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.

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.

A 20-year Study of Intracranial Pyogenic Complications of Sinusitis in Children.

BACKGROUND: Intracranial pyogenic complications of sinusitis in children can lead to serious sequelae. We characterize the clinical, epidemiologic and microbiologic characteristics of children with such complications over a 20-year period. METHODS: Single-center retrospective chart review. Cases were identified based on International Classification of Diseases diagnostic codes (ICD)-9 and ICD-10 depending on the year and by reviewing all intracranial microbiological samples. RESULTS: A total of 104 cases of complicated sinusitis were included after review of 1591 charts. Median age was 12 (IQR 9-14); 72 were male (69%). The most frequent complications were epidural empyema (n = 50, 48%), subdural empyema (n = 46, 44%) and Pott's puffy tumor (n = 27, 26%). 52% (n = 54) underwent neurosurgery and 46% (n = 48) underwent otolaryngological surgery. The predominant pathogen isolated from sterile site specimens was Streptococcus anginosus (n = 40, 63%), but polymicrobial growth was common (n = 24; 38%). The median duration of intravenous antibiotic therapy was 51 days (IQR 42-80). Persistent neurological sequelae (or death, n = 1) were found in 24% (n = 25) and were associated with the presence of cerebritis and extensive disease on neuroimaging ( P = 0.02 and P = 0.04, respectively). CONCLUSIONS: Intracranial complications of sinusitis continue to cause significant morbidity in children. Polymicrobial infections are common, which reinforces the need for broad-spectrum empiric antibiotic therapy and cautious adjustment of the antibiotic regimen based primarily on sterile site cultures. The association of neurologic sequelae with the presence of cerebritis and extensive intracranial involvement on neuroimaging suggest that delayed diagnosis may be a contributor to adverse outcome.

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.

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.

Improving the Hearing Status Discrimination of the Health Utilities Index, Mark 3: Design of the Hearing Status Classification System.

OBJECTIVE: Redesign the health status classification system of the Health Utilities Index, Mark 3 (HUI-3) "hearing" attribute to improve its discrimination of hearing-impaired health states. STUDY DESIGN: Domain and item selection from a previously generated item set guided by a domain and item importance survey, structural independence, and cognitive interviews with patients. SETTING: Tertiary referral center. PARTICIPANTS: Patients with a range of hearing loss severities, etiologies, and treatment experiences participated in the domain and item importance survey (n = 108) and hour-long cognitive interviews (n = 10). MAIN OUTCOME AND MEASURES: Subattributes and levels for the novel Hearing attribute. Domain and item importance was scored on a seven-point Likert scale (1, not at all important; 7, extremely important). RESULTS: Mean domain importance was highest for "speech in noise" (6.21; 95% confidence interval, 5.98-6.43) and lowest for "benefits of hearing loss" (3.46; 95% confidence interval, 3.03-3.89). Domains with moderate or greater ( r ≥ 0.5) domain importance Pearson correlation or construct overlap that interfered with structural independence were combined into single subattributes. Iterative adjustments to instructions, items, and phrasing were guided by cognitive interviews to derive the final instrument with seven subattributes: speech, environmental sounds, localization, listening effort, tinnitus, music, and assistive devices. The novel hearing attribute defines 25,920 unique hearing states. CONCLUSION: The novel HUI-hearing is part of a comprehensive health utility instrument designed for individuals with hearing loss. Pending derivation of a hearing single attribute utility function and evaluation of measurement properties, our innovative approach could be used to improve health utility measurement for impairments described by any of the other HUI-3 attributes.

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.

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.

Hearing Instability in Children with Congenital Cytomegalovirus: Evidence and Neural Consequences.

OBJECTIVE/HYPOTHESIS: Sensorineural hearing loss (SNHL) is a common sequela of congenital cytomegalovirus (cCMV), potentially exacerbating neurocognitive delay. The objectives of this study were to assess: (1) age at which SNHL in children with cCMV; (2) stimulability of the auditory system in children with cCMV following cochlear implantation (CI); and (3) whether features of magnetic resonance imaging (MRI) potentially are predictive of hearing outcomes. METHODS: In this retrospective study of a prospectively acquired cohort, 123 children with cCMV who were referred for hearing loss at a single tertiary referral hospital over 20 years were compared with an unmatched comparative group of 90 children with GJB2-related deafness. Outcome measures were results of newborn hearing screening (NHS), behavioral audiograms, and, in a subgroup of cochlear implant (CI) users, responses from the auditory nerve and brainstem evoked by CI at initial activation, as well as lesional volume of FLAIR-hyperintense signal alterations on MRI. RESULTS: All but 3 of 123 children with cCMV had confirmed and persistent SNHL. At birth, 113 children with cCMV underwent NHS, 31 (27%) passed in both ears and 23 (20%) passed in one ear (no NHS data in 10 children). At the first audiologic assessment, 32 of 123 (26%) had normal hearing bilaterally; 35 of 123 (28%) had unilateral SNHL; and 57 of 123 (46%) had bilateral SNHL. More than half (67 of 123, 54%) experienced hearing deterioration in at least one ear. Survival analyses suggested that 60% of children who developed SNHL did so by 2.5 years and 80% by 5 years. In the children who passed NHS in one or both ears, 50% developed hearing loss by 3.5 years in the ear, which passed unilaterally (n = 23 ears), and 50% by 5 years in bilateral passes (n = 62 ears). Hearing loss was significant enough in all but one child with isolated high-frequency loss for rehabilitation to be indicated. Hearing thresholds in individual ears were in the CI range in 83% (102 of 123), although duration of deafness was sufficient to preclude implantation at our center in 13 children with unilateral SNHL. Hearing aids were indicated in 16% (20 of 123). Responses from the auditory nerve and brainstem to initial CI stimulation were similar in children with cCMV-related SNHL compared with GJB2-related SNHL. Characteristic white matter changes on MRI were seen in all children with cCMV-related SNHL (n = 91), but the lesion volume in each cortical hemisphere did not predict degree of SNHL. CONCLUSIONS: cCMV-related SNHL is often not detected by NHS but occurs with high prevalence in early childhood. Electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2-related SNHL. Hyperintense white matter lesions on FLAIR MRI are consistently present in children with cCMV-related SNHL but cannot be used to predict its time course or degree. Combined, the data show early and rapid deterioration of hearing in children with cCMV-related SNHL with potential for good CI outcomes if SNHL is identified and managed without delay. Findings support universal newborn screening for cCMV followed by careful audiological monitoring. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:S1-S24, 2022.

Vestibular and Balance Impairment Is Common in Children With Primary Ciliary Dyskinesia.

OBJECTIVE: Primary ciliary dyskinesia (PCD) is a rare genetic disorder that presents with a wide range of respiratory complaints. The most common otologic associated disorder is middle ear effusion. We ask if children with PCD suffer from vestibular impairment. STUDY DESIGN: Cross-sectional cohort study. SETTING: Tertiary pediatric referral hospital. PATIENTS/PARTICIPANTS: Children with confirmed PCD. INTERVENTION/METHODS: All included participants were clinically assessed to be at baseline then basic demographics and medical histories were collected, including the specific genetic mutation these patients have. After which, all patients underwent two vestibular tests. The first was to assess their ability to stand on one foot with their eyes open and then with their eyes closed and the second was video head impulse test (vHIT). MAIN OUTCOME MEASURES: Ability standing on one foot with the eyes closed and vHIT. RESULTS: During this period, 25 children with PCD were recruited for the study. The mean age at recruitment was 11.26 years (4-18 yr). There were 11 women and 14 men. All patients were able to participate in both tests. Nineteen participants (76%) had vestibular impairment. Fifteen of them failed to stand on one foot with their eyes closed and six of 25 had abnormal vHIT. The most common involved gene was DNAH5 8/25 (32%) and it was associated with vestibular impairment in seven of eight participants (87.5%). CONCLUSION: The majority of children with PCD that we tested suffered from vestibular impairment that was previously undiagnosed. This potentially indicates that imbalance and vestibular pathology is under-diagnosed in children with PCD.

First Generation Osseointegrated Steady State Implant Benefits in Children With Hearing Loss.

OBJECTIVE: To assess outcomes of a new Osseointegrated Steady State Implant (OSSI) for bone conduction in adolescents. METHOD: In an initial trial, 14 adolescents (14.5 years of age, SD = 2.22) were provided with an OSSI; unilateral OSSI (n = 13), bilateral OSSI in sequential surgeries (n = 1). Outcomes measured were surgical duration, complications, hearing thresholds, speech perception and self-reported hearing benefits using the Speech and Spatial Quality of Hearing Questionnaire. RESULTS: The surgical times were mean 93.6 minutes (SD = 33.3). Surgery was slightly longer in three adolescents who required skin flap reduction (n = 1) or significant bone polishing (n = 2) (121.33 minutes, SD = 8.14). Adverse events occurred in two adolescents post-implant poor external device retention in one child requiring revision flap reduction and inflammation at the incision site due to magnet overuse in another. The "Digital Link Calibration" measure was a good proxy predictor of the strength of magnet required for external device adherence (p = 0.002). The OSSI increased audibility in the implanted ear by mean 31.48 dB HL (SE = 1.58). Aided thresholds were best at 1 kHz (mean 25.33 dB HL, SD = 22.60) and only slightly poorer at 3000 and 4000 Hz (estimate decrease = 8.33 dB HL, SE = 3.54), reflecting good auditory sensitivity even at high frequencies. Speech perception when using the new device alone was good (89.67%, SD = 7.84%) and self-reported hearing by participants and parents improved in all domains assessed by the Speech and Spatial Quality of Hearing Questionnaire (estimate = 1.90 points, SE = 0.25, p < 0.0001). CONCLUSION: The OSSI provides hearing benefits with surgical safety in a carefully selected cohort of adolescents.

Relieving bronchial compression due to cardiomegaly: The role of aortopexy when left ventricular assist device support just is not enough.

Enlarged cardiac structures, especially those on left side have the potential to cause airway compression in pediatric patients with chronic heart failure, owing to their proximity to and impact on the trachea-bronchial tree. Ventricular assist devices are effective in decompressing such hearts thereby alleviating airway problems. Aortopexy serves as an effective airway decompressive measure in cases with persistent airway compression despite effective cardiac decompression by ventricular assist devices. We report a case of 1-year-old male patient with dilated cardiomyopathy in whom airway compression persisted despite ventricular assist device implantation. Aortopexy was effective in relieving airway compression allowing for subsequent extubation and successful heart transplantation.

Risks and Benefits of Adenotonsillectomy in Children With Cerebral Palsy With Obstructive Sleep Apnea: A Systematic Review.

OBJECTIVES/HYPOTHESIS: Assess the risks and benefits of adenotonsillectomy (AT) for obstructive sleep apnea (OSA) in children with cerebral palsy (CP). STUDY DESIGN: Systematic review. METHODS: We conducted a systematic review of Medline, Embase, and Cochrane Central Registry from 1946 to 2021. Broad search concepts included cerebral palsy, pediatric, tonsillectomy/adenoidectomy, and sleep. Additional articles were identified by searching reference lists. Studies on the safety and efficacy of AT for OSA management in children with CP were included. RESULTS: Fifteen articles met inclusion criteria. Articles were classified into one or more of four themes: intraoperative risk (n = 1), postoperative risk (n = 3), postoperative care requirements (n = 6), and surgical outcomes (n = 7). No intraoperative anesthetic complications were reported. Postoperatively, respiratory complications including pneumonia were common and necessitated additional airway management. Following AT, children with CP required close postoperative observation, experienced increased lengths of stay, and had increased odds of unplanned intensive care unit (ICU) admission. Benefits following AT were improvement in OSA as measured by a reduction in obstructive apnea-hypopnea index (OAHI) as well as improved quality of life in some; however, many patients went on to require tracheostomy due to persistent OSA. CONCLUSIONS: Children with CP who undergo AT have a significant risk of developing a postoperative respiratory complication. Realistic counseling of families around increased perioperative risks in this population is imperative and close postoperative monitoring is critical. Many children will obtain a reduction in OAHI, but additional surgical management is often required, including tracheostomy. Further research is needed to determine the best management strategy for OSA in children with CP. Laryngoscope, 132:687-694, 2022.