Variants in Genes Associated with Hearing Loss in Children: Prevalence in a Large Canadian Cohort.

OBJECTIVE: The objective of this study was to assess the prevalence of genetic variants associated with hearing loss in a large cohort of children in Canada using high throughput next generation sequencing (NGS). METHODS: A total of 485 children with hearing loss underwent NGS testing with an 80 gene panel of syndromic and non-syndromic variants known to be associated with hearing loss. Genetic variants were classified as pathogenic, likely pathogenic, likely benign, benign, or variants of uncertain significance (VUS), according to the American College of Medical Genetics and Genomics guidelines. RESULTS: Across the 80 genes tested, 923 variants, predominantly in 28 genes, were identified in 324 children. Pathogenic variants occurred in 19/80 (23.8%) of the hearing loss related genes tested and confirmed the etiology of hearing loss in 73/485 (15.1%) of children. GJB2 was the most prevalent gene, affecting 28/73 (38.4%) children with confirmed genetic hearing loss in our cohort. Most identified variants (748/923, 81.0%, in 76/80 genes) were of uncertain significance. CONCLUSION: Genetic testing using NGS identified the etiology in approximately 15% of childhood hearing loss in a Canadian cohort which is lower than what is typically reported. GJB2 was the most common genetic cause of hearing loss. VUS are commonly identified, presenting clinical challenges for counseling. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 2024.

Head and Eye Movements Reveal Compensatory Strategies for Acute Binaural Deficits During Sound Localization.

The present study aimed to define use of head and eye movements during sound localization in children and adults to: (1) assess effects of stationary versus moving sound and (2) define effects of binaural cues degraded through acute monaural ear plugging. Thirty-three youth (MAge = 12.9 years) and seventeen adults (MAge = 24.6 years) with typical hearing were recruited and asked to localize white noise anywhere within a horizontal arc from -60° (left) to +60° (right) azimuth in two conditions (typical binaural and right ear plugged). In each trial, sound was presented at an initial stationary position (L1) and then while moving at ∼4°/s until reaching a second position (L2). Sound moved in five conditions (±40°, ±20°, or 0°). Participants adjusted a laser pointer to indicate L1 and L2 positions. Unrestricted head and eye movements were collected with gyroscopic sensors on the head and eye-tracking glasses, respectively. Results confirmed that accurate sound localization of both stationary and moving sound is disrupted by acute monaural ear plugging. Eye movements preceded head movements for sound localization in normal binaural listening and head movements were larger than eye movements during monaural plugging. Head movements favored the unplugged left ear when stationary sounds were presented in the right hemifield and during sound motion in both hemifields regardless of the movement direction. Disrupted binaural cues have greater effects on localization of moving than stationary sound. Head movements reveal preferential use of the better-hearing ear and relatively stable eye positions likely reflect normal vestibular-ocular reflexes.

Effects of the BalanCI on Working Memory and Balance in Children and Young Adults With Cochleovestibular Dysfunction.

OBJECTIVES: This study aimed to: (1) determine the interaction between cognitive load and balance in children and young adults with bilateral cochleovestibular dysfunction who use bilateral cochlear implants (CIs) and (2) determine the effect of an auditory balance prosthesis (the BalanCI) on this interaction. Many (20 to 70%) children with sensorineural hearing loss experience some degree of vestibular loss, leading to poorer balance. Poor balance could have effects on cognitive resource allocation which might be alleviated by the BalanCI as it translates head-referenced cues into electrical pulses delivered through the CI. It is hypothesized that children and young adults with cochleovestibular dysfunction will demonstrate greater dual-task costs than typically-developing children during dual balance-cognition tasks, and that BalanCI use will improve performance on these tasks. DESIGN: Study participants were 15 typically-developing children (control group: mean age ± SD = 13.6 ± 2.75 years, 6 females) and 10 children and young adults who use bilateral CIs and have vestibular dysfunction (CI-V group: mean age ± SD=20.6 ± 5.36 years, 7 females). Participants completed two working memory tasks (backward auditory verbal digit span task and backward visuospatial dot matrix task) during three balance conditions: seated, standing in tandem stance with the BalanCI off, and standing in tandem stance with the BalanCI on. Working memory performance was quantified as total number of correct trials achieved. Postural stability was quantified as translational and rotational path length of motion capture markers worn on the head, upper body, pelvis, and feet, normalized by trial time. RESULTS: Relative to the control group, children and young adults in the CI-V group exhibited poorer overall working memory across all balance conditions ( p = 0.03), poorer translational postural stability (larger translational path length) during both verbal and visuospatial working memory tasks ( p < 0.001), and poorer rotational stability (larger rotational path length) during the verbal working memory task ( p = 0.026). The CI-V group also exhibited poorer translational ( p = 0.004) and rotational ( p < 0.001) postural stability during the backward verbal digit span task than backward visuospatial dot matrix task; BalanCI use reduced this stability difference between verbal and visuospatial working memory tasks for translational stability overall ( p > 0.9), as well as for rotational stability during the maximum working memory span (highest load) participants achieved in each task ( p = 0.91). CONCLUSIONS: Balance and working memory were impaired in the CI-V group compared with the control group. The BalanCI offered subtle improvements in stability in the CI-V group during a backward verbal working memory task, without producing a negative effect on working memory outcomes. This study supports the feasibility of the BalanCI as a balance prosthesis for individuals with cochleovestibular impairments.

Importance of early objective auditory testing in the presentation of sudden sensorineural hearing loss in children.

BACKGROUND: Functional hearing loss can be due to an auditory manifestation of functional neurological disorder, previously known as conversion disorder. METHODS: This is a case series of 3 pediatric patients with a diagnosis of idiopathic SSNHL who ultimately were found to have functional neurological disorder. RESULTS: Average age was 12.7 years at presentation (range 10-14 years). All three patients underwent invasive interventions prior to their initial clinic visit. All patients demonstrated profound SNHL on behavioural audiogram, but normal otoacoustic emissions (OAE) and auditory brainstem response testing. With counselling, both patients demonstrated significant hearing improvement. CONCLUSIONS: Early use of OAE's in the workup of SSNHL can avoid unnecessary and potentially harmful therapies and expedite access to counselling services which may help lead to symptom resolution.

Exposure to Spoken Communication During the COVID-19 Pandemic Among Children With Cochlear Implants.

Importance: School closures and other COVID-19-related restrictions could decrease children's exposure to speech during important stages of development. Objective: To assess whether significant decreases in exposure to spoken communication found during the initial phase of the COVID-19 pandemic among children using cochlear implants are confirmed for a larger cohort of children and were sustained over the first years of the COVID-19 pandemic. Design, Setting, and Participants: This cohort study used datalogs collected from children with cochlear implants during clinical visits to a tertiary pediatric hospital in Toronto, Ontario, Canada, from January 1, 2018, to November 11, 2021. Children with severe to profound hearing loss using cochlear implants were studied because their devices monitored and cataloged levels and types of sounds during hourly use per day (datalogs) and because their hearing and spoken language development was particularly vulnerable to reduced sound exposure. Statistical analyses were conducted between January 2022 and August 2023. Main Outcomes and Measures: Daily hours of sound were captured by the cochlear implant datalogging system and categorized into 6 auditory scene categories, including speech and speech-in-noise. Time exposed to speech was calculated as the sum of daily hours in speech and daily hours in speech-in-noise. Residual hearing in the ear without an implant of children with unilateral cochlear implants was measured by pure tone audiometry. Mixed-model regression analyses revealed main effects with post hoc adjustment of 95% CIs using the Satterthwaite method. Results: Datalogs (n = 2746) from 262 children (137 with simultaneous bilateral cochlear implants [74 boys (54.0%); mean (SD) age, 5.8 (3.5 years)], 38 with sequential bilateral cochlear implants [24 boys (63.2%); mean (SD) age, 9.1 (4.2) years], and 87 with unilateral cochlear implants [40 boys (46.0%); mean (SD) age, 7.9 (4.6) years]) who were preschool aged (n = 103) and school aged (n = 159) before the COVID-19 pandemic were included in analyses. There was a slight increase in use among preschool-aged bilateral cochlear implant users through the pandemic (early pandemic, 1.4 h/d [95% CI, 0.3-2.5 h/d]; late pandemic, 2.3 h/d [95% CI, 0.6-4.0 h/d]) and little change in use among school-aged bilateral cochlear implant users (early pandemic, -0.6 h/d [95% CI, -1.1 to -0.05 h/d]; late pandemic, -0.3 h/d [95% CI, -0.9 to 0.4 h/d]). However, use decreased during the late pandemic period among school-aged children with unilateral cochlear implants (-1.8 h/d [95% CI,-3.0 to -0.6 h/d]), particularly among children with good residual hearing in the ear without an implant. Prior to the pandemic, children were exposed to speech for approximately 50% of the time they used their cochlear implants (preschool-aged children: bilateral cochlear implants, 46.6% [95% CI, 46.5%-47.2%] and unilateral cochlear implants, 52.1% [95% CI, 50.7%-53.5%]; school-aged children: bilateral cochlear implants, 47.6% [95% CI, 46.8%-48.4%] and unilateral cochlear implants, 51.0% [95% CI, 49.4%-52.6%]). School-aged children in both groups experienced significantly decreased speech exposure in the early pandemic period (bilateral cochlear implants, -12.1% [-14.6% to -9.4%]; unilateral cochlear implants, -15.5% [-20.4% to -10.7%]) and late pandemic periods (bilateral cochlear implants, -5.3% [-8.0% to -2.6%]; unilateral cochlear implants, -11.2% [-15.3% to -7.1%]) compared with the prepandemic baseline. Conclusions and Relevance: This cohort study using datalogs from children using cochlear implants suggests that a sustained reduction in children's access to spoken communication was found during more than 2 years of COVID-19 pandemic-related lockdowns and school closures.

Age-related increases in right hemisphere support for prosodic processing in children.

Language comprehension is a complex process involving an extensive brain network. Brain regions responsible for prosodic processing have been studied in adults; however, much less is known about the neural bases of prosodic processing in children. Using magnetoencephalography (MEG), we mapped regions supporting speech envelope tracking (a marker of prosodic processing) in 80 typically developing children, ages 4-18 years, completing a stories listening paradigm. Neuromagnetic signals coherent with the speech envelope were localized using dynamic imaging of coherent sources (DICS). Across the group, we observed coherence in bilateral perisylvian cortex. We observed age-related increases in coherence to the speech envelope in the right superior temporal gyrus (r = 0.31, df = 78, p = 0.0047) and primary auditory cortex (r = 0.27, df = 78, p = 0.016); age-related decreases in coherence to the speech envelope were observed in the left superior temporal gyrus (r = - 0.25, df = 78, p = 0.026). This pattern may indicate a refinement of the networks responsible for prosodic processing during development, where language areas in the right hemisphere become increasingly specialized for prosodic processing. Altogether, these results reveal a distinct neurodevelopmental trajectory for the processing of prosodic cues, highlighting the presence of supportive language functions in the right hemisphere. Findings from this dataset of typically developing children may serve as a potential reference timeline for assessing children with neurodevelopmental hearing and speech disorders.

Children with autism spectrum disorder who demonstrate normal language scores use a bottom-up semantic processing strategy: Evidence from N400 recordings.

INTRODUCTION: The N400 is an electrophysiological component that reflects lexical access and integration of words with mental representations. METHODS: Thirty-five young children with a range of language capabilities (n = 21 neurotypical controls, 10 males, mean age = 6.3 ± 0.9 years; n = 14 children with autism, 12 males, mean age = 6.4 ± 1.1 years) completed an auditory semantic categorization paradigm to evoke the N400. Electroencephalograph (EEG) data were acquired with a 64-channel electrode cap as children listened via ear inserts to binaurally presented single syllable words and decided whether the words were congruent (in) or incongruent (out) with a pre-specified category. EEG data were filtered, epoched, and averaged referenced, and global field power (GFP) was computed. The amplitude of the N400 peak in the GFP was submitted to a multiple linear regression analysis. RESULTS: N400 amplitude was found to predict language scores only for the children with ASD who have language scores in the normal range (r2  = 0.72). CONCLUSIONS: This finding that N400 amplitude only predicted language scores in children with ASD and normal language scores suggests that these children may rely more on basic semantic processing (as reflected by the N400) and less on anticipating and predicting upcoming words. This suggests preferential utilization of a bottom-up strategy to access higher order language.

Evaluating the use of a balance prosthesis during balance perturbations in children and young adults with cochleovestibular dysfunction.

Study objectives were to: (1) quantify stability in children and young adults using cochlear implants with concurrent cochleovestibular dysfunction (CI-V) during balance perturbations and (2) to assess effects of an auditory head-referencing device (BalanCI) on their stability. The BalanCI provides auditory feedback via cochlear implants to cue posture and potentially avoid falling in children with CI-V. It was hypothesized that children and young adults with CI-V respond with larger movements to floor perturbations than typically-developing peers (controls) and that BalanCI use decreases these movements. Motion in response to treadmill perturbations was captured by markers on the head, torso, and feet in eight CI-V and 15 control participants. Stability (area under the curve of motion displacement) and peak displacement latencies were measured. The CI-V group demonstrated less stability and slower responses than the control group during medium and large backwards perturbations (p's < 0.01). In the CI-V group, BalanCI use improved stability during large backwards perturbations (p < 0.001), but worsened stability during large sideways perturbations (p's < 0.001). Children and young adults with CI-V move more to remain upright during perturbations than typically-developing peers. The BalanCI has potential to aid physical/vestibular therapy in children with CIs who have poor balance.

Quantification of carpal tunnel morphology using centroid-to-boundary distance shape signatures.

Morphology analysis is valuable to understanding risk factors and the etiology of carpal tunnel (CT) syndrome. The objective of this study was to investigate morphology changes along the length of the CT using shape signatures (SS). Analysis was performed on ten cadaveric specimens in neutral wrist posture. Centroid-to-boundary distance SS were generated for proximal, middle, and distal CT cross-sections. Phase shift and Euclidean distance were quantified relative to a template SS for each specimen. Medial, lateral, palmar, and dorsal peaks were identified on each SS to generate metrics of tunnel width, tunnel depth, peak amplitude, peak angle. Width and depth measures were also performed using previously reported methods to serve as a basis of comparison. The phase shift revealed twisting of 21° between the ends of the tunnel. Distance from the template and width varied significantly over the length of the tunnel, while depth did not. Measures of width and depth using the SS method were consistent with previously reported methods. The SS method afforded the advantage of peak analysis with overall trends of peak amplitude indicating flattening of the tunnel at the proximal and distal ends relative to a rounder shape in the middle.

Effects of Age at Implantation on Outcomes of Cochlear Implantation in Children with Short Durations of Single-Sided Deafness.

OBJECTIVE: Children with single-sided deafness (SSD) show reduced language and academic development and report hearing challenges. We aim to improve outcomes in children with SSD by providing bilateral hearing through cochlear implantation of the deaf ear with minimal delay. STUDY DESIGN: Prospective cohort study of 57 children with SSD provided with cochlear implant (CI) between May 13, 2013, and June 25, 2021. SETTING: Tertiary children's hospital. PARTICIPANTS: Children with early onset (n = 40) or later onset of SSD (n = 17) received CIs at ages 2.47 ± 1.58 years (early onset group) and 11.67 ± 3.91 years (late onset group) (mean ± SD). Duration of unilateral deafness was limited (mean ± SD = 1.93 ± 1.56 yr). INTERVENTION: Cochlear implantation of the deaf ear. MAIN OUTCOMES/MEASURES: Evaluations of device use (data logging) and hearing (speech perception, effects of spatial release from masking on speech detection, localization of stationary and moving sound, self-reported hearing questionnaires). RESULTS: Results indicated that daily device use is variable (mean ± SD = 5.60 ± 2.97, range = 0.0-14.7 h/d) with particular challenges during extended COVID-19 lockdowns, including school closures (daily use reduced by mean 1.73 h). Speech perception with the CI alone improved (mean ± SD = 65.7 ± 26.4 RAU) but, in the late onset group, remained poorer than in the normal hearing ear. Measures of spatial release from masking also showed asymmetric hearing in the late onset group ( t13 = 5.14, p = 0.001). Localization of both stationary and moving sound was poor (mean ± SD error = 34.6° ± 16.7°) but slightly improved on the deaf side with CI use ( F1,36 = 3.95, p = 0.05). Decreased sound localization significantly correlated with poorer self-reported hearing. CONCLUSIONS AND RELEVANCE: Benefits of CI in children with limited durations of SSD may be more restricted for older children/adolescents. Spatial hearing challenges remain. Efforts to increase CI acceptance and consistent use are needed.

Predicting Wrist Posture during Occupational Tasks Using Inertial Sensors and Convolutional Neural Networks.

Current methods for ergonomic assessment often use video-analysis to estimate wrist postures during occupational tasks. Wearable sensing and machine learning have the potential to automate this tedious task, and in doing so greatly extend the amount of data available to clinicians and researchers. A method of predicting wrist posture from inertial measurement units placed on the wrist and hand via a deep convolutional neural network has been developed. This study has quantified the accuracy and reliability of the postures predicted by this system relative to the gold standard of optoelectronic motion capture. Ten participants performed 3 different simulated occupational tasks on 2 occasions while wearing inertial measurement units on the hand and wrist. Data from the occupational task recordings were used to train a convolutional neural network classifier to estimate wrist posture in flexion/extension, and radial/ulnar deviation. The model was trained and tested in a leave-one-out cross validation format. Agreement between the proposed system and optoelectronic motion capture was 65% with κ = 0.41 in flexion/extension and 60% with κ = 0.48 in radial/ulnar deviation. The proposed system can predict wrist posture in flexion/extension and radial/ulnar deviation with accuracy and reliability congruent with published values for human estimators. This system can estimate wrist posture during occupational tasks in a small fraction of the time it takes a human to perform the same task. This offers opportunity to expand the capabilities of practitioners by eliminating the tedium of manual postural assessment.

Longitudinal Effects of Simultaneous and Sequential Bilateral Cochlear Implantation on Cortical Auditory-Evoked Potentials Recorded at Cz in a Large Cohort of Children.

OBJECTIVES: Auditory development after bilateral cochlear implantation in children has been measured using source localization of multi-channel late latency responses. It is not clear, however, whether this development can be tracked using a more clinically feasible method of recording from one active recording electrode placed at mid-line center of the head (Cz). DESIGN: In this prospective cohort study, cortical auditory-evoked potential responses (CAEPs) were recorded from Cz referenced to each earlobe (Cz-CAEP) from 222 children with bilateral cochlear implant (CI); 128 (mean ± SD age: 2.78 ± 3.30 years) received both CIs in the same surgery (simultaneous group) and 94 (aged 7.72 ± 4.45 years) received a second CI after 4.21 ± 2.98 years of unilateral CI use. We sought to (1) identify cortical development over the first couple of years of bilateral CI use; (2) measure known asymmetries in auditory development between the CIs; and (3) detect the effects of bilateral rather than unilateral CI use. 4556 Cz-CAEPs were recorded across the cohort over 33.50 ± 7.67 months duration of bilateral CI use. Given concerns related to peak picking, amplitude areas were measured across two response time windows (50 to 199 ms and 200 to 400 ms). RESULTS: Results indicated that small response amplitudes occur at initial CI use and amplitudes increase in the negative or positive direction rapidly over the first months of CI use in both time windows. Asymmetries between Cz-CAEPs evoked by each CI were found in the sequential group and reduced with bilateral CI use, particularly in the first time window; these differences increased with longer inter-implant delay. Bilaterally evoked Cz-CAEPs were larger in amplitude than unilateral responses from either CI in the simultaneous group. In the sequential group, bilateral responses were similar to responses from the first implanted side but increased in relative amplitude with bilateral CI use. The Cz-CAEP measures were not able to predict asymmetries or bilateral benefits in speech perception measures. CONCLUSIONS: The Cz-CAEP was able to indicate cortical detection of CI input and showed gross morphological changes with bilateral CI use. Findings indicate Cz-CAEPs can be used to identify gross changes in auditory development in children with bilateral CIs, but they are less sensitive to tracking the remaining abnormalities that are measured by multi-channel CAEPs and speech perception testing.

Carpal tunnel volume distribution and morphology changes with flexion-extension and radial-ulnar deviation wrist postures.

Non-neutral wrist postures have been reported to cause decreased carpal tunnel volume (CTV) contributing to impingement of the median nerve and development of carpal tunnel syndrome. Recent analysis found CTV did not change with ±20° flexion-extension (FE), however, CTV decreased with ulnar deviation over the range of -5° to 15° radial-ulnar deviation (RUD). These findings suggest CTV may be too coarse of a measure to reflect the effects of slight non-neutral postures, or that volume is conserved and redistributed due to changes in tunnel morphology with posture. The objective of this study was to assess volume distribution along the length of the carpal tunnel and to quantify regional morphology changes with deviated wrist postures in both FE and RUD. Analysis was performed on a dataset of computed tomography scans collected on ten cadaveric specimens (5 male, 5 female, mean age = 80.7 ± 10.9 years) over a range of FE and RUD postures. The carpal tunnel of each scan was divided into four quartiles of equal length along the tunnel to quantify volume distribution. Volume within the carpal tunnel was seen to redistribute with both FE and RUD. Decreased volume in the distal aspect of the tunnel with flexion and proximal aspect of the tunnel with ulnar deviation may contribute to localized compression of the medial nerve. Measures of mean cross-sectional area, width and depth by quartile provided an indication of the morphology changes associated volume redistribution. Morphology analysis also revealed twisting between the proximal and distal aspects of the tunnel which increased with flexion and ulnar deviation and may further contribute to strain on the median nerve.

Volumetric Analysis of Hearing-Related Structures of Brain in Children with GJB2-Related Congenital Deafness.

Background: Children with non-syndromic hereditary sensorineural hearing loss (SNHL) provide an opportunity to explore the impact of hearing on brain development. Objective: This study investigates volumetric differences of key hearing-related structures in children with gap junction protein beta 2 GJB2-related SNHL compared to controls. Materials and methods: Ninety-four children with SNHL (n = 15) or normal hearing (n = 79) were studied using automated volumetric segmentation. Heschl's gyrus (HG), anterior HG (aHG), planum temporale (PT), medial geniculate nucleus (MGN), and nucleus accumbens (NA) were analyzed relative to total brain volume (TBV) at two different age groups: (1) 7−12 months and (2) 13 months−18 years. Two-sided t-tests were used to evaluate differences between groups. Differences were considered significant if p < 0.007. Results: Significantly smaller aHG-to-TBV ratios were found in 13-month-to-18-year-old patients (p < 0.0055). HG-, PT-, MGN-, and NA-to-TBV ratios were smaller in the same age group, without reaching a significant level. Conversely, HG- and NA-to-TBV were larger in the younger age group. No significant differences were found between the groups for age and TBV. Conclusions: In this exploratory volumetric analysis of key hearing-related structures, we observed age-related changes in volume in children with GJB2-related SNHL.

Effects of Sequential Bilateral Cochlear Implantation in Children: Evidence from Speech-Evoked Cortical Potentials and Tests of Speech Perception.

INTRODUCTION: Benefits of bilateral cochlear implants (CI) may be compromised by delays to implantation of either ear. This study aimed to evaluate the effects of sequential bilateral CI use in children who received their first CI at young ages, using a clinical set-up. METHODS: One-channel cortical auditory evoked potentials and speech perception in quiet and noise were evoked at repeated times (0, 3, 6, 12 months of bilateral CI use) by unilateral and bilateral stimulation in 28 children with early-onset deafness. These children were unilaterally implanted before 3.69 years of age (mean ± SD of 1.98 ± 0.73 years) and received a second CI after 5.13 ± 2.37 years of unilateral CI use. Comparisons between unilaterally evoked responses were used to measure asymmetric function between the ears and comparisons between bilateral responses and each unilateral response were used to measure the bilateral benefit. RESULTS: Chronic bilateral CI promoted changes in cortical auditory responses and speech perception performance; however, large asymmetries were present between the two unilateral responses despite ongoing bilateral CI use. Persistent cortical differences between the two sides at 1 year of bilateral stimulation were predicted by increasing age at the first surgery and inter-implant delay. Larger asymmetries in speech perception occurred with longer inter-implant delays. Bilateral responses were more similar to the unilateral responses from the first rather than the second CI. CONCLUSION: These findings are consistent with the development of the aural preference syndrome and reinforce the importance of providing bilateral CIs simultaneously or sequentially with very short delays.

Response characteristics of vestibular evoked myogenic potentials recorded over splenius capitis in young adults and adolescents.

BACKGROUND AND OBJECTIVES: Examine vestibular evoked myogenic potential (VEMP) responses recorded from surface electrodes over Splenius Capitis (SPC) in a seated position. SPECIFIC AIMS: (1) validate response characteristics of VEMP recordings from surface electrodes over Sternocleidomastoid (SCM) and over SCP and (2) assess age effects on responses in adolescents and young adults. MATERIALS AND METHODS: Simultaneous surface VEMP was recorded bilaterally from electrodes placed over the dorsal neck musculature at a location known from previous work to record from SPC in 15 healthy participants during trials with head rotation toward and away from the stimulated ear. VEMP was also recorded from electrodes over SCM, ipsilateral to the stimulus ear, in the same participants in a supine, head lift/turn position. RESULTS: Response amplitudes significantly increased with contraction strength and decreased with age. Participants were able to maintain sufficient contraction strength (amplitude) with head rotation to reliably measure over SPC. Normalized response amplitudes measured from electrodes over contralateral SPC were largest with head rotation contralateral to the stimulus ear. Normalized amplitudes and peak latencies were comparable to the same measures from SCM obtained in supine, head lift/turn position. CONCLUSIONS: Otolith generated myogenic responses can be recorded seated from electrodes over the dorsal neck with head rotation contralateral to the stimulus ear. In this position, contralateral recordings are consistent with responses known from previous work to arise from SPC; ipsilateral recordings may include crosstalk from activated muscles nearby, including ipsilateral SCM. Overall, techniques targeting contralateral SPC during contralateral head turn may provide additional methods of recording VEMPs.

Response characteristics of vestibular evoked myogenic potentials recorded over splenius capitis in young adults and adolescents

Background and objectives: Examine vestibular evoked myogenic potential (VEMP) responses recorded from surface electrodes over Splenius Capitis (SPC) in a seated position. Specific aims: (1) validate response characteristics of VEMP recordings from surface electrodes over Sternocleidomastoid (SCM) and over SCP and (2) assess age effects on responses in adolescents and young adults. Materials and methods: Simultaneous surface VEMP was recorded bilaterally from electrodes placed over the dorsal neck musculature at a location known from previous work to record from SPC in 15 healthy participants during trials with head rotation toward and away from the stimulated ear. VEMP was also recorded from electrodes over SCM, ipsilateral to the stimulus ear, in the same participants in a supine, head lift/turn position. Results: Response amplitudes significantly increased with contraction strength and decreased with age. Participants were able to maintain sufficient contraction strength (amplitude) with head rotation to reliably measure over SPC. Normalized response amplitudes measured from electrodes over contralateral SPC were largest with head rotation contralateral to the stimulus ear. Normalized amplitudes and peak latencies were comparable to the same measures from SCM obtained in supine, head lift/turn position. Conclusions: Otolith generated myogenic responses can be recorded seated from electrodes over the dorsal neck with head rotation contralateral to the stimulus ear. In this position, contralateral recordings are consistent with responses known from previous work to arise from SPC; ipsilateral recordings may include crosstalk from activated muscles nearby, including ipsilateral SCM. Overall, techniques targeting contralateral SPC during contralateral head turn may provide additional methods of recording VEMPs.(AU)

Antecedentes y objetivos: Examinamos las respuestas de los potenciales evocados miogénicos vestibulares (PEMV) recogidas de los electrodos de superficie sobre el músculo esplenio (ME) en posición sentada. Objetivos específicos: 1) validar las características de los registros de la respuesta de los PEMV recogidos de los electrodos de superficie sobre el músculo esternocleidomastoideo (SCM) y el ME, y 2) evaluar los efectos de la edad en adolescentes y adultos jóvenes. Materiales y métodos: Se registraron simultáneamente los PEMV bilaterales de los electrodos situados en la musculatura dorsal del cuello, en un sitio conocido de un estudio anterior para obtener registros del ME en 15 participantes sanos durante los ensayos, con rotación de cabeza hacia y fuera del oído estimulado. También se registraron los PEMV de los electrodos situados sobre el SCM, en posición ipsilateral al oído estimulado, en los mismos participantes, en posición supina y con elevación/giro de cabeza. Resultados: Las amplitudes de la respuesta se incrementaron significativamente con la fuerza de la contracción y disminuyeron con la edad. Los participantes fueron capaces de mantener suficiente fuerza de contracción (amplitud) con la rotación de cabeza, para realizar mediciones fiables sobre el ME. Las amplitudes de la respuesta normalizada medidas en los electrodos sobre el ME contralateral fueron mayores con la rotación de cabeza contralateral al oído estimulado. Las amplitudes normalizadas y las latencias máximas fueron comparables a las mismas medidas del SCM obtenidas en posición supina, y elevación/giro de cabeza. Conclusiones: Las respuestas miogénicas generadas por otolitos pueden registrarse en posición sentada a partir de los electrodos situados en la parte dorsal del cuello, contralateral al oído estimulado. En esta posición, los registros contralaterales son coherentes con las respuestas conocidas de un estudio previo, derivadas del ME; los registros ipsilaterales pueden incluir interferencias de los músculos activados cercanos, incluyendo el SCM ipsilateral. En general, las técnicas centradas en el ME contralateral durante el giro de cabeza contralateral pueden aportar métodos adicionales de registro de los PEMV.(AU)

Age-related changes to vestibular heave and pitch perception and associations with postural control.

Falls are a common cause of injury in older adults (OAs), and age-related declines across the sensory systems are associated with increased falls risk. The vestibular system is particularly important for maintaining balance and supporting safe mobility, and aging has been associated with declines in vestibular end-organ functioning. However, few studies have examined potential age-related differences in vestibular perceptual sensitivities or their association with postural stability. Here we used an adaptive-staircase procedure to measure detection and discrimination thresholds in 19 healthy OAs and 18 healthy younger adults (YAs), by presenting participants with passive heave (linear up-and-down translations) and pitch (forward-backward tilt rotations) movements on a motion-platform in the dark. We also examined participants' postural stability under various standing-balance conditions. Associations among these postural measures and vestibular perceptual thresholds were further examined. Ultimately, OAs showed larger heave and pitch detection thresholds compared to YAs, and larger perceptual thresholds were associated with greater postural sway, but only in OAs. Overall, these results suggest that vestibular perceptual sensitivity declines with older age and that such declines are associated with poorer postural stability. Future studies could consider the potential applicability of these results in the development of screening tools for falls prevention in OAs.

Cortical imbalance following delayed restoration of bilateral hearing in deaf adolescents.

Unilateral auditory deprivation in early childhood can lead to cortical strengthening of inputs from the stimulated side, yet the impact of this on bilateral processing when inputs are later restored beyond an early sensitive period is unknown. To address this, we conducted a longitudinal study with 13 bilaterally profoundly deaf adolescents who received unilateral access to sound via a cochlear implant (CI) in their right ear in early childhood before receiving bilateral access to sound a decade later via a second CI in their left ear. Auditory-evoked cortical responses to unilateral and bilateral stimulation were measured repeatedly using electroencephalogram from 1 week to 14 months after activation of their second CI. Early cortical responses from the newly implanted ear and bilateral stimulation were atypically lateralized to the left ipsilateral auditory cortex. Duration of unilateral deafness predicted an unexpectedly stronger representation of inputs from the newly implanted, compared to the first implanted ear, in left auditory cortex. Significant initial reductions in responses were observed, yet a left-hemisphere bias and unequal weighting of inputs favoring the long-term deaf ear did not converge to a balanced state observed in the binaurally developed system. Bilateral response enhancement was significantly reduced in left auditory cortex suggesting deficits in ipsilateral response inhibition of new, dominant, inputs during bilateral processing. These findings paradoxically demonstrate the adaptive capacity of the adolescent auditory system beyond an early sensitive period for bilateral input, as well as restrictions on its potential to fully reverse cortical imbalances driven by long-term unilateral deafness.