Vibration-Induced Nystagmus in Patients with Ménière’s Disease: Is There a Correlation to Endolymphatic Hydrops?

Skull vibration induced nystagmus in patients with superior semicircular canal dehiscence

PurposeThis study aimed to investigate correlation between the presence of endolymphatic hydrops(EH) and factors such as causes of hearing loss, patient age, duration of deafness, and results of vestibular function tests.MethodsWe retrospectively reviewed medical charts of 128 ears of cochlear implantees who were not considered relevant to Meniere’s disease.ResultsWhen comparing group with genetic variants of GJB2, SLC26A4, LMX1A and other genetic mutation group, the proportion of vestibular EH and cochlear EH found in group with genetic variants of GJB2, SLC26A4, LMX1A was significantly higher than group with other genetic etiology (p < 0.01) or the group with all the other causes of hearing loss (p < 0.01). The rate of vestibular and cochlear EH detection was higher in younger patients (41.5% and 35.4%) than in older patients (25.4% and 20.6%). A higher ratio of vestibular and cochlear EH was observed in patients with a longer duration of deafness (37.5% and 31.3%) than those with a shorter duration of deafness (29.7% and 25.0%). The group with vestibular EH showed a higher incidence of abnormal findings in the caloric test (42.9%) than the group without vestibular EH (28.2%).ConclusionPatients with genetic variants of GJB2, SLC26A4, LMX1A, younger patients, those with longer deaf durations showed a higher prevalence of vestibular and cochlear EH, implying EH appears to be formed as a developmental disorder in association with a certain set of genetic variants, rather than a phenotypic marker as a result of severe to profound hearing loss.

To help clinicians to further understand the significance of vestibular-evoked myogenic potential (VEMP) examinations to diagnose MD and the quantitative relationship between VEMP and MRI in assessing the location and degree of endolymphatic hydrops (EH) in definite Meniere's disease (MD) patients. Fifty-six patients with unilateral definite MD participated in this study, which used MRIs through intravenous gadolinium administration (IV-Gd), audiometry, caloric tests and VEMP tests. The VEMP results of 26 healthy volunteers were used as a normal reference value. The participants were found through MRI to have differing degrees of vestibular and cochlear EH. Quantitative comparison of MRI and VEMP results found that the response rates of oVEMP decreased with cochlear EH increasing; the asymmetry ratio (AR) of oVEMP can be used to find whether cochlear EH or not, and the P1-N1 amplitude was lower in the extreme cochlear EH group (P < 0.01). The AR of cVEMP was larger in severe vestibular EH group than that of the mild or no vestibular EH group (P < 0.01). The correlation between the degree of cochlear EH and the mean PTA threshold was statistically significant (P < 0.05). The duration of MD correlated positively with vestibular EH (P < 0.05). The abnormal rate of caloric tests was higher in severe vestibular EH group than that of the mild or no vestibular EH group (P < 0.05). The advantages of MRIs by IV-Gd administration were obvious in assessing the location and degree of EH. oVEMP and PTA can be indirectly used to evaluate the extent of cochlear EH, cVEMP and caloric tests can be used to assess the extent of vestibular EH on the condition of absent MRIs.

Background: To establish in patients with peripheral vestibular disorders relations between skull vibration-induced nystagmus (SVIN) different components (horizontal, vertical, torsional) and the results of different structurally related vestibular tests. Methods: SVIN test, canal vestibular test (CVT: caloric test + video head impulse test: VHIT), otolithic vestibular test (OVT: ocular vestibular evoked myogenic potential oVEMP + cervical vestibular evoked myogenic potential cVEMP) performed on the same day in 52 patients with peripheral vestibular diseases (age < 65 years), and 11 control patients were analyzed. Mixed effects logistic regression analysis was performed to assert whether the presence of nystagmus in SVIN (3D analysis) have an association with the presence of peripheral vestibular dysfunction measured by vestibular explorations (CVT or OVT). Results: We obtained different groups: Group-Co (control group), Group-VNT (dizzy patients with no vestibular tests alterations), Group-O (OVT alterations only), Group-C (CVT alterations only), Group-M (mixed alterations). SVIN-SPV horizontal component was significantly higher in Group-M than in the other groups (p = 0.005) and correlated with alterations of lateral-VHIT (p < 0.001), caloric test (p = 0.002) and oVEMP (p = 0.006). SVIN-SPV vertical component was correlated with the anterior-VHIT and oVEMP alterations (p = 0.007; p = 0.017, respectively). SVIN-SPV torsional component was correlated with the anterior-VHIT positivity (p = 0.017). SVIN was the only positive test for 10% of patients (83% of Group-VNT). Conclusion: SVIN-SPV analysis in dizzy patients shows significant correlation to both CVT and OVT. SVIN horizontal component is mainly relevant to both vestibular tests exploring lateral canal and utricle responses. SVIN-SPV is significantly higher in patients with combined canal and otolith lesions. In some patients with dizziness, SVIN may be the only positive test.

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms' competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice.

Introduction and Objectives: Skull vibration-induced nystagmus (SVIN) has become a validated tool for evaluating the vestibular function. The presence of SVIN is a useful indicator of the asymmetry of vestibular function between the two ears. In unilateral vestibular loss, a 100 Hz bone-conducted vibration given to either mastoid immediately causes a primarily horizontal nystagmus. The aim of this study is to review the usefulness of this tool in different clinical situations according to the results published. Methods: We performed an electronic search using PubMed and BVS. Eleven studies were discussed. Results: A progressive linear relationship has been identified between the slow-phase velocity (SPV) of SVIN determined using a 100 Hz skull vibrator and the gain difference (healthy ear/affected ear) measured by video head impulse test (vHIT). The SPV of SVIN may be more sensitive than vHIT in identifying the recovery of vestibular function following intratympanic gentamicin (ITG) administration. A link between a reduction in SPV and the likelihood of vertigo episodes in patients with MD who have been treated with intraympanic gentamicin (ITG) has been illustrated. SVIN in superior canal dehiscence (SCD) patients has greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. SVIN can be combined with vHIT to reveal vestibular asymmetry in nonprogressive vestibular schwannomas. An upbeating SVIN may reveal superior branch vestibular neuritis. Vibration-induced downbeat nystagmus should be added to the list of central vestibular signs and is likely due to cerebellar dysfunction. Conclusions: SVIN has become an interesting screening tool for diagnosing or during the follow-up of many different vestibular pathologies.

Background: Currently, 3 Tesla-MRI following intratympanic gadolinium injection has made it possible to assess the existence and the severity of hydrops in each compartment of the endolymphatic spaces in vivo. However, the relationship between vestibular endolymphatic hydrops (EH) visualized by MRI and vestibular functional tests, especially the correlation between caloric test, video-head impulse test, and semicircular canal hydrops, has not been well-investigated.Objective: The purpose of this study is to investigate the relationship between the severity of EH in each compartment of otoliths and semicircular canal and the results of vestibular functional tests.Methods: In this retrospective study, we performed three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequences following intratympanic gadolinium injection in 69 unilateral patients with definite Menière's disease. Vestibular and lateral semicircular canal hydrops was graded on MRI using a four grade criterion. All patients underwent pure-tone audiometry, cervical vestibular evoked myogenic potential (cVEMP), ocular vestibular evoked myogenic potential (oVEMP), caloric test and video head impulse test (vHIT). The latency, amplitude and asymmetry ratio of VEMP, canal paresis (CP) and vestibulo-ocular reflex (VOR) gain of lateral semicircular canal of vHIT were collected. The correlation analysis were performed between the parameters of function test and EH.Results: Vestibular EH showed correlations with the duration of disease (r = 0.360) and pure tone average (r = 0.326). AR of cVEMP showed correlations with Vestibular EH (r = 0.407). CP (r = 0.367) and VOR gain of lateral semicircular canal at 60 ms (r = 0.311) showed correlations with lateral semicircular canal hydrops.Conclusion: EH in different compartments is readily visualized by using 3D-FLAIR MRI techniques. The degree of vestibular EH correlated with AR of cVEMP and EH in the semicircular canal ampullar affects the caloric and vHIT response in patients with unilateral Meniere‘s disease.

The purpose of this study was to assess the diagnostic performance of 3T MRI after intratympanic injection of gadodiamide for delayed endolymphatic hydrops (DEH), and assess the relationship between endolymphatic hydrops (ELH) and vestibular function in patients diagnosed with DEH and confirmed by 3T MRI. Nineteen patients clinically diagnosed with DEH (11 ipsilateral DEH, 8 contralateral DEH) participated in this study. Diluted gadodiamide was administered to the bilateral tympanic cavity by injection through the tympanic membrane. At 24 hours post-injection, the ELH was evaluated by MRI. Patient vestibular functions were evaluated by caloric testing and cVEMP. ELH was observed in all patients (19/19: positive rate 100%). The distribution patterns of ELH varied between the cochlear or vestibular region. Vestibular ELH was observed in the affected ear in all ipsilateral DEH patients. In the contralateral DEH patients, however, there were individual differences in the distribution patterns of ELH. Six patients (1 ipsilateral DEH, 5 contralateral DEH) had bilateral ELH. No obvious relationships were observed between ELH and vestibular function. ELH distribution was complicated, particularly in the contralateral DEH cases. It was difficult to identify the existence of ELH by vestibular functional testing alone; therefore, 3T MRI is thought to be useful for identifying the affected ear. A significant number of cases had “bilateral” DEH, particularly among the contralateral DEH cases, indicating that we should pay careful attention to this pathology when treating DEH.

Background As described by Dumas, an 80–100 Hz vibration applied to the mastoid produces a horizontal nystagmus, with quick phases beating away from the affected side in patients with unilateral vestibular loss (UVL). Aim/objectives To elucidate the characteristics of skull vibration-induced nystagmus (SVIN) in patients suffering from vestibular neuritis and how these characteristics are related to information provided by the video head impulse test (vHIT). Material and methods Sixty-three patients were enrolled to perform a vHIT to measure the gain in both ears. SVIN was induced with stimulation at 30 Hz, 60 Hz, and 100 Hz. The slow phase velocity (SPV) of the SVIN was measured. Results The SVIN test was positive in 25/63 patients at 30 Hz, 36/63 at 60 Hz and 46/63 at 100 Hz. Mean gain difference between both ears to obtain a positive SVIN at 30 Hz was observed to be 0.38 ± 0.25, decreasing to 0.35 ± 0.23 at 60 Hz, and 0.31 ± 0.24 at 100 Hz (p = .025). We found a significant positive linear correlation between the gain asymmetry measured using horizontal vHIT and SPV in SVIN at 100 Hz. Conclusions and significance There is a close relationship between the difference in the gains of both ears as measured using VHIT and the SPV of the nystagmus induced by SVIN at 100 Hz.

A 100-Hz bone-conducted vibration applied to either mastoid induces instantaneously a predominantly horizontal nystagmus, with quick phases beating away from the affected side in patients with a unilateral vestibular loss (UVL). The same stimulus in healthy asymptomatic subjects has little or no effect. This is skull vibration-induced nystagmus (SVIN), and it is a useful, simple, non-invasive, robust indicator of asymmetry of vestibular function and the side of the vestibular loss. The nystagmus is precisely stimulus-locked: it starts with stimulation onset and stops at stimulation offset, with no post-stimulation reversal. It is sustained during long stimulus durations; it is reproducible; it beats in the same direction irrespective of which mastoid is stimulated; it shows little or no habituation; and it is permanent—even well-compensated UVL patients show SVIN. A SVIN is observed under Frenzel goggles or videonystagmoscopy and recorded under videonystagmography in absence of visual-fixation and strong sedative drugs. Stimulus frequency, location, and intensity modify the results, and a large variability in skull morphology between people can modify the stimulus. SVIN to 100 Hz mastoid stimulation is a robust response. We describe the optimum method of stimulation on the basis of the literature data and testing more than 18,500 patients. Recent neural evidence clarifies which vestibular receptors are stimulated, how they cause the nystagmus, and why the same vibration in patients with semicircular canal dehiscence (SCD) causes a nystagmus beating toward the affected ear. This review focuses not only on the optimal parameters of the stimulus and response of UVL and SCD patients but also shows how other vestibular dysfunctions affect SVIN. We conclude that the presence of SVIN is a useful indicator of the asymmetry of vestibular function between the two ears, but in order to identify which is the affected ear, other information and careful clinical judgment are needed.

Background: Although diagnostic criteria have been established for superior canal dehiscence syndrome, cases in which the diagnosis is not easy are frequent. On those occasions, some tests such as vibration-induced nystagmus or vestibular-evoked myogenic potentials can offer invaluable help due to their high sensitivity and specificity. Methods: We studied 30 patients showing superior canal dehiscence or “near-dehiscence” in a CT scan. Skull vibration-induced nystagmus and high frequency ocular vestibular-evoked myogenic potentials are performed in each patient. The aim of the study is to determine how useful both tests are for detection of superior canal dehiscence or near-dehiscence. Results: Of the 60 temporal bones studied, no dehiscence was the result in 22, near-dehiscence in 17 and a definite finding in 21. In 10/30 patients, there was no SVIN (Skull vibration induced nystagmus) during otoneurological testing, while in 6/30, induced nystagmus was mainly horizontal, and in 14/30 there was vertical up-beating. All patients had a positive oVEMP (Ocular vestibular evoked myiogenic potentials) at 0.5 kHz in both ears and the HFoVEMP (High frequency ocular vestibular evoked myiogenic potentials) response was positive in 25/60 (41.6%) of the ears studied and in 19/30 of the patients evaluated (in 6 it was positive in both ears). Up-beat SVIN will point to a SCD (Superior Canal Dehiscence) mainly when HFoVEMP are present, and when this is negative there is a high probability that it is not a SCD. Conclusions: When SVIN and HFoVEMP results are added (or combined), they not only improve the possibilities of detecting SCD, but also the affected side.

Vertical semicircular canals and endolymphatic hydrops play important roles in the pathophysiological mechanisms of Ménière disease. However, their characteristics and associations with disease progression during medical treatment have not been determined. To examine the function of both the horizontal and vertical semicircular canals in patients with Ménière disease and to evaluate the change in endolymphatic hydrops volume during medical treatment, including treatment with diuretic therapy, over a 2-year period. This prospective longitudinal observational cohort study included 55 patients with definite unilateral Ménière disease and was performed in a tertiary care hospital in Japan. Participants were enrolled between April 1, 2017, and January 31, 2018, and those with vestibular migraine were excluded. All participants received education regarding diet and lifestyle modifications and treatment with betahistine mesylate (36 mg daily) and/or an osmotic diuretic (42-63 mg daily). Patients were followed up for vertigo and hearing evaluations at least once per month for more than 12 months and were instructed to record episodes of vertigo in a self-check diary. Audiometry was performed monthly, video head impulse testing and caloric testing were performed every 4 months, and magnetic resonance imaging was conducted annually. Data were analyzed from May 15, 2017, to January 31, 2020. Neurootological testing to evaluate vestibuloocular reflex gain over time, magnetic resonance imaging to evaluate the change in endolymphatic hydrops volume over time, and monthly vertigo and hearing evaluations for more than 12 months. Among 55 participants with definite Ménière disease, 32 patients (58.2%) were female, and the mean (SD) age was 59.0 (15.1) years. The median disease duration was 2 years (interquartile range, 0-4 years), with 43 patients (78.2%) having an early stage (ie, disease duration ≤4 years) of Ménière disease. Over the 2-year study period, the vestibuloocular reflex gain decreased from 0.76 to 0.56 in the superior semicircular canals, for a difference of 0.20 (95% CI, 0.14-0.26) and from 0.68 to 0.50 in the posterior semicircular canals, for a difference of 0.18 (95% CI, 0.14-0.22). The maximum slow-phase velocity and vestibuloocular reflex gain in the horizontal semicircular canals were maintained. The volume ratio of vestibular endolymphatic hydrops increased from 19.7% to 23.3%, for a difference of 3.6% (95% CI, 1.4%-5.8%). The frequency of vertiginous episodes decreased, and the hearing level over the study period worsened from 40.9 dB to 44.5 dB, for a difference of 3.5 dB (95% CI, 0.7-6.4 dB). In this study, during a 2-year period of medical treatment among patients with Ménière disease, vestibuloocular reflex gain decreased in the vertical semicircular canals but was maintained in the horizontal semicircular canals; the endolymphatic hydrops volume ratio increased, and the frequency of vertiginous episodes decreased. These findings describe the pathological progression of chronic Ménière disease and expand the understanding of its pathophysiological characteristics during the early stage of disease.

This study was performed to reevaluate the diagnostic significance of clinically well-accepted audiological tests in indicating endolymphatic hydrops (EH) in Menière's disease (MD). Retrospective case review. Hospital. Fifty patients (52 affected ears) diagnosed with MD were enrolled. Diagnostic. To analyze the correlations between endolymphatic hydrops and results of audiological test including the pure-tone audiometry threshold, suprathreshold function tests, electrocochleogram, and glycerol test. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging demonstrated EH in either the vestibule or cochlea to various degrees in all of the MD patients, 24 hours after intratympanic gadolinium chelate injection. Both vestibular and cochlear EH were significantly correlated with PTA threshold. However, EH was not associated with alternate binaural loudness balance or the tone decay test, although a correlation was observed with the short-increment sensitivity index. There was also a correlation between vestibular EH, but not cochlear EH, and the negative summating potential/action potential (-SP/AP) ratio. Neither vestibular EH nor cochlear EH was correlated with the glycerol test results. In addition, the frequency of vertigo attacks, the existence of tinnitus, and aural fullness did not correlate with EH. Disrupted ionic homeostasis in the inner ear, but not the EH, may contribute to changes in the -SP/AP ratio. The relevance of glycerol test in identifying EH through detection of hearing changes needs further investigation in the future.

Background/Aim: the aim of this study was to assess the skull vibration-induced nystagmus test (SVINT) results and vestibular residual function after horizontal semicircular canal (HSCC) plugging. Methods: In this retrospective chart review performed in a tertiary referral center, 11 patients who underwent unilateral horizontal semicircular canal plugging (uHSCCP) for disabling Menière’s disease (MD) were included. The skull vibration-induced nystagmus (SVIN) slow-phase velocity (SPV) was compared with the results of the caloric test (CaT), video head impulse test (VHIT), and cervical vestibular-evoked myogenic potentials (cVEMP) performed on the same day. Results: Overall, 10 patients had a strong SVIN beating toward the intact side (Horizontal SVIN-SPV: 8.8°/s ± 5.6°/s), 10 had a significant or severe ipsilateral CaT hypofunction, 10 had an ipsilateral horizontal VHIT gain impairment, and 3 had altered cVEMP on the operated side. Five had sensorineural hearing worsening. SVIN-positive results were correlated with CaT and horizontal VHIT (HVHIT) results (p < 0.05) but not with cVEMP. SVIN-SPV was correlated with CaT hypofunction in % (p < 0.05). Comparison of pre- and postoperative CaT % hypofunction showed a significant worsening (p = 0.028). Conclusion: SVINT results in a human model of horizontal canal plugging are well correlated with vestibular tests exploring horizontal canal function, but not with cVEMP. SVINT always showed a strong lesional nystagmus beating away from the lesion side. SVIN acts as a good marker of HSCC function. This surgical technique showed invasiveness regarding horizontal canal vestibular function.

BackgroundThe relationships of endolymphatic hydrops (EH) and perilymphatic enhancement (PE) with Ménière’s disease (MD) remains unclear. This study aimed to describe the dynamic variation of EH and PE for MD patients over 2 hospitalizations by applying magnetic resonance imaging (MRI) to further clarify the relationships of EH and PE with MD.MethodsA total of 77 MD patients who underwent inner ear MRI after intravenous administration of gadolinium and pure-tone average (PTA) testing during a first and second hospitalization were included. The degree of EH and PE were evaluated via MRI, and the duration and frequency of vertigo attacks and PTA were collected and recorded. The PTA, EH, and PE for the 2 hospitalizations were compared, and the relationships of EH and cochlear PE with the MD stage were investigated.ResultsThere was no difference between the 2 hospitalizations for duration of vertigo attacks or frequency of vertigo attacks. However, there were significant differences in PTA (Z=−3.02, P=0.003). Additionally, the cochlear and vestibular EH in the asymptomatic ear at the second hospitalization was significantly worse than that of the first hospitalization (Z=−2.33 and −2.49, P=0.020 and 0.013, respectively), while there were no differences in EH and PE in the affected ear (all P>0.05). Moreover, the degree of cochlear and vestibular EH was correlated with MD stage (both P<0.01).ConclusionsAlthough EH and PE in the affected ear were unchanged over 2 hospitalizations, an underlying EH in the asymptomatic ear and hearing loss in the affected ear for MD patients developed longitudinally with the duration of disease, and EH varied with the natural course of MD whereas PE did not. Therefore, EH instead of PE is necessary but insufficient to cause the clinical symptoms of MD.