Logo for University of Iowa Health Care This logo represents the University of Iowa Health Care
New Addition: Sleep Apnea Management - Hypoglossal Nerve Stimulation - September 2021Click Here

Meniere's Disease Overview

last modified on: Thu, 11/05/2020 - 10:19

Return to Otology - NeurotologyVertigo

Characteristics

  • Clinical syndrome associated with a specific set of symptoms
  • Pathologically described as endolymphatic hydrops
  • Chronically progressive, destructive disorder involving both the cochlea and labyrinth
  • Permanent hearing loss and vestibular injury
  • Can affect both ears
  • Relapsing, remitting course

Etiology

  • Idiopathic
  • Genetics and environmental exposure play a role in its development

Pathophysiology

  • Accumulation of endolymph in the cochlear duct and vestibular organs (endolymphatic hydrops)

Symptoms

  • Episodes lasting 30 minutes to several hours of:
    • Spontaneous vertigo
    • Unilateral fluctuating sensorineural hearing loss (SNHL)
    • Tinnitus
    • Aural fullness
  • Natural History
    • Vertigo symptoms more common at beginning of disease
    • Hearing loss and vestibular hypofunction vary greatly between patients
      • Hearing loss fluctuates spontaneously in the first few years
      • After repeated attacks hearing loss may progress and become permanent
    • Triggers tend to develop later in the disease as the result of advanced hydrops bringing the membranous labyrinth in close proximity to the stapes foot plate
  • International Classification of Vestibular Disorders (ICVD) Criteria
    • Definite Meniere's Disease
      • > 2 episodes of spontaneous vertigo lasting 20 minutes to 12 hours
      • Audiometrically documented low- to medium frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during or after one of the episodes of vertigo
      • Fluctuating aural symptoms (hearing, tinnitus or fullness) in the affected ear
      • Not better accounted for by another vestibular diagnosis
    • Probable Meniere's Disease
      • > 2 episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
      • Fluctuating aural symptoms (hearing, tinnitus or fullness) in the affected ear
      • Not better accounted for by another vestibular diagnosis
  • Triggers - often occur later in disease
    • Tullio's Phenomenon
      • Episodes of vertigo lasting seconds to minutes triggered by high intensity and low frequency sounds
    • Dietary
      • Excessive consumption of salt or caffeine
    • Changes in Pressure

Differential Diagnosis

  • Transient Ischemic Attack (TIA)
  • Vestibular migraine
  • Vestibular paroxysmia
  • Recurrent unilateral vestibulopathy
  • ​Vestibular schwanomma
  • Endolymphatic sac tumor
  • Autoimmune disease
  • Human Immunodeficiency Virus (HIV)
  • Syphilis

Diagnosis

  • History
    • < 50 y.o. ask about migraine headaches
    • Snoring (association with sleep apnea)
    • Vascular factors
      • Smoking
      • Diabetes mellitus
      • Vasculitis
      • Myocardial infarction (MI)
      • Stroke
    • Family History
      • Familial Meniere's Disease - at least one other relative (first or second degree) fulfills all the criteria of definite or probable MD
        • 8%–9% of sporadic cases in populations of European descent 
        • Genetic heterogeneity, and mitochondrial and recessive inheritance patterns
        • Mutations in DTNA and FAM136A genes
  • MRI - rule out structural cause
  • Electrocochleography (ECoG)
  • Auditory brain stem response (ABR)
  • Electronystagmogram (ENG)
  • Audiometry

Management

  • Surgical
    • Ablative Procedures - greater vertigo control rates compared to non-ablative procedures but require vestibular compensation to limit post-treatment disequilibrium
      • Gentamicin middle ear injections
      • Labyrinthectomy - most reliable
        • Permanent Hearing Loss
        • Removal of all of the vestibular neuroepithelium
      • Vestibular Nerve Section
        • Vestibular division of CN VIII is selectively divided to remove vestibular function from affected side
        • Approaches:
          • Middle fossa
          • Retrolabyrinthine
          • Retrosigmoid
          • Translabyrinthine
        • Complications
          • Facial paralysis
          • Hearing loss
          • CSF leak
          • Persistent disequilibrium
    • Non-ablative Procedures
      • Endolymphatic shunt (see Sham Surgery Trial by Thomsen et al, 1981)
        • shunting - placement of synthetic shunt to drain endolymph
        • drainage - incision of the sac to allow endolypmh drainage
        • decompression - improve sac function of endolymph absorption
      • Intratympanic steroid perfusion
    • American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) on Reporting of Vertigo Control in Meniere's Disease
      • 18-24 months following treatment: divide number of episodes per 6 months by the number of episodes in the 6 months prior to treatment
        • Grade A: complete control (0%)
        • Grade B: Substantial control (1% - 40%)
        • Grade C: Partial control (41% - 80%)
        • Grade D: No control (80% - 120%)
        • Grade E: Worse (>120%)
        • Grade F: Secondary treatment required due to disabling vertigo
  • Pharmacologic
    • Vestibulosuppressants
      • Meclizine [Antivert]
      • Droperidol [Inapsine]
      • Prochlorperazine [Compazine]
      • Benzodiazepines
        • Diazepam [Valium]
        • Lorazepam [Ativan]
        • Alprazolam [Xanax]
    • Diuretics
      • Hydrochlorothiazide and triamterene [Dyazide]
      • Hydrochlorothiazide [Aquazide]
      • Acetazolamide [Diamox]
      • Methazolamide [Neptazane])
    • Steroids
      • Routes of Administration: orally, intramuscularly, intratympanically
    • Aminoglycosides
  • ​Lifestyle
    • Low salt diet (< 2g/day)

image from wikicommons with permission By VestibularSystem.png: *VestibularSystem.gif: user:Thomas.haslwanterderivative work: Ortisa (talk)derivative work: Icewalker cs (talk) - VestibularSystem.png, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=16880697

​References

American Academy of Otolaryngology - Head and Neck Surgery. (2017). Meniere's Disease. http://www.entnet.org/content/menieres-disease

Lopez-Escamez J. A., Carey J., Chung W.-H., Goebel J. A., Magnusson M., Mandalà M., et al. . (2015). Diagnostic criteria for Menière's disease. J. Vest. Res. 25, 1–7. 10.3233/VES-150549

Scholes M. A., Ramakrishnan V. R. (2016). ENT Secrets Fourth Edition. Elsevier. 246, 293-294.

Thomsen J, Bretlau P, Tos M, Johnsen N J. Placebo effect in surgery for Ménière's disease. A double-blind, placebo-controlled study on endolymphatic sac shunt surgery. Arch Otolaryngol. 1981;107(05):271–277.