Meniere's Disease

Aka: Meniere's Disease, Meniere Disease, Labyrinthine Hydrops, Endolymphatic Hydrops

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II. Epidemiology

  1. Incidence: 4.3 per 100,000 persons/year
  2. Prevalence: 17-46 per 100,000 persons
  3. Age of onset
    1. Most common at ages 20 to 60 years old
    2. Peaks at 40-60 years old (with a biphasic peak around age 20 years old)

III. Etiology

  1. Unknown
  2. Exacerbated by stress or emotional disturbance
  3. Associated with concurrent infection in 50% cases

IV. Pathophysiology

  1. Swelling of endolymphatic labyrinthine spaces (increased endolymphatic fluid pressure)
  2. Degeneration of the Organ of Corti

V. Symptoms

  1. Prodrome
    1. Headache
  2. Triad
    1. Vertigo
      1. Recurrent, spontaneous episodes that may last minutes to hours, days when severe (may require bed rest)
      2. Diagnosis requires at least 2 episodes lasting 20 minutes
      3. Associated Nausea, Vomiting and Ataxia
    2. Sensorineural Hearing Loss
      1. Fluctuant, typically low pitched Hearing Loss
    3. Tinnitus
      1. "Roaring", low tone Tinnitus (or aural fullness)
  3. Distribution
    1. Typically unilateral, at least initially (but 33% have bilateral disease)
  4. Other symptoms
    1. Episodic fluctuating, ear pressure or aural fullness (inner ear endolymphatic fluid collection)

VI. Signs

  1. Nystagmus (and associated Ataxia)
    1. Nystagmus is only present when Vertigo present
    2. Unidirectional, horizontal or rotary, torsional Nystagmus
  2. Sensorineural Hearing Loss
    1. Early: Low tones affected (low to medium frequency Sensorineural Hearing Loss)
    2. Later: All tones affected
  3. Hyperacusis
    1. Some noises may seem paradoxically louder (auditory recruitment)

VII. Diagnostics

  1. Audiogram

VIII. Management: Acute

  1. Benzodiazepines (e.g. Diazepam)
  2. Antiemetics (e.g. Ondansetron, Prochlorperazine, Promethazine)
  3. Transdermal Scopolamine

IX. Management: Maintenance

  1. As of 2023, evidence is poor for all of the interventions listed
  2. Diuretics
    1. Hydrochlorothiazide or
    2. Hydrochlorothiazide/Triamterene (Dyazide)
  3. Lifestyle changes
    1. Low salt diet (<2 grams daily)
    2. Decrease Caffeine
    3. Smoking Cessation
    4. Limit Alcohol
    5. Vestibular rehabilitation or Exercises may be effective
  4. Symptomatic Medications for acute episodes
    1. See Vertigo Management for acute symptomatic management
    2. Vestibular balance and rehabilitation therapy

X. Management: ENT

  1. Transtympanic injection of Corticosteroids
  2. Ablation of vestibular hair cells (in those who already have Hearing Loss)
    1. Performed with transtympanic injection of Gentamicin
  3. Surgery: (10% of patients with refractory cases)
    1. Labyrinthectomy to decompress endolymphatic sacs (Symptom relief in 66%)
    2. Vestibular Nerve section (Symptom relief in 95%)
    3. No procedure corrects the Hearing Loss

XI. References

  1. Glasscock (1984) Am J Otol 5:536-42 [PubMed]
  2. Knox (1997) Am Fam Physician 55(4):1185-90 [PubMed]
  3. Muncie (2017) Am Fam Physician 95(3): 154-62 [PubMed]
  4. Rogers (2023) Am Fam Physician 107(5): 514-23 [PubMed]

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Related Studies

Ontology: Meniere Disease (C0025281)

Definition (MEDLINEPLUS)

Meniere's disease is a disorder of the inner ear. It can cause severe dizziness, a roaring sound in your ears called tinnitus, hearing loss that comes and goes and the feeling of ear pressure or pain. It usually affects just one ear. It is a common cause of hearing loss.

Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people have single attacks of dizziness once in a while. Others may have many attacks close together over several days. Some people with Meniere's disease have "drop attacks" during which the dizziness is so bad they lose their balance and fall.

Scientists don't yet know the cause. They think that it has to do with the fluid levels or the mixing of fluids in the canals of your inner ear. Doctors diagnose it based on a physical exam and your symptoms. A hearing test can check to see how it has affected your hearing.

There is no cure. Treatments include medicines to control dizziness, limiting salt in your diet, and taking water pills. A device that fits into the outer ear and delivers air pulses to the middle ear can help. Severe cases may require surgery.

NIH: National Institute on Deafness and Other Communication Disorders

Definition (CSP) fluctuating hearing loss, tinnitus, and vertigo resulting from nonsuppurative disease of the labyrinth; swelling of the endolymph-containing structures is the main pathologic finding.
Definition (MSH) A disease of the inner ear (LABYRINTH) that is characterized by fluctuating SENSORINEURAL HEARING LOSS; TINNITUS; episodic VERTIGO; and aural fullness. It is the most common form of endolymphatic hydrops.
Concepts Disease or Syndrome (T047)
MSH D008575
ICD9 386.00, 386.0
ICD10 H81.0 , H81.09
SnomedCT 13445001, 155235002, 194346003, 194347007, 194352002
English Disease, Meniere, Meniere Syndrome, Meniere's Syndrome, Menieres Syndrome, Disease, Meniere's, Syndrome, Meniere's, MENIERE'S SYNDROME, Meniere's disease, Meniere's disease, unspecified, Unspecified Meniere's disease, MENIERES DISEASE, MENIERE DISEASE, MENIERES DIS, MENIERE DIS, Meniere's Disease, Meniere's disease (diagnosis), Meniere's syndrome, Syndrome Meniere's, Meniere disease, Meniere's disease NOS, Ménière's syndrome or vertigo, Ménière's disease, unspecified ear, Meniere Disease [Disease/Finding], meniere's syndrome, Disease;Menieres, meniere disease, meniere's disease, otogenic vertigo, meniere syndrome, Meniere's disease, unspecified ear, Menieres disease, Ménière's disease, unspecified, Ménière's Diseases, Ménière Disease, Ménières Disease, Ménière Diseases, Ménière's Disease, Disease, Ménière's, Diseases, Ménière, Diseases, Ménière's, Disease, Ménière, Unspecified Meniere's disease (disorder), Meniere syndrome (disorder), Meniere's disease NOS (disorder), Meniere Disease, Auditory vertigo, Meniere disorder, Meniere syndrome, Otogenic vertigo, Ménière's disease (disorder), Ménière's disease, Ménière's syndrome, Ménière's vertigo, Ménière, hydrops; labyrinth, labyrinth; hydrops, Meniere's disease, NOS, Meniere's vertigo, NOS, Ménière's disease, NOS, Ménière's syndrome, NOS, Ménière's vertigo, NOS, Menieres Disease, Meniere's syndrome or vertigo
Portuguese SINDROME DE MENIERE, Síndrome de Ménière, Doença de Ménière NE, Doença de Ménière, Doença de Meniere, Síndrome de Meniere
Spanish SINDROME DE MENIERE, Síndrome de Ménière, Enfermedad de Ménière no especificada, vertigo de Ménière, vértigo otogénico, vértigo de Ménière, enfermedad de Meniere, SAI (trastorno), enfermedad de Ménière no especificada (trastorno), enfermedad de Ménière no especificada, enfermedad de Meniere, SAI, enfermedad de Ménière (trastorno), enfermedad de Ménière, síndrome de Ménière, vértigo auditivo, Enfermedad de Ménière, Enfermedad de Meniere, Síndrome de Meniere
Italian Malattia di Ménière, Malattia di Ménière, non specificata, Sindrome di Meniere, Malattia di Meniere
Dutch syndroom van Ménière, niet-gespecificeerde ziekte van Ménière, Ménière-syndroom, hydrops; labyrint, labyrint; hydrops, Ziekte van Mnirenc, ziekte van Ménière, Syndroom van Ménière, Ziekte van Ménière
French Syndrome de Ménière, Maladie de Ménière, non précisée, SYNDROME DE MENIERE, Vertige labyrinthique, Maladie de Ménière, Syndrome ménièriforme
German Syndrom, Meniere, Meniere-Syndrom, Meniere-Krankheit, unspezifisch, MENIERE SYNDROM, Meniere-Krankheit, Morbus Meniere, Ménière-Krankheit, Ménière-Syndrom, Morbus Ménière
Japanese 詳細不明のメニエール病, ｼｮｳｻｲﾌﾒｲﾉﾒﾆｴｰﾙﾋﾞｮｳ, ﾒﾆｴｰﾙｼｮｳｺｳｸﾞﾝ, ﾒﾆｴｰﾙﾋﾞｮｳ, アレルギー性迷路水症, メニエール症候群, 耳性めまい, めまい-耳性, メニエール病, Meniere症候群, Meniere病
Swedish Menieres sjukdom
Czech Menierova nemoc, Menierův syndrom, Menierova nemoc, blíže neurčená
Finnish Ménièren tauti
Russian GOLOVOKRUZHENIE VESTIBULIARNOE, MEN'ERA BOLEZN', ГОЛОВОКРУЖЕНИЕ ВЕСТИБУЛЯРНОЕ, МЕНЬЕРА БОЛЕЗНЬ
Korean 메니에르 병
Croatian MENIEROVA BOLEST
Polish Choroba Meniere'a
Hungarian Meniere syndroma, Meniere-syndroma, Meniere-betegség, nem meghatározott, Meniére-betegség
Norwegian Menieres sykdom, Méniéres sykdom
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Endolymphatic Hydrops (C0206586)

Definition (MSH) An accumulation of ENDOLYMPH in the inner ear (LABYRINTH) leading to buildup of pressure and distortion of intralabyrinthine structures, such as COCHLEA and SEMICIRCULAR CANALS. It is characterized by SENSORINEURAL HEARING LOSS; TINNITUS; and sometimes VERTIGO.
Concepts Disease or Syndrome (T047)
MSH D018159
ICD10 H81.0
SnomedCT 194346003, 9794007
English Endolymphatic Hydrops, Hydrops, Endolymphatic, labyrinthine hydrops, Endolymphatic Hydrops [Disease/Finding], cochlear hydrops, endolymphatic hydrops, Labyrinthine hydrops, Endolymphatic hydrops, Endolymphatic hydrops (disorder), endolymphatic; hydrops, hydrops; endolymphatic, Cochlear hydrops
Dutch hydrops van het labyrint, cochleaire hydrops, endolymfatisch; hydrops, hydrops; endolymfatisch, endolymfatische hydrops, Hydrops endolymphaticus
French Hypertension endolabyrinthique, Hydrops cochléaire, Hydrops endolymphatique
German Labyrinthhydrops, endolymphatischer Hydrops, Endolymphhydrops, Endolymphatischer Hydrops
Italian Idrope cocleare, Idrope del labirinto, Idrope endolinfatica
Portuguese Hidropsia coclear, Edema labiríntico, Hidropisia Endolinfática, Hidropsia Endolinfática, Hidropsia endolinfática
Spanish Hidropesía laberíntica, Hidrops coclear, Hidropsia Endolinfática, hidropesía endolinfática (trastorno), hidropesía endolinfática, Hidropesía endolinfática, Hidropesía Endolinfática
Japanese 内リンパ水腫, 迷路水腫, 蝸牛水腫, ﾒｲﾛｽｲｼｭ, ﾅｲﾘﾝﾊﾟｽｲｼｭ, ｶｷﾞｭｳｽｲｼｭ
Swedish Endolymfatisk hydrops
Czech endolymfatický hydrops, Vodnatelnost hlemýždě, Endolymfatický hydrops, Hydrops labyrintu
Finnish Labyrinttihydrops
Russian ENDOLIMFATICHESKAIA VODIANKA, ЭНДОЛИМФАТИЧЕСКАЯ ВОДЯНКА
Croatian Not Translated[Endolymphatic Hydrops]
Polish Obrzęk błędnika, Puchlina endolimfatyczna
Hungarian Labyrinthus hydrops, endolymphatikus hydrops, Cochlearis hydrops
Norwegian Not Translated[Endolymphatic Hydrops]
Derived from the NIH UMLS (Unified Medical Language System)

Related Topics in Vestibular

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