What are the clinical presentation and management options for Meniere's disease?

Clinical Presentation of Ménière's Disease

Ménière's disease presents with a classic tetrad: spontaneous vertigo attacks lasting 20 minutes to 12 hours, fluctuating low- to mid-frequency sensorineural hearing loss, tinnitus, and aural fullness in the affected ear. 1

Diagnostic Criteria for Definite Ménière's Disease

To establish a diagnosis of definite Ménière's disease, all of the following must be present 1:

• Two or more spontaneous vertigo attacks, each lasting 20 minutes to 12 hours (not seconds or days) 1
• Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after a vertigo episode 1, 2
• Fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear 1
• Other causes excluded by appropriate testing 1

Core Symptom Characteristics

Vertigo Pattern

• The vertigo must be true rotational spinning, not lightheadedness or presyncope 1
• Episodes are spontaneous (not positional) and last specifically 20 minutes to 12 hours 1
• Attacks are accompanied by nausea and vomiting during the acute phase 3
• Important caveat: Elderly patients or those with long-standing disease may present with "vague dizziness" or severe imbalance rather than classic spinning vertigo 1

Hearing Loss Pattern

• Initially affects low- to mid-frequencies with a fluctuating pattern 1, 4
• Hearing may improve between attacks early in the disease course 5
• Over time, the hearing loss progresses to involve all frequencies and becomes permanent 2, 5
• The Weber tuning fork test lateralizes to the unaffected ear 4

Associated Aural Symptoms

• Tinnitus: Often described as roaring or low-pitched, fluctuates with attacks 5, 3
• Aural fullness: Sensation of pressure or blockage in the affected ear 1, 3
• These symptoms typically worsen before or during vertigo attacks 1

Physical Examination Findings

• Physical examination is typically unremarkable between attacks 1
• During acute attacks, nystagmus may be present 3
• Otoscopic examination is normal (no middle ear pathology) 4
• Neurologic examination should be normal; any focal neurologic signs suggest alternative diagnoses 1

Advanced Disease Features

• Drop attacks (Tumarkin's otolithic crisis): Sudden falls without warning or loss of consciousness, occurring in later stages 1, 2
• Progressive hearing deterioration despite treatment 5
• Bilateral involvement occurs in 25-40% of cases over time 3

Critical Differential Diagnoses

The following conditions must be distinguished from Ménière's disease 1:

Vestibular Migraine

• Vertigo episodes may be shorter (<15 minutes) or longer (>24 hours) than Ménière's disease 1
• Hearing loss is mild, absent, or stable (not fluctuating) 1
• History of migraine headaches with photophobia and motion intolerance 1
• Visual auras more common than in Ménière's disease 1

Benign Paroxysmal Positional Vertigo (BPPV)

• Vertigo lasts seconds, not minutes to hours 1
• Triggered by specific head position changes 2
• No hearing loss, tinnitus, or aural fullness 1

Vestibular Neuritis

• Single prolonged episode of severe vertigo lasting 12-36 hours with gradual improvement over days 1
• No hearing loss, tinnitus, or aural fullness 1, 2
• Not episodic or fluctuating 1

Labyrinthitis

• Sudden severe vertigo with profound hearing loss lasting >24 hours 1
• Symptoms are not episodic and do not fluctuate 1, 2
• May have fever and severe ear pain if infectious etiology 1

Vestibular Schwannoma

• Chronic progressive imbalance rather than episodic vertigo 1
• Hearing loss is progressive and does not fluctuate 1, 2
• Asymmetric hearing loss with poor word discrimination 1

Stroke/Ischemia

• Vertigo may be brief but associated with other neurologic symptoms (dysphagia, dysphonia, visual changes) 1
• Symptoms are permanent, not fluctuating 1
• Usually no hearing loss or tinnitus 1

Essential Diagnostic Testing

Audiometry

• Mandatory for diagnosis to document the characteristic low- to mid-frequency sensorineural hearing loss pattern 2, 4
• Should be performed during or shortly after an attack to capture fluctuation 1
• Serial audiograms are essential for monitoring disease progression 2, 5

Vestibular Testing

• Caloric testing, vestibular-evoked myogenic potentials (VEMP), and head impulse tests may support the diagnosis 6
• Electrocochleography can demonstrate endolymphatic hydrops 1

Common Diagnostic Pitfalls

• Misinterpreting "dizziness" as vertigo: Patients must describe true rotational spinning; lightheadedness or presyncope is not consistent with Ménière's disease 1
• Missing vestibular migraine: Always assess for migraine history, as VM closely mimics Ménière's disease but requires different management 1, 2
• Failing to document hearing loss audiometrically: Clinical suspicion alone is insufficient; objective audiometric documentation is required 1, 2
• Overlooking bilateral disease: Up to 40% of patients develop bilateral involvement, which has significant implications for treatment decisions 3

Probable Ménière's Disease

When the full criteria are not yet met, probable Ménière's disease may be diagnosed with 1:

• At least 2 episodes of vertigo or dizziness lasting 20 minutes to 24 hours
• Fluctuating aural symptoms in the affected ear
• Other causes excluded by testing