Ménière’s Disease- Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

  • An inner ear (labyrinthine) disorder characterized by recurrent attacks of hearing loss, tinnitus, vertigo, and sensations of aural fullness. Generally believed to be caused by, or related to, an increase in the volume and pressure of the inner ear endolymph fluid (endolymphatic hydrops).
  • Often unilateral initially, but it is estimated that nearly half become bilateral over time.
  • Severity and frequency of vertigo may diminish over 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: 40–60 years
  • Predominant gender: Female > Male (1.3:1)
  • Race/ethnicity: white, northern European > Blacks

Incidence

Estimates 1–150/100,000/year

Prevalence

Varies from ∼7.5 to >200/100,000.

Risk Factors

Not well understood, but may include:

  • Stress
  • Allergy
  • Increased salt intake
  • Caffeine, alcohol, or nicotine intake
  • Chronic exposure to loud noise
  • Family history of Ménière
  • Certain vascular abnormalities (patients may also have history of migraines)
  • Certain viral exposures (especially herpes simplex virus [HSV])

Genetics

Some families show increased incidence, but genetic vs environmental influences are not well understood.

General Prevention

Reduce risk factors: Stress; salt, alcohol, and caffeine intake; smoking; noise exposure; ototoxic drugs (aspirin, quinine, aminoglycosides, etc.)

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.

Etiology

Idiopathic (Ménière disease), but Ménière syndrome may be secondary to injury or other disorder (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.

Commonly Associated Conditions

  • Endolymphatic hydrops
  • Anxiety (secondary to the disabling symptoms)
  • Migraines
  • Theorized: Hypothyroidism
  • Hyperprolactinemia

Vestibular schwannoma, Sensorineural hearing loss, vestibular aqueduct, abnormal development, noise exposure, endolymphatic hydrops, environmental influences, inner ear,

Diagnosis

Diagnosis is clinical. Tests rule out other conditions (1)[B].

History

  • Attacks are typically spontaneous but may be preceded by an aura of increasing fullness in the ear and tinnitus. These may occur in clusters with long intervening symptom-free remissions. Signs and symptoms to look for include:
  • Formal criteria for diagnosis from AAO-HNS:
    • At least 2 episodes of rotational-horizontal vertigo >20 minutes in duration
    • Tinnitus or aural fullness
    • Hearing loss: Low frequency (sensorineural) confirmed by audiometric testing
    • Other causes (acoustic neuroma, etc.) excluded
  • During severe attacks: Pallor, sweating, nausea, vomiting, falling, prostration
  • Symptoms are exacerbated by motion.
  • Between attacks, affected patients may experience motion-related imbalance without vertigo.
  • Caution: Many conditions may produce auditory and vestibular findings identical to those associated with Ménière disease.

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 Meniere.

Diagnostic Tests & Interpretation

Testing done to rule out other conditions, and does not necessarily confirm or exclude Meniere disease

Lab

Initial lab tests

  • Serologic tests specific for Treponema pallidum: Microhemagglutination (MHA), fluorescent treponemal antibody (FTA), Treponema immobilization test (TPI) (1)[C]
  • Thyroid, fasting blood sugar, and lipid studies

Imaging

Magnetic resonance imaging (MRI) to rule out acoustic neuroma or other CNS pathology, including tumor, aneurysm, and multiple sclerosis (MS).

Initial approach

  • Detailed history and physical exam
  • Labs, audiometry, consider MRI

Diagnostic Procedures/Surgery

  • 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) will confirm validity of audiometry.
    • Auditory brainstem response audiometry (ABR) to rule out acoustic neuroma
    • Electrocochleography (ECOG) may be useful to confirm etiology (1).
  • Vestibular:
    • Caloric testing: Electronystagmography (ENG) 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.
    • Drugs that may alter lab results: Any sedating medication may affect and invalidate vestibular testing.

Pathological Findings

Histologic temporal bone analysis (at autopsy). Dilation of inner ear fluid system may be seen.

Differential Diagnosis

  • Acoustic neuroma or other CNS tumor
  • Syphilis
  • Perilymphatic fistula
  • Viral labyrinthitis
  • Transient ischemic attack (TIA), migraine
  • Vertebrobasilar disease
  • Other labyrinthine disorders that produce similar symptoms (e.g., Cogan syndrome, benign positional vertigo, temporal bone trauma)
  • Diabetes or thyroid dysfunction
  • Vestibular neuronitis
  • Medication side effects
  • Otitis media

Treatment

  • Can usually be managed in outpatient setting
  • A paucity of evidence-based guidelines exist regarding treatment for Ménière disease; therefore, there is no “gold standard” treatment.
  • Medications are given primarily for symptomatic relief of vertigo and nausea, not to change disease progression.
  • During attacks, bed rest with eyes closed and protection from falling. Attacks rarely last >4 hours.

Medication

First Line

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  • Acute attack: Initial goal is immediate stabilization and symptom relief. For severe episodes, choose one: (2)[C]
    • Benzodiazepines (such as diazepam): Decrease vertigo and anxiety
    • Antihistamines (meclizine/dimenhydrinate): Decrease vertigo and nausea
    • Anticholinergics (transdermal scopolamine): Prevents nausea and emesis associated with “motion sickness”
    • Antidopaminergic (metoclopramide, promethazine): Decreases nausea, anxiety
    • Rehydration therapy and electrolyte replacement
    • Steroid taper for acute hearing loss
  • Maintenance (goal is to prevent/reduce attacks)
  • Lifestyle changes (low-salt diet, etc.) are needed.
  • Diuretics are frequently used, and may help reduce attacks by decreasing the pressure and volume of endolymphatic fluid; however, there is insufficient evidence at this time (2)[C],(3)[A]:
    • Hydrochlorothiazide/Triamterene (Dyazide, Maxzide)
    • Acetazolamide (Diamox)
  • Contraindications:
    • Atropine: Cardiac disease, especially SVT and other arrhythmias, prostatic enlargement
    • Scopolamine: Children and elderly, prostatic enlargement
    • Diuretics: Electrolyte abnormalities, renal disease
  • Precautions:
    • Sedating drugs should be used with caution, particularly in the elderly. Patients should be cautioned not to operate motor vehicles or machinery. Atropine and scopolamine should be used with particular caution.
    • Diuretics: Monitor electrolytes. Use with caution in patients with sulfa allergy.
  • Significant possible interactions: Transdermal scopolamine: Anticholinergics, belladonna products, antihistamines, tricyclic antidepressants, other

Second Line

  • Steroids have been used, both intratympanic and systemically (oral or IV) 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/d to diuretic treatment reduced severity and frequency of tinnitus and vertigo in 1 pilot study (4)[C].
  • In Europe, one of the preferred drugs is betahistine, a histamine agonist (unavailable in US). Insufficient evidence to state effectiveness: Other vasodilators, such as isosorbide dinitrate, niacin and histamine, have also been used, but evidence of their effectiveness is sparse (2)[C].
  • Famvir has also been studied for the treatment of vertigo and stabilization of hearing. Evidence is lacking, but indicates more improvement in hearing than balance (5)[B].
  • Inner ear perfusion with gentamicin and steroids have helped control and stabilize vertigo and hearing loss in Meniere’s disease (6)[A]

Additional Treatment

Issues for Referral

  • Consider ear, nose, throat (ENT)/neurology referral for confirmation, further testing.
  • All patients need formal audiometry to confirm hearing loss.

Additional Therapies

  • Application of intermittent pressures via a myringotomy using a Meniett device has been found in some studies to relieve dizziness:
    • Safe; requires a long-term tympanostomy tube
  • Vestibular rehabilitation may be beneficial for patients with persistent vestibular symptoms (7)[A]:
    • Safe and effective treatment for unilateral vestibular dysfunction
    • When attacks are disabling, the patient should be encouraged to slowly resume activity as soon as able.

Complementary and Alternative Medicine

Insufficient evidence to support effectiveness, but many integrative (CAM) techniques have been tried, including (2)[C]:

  • Acupuncture (8)[A], acupressure, Tai Chi
  • Niacin, bioflavonoids, lipoflavonoids, ginger, ginkgo biloba, and other herbal supplements

Surgery/Other Procedures

  • Interventions that preserve hearing (9)[B]:
    • Endolymphatic sac surgery, either decompression or drainage of endolymph into mastoid or subarachnoid space:
      • Less invasive; may decrease vertigo, may influence hearing or tinnitus
      • There is insufficient evidence of the beneficial effect of endolymphatic sac surgery in Ménière’s disease (10)[A]
    • Vestibular nerve section (intracranial procedure):
      • More invasive due to intracranial location
      • Decreases vertigo and preserves hearing
    • Tympanostomy tube: May decrease symptoms by decreasing the middle ear pressure
  • Interventions for patients with no serviceable hearing (9)[C]:
    • Labyrinthectomy: Very effective at controlling vertigo, but causes deafness
    • Vestibular neurectomy
    • Many patients may be candidates for cochlear implantation if they have lost serviceable hearing.

Ongoing Care

Follow-Up Recommendations

Patient Monitoring

Due to the possibility of progressive hearing loss despite eventual decrease in vertiginous attacks, it is important to have close follow-up to monitor changes in hearing, and continue surveillance for more serious underlying causes (acoustic neuroma, etc.).

Diet

  • Diet is usually not a factor, unless attacks are brought on by certain foods.
  • Some physicians restrict salt, but this is not supported by randomized controlled trials (1)[C].

Patient Education

  • Limit activity during attacks.
  • Between attacks, patient may be fully active but is often limited due to fear or lingering symptoms. This can be severely disabling.
  • Patient information, including support group contacts, is available from the Vestibular Disorders Association: http://www.vestibular.org and the American Academy of Otolaryngology-Head and Neck Surgery: http://www.entnet.org/HealthInformation/menieresDisease.cfm

Prognosis

  • Alternating attacks and remission
  • 1/2 of cases resolve spontaneously within 2–3 years, but can last >20 years. Severity and frequency of attacks diminish, but hearing loss is often progressive.
  • 90% of patients can be managed successfully with medication. 5–10% of patients require surgery for incapacitating vertigo.
  • Clinicians must not overlook possibility of acoustic tumor, which produces an identical clinical picture.

Complications

Loss of hearing; injury during attack; inability to work

References

1. Sajjadi H, Paparella MM. Meniere’s disease. Lancet. 2008;372:406–14.

2. Coelho DH, Lalwani AK. Medical Management Of Ménière’s Disease. Laryngoscope. 2008.

3. Thirlwall AS, Kundu S. Diuretics for Ménière’s disease or syndrome. Cochrane Database Syst Rev. 2006;3:CD003599.

4. Morales-Luckie E, Cornejo-Suarez A, Zaragoza-Contreras MA, et al. Oral administration of prednisone to control refractory vertigo in Ménière’s disease: a pilot study. Otol Neurotol. 2005;26:1022–6.

5. Derebery MJ, Fisher LM, Iqbal Z. Randomized double-blinded, placebo-controlled clinical trial of famciclovir for reduction of Ménière’s disease symptoms. Otolaryngol Head Neck Surg. 2004;131:877–84.

6. Hamid M et al. Medical management of common peripheral vestibular diseases. Curr Opin Otolaryngol Head Neck Surg. 2010;18:407–12.

7. Hillier SL, Hollohan V. Vestibular rehabilitation for unilateral peripheral vestibular dysfunction. Cochrane Database Syst Rev. 2007;CD005397.

8. Long AF, Xing M, Morgan K, et al. Exploring the Evidence Base for Acupuncture in the Treatment of Meniere’s Syndrome–A Systematic Review. Evid Based Complement Alternat Med. 2009.

9. van Benthem PP, Giard JL, Verschuur HP. Surgery for Ménière’s disease (Protocol). Cochrane Database of Systematic Reviews. 2005, Issue 3. Art. No.: CD005395. DOI:10.1002/14651858.CD005395.

10. Pullens B, Giard JL, Verschuur HP, van Benthem PP et al. Surgery for Ménière’s disease. Cochrane Database Syst Rev. 2010;CD005395.

Additional Reading

See Also (Topic, Algorithm, Electronic Media Element)

Labyrinthitis; Tinnitus; Hearing Loss

Algorithm: Vertigo

Codes

ICD9

386.00 Meniere’s disease, unspecified

Snomed

13445001 Meniere’s disease (disorder)

Clinical Pearls

  • Diagnosis of Ménière disease is clinical, based on repeated episodes of vertigo, hearing loss, and tinnitus or aural fullness.
  • Multiple medical, surgical, and rehabilitative treatments are available to decrease the severity and frequency of attacks. Some of these may preserve hearing, while some destroy it.
  • A patient is likely to have progressive hearing loss despite a natural progression toward fewer vertigo attacks.
  • When considering Ménière disease in a patient with vertigo and hearing loss, acoustic neuromas must also be considered.
  • Take symptoms seriously, as they may be disabling.

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