Bilateral Vestibulopathy Research Articles

Exploration of sleep quality and rest-activity rhythms characteristics in Bilateral Vestibulopathy patients

Sleep and circadian timing systems are constantly regulated by both photic and non-photic signals. Connections between the vestibular nuclei and the biological clock raise the question of the effect of peripheral vestibular loss on daily rhythms, such as the sleep-wake cycle and circadian rhythm. To answer this question, we compared the sleep and rest-activity rhythm parameters of 15 patients with bilateral vestibulopathy (BVP) to those of 15 healthy controls. Sleep and rest-activity cycle were recorded by a device coupling actimetry with the heart rate and actigraphy at home over 7 days. Subjective sleep quality was assessed by the Pittsburgh Sleep Quality Index (PSQI). Sleep efficiency and subjective sleep quality were significantly reduced, and sleep fragmentation was increased in BVP patients compared to controls. BVP patients displayed a damped amplitude of the rest-activity rhythm and higher sleep fragmentation, reflected by a higher nocturnal activity compared to controls. These results suggest that both rest-activity and sleep cycles are impaired in BVP patients compared to healthy controls. BVP patients seem to have greater difficulty maintaining good sleep at night compared to controls. BVP pathology appears to affect the sleep-wake cycle and disturb the circadian rhythm synchronization. Nevertheless, these results need further investigation to be confirmed, particularly with larger sample sizes.

Unreliable association between self-reported sense of direction and peripheral vestibular function.

Uni- or bilateral peripheralvestibular impairment causes objective spatial orientation deficits, which can be measured using pen-and-paper-tests or sensorimotor tasks (navigation or pointing). For patients' subjective orientation abilities, questionnaires are commonly used (e.g., Santa Barbara sense of direction scale [SBSODS]). However, the relationship between subjective assessment of spatial skills and objective vestibular function has only been scarcely investigated. A total of 177 patients (mean age 57.86±17.53 years, 90 females) who presented in our tertiary Center for Vertigo and Balance Disorders underwent neuro-otological examinations, including bithermal water calorics, video head impulse test (vHIT), and testing of the subjective visual vertical (SVV), and filled out the SBSODS (German version). Correlation analyses and linear multiple regression model analyses were performed between vestibular test results and self-assessment scores. Additionally, groupwise vestibular function for patients with low, average, and high self-report scores was analyzed. Forty-two patients fulfilled the diagnostic criteria for bilateral vestibulopathy, 93 for chronic unilateral vestibulopathy (68 unilateral caloric hypofunction and 25 isolated horizontal vestibulo-ocular reflex deficits), and 42 patients had normal vestibular test results. SBSODS scores showed clear sex differences with higher subjective skill levels in males (mean score males: 4.94±0.99, females 4.40±0.94; Student's t-test: t-3.78, p<.001***). No stable correlation between objective vestibular function and subjective sense of spatial orientation was found. A multiple linear regression model could not reliably explain the self-reported variance. The three patient groups with low, average, and high self-assessment-scores showed no significant differences of vestibular function. Self-reported assessment of spatial orientation does not robustly correlate with objective peripheral vestibular function. Therefore, other methods of measuring spatial skills in real-world and virtual environments are required to disclose orientation deficits due to vestibular hypofunction.

Simple dynamic stability indicators for characterising and supporting the diagnosis of patients suffering from severe bilateral vestibulopathy

Simple dynamic stability indicators for characterising and supporting the diagnosis of patients suffering from severe bilateral vestibulopathy

Electrical stimulation of the vestibular nerve: evaluating effects and potential starting points for optimization in vestibular implants.

Oscillopsia and unsteadiness are common and highly debilitating symptoms in individuals with bilateral vestibulopathy. A lack of adequate treatment options encouraged the investigation of vestibular implants, which aim to restore vestibular function with motion-modulated electrical stimulation. This review aims to outline the ocular and postural responses that can be evoked with electrical prosthetic stimulation of the semicircular canals and discuss potential approaches to further optimize evoked responses. Particular focus is given to the stimulation paradigm. Feasibility studies in animals paved the way for vestibular implantation in human patients with bilateral vestibulopathy. Recent human trials demonstrated prosthetic electrical stimulation to partially restore vestibular reflexes, enhance dynamic visual acuity, and generate controlled postural responses. To further optimize prosthetic performance, studies predominantly targeted eye responses elicited by the vestibulo-ocular reflex, aiming to minimize misalignments and asymmetries while maximizing the response. Changes of stimulation parameters are shown to hold promise to increase prosthetic efficacy, together with surgical refinements and neuroplastic effects. Optimization of the stimulation paradigm, in combination with a more precise electrode placement, holds great potential to enhance the clinical benefit of vestibular implants.

Bilateral vestibulopathy – Loss of vestibular function and experience of emotions

ObjectiveThe vestibular system is closely connected to emotion-processing neuronal circuits. Patients with bilateral vestibulopathy (BVP), a chronic loss of vestibular function, show remarkably lower rates of psychiatric comorbidities and vertigo-related anxiety (VRA) than those with episodic vertigo/dizziness (v/d). This study aimed to evaluate whether patients with BVP differ from those with episodic v/d in terms of VRA, general anxiety, and cognitions about body and health. MethodsThis cross-sectional study involved a subsample of 202 patients with episodic v/d (i.e., vestibular migraine, vestibular paroxysmia, or Menière's disease) and 43 patients with BVP. All patients underwent standardised neurological/neurotological examinations, structured clinical interviews (SCID-I), and self-report questionnaires, such as the Vertigo Handicap Questionnaire (VHQ), Beck Anxiety Inventory (BAI), Trait Anxiety from the State-Trait-Anxiety Inventory (STAI-T), and Cognitions About Body and Health questionnaire (CABAH). Non-parametric tests were used for analysis. Due to multiple testing, the significance level was set at p ≤ .008. ResultsPatients with episodic v/d exhibited higher VRA levels than those with BVP. However, this difference was not statistically significant (p = .04; r = 0.15, small effect). Additionally, patients with BVP reported more catastrophizing cognitions (p < .001; r = 0.25, small effect) and bodily weakness (p = .003; r = 0.22, small effect) compared to those with episodic v/d. There were no differences in general anxiety levels (BAI and STAI-T) between patients with v/d and those with BVP. ConclusionPatients with episodic v/d and BVP differed in their appraisal (cognition). The difference in VRA (subjective feeling) was not statistically significant. These preliminary results are discussed using a component approach to emotions.

Advances in diagnostic and therapeutic research on bilateral vestibulopathy

Advances in diagnostic and therapeutic research on bilateral vestibulopathy

Using Pupillometry to Evaluate Balance in Patients Implanted with a Cochleo-Vestibular Implant.

Maintaining balance comes naturally to healthy people. In subjects with vestibulopathy, even when compensated, and especially if it is bilateral, maintaining balance requires cognitive effort. Pupillometry is an established method of quantifying cognitive effort. Background/Objectives: We hypothesized that pupillometry would be able to capture the increased effort required to maintain posture in subjects with bilateral vestibulopathy in increasingly difficult conditions. Additionally, we hypothesized that the cognitive workload during balance tasks, indexed by pupil size, would decrease with the activation of the BionicVEST cochleo-vestibular implants. Methods: Subjects with a cochleo-vestibular implant as of March 2023 were recruited, excluding those with ophthalmological issues that precluded pupillometry. Pupillometry was performed using a validated modified videonystagmography system. Computed dynamic posturography and a Modified Clinical Test of Sensory Integration on Balance were performed while the pupil was recorded. Tests were first performed after 24 h of deactivating the vestibular component of the implant. Thereafter, it was reactivated, and after 1 h of rest, the tests were repeated. The pupil recording was processed using custom software and the mean relative pupil diameter (MRPD) was calculated. Results: There was an average of 10.7% to 24.2% reduction in MRPD when the vestibular implant was active, with a greater effect seen in tasks of moderate difficulty, and lesser effect when the task was easy or of great difficulty. Conclusions: Despite technical challenges, pupillometry appears to be a promising method of quantifying the cognitive effort required for maintaining posture in subjects with bilateral vestibulopathy before and after vestibular implantation.

High-Frequency Vestibular Function Is Vulnerable to Presbyvestibulopathy.

In 2019, mild vestibular function deficiency in elder populations was defined as presbyvestibulopathy (PVP) by the Classification Committee of the Bárány Society. The diagnostic criteria include tests for low-, mid-, and high-frequency vestibular function, represented by caloric testing, rotary chair testing, and head impulse testing, respectively. However, there is still a lack of large-scale reports supporting the relationship between vestibular function tests (VFTs) and aging. In this study, we evaluated whether each test is correlated with aging in the elderly population aged over 50. This study retrospectively enrolled 1043 subjects from a single university hospital database after excluding those with unilateral and bilateral vestibulopathy, central dizziness, and acute dizziness. Enrolled subjects had caloric canal paresis <20%, vHIT lateral canal gain >0.6, vHIT interaural difference <0.3, and age >50 years old. Significant negative correlations with age were identified in the vHIT (p < 0.001) and rotary chair test (RCT) 1.0 Hz gain (p = 0.030). However, the caloric test (p = 0.739 and 0.745 on the left and right sides, respectively) and RCT 0.12 Hz gain (p = 0.298) did not show a significant correlation with age. A total of 4.83% of subjects aged 70 years or older showed sub-normal vHIT gain that met the criteria of PVP, whereas only 0.50% of subjects aged 60 to 69 did. The prevalence of sub-normal caloric test results, however, was not significantly different between the two age groups (21.55% in the 60-69 age group and 26.59% in the >70 age group). The high-frequency range vestibular function seems vulnerable to aging, and this is more discernible at age >70 years. The weak correlation between age and low-frequency vestibular function tests, such as the caloric test and low-frequency rotary chair testing, suggests the need to revisit the diagnostic criteria for PVP.

Full-body kinematics and head stabilisation strategies during walking in patients with chronic unilateral and bilateral vestibulopathy

Chronic imbalance is a frequent and limiting symptom of patients with chronic unilateral and bilateral vestibulopathy. A full-body kinematic analysis of the movement of patients with vestibulopathy would provide a better understanding of the impact of the pathology on dynamic tasks such as walking. Therefore, this study aimed to investigate the global body movement during walking, its variability (assessed with the GaitSD), and the strategies to stabilise the head (assessed with the head Anchoring Index). The full-body motion capture data of 10 patients with bilateral vestibulopathy (BV), 10 patients with unilateral vestibulopathy (UV), and 10 healthy subjects (HS) walking at several speeds (slow, comfortable, and fast) were analysed in this prospective cohort study. We observed only a few significant differences between groups in parts of the gait cycle (shoulder abduction–adduction, pelvis rotation, and hip flexion–extension) during the analysis of kinematic curves. Only BV patients had significantly higher gait variability (GaitSD) for all three walking speeds. Head stabilisation strategies depended on the plan of motion and walking speed condition, but BV and UV patients tended to stabilise their head in relation to the trunk and HS tended to stabilise their head in space. These results suggest that GaitSD could be a relevant biomarker of chronic instability in BV and that the head Anchoring Index tends to confirm clinical observations of abnormal head-trunk dynamics in patients with vestibulopathy while walking.

A rare case report of bilateral vestibulopathy due to otosyphilis.

Bilateral vestibulopathy is an important cause of imbalance. There are multiple etiologies of bilateral vestibulopathy (BVP), but reports of BVP due to otosyphilis are rare. A 39-year-old male was referred to our medical center due to vertigo, persistent dizziness and gait disturbance for 2 months. Bilateral vestibulopathy due to otosyphilis was considered in this case, as confirmed through analyses of vestibular function, laboratory tests, and penicillin treatment. The patient was was treated with a high dose of penicillin G (24 × 106 IU/d) for 14 days. The patient's symptoms had improved greatly following treatment, with dizziness and gait disturbance having completely resolved at 3 months following hospital discharge. Bilateral vestibulopathy should be considered when evaluating patients with acute or subacute persistent dizziness. Clinicians should also be aware of the potential for otosyphilis among patients who report BVP.

Development and content validity of the DizzyQuest

IntroductionVestibular disorders have a high prevalence and impact on general health. Currently questionnaires to measure vestibular symptoms and the impact on daily life, do not capture disease-specific symptoms or induce recall bias. The DizzyQuest was introduced, to measure vestibular symptoms in the flow of daily life, using the ecological momentary assessment (EMA) strategy. The development and content validity of the DizzyQuest are described in this article.MethodsThe development process consisted of two stages: First, the development of a concept version of the DizzyQuest based on the conceptual model of positive health, semi-structured interviews with patients diagnosed with bilateral vestibulopathy (BV) and expert input. Second, the assessment of content validity through a 4-week pilot trial, followed by focus group meetings with patients and professionals separately.ResultsPatient interviews, an expert meeting and a conceptual model resulted in the first pilot version of the DizzyQuest, which included a morning-, within-day-, evening- and attack-questionnaire. To report patient characteristics, complimentary patient- and doctor-questionnaires were added. The pilot trial (N = 5) and two patient focus group meetings resulted in multiple suggestions for response options. Two professional focus group meetings provided recommendations for sampling time and sampling schedule. As a result, two versions of the DizzyQuest were developed, consisting of a regular DizzyQuest (DQ) and an extended DizzyQuest-XL (DQ-XL).ConclusionThe DizzyQuest enables the administration of multiple questionnaires during the flow of daily life, therefore increasing reliability and ecological validity. The DQ and DQ-XL, using a big data approach, has the potential to improve precision medicine.

The vestibular symptomatology of Machado-Joseph Disease.

Machado Joseph Disease (MJD) is an autosomal dominant neurodegenerative disease. In previous studies, we described significant bilateral horizontal Vestibulo-Ocular Reflex (VOR) deficit within this population without any reference to the presence of vestibular symptomatology. To evaluate whether, beyond cerebellar ataxia complaints, MJD patients have typical vestibular symptomatology corresponding to the accepted diagnostic criteria of Bilateral Vestibulopathy (BVP) according to the definition of the International Barany Society of Neuro-Otology. Twenty-one MJD, 12 clinically stable chronic Unilateral Vestibulopathy (UVP), 15 clinically stable chronic BVP, and 22 healthy Controls underwent the video Head Impulse Test (vHIT) evaluating VOR gain and filled out the following questionnaires related to vestibular symptomatology: The Dizziness Handicap Inventory (DHI), the Activities-specific Balance Confidence Scale (ABC), the Vertigo Visual Scale (VVS) and the Beck Anxiety Inventory (BAI). The MJD group demonstrated significant bilateral vestibular impairment with horizontal gain less than 0.6 in 71% of patients (0.54±0.17). Similar to UVP and BVP, MJD patients reported a significantly higher level of symptoms than Controls in the DHI, ABC, VVS, and BAI questionnaires. MJD demonstrated significant VOR impairment and clinical symptoms typical of BVP. We suggest that in a future version of the International Classification of Vestibular Disorders (ICVD), MJD should be categorized under a separate section of central vestibulopathy with the heading of bilateral vestibulopathy. The present findings are of importance regarding the clinical diagnosis process and possible treatment based on vestibular rehabilitation.

Bilateral vestibulopathy as the initial presentation of CANVAS

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowly progressing autosomal recessive ataxic disorder linked to an abnormal biallelic intronic (most commonly) AAGGG repeat expansion in the replication factor complex subunit 1 (RFC1). While the clinical diagnosis is relatively straightforward when the three components of the disorder are present, it becomes challenging when only one of the triad (cerebellar ataxia, neuropathy or vestibular areflexia) manifests. Isolated cases of Bilateral Vestibulopathy (BVP) or vestibular areflexia that later developed the other components of CANVAS have not been documented. We report four cases of patients with chronic imbalance and BVP that, after several years, developed cerebellar and neuropathic deficits with positive genetic testing for RFC1. Our report supports the concept that CANVAS should be considered in every patient with BVP of unknown etiology, even without the presence of the other triad components. This is especially important given that about 50% of cases in many BVP series are diagnosed as idiopathic, some of which may be undiagnosed CANVAS.

Vestibular dysfunction and its association with cognitive impairment and dementia.

The vestibular system plays an important role in maintaining balance and posture. It also contributes to vertical perception, body awareness and spatial navigation. In addition to its sensory function, the vestibular system has direct connections to key areas responsible for higher cognitive functions, such as the prefrontal cortex, insula and hippocampus. Several studies have reported that vestibular dysfunction, in particular bilateral vestibulopathy, is associated with an increased risk of cognitive impairment and the development of dementias such as Alzheimer's disease. However, it is still controversial whether there is a causal relationship between vestibular damage and cognitive dysfunction. In this mini-review, we will explore the relationship between the vestibular system, cognitive dysfunction and dementia, hypotheses about the hypothesis and causes that may explain this phenomenon and also some potential confounders that may also lead to cognitive impairment. We will also review multimodal neuroimaging approaches that have investigated structural and functional effects on the cortico-vestibular network and finally, describe some approaches to the management of patients with vestibular damage who have shown some cognitive impairment.

RFC1 and FGF14 Repeat Expansions in Serbian Patients with Cerebellar Ataxia.

The newly discovered intronic repeat expansions in the genes encoding replication factor C subunit 1 (RFC1) and fibroblast growth factor 14 (FGF14) frequently cause late-onset cerebellar ataxia. To investigate the presence of RFC1 and FGF14 pathogenic repeat expansions in Serbian patients with adult-onset cerebellar ataxia. The study included 167 unrelated patients with sporadic or familial cerebellar ataxia. The RFC1 repeat expansion analysis was performed by duplex PCR and Sanger sequencing, while the FGF14 repeat expansion was tested for by long-range PCR, repeat-primed PCR, and Sanger sequencing. We identified pathogenic repeat expansions in RFC1 in seven patients (7/167; 4.2%) with late-onset sporadic ataxia with neuropathy and chronic cough. Two patients also had bilateral vestibulopathy. Repeat expansions in FGF14 were found in nine unrelated patients (9/167; 5.4%) with ataxia, less than half of whom presented with neuropathy and two-thirds with global brain atrophy. Tremor and episodic features were the most frequent additional characteristics in carriers of uninterrupted FGF14 repeat expansions. Among the 122 sporadic cases, 12 (9.8%) carried an expansion in either RFC1 or FGF14, comparable to 4/45 (8.9%) among the patients with a positive family history. Pathogenic repeat expansions in RFC1 and FGF14 are relatively frequent causes of adult-onset cerebellar ataxia, especially among sporadic patients, indicating that family history should not be considered when prioritizing ataxia patients for testing of RFC1 or FGF14 repeat expansions.

Improvement of postural instability in patients with bilateral vestibulopathy in response to low-intensity vestibular noise stimulation

Improvement of postural instability in patients with bilateral vestibulopathy in response to low-intensity vestibular noise stimulation

A clinical 3D pointing test differentiates spatial memory deficits in dementia and bilateral vestibular failure

BackgroundDeficits in spatial memory, orientation, and navigation are often neglected early signs of cognitive impairment or loss of vestibular function. Real-world navigation tests require complex setups. In contrast, simple pointing at targets in a three-dimensional environment is a basic sensorimotor ability which provides an alternative measure of spatial orientation and memory at bedside. The aim of this study was to test the reliability of a previously established 3D-Real-World Pointing Test (3D-RWPT) in patients with cognitive impairment due to different neurodegenerative disorders, bilateral vestibulopathy, or a combination of both compared to healthy participants.MethodsThe 3D-RWPT was performed using a static array of targets in front of the seated participant before and, as a transformation task, after a 90-degree body rotation around the yaw-axis. Three groups of patients were enrolled: (1) chronic bilateral vestibulopathy (BVP) with normal cognition (n = 32), (2) cognitive impairment with normal vestibular function (n = 28), and (3) combined BVP and cognitive impairment (n = 9). The control group consisted of age-matched participants (HP) without cognitive and vestibular deficits (n = 67). Analyses focused on paradigm-specific mean angular deviation of pointing in the azimuth (horizontal) and polar (vertical) spatial planes, of the preferred pointing strategy (egocentric or allocentric), and the resulting shape configuration of the pointing array relative to the stimulus array. Statistical analysis was performed using age-corrected ANCOVA-testing with Bonferroni correction and correlation analysis using Spearman’s rho.ResultsPatients with cognitive impairment employed more egocentric pointing strategies while patients with BVP but normal cognition and HP used more world-based solutions (pBonf 5.78 × 10-3**). Differences in pointing accuracy were only found in the azimuth plane, unveiling unique patterns where patients with cognitive impairment showed decreased accuracy in the transformation tasks of the 3D-RWPT (pBonf < 0.001***) while patients with BVP struggled in the post-rotation tasks (pBonf < 0.001***). Overall azimuth pointing performance was still adequate in some patients with BVP but significantly decreased when combined with a cognitive deficit.ConclusionThe 3D-RWPT provides a simple and fast measure of spatial orientation and memory. Cognitive impairment often led to a shift from world-based allocentric pointing strategy to an egocentric performance with less azimuth accuracy compared to age-matched controls. This supports the view that cognitive deficits hinder the mental buildup of the stimulus pattern represented as a geometrical form. Vestibular hypofunction negatively affected spatial memory and pointing performance in the azimuth plane. The most severe spatial impairments (angular deviation, figure frame configuration) were found in patients with combined cognitive and vestibular deficits.

Is vestibular function related to human hippocampal volume?

Recent studies implicate the effect of vestibular loss on cognitive decline, including hippocampal volume loss. As hippocampal atrophy is an important biomarker of Alzheimer's disease, exploring vestibular dysfunction as a risk factor for dementia and its role in hippocampal atrophy is of interest. To replicate previous literature on whole-brain and hippocampal volume in semicircular canal dysfunction (bilateral vestibulopathy; BV) and explore the association between otolith function and hippocampal volume. Hippocampal and whole-brain MRI volumes were compared in adults aged between 55 and 83 years. Participants with BV (n = 16) were compared to controls individually matched on age, sex, and hearing status (n = 16). Otolith influence on hippocampal volume in preserved semicircular canal function was evaluated (n = 34). Whole-brain and targeted hippocampal approaches using volumetric and surface-based measures yielded no significant differences when comparing BV to controls. Binary support vector machines were unable to classify inner ear health status above chance level. Otolith parameters were not associated with hippocampal volume in preserved semicircular canal function. No significant differences in whole-brain or hippocampal volume were found when comparing BV participants with healthy controls. Saccular parameters in subjects with preserved semicircular canal function were not associated with hippocampal volume changes.

Postural impairments in unilateral and bilateral vestibulopathy.

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.

Multi-frequency VEMPs improve detection of present otolith responses in bilateral vestibulopathy.

To investigate whether multi-frequency Vestibular Evoked Myogenic Potential (VEMP) testing at 500, 750, 1,000, and 2,000 Hz, would improve the detection of present dynamic otolith responses in patients with bilateral vestibulopathy (BV). Prospective study in a tertiary referral center. BV patients underwent multi-frequency VEMP testing. Cervical VEMPs and ocular VEMPs were recorded with the Neuro-Audio system (v2010, Neurosoft, Ivanovo, Russia). The stimuli included air-conducted tone bursts of 500, 750, 1,000, and 2,000 Hz, at a stimulation rate of 13 Hz. Outcome measures included the percentage of present and absent VEMP responses, and VEMP thresholds. Outcomes were compared between frequencies and type of VEMPs (cVEMPs, oVEMPs). VEMP outcomes obtained with the 500 Hz stimulus, were also compared to normative values obtained in healthy subjects. Forty-nine BV patients completed VEMP testing: 47 patients completed cVEMP testing and 48 patients completed oVEMP testing. Six to 15 % more present VEMP responses were obtained with multifrequency testing, compared to only testing at 500 Hz. The 2,000 Hz stimulus elicited significantly fewer present cVEMP responses (right and left ears) and oVEMP responses (right ears) compared to the other frequencies (p ≤ 0.044). Using multi-frequency testing, 78% of BV patients demonstrated at least one present VEMP response in at least one ear. In 46% a present VEMP response was found bilaterally. BV patients demonstrated a significantly higher percentage of absent VEMP responses and significantly higher VEMP thresholds than healthy subjects, when corrected for age (p ≤ 0.002). Based on these results, a pragmatic VEMP testing paradigm is proposed, taking into account multi-frequency VEMP testing. Multi-frequency VEMP testing improves the detection rate of present otolith responses in BV patients. Therefore, multi-frequency VEMPs should be considered when evaluation of (residual) otolith function is indicated.