Visually Enhanced Vestibulo-ocular Reflex Research Articles

Visually enhanced vestibulo-ocular reflex gain in patients with vestibular disease.

Vestibular migraine (VM) is a diagnostic challenge. Visually enhanced vestibulo-ocular reflex (VVOR) gain, a measure of the visual-vestibular interaction, has been proposed as a tool for diagnosing VM. This study seeks to evaluate VVOR gain's diagnostic capability to predict VM and to compare the phenotypes of vestibular patients with elevated versus normal/low VVOR gain. A retrospective review of consecutive adult patients at a dizziness clinic from October 2016 and December 2020 was conducted. VVOR gain's diagnostic performance was assessed with the area under the receiver operating characteristic (AUROC) analysis. Demographic factors and clinical presentations were compared between vestibular patients with elevated versus normal/low VVOR gain. One hundred forty patients (70 with VM) were analyzed. VVOR gain was elevated in 68.6% of patients with VM, compared to 52.9% of patients without VM (p = .057). The AUROC of VVOR gain was 0.5902 (95% confidence interval: 0.4958-0.6846). Vestibular patients with elevated VVOR gain were younger than those with normal/low VVOR gain (mean age 50 vs. 62, p < .0001). A higher proportion of subjects with elevated VVOR gain had symptoms triggered by certain foods (17.6% vs. 5.5%, p = .040) and experienced sound sensitivity (34.1% vs. 18.2%, p = .040) and motion sensitivity (23.5% vs. 9.1%, p = .041). A greater proportion of VM patients with elevated VVOR gain were triggered by certain foods (27.1% vs. 0%, p = .006). VVOR gain alone has limited ability to discriminate VM from other vestibular conditions and must be interpreted carefully. VVOR gain elevation may be associated with food triggers and motion and sound sensitivity. IV.

A New Method for Gain Calculation in Quantified Visuo-vestibular Interaction Test.

To develop a new method to quantify visually-enhanced vestibulo-ocular reflex (VVOR) gain, in patients with vestibular function loss, that is mathematically suitable given the nature of the test, and determine the reliability of the method by comparing results with those of the gold standard, the video head impulse test (vHIT). We developed a new method for VVOR gain quantification and conducted a cross-sectional study in patients diagnosed with vestibular function loss and controls, all participants undergoing both a VVOR test and a vHIT. We measured VVOR gain with three different methods: area under the curve (AUC), slope regression, and a Fourier method (VVORAUC , VVORSP , and VVORFR , respectively); and compared these gain values with vHIT gain calculated using the AUC method. Overall, 111 patients were included: 29 healthy subjects and 82 patients with vestibular function loss. Intraclass correlation coefficients (ICC(1,1)) between gain from the gold standard and each of the VVOR gain methods were: 0.68 (CI: 0.61-0.75) for VVORAUC , 0.66 (CI: 0.58-0.73) for VVORSP and 0.71 (CI: 0.64-0.77) for VVORFR . No interference was found between VVOR gain calculation methods and potentially influential variables considered (p ≥ 0.98). The new method for quantifying VVOR gain showed good concordance with the vHIT method. 2: Individual cross-sectional studies with consistently applied reference standard and blinding (Diagnosis) Laryngoscope, 133:3554-3563, 2023.

Effectiveness of vestibular rehabilitation therapy in patients with idiopathic Cerebellar Ataxia with Bilateral Vestibulopathy (iCABV).

To evaluate the benefit of vestibular rehabilitation therapy (VRT) in the management of patients with idiopathic cerebellar ataxia with bilateral vestibulopathy (iCABV). iCABV is a hindbrain degenerative disorder with impairment of both central and peripheral vestibular pathways. There is combined failure of four compensatory eye movement systems including the vestibulo-ocular reflex (VOR), optokinetic reflex, smooth pursuit and the visually enhanced vestibulo-ocular reflex (VVOR). Phenotypic presentation includes postural and gait instability, oscillopsia and dizziness with active head movement. The benefit of VRT in iCABV patients has not been established. A retrospective review was performed on a cohort of twelve patients diagnosed with iCABV in a multidisciplinary neuro-otology clinic. All participated in VRT and completed their suggested course of VRT. The following clinical measures were assessed before starting and after finishing VRT: 1) Dizziness Handicap Inventory (DHI), 2) Activities-Specific Balance Confidence (ABC) Scale, 3) Catastrophization scale, 4) Positive Affective Negative Affective Score (PANAS), 5) Dynamic Gait Index (DGI) and 6) Modified Clinical Test of Sensory Interaction in Balance (mCTSIB). The number of falls historically was recorded in addition to gait speed (ft./sec). Following VRT, patients were found to have improved balance on mCTSIB (condition 4 : 7 vs 18 seconds, P = 0.04) and a better postural stability with a reduced number of falls (p = 0.01). No statistically significant improvement was seen in the DHI, ABC, Catastrophization scale, DGI, PANAS and gait speed (p > 0.05). iCABV patients who underwent VRT were found to have a better postural stability and reduced risk of falls. VRT was not found to significantly improve patients' overall subjective perception of their symptoms or their psychological status.

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.

VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words "bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy." Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

Perioperative vestibular assessment and testing

Vestibular disorders can be difficult to accurately diagnose and manage. A careful history and focused physical examination are typically adequate to establish a diagnosis and initiate medical treatment. Vestibular testing is an important component of the work-up, but it is particularly essential for patients being considered for surgical intervention for a vestibular disorder, where the testing can be used to more definitively confirm a suspected diagnosis and to determine baseline vestibular organ function. In this article, we will first briefly review key components of the history and physical examination of patients with vestibular complaints. We will then discuss the most commonly used vestibular tests and their role in the preoperative assessment of patients undergoing vestibular surgery, including nystagmography, caloric testing, rotary chair testing, video head impulse testing, and vestibular evoked myogenic potential testing.

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.

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.

Mathematical Methods for Measuring the Visually Enhanced Vestibulo-Ocular Reflex and Preliminary Results from Healthy Subjects and Patient Groups.

Visually enhanced vestibulo-ocular reflex (VVOR) is a well-known bedside clinical test to evaluate visuo-vestibular interaction, with clinical applications in patients with neurological and vestibular dysfunctions. Owing to recently developed diagnostic technologies, the possibility to perform an easy and objective measurement of the VVOR has increased, but there is a lack of computational methods designed to obtain an objective VVOR measurement. To develop a method for the assessment of the VVOR to obtain a gain value that compares head and eye velocities and to test this method in patients and healthy subjects. Two computational methods were developed to measure the VVOR test responses: the first method was based on the area under curve of head and eye velocity plots and the second method was based on the slope of the linear regression obtained for head and eye velocity data. VVOR gain and vestibulo-ocular reflex (VOR) gain were analyzed with the data obtained from 35 subjects divided into four groups: healthy (N = 10), unilateral vestibular with vestibular neurectomy (N = 8), bilateral vestibulopathy (N = 12), and cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) (N = 5). Intra-class correlation index for the two developed VVOR analysis methods was 0.99. Statistical differences were obtained by analysis of variance statistical method, comparing the healthy group (VVOR mean gain of 1 ± 0) with all other groups. The CANVAS group exhibited (VVOR mean gain of 0.4 ± 0.1) differences when compared to all other groups. VVOR mean gain for the vestibular bilateral group was 0.8 ± 0.1. VVOR mean gain in the unilateral group was 0.6 ± 0.1, with a Pearson's correlation of 0.52 obtained when VVOR gain was compared to the VOR gain of the operated side. Two computational methods to measure the gain of VVOR were successfully developed. The VVOR gain values appear to objectively characterize the VVOR alteration observed in CANVAS patients, and also distinguish between healthy subjects and patients with some vestibular disorders.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a novel ataxic disorder consisting of the triad of cerebellar impairment, bilateral vestibular hypofunction, and a somatosensory deficit. We report the first Japanese case of CANVAS. The patient is a 68-year-old Japanese male. He was referred to our university for further evaluation of progressive gait disturbance and ataxia. He exhibited horizontal gaze-evoked nystagmus and sensory deficit. Nerve conduction studies showed sensory neuronopathy. Magnetic resonance imaging showed the atrophy of vermis but not of the brainstem. The caloric stimulation and video head impulse test (vHIT) showed bilateral vestibulopathy. The visually enhanced vestibulo-ocular reflex (VVOR) was also impaired. In addition to neurological and electrophysiological examinations, simple neuro-otological examinations (i.e., caloric stimulation, vHIT, and VVOR) may reveal more non-Caucasian cases.

Superposition Violations in the Compensatory Eye Movement System.

Compensatory eye movements (CEM) maintain a stable image on the retina by minimizing retinal slip. The optokinetic reflex (OKR) and vestibulo-ocular reflex (VOR) compensate for low and high velocity stimuli, respectively. The OKR system is known to be highly nonlinear. The VOR is generally modeled as a linear system and assumed to satisfy the superposition and homogeneity principles. To probe CEM violation of the superposition principle, we recorded eye movement responses in C57BL/6 mice to sum of sine (SoS) stimulation, a combination of multiple nonharmonic inputs. We tested the VOR, OKR, VVOR (visually enhanced VOR), and SVOR (suppressed VOR). We used stimuli containing 0.6 Hz, 0.8 Hz, 1.0 Hz, and 1.9 Hz. Power spectra of SoS stimuli did not yield distortion products. Gains and delays of SoS and single sine (SS) responses were compared to yield relative gains and delays. We find the superposition principle is violated primarily in the OKR, VOR, and SVOR conditions. In OKR, we observed relative gain suppression of the lower SoS stimulus frequency component irrespective of the absolute frequency. Conversely, SVOR and VOR results showed gain enhancement of the lower frequency component and overall decrease in lead. Visually enhanced VOR results showed trends for overall gain suppression and delay decrease. Compensatory eye movements arguably depend on predictive signals. These results may reflect better prediction for SS stimuli. Natural CEM system stimulation generally involves complex frequency spectra. Use of SoS stimuli is a step toward unravelling the signals that really drive CEM and the predictive algorithms they depend on.

The pivotal sign of CANVAS

A 75-year-old woman complained about insecure gait since age 55. Clinical examination revealed signs of cerebellar ataxia, bilateral vestibulopathy, and peripheral sensory impairment. Sensory nerve action potentials were absent. The visually enhanced vestibulo-ocular reflex (VVOR) was impaired (video on the Neurology(®) Web site at www.neurology.org, figure 1) and the diagnosis of cerebellar ataxia (figure 2) with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) was made.(1) CANVAS is considered to be a recessive disorder with a mean age at onset of 60 years.(2) VVOR impairment is its characteristic clinical sign.(2) It can only be elicited if both smooth-pursuit eye movements and the vestibulo-ocular reflex are deficient. Normally, both are redundant at low head velocities.(2.)

Clinico-pathological correlates in cerebellar ataxia with neuronopathy and vestibular areflexia syndrome (CANVAS)

Background: CANVAS is a novel vestibulo-cerebellar ataxia with characteristic clinical features, atrophy of the cerebellum and cranial and spinal nerve sensory ganglia. CANVAS is comprised of the clinical triad of cerebellar ataxia, bilateral vestibular hypofunction and a somatic sensory deficit, and is associated with an abnormal visually enhanced vestibulo-ocular reflex (VVOR).

Video-oculography in Mice

Eye movements are very important in order to track an object or to stabilize an image on the retina during movement. Animals without a fovea, such as the mouse, have a limited capacity to lock their eyes onto a target. In contrast to these target directed eye movements, compensatory ocular eye movements are easily elicited in afoveate animals1,2,3,4. Compensatory ocular movements are generated by processing vestibular and optokinetic information into a command signal that will drive the eye muscles. The processing of the vestibular and optokinetic information can be investigated separately and together, allowing the specification of a deficit in the oculomotor system. The oculomotor system can be tested by evoking an optokinetic reflex (OKR), vestibulo-ocular reflex (VOR) or a visually-enhanced vestibulo-ocular reflex (VVOR). The OKR is a reflex movement that compensates for "full-field" image movements on the retina, whereas the VOR is a reflex eye movement that compensates head movements. The VVOR is a reflex eye movement that uses both vestibular as well as optokinetic information to make the appropriate compensation. The cerebellum monitors and is able to adjust these compensatory eye movements. Therefore, oculography is a very powerful tool to investigate brain-behavior relationship under normal as well as under pathological conditions (f.e. of vestibular, ocular and/or cerebellar origin).Testing the oculomotor system, as a behavioral paradigm, is interesting for several reasons. First, the oculomotor system is a well understood neural system5. Second, the oculomotor system is relative simple6; the amount of possible eye movement is limited by its ball-in-socket architecture ("single joint") and the three pairs of extra-ocular muscles7. Third, the behavioral output and sensory input can easily be measured, which makes this a highly accessible system for quantitative analysis8. Many behavioral tests lack this high level of quantitative power. And finally, both performance as well as plasticity of the oculomotor system can be tested, allowing research on learning and memory processes9.Genetically modified mice are nowadays widely available and they form an important source for the exploration of brain functions at various levels10. In addition, they can be used as models to mimic human diseases. Applying oculography on normal, pharmacologically-treated or genetically modified mice is a powerful research tool to explore the underlying physiology of motor behaviors under normal and pathological conditions. Here, we describe how to measure video-oculography in mice8.

Video-oculography in Mice

Eye movements are very important in order to track an object or to stabilize an image on the retina during movement. Animals without a fovea, such as the mouse, have a limited capacity to lock their eyes onto a target. In contrast to these target directed eye movements, compensatory ocular eye movements are easily elicited in afoveate animals1,2,3,4. Compensatory ocular movements are generated by processing vestibular and optokinetic information into a command signal that will drive the eye muscles. The processing of the vestibular and optokinetic information can be investigated separately and together, allowing the specification of a deficit in the oculomotor system. The oculomotor system can be tested by evoking an optokinetic reflex (OKR), vestibulo-ocular reflex (VOR) or a visually-enhanced vestibulo-ocular reflex (VVOR). The OKR is a reflex movement that compensates for "full-field" image movements on the retina, whereas the VOR is a reflex eye movement that compensates head movements. The VVOR is a reflex eye movement that uses both vestibular as well as optokinetic information to make the appropriate compensation. The cerebellum monitors and is able to adjust these compensatory eye movements. Therefore, oculography is a very powerful tool to investigate brain-behavior relationship under normal as well as under pathological conditions (f.e. of vestibular, ocular and/or cerebellar origin). Testing the oculomotor system, as a behavioral paradigm, is interesting for several reasons. First, the oculomotor system is a well understood neural system5. Second, the oculomotor system is relative simple6; the amount of possible eye movement is limited by its ball-in-socket architecture ("single joint") and the three pairs of extra-ocular muscles7. Third, the behavioral output and sensory input can easily be measured, which makes this a highly accessible system for quantitative analysis8. Many behavioral tests lack this high level of quantitative power. And finally, both performance as well as plasticity of the oculomotor system can be tested, allowing research on learning and memory processes9. Genetically modified mice are nowadays widely available and they form an important source for the exploration of brain functions at various levels10. In addition, they can be used as models to mimic human diseases. Applying oculography on normal, pharmacologically-treated or genetically modified mice is a powerful research tool to explore the underlying physiology of motor behaviors under normal and pathological conditions. Here, we describe how to measure video-oculography in mice8.

Cerebellar Ataxia With Neuropathy and Bilateral Vestibular Areflexia Syndrome

Szmulewicz, David J.*; Merchant, Saumil N.†; Halmagyi, Gabor Michael‡ Author Information

The attenuation of perceived motion smear during combined eye and head movements

The extent of perceived motion smear was compared for targets that underwent similar velocities of retinal image motion during the vestibulo-ocular reflex (VOR) in the dark, the visually enhanced VOR (VVOR), VOR suppression, and fixation. Compared to the extent of perceived motion smear during fixation, observers reported significantly less smear when the target moved either in the same direction or against the direction of the head movement during the VVOR and VOR. We also confirmed a previous finding that perceived smear is attenuated asymmetrically during VOR suppression, with attenuation occurring primarily for targets that move against the direction of the observer’s head motion. The results support the hypothesis that the visual system employs extra-retinal signals that accompany eye and head movements to reduce the perception of motion smear for targets that move physically in the opposite direction of eye and/or head movements.

THE VESTIBULO-OCULAR REFLEX IN CHIARI TYPE II MALFORMATION

Background and Objectives: Chiari type II malformation (CII) is a congenital anomaly of the cerebellum and brainstem, both important structures for processing the vestibulo-ocular reflex (VOR). Little information is available on the VOR in CII. The study objectives were to determine VOR characteristics in darkness and during visual fixation (visually-enhanced VOR or VVOR) in CII.

Validity and limitation of manual rotational test to detect impaired visual–vestibular interaction due to cerebellar disorders

The aim of this study was to investigate validity and limitation of the novel infrared system to record and analyze horizontal visual–vestibular interaction using whole-body rotation rapidly and conveniently in the routine vestibular clinic. We examined 11 patients with cerebellar dysequilibrium and 25 patients with peripheral dysequilibrium for vestibulo-ocular reflex in darkness (DVOR), visually-enhanced vestibulo-ocular reflex (VEVOR), and fixation suppression of vestibulo-ocular reflex (FSVOR), and compared the results with those of examination for head-fixed smooth pursuit and fixation suppression during caloric stimulation. The manual rotation stimuli were 0.5–0.75 Hz in frequecny and 60–90°/s in maximal angular velocity. Gain of vestibulo-ocular reflex in darkness was not significantly correlated with maximal slow phase velocity (MSPV) of caloric-induced nystagmus at that stimulus condition either in patients with peripheral dysequilibrium or in those with cerebellar dysequilibrium. An index for fixation suppression of vestibulo-ocular reflex during rotation stimulus was significantly lower in patients with cerebellar dysequilibrium than in normal control subjects and those with peripheral dysequilbrium. On the other hand, there was no significant difference among the two disease groups and the normal control group in gain of visually-enhanced vestibulo-ocular reflex. In about a half of patients with cerebellar dysequilibrium, measured smooth pursuit gain was lower than estimated smooth pursuit gain calculated based on a simple superposition theory of vestibulo-ocular reflex and smooth pursuit. Testing fixation suppression using the present system is an unusually convenient tool for detection of cerebellar dysequilibrium.

Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign.

We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are still ambulant and self-caring 8-20 years after onset. The cause of CABV in our four patients is unknown. None has a family history of cerebellar or vestibular disease; spinocerebellar ataxia (SCA) 1, 2, 3, 6, 7 and Friedreich's ataxia were excluded by genetic testing. Three of the four have a sensory peripheral neuropathy but none has extrapyramidal or significant autonomic problems, and none has gluten sensitivity. We measured eye rotations in response to head-on-trunk head rotations and in response to head-and-trunk (en bloc) rotations. Horizontal smooth pursuit (SP), vestibulo-ocular reflex (VOR) and VVOR gains were measured in response to head rotations at 0.1, 0.3, 0.6 and 1.0 Hz. The optokinetic reflex (OKR) was tested by measuring optokinetic nystagmus slow phase velocity during constant 50 degrees /s rotation of the subject in light. The results showed that CABV patients had impairment of all three compensatory eye movement reflexes, the VOR, the OKR and SP. During VVOR testing, as the frequency of head rotation increased from 0.1 to 1.0 Hz, eye velocity failed to match head velocity, gaze velocity increased, and gaze position errors developed, which were corrected with bursts of saccades, the basis of the clinical sign of an impaired VVOR.