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Clinical, oculographic and vestibular test characteristics of Ménière’s disease

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Abstract

Seventy Ménière’s disease (MD) patients with spontaneous vertigo (100%), unilateral aural fullness (57.1%), tinnitus (78.6%), and subjective hearing loss (75.7%) self-recorded nystagmus during their episodes of vertigo using portable video oculography goggles. All demonstrated ictal spontaneous nystagmus, horizontal in 94.3% (n = 66) and vertical in 5.7% (n = 4), with a mean slow-phase velocity (SPV) of 42.8 ± 31.1°/s (range 5.3–160.1). Direction reversal of spontaneous horizontal nystagmus was captured in 58.6%, within the same episode in 34.3%, and over different days in 24.3%. In 18.6%, we observed ipsiversive then contraversive nystagmus, and in 12.9% contraversive to ipsiversive direction reversal. Ictal nystagmus SPV (42.8 ± 31.1°/s) was significantly faster than interictal (1.4 ± 3.1°/s, p < 0.001, CI 34.277–48.776). Compared to age-matched healthy controls, interictal video head impulse test gains in MD ears were significantly lower, cumulative and first saccade (S1) amplitudes were significantly larger, and S1 peak velocities were significantly faster (p = 0.038/0.019/0.008/ < 0.001, CI 0.002–0.071/0.130–1.444/0.138–0.909/14.614–41.506). Audiometry showed asymmetrically increased thresholds in 100% of MD ears (n = 70). Significant caloric, air-conducted (AC) cervical vestibular-evoked myogenic potential (VEMP), and AC ocular VEMP asymmetries were found in 61.4, 37.9, and 44.4% of patients (MD ear reduced). Transtympanic electrocochleography tested in 36 ears (23 patients) showed 81.8% of MD ears had a positive result for hydrops (either a summating potential at 1/2 kHz < − 6 µV, or an SP/AP ratio > 40%). Using ictal nystagmus findings of SPV > 12°/s, and a caloric canal paresis > 25%, we correctly separated a diagnosis MD from Vestibular Migraine with a sensitivity and specificity of 95.7% and 85.1% (CI 0.89–0.97).

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Anonymised data may be shared with investigators, upon request, in the context of research collaboration.

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Custom nystagmus analysis software may be shared with interested parties.

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Acknowledgements

This research was funded by the Garnett Passe and Rodney Williams Memorial Foundation, and the National Health and Medical Research Council of Australia. Mario D’Souza (Senior Statistician; Central Clinical School, Faculty of Medicine and Health, University of Sydney, NSW, Australia) provided advice and assistance with statistical analysis.

Funding

This research was funded by the Garnett Passe and Rodney Williams Memorial Foundation (Grant Number RP554), and the National Health and Medical Research Council of Australia (Grant Number APP1126976). These funding sources have no role in any aspect of the study, or the decision to submit for publication.

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Authors and Affiliations

  1. Central Clinical School, Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia

    Allison S. Young, Benjamin Nham, Jacob M. Pogson & Miriam S. Welgampola

  2. Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Camperdown, NSW, Australia

    Allison S. Young, Benjamin Nham, Andrew P. Bradshaw, G. Michael Halmagyi & Miriam S. Welgampola

  3. Department of Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia

    Zeljka Calic

  4. Sydney Cochlear Implant Centre, Sydney, NSW, Australia

    William P. Gibson

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  1. Allison S. Young
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  2. Benjamin Nham
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Contributions

All authors contributed to the study conception and design, read, critically edited, and approved the final manuscript. Original draft of manuscript, and all original figures by AY. Data collection and analysis performed by AY, BN, ZC, and JP. Statistical analysis conducted by AY and AB. Video editing and nystagmus analysis software designed by AB. WPG and GMH consulted with and recruited research patients. Study design, and research patient consultation conducted by MW.

Corresponding author

Correspondence to Miriam S. Welgampola.

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Conflicts of interest

A Young receives scholarship funding from the University of Sydney, and funding from the Garnett Passe and Rodney Williams Memorial Foundation, and reports no conflict of interest. J Pogson receives funding from the Garnett Passe and Rodney Williams Memorial Foundation and reports no conflict of interest. M Welgampola receives funding from the Garnett Passe and Rodney Williams Memorial Foundation, and the National Health and Medical Research Council of Australia, and reports no conflict of interest. B Nham, A Bradshaw, Z Calic, W Gibson, and G Michael Halmagyi report no conflicts of interest.

Ethical approval

This study received local ethics committee approval (Protocol No X18-0087). Written informed consent was obtained from all participants in accordance with the 1964 Declaration of Helsinki and its later amendments.

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Young, A.S., Nham, B., Bradshaw, A.P. et al. Clinical, oculographic and vestibular test characteristics of Ménière’s disease. J Neurol 269, 1927–1944 (2022). https://doi.org/10.1007/s00415-021-10699-z

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