A Case of Bilateral Vestibulopathy Caused by Aminoglycoside-Antibiotics

The use of computerised imaging technologies in vestibular rehabilitation is a new concept. We aimed to examine the effects of virtual reality in a bilateral vestibulopathy patient. The subject was a 22-year old male patient. The bilateral semi-circular channels of patient were ossified, which showed advanced stage sensorineural hearing loss. Balance was analysed with Berg balance Scale (BBS), state of balanced feeling with visual analogue scale (VAS), and daily living activities with the activitiesspecific balance confidence scale (ABC). The scales were applied before and after treatment. The patient’s balance was treated with virtual reality for 18 sessions, after which the patient was feeling his balance better. The patient’s VAS score before rehabilitation was 5 and later it was 7. The ABC scores changed from 60 to 90. The BBS score was 51 before rehabilitation and later it was 56. Balance rehabilitation was successful in bilateral vestibulopathy. Keywords: Bilateral vestibulopathy, virtual reality, balance, vertigo.

Importance: Walking and other dynamic conditions impair visual acuity in individuals with bilateral vestibulopathy (BVP). Dynamic visual acuity (DVA) can be assessed on a treadmill while participants walk at different speeds and this has proven to be a useful assessment in classifying people with BVP. However, there are open questions relating to the effects and interactions of age, presence of BVP and walking speed on DVA loss (DVAL) and DVA assessment dropout.Objective: To investigate the effects of BVP, age and walking speed on DVAL and DVA assessment dropout, by comparing these outcomes between participants with BVP and healthy age-sex-matched participants.. Design, Setting and Participants: Fifty-two participants with BVP and 52 age-sex-matched healthy participants completed a treadmill-based DVA assessment in this cross-sectional study. Data were collected in Maastricht University Medical Centre from June 2021 to August 2024.Main Outcomes and Measures: The measurement included a static condition at 0km/h and walking conditions at 2, 4, and 6km/h. The DVAL was measured as the difference between visual acuity in the static and walking conditions. At all speeds, the drop-out rate, handrail use and DVAL were examined in relation to BVP, age, and the relationship between DVAL and speed in BVP and healthy controls. Results: Age significantly increased the odds of dropping-out (odds ratio = 1.160, P<0.001), while BVP did not increase the odds of dropping-out (odds ratio = 0.792, P=0.733). A significant Group*Speed (P=0.004) interaction effect was found for DVAL, with DVAL being significantly worse in people with BVP across all walking speeds, getting progressively worse as speed increased, which was not seen in the healthy participants. Age did not have a significant effect on DVAL (P=0.06). Conclusions and Relevance: BVP does not appear to restrict the ability to walk at the higher speeds of a DVA assessment and cause an increase in dropout rate, whereas age does. BVP significantly impacts DVA, with increasing DVAL at increasing walking speeds.

Bilateral vestibulopathy (BVP) is a chronic debilitating neurologic disorder with no monogenic cause established so far despite familiar presentations. We hypothesized that replication factor complex subunit 1 (RFC1) repeat expansions might present a recurrent monogenic cause of BVP. The study involved RFC1 screening and in-depth neurologic, vestibulo-oculomotor, and disease evolution phenotyping of 168 consecutive patients with idiopathic at least "probable BVP" from a tertiary referral center for balance disorders, with127 of them meeting current diagnostic criteria of BVP (Bárány Society Classification). Biallelic AAGGG repeat expansions in RFC1 were identified in 10/127 patients (8%) with BVP and 1/41 with probable BVP. Heterozygous expansions in 10/127 patients were enriched compared with those in reference populations. RFC1-related BVP manifested at a median age of 60 years (range 34-72 years) and co-occurred predominantly with mild polyneuropathy (10/11). Additional cerebellar involvement (7/11) was subtle and limited to oculomotor signs in early stages, below recognition of classic cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. Clear dysarthria, appendicular ataxia, or cerebellar atrophy developed 6-8 years after onset. Dysarthria, absent patellar reflexes, and downbeat nystagmus best discriminated RFC1-positive BVP from RFC1-negative BVP, but neither sensory symptoms nor fine motor problems. Video head impulse gains of patients with RFC1-positive BVP were lower relative to those of patients with RFC1-negative BVP and decreased until 10 years disease duration, indicating a potential progression and outcome marker for RFC1-disease. This study identifies RFC1 as the first-and frequent-monogenic cause of BVP. It characterizes RFC1-related BVP as part of the multisystemic evolution of RFC1 spectrum disease, with implications for designing natural history studies and future treatment trials. This study provides Class II evidence that RFC1 repeat expansions cause BVP.

Purpose To estimate the prevalence of bilateral vestibulopathy (BV) and evaluate the association with, and concurrent validity of the Dynamic Gait Index (DGI) and the Dizziness Handicap Inventory (DHI) in diagnosing BV based on video head impulse test (vHIT) among older adults ≥65 years referred to a geriatric falls clinic on suspicion of vestibular impairment. Materials and methods The vHIT was applied as a reference standard of BV to estimate diagnostic parameters for optimal cut-off scores of DGI and DHI applied separately and in combination. Results Two-hundred medical records were reviewed (70% women, mean age 79.4 years). The prevalence of BV was 9%. DGI was weakly associated with BV: Odds Ratio (OR) 1.15 (95% confidence interval (CI): 1.01; 1.31), with a 93% sensitivity and 47% specificity of a cut-off score of 16. The total score of DHI showed no association with BV: OR 1.01 (95% CI: 0.98; 1.04). The concurrent validity for BV and DGI and/or DHI were found to be inadequate. Conclusions A prevalence of 9% underlines the relevance for assessment of BV. Only a weak association between DGI and/or DHI and BV was found. Thus, vHIT remains the preferred test for detecting BV in geriatric fall assessments. IMPLICATIONS FOR REHABILITATION Bilateral vestibulopathy (BV) has numerous negative consequences for older adults and the prevalence is high among older adults referred to a geriatric falls clinic on suspicion of vestibular impairment. The Dynamic Gait Index (DGI) and the Dizziness Handicap Inventory (DHI) are not valid alternatives to the Video Head Impulse Test (vHIT) when assessing BV among geriatric outpatients.

Dizziness is a common leading symptom in bilateral vestibulopathy (BVP) and functional dizziness (FD), with significant negative effects on functional ability and quality of life. Vertigoheel is a widely used non-prescription drug for the treatment of vertigo. In order to generate systematic data for Vertigoheel in BVP and FD, we conducted a non-interventional study assessing vertigo symptoms. This study was conducted as an open-label, prospective, monocentric, non-interventional case series (ClinicalTrials.gov identifier NCT05897853). Patients with BVP and FD received Vertigoheel according to market approval for an observational period of 2 months. Change from baseline after 2 months was assessed for the following endpoints: Dizziness Handicap Inventory (DHI) as the primary endpoint, quality of life (QoL) by EQ-5D-5L, and body sway by static posturography. Patients with FD were additionally assessed for depression and anxiety by PHQ-9 and GAD-7 questionnaires. Patients with BVP were assessed for vestibular function by video head impulse testing and caloric testing. Adverse events and other safety-related observations were evaluated. Of 41 patients with FD and 13 with BVP, two with FD and none with BVP dropped out before the follow-up visit. Both patient groups showed significantly improved disability caused by dizziness after 2 months: In BVP, the DHI decreased on average by 13.2 points from 45.4 to 32.2 (p < 0.001). In FD, the DHI decreased on average by 12.0 points from 46.5 to 34.5 (p < 0.001). In patients with FD, significant improvements were also observed for the secondary endpoints QoL, anxiety, and depression. No significant change was observed for posturography readouts. In patients with BVP, there were no statistically significant improvements for the secondary endpoints QoL, posturography, or vestibular function within the observation period. The study found no evidence of a safety risk. The study provides evidence for Vertigoheel's clinical safety and limited evidence - because of the non-interventional design - for its effectiveness in BVP and FD that are considered disease entities with high medical need for new treatment options. The results may serve as the basis for randomized placebo-controlled trials.

Objective: To evaluate the different peripheral, neurological, genetic, and systemic etiologies of bilateral vestibulopathy (BVP) and the value of vHIT in the diagnostic process.Materials and methods: A retrospective case review was performed on 176 patients diagnosed with BVP in a tertiary referral center, between 2010 and 2020. Inclusion criteria comprised imbalance and/or oscillopsia during locomotion and horizontal angular VOR gain on both sides <0.8. We classified patients into different groups according to (1) their fulfillment of the Barany guideline for bilateral vestibulopathy (2) the definite etiology of BVP and (3) the four clinical subtypes distributed in our population (recurrent vertigo with BVP, rapidly progressive BVP, slowly progressive BVP, and slowly progressive BVP with ataxia). Medical history of vertigo, hypoacusis or migraine, and family background of imbalance and/or oscillopsia were assessed. Horizontal, posterior, and superior semicircular canal angular VOR gain was registered along with saccadic parameters such as velocity, and dispersion of the saccades' latency values.Results: Barany's Society diagnostic criteria for BVP was accomplished in 89 patients. Among our patients, 13.6% had migraines in their medical history and the idiopathic group accounted for 50% of the population. All four clinical subtypes were found in our population, slowly progressive bilateral vestibulopathy without vertigo was the most frequent one. A percentage of our population could not be categorized into any of the former subtypes, many of these patients were diagnosed with BVP after suffering a single vertigo episode. Lower vHIT gains were found in those patients with Barany's criteria for BVP and oscillopsia was significantly more prevalent in this group.Conclusions: Bilateral vestibulopathy manifests with very different patterns representing a very heterogeneous condition. The distribution of the clinical subtypes and Barany's criteria are a useful clinical tool to differentiate groups of patients. The vHIT can serve as an initial tool for identifying patients with BVP. The finding of bilateral vestibular involvement in a clinically suspected unilateral vestibulopathy should be considered in some patients.

ObjectivesDynamic visual acuity (DVA) can be assessed on a treadmill while walking at different speeds and is used to assess people with bilateral vestibulopathy (BVP). However, the effects and interactions of age, BVP, and walking speed on DVA loss and assessment dropout are unclear. Our objective was to investigate the effects of BVP, age, and walking speed on DVA loss and assessment dropout in participants with BVP and healthy age–sex-matched participants.Methods41 participants with BVP and 41 age–sex-matched healthy participants completed a treadmill-based DVA assessment, including a static condition at 0 km/h and walking conditions at 2, 4, and 6 km/h. DVA loss was measured as the visual acuity difference between static and walking conditions. The drop-out rate, handrail use, and DVA loss were examined in relation to BVP, age, and walking speed.ResultsAge significantly increased the odds of dropping out (odds ratio = 1.160, p < 0.001), while BVP did not increase the odds of dropping out (odds ratio = 0.792, p = 0.733). A significant Group*Speed (p = 0.004) interaction effect was found for DVA loss, with DVA loss being significantly worse in people with BVP across all walking speeds, getting progressively worse as speed increased, which was not seen in the healthy participants. Age did not have a significant effect on DVA loss (p = 0.06).ConclusionBVP does not appear to restrict the ability to walk at the higher speeds of a DVA assessment and cause an increase in dropout rate, whereas age does. BVP significantly impacts DVA, with increasing impact at increasing walking speeds.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00415-025-13269-9.

Hearing loss is considered an independent risk factor for dementia. Growing evidence in animal and human studies suggest that not only hearing loss but also vestibular loss might result in cognitive deficits. The objective of this study is to evaluate the presence of spatial and nonspatial cognitive deficits in patients with bilateral vestibulopathy. As different causes of bilateral vestibulopathy are associated with hearing loss, the objective is to evaluate if these cognitive deficits are due to the vestibular loss of the patients with bilateral vestibulopathy, or to their hearing loss, or both. We performed a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. (1) Data sources: MEDLINE and the Cochrane Library. (2) Study selection: Cross-sectional studies investigating cognitive performances in human patients with bilateral vestibulopathy confirmed by quantitative vestibular testing. (3) Data extraction: Independent extraction of articles by three authors using predefined data fields, including patient- and control characteristics and cognitive outcomes. Ten studies reporting on 126 patients with bilateral vestibulopathy matched the inclusion criteria. Cognitive domains evaluated in patients with bilateral vestibulopathy included visuospatial abilities, memory, language, attention, and executive function. In only three studies, hearing performance of the included patients was briefly described. Nearly all studies demonstrated a significant impairment of spatial cognition in patients with bilateral vestibulopathy. In the few papers investigating nonspatial cognition, worse outcome was demonstrated in patients with bilateral vestibular loss performing cognitive tasks assessing attentional performance, memory, and executive function. Strong evidence exists that patients with bilateral vestibulopathy suffer from impaired spatial cognition. Recent studies even suggest impairment in other cognitive domains than spatial cognition. However, in all previous studies, conclusions on the link between cognitive performance and vestibular loss were drawn without taken hearing loss into consideration as a possible cause of the cognitive impairment.

Aims: To determine the susceptibility to visual height intolerance (vHI) in patients with acquired bilateral vestibulopathy (BVP). The question was whether postural instability in BVP, which is partially compensated for by visual substitution of the impaired vestibular control of balance, leads to an increased susceptibility. This is of particular importance since fear of heights is dependent on body posture, and visual control of balance at heights can no longer substitute vestibular input. For comparison susceptibility to vHI was determined in patients with other vestibular or functional disorders.Methods: A total of 150 patients aged 18 or above who had been referred to the German Center for Vertigo and Balance Disorders and diagnosed to have BVP were surveyed with a standardized questionnaire by specifically trained neurological professionals. Further, 481 patients with other vestibular or functional disorders were included.Results: Susceptibility to vHI was reported by 29% (32 % in females, 25% in males) of the patients with BVP. Patients with vHI were slightly younger (67 vs. 71 years). Seventy percent of those with vHI reported avoidance of climbing, hiking, stairs, darkness, cycling or swimming (84% of those without vHI). Mean age for onset of vHI was 40 years. Susceptibility to vHI was higher in patients with other vertigo disorders than in those with BVP: 64% in those with phobic postural vertigo, 61% in vestibular migraine, 56% in vestibular paroxysmia, 54% in benign paroxysmal positional vertigo, 49% in unilateral vestibulopathy and 48% in Menière's disease.Conclusions: The susceptibility to vHI in BVP was not higher than that of the general population (28%).This allows two explanations that need not be alternatives but contribute to each other: (1) Patients with a bilateral peripheral vestibular deficit largely avoid exposure to heights because of their postural instability. (2) The irrational anxiety to fall from heights triggers increased susceptibility to vHI, not the objective postural instability. However, patients with BVP do not exhibit increased comorbid anxiety disorders. This view is supported by the significantly increased susceptibility to vHI in other vestibular syndromes, which are characterized by an increased comorbidity of anxiety disorders.

ABSTRACTBackgroundTwo chronic forms of dizziness—bilateral vestibulopathy (BVP) with a loss of vestibular input, and functional dizziness with normal vestibular function—present with the key symptom of postural and gait imbalance. In BVP, this is associated with spatial disorientation. Here, we investigated whether persistent postural–perceptual dizziness (PPPD) in patients with normal vestibular function also affects spatial orientation, because there is evidence that central multisensory misintegration plays a crucial role in PPPD.MethodsThirty‐two patients with BVP (mean age 52.44 ± 12.00 years; 17 females), 43 patients with PPPD (mean age 45.93 ± 11.72 years; 25 females), and 32 healthy controls (HC, mean age 44.78 ± 14.40 years; 15 females) participated in a clinical bedside test investigating spatial orientation abilities (three‐dimensional real‐world pointing task, 3D‐RWPT). This test includes a cognitive (mental rotation) and a vestibular paradigm (body rotation around yaw axis with eyes closed). Participants reported their perceived spatial abilities and levels of spatial anxiety /orientation‐related discomfort through standardized questionnaires.ResultsPatients with BVP and PPPD showed significantly lower accuracy (i.e., larger angular deviations) in the 3D‐RWPT compared to HC (BVP: 9.62° ± 3.21°, PPPD: 9.16° ± 3.85°, HC: 7.77° ± 2.86°; p = 0.03), especially in the subtasks that rely on vestibular function (BVP: 8.11° ± 5.51°, PPPD: 6.62° ± 4.46°, HC: 4.45° ± 2.33°; p < 0.01). All cohorts had comparable levels of self‐assessed spatial abilities, while both BVP and PPPD patients showed higher levels of spatial orientation discomfort.ConclusionsThis impairment of spatial orientation in PPPD patients with normal vestibular function could be a sign of (potentially anxiety‐driven) central suppression of vestibular input, which is required for the continuous updating of the internal representation of body motion and position relative to the environment.

To assess visual acuity (VA) while the patient is walking and to evaluate oscillopsia severity in patients with bilateral vestibulopathy (BV) and in patients with unilateral vestibular loss (UVL). Prospective study with a group of patients with BV, a group of patients with UVL, and a control group of healthy subjects. Tertiary academic center. Thirty seven patients with BV(age range, 29-80 years), 11 patients with UVL (age range, 48-75 years), and 57 healthy subjects (age range 20-77 years). Computation of the difference between the VA measured in static conditions and in dynamic conditions while walking on a treadmill at 2, 4, and 6 km/h. Oscillopsia severity was assessed with a questionnaire that we developed. Differences in VA at 2, 4, and 6 km/h and oscillopsia severity score. As a group, patients with BV showed a significant increase of the VA differences compared with healthy subjects (P < .001) and patients with UVL (P < .001) for all 3 walking velocities. Normality thresholds were defined as healthy subjects' 95% CI. Sensitivity of the test was 97% for discriminating patients with BV. Moderate to extreme oscillopsia severity was found in 81% of patients with BV and in 9% of patients with UVL. Differences in VA did not correlate with oscillopsia severity scores in patients with BV (P > .05 for all comparisons). We designed a highly sensitive, simple, cost-effective protocol to assess dynamic VA under physiologic conditions and a questionnaire to determine oscillopsia severity. Both tools could be used for the evaluation of new treatments for BV and patients with UVL.

The diagnosis of bilateral vestibulopathy (BV) is typically established based on bilateral semicircular canal dysfunction. The degree to which both otolith organs-the saccule and utricle-are also impaired in BV is not well-established, particularly with respect to the etiology and severity of BV. The aim of this study was to evaluate semicircular canal, saccular and utricular function in patients with BV due to aminoglycoside ototoxicity and bilateral Menière's disease, and with different severities of BV. Caloric and head impulse testing were used as measures of canal function. Cervical vestibular-evoked myogenic potentials (cVEMP) and ocular VEMPs (oVEMP) were used as measures of saccular and utricular function, respectively. We enrolled 34 patients with BV and 55 controls in a prospective case-control study. Patients with BV were less likely to have saccular (61%) or utricular (64%) dysfunction relative to canal dysfunction (100%). Utricular function differed significantly between patients by etiologic group: the poorest function was found in patients with BV due to aminoglycoside toxicity, and the best function in Menière's disease patients. Canal and saccular function did not vary according to etiology. Further, utricular but not saccular function was significantly correlated with canal function. Saccular and utricular function had the strongest association with Dizziness Handicap Inventory scores relative to canal function. These data suggest that when a patient with BV is identified in a clinical context, oVEMP testing is the most sensitive test in distinguishing between aminoglycoside toxicity and bilateral Menière's disease. Both cVEMP and oVEMP testing may be considered to evaluate the functional impact on the patient.

Bilateral vestibulopathy is a rare, but important cause of imbalance that is underrecognized and not well understood. Clinically heterogeneous, it is variably associated with recurrent vertigo, hearing loss, migraine, peripheral neuropathy, or cerebellar degeneration. In about half of all patients with bilateral vestibulopathy, no cause can be identified. There have been several reports of familial bilateral vestibulopathy, suggesting genetic predisposition. The identification of genetic defects underlying hereditary deafness syndromes has greatly advanced the understanding of the functional components and the development of cochlea. In contrast, the progress in bilateral vestibulopathy has been slow, likely hampered by the difficulty in diagnosis outside of academic centers and a lack of animal models that recapitulate the progressive clinical features that are not apparent from birth. It is reasonable to anticipate that there will be an equally large number of genetic disorders underlying bilateral vestibulopathy as in deafness. Understanding the pathophysiology of bilateral vestibulopathy may suggest possible causes for the gradual decline in vestibular function that occurs with normal aging. Furthermore, the study of bilateral vestibulopathy may shed light on the pathophysiology of more common vestibular syndromes such as benign recurrent vertigo and vestibular migraine.

Bilateral vestibulopathy (BV) is a chronic vestibular disorder, characterized by bilaterally absent or significantly impaired vestibular function. Symptoms typically include, but are not limited to, unsteadiness and movement-induced blurred vision (oscillopsia). This prospective case–control study aimed to elucidate the impact of BV on cognitive and motor performance and on cognitive–motor interference. Cognitive and motor performance, as well as cognitive–motor interference were measured in persons with BV and normal hearing using the 2BALANCE dual-task protocol. The experimental group was matched to a healthy control group based on age, sex, and educational level. The 2BALANCE protocol comprises cognitive tests assessing visuospatial memory, mental rotation, visual and auditory response inhibition, visual and auditory working memory, and processing speed. The cognitive tests were performed in single-task condition (while seated), and in dual-task condition (during a static and a dynamic motor task). The static motor task consisted of balancing on a force platform with foam pad. The dynamic motor task consisted of walking at a self-selected speed. These motor tasks were also performed in single-task condition. A generalized estimating equations model was used to investigate group differences for all cognitive and motor outcome measures. The estimated marginal means, as well as the odds ratios (OR), and their 95% confidence intervals (CI) were calculated. For the backward digit recall test, a baseline measurement was performed and analyzed using a student-t test. A total of 22 patients with BV and normal hearing and 22 healthy control subjects were assessed [mean age (SD), BV = 53.66 (13.35) and HC = 53.21 (13.35), 68% male]. The BV group had poorer mental rotation skills in single-task condition, compared to the control group [odds ratio (OR) = 2.30, confidence interval (CI) = 1.12–4.73, P = 0.024]. Similarly, auditory and visual working memory were also poorer in the BV group in single-task condition (P = 0.028 and P = 0.003, respectively). The BV group also performed poorer on the mental rotation task and the visual response inhibition task in dual-task condition (OR = 2.96, CI = 1.57–5.59, P < 0.001 and OR = 1.08, CI = 1.01–1.16, P = 0.032, respectively). Additionally, an interaction effect, indicating increased cognitive–motor interference in the BV group, was observed for mental rotation, response inhibition, and auditory working memory (P = 0.003 to 0.028). All static motor outcome parameters indicated more postural sway in the BV group compared to the control group for all test conditions (P < 0.001 to 0.026). No group differences were noted for the dynamic motor task. These findings suggest a link between vestibular function and cognitive performance, as well as a greater interference between cognitive and motor performance in BV, compared to healthy controls.

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.