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. METHODS: Thirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well-being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow-up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years). RESULTS: Patients showed normal neuropsychological test results at a group level, both at baseline and follow-up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2-year period. In addition, patients reported significantly lower mental and physical well-being, both at baseline and follow-up testing. CONCLUSIONS: Our results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho-emotional burden of the disease was reported, for which screening and follow-up are necessary.

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. RESULTS: A moderate to good between-session reliability with intraclass correlation coefficient (ICC) values ranging from 0.52 to 0.82 was observed for cVEMP latency, amplitude, and asymmetry ratio evoked by 500 Hz NB CE-Chirp, as well as for the BB CE-Chirp cVEMP amplitude (ICC of 0.70 and 0.84). In contrast, an overall poor reliability ICC values between 0.30 and 0.42 for cVEMP latency and asymmetry ratio were observed for BB CE-Chirp. For the oVEMP, overall poor between-session reliability for all response parameters evoked by the 500 Hz NB CE-Chirp and the BB CE-Chirp was observed. CONCLUSIONS: The 500 Hz NB CE-Chirp was more reliable than the BB CE-Chirp in terms of cVEMP latency, amplitude, and asymmetry ratio. Further investigation using the standard electrode montage is necessary to assess the test-retest reliability of the chirp-evoked oVEMP.

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 ± 2.04 years) were recruited to provide a representative group of 20 children for each of the seven age groups that were composed of children aged from 6 to 13 years in 1-year intervals. Each age group consisted of 10 boys and 10 girls. The protocol comprises the video head impulse test, and cervical and ocular vestibular evoked myogenic potential assessments to provide a child-friendly, noninvasive, short, and portable test battery, which is equally applicable in the hospital and office-practice, and which provides information on the integrity of all five parts of the peripheral vestibular system. RESULTS: The study demonstrates that all included tests and methods, with an overall test duration of 25 min 12 sec ± 5 min 10 sec, were feasible to perform in primary school-aged children, taking into account some practical adaptations. Concerning the video head impulse test, no clinically relevant sex and age effects were noted. However, t tests revealed significant differences for the mean gain of the horizontal (right > left; t[139] = 14.563; p < 0.001) and posterior semicircular canals (left > right; t[139] = -4.823; p < 0.001) between both sides. For the cVEMP assessment, no laterality differences were observed for any of the parameters, but a significantly shorter N1 latencies in the youngest age categories (<8 years), compared with the oldest groups were observed [F(6,118) = 8.336; p < 0.001; partial ƞ² = 0.298]. For all oVEMP parameters, no laterality, sex, or age differences were seen. On the basis of the presented normative data, cutoff criteria were proposed with accompanying clinical recommendations to perform vestibular function testing in this target population. CONCLUSIONS: This is the first study in a large group of school-aged children offering normative data and age trends of an age-appropriate vestibular test protocol that evaluates the integrity of all parts of the peripheral vestibular organ. The reported normative values and clinical cutoff values will enable appropriate and age-specific interpretation of clinical and scientific results. Moreover, in combination with extensive history taking, and additional vestibular testing (e.g., rotatory chair test, caloric testing) when needed, the results of this study may support clinicians in the diagnosis of side-specific and location-specific vestibular deficits, which is required for accurate counseling and referral for further follow-up and/or intervention.

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. DESIGN: This normative study included 133 healthy children below the age of 4 years (mean: 22 mo, standard deviation: 12.3 mo, range: 5-47 mo) without history of hearing loss or vestibular symptoms. Children were divided into four age categories: 38 children younger than 1 year old, 37 one-year olds, 33 two-year olds, and 25 three-year olds. Children younger than 3 years of age were examined with the video Head Impulse Test (vHIT) of the horizontal semicircular canals, cervical vestibular evoked myogenic potentials (cVEMP) with bone conduction stimuli, and the rotatory test at 0.16, 0.04, and 0.01 Hz. In 3-year old children, the vHIT of the vertical semicircular canals and ocular vestibular evoked myogenic potentials (oVEMP) using a minishaker were added to the protocol. RESULTS: The horizontal vHIT appeared to be the most feasible test across age categories, except for children younger than 1-year old in which the success rate was the highest for the cVEMP. Success rates of the rotatory test varied the most across age categories. Age trends were found for the vHIT as the mean vestibulo-ocular reflex (VOR) gain increased significantly with age (r = 0.446, p < 0.001). Concerning the cVEMP, a significant increase with age was found for latency P1 (r = 0.420, p < 0.001), rectified interpeak amplitude P1-N1 (r = 0.574, p < 0.001), and averaged electromyographic (EMG) activity (r = 0.430, p < 0.001), whereas age trends for the latency N1 were less pronounced (r = 0.264, p = 0.004). Overall, the response parameters of the rotatory test did not show significant age effects ( p > 0.01), except for the phase at 0.01 Hz (r = 0.578, p < 0.001). Based on the reported success rates and age-dependent normative vestibular data, straightforward cutoff criteria were proposed (vHIT VOR gain < 0.7, cVEMP rectified interpeak amplitude < 1.3, oVEMP interpeak amplitude < 10 µV) with accompanying clinical recommendations to diagnose early vestibular impairment. CONCLUSIONS: In this large cohort of typically developing children below the age of 4 years, the vHIT and cVEMP were the most feasible vestibular tests. Moreover, the age-dependent normative vestibular data could specify age trends in this group of young children. Finally, based on the current results and clinical experience of more than ten years at the Ghent University Hospital (Belgium), a clinical framework to diagnose early vestibular deficits in young patients is proposed.

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. Therefore, this study aimed to determine the most appropriate tool to screen for vestibular deficits, to assess the necessity of vestibular follow-up, and to set clinical guidelines for vestibular screening in children with hearing loss. DESIGN: In total, 71 children with congenital or early-onset sensorineural hearing loss were enrolled (mean age at first appointment = 6.7 months). Follow-up was provided at 6 months, 1, 2, and 3 years of age. Below three years of age, the video Head Impulse Test (vHIT) of the horizontal semicircular canals (SCC), the cVEMP, and the rotatory test at 0.16, 0.04, and 0.01 Hz were applied. At 3 years of age, the vHIT of the vertical SCC and ocular vestibular evoked myogenic potentials (oVEMP) were added. To evaluate early motor development, the Alberta Infant Motor Scale (AIMS) results at 6 months and 1-year old were included. RESULTS: At 6 months of age, the highest success rate was obtained with the cVEMP (90.0%) compared to the vHIT (70.0%) and the rotatory test (34.3-72.9%). Overall, vestibular deficits were found in 20.0% of the children, consisting of 13.9% with both SCC and otolith deficits (bilateral: 9.3%, unilateral: 4.6%), and 6.1% with unilateral isolated SCC (4.6%) or otolith (1.5%) deficits. Thus, vestibular deficits would not have been detected in 4.6% of the children by only using the cVEMP, whereas 1.5% would have been missed when only using the vHIT. Although vestibular deficits were more frequently found in severe to profound hearing loss (28.6%), characteristics of vestibular function were highly dependent on the underlying etiology. The AIMS results showed significantly weaker early motor development in children with bilateral vestibular deficits ( p = 0.001), but could not differentiate children with bilateral normal vestibular function from those with unilateral vestibular deficits ( p > 0.05). Progressive or delayed-onset vestibular dysfunction was only found in a few cases (age range: 12-36 months), in which the hearing loss was mainly caused by congenital cytomegalovirus (cCMV). CONCLUSIONS: The cVEMP is the most feasible screening tool to assess vestibular function in 6-months-old children with hearing loss. Although the majority of children with vestibular deficits are detected with the cVEMP, the vHIT seems even more sensitive as isolated SCC deficits are associated with specific etiologies of hearing loss. As a result, the cVEMP is an appropriate vestibular screening tool, which is advised at least in severe to profound hearing loss, but certain etiologies require the addition of the vHIT (i.e., cCMV, meningitis, cochleovestibular anomalies with or without syndromic hearing loss).

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. RESULTS: Out of the 51 patients, 61% found riding a bicycle difficult, 29% were not able to cycle anymore, and 10% reported not having any problems riding a bicycle. Their mean cycling time decreased from 3.15 hrs weekly before to 1.27 hrs weekly after disease onset ( p = 0.002). The latter also differed significantly from the mean cycling time of 3.51 hrs weekly in control subjects ( p < 0.001). The main complaints while cycling were not being able to look behind (88%), having difficulties with visually stabilizing the environment (65%), and not being able to ride in a straight line (61%). The fall concern was significantly higher in the patient group, with cycling in the dark and at dusk, and turning the head as their main concerns ( p < 0.001). Falls and near-falls were respectively reported in 50% and 65% of the patient group. Only the latter differed significantly from the control group. However, the number of reported falls differed significantly between both groups. CONCLUSIONS: This study demonstrates cycling difficulties in persons with bilateral vestibulopathy, related to problems with postural control and gaze stabilization. Knowledge about specific complaints and triggers is important to prevent future falls. A combination of coping strategies and aids, with vestibular rehabilitation focused on gaze stabilization might be considered, as well as avoidance of cycling in specific cases.

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. Therefore, this study aims to propose an evidence-based vestibular follow-up program with proper balance between its feasibility and sensitivity. DESIGN: In this longitudinal cohort study, 185 cCMV-patients (mean age 3.2 years, SD 1.6 years, range 0.5-6.7 years) were included. Vestibular follow-up data were obtained through lateral video head impulse test (vHIT) and cervical vestibular evoked myogenic potential (cVEMP) evaluations around the ages of 6 months, 1 year, and 2 years. Around 3 and 4.5 years of age, data from vertical vHIT and ocular vestibular evoked myogenic potentials (oVEMP) were also collected. RESULTS: At birth, 55.1% (102/185) of patients were asymptomatic and 44.9% (83/185) were symptomatic. The mean duration of follow-up for all patients was 20.8 (SD 16.3) months (mean number of follow-up assessments: 3.2, SD 1.5). Vestibular loss occurred at some point during follow-up in 16.8% (31/185) of all patients. Six percent (10/164) of patients with normal vestibular function at first assessment developed delayed-onset vestibular loss; 80.0% (8/10) of these within the first 2 years of life. Vestibular deterioration was reported both in patients who had been treated with postnatal antiviral therapy and untreated patients. At final evaluation, both the semicircular and the otolith system were impaired in the majority of vestibular-impaired ears (29/36, 80.6%). Dysfunctions limited to the semicircular system or the otolith system were reported in 4 (4/36, 11.1%) and 3 (3/36, 8.3%) ears, respectively. The occurrence of vestibular loss was highest in patients with first trimester seroconversion (16/59, 27.1%) or with an unknown timing of seroconversion (13/71, 18.3%), patients with sensorineural hearing loss (16/31, 51.6%), and patients with periventricular cysts on magnetic resonance imaging (MRI) (7/11, 63.6%). CONCLUSIONS: Longitudinal vestibular follow-up, most intensively during the first 2 years of life, is recommended in cCMV-patients with vestibular risk factors (first trimester or unknown timing of seroconversion; sensorineural hearing loss; periventricular cysts on MRI). If those risk factors can be ruled out, a single evaluation early in life (around 6 months of age) might be sufficient. Both semicircular and otolith system evaluation should be part of the follow-up program, as partial losses were reported.

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. METHODS: Pooled prevalence rates with 95% confidence intervals (CIs) were computed using random-effects models. Heterogeneity among studies was assessed using the I2 statistic. RESULTS: A total of 54 studies (7546 participants) were included, and random-effects meta-analysis yielded a pooled proportional fall rate of 0.48 (95% CI [0.43-0.52], I2 = 93%, 46 studies, 6874 participants) for classification 1 (≥1 fall) and a pooled proportional fall rate of 0.32 (95% CI [0.27-0.37], I2 = 78%, 31 studies, 5672 participants) for classification 2 (≥2 falls). Subgroup analysis on the classification of falls, and length and method of monitoring falls did not reveal significant differences and did not reduce between-study variability. CONCLUSION: Pooled estimates suggest that one in two persons with PD fall at least once, and one in three fall at least twice within the registered time period. Substantial variability remains after pooling fall prevalence rates according to the length and method of monitoring. Therefore, no recommendations can be made concerning these methodological aspects. Future research on falls in PD is encouraged to implement best practice recommendations to monitor and report fall data.

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). RESULTS: For the cVEMP, the highest response rate was found for NB chirp (100%), followed by TB (91%), BB chirp (87%) and finally click (85%). A similar order was seen for oVEMP with percentages of 100%; 57%, 57%, and 43%. The 500 Hz NB CE-Chirp elicited significantly shorter cVEMP P1 and N1 latencies and significantly larger c&oVEMP amplitudes compared to all other stimuli. BB CE-Chirp elicited significantly shorter c&oVEMP P1 and N1 latencies with smaller amplitudes compared to TB. Asymmetry ratios were not statistically significant for all comparisons. CONCLUSION: The 500 Hz NB CE-chirp provides the highest response rates, shorter latencies and larger amplitudes, and therefore seem a promising stimulus for reliably measuring c&oVEMPs in clinical practice.

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. 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.

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. DESIGN: Fifty patients (32 to 68 years) with a primary unilateral/bilateral MoM hip implant were matched for age, gender, and noise exposure to 50 nonimplanted control subjects. Both groups underwent the same protocol, consisting of an objective auditory [i.e., conventional and high-frequency audiometry, transient-evoked and distortion (TEOAEs and DPOAEs), auditory brainstem responses] and vestibular (i.e., cervical and ocular vestibular evoked myogenic potentials, horizontal and vertical video head impulse tests) test battery, a questionnaire inquiring auditory, balance, and general neurological symptoms, and a blood sample collection to determine the plasma Co concentration. RESULTS: The auditory test battery presented consistently higher audiometric thresholds in the MoM patient group, with group differences ranging from 2.1 to 5.7 dB in the lower frequencies (0.25 to 6.0 kHz) and from 4.6 to 9.3 dB in the high frequencies (8.0 to 14.0 kHz). Group differences at high frequencies were statistically significant ( p ≤ 0.001). Additionally, significantly lower TEOAE ( p = 0.009) and DPOAE ( p < 0.001) amplitudes were observed in the MoM patients when the better ear was included in the analysis, and more absent TEOAE and DPOAE responses were found between 1.0 and 4.0 kHz (0.008 ≤ p ≤ 0.039). Within the vestibular test battery, the MoM patients showed longer N1 and P1 ocular vestibular evoked myogenic potentials latencies for the left ear, and lower video head impulse test gains for the left anterior and right posterior semicircular canals (0.005 ≤ p ≤0.035). The patient-reported (questionnaire) outcome delivered no significant group differences ( p > 0.01). Six patients had elevated Co levels according to our local institutional threshold (>4 or 5 µg/l for unilateral or bilateral MoM hip implants, resp.), but their audiovestibular outcome measures did not differ significantly from those of the other patients. CONCLUSIONS: Corresponding to our preliminary investigations, the results indicate possibly Co-induced (predominantly high-frequency) auditory impairment, probably triggered by toxic damage to the cochlear structures. However, the low mean difference values, the lack of group differences for the patient-reported outcome measures, and the lack of any relationship with the blood Co levels strongly reduce the clinical relevance of these findings. Therefore, the risk of Co-induced ototoxic impairment is considered to be clinically negligible for the majority of MoM hip implant patients, and the use of auditory tests in the risk estimation of systemic Co toxicity should be decided on a case-by-case basis.

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. RESULTS: Of 15,363 screened athletes with intellectual disability, more than half (58.7%) needed referral for ear and/or hearing problems, ranging from 51.7% (<20 years old) to 81.4% (≥60 years old). The most commonly detected conditions were excessive/impacted earwax (40.3%), middle ear problems (30.1%) and hearing loss (27.0%), with significant differences between age groups and countries. DISCUSSION: This study demonstrates an increased risk of ear and hearing problems in people with intellectual disability as compared to the general population. Considering the unawareness and impact of these problems, it is highly recommended to organise systematic screening. Follow-up for diagnostic elaboration, therapeutic management and long-term guidance should be provided.

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. 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.

Vestibular Function in Children With a Congenital Cytomegalovirus Infection: 3 Years of Follow-Up.

OBJECTIVES: Congenital cytomegalovirus (cCMV) infection is the most common nongenetic cause of sensorineural hearing loss in children. Due to the close anatomical relationship between the auditory and the vestibular sensory organs, cCMV can also be an important cause of vestibular loss. However, the prevalence and nature of cCMV-induced vestibular impairment is still underexplored. The aim of this study was to investigate the occurrence and characteristics of vestibular loss in a large group of cCMV-infected children, representative of the overall cCMV-population. DESIGN: Ninety-three children (41 boys, 52 girls) with a confirmed diagnosis of cCMV were enrolled in this prospective longitudinal study. They were born at the Ghent University Hospital or referred from another hospital for multidisciplinary follow-up in the context of cCMV. The test protocol consisted of regular vestibular follow-up around the ages of 6 months, 1 year, 2 years, and 3 years with the video Head Impulse Test, the rotatory test, and the cervical Vestibular Evoked Myogenic Potential test. RESULTS: On average, the 93 patients (52 asymptomatic, 41 symptomatic) were followed for 10.2 months (SD: 10.1 mo) and had 2.2 examinations (SD: 1.1). Seventeen (18%) patients had sensorineural hearing loss (7 unilateral, 10 bilateral). Vestibular loss was detected in 13 (14%) patients (7 unilateral, 6 bilateral). There was a significant association between the occurrence of hearing loss and the presence of vestibular loss (p < 0.001), with 59% (10/17) vestibular losses in the group of hearing-impaired children compared to 4% (3/76) in the group of normal-hearing subjects. In the majority of the cases with a vestibular dysfunction (85%, 11/13), both the semicircular canal system and the otolith system were affected. The remaining subjects (15%, 2/13) had an isolated semicircular canal dysfunction. Sixty-one patients already had at least one follow-up examination. Deterioration of the vestibular function was detected in 6 of them (10%, 6/61). CONCLUSIONS: cCMV can impair not only the auditory but also the vestibular function. Similar to the hearing loss, vestibular loss in cCMV can be highly variable. It can be unilateral or bilateral, limited or extensive, stable or progressive, and early or delayed in onset. As the vestibular function can deteriorate over time and even normal-hearing subjects can be affected, vestibular evaluation should be part of the standard otolaryngology follow-up in all children with cCMV.

The ototoxic potential of cobalt from metal-on-metal hip implants: a pilot study on the patient-reported auditory, vestibular, and general neurological outcome.

OBJECTIVE: This study aimed to systematically investigate the ototoxic potential of cobalt in patients with a metal-on-metal (MoM) hip implant, using objective auditory and vestibular assessments and a questionnaire. The results of the objective evaluation were published previously, whereas the current study focused on the questionnaire outcome and its relationship to the blood cobalt level. Design and study sample: Twenty patients (33-65 years) with a primary MoM hip implant and 20 non-implanted control subjects, matched for age, gender, and noise exposure, received a questionnaire to evaluate the presence of several hearing and balance symptoms (part 1) and general neurological issues (part 2). RESULTS: Concerning part 1, the proportion of auditory-related symptoms in general (p = 0.022) and tinnitus (p = 0.047) was significantly higher in the MoM patient group, whereas no group difference was found for hyperacusis, increased listening effort, and decreased speech understanding. Concerning part 2, no significant group differences were detected. Within the MoM patient group, the questionnaire outcome was not significantly different between the low-exposure and high-exposure subgroups according to the blood Co level. CONCLUSIONS: In line with our previous study, these results potentially imply Co-induced impairment to the auditory system, despite the lack of a clear dose-response relationship.

Vestibular function in adults with intellectual disabilities: feasibility and outcome of a vestibular screening protocol in Special Olympics athletes.

OBJECTIVE: This study aimed to evaluate the feasibility of a well-adapted vestibular screening battery to objectively examine the (peripheral) vestibular function, and to explore the characteristics of potential vestibular deficits in the adult ID population. DESIGN: Cross-sectional study design. STUDY SAMPLE: Compared to an age- and gender-weighted control group, a heterogeneous group of forty-five adults with ID participated in the vestibular screening at the National Games of Special Olympics Belgium (2019), which consisted of a bone conduction cervical Vestibular Evoked Myogenic Potential (cVEMP) measurement and video Head Impulse Test (vHIT). RESULTS: The screening battery appeared to be feasible in the majority of the participants (cVEMP: 92%; vHIT: 72%). Overall, the occurrence of abnormal cVEMP and vHIT responses was significantly higher in the ID group, with significantly lower corrected peak-to-peak cVEMP amplitudes (p < 0.001), lower vHIT gains (p < 0.001), and higher cVEMP and vHIT asymmetry ratios in the ID group (p = 0.008 and p < 0.001 resp.). CONCLUSIONS: Vestibular assessment using the cVEMP and vHIT technique shows a promising feasibility in adults with ID. In addition, this study suggests that people with ID exhibit an increased prevalence of (peripheral) vestibular deficits relative to the general population.

Psychometric Properties of Cognitive-Motor Dual-Task Studies With the Aim of Developing a Test Protocol for Persons With Vestibular Disorders: A Systematic Review.

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.

The Ototoxic Potential of Cobalt From Metal-on-Metal Hip Implants: Objective Auditory and Vestibular Outcome.

OBJECTIVES: During the past decade, the initial popularity of metal-on-metal (MoM) hip implants has shown a progressive decline due to increasingly reported implant failure and revision surgeries. Local as well as systemic toxic side effects have been associated with excessive metal ion release from implants, in which cobalt (Co) plays an important role. The rare condition of systemic cobaltism seems to manifest as a clinical syndrome with cardiac, endocrine, and neurological symptoms, including hearing loss, tinnitus, and imbalance. In most cases described in the literature, revision surgery and the subsequent drop in blood Co level led to (partial) alleviation of the symptoms, suggesting a causal relationship with Co exposure. Moreover, the ototoxic potential of Co has recently been demonstrated in animal experiments. Since its ototoxic potential in humans is merely based on anecdotal case reports, the current study aimed to prospectively and objectively examine the auditory and vestibular function in patients implanted with a MoM hip prosthesis. DESIGN: Twenty patients (15 males and 5 females, aged between 33 and 65 years) implanted with a primary MoM hip prosthesis were matched for age, gender, and noise exposure to 20 non-implanted control subjects. Each participant was subjected to an extensive auditory (conventional and high-frequency pure tone audiometry, transient evoked and distortion product otoacoustic emissions [TEOAEs and DPOAEs], auditory brainstem responses [ABR]) and vestibular test battery (cervical and ocular vestibular evoked myogenic potentials [cVEMPs and oVEMPs], rotatory test, caloric test, video head impulse test [vHIT]), supplemented with a blood sample collection to determine the plasma Co concentration. RESULTS: The median [interquartile range] plasma Co concentration was 1.40 [0.70, 6.30] µg/L in the MoM patient group and 0.19 [0.09, 0.34] µg/L in the control group. Within the auditory test battery, a clear trend was observed toward higher audiometric thresholds (11.2 to 16 kHz), lower DPOAE (between 4 and 8 kHz), and total TEOAE (1 to 4 kHz) amplitudes, and a higher interaural latency difference for wave V of the ABR in the patient versus control group (0.01 ≤ p < 0.05). Within the vestibular test battery, considerably longer cVEMP P1 latencies, higher oVEMP amplitudes (0.01 ≤ p < 0.05), and lower asymmetry ratio of the vHIT gain (p < 0.01) were found in the MoM patients. In the patient group, no suggestive association was observed between the plasma Co level and the auditory or vestibular outcome parameters. CONCLUSIONS: The auditory results seem to reflect signs of Co-induced damage to the hearing function in the high frequencies. This corresponds to previous findings on drug-induced ototoxicity and the recent animal experiments with Co, which identified the basal cochlear outer hair cells as primary targets and indicated that the cellular mechanisms underlying the toxicity might be similar. The vestibular outcomes of the current study are inconclusive and require further elaboration, especially with respect to animal studies. The lack of a clear dose-response relationship may question the clinical relevance of our results, but recent findings in MoM hip implant patients have confirmed that this relationship can be complicated by many patient-specific factors.

The test-retest reliability of questionnaires regarding attitudes and beliefs toward noise, hearing loss, and hearing protector devices in young adults.

CONTEXT: Young people expose themselves to high noise levels during leisure activities, and might thus be at risk of acquiring hearing-related problems. Therefore, information regarding risk-taking behavior is necessary to prevent future hearing problems and to optimize future preventive campaigns. AIM: This study evaluated the test-retest reliability of the Youth Attitude to Noise Scale (YANS) and beliefs about hearing protection and hearing loss (BAHPHL) instrument. SETTINGS AND DESIGN: Forty-three young adults between 18 and 29 years filled in a questionnaire at two test moments. MATERIALS AND METHODS: The YANS and BAHPHL instrument were used to evaluate the attitudes toward noise, hearing loss, and hearing protection. Each participant completed the retest within 21-55 days after the first administration of the questionnaire. RESULTS: Paired Student's t-tests showed no significant differences in mean scores between test and retest for both the entire YANS and BAHPHL instrument as well as their factors. Furthermore, a good agreement between test and retest scores was seen by Bland-Altman analyses. Intraclass correlation coefficients were above 0.70 for the entire YANS and the factor related to youth culture as well as for the entire BAHPHL and all the factors of the BAHPHL instrument, except for the factor related to the severity of the consequences of hearing loss. CONCLUSION: Reliable test-retest measurements of the YANS and BAHPHL instrument can be performed. Hence, these questionnaires can be used in longitudinal studies to explore young adults' changes in attitudes toward noise, hearing loss, and hearing protection, with or without an educational intervention.

Comparison of Different Electrode Configurations for the oVEMP With Bone-Conducted Vibration.

OBJECTIVES: This study was performed to compare three electrode configurations for the ocular vestibular evoked myogenic potentials (oVEMPs)-"standard," "sternum," and "nose"-by making use of bone-conducted stimuli (at the level of Fz with a minishaker). In the second part, we compared the test-retest reliability of the standard and nose electrode configuration on the oVEMP parameters. DESIGN: This study had a prospective design. Fourteen healthy subjects participated in the first part (4 males, 10 females; average age = 23.4 (SD = 2.6) years; age range 19.9 to 28.3 years) and second part (3 males, 11 females; average age = 22.7 (SD = 2.4) years; age range 20.0 to 28.0 years) of the study. OVEMPs were recorded making use of a hand-held bone conduction vibrator (minishaker). Tone bursts of 500 Hz (rise/fall time = 2 msec; plateau time = 2 msec; repetition rate = 5.1 Hz) were applied at a constant stimulus intensity level of 140 dB FL. RESULTS: PART 1: The n10-p15 amplitude obtained with the standard electrode configuration (mean = 15.8 µV; SD = 6.3 µV) was significantly smaller than the amplitude measured with the nose (Z = -3.3; p = 0.001; mean = 35.0 µV; SD = 19.1 µV) and sternum (Z = -3.3; p = 0.001; mean = 27.1 µV; SD = 12.2 µV) electrode configuration. The p15 latency obtained with the nose electrode configuration (mean = 14.2 msec; SD = 0.54 msec) was significantly shorter than the p15 latency measured with the standard (Z = -3.08; p = 0.002) (mean = 14.9 msec; SD = 0.75 msec) and sternum (Z = -2.98; p = 0.003; mean = 15.4 msec; SD = 1.07 msec) electrode configuration. There were no differences between the n10 latencies of the three electrode configurations. The 95% prediction intervals (given by the mean ± 1.96 * SD) for the different interocular ratio values were [-41.2; 41.2], [-37.2; 37.2], and [-25.9; 25.9] for standard, sternum, and nose electrode configurations, respectively. PART 2: Intraclass correlation (ICC) values calculated for the oVEMP parameters obtained with the standard electrode configuration showed fair to good reliability for the parameters n10-p15 amplitude (ICC = 0.51), n10 (ICC = 0.52), and p15 (ICC = 0.60) latencies. The ICC values obtained for the parameters acquired with the nose electrode configuration demonstrated a poor reliability for the n10 latency (ICC = 0.37), a fair to good reliability for the p15 latency (ICC = 0.47) and an excellent reliability for the n10-p15 amplitude (ICC = 0.85). CONCLUSIONS: This study showed the possible benefits from alternative electrode configurations for measuring bone-conducted-evoked oVEMPs in comparison with the standard electrode configuration. The nose configuration seems promising, but further research is required to justify clinical use of this placement.