CASE REPORT
Nagoya J. Med. Sci. 85. 375–379, 2023
doi:10.18999/nagjms.85.2.375

Significance of an augmented response on cervical vestibular
evoked myogenic potential testing in Meniere’s disease
Masumi Kobayashi1, Naomi Katayama1,2, Tadao Yoshida1,Satofumi Sugimoto1, Shinji Naganawa3 and Michihiko Sone1
21
Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, Nagoya, Japan
Department of Health and Nutrition, Faculty of Health and Sciences, Nagoya Women’s University, Nagoya,Japan
3
Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya, JapanABSTRACT
The potential mechanism of augmented response on cervical vestibular evoked myogenic potential(cVEMP) testing and its decrease following treatment in a patient with Meniere’s disease (MD) arediscussed. Changes of static posturography and cVEMP testing before and after a glycerol drip in a
69-year-old man with unilateral MD, in which significant endolymphatic hydrops (EH) was confirmed onmagnetic resonance imaging (MRI) on the diseased side, were evaluated. Values of total locus lengths,
areas of postural sway, and their Romberg ratios were decreased after the glycerol drip. On cVEMP testing,the diseased ear demonstrated a 375% larger amplitude than the contralateral ear before treatment, butboth ears showed almost the same responses after treatment. An augmented response on cVEMP testing
and a decrease following treatment for MD reflect the diversity of clinical findings in MD. Responses oncVEMP testing may relate not only to the degree of EH, but also be due to abnormal acoustic energy
absorbance transmitted into the saccule.
Keywords: augmented cVEMP, Meniere’s disease, static posturography, glycerol drip
Abbreviations:cVEMP: cervical vestibular evoked myogenic potential
MD: Meniere’s disease
EH: endolymphatic hydrops
MRI: magnetic resonance imaging
This is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
License. To view the details of this license, please visit (http://creativecommons.org/licenses/by-nc-nd/4.0/).

INTRODUCTION
Since the first reported visualization of endolymphatic hydrops (EH) on magnetic resonanceimaging (MRI) in the living human body,1 correlations between audio-vestibular functional tests
and the degrees of EH on MRI have been demonstrated, including a significant association ofReceived: April 1, 2022; accepted: June 22, 2022
Corresponding Author: Masumi Kobayashi, MD, PhD
Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho,Showa-ku, Nagoya 466-8550, Japan
Tel: +81-52-744-2323, Fax: +81-52-744-2325, E-mail: masumi@med.nagoya-u.ac.jp375

Masumi Kobayashi et al

EH and the disappearance of cervical vestibular evoked myogenic potentials (cVEMPs)2 or ahigher degree of asymmetry of cVEMP amplitude in Meniere’s disease (MD). 3 In ears withMD, cVEMP responses are generally weak, but Young et al reported an augmented cVEMP
response.4 They speculated that augmented cVEMP responses were an indication of distended
saccular hydrops, where the distended wall was in contact with the footplate. Since then, a similarfinding has not been reported, and the evidence of its existence and the exact mechanisms havenot been elucidated.
A case with unilateral MD, which demonstrated an augmented response on cVEMP testingon the diseased side with significant EH confirmed on MRI, is presented. After treatment for
MD, the patient showed improvement on static posturography and a decreased cVEMP response.
The mechanism of the augmented response on cVEMP in the ear with EH is considered, andits significance for evaluating vestibular function in MD is discussed.

CASE PRESENTATION
A 69-year-old man with MD, who had repeated vertigo and sensorineural hearing loss (SNHL)of the left ear for 9 years, was referred to our hospital due to resistance to conventional therapies.
Audiogram, static posturography and cVEMP examinations were performed before and after a
200-ml glycerol drip, and changes in their values were evaluated.
Magnetic resonance imaging examination
MRI examination for EH was performed 4 h after intravenous injection of a standard dose
(0.1 ml/kg body weight) of gadolinium hydrate. All scans were performed using a 3-T MRI
scanner (Magnetom Verio; Siemens, Erlangen, Germany) equipped with a receive-only, 32-channel, phased-array coil. The details of the parameters of hybrid of reversed image of positive
endolymph signal and native image of positive perilymph signal (HYDROPS) sequences detecting
EH have been described elsewhere.5 Radiologists who did not know the patient’s clinical course
classified the degree of EH using three grades.6 When the ratio of the area of endolymphatic
space to the vestibular space (sum of the endolymphatic and perilymphatic spaces) exceeded 1/2
on MRI and the area of the cochlear duct exceeded the area of the scala vestibuli, the case wasclassified as significant EH.
Static posturography examination
The static posturography evaluation was performed using computer-driven coordination anda balance analysis device. After a sufficient period of adjustment to standing, the subject wasinstructed to maintain a stable posture for one minute with the eyes opened, followed by anotherminute with the eyes closed. Total locus length (mm), the area of postural sway (mm2) of thecenter of gravity movement, and the Romberg ratio of the values when the eyes were closed tothose when the eyes were opened were calculated (UM-BAR, Unimec Corporation, Tokyo, Japan).
Cervical vestibular-evoked myogenic potential
Surface myogenic potentials in the sternocleidomastoid muscles were added 200 times witha reference electrode over the sternum, while click stimuli of 105 dB sound pressure level indecibels (dB SPL) were presented to the ipsilateral ear being tested, and white noise (75 dBSBL) was presented to the contralateral ear. The stimulation rate of the clicks was 5 Hz, andthe electromyogenic signal was amplified through a bandpass filter (20–2000 Hz) (Synax2100,NEC Medical Systems, Tokyo, Japan). Amplitudes between the first positive (p13) and the first
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Augmentation cVEMP in Meniere’s disease

negative peak (n23) of the responses were calculated as parameters of saccular function.

RESULTS
The patient had SNHL of 45 dB on the left side, which was not improved after the drip, in
which MRI showed significant EH both in the cochlea and vestibule, nonadjacent to the stapes
footplate (Figure 1). On static posturography, the Romberg ratios of total locus length or thearea of postural sway were 2.7 or 2.0, respectively, which decreased to 2.0 or 1.3 after the drip
(Figure 2). On cVEMP, the amplitude in the left ear with EH was 103.8 μV, which was 375%
larger than that in the right ear (27.8 μV). After the drip, the amplitudes in the left and rightears were 35.2 μV and 36.4 μV, respectively, almost the same (Figure 3).

Fig. 1 Magnetic resonance imaging (MRI) performed 4 h after intravenous injection of gadolinium hydrate
A 69-year-old man with left Meniere’s disease shows significant endolymphatic hydrops on the left side on MRI(white arrowheads).

Fig. 2 Posturographic examination before and after the glycerol drip
The Romberg ratios are lower for total locus length and the area of postural sway after the drip.
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Masumi Kobayashi et al

Fig. 3 Cervical vestibular-evoked myogenic potential testing before and after the glycerol drip
On cervical vestibular-evoked myogenic potential testing, an augmented response on the affected side appears
before the glycerol drip, and a decrease in amplitude is seen after the drip.

DISCUSSION
Saccular dysfunction is more common than utricular dysfunction in MD, and cVEMP testing is suitable for evaluation of vestibular function in ears with MD. Interaural asymmetry of
amplitudes on cVEMP is a frequently observed finding in cases with unilateral MD. Amplitudes
in diseased ears are usually small or absent, but augmentation of the cVEMP response has beenreported by Young et al.4 They suggested that an augmented response would appear in ears that
had a distended saccule, contacting the stapes footplate due to EH, and result in enhancement ofsensitivity to acoustic stimulation in the saccular macula. Their conclusion was that an augmentedcVEMP response would correlate with the severity of EH in the saccule.
Advanced MRI technology has enabled effective visualization of significant EH in the vestibule
adjacent to the stapes footplate.7 The present case showed an augmented cVEMP response in theear with vestibular EH nonadjacent to the stapes footplate, but its amplitude was much larger
than the amplitudes reported by Young et al.4 We have evaluated responses on cVEMP in manycases with significant EH adjacent to the stapes footplate, but we have not encountered suchan augmented response.2 Since the paper by Young et al, no similar cVEMP finding has beenreported.
Occasionally, significant EH adjacent to the stapes footplate could induce air-bone gaps(ABGs) at low frequencies, which have been demonstrated to be evidence supported by findingson MRI.7 A previously proposed mechanism included that increased perilymphatic pressure orsaccular dilatation caused by EH might decrease the mobility of the stapes8,9; however, a recent
study showed that significantly higher absorbance of acoustic energy was observed in ears withsignificant vestibular EH at low frequencies.10 Though absorbed acoustic energy is high, the presence of EH in the vestibule might cause an obstacle to the transmission of energy to the innerear. The increased amplitude observed in the present case may reflect more strongly transmitted
acoustic energy to the saccule than usual, even in a case with vestibular EH nonadjacent to thestapes footplate. The characteristic augmented response on cVEMP could be observed in ears
with saccular EH nonadjacent to the stapes footplate, too. Responses on cVEMP testing may
relate not only to the degree of EH, but also to the transmission of acoustic energy absorbed
into the saccule.
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The improvement on static posturography indicated the effect of the treatment for MD. The
decrease of cVEMP amplitude may also demonstrate functional improvement of the saccule
according to the previous report.11 A phenomenon of augmented cVEMP responses is rarely
observed in cases with MD, but such a finding might show the diversity of clinical findings inMD.

CONCLUSION
MD patient who showed an augmented cVEMP amplitude and its reduction after drip treatment, demonstrating functional improvement in the vestibule, was reported. Responses on cVEMP
testing may relate not only to the degree of EH, but also be due to abnormal acoustic energy
absorption transmitted into the saccule.

DISCLOSURE STATEMENTThe authors have no conflict of interest.

ETHICAL CONSIDERATIONS
Written informed consent was obtained from the patient for the publication.

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