Cerebellar Dizziness and Vertigo: Etiologies, Diagnostic Assessment, and Treatment

2019 ◽  
Vol 40 (01) ◽  
pp. 087-096 ◽  
Author(s):  
Andreas Zwergal ◽  
Katharina Feil ◽  
Roman Schniepp ◽  
Michael Strupp
Keyword(s):  
Balance Training ◽  
Vestibular Function ◽  
Downbeat Nystagmus ◽  
Ocular Reflex ◽  
Assessment And Treatment ◽  
Gaze Holding ◽  
Risk Of Falls ◽  
Vestibulo Ocular Reflex ◽  
Cerebellar Ataxias ◽  
Vertical Nystagmus

AbstractCerebellar dizziness and vertigo account for approximately 10% of diagnoses in a tertiary dizziness center. This term summarizes a large group of disorders with chronic (degenerative, hereditary, acquired cerebellar ataxias), recurrent (episodic ataxias), or acute (stroke, inflammation) presentations. Key to the diagnosis is a comprehensive examination of central ocular motor and vestibular function. Patients with cerebellar dizziness and vertigo usually show a pattern of deficits in smooth pursuit, gaze-holding, saccade accuracy, or fixation-suppression of the vestibulo-ocular reflex. Central fixation nystagmus (e.g., downbeat nystagmus), gaze-evoked nystagmus, central positional nystagmus, or head-shaking nystagmus with cross-coupling (i.e., horizontal head shaking causing inappropriate vertical nystagmus) occurs frequently. Overlap syndromes with peripheral vestibular disorders, such as cerebellar ataxia, neuropathy, and vestibular areflexia, exist rarely. Posturography and gait analysis can contribute to diagnostic differentiation, estimation of the risk of falls, as well as quantification of progression and treatment effects. Patients with cerebellar dizziness and vertigo should receive multimodal treatment, including balance training, occupational therapy, and medication.

scholarly journals Clinical dynamic visual acuity in patients with cerebellar ataxia and vestibulopathy

PLoS ONE ◽  
2021 ◽  
Vol 16 (7) ◽  
pp. e0255299
Author(s):  
Michaela Dankova ◽  
Jaroslav Jerabek ◽  
Dylan J. Jester ◽  
Alena Zumrova ◽  
Jaroslava Paulasova Schwabova ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Vestibular Function ◽  
Head Impulse Test ◽  
Dynamic Visual Acuity ◽  
Bilateral Vestibulopathy ◽  
Caloric Irrigation ◽  
Ocular Reflex ◽  
Vestibulo Ocular Reflex ◽  
Cerebellar Ataxias ◽  
Central Adaptation

Deterioration of dynamic visual acuity (DVA) as a result of impaired vestibulo-ocular reflex (VOR) has been well described in peripheral vestibulopathies, however, changes in DVA in patients with degenerative cerebellar ataxias (CA) and its relation to VOR impairment in these patients has not yet been evaluated. Our aim was to assess the alterations of DVA in CA and to evaluate its relation to vestibular function. 32 patients with CA and 3 control groups: 13 patients with unilateral and 13 with bilateral vestibulopathy and 21 age matched healthy volunteers were examined by clinical DVA test, VOR was assessed by video Head Impulse Test and caloric irrigation. The severity of ataxia in CA was assessed by Scale for the assessment and rating of ataxia (SARA). Relationship between DVA and vestibular function in CA patients was examined by linear regressions. DVA impairment was highly prevalent in CA patients (84%) and its severity did not differ between CA and bilateral vestibulopathy patients. The severity of DVA impairment in CA was linked mainly to VOR impairment and only marginally to the degree of ataxia. However, DVA impairment was present also in CA patients without significant vestibular lesion showing that central mechanisms such as impairment of central adaptation of VOR are involved. We suggest that the evaluation of DVA should be a standard part of clinical evaluation in patients with progressive CA, as this information can help to target vestibular and oculomotor rehabilitation.

scholarly journals Finding a Balance: A Systematic Review of the Biomechanical Effects of Vestibular Prostheses on Stability in Humans

2020 ◽  
Vol 5 (2) ◽  
pp. 23
Author(s):  
Felix Haxby ◽  
Mohammad Akrami ◽  
Reza Zamani
Keyword(s):  
Vestibular System ◽  
Postural Sway ◽  
Vestibular Function ◽  
Vestibular Stimulation ◽  
Significant Heterogeneity ◽  
Vestibular Loss ◽  
Ocular Reflex ◽  
Gait Characteristics ◽  
Vestibulo Ocular Reflex ◽  
Meta Analyses

The vestibular system is located in the inner ear and is responsible for maintaining balance in humans. Bilateral vestibular dysfunction (BVD) is a disorder that adversely affects vestibular function. This results in symptoms such as postural imbalance and vertigo, increasing the incidence of falls and worsening quality of life. Current therapeutic options are often ineffective, with a focus on symptom management. Artificial stimulation of the vestibular system, via a vestibular prosthesis, is a technique being explored to restore vestibular function. This review systematically searched for literature that reported the effect of artificial vestibular stimulation on human behaviours related to balance, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) technique. A total of 21 papers matched the inclusion criteria of the literature search conducted using the PubMed and Web of Science databases (February 2019). The populations for these studies included both healthy adults and patients with BVD. In every paper, artificial vestibular stimulation caused an improvement in certain behaviours related to balance, although the extent of the effect varied greatly. Various behaviours were measured such as the vestibulo-ocular reflex, postural sway and certain gait characteristics. Two classes of prosthesis were evaluated and both showed a significant improvement in at least one aspect of balance-related behaviour in every paper included. No adverse effects were reported for prostheses using noisy galvanic vestibular stimulation, however, prosthetic implantation sometimes caused hearing or vestibular loss. Significant heterogeneity in methodology, study population and disease aetiology were observed. The present study confirms the feasibility of vestibular implants in humans for restoring balance in controlled conditions, but more research needs to be conducted to determine their effects on balance in non-clinical settings.

Comparison of Horizontal and Vertical Dynamic Visual Acuity in Patients with Vestibular Dysfunction and Nonvestibular Dizziness

2007 ◽  
Vol 18 (03) ◽  
pp. 236-244 ◽  
Author(s):  
Richard A. Roberts ◽  
Richard E. Gans
Keyword(s):  
Visual Acuity ◽  
Head Movement ◽  
Vestibular Function ◽  
Vestibular Dysfunction ◽  
Common Symptom ◽  
Dynamic Visual Acuity ◽  
Blurred Vision ◽  
Ocular Reflex ◽  
Vestibulo Ocular Reflex ◽  
Horizontal Head

Blurred vision with head movement is a common symptom reported by patients with vestibular dysfunction affecting the vestibulo-ocular reflex (VOR). Impaired VOR can be measured by comparing visual acuity in which there is no head movement to visual acuity obtained with head movement. A previous study demonstrated that dynamic visual acuity (DVA) testing using vertical head movement revealed deficits in impaired VOR. There is evidence that horizontal head movement is more sensitive to impaired VOR. The objective of this investigation was to compare horizontal and vertical DVA in participants with normal vestibular function (NVF), impaired vestibular function (IVF), and participants with nonvestibular dizziness (NVD). Participants performed the visual acuity task in a baseline condition with no movement and also in two dynamic conditions, horizontal head movement and vertical head movement. Horizontal DVA was twice as sensitive to impaired VOR than vertical DVA. Results suggest that horizontal volitional head movement should be incorporated into tasks measuring functional deficits of impaired VOR. Una visión borrosa con los movimientos de la cabeza es un síntoma común reportado por los pacientes con una disfunción vestibular que afecta el reflejo vestíbulo-ocular (VOR). La alteración en el VOR puede ser medida comparando la aguda visual no acompañada de movimientos de la cabeza, con la aguda visual obtenida con movimientos cefálicos. Un estudio previo demostró que la prueba de aguda visual dinámica (DVA) usando movimiento vertical de la cabeza revelaba deficiencias relacionados con un VOR alterado. Existe evidencia que el movimiento cefálico horizontal es más sensible a un VOR alterado. El objetivo de esta investigación fue comparar el DVA horizontal y vertical en participantes con funcional vestibular normal (NVF), con función vestibular alterada (IVF) y en sujetos con mareo no vestibular (NVD). Los participantes realizaron sus tareas de agudeza visual en una condición basal, sin movimiento, y también en dos condiciones dinámicas, con movimientos de cabeza horizontales y verticales. El DVA horizontal fue dos veces más sensible a un VOR alterado que el DVA vertical. Los resultados sugieren que los movimientos volitivos horizontales de la cabeza deben incorporarse en las tareas que midan deficiencias funcionales con un VOR alterado.

scholarly journals A – C.A.N.V.A.S (CEREBELLAR ATAXIA, NEURONOPATHY AND VESTIBULAR AREFLEXIA WITH AUTONOMIC DYSFUNCTION): A FASCINATING CLINICAL PORTRAIT

2018 ◽  
Vol 4 (3-4) ◽  
pp. 3-8
Author(s):  
Carlo Canepa-Raggio
Keyword(s):  
Cerebellar Ataxia ◽  
Autonomic Dysfunction ◽  
Cerebellar Atrophy ◽  
Sensory Neuropathy ◽  
Nerve Biopsy ◽  
Sural Nerve Biopsy ◽  
Downbeat Nystagmus ◽  
Charcot Marie Tooth Disease ◽  
Ocular Reflex ◽  
Vestibulo Ocular Reflex

57-year-old male patient with a 30-year history chronic cough, balance difficulties (most noticeable in the dark), ataxia and sensory neuropathy. There was also evidence of orthostatic hypotension and hypohydrosis. Examination revealed downbeat nystagmus, an abnormal visually-enhanced vestibulo-ocular reflex, length-dependent sensory neuropathy, high-stepping tandem ataxia and bilateral dysmetria. MRI brain shows marked vermian cerebellar atrophy (more noticeable in lobes VI and VIIa/b) and nerve conduction studies reveal absent sensory conductions (ganglionopathy). Genetic testing for Friedrich’s ataxia, spinocerebellar ataxia and hereditary motor sensory neuropathy (Charcot-Marie-Tooth disease) were all negative. Sural nerve biopsy showed pattern of severe loss of myelinated fibres. This patient was diagnosed with cerebellar ataxia, neuronopathy (ganglionopathy) and vestibular areflexia with autonomic dysfunction. Keywords: ataxia, neuronopathy, vestibular areflexia, autonomic dysfunction.

scholarly journals Relation Between Perception of Vertical Axis Rotation and Vestibulo-Ocular Reflex Symmetry

1992 ◽  
Vol 2 (1) ◽  
pp. 59-69
Author(s):  
Robert J. Peterka ◽  
Martha S. Benolken
Keyword(s):  
Measurement Errors ◽  
Rotational Velocity ◽  
Vestibular Function ◽  
Vertical Axis ◽  
Perceptual Bias ◽  
Common Source ◽  
Ocular Reflex ◽  
Small Constant ◽  
Vestibulo Ocular Reflex ◽  
Axis Rotation

Subjects seated in a vertical axis rotation chair controlled their rotational velocity by adjusting a potentiometer. Their goal was to null out pseudorandom rotational perturbations in order to remain perceptually stationary. Most subjects showed a slow linear drift of velocity (a constant acceleration) to one side when they were deprived of an earth-fixed visual reference. The amplitude and direction of this drift can be considered a measure of a static bias in a subject’s perception of rotation. The presence of a perceptual bias is consistent with a small, constant imbalance of vestibular function that could be of either central or peripheral origin. Deviations from perfect vestibulo-ocular reflex (VOR) symmetry are also assumed to be related to imbalances in either peripheral or central vestibular function. We looked for correlations between perceptual bias and various measures of vestibular reflex symmetry that might suggest a common source for both reflexive and perceptual imbalances. No correlations were found. Measurement errors could not account for these results since repeated tests in the same subjects of both perceptual bias and VOR symmetry were well correlated.

Ictal downbeat nystagmus in Ménière disease

Neurology ◽  
2020 ◽  
Vol 95 (17) ◽  
pp. e2409-e2417
Author(s):  
Sun-Uk Lee ◽  
Hyo-Jung Kim ◽  
Jeong-Yoon Choi ◽  
Ji-Soo Kim
Keyword(s):  
Semicircular Canals ◽  
Vestibular Evoked Myogenic Potentials ◽  
Slow Phase ◽  
Downbeat Nystagmus ◽  
Meniere Disease ◽  
Head Impulse ◽  
Slow Phase Velocity ◽  
Ocular Reflex ◽  
Vestibulo Ocular Reflex ◽  
Menière Disease

ObjectiveTo determine the mechanism of ictal downbeat nystagmus in Ménière disease (MD), we compared the head impulse gain of the vestibulo-ocular reflex (VOR) for each semicircular canal between patients with (n = 7) and without (n = 70) downbeat nystagmus during attacks of MD.MethodsWe retrospectively analyzed the results of video-oculography, video head-impulse tests, and cervical vestibular-evoked myogenic potentials (VEMPs) in 77 patients with definite MD who were evaluated during an attack.ResultsPure or predominant downbeat nystagmus was observed in 7 patients (9%) with unilateral MD during the attacks. All 7 patients showed spontaneous downbeat nystagmus without visual fixation with a slow phase velocity ranging from 1.5 to 11.2°/s (median 5.4, interquartile range 3.7–8.5). All showed a transient decrease of the head impulse VOR gains for the posterior canals (PCs) in both ears (n = 4) or in the affected ear (n = 3). Cervical VEMPs were decreased in the affected (n = 2) or both ears (n = 2) when evaluated during the attacks. Downbeat nystagmus disappeared along with normalization of the VOR gains for PCs after the attacks in all patients. During the attacks, the head impulse VOR gains for the PC on the affected side were lower in the patients with ictal downbeat nystagmus than in those without (Mann-Whitney U test, p < 0.001), while the gains for other semicircular canals did not differ between the groups.ConclusionDownbeat nystagmus may be observed during attacks of MD due to an asymmetry in the vertical VOR or saccular dysfunction. MD should be considered in recurrent audiovestibulopathy and ictal downbeat nystagmus.

Recovery of vestibulo-ocular reflex-function in subjects with an acute unilateral peripheral vestibular deficit

1999 ◽  
Vol 9 (2) ◽  
pp. 135-144 ◽  
Author(s):  
J.H.J. Allum ◽  
T. Ledin
Keyword(s):  
Low Frequency ◽  
Vestibular Function ◽  
Vertical Axis ◽  
Whole Body ◽  
Horizontal Semicircular Canal ◽  
Ocular Reflex ◽  
Vestibulo Ocular Reflex ◽  
Central Compensation ◽  
Head Rotations ◽  
Vestibular Deficit

The centrally controlled compensation for a reduced horizontal vestibulo-ocular reflex (VOR) gain caused by a unilateral afferent deficit is usually studied following a selective surgical procedure which completely lesions the vestibular nerve or blocks the horizontal semicircular canal. The more common, unilateral, vestibular deficit encountered clinically, is a partial loss of peripheral vestibular function, following which peripheral recovery and/or central compensation may occur. We investigated changes of the VOR gain in response to a sudden, idiopathic, unilateral vestibular deficit in 64 subjects by examining the responses to low-frequency, whole-body, rotations about an earth vertical axis with different accelerations (5, 20 and 40 deg / sec 2 ) during in- and out-patient visits separated by 4 months in an attempt to identify changes brought about by peripheral recovery and by central compensation processes. Peripheral function was assumed to be measured by the response to caloric irrigation. It improved some 30% between the two visits. VOR responses for rotations towards the deficit side also improved between the two visits. Most improvement occurred for 20 deg / sec 2 accelerations. However, the correlation coefficient between rotation and caloric responses was always less than 0.6. Unlike caloric responses which improved over time, responses for rotations to the intact side did not change between the visits. For this reason, the majority of observed VOR rotation responses were nearly symmetrical at the time of the second visit, despite being below normal levels. These findings suggest that both peripheral recovery and central compensation processes help restore symmetrical VOR function for head rotations after a partial unilateral vestibular deficit. However the improvement of VOR response symmetry, particularly to slow ( < 40 deg / sec 2 ) accelerations, is largely independent of the recovery of peripheral sensitivity.

Effect of aging on the otolith-ocular reflex

2001 ◽  
Vol 11 (2) ◽  
pp. 91-103
Author(s):  
Joseph M. Furman ◽  
Mark S. Redfern
Keyword(s):  
Semicircular Canal ◽  
Head Tilt ◽  
Vestibular Function ◽  
Vertical Axis ◽  
Ocular Reflex ◽  
Age Related ◽  
Vestibulo Ocular Reflex ◽  
Older Subjects ◽  
Axis Rotation ◽  
Reflex Time

We assessed the influence of age on the otolith-ocular reflex and semicircular canal-otolith interaction. Healthy young (n=30) and healthy older (n=60) subjects were rotated about an earth vertical axis, and about a 30 degree off-vertical axis. Eye movements during and following rotation were recorded using electro-oculography. Results indicated that there were statistically significant changes in the otolith-ocular reflex and semicircular canal-otolith interaction as a function of age. The modulation component during off-vertical axis rotation (OVAR) was greater in the older group compard to the young adults, whereas the bias component was smaller with advanced age. The foreshortening of the vestibulo-ocular reflex time constant induced by post-rotatory head tilt following cessation of rotation was less prominent in the older group. There were no consistent changes in the semicircular canal-ocular reflex. Overall, response parameters showed more variability in the older subjects. We conclude that age related changes in the otolith-ocular reflex and semicircular canal-otolith interaction are a result primarily of a degradation of central vestibular processing of otolith signals rather than a decline of peripheral vestibular function.

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