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.

Three-Dimensional High-Resolution Temporal Bone Histopathology Identifies Areas of Vascular Vulnerability in the Inner Ear.

OBJECTIVES: Hypothesized causes of vestibular neuritis/labyrinthitis include neuroinflammatory or vascular disorders, yet vascular disorders of the inner ear are poorly understood. Guided by known microvascular diseases of the retina, we developed 2 hypotheses: (1) there exist vascular vulnerabilities of artery channels in cases of hypothetical nerve swelling for the superior, inferior, and vestibulocochlear artery and (2) there are arteriovenous crossings that could compromise vascular flow in disease states. METHODS: Two fully mounted and stained temporal bones were used to render three-dimensional reconstructions of the labyrinth blood supply. Using these maps, areas of potential vascular compression were quantified in 50 human temporal bones. RESULTS: Although inner ear arteries and veins mostly travel within their own bony channels, they may be exposed (1) at the entrance into the otic capsule, and (2) where the superior vestibular vein crosses the inferior vestibular artery. At the entry into the otic capsule, the ratio of the soft tissue to total space for the superior vestibular artery was significantly greater than the inferior vestibular artery/cochleovestibular artery (median 44, interquartile range 34-55 vs. 14 [9-17], p < 0.0001). CONCLUSIONS: Three-dimensional reconstruction of human temporal bone histopathology can guide vascular studies of the human inner ear. Studies of retinal microvascular disease helped identify areas of vascular vulnerability in cases of hypothetical nerve swelling at the entrance into the otic capsule and at an arteriovenous crossing near the saccular macula. These data may help explain patterns of clinical findings in peripheral vestibular lesions.

Clinical, oculographic, and vestibular test characteristics of vestibular migraine.

BACKGROUND: We characterise the history, vestibular tests, ictal and interictal nystagmus in vestibular migraine. METHOD: We present our observations on 101 adult-patients presenting to an outpatient facility with recurrent spontaneous and/or positional vertigo whose final diagnosis was vestibular migraine (n = 27) or probable vestibular migraine (n = 74). Ictal and interictal video-oculography, caloric and video head impulse tests, vestibular-evoked myogenic potentials and audiometry were performed. RESULTS: Common presenting symptoms were headache (81.2%), spinning vertigo (72.3%), Mal de Débarquement (58.4%), and motion sensitivity (30.7%). With fixation denied, ictal and interictal spontaneous nystagmus was observed in 71.3 and 14.9%, and purely positional nystagmus in 25.8 and 55.4%. Spontaneous ictal nystagmus was horizontal in 49.5%, and vertical in 21.8%. Ictal spontaneous and positional nystagmus velocities were 5.3 ± 9.0°/s (range 0.0-57.4), and 10.4 ± 5.8°/s (0.0-99.9). Interictal spontaneous and positional nystagmus velocities were <3°/s in 91.8 and 23.3%. Nystagmus velocities were significantly higher when ictal (p < 0.001/confidence interval: 2.908‒6.733, p < 0.001/confidence interval: 5.308‒10.085). Normal lateral video head impulse test gains were found in 97.8% (mean gain 0.95 ± 0.12) and symmetric caloric results in 84.2% (mean canal paresis 7.0 ± 23.3%). Air- and bone-conducted cervical-vestibular-evoked myogenic potential amplitudes were symmetric in 88.4 and 93.4% (mean corrected amplitude 1.6 ± 0.7, 1.6 ± 0.8) with mean asymmetry ratios of 13.0 and 9.0%. Air- and bone-conducted ocular-vestibular-evoked myogenic potentials were symmetric in 67.7 and 97.2% (mean amplitude 9.2 ± 6.4 and 20.3 ± 12.8 µV) with mean asymmetry ratios of 15.7 and 9.9%. Audiometry was age consistent and symmetric in 85.5%. CONCLUSION: Vestibular migraine is characterised by low velocity ictal spontaneous nystagmus, which can be horizontal, vertical, or torsional, and normal audiovestibular test results.

The human vestibulo-ocular reflex and saccades: normal subjects and the effect of age.

Here we characterize in 80 normal subjects (16-84 yr (means ± SD, 47 ± 19 yr) the vestibulo-ocular reflex (VOR) and saccades in response to three-dimensional head impulses with a monocular video head impulse test (vHIT) of the right eye. Impulses toward the right lateral, right anterior, and left posterior canals (means: 0.98, 0.91, 0.79) had slightly higher mean gains compared with their counterparts (0.95, 0.86, 0.76). In the older age group (>60 yr), gains of the left posterior canal dropped 0.09 and left anterior canals rose 0.09 resulting in symmetry. All canal gains reduced with increasing head velocity (0.02-0.13 per 100°/s). Comparison of lateral canal gains calculated using five published algorithms yielded lower values (~0.80) when a narrow detection window was used. Low-amplitude refixation saccades (amplitude: 1.11 ± 0.98°, peak velocity: 63.9 ± 34.0°/s at 262.0 ± 93.9 ms) were observed among all age groups (frequency: 40.2 ± 23.4%), increasing in amplitude, peak velocity, and frequency in older subjects. Impulses toward anterior canals showed the least frequent saccades and lateral and posterior canals were similar, but lateral canal impulses showed the smallest saccades and the posterior canal showed the largest saccades. Saccade peak-velocity approximate amplitude "main sequence" slope was steeper for the horizontal canals compared with the vertical planes (60 vs. <40°/s per 1°). In summary, we found small but significant asymmetries in monocular vHIT gain that changed with age. Healthy subjects commonly have minuscule refixation saccades that are moderately to strongly correlated with vHIT gain. NEW & NOTEWORTHY Gaze fixation is normally stabilized during rapid "head-impulse" movements by the bisynaptic vestibulo-ocular reflex (VOR), but earlier studies of normal subjects also report small amplitude saccades. We found that with increased age of the subject the vertical VOR became more variable, while in all semicircular canal directions the saccade frequency, amplitude, and peak velocity increased. We also found that the VOR gain algorithm significantly influences values.

Dizziness demystified.

Four vestibular presentations caused by six different disorders constitute most of the neuro-otology cases seen in clinical practice. 'Acute vestibular syndrome' refers to a first-ever attack of acute, spontaneous, isolated vertigo and there are two common causes: vestibular neuritis / labyrinthitis and cerebellar infarction. Recurrent positional vertigo is most often caused by benign paroxysmal positional vertigo and less commonly is central in origin. Recurrent spontaneous vertigo has two common causes: Ménière's disease and vestibular migraine. Lastly, chronic vestibular insufficiency (imbalance) results from bilateral, or severe unilateral, peripheral vestibular impairment. These six disorders can often be diagnosed on the basis of history, examination, audiometry, and in some cases, basic vestibular function testing. Here we show that most common neuro-otological problems can be readily managed by general neurologists.

Neural basis of new clinical vestibular tests: otolithic neural responses to sound and vibration.

Extracellular single neuron recording and labelling studies of primary vestibular afferents in Scarpa's ganglion have shown that guinea-pig otolithic afferents with irregular resting discharge are preferentially activated by 500 Hz bone-conducted vibration (BCV) and many also by 500 Hz air-conducted sound (ACS) at low threshold and high sensitivity. Very few afferent neurons from any semicircular canal are activated by these stimuli and then only at high intensity. Tracing the origin of the activated neurons shows that these sensitive otolithic afferents originate mainly from a specialized region, the striola, of both the utricular and saccular maculae. This same 500 Hz BCV elicits vestibular-dependent eye movements in alert guinea-pigs and in healthy humans. These stimuli evoke myogenic potentials, vestibular-evoked myogenic potentials (VEMPs), which are used to test the function of the utricular and saccular maculae in human patients. Although utricular and saccular afferents can both be activated by BCV and ACS, the differential projection of utricular and saccular afferents to different muscle groups allows for differentiation of the function of these two sensory regions. The basic neural data support the conclusion that in human patients in response to brief 500 Hz BCV delivered to Fz (the midline of the forehead at the hairline), the cervical VEMP indicates predominantly saccular function and the ocular VEMP indicates predominantly utricular function. The neural, anatomical and behavioural evidence underpins clinical tests of otolith function in humans using sound and vibration.

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.

Longer duration entry mitigates nystagmus and vertigo in 7-Tesla MRI.

Introduction: Patients and technologists commonly describe vertigo, dizziness, and imbalance near high-field magnets, e.g., 7-Tesla (T) magnetic resonance imaging (MRI) scanners. We sought a simple way to alleviate vertigo and dizziness in high-field MRI scanners by applying the understanding of the mechanisms behind magnetic vestibular stimulation and the innate characteristics of vestibular adaptation. Methods: We first created a three-dimensional (3D) control systems model of the direct and indirect vestibulo-ocular reflex (VOR) pathways, including adaptation mechanisms. The goal was to develop a paradigm for human participants undergoing a 7T MRI scan to optimize the speed and acceleration of entry into and exit from the MRI bore to minimize unwanted vertigo. We then applied this paradigm from the model by recording 3D binocular eye movements (horizontal, vertical, and torsion) and the subjective experience of eight normal individuals within a 7T MRI. The independent variables were the duration of entry into and exit from the MRI bore, the time inside the MRI bore, and the magnetic field strength; the dependent variables were nystagmus slow-phase eye velocity (SPV) and the sensation of vertigo. Results: In the model, when the participant was exposed to a linearly increasing magnetic field strength, the per-peak (after entry into the MRI bore) and post-peak (after exiting the MRI bore) responses of nystagmus SPV were reduced with increasing duration of entry and exit, respectively. There was a greater effect on the per-peak response. The entry/exit duration and peak response were inversely related, and the nystagmus was decreased the most with the 5-min duration paradigm (the longest duration modeled). The experimental nystagmus pattern of the eight normal participants matched the model, with increasing entry duration having the strongest effect on the per-peak response of nystagmus SPV. Similarly, all participants described less vertigo with the longer duration entries. Conclusion: Increasing the duration of entry into and exit out of a 7T MRI scanner reduced or eliminated vertigo symptoms and reduced nystagmus peak SPV. Model simulations suggest that central processes of vestibular adaptation account for these effects. Therefore, 2-min entry and 20-s exit durations are a practical solution to mitigate vertigo and other discomforting symptoms associated with undergoing 7T MRI scans. In principle, these findings also apply to different magnet strengths.

Association Between Retinal Pathology and Hearing in Older Adults.

We investigated the association between retinal microvascular changes and hearing loss based on the hypothesis that both may result from shared microvascular pathology. Data from 536 older adults from the National Health and Nutritional Examination Survey 2005 to 2006 including sociodemographic and health characteristics, pure-tone hearing thresholds, and retinal pathologies were collected and analyzed. Associations between retinal and hearing pathologies were modeled with multivariable-adjusted linear regressions. 75% of participants had hearing loss and 15% of participants had retinopathy. The association between retinopathy, microaneurysms, and blot hemorrhages with better speech-frequency pure tone average was -2.81 (95% confidence interval [CI]: -5.72 to 0.10), -4.75 (95% CI: -8.73 to -0.78), and -5.34 (95% CI: -8.68 to -2.00), respectively. The presence of retinopathy, microaneurysms, and blot hemorrhages was inversely associated with hearing loss. Further studies are needed to better understand the potential relationship between microvascular pathologies of the eye and ear.

Otolith Membrane Herniation, not Semicircular Canal Duct Dilation, Is Associated with Decreased Caloric Responses in Ménière's Disease.

Ménière's disease (MD) is a debilitating disorder with unclear pathophysiology whose diagnosis often relies on clinical judgment rather than objective testing. To complicate matters further, a dissociation has emerged between two vestibular function tests commonly used in patients with MD to examine the same end-organ (the semicircular canals): the caloric test and video head impulse testing (vHIT). Caloric responses are often abnormal, while vHIT results remain normal. Explaining this dissociation could reveal novel insights into MD pathophysiology. Here, we conduct a histopathological study using temporal bone specimens (N = 58, 21 MD-affected ears and 37 age-matched controls) and their clinical testing data to examine current hypotheses aimed at this dissociation. We find otolith membrane herniation into the horizontal semicircular canal in 69% of MD ears, with 90% of these ears demonstrating a diminished caloric response. No ears with a normal response had this herniation. Moreover, we evaluated the semicircular canals for endolymphatic hydrops, which had been hypothesized to contribute to the dissociation, and found no evidence of duct dilation/hydrops. We did, however, note a potentially novel morphologic finding-smaller bony labyrinth cross-sectional diameters/areas in some MD ear canals compared to controls, suggesting relative size of the membranous duct to the bony canal rather than absolute size may be of importance. Taken together, this study refines hypotheses on the vestibular test dissociation in MD, holding diagnostic implications and expanding our understanding of the mechanisms underlying this enigmatic disease.

Subjective visual horizontal correlates better with ocular than with cervical vestibular evoked myogenic potentials.

OBJECTIVE: To examine the relationship between widely used otolith function tests: the Subjective Visual Horizontal (SVH) and Vestibular Evoked Myogenic Potentials (VEMP). METHODS: A retrospective analysis was performed on 301 patients who underwent SVH, ocular and cervical VEMP (oVEMP and cVEMP) tests on the same day. Correlations between the mean SVH tilt and amplitude asymmetry ratios for bone-conducted (BC) oVEMP and air-conducted (AC) cVEMP were examined. Diagnoses included vestibular neuritis, stroke, vestibular migraine, Meniere's disease, sudden sensorineural hearing loss (SSNHL) and vestibular schwannoma. RESULTS: SVH results were concordant with the oVEMP in 64% of cases and the cVEMP in 51%. Across all patients, SVH demonstrated a significant moderate correlation with BC oVEMP amplitude asymmetry ratios (r = 0.55, p < 0.001) and a weak correlation with AC cVEMP amplitude asymmetry ratios (r = 0.35, p < 0.001). A stronger correlation between SVH and oVEMPs was observed in patients with vestibular neuritis (r = 0.67, p < 0.001) and SSNHL (r = 0.76, p = 0.001). CONCLUSIONS: SVH correlates better with oVEMP than cVEMP symmetry. SIGNIFICANCE: This finding reinforces the hypothesis of a common utricular origin for both SVH and oVEMPs which is distinct from the saccular origin of cVEMPs.

Clinical, oculographic and vestibular test characteristics of Ménière's disease.

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).

The human vestibulo-ocular reflex and compensatory saccades in schwannoma patients before and after vestibular nerve section.

OBJECTIVE: To examine the vestibulo-ocular reflex (VOR) and compensatory-saccades before and after complete unilateral vestibular deafferentation (UVD). METHODS: Forty patients were studied before and after surgery for vestibular or facial schwannoma using the video head-impulse test (vHIT) and multivariable regression. RESULTS: Prior to UVD (median(IQR), 14(58.4) days), the average VOR-gain towards the lesioned-ear was lower than in normal for all semicircular canals (lateral, anterior, posterior: 0.69, 0.72, 0.49). One-week after UVD (5(3.0) days) VOR gains were further reduced (0.22, 0.37, 0.27), however, within one-year after UVD (171(125.0) days) the lesioned-ear VOR gains had slightly increased (+0.08, +0.11, +0.03), maximally for the anterior-canal. After UVD, the VOR gain asymmetry (gain towards minus away from intact-ear) was lower for the intact posterior-canal plane (0.56, 0.56, 0.22). For the lesioned canals, the frequency and amplitude of the first compensatory-saccade increased from 61-93% and 1.9-3.6° pre-surgery, to 98-99% and to 3.1-5.9° one-week post-surgery and remained unchanged over one-year; second saccade frequency and amplitude decreased over the same timespan. CONCLUSIONS: After UVD the high-acceleration VOR for the intact posterior-canal plane is more symmetrical than the other canals. First compensatory-saccades adapt within one week and subsequently change only marginally. SIGNIFICANCE: Saccade compensation from surgical UVD is near complete by one-week.

MRI With Gadolinium as a Measure of Blood-Labyrinth Barrier Integrity in Patients With Inner Ear Symptoms: A Scoping Review.

Objective: Capillaries within the inner ear form a semi-permeable barrier called the blood-labyrinth barrier that is less permeable than capillary barriers elsewhere within the human body. Dysfunction of the blood-labyrinth barrier has been proposed as a mechanism for several audio-vestibular disorders. There has been interest in using magnetic resonance imaging (MRI) with intravenous gadolinium-based contrast agents (GBCA) as a marker for the integrity of the blood labyrinth barrier in research and clinical settings. This scoping review evaluates the evidence for using intravenous gadolinium-enhanced MRI to assess the permeability of the blood-labyrinth barrier in healthy and diseased ears. Methods: A systematic search was conducted of three databases: PubMed, EMBASE, CINAHL PLUS. Studies were included that used GBCA to study the inner ear and permeability of the blood-labyrinth barrier. Data was collected on MRI protocols used and inner ear enhancement patterns of healthy and diseased ears in both human and animal studies. Results: The search yielded 14 studies in animals and 53 studies in humans. In healthy animal and human inner ears, contrast-enhanced MRI demonstrated gradual increase in inner ear signal intensity over time that was limited to the perilymph. Signal intensity peaked at 100 min in rodents and 4 h in humans. Compared to controls, patients with idiopathic sudden sensorineural hearing loss and otosclerosis had increased signal intensity both before and shortly after GBCA injection. In patients with Ménière's disease and vestibular schwannoma, studies reported increased signal at 4 h, compared to controls. Quality assessment of included studies determined that all the studies lacked sample size justification and many lacked adequate control groups or blinded assessors of MRI. Conclusions: The included studies provided convincing evidence that gadolinium crosses the blood-labyrinth barrier in healthy ears and more rapidly in some diseased ears. The timing of increased signal differs by disease. There was a lack of evidence that these findings indicate general permeability of the blood-labyrinth barrier. Future studies with consistent and rigorous methods are needed to investigate the relationship between gadolinium uptake and assessments of inner ear function and to better determine whether signal enhancement indicates permeability for molecules other than gadolinium.

A Window Into the Whole Story: Temporal Bone Plasmacytoma Presenting With a Mobile Third Window.

A 63-year-old man presented with imbalance when coughing due to a respiratory tract infection. He had a history of multiple myeloma with a plasmacytoma of the left temporal bone. Examination revealed a positive leftward head impulse test, no spontaneous nystagmus, left-beating positional nystagmus, and left-beating Valsalva-induced nystagmus. Videonystagmography, audiology, and comprehensive vestibular function tests revealed a subtotal left peripheral audio-vestibular loss. Temporal bone computed tomography showed an unchanged bony erosion of the left labyrinth from 2 years prior. Vertigo subsided after treatment of the respiratory tract infection. Although no tumor progression was evident, coughing had triggered a preexisting third mobile window to declare itself. Laryngoscope, 131:E966-E969, 2021.

Opinion and Special Articles: Remote Evaluation of Acute Vertigo: Strategies and Technological Considerations.

Patients with acute vestibular disorders are often a diagnostic challenge for neurologists, especially when the evaluation must be conducted remotely. The clinical dilemma remains: Does the patient have a benign peripheral inner ear problem or a worrisome central vestibular disorder, such as a stroke? The use of a focused history and the virtual HINTS (head impulse test, nystagmus evaluation, and test of skew) examination are key steps towards correctly diagnosing and triaging the acute vertiginous patient. When looking for signs of vestibulo-ocular dysfunction, there are important technological and practical considerations for an effective clinical interpretation.

Bone-Conducted oVEMP Latency Delays Assist in the Differential Diagnosis of Large Air-Conducted oVEMP Amplitudes.

Background: A sensitive test for Superior Semicircular Canal Dehiscence (SCD) is the air-conducted, ocular vestibular evoked myogenic potential (AC oVEMP). However, not all patients with large AC oVEMPs have SCD. This retrospective study sought to identify alternate diagnoses also producing enlarged AC oVEMPs and investigated bone-conducted (BC) oVEMP outcome measures that would help differentiate between these, and cases of SCD. Methods: We reviewed the clinical records and BC oVEMP results of 65 patients (86 ears) presenting with dizziness or balance problems who underwent CT imaging to investigate enlarged 105 dB nHL click AC oVEMP amplitudes. All patients were tested with BC oVEMPs using two different stimuli (1 ms square-wave pulse and 8 ms 125 Hz sine wave). Logistic regression and odds ratios were used to determine the efficacy of BC oVEMP amplitudes and latencies in differentiating between enlarged AC oVEMP amplitudes due to dehiscence from those with an alternate diagnosis. Results: Fifty-three ears (61.6%) with enlarged AC oVEMP amplitudes were identified as having frank dehiscence on imaging; 33 (38.4%) had alternate diagnoses that included thinning of the bone covering (near dehiscence, n = 13), vestibular migraine (n = 12 ears of 10 patients), enlarged vestibular aqueduct syndrome (n = 2) and other causes of recurrent episodic vertigo (n = 6). BC oVEMP amplitudes of dehiscent and non-dehiscent ears were not significantly different (p > 0.05); distributions of both groups overlapped with the range of healthy controls. There were significant differences in BC oVEMP latencies between dehiscent and non-dehiscent ears for both stimuli (p < 0.001). A prolonged n1 125 Hz latency (>11.5 ms) was the best predictor of dehiscence (odd ratio = 27.8; 95% CI:7.0-111.4); abnormal n1 latencies were identified in 79.2% of ears with dehiscence compared with 9.1% of ears without dehiscence. Conclusions: A two-step protocol of click AC oVEMP amplitudes and 125 Hz BC oVEMP latency measures optimizes the specificity of VEMP testing in SCD.

Head impulse compensatory saccades: Visual dependence is most evident in bilateral vestibular loss.

The normal vestibulo-ocular reflex (VOR) generates almost perfectly compensatory smooth eye movements during a 'head-impulse' rotation. An imperfect VOR gain provokes additional compensatory saccades to re-acquire an earth-fixed target. In the present study, we investigated vestibular and visual contributions on saccade production. Eye position and velocity during horizontal and vertical canal-plane head-impulses were recorded in the light and dark from 16 controls, 22 subjects after complete surgical unilateral vestibular deafferentation (UVD), eight subjects with idiopathic bilateral vestibular loss (BVL), and one subject after complete bilateral vestibular deafferentation (BVD). When impulses were delivered in the horizontal-canal plane, in complete darkness compared with light, first saccade frequency mean(SEM) reduced from 96.6(1.3)-62.3(8.9) % in BVL but only 98.3(0.6)-92.0(2.3) % in UVD; saccade amplitudes reduced from 7.0(0.5)-3.6(0.4) ° in BVL but were unchanged 6.2(0.3)-5.5(0.6) ° in UVD. In the dark, saccade latencies were prolonged in lesioned ears, from 168(8.4)-240(24.5) ms in BVL and 177(5.2)-196(5.7) ms in UVD; saccades became less clustered. In BVD, saccades were not completely abolished in the dark, but their amplitudes decreased from 7.3-3.0 ° and latencies became more variable. For unlesioned ears (controls and unlesioned ears of UVD), saccade frequency also reduced in the dark, but their small amplitudes slightly increased, while latency and clustering remained unchanged. First and second saccade frequencies were 75.3(4.5) % and 20.3(4.1) %; without visual fixation they dropped to 32.2(5.0) % and 3.8(1.2) %. The VOR gain was affected by vision only in unlesioned ears of UVD; gains for the horizontal-plane rose slightly, and the vertical-planes reduced slightly. All head-impulse compensatory saccades have a visual contribution, the magnitude of which depends on the symmetry of vestibular-function and saccade latency: BVL is more profoundly affected by vision than UVD, and second saccades more than first saccades. Saccades after UVD are probably triggered by contralateral vestibular function.

NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome.

Opsoclonus-myoclonus syndrome (OMS) is a brainstem/cerebellar syndrome producing disabling multi-directional saccadic oscillations with oscillopsia, with or without somatic myoclonus and cerebellar ataxia (Wong et al., 2001; Armangué et al., 2016). OMS is presumed to have an autoimmune basis and patients with it are tested for antineuronal antibodies and have imaging to locate any tumors. Here we report a unusual case of a young woman who had NMDAR antibody (NMDAR-ab) positive, teratoma-related, isolated OMS without encephalopathy. Removal of her ovarian teratoma, and immunotherapy with steroids, intravenous immunoglobulin (IVIg), plasma exchange (PLEX), and ultimately with B-cell depletion with rituximab resulted in total recovery after 3 months. Patients with teratoma-related OMS very rarely have NMDAR-ab which suggests that it is not the NMDAR-ab per se that causes the OMS.