Objective To optimize the current diagnostic and treatment procedures for patients with bilateral vestibulopathy (BV), this study aimed to determine the complete spectrum of symptoms associated with BV. Method A prospective mixed-method study design was used. Qualitative data were collected by performing semi-structured interviews about symptoms, context, and behavior. The interviews were recorded and transcribed until no new information was obtained. Transcriptions were analyzed in consensus by two independent researchers. In comparison to the qualitative results, quantitative data were collected using the Dizziness Handicap Inventory (DHI), Hospital Anxiety and Depression Scale (HADS) and a health-related quality of life questionnaire (EQ-5D-5L). Results Eighteen interviews were transcribed. Reported symptoms were divided into fourteen physical symptoms, four cognitive symptoms, and six emotions. Symptoms increased in many situations, such as darkness (100%), uneven ground (61%), cycling (94%) or driving a car (56%). These symptoms associated with BV often resulted in behavioral changes: activities were performed more slowly, with greater attention, or were avoided. The DHI showed a mean score of severe handicap (54.67). The HADS questionnaire showed on average normal results (anxiety = 7.67, depression = 6.22). The EQ-5D-5L demonstrated a mean index value of 0.680, which is lower compared to the Dutch age-adjusted reference 0.839 (60–70 years). Conclusion BV frequently leads to physical, cognitive, and emotional complaints, which often results in a diminished quality of life. Importantly, this wide range of symptoms is currently underrated in literature and should be taken into consideration during the development of candidacy criteria and/or outcome measures for therapeutic interventions such as the vestibular implant.

A homozygous mutation in the flavoprotein (Fp) gene associated with complex II deficiency was demonstrated in a patient with consanguineous parents. She succumbed at 5(1/2) months of age following a respiratory infection. The c1664G-->A transition detected, predicted the substitution of the small uncharged glycine at position 555 by glutamic acid. Her clinical course was at variance with the Leigh syndrome in three previously reported patients due to Fp gene mutations. In this proband, CRM for flavoprotein as well as iron-containing protein (Ip) was decreased, CRM for the entire complex II (130 kDa) being reduced even more. This observation prompts speculation of a labile interaction between Ip and Fp polypeptides and of a key role of the amino acid at position 555 in the interacting domain.

Objectives: Patients suffering from vestibular disorders (VD) often present with impairments in cognitive domains such as visuospatial ability, memory, executive function, attention, and processing speed. These symptoms can be attributed to extensive vestibular projections throughout the cerebral cortex and subcortex on the one hand, and to increased cognitive-motor interference (CMI) on the other hand. CMI can be assessed by performing cognitive-motor dual-tasks (DTs). The existing literature on this topic is scarce and varies greatly when it comes to test protocol, type and degree of vestibular impairment, and outcome. To develop a reliable and sensitive test protocol for VD patients, an overview of the existing reliability and validity studies on DT paradigms will be given in a variety of populations, such as dementia, multiple sclerosis, Parkinson’s disease, stroke, and elderly. Design: The systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. An extensive literature search on psychometric properties of cognitive-motor DTs was run on MEDLINE, Embase, and Cochrane Databases. The studies were assessed for eligibility by two independent researchers, and their methodological quality was subsequently evaluated using the Consensus-based Standards for the selection of health Measurement Instruments (COSMIN). Results and Conclusions: Thirty-three studies were included in the current review. Based on the reliability and validity calculations, including a static as well as dynamic motor task seems valuable in a DT protocol for VD patients. To evoke CMI maximally in this population, both motor tasks should be performed while challenging the vestibular cognitive domains. Out of the large amount of cognitive tasks employed in DT studies, a clear selection for each of these domains, except for visuospatial abilities, could be made based on this review. The use of the suggested DTs will give a more accurate and daily life representation of cognitive and motor deficiencies and their interaction in the VD population.

Objectives: Given the close interconnection between the auditory and vestibular end organs, the increasingly broad application of (bilateral) cochlear implantation (CI) in children raises concern about its impact on the vestibular function. Unfortunately, literature on this matter is inconclusive and subject to several limitations. Therefore, this study aimed to elucidate the impact of pediatric CI on the vestibular function in a large sample of children, representative for the current CI population. Design: Fifty hearing-impaired children followed in the Ghent University Hospital were included in this prospective study. Twenty-seven patients underwent unilateral CI, and 23 were bilaterally implanted (9 sequentially, 14 simultaneously), adding up to 73 implanted ears. Children’s median age at first implantation was 29 (range 8 to 194) months. Vestibular assessment was scheduled on average 2.8 months (SD: 3.6) before and 4.6 (SD: 4.0) months after implantation and consisted of video Head Impulse Testing of the lateral semicircular canals, rotatory testing (0.16, 0.04, and 0.01 Hz) and cervical vestibular evoked myogenic potential (cVEMP) testing with bone conduction stimulation. Caloric testing was added in children older than 3 years of age. Results: Overall, group analysis in our sample of 73 CI-ears did not reveal any significant impact on the vestibular function, except for a significantly shortened ipsilateral N1 latency of the cVEMP responses (p = 0.027) after CI. Complete ipsilateral loss of function after implantation was seen in 5% (3/54) of all CI-ears on the video head impulse testing, in 0% (0/10) on the caloric test and in 2% (1/52) on the cVEMP, notably all patients deafened by a congenital cytomegalovirus infection. Conclusions: The impact of CI on the vestibular function in our dataset was limited. Therefore, the many advantages of simultaneous bilateral implantation may outweigh the risk for vestibular damage postoperatively. However, the impact on the vestibular function may be dependent on various factors (e.g., etiology of the hearing loss), and the clinical outcome is still difficult to predict. Vestibular assessment remains thus an important aspect in the pediatric CI population; first because the vestibular function should be considered in the decision-making process on (simultaneous or sequential bilateral) CI and second because it is essential to reveal a possible additional sensory deficit, allowing an opportunity for rehabilitation to improve the overall outcome of these children.

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Because type I hair cells (particularly of the semicircular canals) are more susceptible to ototoxicity, video Head Impulse test and vestibular evoked myogenic potential testing seem more promising for the early detection of vestibulotoxicity than caloric and rotatory testing. Prospective studies using an extensive vestibular test battery are needed to further characterize the impact of AGs on the different vestibular end organs and to identify the most sensitive vestibular technique for the early detection of vestibulotoxicity.

Background This pilot study aimed to explore the impact of visual, auditory and vestibular dysfunctions on the postural balance performance in adults with intellectual disabilities. Additionally, a comparison was made between the subjects with intellectual disabilities and a control group concerning static and dynamic balance tasks. Method Thirty adults with intellectual disabilities and 25 control subjects received a postural balance assessment. Additionally, the experimental group was subjected to a visual, auditory and vestibular screening. Results The experimental group performed significantly worse and/or showed more sway compared to the control group for all balance tasks (p < .01) except the timed up and go test. Within the experimental group, a significant positive correlation (rs(24) = 0.513, p = .007) was observed between the number of failed sensory screening items and the number of failed balance tasks. Conclusions These findings suggest significant involvement of peripheral sensorial deficits in the balance problems that people with intellectual disabilities often experience.

IntroductionAside from primary vestibular symptoms such as vertigo and dizziness, persons with vestibular dysfunction frequently express cognitive and motor problems. These symptoms have mainly been assessed in single-task setting, which might not represent activities of daily living accurately. Therefore, a dual-task protocol, consisting of the simultaneous performance of cognitive and motor tasks, was developed. This protocol assesses cognitive and motor performance in general, as well as cognitive-motor interference in specific.Methods and analysisThe motor component of the 2BALANCE protocol consists of a static and dynamic postural task. These motor tasks are combined with different cognitive tasks assessing visuospatial cognition, processing speed, working memory and response inhibition. First, test–retest reliability will be assessed with an interval of 2 weeks in a group of young adults. Second, the 2BALANCE protocol will be validated in persons with bilateral vestibulopathy. Finally, the protocol will be implemented in persons with unilateral vestibular loss.Discussion and conclusionsThe 2BALANCE project aims to elucidate the impact of vestibular dysfunction on cognitive and motor performance in dual-task setting. This protocol represents everyday situations better than single-task protocols, as dual-tasks such as reading street signs while walking are often encountered during daily activities. Ultimately, this project could enable individualised and holistic clinical care in these patients, taking into account single as well as dual-task performance.Ethics and disseminationThe current study was approved by the ethics committee of Ghent University Hospital on 5 July 2019 with registration number B670201940465. All research findings will be disseminated in peer-reviewed journals and presented at vestibular as well as multidisciplinary international conferences and meetings.Trials registration numberNCT04126798.