Etiologies and hearing status in bilateral vestibulopathy: a retrospective study of 315 patients.

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

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.

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.

Driving ability in patients with dizziness: a systematic review.

PURPOSE: The aim of this systematic review was to identify and evaluate studies dealing with driving performance of dizzy patients or patients with a vestibular disorder. METHODS: A systematic review was performed according to the preferred reporting items for systematic reviews and meta-analysis guidelines. (1) PubMed, Embase, and Cochrane library. (2) Study selection: articles about driving ability and reported driving difficulties in patients with dizziness, or a diagnosed vestibular disorder, were included. (3) Data extraction was performed by two independent authors using predefined data fields: patient's characteristics, diagnostic criteria, sample size, and type of evaluation of driving ability and outcome of the study. RESULTS: Eight out of 705 articles matched the inclusion criteria but varied widely regarding the study population, study design, and outcome measures. The majority of studies reported a negative impact of dizziness and/or vestibular disorders on self-reported driving ability and car accidents. Yet several studies could not identify any impairment of driving ability. CONCLUSIONS: Driving ability was negatively affected by dizziness or a vestibular disorder in the majority of included studies with low risk of bias. This systematic review revealed a significant heterogeneity in studies reporting driving performance and contradictory results. We were, therefore, unable to identify a causal relationship between dizziness and driving ability. There is a need for prospective studies in populations with different vestibular disorders using subjective and objective outcome measures that have been validated to evaluate driving performance.

The Relationship Between the Activities-Specific Balance Confidence Scale and Balance Performance, Self-perceived Handicap, and Fall Status in Patients With Peripheral Dizziness or Imbalance.

OBJECTIVE: Describe the relationship between the Activities-Specific Balance Confidence (ABC) scale and Dizziness Handicap Inventory (DHI) with balance performance, as well as fall status in patients with peripheral vestibular disorders. STUDY DESIGN: Retrospective. SETTING: Outpatient balance clinic, tertiary referral center. PATIENTS: Data from 97 patients (age: 54.8 ±â€Š12.3 yrs; 48 women) with dizziness or imbalance symptoms of peripheral vestibular origin were used for analysis. INTERVENTIONS: /. MAIN OUTCOME MEASURES: ABC-scores, DHI-scores, static and dynamic balance tests, and fall status of the past 4 weeks, 2 months, and 6 months before the time of measurement were collected. Spearman's rho correlations, χ2 with post-hoc testing, and Kruskal-Wallis with post-hoc Mann-Whitney U test results were interpreted. RESULTS: The ABC- and DHI-scores show moderate correlations with static balance (ABC: r = 0.44; DHI: r = -0.34) and dynamic balance tests (ABC: r = [-0.47;0.56]; DHI: r = [-0.48;0.39]) and a strong inverse correlation with each other (ABC: 70 ±â€Š25; DHI: 33 ±â€Š26; r = -0.84). Related to fall status, weak correlations were found (ABC: r = [-0.29;-0.21]; DHI: r = [0.29;0.33]). Additional results show that subjects in the low-level functioning (ABC) or severe self-perceived disability (DHI) categories have a poorer balance assessed by standing balance, Timed-Up-and-Go and Functional Gait Assessment and are more likely to have experienced multiple falls. CONCLUSIONS: The ABC-scale and DHI showed a strong convergent validity, additionally the ABC-scale showed a better concurrent validity with balance performances and the DHI with fall history. In general, patients with peripheral vestibular impairments reporting a lower self-confidence or a more severe self-perceived disability show worse balance performances and a higher fall incidence.

Paving the Way Toward Distinguishing Fallers From Non-fallers in Bilateral Vestibulopathy: A Wide Pilot Observation.

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

The Virtual Morris Water Task in 64 Patients With Bilateral Vestibulopathy and the Impact of Hearing Status.

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

Prospective cohort study on the predictors of fall risk in 119 patients with bilateral vestibulopathy.

OBJECTIVES: To identify predictive factors for falls in patients with bilateral vestibulopathy (BV). Specific variables contributing to the general work-up of a vestibular patient were compared between BV patients experiencing falls and those who did not. DESIGN: Prospective multi-centric cohort study. SETTING: Department of Otorhinolaryngology & Head and Neck Surgery at two tertiary referral centers: Antwerp University Hospital and Maastricht University Medical Center. PARTICIPANTS: In total, 119 BV patients were included. BV diagnosis was defined in accordance with the diagnostic BV criteria, established by the Bárány Society in 2017. MAIN OUTCOME MEASURES: Patients were divided into fallers and non-fallers, depending on the experience of one or more falls in the preceding 12 months. Residual vestibular function on caloric testing, rotatory chair testing, video head impulse test (vHIT) and cervical vestibular evoked myogenic potentials (cVEMP) was evaluated as a predictive factor for falls. Furthermore, hearing function (speech perception in noise (SPIN)), sound localization performance, etiology, disease duration, sport practice, scores on the Dizziness Handicap Inventory (DHI) and the Oscillopsia Severity Questionnaire (OSQ) were compared between fallers and non-fallers. RESULTS: Forty-five (39%) patients reported falls. In a sub-analysis in the patients recruited at UZA (n = 69), 20% experienced three or more falls and three patients (4%) suffered from severe fall-related injuries. The DHI score and the OSQ score were significantly higher in fallers. Residual vestibular function, SPIN, sound localization performance, etiology, disease duration, age and sport practice did not differ between fallers and non-fallers. CONCLUSIONS: Falls and (severe) fall-related injuries are frequent among BV patients. A DHI score > 47 and an OSQ score > 27.5 might be indicative for BV patients at risk for falls, with a sensitivity of 70% and specificity of 60%. Residual vestibular function captured by single vestibular tests (vHIT, calorics, rotatory chair, cVEMP) or by overall vestibular function defined as the number of impaired vestibular sensors are not suitable to distinguish fallers and non-fallers in a BV population.

Impact of Bilateral Vestibulopathy on Spatial and Nonspatial Cognition: A Systematic Review.

OBJECTIVES: 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. DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

Cognitive Function in Acquired Bilateral Vestibulopathy: A Cross-Sectional Study on Cognition, Hearing, and Vestibular Loss.

BACKGROUND: Several studies have demonstrated cognitive deficits in patients with bilateral vestibulopathy (BVP). So far, little attention has been paid to the hearing status of vestibular patients when evaluating their cognition. Given the well-established link between sensorineural hearing loss (SNHL) and cognitive decline and the high prevalence of SNHL in BVP patients, it is therefore uncertain if the cognitive deficits in BVP patients are solely due to their vestibular loss or might be, partially, explained by a concomitant SNHL. OBJECTIVE: To evaluate the link between cognition, hearing, and vestibular loss in BVP patients. DESIGN: Prospective cross-sectional analysis of cognitive performance in patients with BVP and control participants without vestibular loss. Both groups included subjects with a variety of hearing (dys)function. Cognition was assessed by means of the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing Impaired Individuals (RBANS-H). RESULTS: Sixty-four BVP patients were evaluated and compared with 83 control participants. For each subscale and the totale RBANS-H scale a multiple linear regression model was fitted with the following variables: vestibular loss, hearing loss, age, gender, and education. Hearing loss seemed to be associated with worse outcome on the total RBANS-H scale and subscales immediate memory and language. Vestibular loss, on the other hand, was linked to worse performance on the attention subscale of the RBANS-H. Furthermore, we did not observe a correlation between saccular function and cognition. CONCLUSION: This study has found general cognitive deficits in a large sample size of BVP patients. Multiple linear regression models revealed that both vestibular and hearing dysfunction were associated with different subscales of the cognitive test battery, the RBANS-H. Whereas hearing loss was associated with worse performance on total RBANS-H score, immediate memory and language, vestibular loss was observed to negatively affect attention performance.

Early versus late pulmonary valve replacement in patients with transannular patch-repaired tetralogy of Fallot.

OBJECTIVES: Although the effects of pulmonary regurgitation after tetralogy of Fallot repair are detrimental, timing of pulmonary valve replacement (PVR) is unclear. Our goal was to evaluate the midterm efficacy and safety of early PVR. METHODS: Patients with tetralogy of Fallot who underwent repair from 1962 to 2015 were included from the local database. Statistical analyses compared patients who underwent early PVR (age ≤16 years), late PVR and no PVR. The timing of the intervention was compared for efficacy-all-cause mortality and the combined end-point of all-cause mortality, ventricular tachycardia and defibrillator implantation-and for safety-the combined end-point of 1-year postoperative mortality after PVR, endocarditis and reintervention. Echocardiographic and electrocardiographic data at the last follow-up examination were compared across the 3 groups. RESULTS: Two hundred seventy-three patients (age 21 ± 5 years; 52% female) were included. The mean follow-up was 24 (95% confidence interval 22.7-26.2) years; the observed median was 21 years (interquartile range 11-31). No significant difference in survival was found between the early PVR (n = 106; 39%), the late PVR (n = 47; 17%) and the no PVR groups (n = 120; 44%) (P = 0.990). No significant difference in the combined efficacy end-point was noted between patients who underwent early PVR compared with patients who underwent late PVR (P = 0.247). Worse event-free survival for the 3-point safety end-point was observed after early PVR (P < 0.001). Right ventricular morphology (P < 0.001) and function (P < 0.001) were better preserved in the patient group that underwent PVR before the age of 16 years. CONCLUSIONS: As expected, PVR-related morbidity was higher in patients who underwent early PVR but the midterm outcome was similar. Nevertheless, better preservation of right ventricular morphology and function in the early PVR group might result in better long-term survival.

Ventricular arrhythmia during ajmaline challenge for the Brugada syndrome.

The Brugada syndrome is a genetic disease characterized by an abnormal electrocardiogram (ECG) and an elevated risk of sudden cardiac death. Sodium channel blockers (SCBs), such as ajmaline, are used to unmask the characteristic type 1 Brugada electrocardiographic pattern. We review the literature on the incidence of ventricular arrhythmia (VA) during SCB challenge. We evaluate the clinical and electrocardiographic characteristics of these patients as well as their prognosis. All articles published from January 2000 until August 2015, in which the incidence and predictors of VAs during SCB challenge were reported, are reviewed. The occurrence of VA during SCB challenge ranges from 0 to 17.8%. The weighted average for induction of any VA during sodium blocking challenge is 2.4%; for non-sustained ventricular tachycardia (VT), it is 0.34% and for sustained VT 0.59%. No fatal cases were reported. Predictors may be young age, conduction disturbance at baseline ECG, and mutations in the SCN5A gene. All other clinical and electrocardiographic characteristics failed to be consistent predictors. Life-threatening arrhythmias during SCB challenge are not an exceptional event. Therefore, provocation testing must necessarily be performed in an appropriate environment in which advanced life support facilities are present. Patients who have a higher risk for induced arrhythmias might be those who display a conduction disturbance at baseline ECG or have certain SCN5A mutations or are of a younger age. However, survivors of these induced arrhythmias do not seem to suffer from a worse prognosis.