Vestibular Neurology

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neuro-otology and Neuro-ophthalmology".

Deadline for manuscript submissions: closed (30 November 2022) | Viewed by 43291

Special Issue Editors


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Guest Editor
Consultant in Neuro-otology and Audiovestibular Medicine, Attikon Hospital, University of Athens – Honorary post, Leicester University Hospitals, Leicester, UK
Interests: vertigo; balance; vestibular; hearing loss; middle and inner ear surgery; otology; newborn hearing screening; molecular genetics in hearing loss and balance disorders

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Guest Editor
Centre for Vestibular and Behavioural Neurosceinces, University College London, London, UK
Interests: dizziness; vertigo; balance disorders; eye movements; neurology; neuroscience; vestibular

Special Issue Information

Dear Colleagues,

It is our pleasure to introduce a Special Issue of Brain Science dedicated to basic neuroscience and clinical research relating to vestibular neurology and neuro-otology.

The world of dizziness has experienced a dramatic change over the last 3 decades, as new treatable syndromes have been identified, novel genes identified for common balance disorders and novel treatments developed for existing vestibular diagnoses.

The diagnosis and management of dizziness and vertigo remains a challenge worldwide, with a relative paucity of vestibular neurologists, and a real need to translate findings from vestibular neuroscience – from both animal and human models – into the clinical arena. There remains a knowledge gap in understanding the neurobiology of vestibular disorders, from syndromes of “unexplained dizziness” in the elderly through to variants of benign paroxysmal positional vertigo (BPPV), vestibular migraine, and more persistent symptomatic states such as persistent postural perceptual dizziness. Finally, vestibular neurology is rich in clinical bedside skills; indeed, an evaluation of eye movements may more precisely identify and localise a stroke than state-of-the-art imaging.

The aim of this Special Issue is to provide novel insights from clinical and basic research perspectives on vestibular neurology and neuro-otology. A non-exhaustive list of potential papers may include research involving the use of neurophysiology techniques, genetics, pharmacological therapy, physical therapy, psychological intervention in clinical populations, as well as preclinical studies with animal models.

With regard to the format of papers, we will consider research articles, opinion/perspective articles, and review articles (narrative review, systematic review, meta-analysis).

Dr. George S. Korres
Prof. Dr. Diego Kaski
Guest Editors

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Keywords

  • vestibular neuroscience
  • vertigo
  • balance disorders
  • eye movement disorders
  • dizziness
  • genetics of balance disorders
  • immune response in balance disorders

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Published Papers (10 papers)

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Research

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10 pages, 1472 KiB  
Article
Exploring Heading Direction Perception in Cervical Dystonia, Tremor, and Their Coexistence
by Aratrik Guha, Hanieh Agharazi, Palak Gupta and Aasef G. Shaikh
Brain Sci. 2024, 14(3), 217; https://doi.org/10.3390/brainsci14030217 - 27 Feb 2024
Viewed by 1270
Abstract
Objective: Dystonias, characterized by excessive muscle contractions resulting in involuntary postures and movements, impact 3 million people globally, making them the third most common movement disorder. Often accompanied by tremors, dystonias have epidemiological links and non-motor features shared with isolated tremor, such as [...] Read more.
Objective: Dystonias, characterized by excessive muscle contractions resulting in involuntary postures and movements, impact 3 million people globally, making them the third most common movement disorder. Often accompanied by tremors, dystonias have epidemiological links and non-motor features shared with isolated tremor, such as essential tremor. Both dystonia and tremor present with balance dysfunction and abnormal involuntary movements, potentially linked to abnormal cerebellar function. This study explores the perception of one’s own linear movement, heading, particularly discrimination of heading direction, in isolated cervical dystonia, isolated tremor, and their combination. We compare such perception behavior in visual and vestibular domains, predicting that visual heading perception would be superior to vestibular heading perception. Methods: Following the focus on the perception of heading direction, we used psychophysics techniques, such as two-alternative-forced-choice task, to examine perception of direction of one’s own movements as they see isolated visual star-cloud movement (visual heading perception) and en bloc body movement (vestibular heading perception). We fitted a sigmoidal psychometric function curve to determine the threshold for visual or vestibular heading perception in our participants. Results: Nineteen participants underwent a two-alternative forced-choice task in the vestibular and visual domains. Results reveal elevated vestibular heading perception thresholds in cervical dystonia with or without tremor, and isolated tremor compared to healthy controls. Vestibular heading perception threshold was comparable in cervical dystonia with tremor and isolated tremor, but it was even worse in isolated cervical dystonia. Visual heading perception, however, remained less affected all three conditions—isolated cervical dystonia, isolated tremor, and their combination. Conclusion: These findings indicate shared deficits and distinctions in the perception of linear translational heading across movement disorders, such as isolated cervical dystonia, tremor, or their combination, offering insights into their pathophysiology, particularly the involvement of cerebellum regions responsible for vestibular processing. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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11 pages, 1848 KiB  
Article
The Effect of Roll Circular Vection on Roll Tilt Postural Responses and Roll Subjective Postural Horizontal of Healthy Normal Subjects
by Taylor W. Cleworth, John H. J. Allum, Emma I. Nielsen and Mark G. Carpenter
Brain Sci. 2023, 13(11), 1502; https://doi.org/10.3390/brainsci13111502 - 24 Oct 2023
Cited by 1 | Viewed by 1550
Abstract
Background: Falls and related injuries are critical issues in several disease states, as well as aging, especially when interactions between vestibular and visual sensory inputs are involved. Slow support surface tilt (0.6 deg/s) followed by subjective postural horizontal (SPH) assessments have been [...] Read more.
Background: Falls and related injuries are critical issues in several disease states, as well as aging, especially when interactions between vestibular and visual sensory inputs are involved. Slow support surface tilt (0.6 deg/s) followed by subjective postural horizontal (SPH) assessments have been proposed as a viable method for assessing otolith contributions to balance control. Previous assessments of perceived body alignment to vertical, including subjective visual vertical, have suggested that visual inputs are weighted more when vestibular information is near the threshold and less reliable during slow body tilt. To date, no studies have examined the influence of visual stimuli on slow roll-tilt postural responses and the SPH. Therefore, this study investigated how dynamic visual cues, in the form of circular vection (CV), influence postural responses and the perception of the horizontal during and after support surface tilt. Methods: Ten healthy young adults (6 female, mean age 23) wore a head-mounted display while standing on a tilting platform. Participants were asked to remain upright for 30 s, during which (1) the visual scene rotated, inducing roll CV clockwise (CW) or counter-clockwise (CCW) at 60°/s; (2) the platform only (PO) rotated in roll to test SPH (0.6°/s, 2°, CW or CCW); (3) a combination of both; or (4) neither occurred. During SPH trials, participants used a hand-held device to reset the position of the platform to 0.8°/s to their perceived SPH. The angular motion of body segments was measured using pairs of light-emitting diodes mounted on the head, trunk and pelvis. Segment motion, prior to platform motion, was compared to that at peak body motion induced by platform motion and when SPH had been set. Results: When the support surface was tilted 2°, peak upper body tilt significantly increased for congruent CV and platform tilt and decreased at the pelvis for incongruent CV when compared to PO, leading to significant differences across body segments for congruent and incongruent conditions (p ≤ 0.008). During PO, participants’ mean SPH deviated from horizontal by 0.2°. The pelvis deviated 0.2°, the trunk 0.3°, and the head 0.5° in the direction of initial platform rotation. When platform tilt and CV directions were congruent or incongruent, only head tilt at SPH reset under congruent conditions was significantly different from the PO condition (1.7° vs. 0.5°). Conclusions: Roll CV has a significant effect on phasic body responses and a less significant effect on tonic body responses to lateral tilt. The SPH of the support surface was not altered by CV. Responses during tilt demonstrated enhanced reactions for congruent and reduced reactions for incongruent CV, both different from responses to CV alone. Tonic body displacements associated with SPH were changed less than those during tilt and were only slightly larger than displacements for CV alone. This study supports the hypothesis of weighted multisensory integration during dynamic postural tasks being highly dependent on the direction of visual cues during tilt and less dependent on tonic SPH offsets. These techniques could be used to examine vestibular and visual interactions within clinical populations, particularly those with visual vertigo and dizziness. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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18 pages, 4115 KiB  
Article
The Effects of Vibro-Tactile Biofeedback Balance Training on Balance Control and Dizziness in Patients with Persistent Postural-Perceptual Dizziness (PPPD)
by Claudia Candreia, Heiko M. Rust, Flurin Honegger and John H. J. Allum
Brain Sci. 2023, 13(5), 782; https://doi.org/10.3390/brainsci13050782 - 11 May 2023
Cited by 3 | Viewed by 2988
Abstract
Background: Patients with persistent postural-perceptual dizziness (PPPD) frequently report having problems with balance control. Artificial systems providing vibro-tactile feedback (VTfb) of trunk sway to the patient could aid recalibration of “falsely” programmed natural sensory signal gains underlying unstable balance control and dizziness. Thus, [...] Read more.
Background: Patients with persistent postural-perceptual dizziness (PPPD) frequently report having problems with balance control. Artificial systems providing vibro-tactile feedback (VTfb) of trunk sway to the patient could aid recalibration of “falsely” programmed natural sensory signal gains underlying unstable balance control and dizziness. Thus, the question we examine, retrospectively, is whether such artificial systems improve balance control in PPPD patients and simultaneously reduce the effects of dizziness on their living circumstances. Therefore, we assessed in PPPD patients the effects of VTfb of trunk sway on balance control during stance and gait tests, and on their perceived dizziness. Methods: Balance control was assessed in 23 PPPD patients (11 of primary PPPD origin) using peak-to-peak amplitudes of trunk sway measured in the pitch and roll planes with a gyroscope system (SwayStar™) during 14 stance and gait tests. The tests included standing eyes closed on foam, walking tandem steps, and walking over low barriers. The measures of trunk sway were combined into a Balance Control Index (BCI) and used to determine whether the patient had a quantified balance deficit (QBD) or dizziness only (DO). The Dizziness Handicap Inventory (DHI) was used to assess perceived dizziness. The subjects first underwent a standard balance assessment from which the VTfb thresholds in eight directions, separated by 45 deg, were calculated for each assessment test based on the 90% range of the trunk sway angles in the pitch and roll directions for the test. A headband-mounted VTfb system, connected to the SwayStar™, was active in one of the eight directions when the threshold for that direction was exceeded. The subjects trained for 11 of the 14 balance tests with VTfb twice per week for 30 min over a total of 2 consecutive weeks. The BCI and DHI were reassessed each week and the thresholds were reset after the first week of training. Results: On average, the patients showed an improved balance control in the BCI values after 2 weeks of VTfb training (24% p = 0.0001). The improvement was greater for the QBD patients than for the DO patients (26 vs. 21%), and greater for the gait tests than the stance tests. After 2 weeks, the mean BCI values of the DO patients, but not the QBD patients, were significantly less (p = 0.0008) than the upper 95% limit of normal age-matched reference values. A subjective benefit in balance control was spontaneously reported by 11 patients. Lower (36%), but less significant DHI values were also achieved after VTfb training (p = 0.006). The DHI changes were identical for the QBD and DO patients and approximately equal to the minimum clinical important difference. Conclusions: These initial results show, as far as we are aware for the first time, that providing VTfb of trunk sway to PPPD subjects yields a significant improvement in balance control, but a far less significant change in DHI-assessed dizziness. The intervention benefitted the gait trials more than the stance trials and benefited the QBD group of PPPD patients more than the DO group. This study increases our understanding of the pathophysiologic processes underlying PPPD and provides a basis for future interventions. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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10 pages, 1365 KiB  
Article
Early Diagnosis of Central Disorders Mimicking Horizontal Canal Cupulolithiasis
by Paula Peña Navarro, Sofía Pacheco López, Cristina Nicole Almeida Ayerve, Susana Marcos Alonso, José Manuel Serradilla López, Santiago Santa Cruz Ruiz, José Carlos Gómez Sánchez, Diego Kaski and Ángel Batuecas Caletrío
Brain Sci. 2023, 13(4), 562; https://doi.org/10.3390/brainsci13040562 - 27 Mar 2023
Cited by 4 | Viewed by 2625
Abstract
Background: Horizontal Canal Cupulolithiasis (hc-BPPV-cu) can mimic a pathology of central origin, so a careful examination is essential to prevent misdiagnosis. Methods: Retrospective cross-sectional cohort study of 45 patients suffering from suspected hc-BPPV-cu. We recorded whether patients first presented through an ENT Emergency [...] Read more.
Background: Horizontal Canal Cupulolithiasis (hc-BPPV-cu) can mimic a pathology of central origin, so a careful examination is essential to prevent misdiagnosis. Methods: Retrospective cross-sectional cohort study of 45 patients suffering from suspected hc-BPPV-cu. We recorded whether patients first presented through an ENT Emergency Department (ED) or through an Outpatient Otolaryngology Clinic (OC). Results: We found statistically significant differences (p < 0.05) between the OC versus the ED in relation to the time between symptom onset and first assessment (79.7 vs. 3.6 days, respectively), the number of therapeutic maneuvers (one maneuver in 62.5% vs. 75.9%, and more than one in 25.1% vs. 13.7%), and multi-canal BPPV rate (43.8% vs. 3.4%). hc-BPPV-cu did not resolve in 2 patients (12.5%) from the OC and in 3 (10.3%) from de ED, all of which showed central pathology. Discussion: There are no prior studies that analyze the approach to hc-BPPV-cu in the ED. The benefits of early specialist input are early identification of central positional nystagmus, a decrease in symptom duration, reduced number of therapeutic maneuvers required for symptom resolution, and lower rates of iatrogenic multi-canal BPPV. Conclusion: A comprehensive approach to hc-BPPV-cu in the ED allows both more effective treatment and early identification of central disorder mimics. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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7 pages, 6345 KiB  
Communication
Patient Experience of Flunarizine for Vestibular Migraine: Single Centre Observational Study
by Sk Mamun Ur Rashid, Sheetal Sumaria, Nehzat Koohi, Qadeer Arshad and Diego Kaski
Brain Sci. 2022, 12(4), 415; https://doi.org/10.3390/brainsci12040415 - 22 Mar 2022
Cited by 12 | Viewed by 4451
Abstract
Vestibular migraine (VM) is a leading cause of episodic vertigo, affecting up to 1% of the general population. Despite established diagnostic criteria, there is currently no evidence-based approach for acute treatment of VM, with treatment recommendations generally extrapolated from studies on classical migraine [...] Read more.
Vestibular migraine (VM) is a leading cause of episodic vertigo, affecting up to 1% of the general population. Despite established diagnostic criteria, there is currently no evidence-based approach for acute treatment of VM, with treatment recommendations generally extrapolated from studies on classical migraine headache. Several small-scale studies have identified flunarizine as a potentially effective prophylactic medication in VM. We conducted a single-centre observational service evaluation study exploring patient experiences of preventative medications over a 28-month period, including flunarizine, for control of VM symptoms. To compare patient experience of flunarizine with other medications, data from patients taking flunarizine were separately analysed. A total of 90% of VM patients taking flunarizine reported symptomatic improvement, compared to only 32% of patients on other medications. Whilst 50% of patients on flunarizine reported side effects. these were not deemed to outweigh the clinical benefits, with most patients deciding to continue treatment. Our data supports the use of flunarizine in VM. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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10 pages, 4110 KiB  
Communication
Histopathological Investigation of Dura-like Membrane in Vestibular Schwannomas
by Yumiko Oishi, Ryota Tamura, Kazunari Yoshida and Masahiro Toda
Brain Sci. 2021, 11(12), 1649; https://doi.org/10.3390/brainsci11121649 - 15 Dec 2021
Cited by 2 | Viewed by 2270
Abstract
The dura-like membrane (DLM) is an outermost membranous structure arising from the dura mater adjacent to the internal auditory meatus (IAM) that envelops some vestibular schwannomas (VSs). Its recognition is important for the preservation of the facial and cochlear nerves during tumor resection. [...] Read more.
The dura-like membrane (DLM) is an outermost membranous structure arising from the dura mater adjacent to the internal auditory meatus (IAM) that envelops some vestibular schwannomas (VSs). Its recognition is important for the preservation of the facial and cochlear nerves during tumor resection. This study analyzes the histopathological characteristics of the DLM. The expression of CD34 and αSMA was histopathologically analyzed in tumor and DLM tissue of 10 primary VSs with and without a DLM. Tumor volume, resection volume percentage, microvessel density (MVD), and vessel diameter were analyzed. Volumetric analysis revealed that the presence of a DLM was significantly associated with lower tumor resection volume (p < 0.05). Intratumoral vessel diameter was significantly larger in the DLM group than the non-DLM group (p < 0.01). Larger VSs showed a higher intratumoral MVD in the DLM group (p < 0.05). Multilayered αSMA-positive vessels were identified in the DLM, tumor, and border; there tended to be more of these vessels within the tumor in the DLM group compared to the non-DLM group (p = 0.08). These arteriogenic characteristics suggest that the DLM is formed as the tumor induces feeding vessels from the dura mater around the IAM. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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Review

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10 pages, 985 KiB  
Review
The Prevalence of Dizziness and Vertigo in COVID-19 Patients: A Systematic Review
by George Korres, Dimitrios K. Kitsos, Diego Kaski, Anthi Tsogka, Sotirios Giannopoulos, Vasileios Giannopapas, Giorgos Sideris, Giorgos Tyrellis and Konstantine Voumvourakis
Brain Sci. 2022, 12(7), 948; https://doi.org/10.3390/brainsci12070948 - 20 Jul 2022
Cited by 15 | Viewed by 6758
Abstract
Clinical manifestations of COVID-19 include symptoms of vertigo and dizziness, which is rather unsurprising, since SARS-CoV-2 neurotropism may inflict a broad spectrum of neuropathic effects. The widespread nature of central and peripheral audiovestibular pathways suggests that there may be several probable pathophysiological mechanisms. [...] Read more.
Clinical manifestations of COVID-19 include symptoms of vertigo and dizziness, which is rather unsurprising, since SARS-CoV-2 neurotropism may inflict a broad spectrum of neuropathic effects. The widespread nature of central and peripheral audiovestibular pathways suggests that there may be several probable pathophysiological mechanisms. The cytokine storm, CNS infiltration of the virus through ACE 2 receptors, and other systemic factors can be responsible for the significant number of COVID-19 patients reported to experience symptoms of vertigo and dizziness. In this paper, we present a systematic review of clinical studies reporting the detection of dizziness and vertigo as clinical manifestations of COVID-19 and discuss their etiopathogenesis. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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Other

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11 pages, 5788 KiB  
Perspective
Persistent Postural-Perceptual Dizziness (PPPD) from Brain Imaging to Behaviour and Perception
by Patricia Castro, Matthew J. Bancroft, Qadeer Arshad and Diego Kaski
Brain Sci. 2022, 12(6), 753; https://doi.org/10.3390/brainsci12060753 - 8 Jun 2022
Cited by 15 | Viewed by 10699
Abstract
Persistent postural-perceptual dizziness (PPPD) is a common cause of chronic dizziness associated with significant morbidity, and perhaps constitutes the commonest cause of chronic dizziness across outpatient neurology settings. Patients present with altered perception of balance control, resulting in measurable changes in balance function, [...] Read more.
Persistent postural-perceptual dizziness (PPPD) is a common cause of chronic dizziness associated with significant morbidity, and perhaps constitutes the commonest cause of chronic dizziness across outpatient neurology settings. Patients present with altered perception of balance control, resulting in measurable changes in balance function, such as stiffening of postural muscles and increased body sway. Observed risk factors include pre-morbid anxiety and neuroticism and increased visual dependence. Following a balance-perturbing insult (such as vestibular dysfunction), patients with PPPD adopt adaptive strategies that become chronically maladaptive and impair longer-term postural behaviour. In this article, we explore the relationship between behavioural postural changes, perceptual abnormalities, and imaging correlates of such dysfunction. We argue that understanding the pathophysiological mechanisms of PPPD necessitates an integrated methodological approach that is able to concurrently measure behaviour, perception, and cortical and subcortical brain function. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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20 pages, 3848 KiB  
Case Report
Autoimmune Vestibulopathy—A Case Series
by Surangi Mendis, Nicola Longley, Simon Morley, George Korres and Diego Kaski
Brain Sci. 2022, 12(3), 306; https://doi.org/10.3390/brainsci12030306 - 24 Feb 2022
Cited by 6 | Viewed by 5608
Abstract
Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed [...] Read more.
Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed investigation given that the auditory symptoms and their responsiveness to immunosuppression are the focus of the few proposed diagnostic criteria for AIED. We present three cases of vestibulopathy occurring in the context of autoimmune inner ear conditions, including the first known report of autoimmune inner ear pathology arising with a temporal association to administration of the Pfizer-BioNTech SARS-CoV2 vaccination. We review the available literature pertinent to each case and summarise the key learning points, highlighting the variable presentation of vestibular impairment in AIED. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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8 pages, 976 KiB  
Case Report
Recovery Nystagmus in Vestibular Neuritis with Minimal Canal Paresis. Clinical Observation and Interpretation
by Eleni Zoe Gkoritsa
Brain Sci. 2022, 12(1), 110; https://doi.org/10.3390/brainsci12010110 - 14 Jan 2022
Cited by 2 | Viewed by 2961
Abstract
Recovery nystagmus in vestibular neuritis patients is a reversal of spontaneous nystagmus direction, beating towards the affected ear, observed along the time course of central compensation. It is rarely registered due either to its rarity as a phenomenon per se, or to the [...] Read more.
Recovery nystagmus in vestibular neuritis patients is a reversal of spontaneous nystagmus direction, beating towards the affected ear, observed along the time course of central compensation. It is rarely registered due either to its rarity as a phenomenon per se, or to the fact that it is missed between follow-up appointments. The aim of the manuscript is to describe in detail a case of recovery nystagmus found in an atypical case of vestibular neuritis and discuss pathophysiology and clinical considerations regarding this rare finding. A 26-year-old man was referred to our Otorhinolaryngology practice reporting “dizziness” sensation and nausea in the last 48 h. Clinical examination revealed left beating spontaneous nystagmus (average slow phase velocity aSPV 8.1°/s) with absence of fixation. The head impulse test (H.I.T.) was negative. Cervical vestibular evoked myogenic potentials (cVEMP) and Playtone audiometry (PTA) were normal. Romberg and Unterberger tests were not severely affected. A strong directional preponderance to the left was found in caloric vestibular test with minimal canal paresis (CP 13%) on the right. The first follow-up consultation took place on the 9th day after the onset of symptoms. Right beating weak (aSPV 2.4°/s) spontaneous nystagmus was observed with absence of fixation, whereas a strong right directional preponderance (DP) was found in caloric vestibular test. A brain MRI scan was ordered to exclude central causes of vertigo, which was normal. The patient was seen again completely free of symptoms 45 days later. He reported feeling dizzy during dynamic movements of the head and trunk for another 15 days after his second consultation. The unexpected observation of nystagmus direction reversal seven days after the first consultation is a typical sign of recovery nystagmus. Recovery nystagmus (RN) is centrally mediated and when found, it should always be carefully assessed in combination with the particularities of vestibular neuritis. Full article
(This article belongs to the Special Issue Vestibular Neurology)
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