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.

Auditory, Visual, and Cognitive Abilities in Normal-Hearing Adults, Hearing Aid Users, and Cochlear Implant Users.

OBJECTIVES: Speech understanding is considered a bimodal and bidirectional process, whereby visual information (i.e., speechreading) and also cognitive functions (i.e., top-down processes) are involved. Therefore, the purpose of the present study is twofold: (1) to investigate the auditory (A), visual (V), and cognitive (C) abilities in normal-hearing individuals, hearing aid (HA) users, and cochlear implant (CI) users, and (2) to determine an auditory, visual, cognitive (AVC)-profile providing a comprehensive overview of a person's speech processing abilities, containing a broader variety of factors involved in speech understanding. DESIGN: Three matched groups of subjects participated in this study: (1) 31 normal-hearing adults (mean age = 58.76), (2) 31 adults with moderate to severe hearing loss using HAs (mean age = 59.31), (3) 31 adults with a severe to profound hearing loss using a CI (mean age = 58.86). The audiological assessments consisted of pure-tone audiometry, speech audiometry in quiet and in noise. For evaluation of the (audio-) visual speech processing abilities, the Test for (Audio) Visual Speech perception was used. The cognitive test battery consisted of the letter-number sequencing task, the letter detection test, and an auditory Stroop test, measuring working memory and processing speed, selective attention, and cognitive flexibility and inhibition, respectively. Differences between the three groups were examined using a one-way analysis of variance or Kruskal-Wallis test, depending on the normality of the variables. Furthermore, a principal component analysis was conducted to determine the AVC-profile. RESULTS: Normal-hearing individuals scored better for both auditory, and cognitive abilities compared to HA users and CI users, listening in a best aided condition. No significant differences were found for speech understanding in a visual condition, despite a larger audiovisual gain for the HA users and CI users. Furthermore, an AVC-profile was composed based on the different auditory, visual, and cognitive assessments. On the basis of that profile, it is possible to determine one comprehensive score for auditory, visual, and cognitive functioning. In the future, these scores could be used in auditory rehabilitation to determine specific strengths and weaknesses per individual patient for the different abilities related to the process of speech understanding in daily life. CONCLUSIONS: It is suggested to evaluate individuals with hearing loss from a broader perspective, considering more than only the typical auditory abilities. Also, cognitive and visual abilities are important to take into account to have a more complete overview of the speech understanding abilities in daily life.

Apparent diffusion coefficient values of the white matter in magnetic resonance imaging of the neonatal brain may help predict outcome in congenital cytomegalovirus infection.

BACKGROUND: White matter change is a well-known abnormality in congenital cytomegalovirus (cCMV) infection, but grading remains challenging and clinical relevance unclear. OBJECTIVE: To investigate if quantitative measurement of white matter apparent diffusion coefficient (ADC) values in magnetic resonance imaging (MRI) of the neonatal brain can predict outcome in cCMV. MATERIALS AND METHODS: A retrospective, single-center observational study, including patients with cCMV who had a neonatal brain MRI with diffusion-weighted imaging, was performed between 2007 and 2020. Regions of interest were systematically placed in the white matter on the ADC maps. Two pediatric radiologists independently scored additional brain abnormalities. Outcome measures were neonatal hearing and cognitive and motor development. Statistical analysis included simple and penalized elastic net regression. RESULTS: Neonatal brain MRI was evaluated in 255 patients (median age 21 days, 25-75 percentiles: 14-28 days, 121 male). Gyral abnormalities were noted in nine patients (3.5%), ventriculomegaly in 24 (9.4%), and subependymal cysts in 58 (22.7%). General white matter ADC was significantly higher in patients with neonatal hearing loss and cognitive and motor impairment (P< 0.05). For neonatal hearing loss, simple logistic regression using only general white matter was the best prediction model, with a receiver operating characteristic area under the curve (AUC)=0.76. For cognitive impairment, interacting elastic net regression, including other brain abnormalities and frontoparietal white matter ADC, performed best, with AUC=0.89. For motor impairment, interacting elastic net regression, including other brain abnormalities and deep anterior frontal white matter performed best, with AUC=0.73. CONCLUSION: Neonatal white matter ADC was significantly higher in patients with clinical impairments. Quantitative ADC measurement may be a useful tool for predicting clinical outcome in cCMV.

Risk Factors for Natural Hearing Evolution in Newborns With Congenital Cytomegalovirus Infection.

Importance: Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking. Objective: To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss. Design, Setting, and Participants: This multicenter cohort study included patients from 6 secondary and tertiary hospitals enrolled in the Flemish CMV registry (Belgium). Newborns with untreated cCMV infection with at least 4-year audiological follow-up were included. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Data were collected for 15 years (January 1, 2007, to February 7, 2022) and analyzed from September 26, 2022, to January 16, 2023. Main Outcomes and Measures: Primary outcome was hearing evolution (per-ear analysis; described as stable hearing, improvement, or deterioration). The association of gestational characteristics, clinical findings, timing of seroconversion, viral load, and hearing status at birth with hearing evolution was investigated using effect sizes (Cramer V, odds ratio [OR], or Hedges g). Results: Of the 387 children, 205 of 385 with nonmissing data were male (53.2%), 113 (29.2%) had a symptomatic infection, and 274 (70.8%) had an asymptomatic infection. Every child was 4 years or older at final hearing evaluation. A total of 701 of 774 ears (90%) showed stable hearing (normal hearing or stable hearing loss since birth) over time. Late-onset hearing loss (normal hearing at birth followed by hearing loss) was present in 43 of 683 ears (6.3%). Among children with hearing loss present at birth, 24 of 34 ears (70.6%) had hearing deterioration, and 6 of 91 ears (6.6%) had hearing improvement. Prematurity was associated with a higher chance of hearing improvement (OR, 12.80; 95% CI, 2.03-80.68). Late-onset hearing loss was more prevalent in a first trimester infection (OR, 10.10; 95% CI, 2.90-34.48). None of the 104 ears of children with a third trimester seroconversion developed late-onset hearing loss. Conclusions and Relevance: Findings of this cohort study support that ongoing audiological follow-up for untreated children with congenital hearing loss is important, as the majority of patients had hearing deterioration. The timing of seroconversion was associated with the risk of developing late-onset hearing loss. These insights can aid in parental counseling, patient stratification, and follow-up. Future research should focus on the effect of treatment, the influence of determined risk factors, and the study of eventual new risk factors in patients at high risk to develop hearing loss.

Evaluation of Lifetime Noise Exposure History Reporting.

PURPOSE: The purpose of this study is to critically evaluate lifetime noise exposure history (LNEH) reporting. First, two different approaches to evaluate the cumulative LNEH were compared. Second, individual LNEH was associated with the subjects' hearing status. Third, loudness estimates of exposure activities, by means of Jokitulppo- and Ferguson-based exposure levels, were compared with dosimeter sound-level measurements. METHOD: One hundred one young adults completed the questionnaires, and a subgroup of 30 subjects underwent audiological assessment. Pure-tone audiometry, speech-in-noise intelligibility, distortion product otoacoustic emissions, auditory brainstem responses, and envelope following responses were included. Fifteen out of the 30 subjects took part in a noisy activity while wearing a dosimeter. RESULTS: First, results demonstrate that the structured questionnaire yielded a greater amount of information pertaining to the diverse activities, surpassing the insights obtained from an open-ended questionnaire. Second, no significant correlations between audiological assessment and LNEH were found. Lastly, the results indicate that Ferguson-based exposure levels offer a more precise estimation of the actual exposure levels, in contrast to Jokitulppo-based estimates. CONCLUSIONS: We propose several recommendations for determining the LNEH. First, it is vital to define accurate loudness categories and corresponding allocated levels, with a preference for the loudness levels proposed by Ferguson et al. (2019), as identified in this study. Second, a structured questionnaire regarding LNEH is recommended, discouraging open-ended questioning. Third, it is essential to include a separate category exclusively addressing work-related activities, encompassing various activities for more accurate surveying.

Changes in Tinnitus Characteristics and Residual Inhibition following Cochlear Implantation: A Prospective Analysis.

This study aims to explore the effect of cochlear implantation on tinnitus perception. A prospective study was conducted on 72 adult hearing-impaired patients to evaluate tinnitus perception before and after cochlear implantation, using standardized tinnitus questionnaires (the tinnitus sample case-history questionnaire, tinnitus functional index (TFI), and tinnitus handicap inventory (THI)). A large variety of demographic and hearing- and implant-related data was collected from patient hospital records to explore possible associations with the implantation effect. The prevalence of tinnitus complaints before implantation was 58.3%. The temporary induction or aggravation of tinnitus immediately after surgery was noted in 20% and 46.7% of patients, respectively. When evaluated 3 months after implantation, 60% of tinnitus patients experienced a clinically significant reduction in their complaints; most of the improvements were experienced immediately after activation of the implant. Only the scores for TFI and THI at baseline were found to be significantly correlated with a reduction in TFI scores after implantation. In 80% of tinnitus patients, the tinnitus remained suppressed for some time after taking off the device. The large subset of patients with residual inhibition supports the involvement of central pathophysiological processes in implantation effects on tinnitus, which are explored in this paper.

Implications of isolated white matter abnormalities on neonatal MRI in congenital CMV infection: a prospective single-centre study.

OBJECTIVE: Investigating the clinical implications of isolated white matter abnormalities on neonatal brain MRI in congenital cytomegalovirus (CMV). DESIGN: Prospective, observational. PATIENTS/INTERVENTIONS: Two paediatric radiologists, blinded to clinical data, independently scored the white matter in 286 newborns with congenital CMV. After assessing interobserver variability, mean score was used to categorise white matter (normal, doubtful or abnormal). Patients with other brain abnormalities were excluded. MAIN OUTCOME MEASURES: Hearing and neuromotor evaluation. RESULTS: Cohen's weighted kappa was 0.79 (95% CI 0.73 to 0.84). White matter was normal in 121 patients, doubtful in 62, abnormal in 28. Median clinical follow-up was 12.0 months (IQR 12.0-27.7 months). Neonatal hearing loss occurred in 4/27 patients (14.8%) with abnormal, 1/118 patients (0.8%) with normal and 1/62 patients (1.6%) with doubtful white matter (p<0.01). Impaired cognitive development was seen in 3/27 patients (11.1%) with abnormal, 3/114 patients (2.6%) with normal and 1/59 patients (1.7%) with doubtful white matter (p=0.104). Alberta Infant Motor Scale (AIMS) was below P75 in 21/26 patients (80.8%) with abnormal, 73/114 patients (64.0%) with normal and 36/57 patients (63.2%) with doubtful white matter (p=0.231). In a subgroup of patients with minimal clinical follow-up of 18 months, AIMS score was below P75 in 10/13 patients (76.9%) with abnormal, 13/34 patients (38.2%) with normal and 7/20 patients (35.0%) with doubtful white matter (p<0.05). CONCLUSIONS: Abnormal white matter was associated with neonatal hearing loss and mild, lower motor scores. A tendency towards impaired cognitive development was seen. Patients with doubtful white matter did not show worse clinical outcome.

Early cochlear implantation supports narrative skills of children with prelingual single-sided deafness.

Prelingual single-sided deafness (SSD) not only affects children's hearing skills, but can also lead to speech-language delays and academic underachievement. Early cochlear implantation leads to improved spatial hearing, but the impact on language development is less studied. In our longitudinal study, we assessed the language skills of young children with SSD and a cochlear implant (CI). In particular, we investigated their narrative skills in comparison to two control groups: children with SSD without a CI, and children with bilateral normal hearing. We found that children with SSD and a CI performed in line with their normal-hearing peers with regard to narrative and verbal short-term memory skills. Children with SSD without a CI had worse narrative (group difference = - 0.67, p = 0.02) and verbal short-term memory (group difference = - 0.68, p = 0.03) scores than the implanted group. Verbal short-term memory scores and grammar scores each correlated positively with narrative scores across all groups. Early grammar scores (at 2-3 years of age) could partially predict later narrative scores (at 4-6 years of age). These results show that young children with prelingual SSD can benefit from early cochlear implantation to achieve age-appropriate language skills. They support the provision of a CI to children with prelingual SSD.

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. Cognitive and motor performance, as well as cognitive-motor interference were measured in persons with BV and normal hearing using the 2BALANCE dual-task protocol. The experimental group was matched to a healthy control group based on age, sex, and educational level. The 2BALANCE protocol comprises cognitive tests assessing visuospatial memory, mental rotation, visual and auditory response inhibition, visual and auditory working memory, and processing speed. The cognitive tests were performed in single-task condition (while seated), and in dual-task condition (during a static and a dynamic motor task). The static motor task consisted of balancing on a force platform with foam pad. The dynamic motor task consisted of walking at a self-selected speed. These motor tasks were also performed in single-task condition. A generalized estimating equations model was used to investigate group differences for all cognitive and motor outcome measures. The estimated marginal means, as well as the odds ratios (OR), and their 95% confidence intervals (CI) were calculated. For the backward digit recall test, a baseline measurement was performed and analyzed using a student-t test. A total of 22 patients with BV and normal hearing and 22 healthy control subjects were assessed [mean age (SD), BV = 53.66 (13.35) and HC = 53.21 (13.35), 68% male]. The BV group had poorer mental rotation skills in single-task condition, compared to the control group [odds ratio (OR) = 2.30, confidence interval (CI) = 1.12-4.73, P = 0.024]. Similarly, auditory and visual working memory were also poorer in the BV group in single-task condition (P = 0.028 and P = 0.003, respectively). The BV group also performed poorer on the mental rotation task and the visual response inhibition task in dual-task condition (OR = 2.96, CI = 1.57-5.59, P < 0.001 and OR = 1.08, CI = 1.01-1.16, P = 0.032, respectively). Additionally, an interaction effect, indicating increased cognitive-motor interference in the BV group, was observed for mental rotation, response inhibition, and auditory working memory (P = 0.003 to 0.028). All static motor outcome parameters indicated more postural sway in the BV group compared to the control group for all test conditions (P < 0.001 to 0.026). No group differences were noted for the dynamic motor task. These findings suggest a link between vestibular function and cognitive performance, as well as a greater interference between cognitive and motor performance in BV, compared to healthy controls.

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. Objective: To uncover the association of BV with and without hearing loss with cognitive and motor performance and cognitive-motor interference. Design, Setting, and Participants: This prospective case-control study assessed persons with an isolated BV and persons with BV and a concomitant hearing loss compared with a healthy control group. Data were analyzed in December 2022. The study was conducted at Ghent University (Ghent, Belgium). Data collection took place between March 26, 2021, and November 29, 2022. Main Outcomes and Measures: All participants completed the 2BALANCE dual-task protocol, comprising a static and a dynamic motor task that was combined with 5 visual cognitive tasks. These cognitive tasks assessed mental rotation, visuospatial memory, working memory, response inhibition (executive function), and processing speed. All cognitive tasks were performed in a single-task condition (while seated) and in a dual-task condition (combined with a static and a dynamic motor task). The static task comprised balancing on a force platform with foam pad, and the dynamic task comprised walking at a self-selected speed on the GAITRite Walkway. Both motor tasks were performed in the single-task and dual-task condition. Results: Nineteen persons with BV and hearing loss (mean [SD] age, 56.70 [10.12] years; 10 women [52.6%]), 22 persons with an isolated BV (mean [SD] age, 53.66 [13.35] years; 7 women [31.8%]), and 28 healthy control participants were included (mean [SD] age, 53.73 [12.77] years; 12 women [42.9%]). Both patient groups had mental rotation and working memory impairment in a single-task condition and slower processing speed when walking (ie, during the dynamic dual-task condition). Additionally, the patient group with hearing loss had impaired visuospatial memory and executive function deficits in single-task and dual-task conditions, while this could only be elicited when performing a motor task in persons with isolated BV (ie, when dual-tasking). Conclusion and Relevance: The findings of this case-control study suggest an association between vestibular function and cognitive and motor performance, even greater in persons with a concomitant hearing loss than in persons with an isolated BV.

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.

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. The protocol comprises cognitive tests assessing response inhibition, emotion recognition, visuospatial memory, selective and sustained attention, visual memory and visual-motor integration.Based on the results, the VI group had significantly reduced scores on 'social cognition' (p = 0.018), 'executive functions' (p < 0.01), and 'perceptual-motor functioning' (p = 0.020) compared to their TD and HI peers. For the categories 'complex attention' and 'learning and memory' no differences could be observed. Analogous to the findings of previous literature, the symptoms of a VI are often not limited to the primary functions of the system, but also comprise an impact on emotional and cognitive performance. Therefore, more holistic rehabilitation approaches should be encouraged, with a screening and attention for cognitive, emotional and behavioral dysfunctions in the vestibular population. Since this is one of the first studies to investigate the involvement of a VI in a child's cognitive development, these findings support the need for studies further characterizing the impact of a VI, the underlying pathophysiology and the effect of different rehabilitation procedures.

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.

Comparing tinnitus, pain, psychosocial and cognitive factors between patients with tinnitus and pain: A systematic review.

OBJECTIVE: Tinnitus is a multifactorial symptom, which shows similarities with the involved mechanisms in chronic pain. The aim of this systematic review is to provide an overview of studies comparing patients with only tinnitus to patients with pain (headache, temporomandibular joint (TMJ) pain or neck pain) with or without tinnitus, regarding tinnitus-related, pain-related, psychosocial and cognitive factors. METHODS: This systematic review was written following the PRISMA guidelines. To identify relevant articles, PubMed, Web of Science and Embase databases were searched. The risk of bias was rated using the Newcastle Ottawa scale for case-control studies. RESULTS: Ten articles were included in the qualitative analysis. The risk of bias ranged from low to moderate. Low to moderate evidence shows that patients with tinnitus experience higher mean symptom intensity, but lower psychosocial and cognitive distress, compared to patients with pain. Inconsistent results were found for tinnitus-related factors. Low to moderate evidence points to a higher severity of hyperacusis and psychosocial distress in patients with both pain and tinnitus, compared to patients with tinnitus only, as well as for positive associations between tinnitus-related factors and the presence or intensity of pain. CONCLUSION: This systematic review shows that psychosocial dysfunctions are more clearly present in patients with pain only, compared to patients with tinnitus only and the co-occurrence of tinnitus and pain increases psychosocial distress as well as hyperacusis severity. Some positive associations were identified between tinnitus-related and pain-related factors.

Envelope following responses for hearing diagnosis: Robustness and methodological considerations.

Recent studies have found that envelope following responses (EFRs) are a marker of age-related and noise- or ototoxic-induced cochlear synaptopathy (CS) in research animals. Whereas the cochlear injury can be well controlled in animal research studies, humans may have an unknown mixture of sensorineural hearing loss [SNHL; e.g., inner- or outer-hair-cell (OHC) damage or CS] that cannot be teased apart in a standard hearing evaluation. Hence, a direct translation of EFR markers of CS to a differential CS diagnosis in humans might be compromised by the influence of SNHL subtypes and differences in recording modalities between research animals and humans. To quantify the robustness of EFR markers for use in human studies, this study investigates the impact of methodological considerations related to electrode montage, stimulus characteristics, and presentation, as well as analysis method on human-recorded EFR markers. The main focus is on rectangularly modulated pure-tone stimuli to evoke the EFR based on a recent auditory modelling study that showed that the EFR was least affected by OHC damage and most sensitive to CS in this stimulus configuration. The outcomes of this study can help guide future clinical implementations of electroencephalography-based SNHL diagnostic tests.

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 8.9, standard deviation 3.27 months). Motor results using the Alberta Infant Motor Scale were collected in 152 of these patients. Logistic and linear regression models were applied to identify risk factors for the vestibular and motor outcomes, respectively. RESULTS: The odds of developing early vestibular dysfunction were 6 times higher in patients presenting with hearing loss at birth compared to those born with normal hearing (p = .002). Within the latter group, significant predictors for vestibular dysfunction were (delayed-onset) hearing impairment at the time of vestibular testing (p = .003) and the presence of periventricular cysts on magnetic resonance imaging (p = .005). Remarkably, none of the patients infected during the third trimester of pregnancy (n = 14) developed early vestibular dysfunction. On average, vestibular-impaired patients had a z-score on the Alberta Infant Motor Scale that was 1.17 points lower than patients without vestibular deficit (p < .001). CONCLUSION: Early vestibular loss can have a significant adverse effect on motor development. Hearing and cranial imaging findings could facilitate the widespread implementation of a (targeted) vestibular assessment approach in the cCMV-population. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:1757-1765, 2023.

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).

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.

Risk Factors for Hearing Loss at Birth in Newborns With Congenital Cytomegalovirus Infection.

Importance: With a prevalence between 0.2% and 6.1% of all live births, congenital cytomegalovirus (cCMV) infection is a major cause of congenital nonhereditary sensorineural hearing loss. Despite the large amount of research on cCMV-related hearing loss, it is still unclear which newborns are at risk of hearing loss. Objective: To identify independent risk factors for cCMV-related congenital hearing loss and predictors of hearing loss severity at birth. Design, Setting, and Participants: This cross-sectional study of newborns with cCMV infection used data included in the Flemish CMV registry that was collected from 6 secondary and tertiary hospitals in Flanders, Belgium, over 15 years (January 1, 2007, to February 7, 2022). Data were analyzed March 3 to October 19, 2022. Patients were included in the study after confirmed diagnosis of cCMV infection and known hearing status at birth. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Main Outcomes and Measures: Primary outcome was hearing status at birth. Clinical, neurological, and laboratory findings along with the timing of seroconversion and blood viral load were separately considered as risk factors. Binary logistic regression was performed to identify independent risk factors for congenital hearing loss in newborns with cCMV. Effect sizes were measured using Hedges g, odds ratio, or Cramer V. Results: Of the 1033 newborns included in the study (553 of 1024 [54.0%] boys), 416 (40.3%) were diagnosed with symptomatic cCMV infection and 617 (59.7%) with asymptomatic cCMV infection. A total of 15.4% of the patients (n = 159) presented with congenital hearing loss; half of them (n = 80 [50.3%]) had isolated hearing loss. The regression model revealed 3 independent risk factors for congenital hearing loss: petechiae at birth (adjusted odds ratio [aOR], 6.7; 95% CI, 1.9-23.9), periventricular cysts on magnetic resonance imaging (MRI; aOR, 4.6; 95% CI, 1.5-14.1), and seroconversion in the first trimester (aOR, 3.1; 95% CI, 1.1-9.3). Lower viral loads were seen in patients with normal hearing compared with those with congenital hearing loss (median [IQR] viral load, 447.0 [39.3-2345.8] copies per milliliter of sample [copies/mL] vs 1349.5 [234.3-14 393.0] copies/mL; median difference, -397.0 [95% CI, -5058.0 to 174.0] copies/mL). Conclusions and Relevance: Findings of this cross-sectional study suggest that newborns with cCMV infection and petechiae at birth, periventricular cysts on MRI, or a seroconversion in the first trimester had a higher risk of congenital hearing loss. Clinicians may use these risk factors to counsel parents in the prenatal and postnatal periods about the risk of congenital hearing loss. Moreover, linking clinical features to hearing loss may provide new insights into the pathogenesis of cCMV-related hearing loss. The importance of viral load as a risk factor for congenital hearing loss remains unclear.

Development of the hearing-related quality of life questionnaire for auditory-visual, cognitive and psychosocial functioning (hAVICOP).

INTRODUCTION: There is a need for a validated and standardized self-assessment instrument to assess the subjective effect of hearing aid (HA) use and/or cochlear implantation (CI) on different aspects of functioning in daily life. The aim of this study was to develop a new holistic Patient Reported Outcome Measure (PROM) to assess hearing-related quality of life. The new PROM is titled the hearing-related quality of life questionnaire for Auditory-VIsual, COgnitive and Psychosocial functioning (hAVICOP). METHODS: A conceptual framework was set up and test items were prepared per domain. Preliminary testing involved a semi-structured interview-based assessment in normal-hearing and hearing-impaired adults and an expert panel. For the further psychometric evaluation, a new sample of 15 adult HA users, 20 adult CI users and 20 normal-hearing adults filled in the refined version of the hAVICOP, the Speech, Spatial and Qualities of Hearing Scale, the Nijmegen Cochlear Implant Questionnaire and the TNO-AZL Questionnaire for Adult's Health-Related Quality of Life. Based on these results, a factor analysis was conducted and internal consistency, discriminant validity and concurrent construct validity were determined. RESULTS: The final version of the hAVICOP consists of three domains for hearing-related quality of life: (1) auditory-visual functioning, (2) cognitive functioning, and (3) psychosocial functioning. A sufficient internal consistency was found, and discriminant validity and concurrent construct validity were good. CONCLUSIONS: A new PROM to assess hearing-related quality of life was developed, named the hAVICOP. In the future the validity and reliability should be examined further.