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Vestibular deficits often lead to unsteady gait, affecting quality of life and increasing fall risk. This study aimed to identify gait impairments in chronic vestibulopathy. Ten patients with bilateral vestibulopathy (BV), 10 patients with chronic unilateral vestibulopathy (UV), and 10 healthy participants (HS) participated. Spatio-temporal parameters were computed during walking at various self-selected walking speeds (slow, comfortable, and fast) using motion capture system with additional assessment usingclinical gait tests [functional gait assessment (FGA), tandem walk (TW), Timed Up and Go test (TUG)], and symptom severity [Dizziness Handicap Inventory (DHI)] were assessed and compared between the three groups. BV and UV patients showed significantly slower walking speeds, shorter step lengths, and broader step widths compared to HS, but similar cadence. Significant differences were also seen in stance phase, double and single support phases at comfortable and slow speeds, but not at fast speed. BV patients, but not UV patients, had worse FGA scores than HS, reflecting their reported difficulties in specific tasks requiring greater postural control. Tandem walk performance was lower in BV patients compared to the other groups, whereas there was no significant differences in TUG scores. Cluster analysis revealed two distinct clusters: one with all HS and most UV patients (70%), and another with most BV patients and 30% of UV. Overall, this study highlights how altered vestibular function impacts gait outcomes. These findings can aid clinicians in evaluating gait in patients with vestibular deficits and monitoring rehabilitation interventions.

[This corrects the article DOI: 10.1371/journal.pone.0301032.].

Chronic imbalance is a major complaint of patients suffering from bilateral vestibulopathy (BV) and is often reported by patients with chronic unilateral vestibulopathy (UV), leading to increased risk of falling. We used the Central SensoriMotor Integration (CSMI) test, which evaluates sensory integration, time delay, and motor activation contributions to standing balance control, to determine whether CSMI measures could distinguish between healthy control (HC), UV, and BV subjects and to characterize vestibular, proprioceptive, and visual contributions expressed as sensory weights. We also hypothesized that sensory weight values would be associated with the results of vestibular assessments (vestibulo ocular reflex tests and Dizziness Handicap Inventory scores). Twenty HCs, 15 UVs and 17 BVs performed three CSMI conditions evoking sway in response to pseudorandom (1) surface tilts with eyes open or, (2) surface tilts with eyes closed, and (3) visual surround tilts. Proprioceptive weights were identified in surface tilt conditions and visual weights were identified in the visual tilt condition. BVs relied significantly more on proprioception. There was no overlap in proprioceptive weights between BV and HC subjects and minimal overlap between UV and BV subjects in the eyes-closed surface-tilt condition. Additionally, visual sensory weights were greater in BVs and were similarly able to distinguish BV from HC and UV subjects. We found no significant correlations between sensory weights and the results of vestibular assessments. Sensory weights from CSMI testing could provide a useful measure for diagnosing and for objectively evaluating the effectiveness of rehabilitation efforts and future treatments designed to restore vestibular function such as hair cell regeneration and vestibular implants.

BackgroundA combined vestibular (VI) and cochlear implant (CI) device, also known as the vestibulocochlear implant (VCI), was previously developed to restore both vestibular and auditory function. A new refined prototype is currently being investigated. This prototype allows for concurrent multichannel vestibular and cochlear stimulation. Although recent studies showed that VCI stimulation enables compensatory eye, body and neck movements, the constraints in these acute study designs prevent them from creating more general statements over time. Moreover, the clinical relevance of potential VI and CI interactions is not yet studied. The VertiGO! Trial aims to investigate the safety and efficacy of prolonged daily motion modulated stimulation with a multichannel VCI prototype.MethodsA single-center clinical trial will be carried out to evaluate prolonged VCI stimulation, assess general safety and explore interactions between the CI and VI. A single-blind randomized controlled crossover design will be implemented to evaluate the efficacy of three types of stimulation. Furthermore, this study will provide a proof-of-concept for a VI rehabilitation program. A total of minimum eight, with a maximum of 13, participants suffering from bilateral vestibulopathy and severe sensorineural hearing loss in the ear to implant will be included and followed over a five-year period. Efficacy will be evaluated by collecting functional (i.e. image stabilization) and more fundamental (i.e. vestibulo-ocular reflexes, self-motion perception) outcomes. Hearing performance with a VCI and patient-reported outcomes will be included as well.DiscussionThe proposed schedule of fitting, stimulation and outcome testing allows for a comprehensive evaluation of the feasibility and long-term safety of a multichannel VCI prototype. This design will give insights into vestibular and hearing performance during VCI stimulation. Results will also provide insights into the expected daily benefit of prolonged VCI stimulation, paving the way for cost-effectiveness analyses and a more comprehensive clinical implementation of vestibulocochlear stimulation in the future.Trial registrationClinicalTrials.gov: NCT04918745. Registered 28 April 2021.

Bilateral vestibulopathy (BVP) is characterized by its heterogeneous and chronic nature with various clinical presentations and multiple etiologies. This current narrative review reflects on the main insights and developments regarding clinical presentation. In addition, it proposes a new diagnostic algorithm, and describes available and potential future therapeutic modalities.

BackgroundThe Bilateral Vestibulopathy Questionnaire (BVQ) is a recently developed 54-item Patient Reported Outcome Measure (PROM) that evaluates the clinically important symptoms of bilateral vestibulopathy (BVP) and its impact on daily life. This study aimed to assess the construct validity and reliability of the BVQ in a large BVP cohort.MethodsPatients diagnosed with BVP were asked to complete a set of questionnaires, including the BVQ, the EuroQol-5D-5L, the Health Utilities Index, the Dizziness Handicap Inventory, the Hospital Anxiety and Depression Scale, and the Oscillopsia Severity Questionnaire. The construct validity of the BVQ was evaluated by confirmatory and exploratory factor analyses (CFA and EFA), followed by hypotheses testing and known groups validity. Structural properties were explored for each individual item. Reliability was assessed by testing the internal consistency of the BVQ constructs (Cronbach's alpha) and test–retest reliability [intraclass correlation coefficients (ICCs)].ResultsA total of 148 patients with BVP (50% women, mean age 66 years) completed the set of questionnaires. The CFA did not show a satisfactory model in the original BVQ. However, the EFA showed a four-factor solution with 20 Likert-scale items related to oscillopsia, imbalance, emotion, and cognition. The succeeding CFA provided evidence for construct validity and an acceptable model of fit. Hypothesis testing confirmed that this shortened version validly measures the constructs to be measured. Statistically significant differences in scores between known groups were found, providing further support for good construct validity. The structural properties were acceptable. Cronbach's alpha confirmed good internal consistency for the four constructs, ranging from 0.80 to 0.89. The ICCs of the 20 Likert-scale items and four visual analog scale (VAS) items were interpreted as good (range 0.76–0.93).ConclusionThis study showed evidence of good construct validity of the new shortened version of the BVQ, consisting of four constructs with a total of 20 Likert-scale items and four VAS items. The final 24-item BVQ proved to be a reliable and valid multi-item PROM that captures the clinically important symptoms of BVP and evaluates its impact on daily life. Consequently, the BVQ enables the gathering of high-level evidence of treatment effectiveness in a systematic and quantitative manner.

ImportanceThe development of a vestibular implant has reached milestones and seems to be a promising therapeutic tool for bilateral vestibulopathy (BV). Given the former lack of therapeutic options for BV, the disease has received scant attention in the previous research literature. It is therefore of major importance to gain more insight into the underlying pathology of BV. Furthermore, as some research groups specifically use a combined vestibulo-cochlear implant, the size of the group of BV patients with associated hearing loss is of special interest.ObjectivesThe study aimed to determine the definite and probable etiology in bilateral vestibulopathy (BV) patients and to report on their hearing status.DesignThis study involves multicenter retrospective study design.SettingThe research setting is at tertiary referral centers.ParticipantsConsecutive BV patients diagnosed at the Antwerp University Hospital between 2004 and 2018 at the Maastricht University Medical Center between 2002 and 2015 and at the Geneva University Hospital between 2013 and 2018, who met the BV diagnostic criteria of the Bárány Society.Main outcome measuresPrimary interests were the etiology and hearing status of BV patients. Moreover, the data of vestibular tests were examined (caloric irrigation, rotatory chair tests, and video-head impulse test).ResultsThe authors identified 315 BV patients, of whom 56% were male patients. Mean age at diagnosis was 58.6 ± 15.1 (range 7–91) years. The definite cause was determined in 37% of the patients and the probable cause in 26% of the patients. No cause was identified in 37% of BV patients. The largest subgroup included patients with genetic etiology (31%), most frequently COCH mutation. Only 21% of patients (n = 61) had bilateral normal hearing. Almost half of the patients (45%, n = 134) had profound hearing loss in at least one ear.ConclusionBV is a heterogeneous condition, with over a third of cases remaining idiopathic, and nearly three-quarters affected by hearing loss. COCH mutation is the most common non-idiopathic cause of BV in our population. Only 21% of our BV patients presented with bilateral normal hearing.

ObjectiveTo systematically evaluate the full spectrum of self-reported chronic symptoms in patients with unilateral vestibular hypofunction (UVH) and to investigate the effect of interventions on these symptoms.MethodsA systematic review was conducted following the guidelines of the Preferred Reporting Items for Systematic Review and Meta-Analysis Statement (PRISMA). A literature search was performed in Pubmed, Web of Science, Embase, and Scopus to investigate self-reported symptoms and self-report questionnaires in patients with UVH. All original studies ranging from full-text clinical trials to case reports, written in English, German, and French, were included. The frequency of self-reported symptoms was presented. For self-report questionnaires, a meta-analysis was carried out to synthesize scale means by the pre- and post-intervention means and mean changes for studies that investigated interventions.ResultsA total of 2,110 studies were retrieved. Forty-seven studies were included after title-abstract selection and full-text selection by two independent reviewers. The symptoms of UVH patients included chronic dizziness (98%), imbalance (81%), symptoms worsened by head movements (75%), visually induced dizziness (61%), symptoms worsened in darkness (51%), and oscillopsia (22%). Additionally, UVH could be accompanied by recurrent vertigo (77%), tiredness (68%), cognitive symptoms (58%), and autonomic symptoms (46%). Regarding self-report questionnaires, UVH resulted on average in a moderate handicap, with an estimated mean total score on the Dizziness Handicap Inventory (DHI) and the Vertigo Symptom Scale (VSS) of 46.31 (95% CI: 41.17–51.44) and 15.50 (95% CI: 12.59–18.41), respectively. In studies that investigated the effect of vestibular intervention, a significant decrease in the estimated mean total DHI scores from 51.79 (95% CI: 46.61–56.97) (pre-intervention) to 27.39 (95% CI: 23.16–31.62) (post intervention) was found (p

Background: A vestibular implant can partially restore vestibular function by providing motion information through implanted electrodes. During vestibular implantation, various obstructions of the semicircular canals, such as protein deposits, fibrosis, and ossification, can be encountered. The objective was to explore the relationship between preoperative imaging and intraoperative findings of semicircular canal obstruction and to develop surgical strategies for dealing with obstructions of the semicircular canal(s) in patients eligible for vestibular implantation. Methods: Patients undergoing vestibulocochlear implantation (in an active clinical trial) were included in the current study when preoperative imaging indicated an obstruction in the semicircular canal. Preoperative imaging consisted of CT and MRI scans. During surgery, the bony semicircular canals were skeletonized ("bluelined") to identify the course of the canals and create a fenestration to insert the electrodes. The aim was to place the electrodes in the semicircular canal ampullae. Surgical strategies were developed to deal with the soft tissue obstructions. These procedures were evaluated intraoperatively with microscopic visualization, postoperatively with CT imaging. Results: The three included patients suffered from bilateral vestibulopathy and hearing loss due to autosomal dominant nonsyndromic sensorineural deafness 9 (DFNA9). A soft tissue obstruction was predicted in one semicircular canal (2 patients) or two semicircular canals (1 patient), based on preoperative imaging. Intraoperatively, bluelining the semicircular canals aided in identifying these locations, by revealing a "whiteline" instead of blueline. Depending on the nature and location of the obstruction, different surgical procedures were employed to facilitate proper electrode insertion. These were as follows: a dummy electrode was used to probe the soft tissue, the obstructive tissue was removed, and/or a bypass fenestration was created. In all patients, the electrodes could be implanted in the semicircular canal ampullae. Based on these first experiences, a diagnostic and surgical guide to deal with obstructions of the semicircular canals during vestibular implantation was developed. Conclusions: Preoperative imaging can indicate locations of obstructions in the SCCs. Different surgical procedures can be applied to enable appropriate electrode positioning in the SCC ampulla. This article describes the first experiences with obstructions of the semicircular canals during intralabyrinthine vestibular implantation and presents a diagnostic and surgical guide. Trial registration: ABR NL73492.068.20, METC20-087 (Maastricht University Medical Center) and NAC 11-080 (Geneva University Hospitals). [ABSTRACT FROM AUTHOR]

IntroductionVestibular deficits are considered rare in children, but the lack of systematic screening leads to underdiagnosis. It has been demonstrated that chronic vestibular dysfunction impacts the normal psychomotor development of children. Early identification is needed to allow for clinical management, ensuring better global development. For this purpose, our research group has developed the Geneva Balance Test (GBT), aiming to objectively quantify the balance capacity of children over a broad age range, to screen for bilateral vestibulopathy (BV), and to quantify the improvement of balance abilities in children.MethodsTo determine the capacity of the GBT to quantify the balance capacity of children with BV, we conducted an observational prospective study with three populations: 11 children with BV, and two age-matched control groups composed of (1) 15 healthy subjects without the vestibular or auditory disorder (HS) and (2) 11 pediatric cochlear implant recipients (CIs) without vestibular disorders. Results of the three populations have been compared in three different age sub- groups (3–5, 6–9, and ≥10 years), and with results of a short, modified version of the Bruininks-Oseretsky test of Motor proficiency Ed. 2 (mBOT-2).ResultsStatistical analyses demonstrated significant differences in the scores of the GBT between children aged 3–5, 6–9, and ≥10 years with BV and in both control populations (HS and CI). BV scores reflected poorer balance capacities at all ages. Children in the youngest CI sub-group (3–5 years) showed intermediate GBT scores but reached HS scores at 6–9 years, reflecting an improvement in their balance capacities. All the results of the GBT were significantly correlated with mBOT-2 results, although only a few BV completed the entire mBOT-2.DiscussionIn this study, the GBT allowed quantifying balance deficits in children with BV. The BOT-2 test is not validated for children

BackgroundTo date, the burden and severity of the full spectrum of bilateral vestibulopathy (BVP) symptoms has not yet been measured in a standardized manner. Since therapeutic interventions aiming to improve BVP symptoms are emerging, the need for a new standardized assessment tool that encompasses the specific aspects of BVP arises. Therefore, the aim of this study was to develop a multi-item Patient Reported Outcome Measure (PROM) that captures the clinically important symptoms of BVP and assesses its impact on daily life.MethodsThe development of the Bilateral Vestibulopathy Questionnaire (BVQ) consisted of two phases: (I) initial item generation and (II) face and content validity testing. Items were derived from a literature review and individual semi-structured interviews focusing on the full spectrum of reported BVP symptoms (I). Subsequently (IIa), individual patient interviews were conducted using “thinking aloud” and concurrent verbal probing techniques to assess the comprehensibility of the instructions, questions and response options, and the relevance, missing domains, or missing items. Interviews continued until saturation of input was reached. Finally, international experts with experience in the field of the physical, emotional, and cognitive symptoms of BVP participated in an online focus group to assess the relevance and comprehensiveness of the BVQ (IIb).ResultsThe BVQ consisted of two sections. The first section included 50 items scored on a six-point Likert scale arranged into seven constructs (i.e., imbalance, oscillopsia, other physical symptoms, cognitive symptoms, emotional symptoms, limitations and behavioral changes and social life). The second section consisted of four items, scored on a visual analog scale from 0 to 100, to inquire about limitations in daily life, perceived health and expectations regarding future recovery. Interviews with BVP patients [n = 8, 50% female, mean age 56 years (range 24–88 years)] and the expert meeting confirmed face and content validity of the developed BVQ.ConclusionThe BVQ, which was developed to assess the spectrum of BVP symptoms and its impact on daily life, proved to have good face and content validity. It can be used to characterize current self-reported symptoms and disability and to evaluate symptom burden before and after therapeutic interventions in future research and clinical practice.

ObjectiveThis study aimed to investigate (1) the patterns of vestibular impairment in bilateral vestibulopathy (BVP) and subsequently, the implications regarding patient eligibility for vestibular implantation, and (2) whether this pattern and severity of vestibular impairment is etiology dependent.MethodsA total of one hundred and seventy-three subjects from three tertiary referral centers in Europe were diagnosed with BVP according to the Bárány Society diagnostic criteria. The subjects underwent vestibular testing such as the caloric test, torsion swing test, video Head Impulse Test (vHIT) in horizontal and vertical planes, and cervical and/or ocular vestibular evoked myogenic potentials (c- and oVEMPs). The etiologies were split into idiopathic, genetic, ototoxicity, infectious, Menière's Disease, (head)trauma, auto-immune, neurodegenerative, congenital, and mixed etiology.ResultsThe caloric test and horizontal vHIT more often indicated horizontal semicircular canal impairment than the torsion swing test. The vHIT results showed significantly higher gains for both anterior canals compared with the horizontal and posterior canals (p < 0.001). The rates of bilaterally absent oVEMP responses were higher compared to the bilaterally absent cVEMP responses (p = 0.010). A total of fifty-four percent of the patients diagnosed with BVP without missing data met all three Bárány Society diagnostic test criteria, whereas 76% of the patients were eligible for implantation according to the vestibular implantation criteria. Regarding etiology, only horizontal vHIT results were significantly lower for trauma, neurodegenerative, and genetic disorders, whereas the horizontal vHIT results were significantly higher for Menière's Disease, infectious and idiopathic BVP. The exploration with hierarchical cluster analysis showed no significant association between etiology and patterns of vestibular impairment.ConclusionThis study showed that caloric testing and vHIT seem to be more sensitive for measuring vestibular impairment, whereas the torsion swing test is more suited for measuring residual vestibular function. In addition, no striking patterns of vestibular impairment in relation to etiology were found. Nevertheless, it was demonstrated that although the implantation criteria are stricter compared with the Bárány Society diagnostic criteria, still, 76% of patients with BVP were eligible for implantation based on the vestibular test criteria. It is advised to carefully examine every patient for their overall pattern of vestibular impairment in order to make well-informed and personalized therapeutic decisions.

Chronic imbalance is a major complaint of patients suffering from bilateral vestibulopathy (BV) and is often reported by patients with chronic unilateral vestibulopathy (UV), leading to increased risk of falling. We used the Central SensoriMotor Integration (CSMI) test, which evaluates sensory integration, time delay, and motor activation contributions to standing balance control, to determine whether CSMI measures could distinguish between healthy control (HC), UV, and BV subjects and to characterize vestibular, proprioceptive, and visual contributions expressed as sensory weights. We also hypothesized that sensory weight values would be associated with the results of vestibular assessments (vestibulo ocular reflex tests and Dizziness Handicap Inventory scores). Twenty HCs, 15 UVs and 17 BVs performed three CSMI conditions evoking sway in response to pseudorandom (1) surface tilts with eyes open or, (2) surface tilts with eyes closed, and (3) visual surround tilts. Proprioceptive weights were identified in surface tilt conditions and visual weights were identified in the visual tilt condition. BVs relied significantly more on proprioception. There was no overlap in proprioceptive weights between BV and HC subjects and minimal overlap between UV and BV subjects in the eyes-closed surface-tilt condition. Additionally, visual sensory weights were greater in BVs and were similarly able to distinguish BV from HC and UV subjects. We found no significant correlations between sensory weights and the results of vestibular assessments. Sensory weights from CSMI testing could provide a useful measure for diagnosing and for objectively evaluating the effectiveness of rehabilitation efforts and future treatments designed to restore vestibular function such as hair cell regeneration and vestibular implants. [ABSTRACT FROM AUTHOR]

Introduction: Different eye movement analysis algorithms are used in vestibular implant research to quantify the electrically evoked vestibulo-ocular reflex (eVOR). Often, standard techniques are used as applied for quantification of the natural VOR in healthy subjects and patients with vestibular loss. However, in previous research, it was observed that the morphology of the VOR and eVOR may differ substantially. In this study, it was investigated if the analysis techniques for eVOR need to be adapted to optimize a truthful quantification of the eVOR (VOR gain, orientation of the VOR axis, asymmetry, and phase shift). Methods: "Natural" VOR responses were obtained in six age-matched healthy subjects, and eVOR responses were obtained in eight bilateral-vestibulopathy patients fitted with a vestibular implant. Three conditions were tested: "nVOR" 1-Hz sinusoidal whole-body rotations of healthy subjects in a rotatory chair, "eVOR" 1-Hz sinusoidal electrical vestibular implant stimulation without whole-body rotations in bilateral-vestibulopathy patients, and "dVOR" 1-Hz sinusoidal whole-body rotations in bilateral-vestibulopathy patients using the chair-mounted gyroscope output to drive the electrical vestibular implant stimulation (therefore also in sync 1 Hz sinusoidal). VOR outcomes were determined from the obtained VOR responses, using three different eye movement analysis paradigms: (1) peak eye velocity detection using the raw eye traces; (2) peak eye velocity detection using full-cycle sine fitting of eye traces; (3) peak eye velocity detection using half-cycle sine fitting of eye traces. Results: The type of eye movement analysis algorithm significantly influenced VOR outcomes, especially regarding the VOR gain and asymmetry of the eVOR in bilateral-vestibulopathy patients fitted with a vestibular implant. Full-cycle fitting lowered VOR gain in the eVOR condition (mean difference: 0.14 ± 0.06 95% CI, p = 0.018). Half-cycle fitting lowered VOR gain in the dVOR condition (mean difference: 0.08 ± 0.04 95% CI, p = 0.009). In the eVOR condition, half-cycle fitting was able to demonstrate the asymmetry between the excitatory and inhibitory phases of stimulation in comparison with the full-cycle fitting (mean difference: 0.19 ± 0.12 95% CI, p = 0.024). The VOR axis and phase shift did not differ significantly between eye movement analysis algorithms. In healthy subjects, no clinically significant effect of eye movement analysis algorithms on VOR outcomes was observed. Conclusion: For the analysis of the eVOR, the excitatory and inhibitory phases of stimulation should be analysed separately due to the inherent asymmetry of the eVOR. A half-cycle fitting method can be used as a more accurate alternative for the analysis of the full-cycle traces. [ABSTRACT FROM AUTHOR]

Cytomegalovirus is the most common cause of congenital infection worldwide. 90 % of children infected in utero are born without symptoms, but 15 % of them will develop disorders within the first five years of life. The most common disorders affect the inner ear, resulting in sensorineural hearing loss and/or vestibular dysfunction (VD). VD is often unrecognized and confused with conditions -affecting the central nervous system. It can cause delays in psychomotor development and predispose to overall developmental delay. Early diagnosis and treatment are essential to prevent or limit these sequelae. Antiviral treatment during the pre- and neonatal periods should be considered., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.