What is Meniere's disease?

What is Ménière's Disease?

Ménière's disease is a chronic inner ear disorder characterized by recurrent episodes of spontaneous vertigo lasting 20 minutes to 12 hours, documented low- to mid-frequency sensorineural hearing loss, fluctuating tinnitus, and aural fullness. 1, 2

Core Clinical Features

The disease presents with a classic tetrad of symptoms:

• Episodic vertigo attacks lasting specifically 20 minutes to 12 hours (not seconds or days), which distinguishes it from other vestibular disorders 1, 2
• Fluctuating sensorineural hearing loss that initially affects low- to mid-frequency ranges, documented by audiometry 1, 3
• Tinnitus that fluctuates in intensity, often worsening during acute attacks 1
• Aural fullness or pressure in the affected ear, described as a sensation of blockage or pressure 1, 2

Pathophysiology

• The hallmark pathological finding is endolymphatic hydrops (ELH), an excessive accumulation of endolymph fluid in the membranous labyrinth of the inner ear 4, 5
• The exact mechanism linking ELH to symptoms remains incompletely understood, as some patients develop symptoms early while others only after considerable endolymph accumulation 4
• Multiple biochemical pathways are involved, including the endocrine system, innate immune response, and autonomic nervous system 6
• Genetic susceptibility plays a role, with allergens, infectious agents, vascular events, or genetic factors potentially triggering the disease 6

Disease Progression Patterns

• Hearing loss initially fluctuates in the low- to mid-frequency range but may eventually involve all frequencies as the disease progresses 1, 2
• Hearing often worsens over time despite treatment interventions 1
• Bilateral involvement occurs in 25-40% of cases, though it typically starts unilaterally 6
• Drop attacks (Tumarkin's otolithic crisis) may occur in later stages, where patients suddenly fall without warning or loss of consciousness 2

Diagnostic Criteria

The American Academy of Otolaryngology-Head and Neck Surgery defines definite Ménière's disease as:

• Two or more spontaneous vertigo attacks, each lasting 20 minutes to 12 hours 2
• Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear 2
• Fluctuating aural symptoms (hearing loss, tinnitus, and/or fullness) 2
• Other causes excluded by appropriate testing 2

Key Diagnostic Considerations

• Audiometry is essential for documenting the characteristic sensorineural hearing loss pattern 3, 2
• The Weber tuning fork test in unilateral sensorineural hearing loss will lateralize to the unaffected ear (opposite to the affected ear) 3
• Normal otoscopic examination with hearing loss should raise suspicion for sensorineural rather than conductive hearing loss 3
• MRI can directly visualize endolymphatic hydrops in the cochlea, vestibule, and semicircular canals, shifting from research to clinical use 4

Important Differential Diagnoses to Exclude

• Vestibular migraine: vertigo episodes lasting minutes to days, often with headache history 2
• Benign paroxysmal positional vertigo (BPPV): brief vertigo episodes provoked by head position changes 2
• Labyrinthitis: sudden severe vertigo with prolonged symptoms (>24 hours) and hearing loss 2
• Vestibular neuritis: acute prolonged vertigo without hearing loss 2
• Cerebellopontine angle tumors: progressive unilateral hearing loss with imbalance 2

Clinical Pitfalls

• Distinguishing between temporary fluctuations and permanent progression of hearing loss is crucial for treatment decisions 1
• Headache is commonly found during attacks, which can lead to confusion with vestibular migraine 6
• Patients must understand that complete restoration of hearing is not currently possible, though symptoms can be managed 1