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This chapter starts by comparing two different models to account for optokinetic-vestibular symbiosis. In the first, there are two separate velocity storage (integrator) elements for vestibular and optokinetic systems, and in the second model, velocity storage is shared between the two systems. Behavioral and electrophysiological evidence is presented to support the model with shared velocity storage and its ability to provide a linear addition of vestibular and optokinetic signals, account for different time constants of optokinetic and vestibular responses and separate adaptive properties of the two systems. This model is then extended to account for an unexplained clinical disorder-periodic alternating nystagmus-and provide insights into its pathogenesis and treatment.

I ntracranial vertebral dissections have polymorphs clinical presentations and can lead to haemorrhagic complications if they are intracranial. We here describe a case of a thrombosed dissecting aneurysm of postero-inferior cerebellar artery (PICA) revealed by a Wallenberg syndrome preceded by headaches.A 23-year-old patient, without neurological or vascular past medical history, was admitted for dizziness preceded by headache. The clinical examination on admission revealed an incomplete Wallenberg syndrome, associating hemiface sensitive deficit, Horner's syndrome, dysmetria and nystagmus. The brain MRI showed a latero-medullary infarct with a homolateral PICA thrombosed dissecting aneurysm.The diagnosis of intracranial dissecting aneurysms needs particular caution because aneurysm sac thrombosis can give false reassurance on angiographic MR sequences. Moreover, the anatomic features of intracranial artery walls make them prone to sub-adventitial dissection and subsequent subarachnoid haemorrhages. Therefore, antithrombotic therapy should be used with caution, due to the risk of bleeding in these intracranial dissections.

The article discusses two main causes of acute vestibular dizziness - vertebrobasilar ischemic stroke and vestibular neuritis. The features of acute vestibular syndrome depending on the localization of cerebral infarction - in the territory of the posterior inferior, anterior inferior and superior cerebellar arteries, as well as in the brain stem are presented. Detailed clinical characteristics of vestibular neuritis is given. The issues of differential diagnosis of diseases, including the features of nystagmus and head impulse test, are discussed. The approaches to the treatment of acute vestibular syndrome depending on its etiology are considered. The authors present a treatment and diagnostic algorithm and consider features of clinical practice in acute dizziness. Fundamental differences in the treatment of vestibular neuritis and vertebrobasilar stroke dictate the need for neurologists of vascular departments to master the skills of otoneurological examination, which is the key to differential diagnosis. When choosing a treatment method, the most individualized approach is required.В статье рассмотрены две основные причины острого вестибулярного головокружения — ишемический инсульт в вертебрально-базилярной системе и вестибулярный нейронит (ВН). Представлены особенности острого вестибулярного синдрома в зависимости от локализации инфаркта мозга — в бассейнах задней нижней, передней нижней и верхней мозжечковых артерий, а также в стволе головного мозга. Дана подробная клиническая характеристика ВН. Обсуждены вопросы дифференциальной диагностики данных заболеваний, включая особенности нистагма и импульсного теста поворота головы. Рассмотрены подходы к лечению острого вестибулярного синдрома в зависимости от его этиологии. Представлен лечебно-диагностический алгоритм и обсуждены особенности реальной клинической практики при остром головокружении. Принципиальные различия в терапии при ВН и вертебрально-базилярном инсульте диктуют необходимость освоения неврологами сосудистых отделений навыков отоневрологического обследования, которое является ключом к дифференциальной диагностике. При выборе метода лечения требуется максимально индивидуализированный подход.

Positional nystagmus (PN) is a heterogeneous clinical phenomenon. Most often it develops in benign positional paroxysmal vertigo as a result of otolithiasis. Rarely PN is observed in cases of lesion location in the cerebellum and brainstem. Recently a theory of light cupula was suggested to explain persisting geotropic PN. It explains the development of nystagmus by the changes in the ratio of the density of cupula to the density of endolymph, making the former sensitive to gravitational forces. Several theories were suggested: lighter cupula theory, heavier endolymph theory, theories of light debris and of density difference between perilymph and endolymph. However, the exact mechanism of light cupula syndrome is unclear. Clear diagnosis of the causes of PN requires detailed evaluation of neurological status with the use of diagnostic tests and additional research methods.Позиционный нистагм (ПН) гетерогенный клинический феномен. Наиболее часто он наблюдается при доброкачественном позиционном пароксизмальном головокружении как следствие отолитиаза. Изредка ПН наблюдается при поражениях мозжечка и ствола головного мозга. Недавно для объяснения случаев персистирующего варианта позиционного геотропного нистагма была предложена теория легкой купулы. Она объясняет развитие нистагма изменением соотношения плотности купулы и окружающей эндолимфы, что делает ее чувствительной к гравитационным силам. Предложено несколько гипотез: более легкой купулы, более тяжелой эндолимфы, легких осколков, более тяжелой перилимфы. Однако точный механизм развития синдрома легкой купулы неясен. Точная диагностика причин ПН требует детальной оценки неврологического статуса с использованием диагностических проб и дополнительных методов исследования.

The aim was to characterize a combined vestibular, ocular motor and postural syndrome induced by deep brain stimulation (DBS) of the subthalamic nucleus in a patient with Parkinson's disease.In a systematic DBS programming session, eye, head and trunk position in roll and pitch plane were documented as a function of stimulation amplitude and field direction. Repeat ocular coherence tomography was used to estimate ocular torsion. The interstitial nucleus of Cajal (INC), zona incerta (ZI) and ascending vestibular fibre tracts were segmented on magnetic resonance imaging using both individual and normative structural connectomic data. Thresholded symptom-associated volumes of tissue activated (VTA) were calculated based on documented stimulation parameters.Ipsilateral ocular tilt reaction and body lateropulsion as well as contralateral torsional nystagmus were elicited by the right electrode in a current-dependent manner and subsided after DBS deactivation. With increasing currents, binocular tonic upgaze and body retropulsion were observed. Symptoms were consistent with an irritative effect on the INC. Symptom-associated VTA was found to overlap with the dorsal ZI and the ipsilateral vestibulothalamic tract, while lying rather distant to the INC proper. A ZI-to-INC 'incerto-interstitial' tract with contact to the medial-uppermost portion of the VTA could be traced.Unilateral stimulation of INC-related circuitry induces an ipsilateral vestibular, ocular motor and postural roll-plane syndrome, which converts into a pitch-plane syndrome when functional activation expands bilaterally. In this case, tractography points to an incerto-interstitial pathway, a tract previously only characterized in non-human primates. Directional current steering proved useful in managing this rare side effect.

Objective: Horizontal semicircular canal site pathology of benign paroxysmal positional vertigo demonstrating three types of nystagmi on positional test were studied. We have attempted to design a protocol for its diagnosis and treatment. Methods: 320 patients of HSC-BPPV were subjected to two types of positional tests. Of these, patients with bilateral steady apogeotropic nysatgmus were treated with VAV modification of Semont’s maneuver. Patients with unsteady or changing apo/geotropic signs were converted into steady geotropic ones by repetitive positional tests; followed by barbecue maneuver with forced prolong positioning. Results: Overall 88% of patients had a total recovery. 92% of patients with geotropic nystagmus showed no symptoms after second maneuveral sitting. 85% of patients with apogeotropic nystagmus recovered fully after third maneuveral sitting. Conclusion: Correct identification of subtypes of HSC-BPPV is based on provoked nystagmus by positional tests. After locating the site and side on the basis of nystagmic pattern, physician can apply the appropriate PRM. Level of evidence: II a.

The vestibulo-ocular reflex (VOR) in neurotologic patients can be evaluated most quickly by spontaneous nystagmus examinations, and the video Head Impulse Test (vHIT) quantitatively evaluates the VOR of the semicircular canals. We aimed to clarify the concordance and discrepancies between spontaneous nystagmus and vHIT findings, to provide guidance on screening vestibular functions by initial spontaneous nystagmus examination then vHIT. We evaluated 169 outpatients by spontaneous nystagmus examination in the dark using a charge-coupled device camera, then by horizontal vHIT the same day. Vestibular loss on vHIT was defined if both reduced VOR gain (0.8) and corrective saccade were observed. Adjusted logistic regression modelling revealed that differences in right and left VOR gain positively impacted the presence of nystagmus, which suggests lateralised vestibular dysfunction (P0.05; odds ratio 1.39 [95% confidence interval, 1.1-1.8] per 0.1 increment). When vHIT is regarded as the standard clinical test, the positive predictive value of nystagmus for vestibular loss on vHIT was 44.4%, and the negative predictive value was 93.5%. The adjusted odds ratio of adults ≥65 years old compared to younger patients associated with a discrepancy of nystagmus and vHIT was significant (2.4 [1.1-5.3]). In conclusion, if spontaneous nystagmus is initially observed in patients, further assessment by vHIT could confirm vestibular dysfunction in 40-50% of cases. If no nystagmus is observed, vHIT might also result in a negative finding in 90% of cases. Older adults appear more likely to show discrepancies between nystagmus and vHIT.

Objective: To develop a method of visualising electrooculography data to improve the interpretability of nystagmus eye-movements captured using the Continuous Ambulatory Vestibular Assessment (CAVA®) device. Design: We are currently undertaking a clinical investigation to evaluate the capabilities of the CAVA® device to detect periods of pathological nystagmus. The work presented here was undertaken using unblinded data obtained from the preliminary phase of this investigation. Study sample: One patient with Ménière’s disease and one with Benign Paroxysmal Positional Vertigo. Results: Using the electrooculography data captured by the CAVA® device, we reconstructed 2D animations of patients’ eye movements during attacks of vertigo. We were able to reanimate nystagmus produced as a consequence of two conditions. Concurrent video footage showed that the animations were visually very similar to the patient’s actual eye-movements, excepting torsional eye-movements. Conclusions: The reconstructed animations provide an alternative presentation modality, enabling clinicians to largely interpret electrooculography data as if they were present during a vertigo attack. We were able to recreate nystagmus from attacks experienced in the community rather than a clinical setting. This information provides an objective record of a patient’s nystagmus and could be used to complement a full neurotologic history when considering diagnosis and treatment options.

To analyse the neuro-ophthalmological data of children referred for further work-up of infantile nystagmus where ophthalmological evaluation had not achieved a diagnosis.We retrospectively reviewed medical records of patients presenting with infantile nystagmus at our institution between 2007 and 2019. Inclusion criteria were onset before 6 months of age, availability of complete ophthalmic examination, visual electrophysiological tests, and neurological examination. Children with a previous definite ophthalmological diagnosis at onset and those with uncertain nystagmus onset age were not recruited.Out of 142 infants (mean age at nystagmus onset 3.6 mo, SD 1.7, range 0-6 mo; 56 females, 86 males), 23% had neurological nystagmus, 7% mixed neurological and sensory nystagmus, 48% sensory defect, and 22% idiopathic infantile nystagmus. The neurological diagnoses were inborn errors of metabolism, white matter genetic disorders, and brain malformations. The prevalent diagnosis in the sensory defect subgroup was retinal dystrophy.Infantile nystagmus without diagnostic ocular findings may be due to neurological, retinal, and optic nerve disorders or be a benign idiopathic condition. In infants with and without neurological abnormalities, the search for a sensory defect should include visual electrophysiology performed early in the diagnostic pathway.Infantile nystagmus without diagnostic ophthalmological signs has an underlying neurological cause in 30% of cases. Neurological diagnoses include congenital brain malformations, and metabolic and genetic disorders. Sensory defects are part of systemic neurological disorders in 23% of infants. Electrophysiology is useful when ophthalmological examination is uninformative.

BACKGROUND Acquired pendular nystagmus (APN) is a back and forth, oscillatory eye movement in which the 2 oppositely directed slow phases have similar waveforms. APN occurs commonly in multiple sclerosis and causes a disabling oscillopsia that impairs vision. Previous studies have proven that symptomatic therapy with gabapentin or memantine can reduce the nystagmus amplitude or frequency. However, the effect of these medications on visual acuity (VA) is less known and to our knowledge the impact of non-pharmacological strategies such as blinking on VA has not been reported. This is a single observational study without controls (Class IV) and is meant to suggest a future strategy for study of vision in patients with disabling nystagmus and impaired vision. CASE REPORT A 49-year-old woman with primary progressive multiple sclerosis with spastic paraparesis and a history of optic atrophy presented with asymmetrical binocular APN and bothersome oscillopsia. We found that in the eye with greater APN her visual acuity improved by 1 line (from 0.063 to 0.08 decimals) immediately after blinking. During treatment with memantine, her VA without blinking increased by 2 lines, from 0.063 to 0.12, but improved even more (from 0.12 to 0.16) after blinking. In the contralateral eye with a barely visible nystagmus, VA was reduced by 1 line briefly (~500 ms) after blinking. CONCLUSIONS In a patient with APN, blinking transiently improved vision. The combination of pharmacological treatment with memantine and the blinking strategy may induce better VA and less oscillopsia than either alone.

Background: nystagmus is a disorder of rhythmic, involuntary oscillations of the eyes and can be classified aseither infantile or acquired. Whether it occurs in isolation or as a part of other visual or neurological disorders, itcauses significant visual dysfunction and problems with social functioning. In this study, we seek to understandways in which people with nystagmus are currently supported across the UK and identify any geographicalvariations or disconnects between current practice and best practice, as judged by patients and their carers.Methods: a nationwide, qualitative, cross-sectional, questionnaire study of people with nystagmus andtheir carers. Recruitment was achieved through specialist clinics, charity events, online advertisementsand social media calls. Data was gathered using five, age-appropriate questionnaires which werecompleted and returned to the research team between November 2016 and August 2018.Results: 184 respondents were included (89 carers, 47 patients aged 4–10 years, 5 aged 11–14 years, 4aged 15–17 years and 39 > 18 years). Notably, respondents rated social media as the best source ofinformation they have received, even compared with face-to-face consultation with medical professionals. Additionally, only 33% of the respondents had been offered visual impairment support. Notably,patterns of clinical practice and patient experience emerged according to geographical location,particularly provision of initial information and ongoing VI support.Conclusions: this study highlights a significant variation in the support and information received bypeople in the UK with nystagmus. It also supports the role of charities and increasingly, social media inthe provision of patient information. The study also highlights the need for standardized guidelines forthe management of patients with nystagmus, particularly with regard to support and information.

Objectives The aim of the present study was to evaluate the ability of head-shaking nystagmus (HSNy), evoked after the resolution of a vertigo spell, to predict an imminent crisis in the early stage of Meniere's disease (MD). Materials and methods A total of 20 patients in the early stage of MD were included in the study. The head-shaking test (HST) was performed twice, during the first visit within 24 h of vertigo spell (T0) and 48 h later (T1). The onset of a new vertigo episode during the 2 weeks following the first visit was recorded in each patient's medical record. The sensitivity and specificity of HSNy toward predicting a new vertigo episode were calculated. Results At T0, an evoked ipsilesional HSNy in 15 (75%) patients was observed; in four of them, the HSNy had a biphasic component. The HSNy was present and persistent at T1 in 8 (42.1%) patients; among these cases, 6 patients had ipsilesional HSNy, and 2 patients a contralesional HSNy. None of the patients presented with a biphasic HSNy at T1. Seven (36.8%) patients experienced the recurrence of a vertigo crisis. Among these, 6 patients had ipsilesional HSNy at T1. Only 8 patients with ipsilesional HSNy at T0 did not have recurrence. The sensitivity of the ipsilesional HSNy in predicting the recurrence of vertigo in patients with MD was 100% at T0 and 85.7% at T1. The specificity was 46.6% and 100% at T0 and T1, respectively. Conclusion The HST can be a useful test in the early stages of MD to predict a new vertigo attack.

BackgroundPeriodic alternating nystagmus is a rare condition characterised by spontaneous horizontal nystagmus that periodically reverses direction, indicating an alteration of the velocity storage mechanism. Windmill nystagmus is a peculiar and rare variant of periodic alternating horizontal nystagmus with a superimposed periodic alternating vertical nystagmus. It is generally observed in blind patients.Case reportThis paper presents the unique case of a normally sighted patient with a windmill nystagmus triggered by an episode of benign paroxysmal positional vertigo due to bilateral posterior canalolithiasis. Videonystagmography revealed an anticlockwise up-beating nystagmus followed by a clockwise down-beating nystagmus with a cycle lasting 2 minutes, followed by a brief burst of horizontal left-beating nystagmus.ConclusionThis case report represents the first observation of a new type of windmill nystagmus, probably provoked by a malfunction of the velocity storage mechanism, gaze-stabilisation and short-adaptation networks, with a loss of cerebellar inhibition.

An absence of skin lesions at the neurological onset may obscure the diagnosis of neuro-Sweet disease (NSD). We herein report a 32-year-old man with NSD in whom neurological symptoms preceded the development of skin lesions by 10 years. The patient exhibited four distinct neurological episodes: meningoencephalitis, scattered brain lesions, ocular flutter, and isolated seizures. Acute relapses responded to corticosteroid therapy, and the patient was successfully maintained on corticosteroid and dapsone combination therapy. NSD should be considered in the differential diagnosis of patients with recurrent neurological manifestations, especially with both meningeal and brain parenchymal involvement, even if no skin lesions are observed.

Pseudo-spontaneous nystagmus (PSN) can be detected in patients with lateral canal benign paroxysmal positional vertigo (LC-BPPV). Its frequency, and correlation with the therapeutic outcome have been less described and conflicting results have been reported.This study aims to investigate its clinical and prognostic significance.One hundred and eighty-four patients with LC BPPV (98 apogeotropic, 86 geotropic type) were enrolled for the study. Clinical parameters were reviewed in patients with or without PSN. The Chi-square and one way ANOVA tests were used to compare the difference between study groups. Statistical significance was set atTwenty-two patients with apogeotropic (22.4%; 98/22) and 17 patients with geotropic nystagmus (19.7%; 86/17) had PSN. The incidence, age, male-female ratio, mean slow phase velocity (SPV), duration of BPPV and the rate of recurrence were not significant in patients with LC-BPPV whether they have PSN or not.Prognostic role of PSN in patients with LC BPPV seems to be questionable.Appearance and disappearance of PSN with regard to head position helps to differentiate BPPV from other acute vestibular disorders. Additionally, direction of nystagmus assists to determine the site of the affected canal. However, prognostic significance is obscure.

Vestibular compensatory eye movements provide visual fixation stabilization during head movement. The anatomic pathways mediating a normal horizontal vestibulo-ocular reflex (h-VOR), when lesioned, cause spontaneous nystagmus. While previous reports address the effect of convergence on different spontaneous nystagmus types, to our knowledge, a study of acute vestibular nystagmus suppression viewing near targets comparing patients with peripheral or central vestibular lesions has not been previously reported.We attempt to clarify potential vestibular and near-reflex interaction by comparing near and far h-VOR gain in 19 healthy controls, six patients with acute/subacute peripheral vestibular lesion (PVL), and one patient with unilateral vestibular nuclear lesion (VNL) in the pontine tegmentum.The horizontal (h)-VOR in normal subjects increased with convergence in both eyes (P = 0.027, P0.001). In unilateral PVL patients, gain failed to increase in either direction (P = 0.25, P = 0.47). In contrast, when fixating at 15 cm, the h-aVOR in the VNL lesion, gain did not increase, and a right h-nystagmus developed. Even though we found inability to increase gain in PVL with near target fixation, this did not interfere with h-nystagmus suppression upon converging. Our VNL patient had normal h-nystagmus suppression viewing far distance targets and lacked near target h-nystagmus suppression.We hypothesize that normal IO/flocculus pathway suppressed spontaneous nystagmus in PVL. Impaired h-VOR near adaptation in the medial vestibular nucleus was responsible for h-nystagmus direction with fixation block. Additionally, impaired viewing distance estimate contributed to near h-nystagmus suppression failure.

Purpose of review Major therapeutic advances have been made in patients with episodic and progressive cerebellar ataxias, downbeat nystagmus and some vestibular disorders. We provide an update review on this subject highlighting important research findings from the last two years. Recent findings Recently, the use of omaveloxolone for 2 years significantly improved upright stability in Friedreich's ataxia patients. In an open-label study, N-acetyl-L-leucine administered for 6-weeks significantly improved clinical impression of change, ataxia, and quality of life in patients with Niemann-Pick disease type C1. A 12-week treatment with dalfampridine was associated with improved standing balance in a subgroup of patients with multiple sclerosis. A gluten-free diet alone improved ataxia in half of patients with antiglutamic acid decarboxylase (GAD) ataxia, suggesting that gluten sensitivity might be part of the underlying pathogenesis in anti-GAD ataxia. In a head-to-head trial, both prolonged-release 4-aminopyridine (4-AP) and acetazolamide effectively reduced the attacks up to 60% in patients with episodic ataxia type 2 (EA2), albeit 4-AP had fewer adverse effects. Small observational studies have shown that patients with episodic vestibular syndrome who cannot be diagnosed as definite or probable vestibular migraine, might still improve vestibular symptoms following preventive treatment for migraine. The use of vitamin D supplementation in benign paroxysmal positional vertigo, steroids in acute unilateral vestibulopathy, and betahistine in Meniere's disease patients remains controversial. Summary Although the use of several therapies is being established in the treatment of cerebellar and vestibular disorders, there is an urgent need for prospective controlled therapeutic trials.

Patients with benign paroxysmal positional vertigo (BPPV) occasionally experience severe dizziness, could not maintain the sitting posture, and then fall onto or off the examination table when they return to the sitting position, which is the last step of the barbecue maneuver and Epley maneuver (EM); down-beating nystagmus is also observed. This study aims to investigate the clinical characteristics and significance of these findings. We retrospectively reviewed video data showing nystagmus and medical records of adult patients diagnosed with canalolithiasis of the horizontal canal and the posterior canal (PC) BPPV who underwent barbecue maneuver and EM, respectively, in outpatient clinics from April 2014 to March 2019. This study included 112 patients (28 horizontal canal BPPV and 94 PC BPPV cases). Among the 122 BPPV cases, only 14 (14.9%) were analyzed, due to their occurrence during EM. Down-beating nystagmus appeared at 3.6 seconds on average after returning to the sitting position, and the patients fell onto or off the examination table at 4.4 seconds on average after the onset of the nystagmus. The average duration of the down-beating nystagmus was 20.3 seconds. In all 14 cases, no nystagmus was induced by the Dix-Hallpike test performed again after EM, confirming that the treatment was successful. During the EM, down-beating nystagmus and falling onto or off the examination table occurred in approximately 15% of cases. As the risk of falls increases, the patient should be secured immediately after EM. Moreover, it can be inferred that the findings occur when otoconia in the PC enter the utricle, suggesting a successful treatment.

Seesaw nystagmus (SSN) is a rare, debilitating neurologic syndrome with few known etiologies. Typically, patients present with oscillopsia subsequent to a recent neurologic insult, with only a handful of patients showing delayed onset after trauma. We report a case of seesaw nystagmus in a patient with a large meso-diencephalic mass treated with external beam radiation therapy (XRT), who developed asymptomatic SSN nearly 8 years after treatment.

Pineal masses commonly present with Parinaud syndrome, a triad consisting of upgaze restriction, convergence-retraction nystagmus, and pupillary light-near dissociation from compression of the quadrigeminal plate.

Introduction Medial longitudinal fasciculus infarction is rare in clinical practice and generally accompanied by brain tissue damage around the medial longitudinal fasciculus. Isolated medial longitudinal fasciculus midbrain infarction was seldom reported. Case report An 81-year-old man with hypertension was admitted to our hospital because of sudden onset diplopia. A neurological examination revealed right adduction paresis without abducting nystagmus in the left eye, whereas the convergence reflex was normal. Diffusion-weighted magnetic resonance imaging demonstrated a small acute lacunar medial longitudinal fasciculus infarction in the right midbrain at the level of the inferior colliculus. Diffusion-tensor imaging showed a reduction of the right medial longitudinal fasciculus. Medial longitudinal fasciculus infarction is rare and occurs most commonly in the pons. The authors report on a case of isolated medial longitudinal fasciculus infarction that was diagnosed because of sudden diplopia and manifested as simple internal rectus paralysis with no abducting nystagmus on the contralateral side of the lesion. Conclusion Isolated midbrain-medial longitudinal fasciculus infarction without contralateral abducting nystagmus is a rare occurrence. It can be differentiated from partial oculomotor palsy by assessing the convergence reflex, primary gaze, and diffusion-tensor imaging.

Nystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central compensatory movement back to the primary position (refixation saccade). The direction, however, is reported according to the fast phase. The cardinal symptoms are, on the one hand, blurred vision, jumping images (oscillopsia), reduced visual acuity and, sometimes, double vision; many of these symptoms depend on the eye position. On the other hand, depending on the etiology, patients may suffer from the following symptoms: 1. permanent dizziness, postural imbalance, and gait disorder (typical of downbeat and upbeat nystagmus); 2. if the onset of symptoms is acute, the patient may experience spinning vertigo with a tendency to fall to one side (due to ischemia in the area of the brainstem or cerebellum with central fixation nystagmus or as acute unilateral vestibulopathy with spontaneous peripheral vestibular nystagmus); or 3. positional vertigo. There are two major categories: the first is spontaneous nystagmus, i.e., nystagmus which occurs in the primary position as upbeat or downbeat nystagmus; and the second includes various types of nystagmus which are induced or modified by certain factors. Examples are gaze-evoked nystagmus, head-shaking nystagmus, positional nystagmus, and hyperventilation-induced nystagmus. In addition, there are disorders similar to nystagmus, such as ocular flutter or opsoclonus. The most common central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of 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 midbrain or medulla. Examples of drug treatment are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for pendular fixation nystagmus, or baclofen for periodic alternating nystagmus. In this article we are focusing on nystagmus. In a second article we will focus on central ocular motor disorders, such as saccade or gaze palsy, internuclear ophthalmoplegia, and gaze-holding deficits. Therefore, these types of eye movements will not be described here in detail.Unter einem Nystagmus versteht man rhythmische Augenbewegungen, die i. d. R. aus einer langsamen (ursächlichen bzw. pathologischen) Augendrift und einer schnellen kompensatorischen Rückstellbewegung (Rückstellsakkade) bestehen; die Richtung wird klinisch nach der schnellen Phase angegeben. Leitsymptome sind zum einen Verschwommensehen, „hüpfende oder laufende Bilder“ (Oszillopsien), verminderte Sehschärfe sowie manchmal Doppelbilder, die oft blickpositionsabhängig sind. Zum anderen können die Patienten, je nach Ursache, leiden unter 1. permanentem Schwankschwindel und Gangunsicherheit (wie beim Downbeat- [DBN] oder Upbeat-Nystagmus [UBN]), 2. Drehschwindel mit gerichteter Fallneigung (bei akutem Beginn) sowie 3. Lagerungsschwindel. Es lassen sich im Wesentlichen zwei Kategorien unterscheiden: 1. Spontannystagmus, d. h. ein Nystagmus, der in Geradeausblickposition auftritt, wie DBN oder UBN, und 2. verschiedene Nystagmusformen, die durch bestimmte Faktoren ausgelöst oder moduliert werden, wie Blickrichtungs-, Kopfschüttel-, Rebound-, Lagerungs-, hyperventilations- oder druckinduzierter Nystagmus. Daneben gibt es noch nystagmusähnliche schnelle Augenbewegungen wie Ocular Flutter oder Opsoklonus. Die häufigsten zentralen Formen eines Spontannystagmus sind DBN, UBN, infantiler Nystagmus, seltener sind Fixationspendelnystagmus, rein torsioneller, periodisch alternierender und See-saw-Nystagmus. Viele Nystagmusformen erlauben eine genaue anatomische Lokalisation, wie z. B. der DBN, der meistens auf einer beidseitigen zerebellären Flocculusläsion beruht oder der UBN auf einer Läsion im Mesenzephalon oder der Medulla oblongata. Beispiele einer Pharmakotherapie sind die Gabe von 4-Aminopyridin beim DBN und UBN, Memantin oder Gabapentin beim Fixationspendelnystagmus oder Baclofen beim periodisch alternierenden Nystagmus. Abschließend: in einem nachfolgenden Artikel werden in dieser Zeitschrift die häufigsten und klinisch relevanten zentralen Okulomotorikstörungen wie Sakkaden und Blickparesen, internukleäre Ophthalmoplegie oder Blickhaltedefekte dargestellt, sodass auf diese hier nicht detailliert eingegangen wird.

Rationale: Vestibular paroxysmia (VP) is characterized by spontaneous, recurrent, short, paroxysmal attacks of vertigo with or without tinnitus. Patient concerns: We report a case of paroxysmal recurrent vertigo accompanying clicking tinnitus on the left side in a 61-year-old patient. He had undergone microvascular decompression to treat the left-side hemifacial spasm 6 years prior. The patient first developed vertigo attacks about 3 years after microvascular decompression, and the attacks increased in frequency over the last 4 months. Video-nystagmography revealed a background right-beating nystagmus which was reversed every 55 seconds, to left-beating nystagmus for 17 seconds. Diagnosis: Brain magnetic resonance imaging and angiography demonstrated a compression of the cisternal segment of the left vestibulocochlear nerve between the tortuous right vertebral artery and the posterior wall of the left porus acusticus internus. Interventions and outcomes: Under the diagnosis of VP, 300 mg oxcarbazepine was administered daily, which relieved the symptoms dramatically. Lesson: The neurovascular cross-compression of the vestibulocochlear nerve by the contralateral vertebral artery tortuosity can cause VP. Periodic paroxysms of right-beating nystagmus accompanying the left-side tinnitus during vertigo attacks in our patient can be explained by secondary central hyperactivity in both vestibular and cochlear nuclei following long-standing neurovascular cross-compression.

Introduction: Clinically, there is a kind of patients with positional vertigo or dizziness, which occurs when they turn left or right, look down or up, lie down or sit up. With a long duration and varying frequency, it is not consistent with the manifestations of benign paroxysmal positional vertigo (BPPV). In addition, the persistent geotropic direction-changing positional nystagmus (PG-DCPN) was observed in a supine head-roll test. Patient concerns: With no apparent trigger for visual rotation and a sense of self instability, an 81-year-old female patient had suffered from vertigo for 3 days. The vertigo occurred every day, lasting several minutes each time, and associated with head movements and changes in body position. In a supine head-roll test, it appeared persistent geotropic direction-changing positional nystagmus for a long time, without latency, fatigability and in the presence of 3 zero planes. Diagnosis: Light cupula. Interventions: Difenidol hydrochloride 25 mg orally 3 times/day for 2 weeks and betahistine hydrochloride 12 mg orally 3 times/day for 1 month were administered. Outcomes: After 1 month of treatment, the patient's vertigo symptoms disappeared. And in the supine head-roll test, the persistent geotropic direction-changing positional nystagmus disappeared. Conclusion: We report the characteristics of nystagmus produced in a typical patient with light cupula during the supine head-roll test. After reviewing the relevant literatures, we believe that a simpler method can be used to identify canalolithiasis and cupula disease, to distinguish light and heavy cupula, and to determine the pathological semicircular canal to which the lesion belongs.

The optokinetic nystagmus is a gaze-stabilizing mechanism reducing motion blur by rapid eye rotations against the direction of visual motion, followed by slower syndirectional eye movements minimizing retinal slip speed. Flies control their gaze through head turns controlled by neck motor neurons receiving input directly, or via descending neurons, from well-characterized directional-selective interneurons sensitive to visual wide-field motion. Locomotion increases the gain and speed sensitivity of these interneurons, while visual motion adaptation in walking animals has the opposite effects. To find out whether flies perform an optokinetic nystagmus, and how it may be affected by locomotion and visual motion adaptation, we recorded head movements of blowflies on a trackball stimulated by progressive and rotational visual motion. Flies flexibly responded to rotational stimuli with optokinetic nystagmus-like head movements, independent of their locomotor state. The temporal frequency tuning of these movements, though matching that of the upstream directional-selective interneurons, was only mildly modulated by walking speed or visual motion adaptation. Our results suggest flies flexibly control their gaze to compensate for rotational wide-field motion by a mechanism similar to an optokinetic nystagmus. Surprisingly, the mechanism is less state-dependent than the response properties of directional-selective interneurons providing input to the neck motor system.

To evaluate how often the positive sign of HINTS (Head-Impulse, Gaze Evoked Nystagmus, Test of Skew) appears in patients with acute peripheral vestibular lesion, HINTS findings were quantitatively measured and analyzed in patients with peripheral vestibulopathy accompanying spontaneous nystagmus.HINTS was evaluated in 14 vertigo patients with spontaneous nystagmus. Horizontal vestibulo-ocular reflex (VOR) gain was measured using the video head impulse test (vHIT). To evaluate gaze-evoked nystagmus (GEN), slow-phase velocities at different points of lateral gaze were measured and plotted, then the slope and its inverse value, the neural integrator time constant, were calculated. Skew deviation was tested using anaglyph filters to simulate the alternate cover test, and the degree and latency of vertical eyeball deviation were measured. The ABCD2 score was calculated to evaluate the risk of stroke.Among 13 patients of peripheral vestibulopathy, 7 showed positive signs in HINTS (normal vHIT: 5, direction-changing GEN: 0, skew deviation: 3). One patient with a cerebellopontine angle tumor presented with both a peripheral and central pattern and showed positive HINTS findings (presence of direction-changing GEN). The mean VOR gain of patients with abnormal vHIT was 0.58±0.29 and 1.10±0.11 in the affected and contralateral side, respectively, while those in patients with normal vHIT were 1.04±0.21 and 1.13±0.12, respectively. The neural integrator time constant calculated from the mean slope of horizontal slow-phase velocity according to horizontal eye position was 42.9 s. The mean vertical eyeball deviation of patients with positive skew was 2.14±1.18° while uncovering the eye on the affected side, and -1.97±1.59° while uncovering the eye on the unaffected side. The median ABCD2 score of 14 patients was 2 (range, 1-3).HINTS findings were objectively measured in vertigo patients with spontaneous nystagmus. Although positive findings of HINTS have been recognized as a central sign, 54% (7/13) of cases with peripheral vestibulopathy showed positive HINTS signs. HINTS results should be interpreted carefully considering that a substantial proportion of peripheral vestibulopathy shows a positive HINTS sign.

When diagnosing benign paroxysmal positional vertigo, extraocular muscle contraction and the nystagmus it causes, though recognized as an important indicator, is less commonly seen as a principal method of diagnosis. However, through determining the direction of resulting nystagmus during diagnostic tests such as the supine roll test and the Dix-Hallpike test, which semicircular canals are involved in individual cases of benign paroxysmal positional vertigo can be ascertained, in both cases involving only one semicircular canal or cases of multi-canal benign paroxysmal positional vertigo.

As we know, benign paroxysmal positional vertigo is a peripheral vestibular disorder,while vestibular migraine is a central vestibular disease. Although they are two different independent diseases, patients often choose to consult otolaryngology and neurology. The clinical manifestations of some patients with these two diseases are similar and the diagnosis is easy to be confused, but the treatment methods are completely different, and incorrect treatment methods will inevitably affect the curative effect and prognosis. Vestibular migraine is characterized by a diversity of clinical manifestations and signs, and the migraine symptoms of some patients do not match with the vestibular symptoms. There are 30% of vestibular migraine patients who showed isolated paroxysmal vertigo/dizziness and nystagmus, which is easily confused with benign paroxysmal positional vertigo and needs to be identified. Under the framework of the international classification of vestibular diseases, according to the new guideline of nystagmus examination and classification issued by Barany Association and new diagnostic criteria of two diseases, differential diagnosisof benign paroxysmal positional vertigo from vestibular migrainecan make up for one-sided understanding caused by the specialization of disciplines, improve thediagnosis and treatment of vestibular diseases, and thus reduce the misdiagnosis and mistreatment.良性阵发性位置性眩晕是外周性前庭疾病，前庭性偏头痛是中枢性前庭疾病，虽然二者是不同的独立疾病，但常选择耳鼻咽喉科和神经科两个专科就诊。这两种疾病的部分患者临床表现相似，诊断易混淆，但治疗手段完全不同，治疗方法不正确必然影响疗效和预后。前庭性偏头痛的特点是临床表现和体征具有多样性，部分患者的偏头痛症状与前庭症状不同步，约30%表现为孤立性发作性眩晕/头晕和眼震，极易与良性阵发性位置性眩晕混淆而需鉴别。在前庭疾病国际分类的框架下，依据Barany协会新颁布的眼震检查与分类，依据这两种疾病新的诊断标准进行甄别，可弥补由于学科专业化导致的对二者认识的片面性，提高对包括这两种疾病在内的前庭疾病诊疗水平，以期减少误诊误治。.

Background: There is no authorized treatment for ataxia telangiectasia (AT). As cerebellar symptoms of storage diseases were improved by acetyl-DL-leucine (ADLL), the authors hypothesized a symptomatic and disease-modifying effect in AT upon supplementation with ADLL. Methods: Six patients were treated with ADLL 3 g/day for 1 week followed by 5g/day for 3 weeks to 1 year. Cerebellar ataxia was evaluated by validated scales. Gaze-holding, saccades and smooth pursuit were examined by video-oculography. Measurements took place at baseline, at 1 month of therapy in 5 patients, and after 6 and 12 months in 1 patient. Results: The Scale for Assessment and Rating of Ataxia changed from the baseline, mean, (SD, min-max) of 22.1 (5.88, 11-28.5) to 18 points (5.39, 8.5-23.5) after 1 month on medication ( P = .0028). All patients demonstrated gaze-holding deficits; 3 patients had central-position downbeat-nystagmus. Mean slow-phase velocity of this nystagmus with the gaze straight-ahead changed from 5.57°/s (1.8, 3.53-6.99) to 4.7°/s (0.79, 3.97-5.56) after 1 month on treatment (1.35, -2.56-4.17) ( P = .046). Interpretation: ADLL may improve ataxia and ocular stability in AT patients, while the molecular basis still remains to be elucidated. A multicentric, rater-blinded, phase II trial currently investigates the effects of acetyl-L-leucine in AT (NCT03759678).

Purpose Myoblast determination protein 1 (MYOD) is a critical myogenic regulatory factor in muscle development, differentiation, myofiber repair, and regeneration. As the extraocular muscles significantly remodel their myofibers throughout life compared with limb skeletal muscles, we hypothesized that the absence of MYOD would result in their abnormal structure and function. To assess structural and functional changes in the extraocular muscles in MyoD-/- mice, fiber size and number and optokinetic nystagmus reflex (OKN) responses were examined. Methods OKN was measured in MyoD-/- mice and littermate wild-type controls at 3, 6, and 12 months. The extraocular muscles were examined histologically for changes in mean myofiber cross-sectional area, total myofiber number, and nuclei immunostained for PAX7 and PITX2, markers of myogenic precursor cells. Results The MyoD-/- mice developed nystagmus, with both jerk and pendular waveforms, in the absence and in the presence of moving visual stimulation. At 12 months, there were significant losses in mean myofiber cross-sectional area and in total number of orbital layer fibers in all rectus muscles, as well as in global layer fibers in the superior and inferior rectus muscles. Haploinsufficient mice showed abnormal OKN responses. PITX2-positive cell entry into myofibers of the MyoD-/- mice was significantly reduced. Conclusions This study is the first demonstration of the development of nystagmus in the constitutive absence of expression of the muscle-specific transcription factor MYOD. We hypothesize that myofiber loss over time may alter anterograde and/or retrograde communication between the motor nerves and extraocular muscles that are critical for maintaining normalcy of extraocular muscle function.

Microcephaly is a prominent feature of patients with primary autosomal recessive microcephaly 2 (MCPH2) caused by mutations in the WD Repeat Domain 62 (WDR62; OMIM: 613,583).The study aimed to identify the underlying genetic factor(s) causing microcephaly in two patients in a consanguineous Iranian family.Two male patients (11 and 27 years old) were noticed due to microcephaly, neurodevelopmental delay, and occasional seizures. The younger patient (the proband) was subjected to paired-end whole-exome sequencing followed by Sanger sequencing to detect any underlying genetic factor.Upon examination, both patients showed microcephaly as a prominent manifestation; they were under-weighted as well. The patients had a moderate gross motor impairment, severe cognitive disability and speech delay, increased deep tendon reflexes, flexible joint contractures, sensorineural hearing loss, and vertical nystagmus as a new ocular finding. The proband had more severe neurodevelopmental delay symptoms. The brain magnetic resonance imaging series revealed severe structural and cortical brain abnormalities in addition to hemiatrophy. Using Whole-exome Sequencing, a novel homozygous missense variant-NM_001083961.2; c.1598A G: p.(His533Arg)-was identified in the WDR62. Subsequently, in silico analyses determined the possible impacts of the novel variant on the structure and function of WDR62 protein.Herein, we identified a novel homozygous missense variant in the WDR62 in two patients with MCPH2. Vertical nystagmus and sensorineural hearing loss were detected as novel neurological findings. The present study expands the phenotype and genotype spectrum of MCPH2.

Downbeat nystagmus (DBN) is a common form of acquired fixation nystagmus related to vestibulo-cerebellar impairments and associated with impaired vision and postural imbalance. DBN intensity becomes modulated by various factors such as gaze direction, head position, daytime, and resting conditions. Further evidence suggests that locomotion attenuates postural symptoms in DBN. Here, we examined whether walking might analogously influence ocular-motor deficits in DBN. Gaze stabilization mechanisms and nystagmus frequency were examined in 10 patients with DBN and 10 age-matched healthy controls with visual fixation during standing vs. walking on a motorized treadmill. Despite their central ocular-motor deficits, linear and angular gaze stabilization in the vertical plane were functional during walking in DBN patients and comparable to controls. Notably, nystagmus frequency in patients was considerably reduced during walking compared to standing (p p = 0.015). These attenuating effects on nystagmus intensity during walking suggest that ocular-motor control disturbances are selectively suppressed during locomotion in DBN. This suppression is potentially mediated by locomotor efference copies that have been shown to selectively govern gaze stabilization during stereotyped locomotion in animal models.

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.

A mono-positional persistent, direction-fixed apogeotropic nystagmus (MPosApoNy) is very challenging for the neuro-otologist. MPosApoNy can be found in patients suffering from a partially compensated acute unilateral vestibulopathy; with a normal caloric test, one can speculate the presence of "trapped" otolithic debris located close to the ampulla of the horizontal semicircular canal.Among 957 patients suffering from vertigo and dizziness, we selected 53 cases of MPosApoNy.In 28 patients, caloric test showed a canal paresis on the same side of the MPosApoNy. In the remaining 25 cases, MPosApoNy was the only clinical finding. We hypothesised the presence of horizonal canal lithiasis and patients were treated with a Gufoni manoeuvre, followed by a forced prolonged position.Performing bedside examination in a patient suffering from vertigo, the presence of MPosApoNy may be due to: a) facilitation of a subclinical nystagmus due to the mechanism of apogeotropic reinforcement; b) horizontal canal lithiasis with 'trapped' otoliths close to the ampulla. The disappearance of MPosApoNy following a repositioning manoeuvre or conversion in a typical form of canalolithiasis may represent the best method to confirm this hypothesis.Il significato clinico del nistagmo monoposizionale orizzontale apogeotropo.Il riscontro di un nistagmo monoposizionale orizzontale apogeotropo (MPosApoNy) rappresenta una sfida diagnostica per il vestibologo. Tale nistagmo può indicare, in presenza di un deficit al test calorico, una labirintopatia periferica non compensata. In assenza di tale reperto, il MPosApoNy potrebbe essere riconducibile ad una forma atipica di litiasi del canale semicircolare orizzontale (CSL).Su 957 pazienti con vertigini, ne abbiamo selezionati 53 che presentavano MPosApoNy.In 28 pazienti il test calorico evidenziò un deficit ipsilaterale al MPosApoNy. Nei rimanenti 25 in cui il test calorico risultò nella norma, si ipotizzò una litiasi del CSL con otoliti intrappolati in prossimità dell’ampolla. Questi pazienti vennero trattati con manovra di Gufoni e successiva posizione coatta.Il riscontro di un MPosApoNy può riconoscere una duplice causa. Esso può essere l’espressione di un nistagmo subclinico esito di labirintopatia acuta monolaterale. Tuttavia, la presenza di otoliti intrappolati in stretta vicinanza dell’ampolla ne può spiegare la genesi. In quest’ultimo caso, le manovre liberatorie, attraverso la risoluzione del MPosApoNy o per la conversione in una forma tipica di canalolitiasi, possono confermarne l’origine litiasica.

When assessing the acutely dizzy patient, the HINTS 'Plus' (Head Impulse, Nystagmus, Test of Skew, 'Plus' a bedside assessment of auditory function) exam is a crucial component of the bedside exam. However, there are additional ocular motor findings that can help the clinician distinguish peripheral from central etiologies and enable accurate localization, especially when the patient has acute dizziness, vertigo and/or imbalance but without spontaneous nystagmus. We will review the literature on these findings which are 'beyond HINTS' and include saccades/ocular lateropulsion, smooth pursuit, and provocative maneuvers including head-shaking and positional testing (not part of the HINTS exam). Additionally, we will expound on the localizing value of nystagmus, ocular alignment and the ocular tilt reaction (parts of the HINTS exam). The paper has been organized neuroanatomically, based on brainstem and cerebellar structures that have been reported to cause the acute vestibular syndrome.

The video head impulse test (vHIT) assesses the vestibulo-ocular reflex (VOR) during a rapid high-velocity low amplitude (10°-20°) head rotation. Patients with peripheral vestibulopathy have a reduced VOR gain with corrective catch-up saccades during the head turn. There are several pitfalls, mainly technical, which may interfere with interpretation of vHIT data. In addition, intrusive eye movement disorders such as spontaneous nystagmus that affect normal eye position and tracking can affect the vHIT results. To date there has been little study of neurological saccadic eye movements that may interfere with the interpretation of vHIT data. Here, in ten patients with a range of central neurological disorders, we describe oculomotor abnormalities on vHIT in the presence of normal range VOR gain values, recorded at a tertiary vestibular neurology service.

Purpose of review This review will extensively cover the clinical manifestations, causes, diagnostic evaluation, and management strategies of downbeat nystagmus (DBN). Recent findings Historically, MRI to assess for structural lesions at the cervicomedullary junction has been the primary diagnostic test in the evaluation of DBN since the 1980s. In recent years, there is increasing awareness of nonstructural causes of DBN including gluten ataxia, nutritional deficiencies, and paraneoplastic syndromes, among others. Medical management with aminopyridines has become first-line therapy in addition to disease-specific therapies. Summary DBN is a common form of acquired nystagmus and the differential diagnosis remains broad, including both benign and potentially fatal causes. For practical purposes, the causes can be categorized as structural vs. nonstructural with MRI as the ideal, initial diagnostic study to differentiate the two. General therapeutic options include pharmacotherapy to enhance Purkinje cell function, strabismus surgery or prisms to shift null points, and behavioural changes. Disease-specific treatment is necessarily broad, though a significant proportion of patients will be idiopathic.

Objective To evaluate the performance of different vestibular indicators in disease classification based on machine learning method. Methods This study use retrospective analysis of the vertigo outpatient database from a tertiary care general hospital. 1491 patients with definite clinical diagnoses were enrolled in this study. Spontaneous nystagmus, head-shaking nystagmus, positional nystagmus, unilateral weakness in caloric test, and gain and saccade in video head impulse test (vHIT) were recorded as variables. Diagnoses were mainly reorganized as acute vestibular syndrome, episodic vestibular syndrome, and chronic vestibular syndrome. The trained random forest model was applied based on exploratory data analysis results. Results Random forest accuracies on acute, episodic, and chronic vestibular syndrome are 90%, 81.74%, and 91.3%, respectively. The most important features in acute vestibular syndrome are spontaneous nystagmus, and vHIT variables. In episodic vestibular syndrome, unilateral weakness in caloric test, gain and saccades on lateral semicircular canal are the top three parameters. Lateral vHIT gain, head-shaking nystagmus, and unilateral weakness in caloric test are the main parameters on chronic vestibular syndrome. In acute vestibular syndrome, spontaneous nystagmus and vHIT make major contributions in vestibular disorders distinction. When the disease course prolongation, unilateral weakness and head-shaking nystagmus become increasingly important. Conclusion Fast clinical test sets including spontaneous nystagmus, head shaking nystagmus, and vHIT should be the first consideration in screening vestibular disorders.