Disorder-Not CHARGE Syndrome-Presenting as Isolated Cochleovestibular Dysfunction.

CHARGE syndrome, characterized by a distinct set of clinical features, has been linked primarily to mutations in the gene. Initially defined by specific clinical criteria, including coloboma, heart defects, choanal atresia, delayed growth, and ear anomalies, CHARGE syndrome's diagnostic spectrum has broadened since the identification of . Variants in this gene exhibit considerable phenotypic variability, leading to the adoption of the term " disorder" to encompass a wider range of associated symptoms.

A Clinical Framework for Video Head Impulse Testing and Vestibular Evoked Myogenic Potential Assessments in Primary School-Aged Children.

Objectives: This study aimed to offer normative data and age trends of an age-appropriate vestibular test protocol in a large group (n = 140) of school-aged children (6 to 13 years old) as well as to provide a practical and clinical framework for accurate performance and interpretation of vestibular test results in this specific age group.

Design: The typically developing participants (mean age of 9.51 ± 2.

Cognitive outcomes in Susac syndrome: A 2-year neuropsychological follow-up study.

Background And Purpose: Susac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long-term neuropsychological outcomes of patients with SuS.

The impact of vestibular function on cognitive-motor interference: a case-control study on dual-tasking in persons with bilateral vestibulopathy and normal hearing.

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.

Chirp-Evoked VEMPs: A Test-Retest Reliability Study.

Objectives: To determine the test-retest reliability of cervical and ocular vestibular-evoked myogenic potentials (c&oVEMP) evoked by 500 Hz narrowband (NB) CE-Chirp and broadband (BB) CE-Chirp stimuli.

Design: Twenty healthy participants (10 female) were tested twice on the same day to determine the within-session reliability and 1 week later to determine the between-session reliability. The latency, amplitude, and asymmetry ratio of c&oVEMPs elicited by 95 dB nHL air-conducted (AC) 500 Hz NB CE-Chirp and BB CE-Chirp were recorded bilaterally.

Association of Bilateral Vestibulopathy With and Without Hearing Loss With Cognitive-Motor Interference.

Importance: The past years, evidence suggested that the primary symptoms traditionally associated with bilateral vestibulopathy (BV) do not represent the full picture of this patient population. Recent literature also demonstrated cognitive impairment. However, although multitasking and dual-tasking are widely present in everyday activities, most of these studies assessed cognitive function only in single-task conditions.

A cross-sectional study on the neurocognitive outcomes in vestibular impaired school-aged children: are they at higher risk for cognitive deficits?

This study aimed to assess if children with a vestibular impairment (VI) are more prone to have neurocognitive deficits compared to typically developing (TD) peers, taking into account important confounding factors with hearing loss being the most important. The neurocognitive performance of fifteen VI children (6-13 years old) was compared to that of an age-, handedness- and sex-weighted group of TD peers (n = 60). Secondly, their performance was also compared to matched groups of TD and hearing impaired (HI) children to evaluate the involvement of HI.

Vestibular Follow-up Program for Congenital Cytomegalovirus Based on 6 Years of Longitudinal Data Collection.

Objectives: Congenital cytomegalovirus (cCMV), the leading nongenetic cause of pediatric sensorineural hearing loss, can also affect vestibular function. Literature findings suggest clinical presentation of vestibular loss in cCMV to be as variable as the hearing loss. Still, probably due to the considerable additional burden it entails for both patients and diagnostic centers, longitudinal vestibular follow-up in cCMV is not well-established in clinical practice.

Self-Reported Cycling Performance and Impact on Falls in Persons With Bilateral Vestibulopathy.

Objectives: The aim of this study was to investigate cycling skills, cycling difficulties, fall concern, and fall history while cycling in persons with bilateral vestibulopathy.

Design: Fifty-one persons with bilateral vestibulopathy and 51 healthy control subjects filled out a questionnaire about cycling abilities. This questionnaire consisted of four parts: (1) general information about audiovestibular function, vision, and physical activity; (2) general cycling abilities, history, and difficulties; (3) fall concern while cycling in various situations; and (4) fall history.

Proportion of falls reported in persons with Parkinson's disease: A meta-analysis.

Objective: Falls can be highly debilitating and have an important negative impact on the quality of life of patients with Parkinson's disease (PD). The aim of this systematic review and meta-analysis is to provide an up-to-date overview of the prevalence of ≥1 fall and ≥2 falls in idiopathic PD.

Data Sources: MEDLINE, Web of Science, Embase and Cinahl databases were systematically searched until 04 July 2022 for prospective studies reporting fall prevalence in persons with idiopathic PD.

Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort.

Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and paraclinical data of 19 SuS patients followed at Ghent University Hospital and highlight some atypical clinical and novel radiological findings. Our findings suggest that spinal involvement expands the clinical phenotype of SuS.

Effects of Oral Levodopa on Balance in People with Idiopathic Parkinson's Disease.

Background: Balance impairment is a frequent cause of morbidity and mortality in people with Parkinson's disease (PD). As opposed to the effects of appendicular motor symptoms, the effects of Levodopa on balance impairment in idiopathic PD are less clear.

Objective: To review the literature on the effects of oral Levodopa on clinical balance test performance, posturography, step initiation, and responses to perturbation in people with idiopathic PD (PwPD).

Pediatric Vestibular Assessment: Clinical Framework.

Objectives: Although vestibular deficits can have severe repercussions on the early motor development in children, vestibular assessment in young children has not yet been routinely integrated in clinical practice and clear diagnostic criteria to detect early vestibular deficits are lacking. In young children, specific adjustments of the test protocol are needed, and normative data are age-dependent as the vestibular pathways mature through childhood. Therefore, this study aims to demonstrate the feasibility of an extensive age-dependent vestibular test battery, to provide pediatric normative data with the concurrent age trends, and to offer a clinical framework for pediatric vestibular testing.

Vestibular Infant Screening-Flanders: What is the Most Appropriate Vestibular Screening Tool in Hearing-Impaired Children?

Objectives: As children with sensorineural hearing loss have an increased risk for vestibular impairment, the Vestibular Infant Screening-Flanders project implemented a vestibular screening by means of cervical vestibular evoked myogenic potentials (cVEMP) at the age of 6 months for each child with hearing loss in Flanders (Belgium). Given that vestibular deficits can affect the child's development, this vestibular screening should allow early detection and intervention. However, less is currently known about which screening tool would be the most ideal and how vestibular impairment can evolve.

Predicting Early Vestibular and Motor Function in Congenital Cytomegalovirus Infection.

Objectives: Congenital cytomegalovirus (cCMV) can affect vestibular function, which is an important cornerstone for early motor development. This study aims to identify risk factors for early vestibular dysfunction with severe repercussions on the motor outcome.

Methods: This prospective cohort study included 169 cCMV-patients with complete vestibular assessment (lateral video Head Impulse Test and cervical Vestibular Evoked Myogenic Potentials) before the age of 18 months (mean 8.

Three Years of Vestibular Infant Screening in Infants With Sensorineural Hearing Loss.

Objectives: Although vestibular deficits are more prevalent in hearing-impaired children and can affect their development on many levels, a pediatric vestibular assessment is still uncommon in clinical practice. Since early detection may allow for timely intervention, this pioneer project has implemented a basic vestibular screening test for each six-month-old hearing-impaired infant in Flanders, Belgium. This study aims to report the vestibular screening results over a period of three years and to define the most important risk factors for abnormal vestibular screening results.

Cervical and ocular vestibular evoked myogenic potential: A comparison of narrowband chirp, broadband chirp, tone burst and click stimulation.

Objectives: To compare the response rate and response parameters of cervical and ocular vestibular evoked myogenic potentials (c&oVEMP) elicited by narrowband (NB) and broadband (BB) CE-Chirp, with the more classical tone burst (TB) and click VEMPs.

Design: The response rate, latency, amplitude and asymmetry ratio of c&oVEMPs elicited by 95 dB nHL air conducted (AC) 500 Hz NB CE-chirp, BB CE-chirp, 500 Hz TB and click stimuli were recorded bilaterally.

Study Sample: 20 male and 38 female participants (19-39 years).

Global Data on Ear and Hearing Screening in an Intellectual Disability Population.

Intellectual disability (ID) and hearing loss are frequent comorbid conditions, although otological problems often go unnoticed until picked up by screening. In the hearing program of Special Olympics (SO), athletes with ID are screened for otological problems. By retrospective analysis of all SO meetings between 2007 and 2017, more than 100,000 screenings could be included.

Assessing the Clinical Value of Objective and Patient-Reported Audiovestibular Outcome Measures in the Risk Estimation of Systemic Cobalt Toxicity for Patients With a Metal-on-Metal Hip Implant.

Objectives: Based on limited evidence from case reports and small cohort studies of metal-on-metal (MoM) hip implant patients with presumed systemic cobalt (Co) toxicity, and a few animal studies on Co-induced damage to the otovestibular system, it was hypothesised that Co exhibits an ototoxic potential alongside other systemic manifestations. Preliminary investigations from our research group in MoM patients confirmed this assumption for the auditory system, whereas no signs of Co-induced vestibular impairment were detected, and a clear dose-response relationship between the auditory function and the blood Co levels was lacking. Therefore, the current study aimed to extend and validate these findings in a larger sample of MoM patients and control subjects, to explore the potential clinical value of audiovestibular outcome measures in the risk estimation of systemic Co toxicity in this patient population.

2BALANCE: Test-retest reliability of a cognitive-motor dual-task protocol.

Purpose: Aside from typical symptoms such as dizziness and vertigo, persons with vestibular disorders often have cognitive and motor problems. These symptoms have been assessed in single-task condition. However, dual-tasks assessing cognitive-motor interference might be an added value as they reflect daily life situations better.

Postural balance problems in people with intellectual disabilities: Do not forget the sensory input systems.

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.

Diagnostic accuracy and usability of the EMBalance decision support system for vestibular disorders in primary care: proof of concept randomised controlled study results.

Background: Dizziness and imbalance are common symptoms that are often inadequately diagnosed or managed, due to a lack of dedicated specialists. Decision Support Systems (DSS) may support first-line physicians to diagnose and manage these patients based on personalised data.

Aim: To examine the diagnostic accuracy and application of the EMBalance DSS for diagnosis and management of common vestibular disorders in primary care.

Changes in Vestibular Function Following Pediatric Cochlear Implantation: a Prospective Study.

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.

Outcome of 10 years of ear and hearing screening in people with intellectual disability in Europe: A multicentre study.

Background: Undetected ear and hearing problems negatively impact the quality of life of people with intellectual disability.

Methods: This multicentre retrospective study describes the outcome of 10 years of ear and hearing screening in athletes with intellectual disability in seven European countries. The screening was conducted by a trained team using a strictly standardised protocol.