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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.

IntroductionThe vestibular implant is a neuroprosthesis which offers a potential treatment approach for patients suffering from vestibulopathy. Investigating the influence of electrical stimulation parameters is essential to improve the vestibular implant response. Optimization of the response focuses on the electrically evoked vestibulo-ocular reflex. It aims to facilitate high peak eye velocities and adequate alignment of the eye movement responses. In this study, the basic stimulation parameters of the vestibular implant were tested for their effect on the electrically evoked vestibulo-ocular reflex.MethodsFour stimulation parameters, including the stimulation amplitude, phase duration, stimulus rate and speed of change of stimulation, were systematically tested in a cohort of nine subjects with a vestibulo-cochlear implant. These parameters were tested to evaluate their effect on fitting settings (i.e., threshold of activation, upper comfortable limit and dynamic range) as well as on the electrically evoked vestibulo-ocular reflex (peak eye velocity and alignment).ResultsIt was confirmed that, in addition to current amplitude, the peak eye velocity of the response can be increased by increasing the phase duration and pulse rate. Both parameters have little effect on the alignment of the eye response. However, a longer phase duration decreased the range between the threshold of activation and the upper comfortable limit of the electrical stimulation (i.e., dynamic range). Furthermore, these results show that next to the amplitude of the stimulation, the speed of change in stimulation has a determinative positive effect on the peak eye velocity.ConclusionThe observations in this study imply that the vestibular implant response, in terms of peak eye velocity, can be optimized with a higher pulse rate and longer phase duration. However, this comes at a trade-off between the dynamic range and power consumption. This study provides essential insights for fitting strategies in future vestibular implant care.

ObjectiveTo investigate the effect of daily use of subconscious vibrotactile stimulation in bilateral vestibulopathy (BVP) patients, who judged the effect of vestibular rehabilitation as insufficient.MethodsBVP patients were asked to wear a subconscious vibrotactile stimulation belt for 2 h. Patients who reported benefit after 2 h of use, were instructed to wear a subconscious vibrotactile stimulation belt in daily life, for up to more than 2 years. Follow-up consultations (mostly by telephone calls) were scheduled after 2 weeks, 2 months, 1 year, and 2 years of use. During these consultations, adherence and the self-reported overall Balance and Mobility Score (BMS) were evaluated.ResultsOne hundred twenty-one BVP patients were included. Regarding adherence, 74% of patients (n = 89) wanted to proceed with daily use at home after 2 h of try out. Of these patients, 90% (n = 80) was still wearing the belt daily after 2 months, and at least 81% (n = 72) after 1 year and 73% (n = 65) after 2 years. It should be noted that lack of adherence after 1 and 2 years resulted from a loss to follow-up. All patients responding to telephone consultations in the 2 years follow up were wearing a subconscious vibrotactile stimulation belt daily. The median BMS score significantly improved within 2 h of use, from 4 to 6 points (p < 0.0001). Compared to baseline, the median BMS score significantly improved with >=3 points after 2 weeks, 2 months, 1 year, and 2 years of daily use (p < 0.0001). Long-term adherence was high in patients who experienced an increase of two or more points on the BMS, after 2 weeks of daily use.ConclusionThe Subconscious vibrotactile stimulation improves self-reported balance and mobility in a subgroup of motivated BVP patients in which vestibular rehabilitation is insufficient.

Vestibular migraine (VM) and Menière’s disease (MD) are characterized by episodes of vertigo of similar duration. It is well known that differentiation between both diseases is not always possible based only on the patient history, physical examination, and audiological testing. In addition, the quantification of the vestibular function can also be helpful since, among patients with MD, there is often a dissociation between a normal/pseudo-normal video head impulse test (vHIT) and reduced caloric testing. The goal of this confirmatory study was to determine the sensitivity, specificity, and positive and negative predictive values (PPV and NPV) of this dissociation to differentiate between MD and VM as well as between MD and other vestibular diseases. We performed a retrospective analysis of 2,101 patients. The examination group consisted of 1,100 patients; of these, 627 (57%) had MD according to the diagnostic criteria of the Bárány Society and 473 (43%) had VM. The comparison group consisted of 1,001 patients with other peripheral, central, or functional vestibular disorders. Statistical analysis revealed the following findings for the dissociation: MD vs. VM: specificity: 83.5%, sensitivity: 58.9%, PPV: 82.6%, and NPV: 60.5%, and MD vs. all other vestibular disorders (VM plus others): specificity: 83.5%, sensitivity: 58.9%, PPV: 60.3%, and NPV: 82.7%. The dissociation between a normal vHIT and a reduced caloric response is due to the high specificity and PPV suited for the differentiation between MD and VM. This part of the study confirms previous findings in a large cohort of patients. When it comes to differentiating between MD and all observed vestibular disorders, if there is no dissociation, the diagnosis of MD is unlikely.

IntroductionVestibular assessment in children with sensorineural hearing loss (SNHL) is critical for early vestibular rehabilitation therapy to promote (motor) development or guide decision making towards cochlear implantation (timing of surgery and laterality). It can be challenging from a clinical viewpoint to decide which vestibular tests should be performed for a pediatric patient. The aim of this study was to evaluate the diagnostic accuracy of several clinically available vestibular tests in children with SNHL, and to provide recommendations for the implementation of vestibular testing of children in clinical practice, to screen for vestibular hypofunction (VH).MethodsA two-center retrospective chart review was conducted. Eighty-six patients between the age of 0 and 18 years were included in this study with SNHL. Vestibular tests included video headimpulse test (VHIT), caloric test (performed at the age of four or higher), rotatory chair and cervical vestibular evoked myogenic potential (cVEMP). A combination of the clinical assessment and (combinations of) vestibular test outcomes determined the diagnosis. The diagnostic quality of tests and combination of tests was assessed by diagnostic accuracy, sensitivity and specificity.ResultsVH was diagnosed in 44% of the patients. The VHIT and caloric test showed the highest diagnostic accuracy compared to the rotatory chair and cVEMP. All combinations of VHIT, caloric test and cVEMP showed improvement of the diagnostic accuracy compared to the respective tests when performed singularly. All combinations of tests showed a relatively similar diagnostic accuracy, with the VHIT combined with the caloric test scoring the highest. Adding a third test did not substantially improve the diagnostic accuracy.DiscussionVestibular testing is feasible and VH is highly prevalent in children with SNHL. A proposed diagnostic algorithm recommends starting with VHIT, followed by cVEMP for children under the age of four, and caloric testing for older children if VH is not confirmed with the first test. Performing a third test is redundant as the diagnostic accuracy does not improve substantially. However, challenges remain, including the lack of a gold standard and the subjective nature of the diagnosis, highlighting the need for standardized testing and increased understanding of VH in this population.

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.

IntroductionPatients with chronic vestibular hypofunction typically suffer from dizziness, imbalance and oscillopsia (blurred vision); symptoms that pose challenges to everyday life. Currently, advice on how to deal with such challenges is mainly provided by health care professionals (i.e., ENT-surgeons, neurologists, physiotherapists and psychologists). However, fellow patients with a similar condition and a true appreciation of the lived experiences, are likely to provide valuable support and advice as well. The purpose of this study, therefore, was to collect tips and advice from patients with chronic vestibular hypofunction.MethodsAn exploratory survey was designed to collect tips from fellow chronic vestibular hypofunction patients on how to cope with disease-related challenges in everyday life. The survey was distributed both online and in person. The list of tips was coded and analyzed thematically and deductively, by using the international classification of functioning, disability, and health (ICF) model.ResultsIn total, 425 tips were obtained from the 179 participants. Most tips were coded under “environmental factors” (46%) and “activities and participation” (39%). The remaining tips were categorized as “body functions” (15%). No tips were about “body structures.” The participants coped with their daily struggles by investing in assistive products and technology, like adapted bikes, special footwear, walking frames. They described the importance of ensuring minimal light intensity for visibility (i.e., installing light sources in dark places). During activities, participants gave the advice to avoid bumpy roads and obstacles, and highlighted the necessity of adequate visual fixation to maintain balance. To ensure optimal activity, participants emphasized the importance of managing energy and taking sufficient rest.DiscussionThis study gives insight into how patients with chronic vestibular hypofunction cope with everyday struggles due to their symptoms. These tips can expand advice given by healthcare professionals. Knowing that fellow patients experience similar struggles and learned to deal with their struggles in adequate ways, might offer support and help patients focus on possibilities rather than on disabilities. Further research should investigate the effect of sharing tips to see whether improvement in (mental) health can be achieved in patients with chronic vestibular hypofunction.

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

A 29-year-old woman, known with polyostotic fibrous dysplasia, presented with facial palsy, vertigo, and ipsilateral neck pain. Radiology revealed a large temporal bone cyst, extruding intracranially, eroding the labyrinth and Fallopian canal. Histopathology of the cyst wall retrieved through transmastoidal biopsy showed a mutation in the Gs-α gene, confirming cystic degeneration of fibrous dysplasia, making this a unique case in which both the inner ear and facial canal are affected by this condition.

IntroductionVideo head impulse testing is frequently used to evaluate the vestibular function. During this test, eye movement responses are recorded with video-oculography (VOG). However, the use of VOG can sometimes be challenging, especially due to pupil detection problems (e.g., blinking, droopy eyelids, etc.). Therefore, this study investigated whether electro-oculography (EOG), a technique that does not depend on pupil tracking but on the orientation of the corneoretinal potential, might be an alternative to VOG for quantifying eye movement responses during head impulse testing.Subjects and methodsHead impulse testing was performed in 19 healthy subjects without a prior history of vestibular symptoms. Horizontal eye movements were recorded simultaneously with EOG (using an EOG system) and VOG (using a VHIT system: ICS Impulse). The eye movement responses to each side of both techniques were compared using a concordance correlation coefficient (rc), t-testing, and Bayes Factor (BF) paired t-testing.ResultsEOG and VOG obtained eye movement traces that correlated well with each other during head impulse testing (average rc = 0.89). Average VOR gains obtained with EOG and VOG were not significantly different from each other for all subjects during left head impulses. However, VOG gains differed between both techniques regarding right head impulses. VOG showed significant VOR gain asymmetry (5% to the right), whereas EOG showed no significant asymmetry (1% to the right).ConclusionThis study demonstrated the use of EOG to record eye movements during head impulse testing for the first time. EOG and VOG obtained eye movement traces that correlated well with each other during horizontal head impulse testing. In addition, EOG showed smaller VOR gain asymmetry in healthy individuals, in contrast to VOG. These findings indicate that EOG might potentially be applicable as an alternative to VOG for collecting eye movement responses during head impulse testing.Trial registration number10192021-38 dated 19.10.21.

IntroductionVideo head impulse testing is frequently used to evaluate the vestibular function. During this test, eye movement responses are recorded with video-oculography (VOG). However, the use of VOG can sometimes be challenging, especially due to pupil detection problems (e.g., blinking, droopy eyelids, etc.). Therefore, this study investigated whether electro-oculography (EOG), a technique that does not depend on pupil tracking but on the orientation of the corneoretinal potential, might be an alternative to VOG for quantifying eye movement responses during head impulse testing.Subjects and methodsHead impulse testing was performed in 19 healthy subjects without a prior history of vestibular symptoms. Horizontal eye movements were recorded simultaneously with EOG (using an EOG system) and VOG (using a VHIT system: ICS Impulse). The eye movement responses to each side of both techniques were compared using a concordance correlation coefficient (rc), t-testing, and Bayes Factor (BF) paired t-testing.ResultsEOG and VOG obtained eye movement traces that correlated well with each other during head impulse testing (average rc = 0.89). Average VOR gains obtained with EOG and VOG were not significantly different from each other for all subjects during left head impulses. However, VOG gains differed between both techniques regarding right head impulses. VOG showed significant VOR gain asymmetry (5% to the right), whereas EOG showed no significant asymmetry (1% to the right).ConclusionThis study demonstrated the use of EOG to record eye movements during head impulse testing for the first time. EOG and VOG obtained eye movement traces that correlated well with each other during horizontal head impulse testing. In addition, EOG showed smaller VOR gain asymmetry in healthy individuals, in contrast to VOG. These findings indicate that EOG might potentially be applicable as an alternative to VOG for collecting eye movement responses during head impulse testing.Trial registration number10192021-38 dated 19.10.21.

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.

Objective: To assess the prevalence of each symptom listed in the acronym DISCOHAT (worsening of symptoms in Darkness and/or uneven ground, Imbalance, Supermarket effect, Cognitive complaints, Oscillopsia, Head movements worsen symptoms, Autonomic complaints, and Tiredness) in patients with bilateral vestibulopathy (BVP), compared to patients with unilateral vestibulopathy (UVP).Methods: A descriptive case-control study was performed on BVP and UVP patients who were evaluated for their vestibular symptoms by two of the authors (RvdB, MCG) at a tertiary referral center, between 2017 and 2020. During history taking, the presence of each DISCOHAT symptom was checked and included in the electronic health record. Presence of a symptom was categorized into: “present,” “not present,” and “missing.”Results: Sixty-six BVP patients and 144 UVP patients were included in this study. Prevalence of single DISCOHAT symptoms varied from 52 to 92% in BVP patients and 18–75% in UVP patients. Patients with BVP reported “worsening of symptoms in darkness,” “imbalance,” “oscillopsia,” and “worsening of symptoms with fast head movements” significantly more than UVP patients (p ≤ 0.004).Conclusion: The DISCOHAT acronym is able to capture a wide spectrum of symptoms related to vestibulopathy, while it is easy and quickly to use in clinic. Application of this acronym might facilitate a more thorough and uniform assessment of bilateral vestibulopathy, within and between vestibular clinics worldwide.

Objective: This study aimed to identify differences in vestibulo-ocular reflex gain (VOR gain) and saccadic response in the suppression head impulse paradigm (SHIMP) between predictable and less predictable head movements, in a group of healthy subjects. It was hypothesized that higher prediction could lead to a lower VOR gain, a shorter saccadic latency, and higher grouping of saccades.Methods: Sixty-two healthy subjects were tested using the video head impulse test and SHIMPs in four conditions: active and passive head movements for both inward and outward directions. VOR gain, latency of the first saccade, and the level of saccade grouping (PR-score) were compared among conditions. Inward and active head movements were considered to be more predictable than outward and passive head movements.Results: After validation, results of 57 tested subjects were analyzed. Mean VOR gain was significantly lower for inward passive compared with outward passive head impulses (p < 0.001), and it was higher for active compared with passive head impulses (both inward and outward) (p ≤ 0.024). Mean latency of the first saccade was significantly shorter for inward active compared with inward passive (p ≤ 0.001) and for inward passive compared with outward passive head impulses (p = 0.012). Mean PR-score was only significantly higher in active outward than in active inward head impulses (p = 0.004).Conclusion: For SHIMP, a higher predictability in head movements lowered gain only in passive impulses and shortened latencies of compensatory saccades overall. For active impulses, gain calculation was affected by short-latency compensatory saccades, hindering reliable comparison with gains of passive impulses. Predictability did not substantially influence grouping of compensatory saccades.

Patients with bilateral vestibulopathy (BVP) present with unsteadiness during standing and walking, limiting their activities of daily life and, more importantly, resulting in an increased risk of falling. In BVP patients, falls are considered as one of the major complications, with patients having a 31-fold increased risk of falling compared to healthy subjects. Thus, highlighting objective measures that can easily and accurately assess the risk of falling in BVP patients is an important step in reducing the incidence of falls and the accompanying burdens. Therefore, this study investigated the interrelations between demographic characteristics, vestibular function, questionnaires on self-perceived handicap and balance confidence, clinical balance measures, gait variables, and fall status in 27 BVP patients. Based on the history of falls in the preceding 12 months, the patients were subdivided in a “faller” or “non-faller” group. Results on the different outcome measures were compared between the “faller” and “non-faller” subgroups using Pearson's chi-square test in the case of categorical data; for continuous data, Mann–Whitney U test was used. Performances on the clinical balance measures were comparable between fallers and non-fallers, indicating that, independent from fall status, the BVP patients present with an increased risk of falling. However, fallers tended to report a worse self-perceived handicap and confidence during performing activities of daily life. Spatiotemporal parameters of gait did not differ between fallers and non-fallers during walking at slow, preferred, or fast walking speed. These results may thus imply that, when aiming to distinguish fallers from non-fallers, the BVP patients' beliefs concerning their capabilities may be more important than the moderately or severely affected physical performance within a clinical setting. Outcome measures addressing the self-efficacy and fear of falling in BVP patients should therefore be incorporated in future research to investigate whether these are indeed able to distinguish fallers form non-fallers. Additionally, information regarding physical activity could provide valuable insights on the contextual information influencing behavior and falls in BVP.

Introduction: Superior canal dehiscence syndrome (SCDS) is a condition characterized by a defect in the bone overlying the superior semicircular canal, creating a third mobile window into the inner ear. Patients can experience disabling symptoms and opt for surgical management. Limited data are available on the impact of SCDS on health-related quality of life (HRQoL) and disease-specific HRQoL more specifically.Objective: To perform a prospective analysis on generic HRQoL in SCDS patients compared to healthy age-matched controls.Methods: A prospective study was performed on patients diagnosed with SCDS and who did not undergo reconstructive surgery yet. Patients were recruited between November 2017 and January 2020 and asked to complete the Health Utility Index (HUI) Mark 2 (HUI2)/Mark 3 (HUI3) questionnaire. For the control group, age-matched participants without otovestibular pathology or other chronic pathology were recruited. The multi-attribute utility function (MAUF) score was calculated for the HUI2 and HUI3. Results of both groups were compared using the Mann-Whitney U test.Results: A total of 20 patients completed the questionnaire. Age ranged from 37 to 79 years with a mean age of 56 years (45% males and 55% females). The control group consisted of 20 participants with a mean age of 56.4 years and ranged from 37 to 82 years (35% males and 65% females). For the case group, median HUI2 MAUF score was 0.75 and median HUI3 MAUF score was 0.65. For the control group, the median scores were 0.88 and 0.86 respectively. There was a statistically significant difference for both HUI2 (p = 0.024) and HUI3 (p = 0.011). SCDS patients had a worse generic HRQoL than age-matched healthy controls. One patient with unilateral SCDS had a negative HUI3 MAUF score (−0.07), indicating a health-state worse than death.Conclusion: SCDS patients have significantly lower health utility values than an age-matched control group. This confirms the negative impact of SCDS on generic HRQoL, even when using an instrument that is not designed to be disease-specific but to assess health state in general. These data can be useful to compare impact on HRQoL among diseases.

Background: Previous studies have demonstrated spatial cognitive deficits in patients with bilateral vestibulopathy (BVP). However, BVP patients frequently present with a concomitant sensorineural hearing loss, which is a well-established risk factor of cognitive impairment and incident dementia. Nonetheless, previous research on spatial cognitive deficits in BVP patients have not taken hearing status into account.Objective: This study aims to compare spatial cognition of BVP patients with healthy controls, with analyses adjusting for hearing status.Methods: Spatial cognition was assessed in 64 BVP patients and 46 healthy controls (HC) by use of the Virtual Morris Water Task (VMWT). All statistical analyses were adjusted for hearing (dys)function, sex, age, education, and computer use.Results: Overall, patients with BVP performed worse on all outcome measures of the VMWT. However, these differences between BVP patients and healthy controls were not statistically significant. Nonetheless, a statistically significant link between sensorineural hearing loss and spatial cognition was observed. The worse the hearing, the longer subjects took to reach the hidden platform in the VMWT. Furthermore, the worse the hearing, the less time was spent by the subjects in the correct platform quadrant during the probe trial of the VMWT.Conclusion: In this study, no difference was found regarding spatial cognition between BVP patients and healthy controls. However, a statistically significant link was observed between sensorineural hearing loss and spatial cognition.

Objective: Clinical vestibular testing mainly consists of testing reflexes, but does not routinely include testing for perceptual symptoms. The objective of this study was to investigate a new and faster test for vestibular perception, and to compare its results with previous studies.Methods: Fifty-five healthy subjects with no prior vestibular complaints were included and divided into three age groups. Vestibular perceptual thresholds were measured using a hydraulic platform in the dark. The platform delivered 12 different movements: six translations (forward, backward, right, left, up, and down) and six rotations/tilt (yaw left, yaw right, pitch forward, pitch backward, roll left, and roll right). The subject had to report the correct type and direction of movements. Thresholds were determined by a double confirmation of the lowest threshold. General trends in thresholds like relative interrelationship and the influence of age were analyzed and compared with values reported previously.Results: Mean thresholds of age groups ranged between 0.092 and 0.221 m/s2 for translations, and between 0.188 and 2.255°/s2 for rotations. The absolute values differed from previous reports, but the relative interrelationship of thresholds between type and direction of motion remained. An association between age and vestibular thresholds was found, similar to previous reports.Conclusion: This new and faster test for vestibular perception showed comparable patterns in perceptual thresholds when compared to more research oriented, lengthy tests. This might pave the way for establishing vestibular perception testing protocols useful for the clinic.

ObjectiveTo systematically review the symptoms reported by patients with bilateral vestibulopathy (BV) in clinical studies and case reports. This would serve as the first step in establishing a validated patient-reported outcome measures (PROM) for BV.MethodsA search on symptoms reported by patients with BV was performed in PubMed, and all publications covering these symptoms were included. Exclusion criteria comprised reviews and insufficient details about the frequency of occurrence of symptoms.Results1,442 articles were retrieved. 88 studies were included (41 clinical studies, 47 case reports). In consensus, 68 descriptions of symptoms were classified into 6 common and generic symptoms. Frequency of symptoms in clinical studies and case reports were reviewed, respectively; imbalance (91 and 86%), chronic dizziness (58 and 62%), oscillopsia (50 and 70%), and recurrent vertigo (33 and 67%). BV could be accompanied by hearing loss (33 and 43%) and tinnitus (15 and 36%). 15 clinical studies and 10 case reports reported symptoms beyond vestibular and hearing deficits such as limited social activities, depression, concentration, and memory impairment and reduced quality of life in general.ConclusionThe literature on BV symptomatology mainly focuses on classic symptoms such as imbalance and oscillopsia, while only few report additional symptoms such as cognitive memory impairment and performing dual tasks. In fact, none of the reviewed clinical studies and case reports provided a comprehensive overview of BV symptoms. To develop a validated PROM, qualitative research using semi-structured and unstructured interviews is needed to explore the full spectrum of BV symptoms.

ObjectivePatients with bilateral vestibulopathy (BV) can still have residual “natural” function. This might interact with “artificial” vestibular implant input (VI-input). When fluctuating, it could lead to vertigo attacks. Main objective was to investigate how “artificial” VI-input is integrated with residual “natural” input by the central vestibular system. This, to explore (1) whether misalignment in the response of “artificial” VI-input is sufficiently counteracted by well-aligned residual “natural” input and (2) whether “artificial” VI-input is able to influence and counteract the response to residual “natural” input, to show feasibility of a “vestibular pacemaker.”Materials and methodsFive vestibular electrodes in four BV patients implanted with a VI were available. This involved electrodes with a predominantly horizontal response and electrodes with a predominantly vertical response. Responses to predominantly horizontal residual “natural” input and predominantly horizontal and vertical “artificial” VI-input were separately measured first. Then, inputs were combined in conditions where both would hypothetically collaborate or counteract. In each condition, subjects were subjected to 60 cycles of sinusoidal stimulation presented at 1 Hz. Gain, asymmetry, phase and angle of eye responses were calculated. Signal averaging was performed.ResultsCombining residual “natural” input and “artificial” VI-input resulted in an interaction in which characteristics of the resulting eye movement responses could significantly differ from those observed when responses were measured for each input separately (p

The purpose of this study was to evaluate whether it is possible to restore the high-frequency angular vestibulo-ocular reflex (aVOR) in patients suffering from a severe bilateral vestibulopathy (BV) and implanted with a vestibular implant prototype. Three patients (S1–3) participated in the study. They received a prototype vestibular implant with one to three electrode branches implanted in the proximity of the ampullary branches of the vestibular nerve. Five electrodes were available for electrical stimulation: one implanted in proximity of the left posterior ampullary nerve in S1, one in the left lateral and another one in the superior ampullary nerves in S2, and one in the right lateral and another one in the superior ampullary nerves in S3. The high-frequency aVOR was assessed using the video head impulse test (EyeSeeCam; EyeSeeTec, Munich, Germany), while motion-modulated electrical stimulation was delivered via one of the implanted vestibular electrodes at a time. aVOR gains were compared to control measurements obtained in the same patients when the device was not activated. In three out of the five tested electrodes the aVOR gain increased monotonically with increased stimulation strength when head impulses were delivered in the plane of the implanted canal. In these cases, gains ranging from 0.4 to values above 1 were measured. A “reversed” aVOR could also be generated when inversed stimulation paradigms were used. In most cases, the gain for excitatory head impulses was superior to that recorded for inhibitory head impulses, consistent with unilateral vestibular stimulation. Improvements of aVOR gain were generally accompanied by a concomitant decrease of corrective saccades, providing additional evidence of an effective aVOR. High inter-electrode and inter-subject variability were observed. These results, together with previous research, demonstrate that it is possible to restore the aVOR in a broad frequency range using motion-modulated electrical stimulation of the vestibular afferents. This provides additional encouraging evidence of the possibility of achieving a useful rehabilitation alternative for patients with BV in the near future.

BackgroundSuperior canal dehiscence syndrome (SCDS) can be treated surgically in patients with incapacitating symptoms. However, the ideal treatment has not been determined.ObjectivesThis systematic literature review aims to assess available evidence on the comparative effectiveness and risks of different surgical treatments regarding: (1) symptom improvement; (2) objectively measurable auditory and vestibular function; (3) adverse effects, and (4) length of hospitalization.Search method and data sourcesA systematic database search according to PRISMA statement was conducted on Pubmed, Embase, and Cochrane library. In addition, reference lists were searched. No correspondence with the authors was established. The last search was conducted on June 9, 2017.Study eligibility criteriaRetrospective and prospective cohort studies were held applicable under the condition that they investigated the association between a surgical treatment method and the relief of vestibular and/or auditory symptoms. Only studies including quantitative assessment of the pre- to postoperative success rate of a surgical treatment method were included. Case reports, reviews, meta-analysis, and studies not published in English, Dutch, or German were excluded.Data collection and analysisThe first author searched literature and extracted data; the first and last analyzed the data.Main resultsSeventeen studies (354 participants, 367 dehiscences) met the eligibility criteria and were grouped according to surgical approach. Seven combinations of surgical approaches and methods for addressing the dehiscence were identified: plugging, resurfacing, or a combination of both through the middle fossa (middle fossa approach); plugging, resurfacing, or a combination of both through the mastoid (transmastoid approach); round window reinforcement through the ear canal (transcanal approach). Several studies showed high internal validity, but quality was often downgraded due to study design (1). Outcome measures and timing of postsurgical assessment varied among studies, making it unfeasible to pool data to perform a meta-analysis.ConclusionA standardized protocol including outcome measures and timeframes is needed to compare the effectiveness and safety SCDS treatments. It should include symptom severity assessments and changes in vestibular and auditory function before and after treatment.