The VertiGO! Trial protocol: A prospective, single-center, patient-blinded study to evaluate efficacy and safety of prolonged daily stimulation with a multichannel vestibulocochlear implant prototype in bilateral vestibulopathy patients.

BACKGROUND: A 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. METHODS: A 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. DISCUSSION: The 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 REGISTRATION: ClinicalTrials.gov: NCT04918745. Registered 28 April 2021.

The DizzyQuest Combined with Accelerometry: Daily Physical Activities and Limitations among Patients with Bilateral Vestibulopathy Due to DFNA9.

BACKGROUND: DFNA9 is a genetic disease of the inner ear, causing progressive bilateral sensorineural deafness and bilateral vestibulopathy (BV). In this study, DizzyQuest, a mobile vestibular diary, and the MOX accelerometer were combined to assess the daily life functional limitations and physical activity of patients with DFNA9 suffering from BV. These parameters might be appropriate as potential candidacy criteria and outcome measures for new therapeutic interventions for BV. METHODS: Fifteen DFNA9 patients with BV and twelve age-matched healthy controls were included. The DizzyQuest was applied for six consecutive days, which assessed the participants' extent of functional limitations, tiredness, types of activities performed during the day, and type of activity during which the participant felt most limited. The MOX accelerometer was worn during the same six days of DizzyQuest use, measuring the participants intensity and type of physical activity. Mixed-effects linear and logistic regression analyses were performed to compare the DFNA9 patients and control group. RESULTS: DFNA9 patients with BV felt significantly more limited in activities during the day compared to the age-matched controls, especially in social participation (p < 0.005). However, these reported limitations did not cause adjustment in the types of activities and did not reduce the intensity or type of physical activity measured with accelerometry. In addition, no relationships were found between self-reported functional limitations and physical activity. CONCLUSIONS: This study demonstrated that self-reported functional limitations are significantly higher among DFNA9 patients with BV. As a result, these limitations might be considered as part of the candidacy criteria or outcome measures for therapeutic interventions. In addition, the intensity or type of physical activity performed during the day need to be addressed more specifically in future research.

Optimizing vestibular implant electrode positioning using fluoroscopy and intraoperative CT imaging.

PURPOSE:  Vestibular implant electrode positioning close to the afferent nerve fibers is considered to be key for effective and selective electrical stimulation. However, accurate positioning of vestibular implant electrodes inside the semicircular canal ampullae is challenging due to the inability to visualize the target during the surgical procedure. This study investigates the accuracy of a new surgical protocol with real-time fluoroscopy and intraoperative CT imaging, which facilitates electrode positioning during vestibular implant surgery. METHODS:  Single-center case-controlled cohort study with a historic control group at a tertiary referral center. Patients were implanted with a vestibulocochlear implant, using a combination of intraoperative fluoroscopy and cone beam CT imaging. The control group consisted of five patients who were previously implanted with the former implant prototype, without the use of intraoperative imaging. Electrode positioning was analyzed postoperatively with a high-resolution CT scan using 3D slicer software. The result was defined as accurate if the electrode position was within 1.5 mm of the center of the ampulla. RESULTS: With the new imaging protocol, all electrodes could be positioned within a 1.5 mm range of the center of the ampulla. The accuracy was significantly higher in the study group with intraoperative imaging (21/21 electrodes) compared to the control group without intraoperative imaging (10/15 electrodes), (p = 0.008). CONCLUSION:  The combined use of intraoperative fluoroscopy and CT imaging during vestibular implantation can improve the accuracy of electrode positioning. This might lead to better vestibular implant performance.

Construct validity and reliability of the Bilateral Vestibulopathy Questionnaire (BVQ).

Background: The 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. Methods: Patients 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)]. Results: A 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). Conclusion: This 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.

Chronic symptoms in patients with unilateral vestibular hypofunction: systematic review and meta-analysis.

Objective: To 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. Methods: A 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. Results: A 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 < 0.0001). In three studies, the estimated mean total Visual Analog Scale (VAS) scores were 7.05 (95% CI, 5.64-8.46) (pre-intervention) and 2.56 (95% CI, 1.15-3.97) (post-intervention). Finally, a subgroup of patients (≥32%) persists with at least a moderate handicap, despite vestibular rehabilitation. Conclusion: A spectrum of symptoms is associated with UVH, of which chronic dizziness and imbalance are most frequently reported. However, semi-structured interviews should be conducted to define the whole spectrum of UVH symptoms more precisely, in order to establish a validated patient-reported outcome measure (PROM) for UVH patients. Furthermore, vestibular interventions can significantly decrease self-reported handicap, although this is insufficient for a subgroup of patients. It could therefore be considered for this subgroup of patients to explore new intervention strategies like vibrotactile feedback or the vestibular implant. Systematic review registration: [https://www.crd.york.ac.uk/prospero/], identifier [CRD42023389185].

Assessing balance in people with bilateral vestibulopathy using the Mini-Balance Evaluation Systems Test (Mini-BESTest): feasibility and comparison with healthy control data.

OBJECTIVES: Bilateral vestibulopathy (BVP) leads to unsteadiness when walking, which worsens in darkness or on uneven ground, as well as falls. Since simple balance tests struggle to distinguish between BVP and healthy participants, we aimed (1) to test if the Mini-BESTest is feasible in BVP, (2) how people with BVP perform on the Mini-BESTest and (3) to compare these scores with healthy reference data. METHODS: Fifty participants with BVP completed the Mini-BESTest. 12-month falls incidence was obtained by questionnaire. To compare the overall and sub-scores between our participants with BVP and those of healthy participants from the literature (n = 327; obtained via PubMed searches), Mann-Whitney U tests were used. Sub scores within the BVP group were also compared. Spearman correlations were used to investigate the relationships between Mini-BESTest score and age. RESULTS: No floor or ceiling effects were observed. Participants with BVP had significantly lower Mini-BESTest total scores than the healthy group. Anticipatory, reactive postural control and sensory orientation sub scores of the Mini-BESTest were significantly lower in BVP, while dynamic gait sub scores were not significantly different. A stronger negative correlation between age and Mini-BESTest total score was found in BVP than in the healthy group. Scores did not differ between patients with different falls history. CONCLUSION: The Mini-BESTest is feasible in BVP. Our results confirm the commonly reported balance deficits in BVP. The stronger negative association between age and balance in BVP might reflect the age-related decline in the remaining sensory systems with which people with BVP compensate.

Development of a new clinical tool to evaluate the balance abilities of children with bilateral vestibular loss: The Geneva Balance Test.

Introduction: Vestibular 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. Methods: To 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). Results: Statistical 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. Discussion: In this study, the GBT allowed quantifying balance deficits in children with BV. The BOT-2 test is not validated for children <4.5 years of age, and the GBT seems to be better tolerated in all populations than the mBOT-2. Furthermore, mBOT-2 results saturated, reaching maximum values by 6-9 years whereas the GBT did not, suggesting that the GBT could be a useful tool for monitoring the development of balance capacities with age and could be used in the follow-up of children with severe vestibular disorders.

Optimized Signal Analysis to Quantify the Non-Linear Behaviour of the Electrically Evoked Vestibulo-Ocular Reflex in Patients with a Vestibular Implant.

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.

Suppression Head Impulse Test (SHIMP) versus Head Impulse Test (HIMP) When Diagnosing Bilateral Vestibulopathy.

The Suppression Head Impulse (SHIMP) test was introduced as an alternative to the Head Impulse Paradigm (HIMP) to overcome challenges in VOR gain calculation due to the interference of covert saccades. The objectives of this study were (1) to determine if SHIMP, compared to HIMP, reduces covert saccades in BV patients and (2) to define the agreement on diagnosing BV between SHIMP and HIMP. First, the number of covert saccades was compared between SHIMP and HIMP. Secondly, VOR gain was compared between SHIMP and HIMP. Lastly, the agreement between SHIMP and HIMP on identifying BV (horizontal VOR gain <0.6) was evaluated. A total of 98 BV patients were included. To our knowledge, this is the largest study population on SHIMP testing in BV patients. Covert saccades were significantly reduced, and a lower VOR gain was found during SHIMP compared to HIMP (p < 0.001). However, the clinical relevance of these statistically significant differences is small. In 93% of the patients, an agreement was found between the two paradigms regarding the diagnosis of BV, and both paradigms detect BV in the vast majority of patients.

Sound localization in patients with bilateral vestibulopathy.

PURPOSE: The goal of this study was to evaluate if bilaterally (partially) absent vestibular function during static sound localization testing, would have a negative impact on sound localization skills. Therefore, this study compared horizontal static sound localization skills of normal-hearing patients with bilateral vestibulopathy (BV) and healthy controls. METHODS: Thirteen normal-hearing patients with BV and thirteen age-matched healthy controls were included. Sound localization skills were tested using seven loudspeakers in a frontal semicircle, ranging from - 90° to + 90°. Sound location accuracy was analyzed using the root-mean-square error (RMSE) and the mean absolute error (MAE). To evaluate the severity of the BV symptoms, the following questionnaires were used: Dizziness Handicap Inventory (DHI), Oscillopsia severity questionnaire (OSQ), 12-item Spatial, Speech, and Qualities Questionnaire (SSQ12), and Health Utilities Index Mark 3 (HUI3). RESULTS: The RMSE and MAE were significantly larger (worse) in the BV group than in the healthy control group, with respective median RMSE of 4.6° and 0°, and a median MAE of 0.7° and 0°. The subjective reporting of speech perception, spatial hearing, and quality of life only demonstrated a moderate correlation between DHI (positive correlation) and HUI total score (negative correlation), and localization scores. CONCLUSION: Static sound localization skills of patients with BV were only mildly worse compared to healthy controls. However, this difference was very small and therefore most likely due to impaired cognitive function. The vestibular system does not seem to have a modulating role in sound localization during static conditions, and its impact is negligible in contrast to the impact of hearing impairment. Furthermore, the subjective reporting of speech perception, spatial hearing, and quality of life was not strongly correlated with localization scores.

Development and Content Validity of the Bilateral Vestibulopathy Questionnaire.

Background: To 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. Methods: The 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). Results: The 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. Conclusion: The 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.

Patterns of Vestibular Impairment in Bilateral Vestibulopathy and Its Relation to Etiology.

Objective: This 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. Methods: A 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. Results: The 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. Conclusion: This 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.

The resilience of the inner ear-vestibular and audiometric impact of transmastoid semicircular canal plugging.

BACKGROUND: Certain cases of superior semicircular canal dehiscence or benign paroxysmal positional vertigo can be treated by plugging of the affected semicircular canal. However, the extent of the impact on vestibular function and hearing during postoperative follow-up is not known. OBJECTIVE: To evaluate the evolution of vestibular function and hearing after plugging of a semicircular canal. METHODS: Six patients underwent testing before and 1 week, 2 months, and 6 months after plugging of the superior or posterior semicircular canal. Testing included caloric irrigation test, video Head Impulse Test (vHIT), cervical and ocular Vestibular Evoked Myogenic Potentials (VEMPs) and audiometry. RESULTS: Initially, ipsilateral caloric response decreased in all patients and vHIT vestibulo-ocular reflex (VOR) gain of each ipsilateral semicircular canal decreased in 4/6 patients. In 4/6 patients, postoperative caloric response recovered to > 60% of the preoperative value. In 5/6 patients, vHIT VOR gain was restored to > 85% of the preoperative value for both ipsilateral non-plugged semicircular canals. In the plugged semicircular canal, this gain decreased in 4/5 patients and recovered to > 50% of the preoperative value. Four patients preserved cervical and ocular VEMP responses. Bone conduction hearing deteriorated in 3/6 patients, but recovered within 6 months postoperatively, although one patient had a persistent loss of 15 dB at 8 kHz. CONCLUSION: Plugging of a semicircular canal can affect both vestibular function and hearing. After initial deterioration, most patients show recovery during follow-up. However, a vestibular function loss or high-frequency hearing loss can persist. This stresses the importance of adequate counseling of patients considering plugging of a semicircular canal.

Bilateral vestibulopathy decreases self-motion perception.

OBJECTIVE: Current diagnostic criteria for bilateral vestibulopathy (BV) primarily involve measurements of vestibular reflexes. Perceptual self-motion thresholds however, are not routinely measured and their clinical value in this specific population is not yet fully determined. Objectives of this study were (1) to compare perceptual self-motion thresholds between BV patients and control subjects, and (2) to explore patterns of self-motion perception performance and vestibular function in BV patients. METHODS: Thirty-seven BV patients and 34 control subjects were included in this study. Perceptual self-motion thresholds were measured in both groups using a CAREN platform (Motek Medical BV, Amsterdam, The Netherlands). Vestibular function was evaluated (only in BV patients) by the caloric test, torsion swing test, video head impulse test of all semicircular canals, and cervical- and ocular vestibular-evoked myogenic potentials. Differences in thresholds between both groups were analyzed. Hierarchical cluster analysis was performed to visualize patterns between self-motion perception and vestibular function within the group of BV patients. RESULTS: Perceptual self-motion thresholds were significantly higher in BV patients compared to control subjects, regarding nearly all rotations and translations (depending on the age group) (p ≤ 0.001). Cluster analysis showed that within the group of BV patients, higher perceptual self-motion thresholds were generally associated with lower vestibular test results (significant for yaw rotation, caloric test, torsion swing test, and video head impulse test (p ≤ 0.001)). CONCLUSION: Self-motion perception is significantly decreased in BV patients compared to control subjects regarding nearly all rotations and translations. Furthermore, decreased self-motion perception is generally associated with lower residual vestibular function in BV patients. TRIAL REGISTRATION: Trial registration number NL52768.068.15/METC.

Bilateral vestibulopathy patients' perspectives on vestibular implant treatment: a qualitative study.

OBJECTIVES: The aim of this study was to explore expectations of patients with bilateral vestibulopathy regarding vestibular implant treatment. This could advance the definition of recommendations for future core outcome sets of vestibular implantation and help to determine on which characteristics of bilateral vestibulopathy future vestibular implant research should focus. METHODS: Semi-structured interviews were conducted with 50 patients diagnosed with bilateral vestibulopathy at Maastricht UMC + . Interviews followed a semi-structured interview guide and were recorded and transcribed. Transcripts were analyzed thematically by two independent researchers. A consensus meeting took place to produce a joint interpretation for greater dimensionality and to confirm key themes. RESULTS: Overall, patient expectations centralized around three key themes: (physical) symptom reduction, functions and activities, and quality of life. These themes appeared to be interrelated. Patient expectations focused on the activity walking (in a straight line), reducing the symptom oscillopsia and being able to live the life they had before bilateral vestibulopathy developed. In general, patients indicated to be satisfied with small improvements. CONCLUSION: This study demonstrated that patient expectations regarding a vestibular implant focus on three key themes: symptom reduction, functions and activities, and quality of life. These themes closely match the functional improvements shown in recent vestibular implantation research. The results of this study provide a clear guideline from the patient perspective on which characteristics of bilateral vestibulopathy, future vestibular implant research should focus. TRIAL REGISTRATION: NL52768.068.15/METC.

The Effect of Different Head Movement Paradigms on Vestibulo-Ocular Reflex Gain and Saccadic Eye Responses in the Suppression Head Impulse Test in Healthy Adult Volunteers.

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.

Speech Perception With Novel Stimulation Strategies for CombinedCochleo-Vestibular Systems.

Cochlear implants are very well established in the rehabilitation of hearing loss and are regarded as the most successful neuroprostheses to date. While a lot of progress has also been made in the neighboring field of specific vestibular implants, some diseases affect the entire inner ear, leading to both hearing and vestibular hypo- or dysfunction. The proximity of the cochlear and vestibular organs suggests a single combined implant as a means to alleviate the associated impairments. While both organs can be stimulated in a similar way with electric pulses applied through implanted electrodes, the typical phase durations needed in the vestibular system seem to be substantially larger than those typically needed in the cochlear system. Therefore, when using sequential stimulation in a combined implant, the pulse stream to the cochlea is interrupted by comparatively large gaps in which vestibular stimulation can occur. We investigate the impact of these gaps in the auditory stream on speech perception. Specifically, we compare a number of stimulation strategies with different gap lengths and distributions and evaluate whether it is feasible to use them without having a noticeable decline in perception and quality of speech. This is a prerequisite for any practicable stimulation strategy of a combined system and can be investigated even in recipients of a normal cochlear implant. Our results show that there is no significant deterioration in speech perception for the different strategies examined in this paper, leaving the strategies as viable candidates for prospective combined cochleo-vestibular implants.

Simultaneous activation of multiple vestibular pathways upon electrical stimulation of semicircular canal afferents.

BACKGROUND AND PURPOSE: Vestibular implants seem to be a promising treatment for patients suffering from severe bilateral vestibulopathy. To optimize outcomes, we need to investigate how, and to which extent, the different vestibular pathways are activated. Here we characterized the simultaneous responses to electrical stimuli of three different vestibular pathways. METHODS: Three vestibular implant recipients were included. First, activation thresholds and amplitude growth functions of electrically evoked vestibulo-ocular reflexes (eVOR), cervical myogenic potentials (ecVEMPs) and vestibular percepts (vestibulo-thalamo-cortical, VTC) were recorded upon stimulation with single, biphasic current pulses (200 µs/phase) delivered through five different vestibular electrodes. Latencies of eVOR and ecVEMPs were also characterized. Then we compared the amplitude growth functions of the three pathways using different stimulation profiles (1-pulse, 200 µs/phase; 1-pulse, 50 µs/phase; 4-pulses, 50 µs/phase, 1600 pulses-per-second) in one patient (two electrodes). RESULTS: The median latencies of the eVOR and ecVEMPs were 8 ms (8-9 ms) and 10.2 ms (9.6-11.8 ms), respectively. While the amplitude of eVOR and ecVEMP responses increased with increasing stimulation current, the VTC pathway showed a different, step-like behavior. In this study, the 200 µs/phase paradigm appeared to give the best balance to enhance responses at lower stimulation currents. CONCLUSIONS: This study is a first attempt to evaluate the simultaneous activation of different vestibular pathways. However, this issue deserves further and more detailed investigation to determine the actual possibility of selective stimulation of a given pathway, as well as the functional impact of the contribution of each pathway to the overall rehabilitation process.

The vestibular implant: Opinion statement on implantation criteria for research.

This opinion statement proposes a set of candidacy criteria for vestibular implantation of adult patients with bilateral vestibulopathy (BVP) in a research setting. The criteria include disabling chronic symptoms like postural imbalance, unsteadiness of gait and/or head movement-induced oscillopsia, combined with objective signs of reduced or absent vestibular function in both ears. These signs include abnormal test results recorded during head impulses (video head impulse test or scleral coil technique), bithermal caloric testing and rotatory chair testing (sinusoidal stimulation of 0.1 Hz). Vestibular implant (VI) implantation criteria are not the same as diagnostic criteria for bilateral vestibulopathy. The major difference between VI-implantation criteria and the approved diagnostic criteria for BVP are that all included vestibular tests of semicircular canal function (head impulse test, caloric test, and rotatory chair test) need to show significant impairments of vestibular function in the implantation criteria. For this, a two-step paradigm was developed. First, at least one of the vestibular tests needs to fulfill stringent criteria, close to those for BVP. If this is applicable, then the other vestibular tests have to fulfill a second set of criteria which are less stringent than the original criteria for BVP. If the VI-implantation is intended to excite the utricle and/or saccule (otolith stimulation), responses to cervical and ocular vestibular evoked myogenic potentials must be absent in addition to the above mentioned abnormalities of semicircular canal function. Finally, requirements for safe and potentially effective stimulation should be met, including implanting patients with BVP of peripheral origin only, and assessing possible medical and psychiatric contraindications.