Effects of nonprescription therapies on vestibular migraine: a questionnaire-based observational study.

BACKGROUND: Vestibular migraine (VM) is a common cause of recurrent vertigo. Migraine headache preventative therapies are currently prescribed to control vertigo symptoms in VM. Clinical trials of nutraceuticals for migraine headache prevention have shown positive outcomes, but, to date, there have been no studies to assess their effectiveness in the management of VM. AIMS: To report the effects of nonprescription therapy management on VM symptoms. METHODS: We undertook a prospective, questionnaire-based assessment of patients diagnosed with VM between November 2019 and August 2021 at two Sydney tertiary referral clinics. Patients were advised on optimising sleep, hydration, exercise and nutrition and instructed to use an over-the-counter combination product containing riboflavin 200 mg, magnesium 150 mg, coenzyme Q10 75 mg and feverfew 200 mcg. Symptom severity and frequency were assessed using the Dizziness Handicap Inventory (DHI), the Vertigo Symptom Score short-form (VSS-sf) and two visual analogue scales for severity (VAS-s) and frequency (VAS-f) before and 3 months after commencing treatment. RESULT: In 82 participants (78% female; mean age, 44 ± 14 years) we recorded a decrease in DHI (mean, 16.8 [95% confidence interval (CI), 12.8-20.9], VSS-sf (9.3, 7.1-11.5), VAS-s (3.0, 2.2-3.8) and VAS-f (2.8, 2.1-3.4), equating to an improvement of 44.1%, 44.9%, 44.1% and 38.9% for each measure respectively. On the DHI and VSS-sf, 41 (50%) and 44 (53.7%) patients showed improvement in their symptoms; 39 (47.6%) and 36 (43.9%) patients noted no change and two patients reported worsening. The supplement was well-tolerated. CONCLUSIONS: The results provide preliminary evidence that VM symptom frequency and severity can be reduced by using nonprescription therapies.

The role of cochlear and vestibular afferents in long-latency cervical vestibular evoked myogenic potentials.

OBJECTIVE: To examine the origin of cervical vestibular evoked myogenic potential (cVEMP) late waves (n34-p44) elicited with air-conducted click stimuli. DESIGN: Using a retrospective design, cVEMPs from normal volunteers were compared to those obtained from patients with vestibular and auditory pathologies. STUDY SAMPLE: (1) Normal volunteers (n = 56); (2) severe-to-profound sensorineural hearing loss (SNHL) with normal vestibular function (n = 21); (3) peripheral vestibular impairment with preserved hearing (n = 16); (4) total vestibulocochlear deficit (n = 23). RESULTS: All normal volunteers had ipsilateral-dominant early p13-n23 peaks. Late peaks were present bilaterally in 78%. The p13-n23 response was present in all patients with SNHL but normal vestibular function, and 43% had late waves. Statistical comparison of these patients to a subset of age-matched controls showed no significant difference in the frequencies, amplitudes or latencies of their ipsilateral early and late peaks. cVEMPs were absent in all patients with vestibular impairment. CONCLUSION: The presence of long-latency cVEMP waves was not dependent on the integrity of sensorineural hearing pathways, but instead correlated with intact vestibular function. This finding conflicts with the view that these late waves are cochlear in origin, and suggests that vestibular afferents may assume a more prominent role in their generation.

Vestibular function testing in the 21st century: video head impulse test, vestibular evoked myogenic potential, video nystagmography; which tests will provide answers?

PURPOSE OF REVIEW: To most neurologists, assessing the patient with vertigo is an unpleasant and worrisome task. A structured history and focused examination can be complemented by carefully selected laboratory tests, to reach an early and accurate diagnosis. We provide evidence-based recommendations for vestibular test selection. RECENT FINDINGS: The video head impulse test (vHIT), cervical and ocular vestibular evoked myogenic potential (VEMP) and home-video nystagmography are four modern, noninvasive methods of assessing vestibular function, which are equally applicable in the hospital and office-practice. Collectively, they enable assessment of all five vestibular end-organs. The prevalence and patterns of test abnormalities are distinct for each vestibular disorder. We summarize typical abnormalities encountered in four common vestibular syndromes. SUMMARY: In the context of acute vestibular syndrome, an abnormal vHIT with low gain and large amplitude refixation saccades and an asymmetric oVEMP separates innocuous vestibular neuritis from stroke. In episodic spontaneous vertigo, high-velocity ictal nystagmus and asymmetric cVEMP help separate Ménière's disease from vestibular migraine. In chronic imbalance, all three tests help detect unilateral or bilateral vestibular loss as the root cause. Recurrent positional vertigo requires no laboratory test and can be diagnosed and treated at the bedside, guided by video nystagmography.

Subjective Cognitive Dysfunction in Patients with Dizziness and Vertigo.

INTRODUCTION: Patients with vestibular disorders sometimes report cognitive difficulties, but there is no consensus about the type or degree of cognitive complaint. We therefore investigated subjective cognitive dysfunction in a well-defined sample of neuro-otology patients and used demographic factors and scores from a measure of depression, anxiety, and stress to control for potential confounding factors. METHODS: We asked 126 neuro-otology clinic outpatients whether they experienced difficulties with thinking, memory, or concentration as a result of dizziness or vertigo. They and 42 nonvertiginous control subjects also completed the Neuropsychological Vertigo Inventory (NVI, which measures cognitive, emotional, vision, and motor complaints), the Everyday Memory Questionnaire (EMQ), and Depression, Anxiety, and Stress Scales (DASS). RESULTS: In the initial interview questions, 60% of patients reported experiencing cognitive difficulties. Cognitive questionnaire scores were positively correlated with the overall DASS score and to a lesser extent with age and gender. Therefore, we compared patients and controls on the NVI and EMQ, using these mood and demographic variables as covariates. Linear regression analyses revealed that patients scored significantly worse on the total NVI, NVI cognitive composite, and 3 individual NVI cognition subscales (Attention, Space Perception, and Time Perception), but not the EMQ. Patients also scored significantly worse on the NVI Emotion and Motor subscales. CONCLUSIONS: Patients with dizziness and vertigo reported high levels of cognitive dysfunction, affecting attention, perceptions of space and time. Although perceptions of cognitive dysfunction were correlated with emotional distress, they were significantly elevated in patients over and above the impact of depression, anxiety, or stress.

Rejecting Gargoylism: Reflections on the term and its relationship to Hurler syndrome.

Just over a century ago, Dr. Gertrud Hurler described what is now commonly called Hurler syndrome. This rare inherited condition became known by terms describing its cardinal physical signs; the eponymous name was not proposed. In 1936, Ellis et al. coined the term "gargoylism" to name the syndrome. We propose that the film The Hunchback of Notre Dame played a key role in the original association of Hurler syndrome with the architectural figures known as gargoyles and with the persistence of the term. Over time, due in large part to its extremely negative connotations and lack of accuracy, "gargoylism" was abandoned. It has been supplanted by a sophisticated clinical understanding of mucopolysaccharidosis type 1 (MPS1), the underlying enzymatic defect, and dramatic advances in early diagnosis and treatment.

Vestibular-Evoked Myogenic Potential Testing in Vestibular Localization and Diagnosis.

Vestibular-evoked myogenic potentials (VEMPs) are short-latency, otolith-dependent reflexes recorded from the neck and eye muscles. They are widely used in neuro-otology clinics as tests of otolith function. Cervical VEMPs are recorded from the neck muscles and reflect predominantly saccular function, while ocular VEMPs are reflexes of the extraocular muscles and reflect utricular function. They have an important role in the diagnosis of superior canal dehiscence syndrome and provide complementary information about otolith function that is useful in the diagnosis of other vestibular disorders. Like other evoked potentials, they can provide important localizing information about lesions that may occur along the VEMP pathway. This review will describe the VEMP abnormalities seen in common disorders of the vestibular system and its pathways.

Bone-conducted vestibular and stretch reflexes in human neck muscles.

In normal humans, tapping the forehead produces a neck muscle reflex that is used clinically to test vestibular function, the cervical vestibular evoked myogenic potential (cVEMP). As stretch receptors can also be activated by skull taps, we investigated the origin of the early and late peaks of the bone-conducted cVEMP. In twelve normal participants, we differentially stimulated the vestibular and neck stretch receptors by applying vibration to the forehead (activating both vestibular and stretch receptors) and to the sternum (activating mainly stretch receptors). Patients with bilateral vestibulopathy (BVP; n = 26) and unilateral vestibular loss (uVL; n = 17) were also investigated for comparison. Comparison of peaks in normal subjects suggested that the early peaks were vestibular-dependent, while the later peaks had mixed vestibular and stretch input. The late peaks were present but small (1.1 amplitude ratio) in patients with BVP and absent VEMPs, confirming that they do not strictly depend on vestibular function, and largest in age-matched controls (1.5 amplitude ratio, p = 0.049), suggesting that there is an additional vestibular reflex at this latency (approx. 30 ms). Patients with uVL had larger late peaks on the affected than the normal side (1.4 vs 1.0 amplitude ratio, p = 0.034). The results suggest that the early responses in SCM to skull vibration in humans are vestibular-dependent, while there is a late stretch reflex bilaterally and a late vestibular reflex in the contralateral muscle.

Evidence of a Vestibular Origin for Crossed-Sternocleidomastoid Muscle Responses to Air-Conducted Sound.

OBJECTIVES: Small, excitatory surface potentials can sometimes be recorded from the contralateral sternocleidomastoid muscle (SCM) following monaural acoustic stimulation. Little is known about the physiological properties of these crossed reflexes. In this study, we sought the properties of crossed SCM responses and through comparison with other cochlear and vestibular myogenic potentials, their likely receptor origin. DESIGN: Surface potentials were recorded from the ipsilateral and contralateral SCM and postauricular (PAM) muscles of 11 healthy volunteers, 4 patients with superior canal dehiscence and 1 with profound hearing loss. Air-conducted clicks of 105 dB nHL and tone bursts (250 to 4000 Hz) of 100 dB nHL were presented monaurally through TDH 49 headphones during head elevation. Click-evoked responses were recorded under two conditions of gaze in random order: gaze straight ahead and rotated hard toward the contralateral recording electrodes. Amplitudes (corrected and uncorrected) and latencies for crossed SCM responses were compared with vestibular (ipsilateral SCM) and cochlear (PAM) responses between groups and across the different recording conditions. RESULTS: Surface waveforms were biphasic; positive-negative for the ipsilateral SCM, and negative-positive for the contralateral SCM and PAM. There were significant differences in the amplitudes and latencies (p = 0.004) for click responses of healthy controls across recording sites. PAM responses had the largest mean-corrected amplitudes (2.3 ± 2.8) and longest latencies (13.0 ± 1.2 msec), compared with ipsilateral (1.6 ± 0.5; 12.0 ± 0.7 msec) and contralateral (0.8 ± 0.3; 10.4 ± 1.0 msec) SCM responses. Uncorrected amplitudes and muscle activation for PAM increased by 104.4% and 46.8% with lateral gaze respectively, whereas SCM responses were not significantly affected. Click responses of patients with superior canal dehiscence followed a similar latency, amplitude, and gaze modulation trend as controls. SCM responses were preserved in the patient with profound hearing loss, yet all PAM were absent. There were significant differences in the frequency tuning of the three reflexes (p < 0.001). Tuning curves of healthy controls were flat for PAM and down sloping for ipsilateral and contralateral SCM responses. For superior canal dehiscence, they were rising for PAM and slightly down sloping for SCM responses. CONCLUSIONS: Properties of crossed SCM responses were similar, though not identical, to those of ipsilateral SCM responses and are likely to be predominantly vestibular in origin. They are unlikely to represent volume conduction from the PAM as they were unaffected by lateral gaze, were shorter in latency, and had different tuning properties. The influence of crossed vestibulo-collic pathways should be considered when interpreting cervical vestibular-evoked myogenic potentials recorded under conditions of binaural stimulation.

Investigating short latency subcortical vestibular projections in humans: what have we learned?

Vestibular evoked myogenic potentials (VEMPs) are now widely used for the noninvasive assessment of vestibular function and diagnosis in humans. This review focuses on the origin, properties, and mechanisms of cervical VEMPs and ocular VEMPs; how these reflexes relate to reports of vestibular projections to brain stem and cervical targets; and the physiological role of (otolithic) cervical and ocular reflexes. The evidence suggests that both VEMPs are likely to represent the effects of excitation of irregularly firing otolith afferents. While the air-conducted cervical VEMP appears to mainly arise from excitation of saccular receptors, the ocular VEMP evoked by bone-conducted stimulation, including impulsive bone-conducted stimuli, mainly arises from utricular afferents. The surface responses are generated by brief changes in motor unit firing. The effects that have been demonstrated are likely to represent otolith-dependent vestibulocollic and vestibulo-ocular reflexes, both linear and torsional. These observations add to previous reports of short latency otolith projections to the target muscles in the neck (sternocleidomastoid and splenius) and extraocular muscles (the inferior oblique). New insights have been provided by the investigation and application of these techniques.

Laboratory examinations for the vestibular system.

PURPOSE OF REVIEW: In the last decades, researchers suggested that clinical assessment of labyrinthine function in detail became easy thanks to video head impulse tests (VHITs), vestibular evoked myogenic potential test (VEMP) and video-oculography (VOG). It has been argued that they can replace electronystagmography, the caloric and rotatory chair tests. This review addresses the latest evaluations of these tests and the opportunities they offer, but also the limitations in clinical practice. RECENT FINDINGS: The VHIT and suppression head impulse test (SHIMP) are under ideal circumstances able to accurately identify deficits of the VOR in 3D. However, in a relevant part of the patient population, pupil tracking is inaccurate, video-goggles slip and VOR quantification is problematic. The dissociation between the VHIT and caloric test suggests that these tests are complementary. A new 3D-VOG technique claims to quantify eye torsion better than before, opening multiple diagnostic possibilities. VEMPs remain difficult to standardize. Variability in normal cervical vestibular-evoked myogenic potential amplitude is large. VEMPs become smaller or absent with age, raising questions of whether there is a lower normal limit at all. Recent research shows that the labyrinth is directly stimulated in the MRI offering new opportunities for diagnostics and research. SUMMARY: In clinical practice, the VHIT, SHIMP, VEMP and new 3D-VOG techniques improve diagnostic power. Unfortunately, technical issues or variability prevent reliable quantitative evaluation in a part of the regular patient population. The traditional caloric and rotatory chair test can still be considered as valuable complementary tests.

Contrasting phase effects on vestibular evoked myogenic potentials (VEMPs) produced by air- and bone-conducted stimuli.

We have studied the effects of stimulus phase on the latency and amplitude of cVEMPs and oVEMPs by reanalysing data from Lim et al. (Exp Brain Res 224:437-445, 2013) in which alternating phase was used. Responses for the different initial stimulus phase, either positive or negative, were separated and reaveraged. We found that the phase (compressive or rarefactive) of AC 500-Hz stimuli had no significant effect on either latency or amplitude of the responses. Conversely, phase (positive = motor towards subjects) did alter the effects of BC 500-Hz stimulation. For cVEMPs, phase consistently affected initial latency with earlier responses for positive stimuli, while, for stimulation at the mastoid, negative onset phase gave larger responses. For the oVEMP, effects were different for the two sites of BC stimulation. At the forehead, the response appeared to invert, whereas at the mastoid there appeared to be a delay of the initial response. Related to this, the effect of phase for the two sites was opposite: at the mastoid, positive responses were earlier but negative were larger (particularly for long stimuli). At the forehead, the effect was the opposite: negative onset stimuli evoked earlier responses, whereas positive onset evoked larger responses. These findings indicate a basic difference in the way that AC and BC stimuli activate vestibular receptors and also indicate that the effects of phase of BC stimulation depend on location. Stimulus alternation does little to affect the response to AC stimulation but obscures the effects of BC stimuli, particularly for the oVEMP.

TNF receptor superfamily member 13b (TNFRSF13B) hemizygosity reveals transmembrane activator and CAML interactor haploinsufficiency at later stages of B-cell development.

BACKGROUND: Heterozygous C104R or A181E TNF receptor superfamily member 13b (TNFRSF13B) mutations impair removal of autoreactive B cells, weaken B-cell activation, and convey to patients with common variable immune deficiency (CVID) an increased risk for autoimmunity. How mutant transmembrane activator and CAML interactor (TACI) influences wild-type TACI function is unclear; different models suggest either a dominant negative effect or haploinsufficiency. OBJECTIVE: We investigated potential TACI haploinsufficiency by analyzing patients with antibody-deficient Smith-Magenis syndrome (SMS) who possess only 1 TNFRSF13B allele and antibody-deficient patients carrying one c.204insA TNFRSF13B null mutation. METHODS: We tested the reactivity of antibodies isolated from single B cells from patients with SMS and patients with a c.204insA TNFRSF13B mutation and compared them with counterparts from patients with CVID with heterozygous C104R or A181E TNFRSF13B missense mutations. We also assessed whether loss of a TNFRSF13B allele induced haploinsufficiency in naive and memory B cells and recapitulated abnormal immunologic features typical of patients with CVID with heterozygous TNFRSF13B missense mutations. RESULTS: We found that loss of a TNFRSF13B allele does not affect TACI expression, activation responses, or establishment of central B-cell tolerance in naive B cells. Additionally, patients with SMS and those with a c.204insA TNFRSF13B mutation display normal regulatory T-cell function and peripheral B-cell tolerance. The lack of a TNFRSF13B allele did result in decreased TACI expression on memory B cells, resulting in impaired activation and antibody secretion. CONCLUSION: TNFRSF13B hemizygosity does not recapitulate autoimmune features of CVID-associated C104R and A181E TNFRSF13B mutations, which likely encode dominant negative products, but instead reveals selective TACI haploinsufficiency at later stages of B-cell development.

Clinical utility of ocular vestibular-evoked myogenic potentials (oVEMPs).

Over the last years, vestibular-evoked myogenic potentials (VEMPs) have been established as clinical tests of otolith function. Complementary to the cervical VEMPs, which assess mainly saccular function, ocular VEMPs (oVEMPs) test predominantly utricular otolith function. oVEMPs are elicited either with air-conducted (AC) sound or bone-conducted (BC) skull vibration and are recorded from beneath the eyes during up-gaze. They assess the vestibulo-ocular reflex and are a crossed excitatory response originating from the inferior oblique eye muscle. Enlarged oVEMPs have proven to be sensitive for screening of superior canal dehiscence, while absent oVEMPs indicate a loss of superior vestibular nerve otolith function, often seen in vestibular neuritis (VN) or vestibular Schwannoma.

Observation and prediction of recurrent human translocations mediated by NAHR between nonhomologous chromosomes.

Four unrelated families with the same unbalanced translocation der(4)t(4;11)(p16.2;p15.4) were analyzed. Both of the breakpoint regions in 4p16.2 and 11p15.4 were narrowed to large ∼359-kb and ∼215-kb low-copy repeat (LCR) clusters, respectively, by aCGH and SNP array analyses. DNA sequencing enabled mapping the breakpoints of one translocation to 24 bp within interchromosomal paralogous LCRs of ∼130 kb in length and 94.7% DNA sequence identity located in olfactory receptor gene clusters, indicating nonallelic homologous recombination (NAHR) as the mechanism for translocation formation. To investigate the potential involvement of interchromosomal LCRs in recurrent chromosomal translocation formation, we performed computational genome-wide analyses and identified 1143 interchromosomal LCR substrate pairs, >5 kb in size and sharing >94% sequence identity that can potentially mediate chromosomal translocations. Additional evidence for interchromosomal NAHR mediated translocation formation was provided by sequencing the breakpoints of another recurrent translocation, der(8)t(8;12)(p23.1;p13.31). The NAHR sites were mapped within 55 bp in ∼7.8-kb paralogous subunits of 95.3% sequence identity located in the ∼579-kb (chr 8) and ∼287-kb (chr 12) LCR clusters. We demonstrate that NAHR mediates recurrent constitutional translocations t(4;11) and t(8;12) and potentially many other interchromosomal translocations throughout the human genome. Furthermore, we provide a computationally determined genome-wide "recurrent translocation map."

Machine learning models help differentiate between causes of recurrent spontaneous vertigo.

BACKGROUND: Vestibular migraine (VM) and Menière's disease (MD) are two common causes of recurrent spontaneous vertigo. Using history, video-nystagmography and audiovestibular tests, we developed machine learning models to separate these two disorders. METHODS: We recruited patients with VM or MD from a neurology outpatient facility. One hundred features from six "feature subsets": history, acute video-nystagmography and four laboratory tests (video head impulse test, vestibular-evoked myogenic potentials, caloric testing and audiogram) were used. We applied ten machine learning algorithms to develop classification models. Modelling was performed using three "tiers" of data availability to simulate three clinical settings. "Tier 1" used all available data to simulate the neuro-otology clinic, "Tier 2" used only history, audiogram and caloric test data, representing the general neurology clinic, and "Tier 3" used history alone as occurs in primary care. Model performance was evaluated using tenfold cross-validation. RESULTS: Data from 160 patients with VM and 114 with MD were used for model development. All models effectively separated the two disorders for all three tiers, with accuracies of 85.77-97.81%. The best performing algorithms (AdaBoost and Random Forest) yielded accuracies of 97.81% (95% CI 95.24-99.60), 94.53% (91.09-99.52%) and 92.34% (92.28-96.76%) for tiers 1, 2 and 3. The best feature subset combination was history, acute video-nystagmography, video head impulse test and caloric testing, and the best single feature subset was history. CONCLUSIONS: Machine learning models can accurately differentiate between VM and MD and are promising tools to assist diagnosis by medical practitioners with diverse levels of expertise and resources.

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Post Cochlear Implantation Vertigo: Ictal Nystagmus and Audiovestibular Test Characteristics.

OBJECTIVE: To investigate ictal nystagmus and audiovestibular characteristics in episodic spontaneous vertigo after cochlear implantation (CI). STUDY DESIGN: Retrospective and prospective case series. PATIENTS: Twenty-one CI patients with episodic spontaneous vertigo after implantation were recruited. INTERVENTIONS: Patient-initiated home video-oculography recordings were performed during one or more attacks of vertigo, using miniature portable home video-glasses. To assess canal and otolith function, video head-impulse tests (vHITs) and vestibular-evoked myogenic potential tests were conducted. MAIN OUTCOME MEASURES: Nystagmus slow-phase velocities (SPVs), the presence of horizontal direction-changing nystagmus, and post-CI audiovestibular tests. RESULTS: Main final diagnoses were post-CI secondary endolymphatic hydrops (48%) and exacerbation of existing Ménière's disease (29%). Symptomatic patients demonstrated high-velocity horizontal ictal-nystagmus (SPV, 44.2°/s and 68.2°/s in post-CI secondary endolymphatic hydrop and Ménière's disease). Direction-changing nystagmus was observed in 80 and 75%. Two were diagnosed with presumed autoimmune inner ear disease (SPV, 6.6°/s and 172.9°/s). One patient was diagnosed with probable vestibular migraine (15.1°/s).VHIT gains were 0.80 ± 0.20 (lateral), 0.70 ± 0.17 (anterior), and 0.62 ± 0.27 (posterior) in the implanted ear, with abnormal values in 33, 35, and 35% of each canal. Bone-conducted cervical and ocular vestibular-evoked myogenic potentials were asymmetric in 52 and 29% of patients (all lateralized to the implanted ear) with mean asymmetry ratios of 51.2 and 35.7%. Reversible reduction in vHIT gain was recorded in three acutely symptomatic patients. CONCLUSION: High-velocity, direction-changing nystagmus time-locked with vertigo attacks may be observed in post-CI implant vertigo and may indicate endolymphatic hydrops. Fluctuating vHIT gain may be an additional marker of a recurrent peripheral vestibulopathy.

New perspectives on vestibular evoked myogenic potentials.

PURPOSE OF REVIEW: Although the vestibular evoked myogenic potential (VEMP) measured from the cervical muscles (cVEMP, cervical VEMP) is well described and has documented clinical utility, its analogue recorded from the extraocular muscles (oVEMP, ocular VEMP) has been described only recently and is currently emerging as an additional test of otolith function. This review will, therefore, summarize recent developments in VEMP research with a focus on the oVEMP. RECENT FINDINGS: Recent studies suggest that the oVEMP is produced by otolith afferents in the superior vestibular nerve division, whereas the cVEMP evoked by sound is thought to be an inferior vestibular nerve reflex. Correspondingly, the oVEMP correlates better with caloric and subjective visual vertical tests than sound-cVEMPs. cVEMPs are more complicated than often thought, as shown by the presence of crossed responses and conflicting results of recent vibration studies. Altered inner ear mechanics produced by the vestibular diseases superior semicircular canal dehiscence and Ménière's disease lead to changes in the preferred frequency of the oVEMP and cVEMP. SUMMARY: The oVEMP provides complementary diagnostic information to the cVEMP and is likely to be a useful addition to the diagnostic test battery in neuro-otology.

Magnitude, variability and symmetry in head acceleration and jerk and their relationship to cervical and ocular vestibular evoked myogenic potentials.

BACKGROUND: Acceleration and changes in acceleration (jerk) stimulate vestibular otolith afferents. Bone-conducted (BC) vibration applied to the skull accelerates the head and produces short latency reflexes termed vestibular evoked myogenic potentials (VEMPs). OBJECTIVE: To determine the magnitude, variability and symmetry in head acceleration/jerk during VEMP recordings and investigate the relationship between head acceleration/jerk and VEMP properties. METHODS: 3D head accelerometery (sagittal, interaural and vertical axes) was recorded bilaterally in thirty-two healthy subjects during cervical (cVEMP) and ocular (oVEMP) recordings. BC 500 Hz sinusoidal tones were applied to the midline forehead using a positive polarity stimulus. RESULTS: The direction of induced acceleration/jerk was predominately backward, outward and downward on either side of the head during cVEMP and oVEMP recordings.Overall, acceleration/jerk was larger in the sagittal and interaural axes and peaked earlier in the interaural axis bilaterally. Acceleration was more symmetric in the sagittal and interaural axes whereas jerk symmetry did not differ between axes. Regression models did not show a systematic relationship between acceleration/jerk and either VEMP reflex. CONCLUSIONS: The pattern of skull acceleration/jerk was relatively consistent between the two sides of the head and across subjects, but there were differences in magnitude, leading to inter-side and inter-subject variability.

The Relationship between the Subjective Visual Horizontal and Ocular Vestibular Evoked Myogenic Potentials in Acute Vestibular Neuritis.

OBJECT: Vestibular evoked myogenic potentials (VEMPs) and the subjective visual horizontal (SVH) (or vertical [SVV]) have both been considered tests of otolith function: ocular-VEMPs (oVEMPs) utricular function, cervical VEMPs (cVEMPs) saccular function. Some studies have reported association between decreased oVEMPs and SVH, whereas others have not. DESIGN: A retrospective study of test results. SETTING: A tertiary, neuro-otology clinic, Royal Prince Alfred Hospital, Sydney, Australia. METHOD: We analyzed results in 130 patients with acute vestibular neuritis tested within 5 days of onset. We sought correlations between the SVH, oVEMPs, and cVEMPs to air-conducted (AC) and bone-conducted (BC) stimulation. RESULTS: The SVH deviated to the side of lesion, in 123 of the 130 AVN patients, by 2.5 to 26.7 degrees. Ninety of the AVN patients (70%) had abnormal oVEMPs to AC, BC or both stimuli, on the AVN side (mean asymmetry ratio ± SD [SE]): (64 ± 45.0% [3.9]). Forty-three of the patients (35%) had impaired cVEMPs to AC, BC or both stimuli, on the AVN side, [22 ± 41.6% (4.1)]. The 90 patients with abnormal oVEMP values also had abnormal SVH. Correlations revealed a significant relationship between SVH offset and oVEMP asymmetry (r = 0.80, p < 0.001) and a weaker relationship between SVH offset and cVEMP asymmetry (r = 0.56, p < 0.001). CONCLUSIONS: These results indicate that after an acute unilateral vestibular lesion, before there has been a chance for vestibular compensation to occur, there is a significant correlation between the SVH, and oVEMP results. The relationship between SVH offset and oVEMP amplitude suggests that both tests measure utricular function.