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Ménière Disease
Jason E. Cohn, DO, MS
Nadir Ahmad, MD, FACS
Thomas C. Spalla, MD
image BASICS
DESCRIPTION
  • An inner ear (labyrinthine) disorder characterized by recurrent attacks of hearing loss, tinnitus, vertigo, and sensations of aural fullness due to an increase in the volume and pressure of the inner ear endolymph fluid (endolymphatic hydrops)
  • Often unilateral initially, nearly half become bilateral over time.
  • Severity and frequency of vertigo may diminish with time, but hearing loss is often progressive and/or fluctuating.
  • Usually idiopathic (Ménière disease) but may be secondary to another condition causing endolymphatic hydrops (Ménière syndrome)
  • System(s) affected: nervous
  • Synonym(s): Ménière syndrome; endolymphatic hydrops
EPIDEMIOLOGY
  • Predominant age of onset: 40 to 60 years
  • Predominant gender: female > male (1.3:1)
  • Race/ethnicity: white, Northern European > blacks
Incidence
Estimates 1 to 150/100,000 per year
Prevalence
Varies from 7.5 to >200/100,000
ETIOLOGY AND PATHOPHYSIOLOGY
  • Not fully understood; theories include increased pressure of the endolymph fluid due to increased fluid production or decreased resorption. This may be caused by endolymphatic sac pathology, abnormal development of the vestibular aqueduct, or inflammation caused by circulating immune complexes. Increased endolymph pressure may cause rupture of membranes and changes in endolymphatic ionic gradient.
  • Ménière syndrome may be secondary to injury or other disorders (e.g., reduced middle ear pressure, allergy, endocrine disease, lipid disorders, vascular, viral, syphilis, autoimmune). Any disorder that could cause endolymph hydrops could be implicated in Ménière syndrome.
Genetics
Some families show increased incidence, but genetic and environmental influences are incompletely understood.
RISK FACTORS
May include
  • Stress
  • Allergy
  • Increased salt intake
  • Caffeine, alcohol, or nicotine
  • Chronic exposure to loud noise
  • Family history of Ménière
  • Certain vascular abnormalities (including migraines)
  • Certain viral exposures (especially herpes simplex virus [HSV])
GENERAL PREVENTION
Reduce known risk factors: stress; salt, alcohol, and caffeine intake; smoking; noise exposure; ototoxic drugs (e.g., aspirin, quinine, aminoglycosides).
COMMONLY ASSOCIATED CONDITIONS
  • Anxiety (secondary to the disabling symptoms)
  • Migraines
  • Hyperprolactinemia
image DIAGNOSIS
Diagnosis is clinical.
PHYSICAL EXAM
  • Physical exam rules out other conditions; no finding is unique to Ménière disease.
  • Horizontal nystagmus may be seen during attacks.
  • Otoscopy is typically normal.
  • Triggering of attacks in the office with Dix-Hallpike maneuver suggests diagnosis of benign paroxysmal positional vertigo, not Ménière disease.
DIFFERENTIAL DIAGNOSIS
  • Acoustic neuroma or other CNS tumor
  • Syphilis
  • Third window syndromes
  • Endolymphatic sac tumor
  • Viral labyrinthitis
  • Transient ischemic attack (TIA), migraine
  • Vertebrobasilar disease
  • Other labyrinthine disorders (e.g., Cogan syndrome, benign positional vertigo, temporal bone trauma)
  • Diabetes or thyroid dysfunction
  • Vestibular neuronitis
  • Medication side effects
  • Otitis media
  • Autoimmune inner ear disease
  • Autosomal dominant sensorineural hearing loss
DIAGNOSTIC TESTS & INTERPRETATION
Testing is done to rule out other conditions but does not necessarily confirm or exclude Ménière disease.
Initial Tests (lab, imaging)
  • Consider serologic tests specific for Treponema pallidum in at-risk populations.
  • Thyroid, fasting blood sugar, and lipid studies
  • Consider MRI to rule out acoustic neuroma or other CNS pathology, including tumor, aneurysm, and multiple sclerosis (MS).
  • MRI can help diagnosis Ménière disease by identifying endolymphatic hydrops (1,2)[B].
Diagnostic Procedures/Other
  • Auditory
    • Audiometry using pure tone and speech to show low-frequency sensorineural (nerve) loss and impaired speech discrimination. Usually shows low-frequency sensorineural hearing loss
    • Tuning fork tests (i.e., Weber and Rinne) ABR or MRI to rule out acoustic neuroma
    • Electrocochleography may be useful to confirm etiology.
  • Vestibular
    • Caloric testing: Electronystagmography may show reduced caloric response. Can obtain reasonably comparable information with use of 0.8 mL of ice water caloric testing. Reduced activity on either side is consistent with Ménière diagnosis but is not itself diagnostic.
Test Interpretation
  • Histologic temporal bone analysis (at autopsy). Dilation of inner ear fluid system, neuroepithelial damage with hair cell loss, basement membrane thickening, and perivascular microvascular damage
  • Cytochemical analysis can reveal altered AQP4 and AQP6 expression in the supporting cell, altered cochlin, and mitochondrial protein expression (3)[B].
image TREATMENT
  • Usually managed in outpatient setting
  • A paucity of evidence-based guidelines exist; therefore, there is no gold standard treatment.
  • Medications are primarily for symptomatic relief of vertigo and nausea.
  • During attacks, bed rest with eyes closed prevents falls. Attacks rarely last >4 hours.
MEDICATION
First Line
  • Acute attack: Initial goal is stabilization and symptom relief. For severe episodes
    • Benzodiazepines (such as diazepam): decrease vertigo and anxiety
    • Antihistamines (meclizine/dimenhydrinate): decrease vertigo and nausea
    • Anticholinergics (transdermal scopolamine): reduces nausea and emesis associated with motion sickness
    • Antidopaminergic agents (metoclopramide, (promethazine): decrease nausea, anxiety
    • Rehydration therapy and electrolyte replacement
    • Steroid taper for acute hearing loss
  • Maintenance (goal is to prevent/reduce attacks)
    • Lifestyle changes (e.g., low-salt diet) are needed.
    • Diuretics may help reduce attacks by decreasing endolymphatic pressure and volume; there is insufficient evidence to recommend routine use:
      • Hydrochlorothiazide; hydrochlorothiazide/triamterene (Dyazide, Maxzide)
      • Acetazolamide (Diamox)
  • Contraindications/warnings:
    • Atropine: cardiac disease, especially supraventricular tachycardia and other arrhythmias, prostatic enlargement
    • Scopolamine: children and elderly, prostatic enlargement
    • Diuretics: electrolyte abnormalities, renal disease
  • P.651

  • Precautions:
    • Sedating drugs should be used with caution, particularly in the elderly. Patients are cautioned not to operate motor vehicles or machinery. Atropine and scopolamine should be used with particular caution.
    • Diuretics: Monitor electrolytes.
  • Significant possible interactions: transdermal scopolamine: anticholinergics, antihistamines, tricyclic antidepressants, other
Second Line
  • Steroids, both intratympanic and systemic (PO or IV) have been used for longer treatment of hearing loss:
    • Intratympanic administration results in higher steroid levels in the inner ear and may be more effective and safer than systemic (2)[C].
    • Addition of prednisone 30 mg/day to diuretic treatment reduced severity and frequency of tinnitus and vertigo in one pilot study.
  • In Europe, betahistine, a histamine agonist is routinely used (unavailable in the United States). Other vasodilators, such as isosorbide dinitrate, niacin, and histamine, have also been used; evidence of their effectiveness is incomplete.
  • Evidence is lacking for routine use of Famvir, may improve hearing more than balance.
  • Intratympanic gentamicin has shown to improve vertigo (4,5,6)[B].
ISSUES FOR REFERRAL
  • Consider ear, nose, throat/neurology referral.
  • Patients should have formal audiometry to confirm hearing loss.
ADDITIONAL THERAPIES
  • Application of intermittent pressures via a myringotomy using a Meniett device has been shown to relieve vertigo (7)[B]:
    • Safe; requires a long-term tympanostomy tube
  • Vestibular rehabilitation may be beneficial for patients with persistent vestibular symptoms:
    • Safe and effective for unilateral vestibular dysfunction
SURGERY/OTHER PROCEDURES
  • Interventions that preserve hearing:
    • Endolymphatic sac surgery, either decompression or drainage of endolymph into mastoid or subarachnoid space
      • Less invasive; may decrease vertigo; may influence hearing/tinnitus
    • Endolymphatic sac surgery is effective in controlling vertigo in short- and long-term follow-up in at least 75% of patients with Ménière disease who failed medical therapy (8)[A].
    • Vestibular nerve section (intracranial procedure)
      • More invasive
      • Decreases vertigo and preserves hearing
    • Tympanostomy tube: may decrease symptoms by decreasing the middle ear pressure
  • Interventions for patients with no serviceable hearing:
    • Labyrinthectomy: very effective at controlling vertigo but causes deafness
    • Vestibular neurectomy
    • Cochlear implantation has shown to improve tinnitus and quality of life (9)[B].
    • There are new surgeries on the rise, for example, endolymphatic duct blockage, which in one study proved to be superior to endolymphatic sac decompression (10)[B].
COMPLEMENTARY & ALTERNATIVE MEDICINE
Insufficient evidence to support effectiveness, but many integrative techniques have been tried, including the following:
  • Acupuncture, acupressure, tai chi
  • Niacin, bioflavonoids, Lipoflavonoids, ginger, ginkgo biloba, and other herbal supplements
image ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
Due to the possibility of progressive hearing loss despite decrease in vertiginous attacks, it is important to monitor changes in hearing and to monitor for more serious underlying causes (e.g., acoustic neuroma).
DIET
  • Diet is usually not a factor, unless attacks are brought on by certain foods.
  • A low salt is often recommended but not proven effective in randomized controlled trials.
PROGNOSIS
  • Alert patients about the nature of alternating attacks and remission.
  • Between attacks, patient may be fully active but is often limited due to fear or lingering symptoms. This can be severely disabling.
  • 50% resolve spontaneously within 2 to 3 years.
  • Some cases last >20 years.
  • Severity and frequency of attacks diminish, but hearing loss is often progressive.
  • 90% can be treated successfully with medication; 5-10% of patients require surgery for incapacitating vertigo.
  • There is a mental illness burden associated with Ménière disease (11)[B].
REFERENCES
1. Wu Q, Dai C, Zhao M, et al. The correlation between symptoms of definite Ménière disease and endolymphatic hydrops visualized by magnetic resonance imaging [published ahead of print August 29, 2015]. Laryngoscope.
2. Gu X, Fang ZM, Liu Y, et al. Diagnostic advantages of intratympanically gadolinium contrast-enhanced magnetic resonance imaging in patients with bilateral Ménière's disease. Am J Otolaryngol. 2015;36(1):67-73.
3. Ishiyama G, Lopez IA, Sepahdari AR, et al. Meniere's disease: histopathology, cytochemistry, and imaging. Ann N Y Acad Sci. 2015;1343:49-57.
4. Marques P, Manrique-Huarte R, Perez-Fernandez N. Single intratympanic gentamicin injection in Ménière's disease: VOR change and prognostic usefulness. Laryngoscope. 2015;125(8):1915-1920.
5. Rah YC, Han JJ, Park J, et al. Management of intractable Ménière's disease after intratympanic injection of gentamicin. Laryngoscope. 2015;125(4):972-978.
6. Casani AP, Cerchiai N, Navari E, et al. Intratympanic gentamicin for Meniere's disease: short- and long-acting follow-up of two regimens of treatment. Otolaryngol Head Neck Surg. 2014;150(5):847-852.
7. Ahsan SF, Standring R, Wang Y. Systematic review and meta-analysis of Meniett therapy for Meniere's disease. Laryngoscope. 2015;125(1):203-208.
8. Sood AJ, Lambert PR, Nguyen SA, et al. Endolymphatic sac surgery for Ménière's disease: a systematic review and meta-analysis. Otol Neurotol. 2014;35(6):1033-1045.
9. Mick P, Amoodi H, Arnoldner C, et al. Cochlear implantation in patients with advanced Ménière's disease. Otol Neurotol. 2014;35(7):1172-1178.
10. Saliba I, Gabra N, Alzahrani M, et al. Endolymphatic duct blockage: a randomized controlled trial of a novel surgical technique for Ménière's disease treatment. Otolaryngol Head Neck Surg. 2015;152(1):122-129.
11. Furukawa M, Kitahara T, Horii A, et al. Psychological condition in patients with intractable Ménière's disease. Acta Otolaryngol. 2013;133(6):584-589.
Additional Reading
&NA;
  • Long AF, Xing M, Morgan K. Exploring the evidence base for acupuncture in the treatment of Ménière's syndrome—a systematic review. Evid Based Complement Alternat Med. 2011;2011:429102.
  • Lopez-Escamez JA, Carey J, Chung WH, et al. Diagnostic criteria for Ménière's disease. J Vestib Res. 2015;25(1):1-7.
See Also
&NA;
Hearing Loss; Labyrinthitis; Tinnitus
Codes
&NA;
ICD10
  • H81.09 Meniere's disease, unspecified ear
  • H81.01 Meniere's disease, right ear
  • H81.02 Meniere's disease, left ear
Clinical Pearls
&NA;
  • Ménière disease is characterized by vertigo, hearing loss, and tinnitus +/− aural fullness.
  • There is a wide differential diagnosis for Ménière disease; therefore, one must fully investigate symptoms.
  • Multiple medical, surgical, and rehabilitative treatments are available to decrease the severity and frequency of attacks.