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Objective: (1) Compare the non-device-related costs of bilateral simultaneous cochlear Implantation (BSICI) vs. unilateral, and (2) establish a dollar amount for potential cost savings in direct costs for BSiCI vs. sequential cochlear implantation (CI). Methods: Data from all CI cases performed during the fiscal year 2010-2011 were retrospectively reviewed without exception. Fifty-four patients received unilateral CI and 36 received BSiCI. Demographics and data regarding direct costs associated with the operation and immediate In hospital post-operative period were collected. Results: The total operating room (OR) time for unilateral and BSiCI was 3 hours 00 minutes and 4 hours 37 minutes, respectively, with a mean difference of 1 hour and 36 minutes (SD = 0 hours:06 minutes). The cost of OR and post-anesthetic care unit supporting staff was $3102 and $4240 for unilateral and BSiCI, respectively, with a mean difference of $1138 (SD = 216). Unilateral CI supplies totaled $1348 compared to $1822 for BSICI supplies with an average difference of $438 (SD = 123). Conclusion: The total direct costs for a unilateral CI (excluding implantable device and surgeon fees) were $4362 compared to $5823 dollars for BSiCI. Simultaneous implantation can lead to a potential saving of $2901 compared to sequential Implantation. [ABSTRACT FROM AUTHOR]

Objective: To describe the cochleovestibular apparatus on computed tomography (CT) imaging in patients with cholesteatoma. We asked whether cochleovestibular anomalies coexist in individuals with cholesteatoma. Study Design: Randomized, controlled, prospective measurement. Methods: A database search yielded 145 children with cholesteatoma: 31 met inclusion criteria by not having sensorineural hearing loss, not having an associated syndrome, and having digitally stored temporal bone CT imaging available. Prospective measurement of 31 individuals (62 ears) with unilateral cholesteatoma and 32 normally hearing nonsyndromic controls (64 ears) was performed by a neuroradiologist blinded to the study objective. Twenty-six temporal bone aspects on axial imaging were evaluated (16 measurement, 10 calculated from measurement). Results: The cholesteatoma group had a larger endolymphatic fossa and vestibular aqueduct, and there was a trend for the lateral semicircular canal vestibule to be smaller as compared with controls. Subgroup analysis revealed a gradient in prevalence of these findings being most common in the congenital cholesteatoma group, intermediate in the acquired cholesteatoma group, and least common in controls. There were no differences in measurements between ears with cholesteatoma and contralateral disease-free temporal bones. Conclusions: Children with cholesteatoma have abnormal vestibular anatomy. The gradient in prevalence of these findings may suggest a relationship between congenital and acquired cholesteatoma. These may include a generalized temporal bone anomaly that predisposes to cholesteatoma formation, or a third variable such as genetic mutation may predispose to both anomalous cochleovestibular formation and cholesteatoma. [ABSTRACT FROM AUTHOR]

Objective: To describe temporal bone findings on computed tomography (CT) imaging in GJB2-related hearing loss (HL). We asked whether evaluation of the temporal bone is required in individuals with biallelic GJB2 mutations.Study Design: Randomized, blinded, controlled, prospective measurement.Methods: Blood from 264 pediatric cochlear implant users was analyzed for mutations in the GJB2 gene. Thirty-six aspects of the temporal bone on CT imaging were evaluated in 53 individuals (106 ears) with biallelic disease causing GJB2 mutations. A subset of patients was age matched and compared with normally hearing individuals. Subjects with biallelic GJB2 mutations were tested for mutations in the SLC26A4 gene to rule out Pendred syndrome as a confounding cause of large vestibular aqueduct syndrome.Results: Approximately 53% of ears of subjects (72% of subjects) with biallelic GJB2 mutations had at least one temporal bone anomaly. The most common findings were 1) dilated endolymphatic fossa (28%); 2) hypoplastic modiolus (25%); 3) large vestibular aqueduct (8%); 4) hypoplastic horizontal semicircular canal (8%); 5) hypoplastic cochlea (4%). Compared with normally hearing individuals, the GJB2 group had hypoplasia of the cochlear nerve canal, lateral semicircular canal vestibule, internal auditory canal (t tests, P < .001), and were 11 times more likely to have a hypoplastic modiolus. Dilated endolymphatic fossae were 1.4 times more common in the GJB2 group, and large vestibular aqueducts were 3 times more common in the GJB2 group, as compared with normally hearing controls.Conclusions: Temporal bone anomalies are common in GJB2-related HL, and imaging of the temporal bone should be included in routine evaluation of these individuals. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Middle and external ear anomalies are well recognized in Down syndrome (DS, trisomy 21). Inner ear anomalies are much less frequently described. This study reviews inner ear morphology on imaging to determine the prevalence of cochlear and vestibular anomalies in children with DS. Study Design: The authors conducted a retrospective review of imaging features of (DS) inner ear structures. Methods: Fifty-nine sequential patients with DS with imaging of the inner ear were identified by a radiology report text search program. Quantitative biometric assessment of the inner ear was performed on patients with high-resolution computed tomography or magnetic resonance images of the petrous bone. Petrous imaging was performed for evaluation of inflammatory disease or hearing loss. Spinal imaging, which included petrous views, was performed in most cases to exclude C1 to 2 dislocation, a potential complication of DS. Measurements were compared with normative data. Results: Inner ear dysplasia is much more common in DS than previously reported. Inner ear structures are universally hypoplastic. Vestibular malformations are particularly common and a small bony island of the lateral semicircular canal (<3 mm in diameter) appears highly typical. Additional findings in some patients were persistent lateral semicircular anlage with fusion of the lateral semicircular canal and vestibule into a single cavity, vestibular aqueduct and endolymphatic sac fossa enlargement, cochlear nerve canal hypoplasia, and stenosis or duplication of the internal auditory canal. Stenosis of the external meatus, poor mastoid pneumatization, middle ear and mastoid opacification, and cholesteatoma were common, as expected. [ABSTRACT FROM AUTHOR]

Objective/hypothesis: It is reasonable to suppose that the pattern of sensorineural damage along the length of the cochlea depends on the etiology of a hearing loss (HL). In GJB2-related deafness, we hypothesize that gap junction deficits are uniformly distributed and will result in similar damage along the length of the cochlea as compared with non-GJB2 subjects. We assessed this by measuring patterns of neural activity and hearing from apical versus basal cochlear implant electrode regions.Study Design: This was a prospective, blind, controlled study.Methods: Blood from 301 pediatric cochlear implant users was analyzed for mutations in GJB2 by direct sequencing. After exclusion of patients with monoallelic GJB2 mutations, associated syndromes, or risk factors for HL that were not congenital, 39 children with biallelic GJB2 mutations and 58 without GJB2 mutations were evaluated. Hearing was measured before implantation at frequencies ranging from 250 Hz to 8 kHz. After implantation, neural activity at the apical and basal ends of the implanted array was measured using electrically evoked compound action potentials of the auditory nerve (ECAPs) and evoked stapedius reflexes (ESRs).Results: GJB2 and non-GJB2 groups were not significantly different with respect to sex, age at implantation, duration of auditory deprivation, hearing aid use, duration of aided hearing, ear implanted, implant model, or depth of insertion (P>.05). Children with GJB2-related HL had greater similarities between low- and high-frequency residual hearing and between neural activity electrically evoked at apical and basal regions of the cochlea as compared with children with non-GJB2-related HL who demonstrated larger deficits in basal regions.Conclusion: Results suggest more consistent spiral ganglion survival along the length of the cochlea in GJB2-related HL as compared with non-GJB2-related HL, which appears to involve a decreasing gradient of spiral ganglion survival from the apex to the base of the cochlea. Our findings support our premise that in GJB2-related HL, dysfunction of gap junctions likely occurs to a similar degree in the apical and basal regions of the cochlea. This knowledge might be used to customize implantable devices for patients with HL in the future. [ABSTRACT FROM AUTHOR]

Objectives: Early presentation of congenitally deaf children for cochlear implantation is leading to surgery in younger candidates. The safety of cochlear implantation in children aged 12 months and younger is reviewed with radiologic assessment of mastoid bone anatomy and surgical outcome data. Study Design: Analysis of case records and temporal bone computed tomography (CT) scans with description of surgical technique in infants. Methods: Chart analysis of children aged 12 months or younger at cochlear implantation. Mastoid bone anatomy was compared with older children (mean age 2 years) using CT scans. Results: Twenty-five infants received implants at 7 to 12 months of age because of meningitis (n = 4) or early detection of deafness (n = 21). Mastoid marrow content on CT scan was significantly greater in this age group (P < .001 Mann-Whitney rank sum test), but pneumatization was always adequate for safe identification of surgical landmarks. The smaller size of the mastoid bone was not restrictive. An extended postauricular approach was used in the first 11 cases and a 2.5 cm hair-line incision in the remainder. Ligature tie-down of the device was completed in all cases. No complications occurred. All are full-time implant users, except one with other neurologic sequelae of preoperative meningitis. Conclusions: In our experience, cochlear implant surgery is safe in children aged 7 to 12 months with appropriate anesthetic and postoperative support. The small incision technique is particularly suited to this age group. Ligature fixation of the device is considered advisable because of the increased risk of displacement from frequent falls when learning to walk. [ABSTRACT FROM AUTHOR]

The effect of blindness on sound source identification was studied. Four groups of normally-hearing adults, two sighted and two blind, participated. Subjects were tested using arrays of four and eight loudspeakers, surrounding them in the horizontal plane. One sighted group was tested in quiet. The other groups were tested in continuous 60-dB SPL white noise. Three 75-dB SPL 300-ms stimuli were localized: one-third octave noise bands, centered at 0.5 and 4 kHz, and broadband noise. Broadband noise was easiest to localize (both binaural and spectral cues available), and the 0.5-kHz noise band was the most difficult (primarily interaural temporal difference cue available). Subjects with late-onset blindness achieved significantly higher scores than the early blind and blindfolded sighted subjects. The percentage correct decreased with an increase in the number of speakers, but background noise had no effect. The results attest to the benefit of early visual experience for spatial hearing in adulthood, and demonstrate the negative impact of sudden loss of sight. [ABSTRACT FROM AUTHOR]

Titanium osseointegrated implants for bone-anchored hearing aids (BAHAs) have been in use since 1977. A series of 32 children who received implantation since 1990 is reported. The report focuses on the surgical aspects of BAHAs, predisposing factors, and prevention of complications in an unusual pediatric population. The records of 32 children who had undergone two-stage implantation of a BAHA were retrospectively reviewed. The majority of the patients had craniofacial abnormalities. Of the 32 implantees, 29 use their BAHA at present. Five children failed to achieve osseointegration, and eight patients have had revision surgery for lost abutments, trauma, or chronic skin problems. There were no differences between preimplantation and postimplantation bone or air conduction thresholds. The pediatric BAHA carries with it a unique set of challenges and problems but can be successfully implanted and maintained. [ABSTRACT FROM AUTHOR]

We propose a method of obtaining audible and comfort-able stimulation levels with the use of objective measures when reliable behavioral testing is not possible. Electrically evoked compound action potentials of the auditory nerve (ECAPs) and stapedius reflex (ESR) thresholds were measured in 68 children who use Nucleus devices, beginning at implantation in the operating room (OR) and continuing over the first year of implant use at three implant electrodes. Generic correction factors based on group data were subtracted from ECAP thresh-olds obtained in the OR. The correction factors were specified for two groups of electrodes and two age groups of children. Objective thresholds exceeded behavioral values in 75%, of children and were lower than minimum ESR thresholds in 85% of children. Mean dynamic ranges between the two objective measures spanned 23--36 Clinical Units across the implanted array. Maximum levels, based on ESR thresholds measured in the OR, can be globally adjusted to maintain comfort-able stimulation levels. [ABSTRACT FROM AUTHOR]

BACKGROUND: Children with multiple disabilities account for a small percentage of implantees in a cochlear implant program, but they remain the most challenging group for which to predict benefit from the implant and for cooperation with habilitation postoperatively. PURPOSE: To assess the relationship of pre-implant functional disabilities with postoperative speech perception scores and determine the feasibility of predicting outcome with a cochlear implant in a multiply disabled pediatric population. RESEARCH DESIGN: Retrospective cohort study. STUDY SAMPLE: Sixty-six children with a cochlear implant and at least one additional disability. DATA COLLECTION AND ANALYSIS: We retrospectively examined the relationship between pre-implant Graded Profile Analysis (GPA) scores and postimplant speech perception scores. A pre-implant functional disability score (based on the Battelle developmental screen) was applied to the same cohort of patients and its association with postimplant speech perception scores was examined. RESULTS: The functional disability score significantly predicted high (k > 24) and low (k < 7) speech perception scores (p < 0.001 and p < .0001) and had excellent discrimination ability (c statistic = 0.88 and 0.93 respectively). The GPA score was not significantly associated with speech perception scores (p = 0.519 and p = 0.146) and demonstrated no ability to discriminate postimplant speech perception scores in this implant population (c statistic = 0.49 and c = 0.57). CONCLUSIONS: Prediction of outcomes following cochlear implantation in multiply disabled children can be facilitated using this newly developed functional disability score as an adjunct to traditional candidacy assessments. [ABSTRACT FROM AUTHOR]

Objectives: National guidelines advise delaying initiation of solid foods until after 4-6 months of age and avoiding "high-risk" foods under the age of 4 years. However, foreign body aspiration of food remains a common preventable pediatric emergency. Our primary aim was to investigate public knowledge regarding the safe age of introduction of different foods to children and determine if demographic factors affect this knowledge., Methods: An online survey was designed following a literature review and consultation with an expert panel. This was distributed via social media platforms. A review of our institutional data of bronchoscopy/foreign body retrievals was performed to identify trends., Results: There were 1000 survey responses: 79.4% of respondents cared for children and 21.5% were medical professionals; 37.7% of respondents (n = 385) would offer high-risk foods to children <2 years of age and 56.9% (n = 582) to children <3 years. At our institution nuts (65.7%) were the most common food-related foreign body retrieved from a total of 265 over 21 years. Notably, 80% of respondents (n = 800) would offer whole nuts to children <4 years. Respondents with medical training were more likely to hold off on introducing nuts to children until a later age., Conclusion: Although the public has an overall appreciation of food safety, a significant proportion would feel comfortable offering high-risk foods to children under 2 and 3 years. There is a poor understanding of the danger of nuts and the appropriate age of introduction. Further research into effective public education strategies on safe food introduction in children are warranted., Level of Evidence: 5 Laryngoscope, 135:445-451, 2025., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Background & Rationale: In prior work using non-speech stimuli, children with hearing loss show impaired perception of binaural cues and no significant change in cortical responses to bilateral versus unilateral stimulation. Aims of the present study were to: 1) identify bilateral responses to envelope and spectral components of a speech syllable using the frequency-following response (FFR), 2) determine if abnormalities in the bilateral FFR occur in children with hearing loss, and 3) assess functional consequences of abnormal bilateral FFR responses on perception of binaural timing cues., Methods: A single-syllable speech stimulus (/dα/) was presented to each ear individually and bilaterally. Participants were 9 children with normal hearing (M Age = 12.1 ± 2.5 years) and 6 children with bilateral hearing loss who were experienced bilateral hearing aid users (M Age = 14.0 ± 2.6 years). FFR temporal and spectral peak amplitudes were compared between listening conditions and groups using linear mixed model regression analyses. Behavioral sensitivity to binaural cues were measured by lateralization responses as coming from the right or left side of the head., Results: Both temporal and spectral peaks in FFR responses increased in amplitude in the bilateral compared to unilateral listening conditions in children with normal hearing. These measures of "bilateral advantage" were reduced in the group of children with bilateral hearing loss and associated with decreased sensitivity to interaural timing differences., Conclusion: This study is the first to show that bilateral responses in both temporal and spectral domains can be measured in children using the FFR and is altered in children with hearing loss with consequences to binaural hearing., (Copyright © 2024. Published by Elsevier B.V.)

Introduction: This study reviews the feasibility of implanting active osseointegrated bone conduction devices in young children, below the prior age for FDA indication (<12 years), which has recently been reduced to 5 years. Outcomes included differences in adverse event rates and operative time between two groups (<12 and 12 years or older)., Materials and Methods: This study is a retrospective review of children receiving active osseointegrated bone conduction devices at a tertiary referral center academic hospital. One hundred and twenty-four children received 135 active osseointegrated bone conduction devices (May 2018-March 2024)., Results: Of 135 devices, 77 (57%) were in children <12 years (mean age (SD) = 7.9 (2.0) years, range = 4.9-11.9 years) and 58 (43%) were in 12 years or older (mean age (SD) = 15.1 (1.7) years, range = 12-18 years). Adverse events were significantly higher in the older group, occurring in 8 (10%) of 77 devices in children <12 years and 15 (26%) of 58 devices in children 12 years and older (26%) (Fisher's exact test = 0.0217 at p < 0.05). Major adverse events occurred in 5/124 (4%) patients, with 2 in patients <12 years (2/73, 3%) and 3 in children 12 and older (3/51, 6%). The proportion of major events between groups was not significantly different (Fisher's exact test = 0.4, p < 0.05). Mean surgical time was significantly less (t = -2.8799, df = 120.26, p = 0.005) in the children <12 years (mean (SD) = 66.5 (22.4) min) compared to those 12 and over (mean (SD) = 78.32 (23.1) min)., Conclusions: Implantation of active osseointegrated bone conduction devices is feasible in children as young as 5 years and demonstrates low rates of complication. Further miniaturization may allow even earlier safe intervention., Competing Interests: Financial disclosures/conflict of interests: Dr. Cushing, Dr. Papsin, and Dr. Gordon hold sponsored research agreements with Cochlear Americas. Dr. Papsin, Dr. Cushing, and Dr. Gordon receive speaking fees from Cochlear Americas. Dr. Cushing receives speaking fees for Interacoustics. Dr. Cushing earns royalties from the book, Balance Disorders in the Pediatric Population. Dr. Cushing and Dr. Papsin are patent holders for patent no. 7041-0: Systems and Methods for Balance Stabilization. Dr. Gordon holds the Cochlear America Chair in Auditory Development, is the Bastable-Potts Health Clinician Scientist in Hearing Impairment, and is a lecturer at Salus University., (Copyright © 2024, Otology & Neurotology, Inc.)

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: 4 Laryngoscope, 134:3832-3838, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

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., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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 ( M Age  = 12.9 years) and seventeen adults ( M Age  = 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., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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.

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., (© 2023. The Author(s).)

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., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Objective: Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder whereby abnormal cilia cause a wide array of respiratory tract manifestations including chronic rhinosinusitis. The purpose of this study was to determine whether olfaction and gustation are impaired in children with PCD., Study Design: Cross-sectional study., Setting: Tertiary pediatric academic hospital., Methods: Children with confirmed PCD based on having at least 1 of 3 approved diagnostic criteria as per The American Thoracic Society guidelines were recruited from The PCD Clinic in our tertiary care pediatric hospital. Odor identification ability was tested using the Universal Sniff (U-Sniff) test and taste threshold was measured using an electrogustometer. The main outcome of this study is to determine the incidence of olfactory dysfunction in children with PCD and investigate if there is an associated gustatory dysfunction., Results: Twenty-five children participated (14 male, 11 female), The median age was 10.8 years (range: 4.1-17.9 years). Only 4/25 (16%) complained of olfactory dysfunction prior to testing. None of the patients complained of dysgeusia. However, 48% (12/25) scored less than 7 on the U-Sniff, signifying hyposmia or anosmia. In contrast, scores obtained by electrogustometry were in the normal range. There was no correlation between performance on the U-Sniff and electrogustometry testing., Conclusion: Olfactory impairment in children with PCD is common but underrecognized by patients. This is not associated with abnormal gustation. Among other, this places children with PCD at an increased risk with respect to smelling a fire or detecting spoiled or poisonous food., Competing Interests: The authors have no conflicts of interest relevant to this article to disclose., (© 2023 The Authors. OTO Open published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

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., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To describe the novel presentation, implications, and endoscopic management of a congenital round window cochleocele., Patient: A 16-month old girl with profound hearing loss from bilateral incomplete partition type 1 (IP1) anomaly of the cochlea plus left-sided cochlear nerve aplasia and cochleocele., Intervention: Anomalies were identified with computed tomographic scanning and magnetic resonance imaging during cochlear implant candidacy assessment. While under general anesthesia for right-sided cochlear implantation, the cochleocele was removed and packed with temporalis fascia using transcanal endoscopic ear surgery. The endoscope was held by an assistant while the surgeon packed the round window using a two-handed technique to counter the gush of cerebrospinal fluid., Results: The child made an uneventful recovery with no cerebrospinal fluid leak or meningitis., Conclusions: Cochleocele can protrude through the round window of a cochlea with incomplete partition type 1 anomaly. An increased risk of meningitis secondary to acute otitis media is expected given the known risk from cochleocele arising through a stapes footplate fistula. Care should be taken to check for the presence of a cochleocele in hearing loss from congenital malformations involving the basal turn of the cochlea on imaging and also at the time of cochlear implant surgery. Repair should be considered at the first opportunity to prevent meningitis. If early cochlear implant surgery is not feasible or appropriate (as, for example, with cochlear nerve aplasia), transcanal endoscopic ear surgery provides good access for a low-morbidity approach., Competing Interests: Sources of support and disclosure of funding: No sources of financial support. Dr. James has no conflicts of interest. Dr. Papsin is a speaker for Cochlear Corporation but no conflicts of interest relevant to this article., (Copyright © 2022, Otology & Neurotology, Inc.)

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., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

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., (© 2022 The Authors. Human Brain Mapping published by Wiley Periodicals LLC.)

Aim: Most button battery (BB) ingestions in children are unwitnessed leading to prolonged exposures and severe complications. One third of ingestions occur from free BB, that are stored or awaiting disposal. Recommendations have been made to cover the terminals of discarded BB with adhesive tape; however, it is unclear if this practice prevents injury. Our aim was to determine if tape could prevent oesophageal injury in a cadaveric porcine model., Methods: Electrical, masking, packing and duct tape were compared. One BB was left untaped. Taped BBs were placed in a cadaveric porcine oesophagus controlled for temperature and humidification. Specimens were assessed at 0, 0.5, and hourly for 6 h by visual inspection, temperature and pH. BB voltage was measured before and after testing. All tests were repeated in triplicate., Results: Oesophageal specimens demonstrated burn prevention in the packing and duct tape trials. Burns were seen in 2/3 trials with electrical tape and 3/3 trials with masking tape. pH remained neutral throughout the study for all packing and duct tape specimens. pH remained neutral initially for masking tape but increased rapidly to 12 by 2 h. There was no change in battery voltage for the packing tape and duct tape trials. There was a 16.3% reduction in voltage for masking tape which was similar to controls., Conclusions: Taping BB with packing tape and duct tape prevented oesophageal burns. This may provide a novel method of burn prevention for loose BB intended for disposal., (© 2022 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Auriculocondylar syndrome (ACS) is a rare craniofacial disorder characterized by mandibular hypoplasia and an auricular defect at the junction between the lobe and helix, known as a "Question Mark Ear" (QME). Several additional features, originating from the first and second branchial arches and other tissues, have also been reported. ACS is genetically heterogeneous with autosomal dominant and recessive modes of inheritance. The mutations identified to date are presumed to dysregulate the endothelin 1 signaling pathway. Here we describe 14 novel cases and reassess 25 published cases of ACS through a questionnaire for systematic data collection. All patients harbor mutation(s) in PLCB4, GNAI3, or EDN1. This series of patients contributes to the characterization of additional features occasionally associated with ACS such as respiratory, costal, neurodevelopmental, and genital anomalies, and provides management and monitoring recommendations., (© 2022 Wiley Periodicals LLC.)

Objectives: To identify whether mismatched bilateral cochlear implants compromise balanced stimulation of the two auditory nerves and establish asymmetric hearing in children., Methods: Behavioural and electrophysiological measures were completed in 47 children receiving bilateral CIs in the same surgery (simultaneously): 27 children received a peri‑modiolar N24RE array in one ear and a 422 anti-modiolar array in the other (experimental group) and 20 children received 2 peri‑modiolar arrays (control group). Differences in current levels between the two devices were measured by electrically evoked compound action potentials (ECAPs) at the time of surgery. These data were compared with minimum and maximum comfortably loud levels programmed in each speech processor (T-levels, C-levels, respectively) after 12 months of bilateral CI use. Asymmetries in functional hearing between arrays were measured in open set speech perception testing between 3 to 5 years of CI use., Results: Higher current levels were required from the anti-modiolar than peri‑modiolar array to evoke balanced interaural ECAP amplitudes (mismatched group: mean ± SD difference: -9.9 ± 22.6; matched group: -0.8 ± 26.5). This difference was larger in the experimental group than control group (t = -2.51; p = 0.016) and remained constant with increases in current level from ECAP threshold to maximum amplitudes (dynamic range) in many but not all children in both groups. T and C-levels were poorly predictive of levels needed to evoke balanced ECAP amplitudes in children with mismatched devices (F(1, 312) = 1.3, p = 0.263). Speech perception scores were more asymmetric between ears in children using bilateral mismatched arrays (mean ± SD: 73.8 ± 16.4 at the peri‑modiolar array; 57.7 ± 26.4 at the anti-modiolar array), compared to children with bilateral matched arrays (right ear: 78.0 ± 10.4; left ear: 74.9 ± 13.5)., Conclusion: Higher current level requirements at the anti-modiolar array compared to the peri‑modiolar array in children with bilateral mismatched CIs are not fully accounted for in device programming. Mismatched electrodes in children receiving bilateral cochlear implants increases the risk of asymmetric hearing., (Copyright © 2022. Published by Elsevier B.V.)