Meniere disease
What it is
Meniere disease is an episodic inner-ear disorder characterised by recurrent attacks of spontaneous vertigo lasting minutes to hours, fluctuating low-frequency sensorineural hearing loss, tinnitus, and aural fullness. The Bárány Society / AAO-HNS 2015 diagnostic criteria require two or more spontaneous vertigo episodes lasting 20 minutes to 12 hours, audiometrically documented low-to-mid-frequency hearing loss in one ear, and fluctuating aural symptoms on the affected side[Lopez-Escamez JA 2015].
The pathophysiology is widely attributed to endolymphatic hydrops — accumulation of endolymph within the membranous labyrinth — though hydrops on imaging or autopsy is not perfectly specific for clinical Meniere disease, and the causal arrow remains debated.
The dissociation that defines the disease
Here is the clinically useful fact, and the reason Meniere disease has a chapter in a vHIT atlas despite the test usually looking normal: Meniere disease characteristically shows reduced caloric response with preserved vHIT gain on the affected side. This caloric–vHIT dissociation is one of the most specific findings in clinical vestibular testing.
Cordero-Yanza et al. compared caloric and vHIT findings in 84 patients with unilateral Meniere disease[Cordero-Yanza JA 2019]. Around 70% of patients with abnormal calorics had normal or only minimally reduced vHIT gains. The dissociation grew more prominent with disease duration. The proposed explanation is frequency-selective vulnerability: endolymphatic hydrops impairs the low-frequency response (which the cupula needs prolonged fluid displacement to transduce) while sparing the high-acceleration, high-frequency machinery that vHIT probes.
A useful clinical heuristic: an asymmetric caloric response with a symmetric vHIT, in a patient with fluctuating low-frequency hearing loss and episodic vertigo, is Meniere disease until proven otherwise. The dissociation is so characteristic that the absence of it should make you reconsider the diagnosis.
Audiogram
The classic audiogram in Meniere disease shows a low-frequency or peaked sensorineural hearing loss on the affected side, often improving slightly between attacks. With time, the loss becomes flatter and more permanent.
Key teaching points
- Diagnosis is clinical (Bárány/AAO criteria 2015): episodic vertigo, fluctuating low-frequency hearing loss, aural symptoms.
- Caloric–vHIT dissociation (reduced calorics, preserved vHIT) is a highly specific marker on the affected side.
- vHIT looks normal in most early-to-mid Meniere disease; abnormal vHIT does not exclude it but argues for advanced disease or a different cause.
- A unilateral low-frequency hearing loss with normal vHIT in a recurrent vertigo patient is Meniere disease until proven otherwise.
References
- Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandalà M, Newman-Toker DE, Strupp M, Suzuki M, Trabalzini F, Bisdorff A. Diagnostic criteria for Meniere disease. Journal of Vestibular Research 2015;25:1–7. doi:10.3233/VES-150549
- Cordero-Yanza JA, Arrieta Vázquez EV, Hernaiz Leonardo JC, Mancera Sánchez J, Hernández Palestina MS, Pérez-Fernández N. Comparative study between the caloric vestibular and the video head impulse tests in unilateral Meniere disease. Journal of Neurology 2019;266 (Suppl 1):S96–101. doi:10.1007/s00415-018-9112-4
- Halmagyi GM, Chen L, MacDougall HG, Weber KP, McGarvie LA, Curthoys IS. The video head impulse test. Frontiers in Neurology 2017;8:258. doi:10.3389/fneur.2017.00258
- MacDougall HG, Weber KP, McGarvie LA, Halmagyi GM, Curthoys IS. The video head impulse test: diagnostic accuracy in peripheral vestibulopathy. Neurology 2009;73:1134–41. doi:10.1212/WNL.0b013e3181bacf85