases, Meniett therapy may achieve remission, but severe vertigo or tinnitus may only see short-term relief[10].
What surgical treatments are available for Ménière's disease?
Surgical options include endolymphatic sac surgery, semicircular canal occlusion, vestibular nerve section, and labyrinthectomy. These are reserved for patients with failed 6-month conservative treatment or frequent, severe vertigo attacks.
- Endolymphatic sac surgery: Reduces endolymph pressure to alleviate vertigo and tinnitus, usually preserving hearing and vestibular function. Suitable for Stage III and some severe Stage II patients.
- Semicircular canal occlusion: Controls vertigo but may impair hearing/vestibular function. Primarily for Stage IV or failed endolymphatic sac surgery cases.
- Vestibular nerve section: Eliminates vertigo while preserving hearing, for Stage IV patients unresponsive to prior treatments.
- Labyrinthectomy: Destroys vestibular function to stop vertigo but causes permanent hearing loss. For Stage IV patients with no usable hearing and refractory symptoms[6].
What are common side effects of Ménière's disease medications?
- Vestibular suppressants: Drowsiness, headache, fatigue, photosensitivity, stomach upset, rash, blurred vision.
- Vasodilators: Slowed movement, stiffness, constipation, increased appetite, drowsiness, menstrual changes, fatigue.
- Diuretics: Electrolyte imbalance, thirst, chills, difficulty urinating.
Proper use under medical guidance can minimize side effects.
What are the risks of Ménière's disease surgery?
Some procedures (e.g., semicircular canal occlusion) may partially damage hearing/vestibular function, while others (e.g., labyrinthectomy) permanently destroy both[6].
Damage manifests as partial/complete hearing loss, vertigo, imbalance, or nystagmus—all irreversible.
Are there long-term complications after Ménière's disease treatment?
Some patients may experience permanent hearing or vestibular dysfunction, including unilateral/bilateral hearing loss, vertigo, or nystagmus.
What precautions should be taken during Ménière's disease treatment?
- Rest in a quiet, dark room during attacks; resume activity gradually post-recovery. Exercise moderately during remission[2].
- Maintain regular sleep and manage stress/emotional triggers[6].
- Eat a high-protein, high-vitamin, low-salt/fat diet; avoid caffeine, tobacco, alcohol, and strong tea[6].
Is follow-up needed for Ménière's disease? How?
Yes. Hearing and vestibular function tests assess treatment efficacy, typically starting ~3 months post-treatment. Further timing depends on disease progression.
Can Ménière's disease be cured?
No, but medications and surgery can effectively manage symptoms[2].
Can Ménière's disease resolve on its own?
No. Middle/late-stage symptoms require medical or surgical intervention[2].
Is Ménière's disease fatal?
Rarely. However, vertigo attacks may lead to falls—caregivers should take precautions.
DIET & LIFESTYLE
What should patients with Meniere's disease pay attention to in their diet?
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Maintain a low-salt, low-fat, and light diet, and reduce daily salt intake. The current recommendation follows the American Heart Association's salt restriction guidelines (maximum daily sodium intake of 2300 mg, equivalent to 4 g of salt)[5].
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Reduce the intake of coffee, alcohol, and strong tea, and drink more water.
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Some patients may be allergic to certain foods and should avoid allergens[3,5].
What should patients with Meniere's disease pay attention to in daily life?
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During acute episodes, bed rest is the most effective treatment and the best position to minimize dizziness. A semi-liquid diet, such as porridge or soft noodles, is recommended.
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During remission periods, exercise moderately, balance work and rest, and maintain a low-salt diet.
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Adjust daily routines, maintain a regular lifestyle, and reduce emotional fluctuations[2].
Does Meniere's disease affect normal life, study, and work?
Yes.
During episodes, Meniere's disease often causes sudden rotational vertigo without warning, accompanied by nausea, vomiting, cold sweats, pallor, low blood pressure, hearing loss, and tinnitus. These symptoms significantly disrupt daily life, study, and work.
During remission, symptoms like vertigo, hearing loss, tinnitus, and ear fullness may subside, allowing normal activities. However, after repeated episodes, tinnitus and hearing loss may persist even during remission, leading to long-term impacts on life, work, and study[1].
Can patients with Meniere's disease fly, engage in strenuous exercise, or travel to high-altitude areas?
It depends.
During episodes, vestibular function (including balance) is impaired, so flying or strenuous exercise should be avoided. In early-stage disease with no symptoms during remission, patients can fly, exercise vigorously, or travel to high-altitude areas without restrictions.
However, diagnosed patients should minimize these activities, as they may trigger inner ear circulatory disorders and hypoxia. One theory suggests Meniere's disease may be caused by inner ear circulatory dysfunction leading to hypoxia and metabolic disturbances in the membranous labyrinth.
PREVENTION
Can Meniere's Disease Be Prevented? How to Prevent It?
Strictly speaking, Meniere's disease cannot be prevented because it often causes sudden rotational vertigo without warning. However, the frequency of vertigo episodes can be minimized:
- Maintain a regular schedule, get adequate rest, and avoid excessive fatigue.
- Avoid infections, vitamin deficiencies, endocrine dysfunction, allergic reactions, etc.
- Stay in a positive mood, maintain emotional stability, and manage stress appropriately, as these are crucial for disease prevention.
- Individuals with a family history should undergo regular check-ups for early detection and treatment.
How Can Meniere's Disease Patients Prevent Severe Complications?
Patients with Meniere's disease should receive timely symptomatic drug treatment. If medication fails to control the condition and it continues to progress, surgical intervention may be considered to prevent severe complications.