Suppression Of Vestibulo-ocular Reflex Research Articles

Clinical and Video-Oculographic Characteristics of Spinocerebellar Ataxia Type 27B (GAA-FGF14 Ataxia): A Single-Center Retrospective Study

An intronic GAA repeat expansion in the FGF14 gene was recently identified as a common cause of autosomal dominant GAA-FGF14 ataxia (SCA27B). We aimed to characterize in detail the clinical and video-oculographic features in our cohort of SCA27B patients. We genotyped the FGF14 GAA repeat expansion in 52 patients with unsolved late-onset cerebellar ataxia. Brain MRI and nerve conduction study, as well as video-oculographic (VOG) assessment, were performed. Eight patients (15.4%) with pathogenic GAA repeat expansion in the FGF14 gene were found. The median age at onset was 51 years (range—23–63 years). Sensory axonal neuropathy was found in 5/8 patients. Cerebellar atrophy was observed in 5/8 patients, and in one case, pontocerebellar atrophy was found. All tested patients had impaired smooth pursuit, 5/6 patients had impaired vestibulo-ocular reflex suppression, nystagmus, and an increased number of square wave jerks, 4/6 patients had horizontal gaze-evoked nystagmus, 3/6 had spontaneous downbeat nystagmus, and 1/6 had an upbeat one. Video head impulse test gain was lower than 0.8 on both sides in 2/4 patients, along with the presence of overt saccades. Further studies in different cohorts are needed to complete the phenotype of the FGF14-related disorders.

The patterns of anti-compensatory saccades of the SHIMP paradigm can differentiate vestibular migraine from healthy individuals.

The aim of the present study was to characterize the HIMP and SHIMP test findings in individuals with vestibular migraine. The study comprised two groups- group I consisted of twenty five adults diagnosed with Vestibular migraine, and group II comprised twenty-five age matched healthy individuals. HIMP & SHIMP were administered using the ICS impulse system for both groups. Mann-Whitney U test revealed a significant difference (p< 0.05) in VOR gain and VOR suppression gain between the vestibular migraine and healthy individuals. All the subjects in the vestibular migraine group manifested either of the four patterns of anti-compensatory saccades (ACS): (a) bilaterally absent ACS, (b) bilaterally scattered ACS or (c) typical ACS on one side with scattered ACS on the other side (d) unilaterally absent ACS. On the other hand, all the healthy individuals exhibited the presence of an organised ACS bilaterally. VOR pathway is affected in individuals with vestibular migraine. Also, various patterns of ACS are more effective in identifying the subtle vestibular lesion in vestibular migraine. These patterns are useful in differentiating vestibular migraine from healthy individuals.

Update on central oculomotor disorders and nystagmus

The diagnosis of ocular motor disorders and the different forms of a nystagmus is based on a systematic clinical examination of all types of eye movements: eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum, and cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal in the pons. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades.Nystagmus is defined as a rhythmic, most often involuntary eye movement. It normally consists of a slow (pathological) drift of the eyes and a fast central compensatory movement of the eyes back to the primary position (re-fixation saccade). There are three major categories: first, spontaneous nystagmus, i. e. nystagmus which occurs in the gaze straight ahead position as upbeat or downbeat nystagmus; second, nystagmus that becomes visible at eccentric gaze only and third, nystagmus which can be elicited by certain maneuvers, e. g. head-shaking, head positioning, air pressure or hyperventilation, most of which are of peripheral vestibular origin. The most frequent central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of central nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the mesencephalon or medulla oblongata. Examples of pharmacotherapy are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for fixation pendular nystagmus or baclofen for periodic alternating nystagmus.

Speech, Gait, and Vestibular Function in Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome.

(1) Background: Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by late-onset cerebellar ataxia, bilateral vestibulopathy, and sensory neuronopathy mostly due to biallelic RFC1 expansion. (2) Objectives: The aim of this case series is to describe vestibular, gait, and speech alterations in CANVAS via a systematic approach. (3) Methods: All patients (n = 5) underwent a standardized clinical-instrumental examination, including the perceptual and acoustic analysis of speech, instrumental gait, and balance analysis (posturographic data were acquired using a force plate [Kistler, Winterthur, Switzerland] while 3D gait analysis, inclusive of surface electromyography, was acquired using a motion capture system [SMART DX, BTS Bioengineering, Milan, Italy], a wireless electromyograph [FreeEMG, BTS Bioengineering, Milan, Italy]), and vestibular assessment with video-oculography. (4) Results: Five patients were included in the analysis: three females (patients A, B, C) and two males (patients D and E) with a mean age at evaluation of 62 years (SD ± 15.16, range 36-74). The mean age of symptoms' onset was 55.6 years (SD ± 15.04, range 30-68), and patients were clinically and instrumentally evaluated with a mean disease duration of 6.4 years (SD ± 0.54, range 6-7). Video-Frenzel examination documented spontaneous downbeat nystagmus enhanced on bilateral gaze in all patients, except for one presenting with slight downbeat nystagmus in the supine position. All patients exhibited different degrees of symmetrically reduced VOR gain for allsix semicircular canals on the video-head impulse test and an unexpectedly normal ("false negative") VOR suppression, consistent with combined cerebellar dysfunction and bilateral vestibular loss. Posturographic indices were outside their age-matched normative ranges in all patients, while 3D gait analysis highlighted a reduction in ankle dorsiflexion (limited forward rotation of the tibia over the stance foot during the stance phase of gait and fatigue of the dorsiflexor muscles) and variable out-of-phase activity of plantar flexors during the swing phase. Finally, perceptual-acoustic evaluation of speech showed ataxic dysarthria in three patients. Dysdiadochokinesis, rhythm instability, and irregularity were observed in the oral diadochokinesis task. (5) Conclusions: CANVAS is a recently discovered syndrome that is gaining more and more relevance within late-onset ataxias. In this paper, we aimed to contribute to a detailed description of its phenotype.

Normative Ranges for, and Interrater Reliability of, Rotational Vestibular and Balance Tests in U.S. Military Service Members and Veterans.

The objectives of this study were to (a) describe normative ranges-expressed as reference intervals (RIs)-for vestibular and balance function tests in a cohort of Service Members and Veterans (SMVs) and (b) to describe the interrater reliability of these tests. As part of the Defense and Veterans Brain Injury Center (DVBIC)/Traumatic Brain Injury Center of Excellence 15-year Longitudinal Traumatic Brain Injury (TBI) Study, participants completed the following: vestibulo-ocular reflex suppression, visual-vestibular enhancement, subjective visual vertical, subjective visual horizontal, sinusoidal harmonic acceleration, the computerized rotational head impulse test (crHIT), and the sensory organization test. RIs were calculated using nonparametric methods and interrater reliability was assessed using intraclass correlation coefficients between three audiologists who independently reviewed and cleaned the data. Reference populations for each outcome measure comprised 40 to 72 individuals, 19 to 61 years of age, who served either as noninjured controls (NIC) or injured controls (IC) in the 15-year study; none had a history of TBI or blast exposure. A subset of 15 SMVs from the NIC, IC, and TBI groups were included in the interrater reliability calculations. RIs are reported for 27 outcome measures from the seven rotational vestibular and balance tests. Interrater reliability was considered excellent for all tests except the crHIT, which was found to have good interrater reliability. This study provides clinicians and scientists with important information regarding normative ranges and interrater reliability for rotational vestibular and balance tests in SMVs.

Test-Retest Reliability, Effects of Age and Comparison of Horizontal 2 Semicircular Canals Gain Values between Head Impulse and Suppression Head Impulse Paradigms.

This study investigates the test-retest reliability, aging effects, and differences in horizontal semicircular canals gain values between the head impulse paradigm and suppression head impulse paradigm. Sixty healthy adult subjects aged 22-76-year-old (mean ± standard deviation=47.27 ± 18.29) participated in the head impulse paradigm and suppression head impulse paradigm using the video head impulse test. The Head impulse paradigm was used to assess all 6 semicircular canals, while suppression head impulse paradigm measured only the horizontal canals. Twenty subjects aged 22-40-year-old (25.25 ± 4.9) underwent a second session for the test-retest reliability. There were good test-retest reliability for both measures (right horizontal head impulse paradigm, intraclass correlation coefficient=0.80; left horizontal head impulse paradigm, intraclass correlation coefficient=0.77; right anterior head impulse paradigm, intraclass correlation coefficient=0.86; left anterior head impulse paradigm, intraclass correlation coefficient=0.78; right posterior head impulse paradigm, intraclass correlation coefficient=0.78; left posterior head impulse paradigm, intraclass correlation coefficient=0.75; right horizontal suppression head impulse paradigm, intraclass correlation coefficient=0.76; left horizontal suppression head impulse paradigm, intraclass correlation coefficient=0.79). The test-retest reliability for suppression head impulse paradigmanti-compensatory saccade latency and amplitude were moderate (right latency, intraclass correlation coefficient=0.61; left latency, intraclass correlation coefficient=0.69; right amplitude, intraclass correlation coefficient=0.69; left amplitude, intraclass correlation coefficient=0.58). There were no significant effects of age on head impulse paradigm and suppression head impulse paradigm vestibulo-ocular reflex gain values and suppression head impulse paradigmsaccade latency. However, the saccade amplitude became smaller with increasing age, P < .001. The horizontal suppression head impulse paradigm vestibuloocular reflex gain values were significantly lower than the head impulse paradigm for both sides (right, P = .004; left, P = .004). There was good test-retest reliability for both measures, and the gain values stabilized with age. However, suppression head impulse paradigm anti-compensatory saccade latency and amplitude had lower test-retest reliability than the gain. The suppression head impulse paradigm vestibulo-ocular reflex gain was lower than the head impulse paradigm and its anti-compensatory saccade amplitude reduced with increasing age.

Suppression of Vestibulo-Ocular Reflex With Increased Mental Workload While Driving

Suppression of Vestibulo-Ocular Reflex With Increased Mental Workload While Driving

Visual suppression of vestibulo-ocular reflex in patients treated with carotid artery revascularization: A potential biomarker for cerebral perfusion

BackgroundCarotid endarterectomy (CEA) and carotid artery stenting (CAS) are common treatments for ischemic stroke prevention in patients with carotid artery stenosis. However, the beneficial effects of CEA/CAS for cerebral hypoperfusion due to carotid artery stenosis have yet to be fully established. As dizziness is a common symptom in patients with carotid artery stenosis, we aimed to evaluate the effects of CEA/CAS on cerebral function in patients with carotid artery stenosis, using equilibrium function tests. MethodsThis prospective observational cohort study included 50 patients who had carotid artery stenosis and were scheduled to undergo CEA or CAS. Before CEA/CAS, we quantitatively evaluated the vestibulo-ocular reflex (VOR) and vestibular evoked myogenic potential (VEMP), as indicators of brainstem/inner ear functions related to balance, and visual suppression of VOR, as an indicator of cerebral control over the brainstem reflex related to balance. These were then compared with supratentorial cerebral blood flow (CBF). Changes in VOR, VEMP, visual suppression of VOR, CBF, and dizziness after CEA/CAS were also evaluated. ResultsThe visual suppression rates of VOR correlated with supratentorial CBFs before CEA/CAS (correlation coefficient = 0.47, p = 0.003). The visual suppression rates of VOR (p < 0.001) and supratentorial CBFs (p = 0.003) were improved by CEA/CAS, while VOR and VEMP did not change. Symptoms of dizziness improved after CEA/CAS. ConclusionsOur results suggest that visual suppression of the VOR may be a novel and practical marker for the beneficial effects of CEA/CAS on supratentorial cerebral function.

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases.

The key to the diagnosis of ocular motor disorders is a systematic clinical examination of the different types of eye movements, including eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. This is like a window which allows you to look into the brain stem and cerebellum even if imaging is normal. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum and rarely the cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal eye movements in the pons. For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), with impaired vertical saccades only or vertical gaze-evoked nystagmus due to dysfunction of the Interstitial nucleus of Cajal (INC). Lesions of the lateral medulla oblongata (Wallenberg syndrome) lead to typical findings: ocular tilt reaction, central fixation nystagmus and dysmetric saccades. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades. The time course of the development of symptoms and signs is important for the diagnosis of underlying diseases: acute: most likely stroke; subacute: inflammatory diseases, metabolic diseases like thiamine deficiencies; chronic progressive: inherited diseases like Niemann-Pick type C with typically initially vertical and then horizontal saccade palsy or degenerative diseases like progressive supranuclear palsy. Treatment depends on the underlying disease. In this article, we deal with central ocular motor disorders. In a second article, we focus on clinically relevant types of nystagmus such as downbeat, upbeat, fixation pendular, gaze-evoked, infantile or periodic alternating nystagmus. Therefore, these types of nystagmus will not be described here in detail.

Ocular Motor Abnormalities in Anti-IgLON5 Disease.

ObjectiveAnti-IgLON5 disease forms an interface between neuroinflammation and neurodegeneration and includes clinical phenotypes that are often similar to those of neurodegenerative diseases. An early diagnosis of patients with anti-IgLON5 disease and differentiation from neurodegenerative diseases is necessary and may have therapeutic implications.MethodsIn our small sample size study we investigated oculomotor function as a differentiating factor between anti-IgLON5 disease and neurodegenerative disorders. We examined ocular motor and vestibular function in four patients suffering from anti-IgLON5 disease using video-oculography (VOG) and a computer-controlled rotational chair system (sampling rate 60 Hz) and compared the data with those from ten age-matched patients suffering from progressive supranuclear palsy (PSP) and healthy controls (CON).ResultsPatients suffering from anti-IgLON5 disease differed from PSP most strikingly in terms of saccade velocity and accuracy, the presence of square wave jerks (SWJ) (anti-IgLON5 0/4 vs. PSP 9/10) and the clinical finding of supranuclear gaze palsy (anti-IgLON5 1/4). The presence of nystagmus, analysis of smooth pursuit eye movements, VOR and VOR suppression was reliable to differentiate between the two disease entities. Clear differences in all parameters, although not always significant, were found between all patients and CON.DiscussionWe conclude that the use of VOG as a tool for clinical neurophysiological assessment can be helpful in differentiating between patients with PSP and patients with anti-IgLON5 disease. VOG could have particular value in patients with suspected PSP and lack of typical Parkinson’s characteristics. future trials are indispensable to assess the potential of oculomotor function as a biomarker in anti-IgLON5 disease.

Influence of Tasking During Vestibular Testing.

Purpose The purpose of this study was to investigate the effectiveness of different types of tasking on the measurement of peak slow phase velocity (SPV) for caloric testing and rotary chair testing. Method This study evaluated the peak SPV response for caloric testing and rotary chair across five conditions. Three verbal, one tactile, and one condition without tasking were used for both caloric testing and rotary chair. The subjects consisted of 20 young adults (age range: 22-33 years, M = 26.65, SD = 3.72; seven male, 13 female) with normal vestibular function and no history of ear surgery or vestibular disorder. Study participation consisted of two visits with 24 hr minimum between each, one for caloric testing and one for rotary chair testing. The test completed at each visit was counterbalanced. Caloric Testing: The caloric irrigations were performed 5 times, with the ears randomized and tasking conditions randomized. Rotary Chair Testing: Rotary chair sinusoidal harmonic acceleration testing was performed 5 times at 0.08 Hz with the tasking conditions randomized. Results Tasking of any kind resulted in significantly larger peak SPV responses when compared to the no tasking condition for rotary chair testing. When comparing each type of tasking, no significant differences were noted. No significant difference was noted when comparing the conditions with tasking to the no tasking condition for caloric testing. Conclusions Clinically, either mental or tactile tasking can be utilized as a method to reduce VOR suppression during rotary chair testing. As no difference was found when comparing different verbal tasks to each other, the type of tasking can be catered to the patient. If verbal tasking cannot be completed, the braiding tactile task is a valid substitution. Caloric results varied widely across subjects and did not reach statistical significance, so conclusions on the need for tasking cannot be drawn.

Telemedicine Evaluations in Neuro-Ophthalmology During the COVID-19 Pandemic: Patient and Physician Surveys.

The novel coronavirus 2019 (COVID-19) pandemic has transformed health care. With the need to limit COVID-19 exposures, telemedicine has become an increasingly important format for clinical care. Compared with other fields, neuro-ophthalmology faces unique challenges, given its dependence on physical examination signs that are difficult to elicit outside the office setting. As such, it is imperative to understand both patient and provider experiences to continue to adapt the technology and tailor its application. The purpose of this study is to analyze both neuro-ophthalmology physician and patient satisfaction with virtual health visits during the time of the COVID-19 pandemic. Across three institutions (NYU Langone Health, Indiana University Health, and Columbia University Medical Center), telemedicine surveys were administered to 159 patients. Neuro-ophthalmologists completed 157 surveys; each of these were linked to a single patient visit. Patient surveys consisted of 5 questions regarding visit preparation, satisfaction, challenges, and comfort. The physician survey included 4 questions that focused on ability to gather specific clinical information by history and examination. Among 159 patients, 104 (65.4%) reported that they were satisfied with the visit, and 149 (93.7%) indicated that they were comfortable asking questions. Sixty-eight (73.9%) patients found the instructions provided before the visit easy to understand. Potential areas for improvement noted by patients included more detailed preparation instructions and better technology (phone positioning, Internet connection, and software). More than 87% (137/157) of neuro-ophthalmologists surveyed reported having performed an examination that provided enough information for medical decision-making. Some areas of the neuro-ophthalmologic examination were reported to be easy to conduct (range of eye movements, visual acuity, Amsler grids, Ishihara color plates, and pupillary examination). Other components were more difficult (saccades, red desaturation, visual fields, convergence, oscillations, ocular alignment, and smooth pursuit); some were especially challenging (vestibulo-ocular reflex [VOR], VOR suppression, and optokinetic nystagmus). Clinicians noted that virtual health visits were limited by patient preparation, inability to perform certain parts of the examination (funduscopy and pupils), and technological issues. Among virtual neuro-ophthalmology visits evaluated, most offer patients with appointments that satisfy their needs. Most physicians in this cohort obtained adequate clinical information for decision-making. Even better technology and instructions may help improve aspects of virtual health visits.

Effects of parameters of video head impulse testing on visually enhanced vestibulo-ocular reflex and vestibulo-ocular reflex suppression

ObjectiveTo investigate the main effects of some testing and analysis variables on clinically quantified visually enhanced vestibulo-ocular reflex (VVOR) and vestibulo-ocular reflex suppression (VORS) results using video head impulse test. MethodsThis prospective observational clinical study included 19 healthy participants who underwent the VVOR and VORS tests. The effect of demographic variables, head oscillation frequency, rotation direction, visual acuity and analysis time window width and location of the recorded tests on the quantified results of both VVOR and VORS were evaluated. And specifically, for the VORS test the effect of cognitive reinforcement of the participant during testing was evaluated. ResultsA statistically significant difference was observed among the VVOR, non-reinforced VORS, and reinforced VORS tests for mean gain values of 0.91 ± 0.09, 0.6 ± 0.15, and 0.57 ± 0.16, respectively (p < 0.001). The optimized linear mixed-effect model showed a significant influence of frequency on the gain values for the reinforced and non-reinforced VORS tests (p = 0.01 and p = 0.004, respectively). Regarding the gain analysis method, statistically significant differences were found according to the short time interval sample location of the records for the initial location of the VVOR test (p < 0.006) and final location of the reinforced VORS test (p < 0.023). ConclusionSignificant differences were observed in the gain values according to VVOR and VORS testing. Head oscillation frequency is a significant factor that affects the gain values, especially in VORS testing. Moreover, in VORS testing, participant concentration has a significant effect on the test for obtaining suppression gain values. When a short time interval sample is considered for VVOR and VORS testing, intermediate time samples appear the most adequate for both tests. SignificanceThe quantified visually enhanced vestibulo-ocular reflex (VVOR) and vestibulo-ocular reflex suppression (VORS) tests have recently been added to the assortment of available clinical vestibular tests. However, despite the clinical validity of these quantified tests that appear to be of increasing clinical interest, the effects of most of the clinical testing methods and mathematical variables are not well defined.In this research we describe what are the main collecting and analysis variables that could influence to the VVOR and VORS tests. Specially for VORS test, participant concentration on test tasks will have positive effect on the measured vestibulo-ocular reflex (VOR) suppression.

Effect of Intensity Level and Speech Stimulus Type on the Vestibulo-Ocular Reflex.

Accurate vestibulo-ocular reflex (VOR) measurement requires control of extravestibular suppressive factors such as visual fixation. Although visual fixation is the dominant suppressor and has been extensively studied, the mechanisms underlying suppression from nonvisual factors of attention and auditory stimulation are less clear. It has been postulated that the nonvisual suppression of the VOR is the result of one of two mechanisms: (1) activation of auditory reception areas excites efferent pathways to the vestibular nuclei, thus inhibiting the VOR or (2) cortical modulation of the VOR results from directed attention, which implies a nonmodality-specific process. The purpose of this research was to determine if the VOR is affected by the intensity level and/or type of speech stimulus. A repeated measures design was used. The experiment was single-blinded. Participants included 17 adults (14 females, three males) between the ages of 18-34 years who reported normal oculomotor, vestibular, neurological, and musculoskeletal function. Each participant underwent slow harmonic acceleration testing in a rotational chair. VOR gain was assessed at 0.02, 0.08, and 0.32 Hz in quiet (baseline). VOR gain was also assessed at each frequency while a forward running speech stimulus (attentional) or a backward running speech stimulus (nonattentional) was presented binaurally via insert earphones at 42, 62, and 82 dBA. The order of the conditions was randomized across participants. VOR difference gain was calculated as VOR gain in the auditory condition minus baseline VOR gain. To evaluate auditory efferent function, the medial olivocochlear reflex (MOCR) was assayed using transient-evoked otoacoustic emissions (right ear) measured in the presence and absence of broadband noise (left ear). Contralateral acoustic reflex thresholds were also assessed using a broadband noise elicitor. A three-way repeated measures analysis of variance was conducted to evaluate the effect of frequency, intensity level, and speech type on VOR difference gain. Correlations were conducted to determine if difference gain was related to the strength of the MOCR and/or to the acoustic reflex threshold. The analysis of variance indicated that VOR difference gain was not significantly affected by the intensity level or the type of speech stimulus. Correlations indicated VOR difference gain was not significantly related to the strength of the MOCR or the acoustic reflex threshold. The results were in contrast to previous research examining the effect of auditory stimulation on VOR gain as auditory stimulation did not produce VOR suppression or enhancement for most of the participants. Methodological differences between the studies may explain the discrepant results. The removal of an acoustic target from space to attend to may have prevented suppression or enhancement of the VOR. Findings support the hypothesis that VOR gain may be affected by cortical modulation through directed attention rather than due to activation of efferent pathways to the vestibular nuclei.

Corrective Saccades in Unilateral and Bilateral Vestibular Hypofunction During Slow Rotation Expressed by Visually Enhanced VOR and VOR Suppression: Role of the Cerebellum.

In clinical practice, the head impulse test paradigm (HIMP) and the suppression head impulse paradigm (SHIMP) stimulate high-frequency head movements so that the visual system is temporarily suppressed. The two tests could also be useful tools for vestibular assessment at low frequencies: VVOR (visually enhanced vestibulo-ocular reflex) and VORS (vestibulo-ocular reflex suppression). The aim of this study is to analyze the eye movements typically found during VVOR and VORS testing in patients with unilateral and bilateral vestibular hypofunction. Twenty patients with unilateral vestibular hypofunction, three patients with bilateral vestibular hypofunction, and ten patients with normal vestibular function (control group) were analyzed through VVOR and VORS testing with an Otometrics ICS Impulse system. During the VVOR test, patients with unilateral vestibular hypofunction exhibited corrective saccades to the same direction of the nystagmus fast phase toward the healthy side when the head rotates toward the affected side, while patients with bilateral vestibular hypofunction exhibited corrective saccades to the opposite side of head movements to each side. During the VORS test, patients with unilateral vestibular hypofunction seem to exhibit larger corrective saccades to the healthy side when the head was moved to this side, while patients with bilateral vestibular hypofunction did not exhibit corrective saccades during head movements to either side. Our data suggest that the VVOR and VORS tests yield the same diagnostic information as the HIMP and SHIMP tests in unilateral and bilateral vestibular hypofunction, and can contribute to the diagnosis of a peripheral vestibular loss as well as the affected side.

Teaching Video NeuroImages: Cerebellar esotropia

A 17-year-old girl presented with slowly progressive binocular horizontal diplopia, worse for distance viewing. She had distance esotropia (video), gaze-evoked nystagmus, broken smooth pursuit, reduced horizontal optokinetic nystagmus, and impaired vestibulo-ocular reflex suppression (VORS; video), in keeping with cerebellar dysfunction. The patient had full abduction during monocular viewing and head rotations, and normal saccadic velocities, excluding a bilateral sixth nerve palsy. An MRI scan confirmed a type 1 Chiari malformation (figure). A careful oculomotor assessment in patients with esotropia, including bedside VORS,1 may identify cerebellar signs that would suggest the tropia is cerebellar in origin.2 Cerebellar esotropia may arise from dorsal vermis impairment,2 or possibly from floccular and parafloccular dysfunction, explaining the associated abnormalities in smooth pursuit, optokinetic nystagmus, and VORS that share common neural pathways.

Superficial Siderosis of the Central Nervous System: Neurotological Findings Related to Magnetic Resonance Imaging.

To compare the neurotological results of five patients suffering from progressive hearing loss and ataxia due to superficial siderosis (SS) with the magnetic resonance imaging (MRI) findings. Retrospective case review. Primary and hospital care center. Five adult patients with neurotological symptoms of SS underwent MRI with acquisition of our temporal bone protocol including 3D-constructive interference in steady state (3D-CISS) and susceptibility-weighted imaging (SWI). All patients underwent a complete neurotological examination, the results of which were compared with the imaging findings. Cochleovestibular deficits were present in all five patients as determined by uni- or bilateral bithermal caloric testing and/or video head impulse tests. Sacculocollic reflex was present with increased P1 and N1 latencies on both sides in all patients. MRI revealed an extensive hypointense SWI signal outlining the surface of the brain and the VIIIth cranial nerve in all five patients. Desynchronization of the brainstem auditory evoked potentials (BAEP) and partial or complete absence of the visual suppression of vestibulo-ocular reflex during the pendular rotatory test was particularly consistent with the lesions of the cochleovestibular nerves as well as the cerebellar atrophy seen on MRI. The MRI results with SWI were related to neurotological findings in patients suffering from sensorineural deafness with ataxia due to SS. Our findings support the integration of the SWI and 3D-CISS sequences into the MRI protocol for all patients referred for evaluation of the extent of SS.

Pruebas de VVOR y VORS como herramientas de diagnóstico para la hipofunción vestibular unilateral y bilateral

Background: In clinical practice, tests such as the head impulse test paradigm (HIMP) and suppression head impulse paradigm (SHIMP) stimulate high-frequency head movements so the visual and somatosensory system are somehow suppressed. In low frequencies, two tests could be useful tools for vestibular assessment: VVOR (visually enhanced vestibulo-ocular reflex) and VORS (vestibulo-ocular reflex suppression). The aim of this study is to explain the eye movements typically found during VVOR and VORS testing in patients with unilateral and bilateral vestibular hypofunction. Methods: Ten patients with unilateral vestibular hypofunction, two patients with bilateral vestibular hypofunction, and ten patients without vestibular symptoms (control group) were analyzed retrospectively through VVOR and VORS testing in an Otometrics ICS Impulse system. Results: In the VVOR test, patients with unilateral vestibular hypofunction exhibited catch-up saccades beating to the healthy side when moving the head to the affected side, while patients with bilateral vestibular hypofunction exhibited catch-up saccades beating to the opposite side of head movement. In the VORS test, patients with unilateral vestibular hypofunction exhibited catch-up saccades to the healthy side when moving the head to this side, while patients with bilateral vestibular hypofunction did not exhibit catch-up saccades during head movement to either side. Conclusion: Our data suggest that the VVOR and VORS tests yield the same findings as the HIMP and SHIMP tests in unilateral and bilateral vestibular hypofunction, and can contribute to confirming peripheral etiology as well as the affected side.

Enhanced vestibulo-ocular reflex suppression in dancers during passive high-velocity head impulses.

The vestibulo-ocular reflex (VOR) is responsible for stabilizing images on the fovea during head movements. However, in some situations, one needs to suppress the VOR to be able to follow a target moving along with the head. Evidence suggests that the visual mechanism underlying VOR suppression can be modulated by experience. Unfortunately, the non-visual mechanism underlying VOR suppression has never been examined in dancers and, consequently, it is still unsure whether dance training can enhance eye-head tracking accuracy. The goal of the present study was to look at the influence of dance training on the VOR suppression during passive head impulses. Twenty-four individuals participated, 12 controls and 12 dancers. VOR and VOR suppression were assessed using a head impulse paradigm as well as a suppression head impulse test paradigm (SHIMP) with video head impulse test, respectively. The results suggest that dancers display a significantly reduced VOR gain during the SHIMP at 60ms in comparison to controls. Moreover, dancers with more than 10years of dance training exhibited a significantly reduced VOR gain during the SHIMP at 60ms. Overall, the results suggest that dance training improves VOR suppression, but also modulates VOR suppression abilities. Although studies are needed to shed light on the possible mechanisms involved in the modulation of the VOR gain, the observed changes in dancers' vestibulo-cerebellum and its role in the modulation of the VOR gain makes the cerebellar-vestibular nuclei pathway a possible model to explain the present results.

Inaccurate Saccades and Enhanced Vestibulo-Ocular Reflex Suppression during Combined Eye-Head Movements in Patients with Chronic Neck Pain: Possible Implications for Cervical Vertigo.

The primate ocular motor system is designed to acquire peripheral targets of interest by coordinating visual, vestibular, and neck muscle activation signals. The vestibulo-ocular reflex (VOR) is greatly reduced at the onset of large eye-head (gaze) saccades and resumes before the end of the saccades to stabilize eye-in-orbit and ensure accurate target acquisition. Previous studies have relied on manipulating head movements in normal individuals to study VOR suppression and gaze kinematics. We sought to determine if reduced head-on-trunk movement alters VOR suppression and gaze accuracy similar to experiments involving normal subjects and if intentionally increasing head and neck movement affects these dynamics. We measured head and gaze movements using magnetic search coil oculography in eight patients with cervical soft tissue disorders and seven healthy subjects. All participants made horizontal head-free saccades to acquire a laser dot target that stepped pseudorandomly 30-65° to either side of orbital mid-position, first using typical head and eye movements and again after being instructed to increase head amplitudes as much as possible. Compared to healthy subjects, patients made smaller head movements that contributed only 6% to total gaze saccade amplitudes. Head movements were also slowed, prolonged, and delayed. VOR suppression was increased and prolonged. Gaze saccades were inaccurate and delayed with long durations and decreased peak velocities. In patients with chronic neck pain, the internal commands issued for combined eye-head movements have large enough amplitudes to create accurate gaze saccades; however, because of increased neck stiffness and viscosity, the head movements produced are smaller, slower, longer, and more delayed than they should be. VOR suppression is disproportionate to the size of the actual gaze saccades because sensory feedback signals from neck proprioceptors are non-veridical, likely due to prolonged coactivation of cervical muscles. The outcome of these changes in eye-head kinematics is head-on-trunk stability at the expense of gaze accuracy. In the absence of vestibular loss, the practical consequences may be dizziness (cervical vertigo) in the short term and imbalance and falls in the long term.