return to: Otology - Neurotology; Vertigo

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.