Disease 05.2

Ménière's Disease

A clinical diagnosis whose histopathological substrate — endolymphatic hydrops — leaves a recognisable VEMP signature: reduced amplitudes and a frequency-tuning shift.

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Foundation

What is Ménière's disease?

Ménière's disease is a clinical syndrome of episodic vertigo, fluctuating low-frequency sensorineural hearing loss, and tinnitus, often accompanied by aural fullness. Its histopathological hallmark is endolymphatic hydrops — distension of the endolymphatic compartment of the inner ear.

AudiogramThe pure-tone signature
1252505001k2k4k8kFrequency (Hz)020406080100dB HLRL
Classic Ménière's pattern: low-frequency sensorineural hearing loss that fluctuates with attacks. Over years the loss may flatten and progress to a moderate-severe pancochlear shape. Pair with the cVEMP frequency-tuning shift to add specificity.
01020304050Time (ms)NormalAffected ear
cVEMP amplitude is markedly reduced on the affected side; latencies are within normal limits.
Trainee

VEMP signature

  • Reduced amplitude on the affected side — the most common abnormality.
  • Frequency-tuning shift — best response frequency moves from 500 Hz toward 1 kHz in hydropic saccules.
  • Increased IAR, sometimes with raised threshold.
  • Absent responses are seen in advanced disease.
Clinician

Evidence base

A meta-analysis of cVEMP in Ménière's pooled a diagnostic odds ratio of approximately 40 with an area under the ROC curve of 0.78. The 2017 AAN guideline, however, rated routine VEMP evidence as Level C-negative in Ménière's — useful in some research contexts and for monitoring, but not yet diagnostic by itself.[1,8]

VEMP nevertheless plays a useful role in disease staging — declining amplitudes track progression — and in research on the otolithic involvement of subtypes (delayed endolymphatic hydrops, atypical Ménière's, and the role of saccule vs utricle in drop-attack patients).

Reading the report

A unilateral reduced cVEMP amplitude alongside a fluctuating low-frequency hearing loss is consistent with hydrops but cannot establish Ménière's by itself — vestibular neuritis can produce a similar VEMP pattern. The clinical history of episodic vertigo and the audiometric shape remain the diagnostic anchors.