Meniere Disease

from: MedicineToday 2014; 15(3): 18-26

Hallmark of Acute Attack:

• Prolonged vertigo
• Sudden, intense sensation of movement (usually spinning)
• Lasts at least 20 minutes
• Accompanied by nausea, vomiting, diarrhea, sweating

Vertigo Characteristics:

• Vertigo lasting a few seconds or minutes: likely BPPV
• Vertigo lasting 8-12+ hours: vestibular neuritis or cerebellar stroke
• Light-headedness, balance disturbances: non-otological causes (e.g., postural hypotension, hyperglycemia, panic attacks)

Early Stage:

• Vertigo symptoms prominent
• Minor, unnoticed fluctuations in hearing

Middle Stage:

• More marked fluctuating hearing loss (low tones)
• Permanent hearing loss may persist between attacks

Late Stage:

• Permanent, nonfluctuating hearing loss
• Attacks of vertigo lessen
• Dominant symptom: hearing loss (‘burnt out’ Ménière’s disease)
• Tinnitus becomes more prominent
• Drop attacks (sudden loss of balance without losing consciousness)

Natural History:

• Highly variable
• Vertigo attacks: daily clusters over weeks or isolated episodes with remissions lasting months or years
• Later stages: vertigo episodes ‘burn out,’ severe hearing loss dominates
• Long-term: 30%-45% develop the disease in the contralateral ear within 30 years

Variants:

• Classical Ménière’s: vertigo precedes hearing loss
• Lermoyez syndrome: hearing loss precedes vertigo and improves after vertigo attacks
• Cochlear hydrops: fluctuation in hearing thresholds with tinnitus and fullness but no vertigo

Epidemiology:

• Prevalence rates: 3.5 to 513 per 100,000 population
• More common in women
• Prevalence increases with age, peaks at 60-69 years
• Rare in people younger than 20 years

Pathophysiology – Current Understanding:

• Episodic disturbances of endolymph formation and resorption
• Failure of complex inner ear homeostasis caused by various pathologies
• Causes: autoimmune diseases, viral infections, trauma, allergy, hormonal changes, unknown causes

Differential Diagnosis

• Basilar Migraine: Vertigo without aural symptoms.
• Vestibular Neuronitis: Vertigo for several days, no aural symptoms.
• Benign Paroxysmal Positional Vertigo (BPPV): Vertigo related to head movements, no aural symptoms.
• Medications: Aminoglycosides, loop diuretics.
• Central Vertigo: Stroke, multiple sclerosis, seizure disorder.
• Peripheral Vertigo: Non-otogenic origin (e.g., peripheral neuropathy).
• Orthostatic Hypotension: Described as “dizziness” by patients.
• Neoplasm: Vestibular schwannoma, meningioma, malignancy.
• Infectious Causes: Meningitis, syphilis, HIV cerebritis.

Diagnosis

Diagnosis Criteria (Barany Society):

• Definite Meniere Disease:
  • Two or more episodes of vertigo (20 minutes to 12 hours).
  • Low- to medium-frequency sensorineural hearing loss in one ear.
  • Fluctuating aural symptoms (fullness, hearing, tinnitus) in the affected ear.
  • No better explanation by other vestibular diagnoses.
• Probable Meniere Disease:
  • Two or more episodes of dizziness/vertigo (20 minutes to 24 hours).
  • Fluctuating aural symptoms in the affected ear.
  • Better explained by another vestibular diagnosis.

Clinical Examination:

• History:
  • Character of vertigo (differentiate true vertigo from imbalance or presyncope).
  • Hearing loss, duration of episodes, positional triggers.
  • Family history of hearing and balance problems.
• Physical Examination:
  • Comprehensive neurologic examination.
  • Cranial nerves, peripheral sensation, gait, cerebellar testing, Romberg, Fukuda, pronator drift tests.
  • Rinne and Weber Tests: Assess auditory nerve function.
  • Frenzel Goggles: Identify nystagmus.
  • Dix-Hallpike Maneuver: Differentiate BPPV.

Between Attacks:

• Unilateral sensorineural hearing loss or bilateral asymmetrical hearing loss
• Romberg’s and Unterberger’s stepping tests may show mild disturbance of balance

During Acute Attack:

• Patients appear unwell, sweaty, pale, nauseated, vomiting
• Horizontal nystagmus that changes direction as the attack progresses

Investigations:

• Pure Tone Audiogram: Sensorineural hearing loss, commonly low tones
• Vestibular Function Tests: For atypical cases or bilateral disease
• Electrocochleography: Confirms diagnosis in atypical cases
• Imaging (MRI, CT): Exclude acoustic neuromas and other intracranial pathologies

Differential Diagnosis

Common Causes of Disturbed Balance:

• Nonvestibular Causes:
  • Light-headedness, disorientation, floating
• Vestibular Causes:
  • True vertigo (spinning, rocking, tilting)

Conditions Mimicking Ménière’s Disease:

• Vestibular Migraine:
  • Common and may mimic Ménière’s disease
• Herpes Zoster, Ramsay Hunt Syndrome:
  • Pain, vertigo, hearing loss, vesicles in the ear canal
• Meningitis, Carcinoma, Lymphoma, Sarcoid:
  • Vestibular and cochlear dysfunction with other cranial nerve lesions
• Vasculitides:
  • Ear- and eye-specific syndromes (Cogan’s syndrome, Susac’s syndrome)
• Brainstem Lesions:
  • Vestibular nerve root or nucleus involvement (e.g., multiple sclerosis)

Management

Goals:

• Reduce frequency and severity of symptoms
• Improve quality of life for patients and families

Acute Vertiginous Attacks:

• Safety Strategies:
  • While driving or in dangerous situations
• Allow Attack to Pass:
  • Recovery may take a day or two

Support:

• Meniere’s Australia: Patient information and reassurance
• Vestibular Rehabilitation: Referral: For exercises to improve balance and reduce vertigo symptoms.

Lifestyle and Dietary Changes:

• Low-Salt Diet: 1-2 g/day, avoid salty foods, processed and fast foods
• Reducing Caffeine, Chocolate, Alcohol: Beneficial for some patients
• Regular Exercise: 30 minutes brisk walk, three to five days a week

Medications:

• Betahistine:
  • Mainstay treatment to reduce the frequency and severity of vertigo attacks.
  • Histamine analogue, 8-32 mg/day, starting with 16 mg twice daily
• Diuretics:
  • Hydrochlorothiazide, frusemide, spironolactone
  • Helps in reducing fluid retention, thereby preventing endolymphatic hydrops.
• Corticosteroids:
  • Oral prednisone 1 mg/kg/day for 10 days or intratympanic dexamethasone 4 mg single dose
• Acute Management:
  • Antiemetics (e.g., Promethazine, Metoclopramide): For nausea control.
  • Antihistamines (e.g., Diphenhydramine, Meclizine): For short-term use during acute episodes.

Hearing Aids and Tinnitus Management:

• Self-Programming Hearing Aids: Adjustable to current hearing thresholds
• Cochlear Implants: Effective for severe hearing loss
• Tinnitus Management: Education, sound therapy, short-term drug therapy, tinnitus retraining therapy, cognitive behavioral therapy

Semi-Invasive and Surgical Treatments:

• Grommets (Tympanostomy Tubes): Simple, temporary, low-risk procedure
• Micropressure Therapy: Pressure pulses to the inner ear via a portable pressure generator
• Aminoglycoside Treatment (Gentamicin): Chemical labyrinthectomy, transtympanic injection, permanent effect on vestibular hair cells, small risk of hearing loss
• Endolymphatic Sac Surgery: Declined in popularity, no better than placebo
• Labyrinthectomy and Vestibular Nerve Section: Complete unilateral surgical deafferentation, effective but high risk, replaced by transtympanic gentamicin

Role of the GP

• Recognize and differentiate Ménière’s from other causes of vertigo
• Provide education on disease management
• Refer to a specialist for diagnosis and treatment plan

Conclusion

• Ménière’s disease can be frightening and sudden
• Later stages: hearing loss and tinnitus become more intrusive
• Diagnosis: careful history and simple audiogram
• Management: stepwise treatment plan involving lifestyle changes, medication, and potentially surgery

Practice Points

• Ménière’s disease: recurrent vertigo, fluctuating hearing loss, tinnitus, aural fullness
• Prevalence increases with age, peaks at 60-69 years, rare under 20 years
• Differential diagnosis includes migraine-associated vertigo
• Management: lifestyle changes, medical and surgical interventions
• Vertigo control achievable in most patients within two years