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The article examines the ethical considerations of introducing school-based hearing and vision screening programs in Samoa and the Pacific Islands. Topics discussed include the importance of early detection of hearing and vision impairments, the use of mobile technology in low-resource settings, and the collaboration needed among healthcare and educational stakeholders to ensure effective program implementation.

Abstract Objectives Auditory brainstem response (ABR) is the gold standard to assess hearing loss in pediatric patients. Multiple widely accepted ABR protocols with varying parameters are accepted, difference in standards may lead to misdiagnosis or delay in diagnosis and treatment. This study investigates the quality of ABR testing in pediatric patients in addition to changes in diagnoses and management. Study Design Retrospective chart review. Setting University Hospital, Rutgers New Jersey Medical School. Methods Retrospective chart review was conducted for all pediatric patients from 2012 to 2019 who had undergone prior outside ABR testing before presenting to our institution for hearing loss evaluation. The ABR tests were analyzed for completeness following the American Academy of Audiology (AAA), American Speech Language Hearing Association (ASHA), and The Joint Committee on Infant Hearing (JCIH) guidelines. Descriptive statistics on changes in patient diagnoses and interventions after repeat ABR were performed. Results 80 patients met inclusion criteria. The most common reasons for an incomplete ABR were inadequate components of testing including tone burst bone conduction (85.0%), polarity (82.5%), and tone burst air conduction (48.7%). 77 of the patients who presented required a repeat ABR. 37 repeated ABRs resulted in a change of diagnosis, the most common being from unspecified hearing loss to sensorineural hearing loss (10%). 23 cases had a change in ultimate management. Conclusion Incomplete ABR testing may result in misdiagnosis, delay in diagnosis and treatment. Identifying common reasons for incomplete ABR testing may aid Otolaryngologists develop a screening workflow to recognize patients requiring repeat testing. Level of Evidence 4.

Background: Fluctuating conductive hearing loss resulting from middle ear conditions, such as otitis media, is the most common cause of hearing loss in children, with Indigenous Peoples experiencing otitis media at a rate three times higher than non-Indigenous populations. Children with chronic hearing loss face increased educational, social, and economic challenges. However, treating and documenting fluctuating hearing loss remains difficult due to its sporadic and invisible nature, frequently leading to delayed or missed identification and inconsistent management. Methods: A comprehensive literature search was completed with a librarian, but few resources were located for this condition and population. Results: This practical guideline aims to improve the documentation and subsequent management of otitis media in school-aged children, with a focus on rural and Indigenous communities in Canada, where access to healthcare professionals may be limited. Conclusions: Despite efforts to raise awareness about otitis media in rural and Indigenous communities, there are still few accessible tools for caregivers to track the severity of fluctuating hearing loss. This guideline aims to help fill this gap.

Objective: To determine the positivity rate of congenital cytomegalovirus (cCMV) testing among universal, hearing‐targeted CMV testing (HT‐cCMV) and delayed targeted dried blood spot (DBS) testing newborn screening programs, and to examine the characteristics of successful HT‐cCMV testing programs. Study Design: Prospective survey of birth hospitals performing early CMV testing. Setting: Multiple institutions. Methods: Birth hospitals participating in the National Institutes of Health ValEAR clinical trial were surveyed to determine the rates of cCMV positivity associated with 3 different testing approaches: universal testing, HT‐cCMV, and DBS testing. A mixed methods model was created to determine associations between successful HT‐cCMV screening and specific screening protocols. Results: Eighty‐two birth hospitals were surveyed from February 2019 to December 2021. Seven thousand six hundred seventy infants underwent universal screening, 9017 infants HT‐cCMV and 535 infants delayed DBS testing. The rates of cCMV positivity were 0.5%, 1.5%, and 7.3%, respectively. The positivity rate for universal CMV screening was less during the COVID‐19 pandemic than that reported prior to the pandemic. There were no statistically significant drops in positivity for any approach during the pandemic. For HT‐cCMV testing, unique order sets and rigorous posttesting protocols were associated with successful screening programs. Conclusion: Rates of cCMV positivity differed among the 3 approaches. The rates are comparable to cohort studies reported in the literature. Universal CMV prevalence decreased during the pandemic but not significantly. Institutions with specific order set for CMV testing where the primary care physician orders the test and the nurse facilitates the testing process exhibited higher rates of HT‐cCMV testing. [ABSTRACT FROM AUTHOR]

Objective: The primary objective of this pilot study is to describe the impacts of bullying and peer victimization (BPV) in children with hearing loss. Study design: Prospective clinical survey study. Methods: This pilot study enrolled children between 8 and 18 years who were administered validated surveys at an outpatient clinic between July 2020 and March 2022. Surveys included health-related quality of life questionnaires (PedsQL and EQ-5D-Y), along with bullying and peer victimization questionnaires ("My Life in School" and the multidimensional peer victimization scale). Responses were scored with multivariate analysis. Clinical histories and active ICD-10 codes were also collected. Results: About 105 patients were recruited with a mean age of 13.1 years (SD = 3.15) and hearing loss (n = 30) among the top otolaryngological diagnoses. When surveying patients with hearing loss, 50.0% (n = 15) actively used a hearing aid device. Children (ages 8-12 years) with hearing loss reported a significantly lower psychosocial health-related quality of life than their peers without hearing loss (P =.007), though this was not the case for adolescents (ages 13-18 years) with hearing loss (P =.099). These trends did not change significantly before or after students resumed in-person classes. Children who wore hearing aids did not report a different BPV level than their peers. Conclusion: In this small sample of school-aged children, any hearing loss, even with mild severity, was associated with diminished health-related quality of life; however, this was unrelated to hearing aid use. Wearing a hearing aid did not appear to be linked to higher bullying and peer victimization rates. Along with further studies on BPV with larger sample sizes, the findings in this study may help physicians counsel parents and children on the psychosocial aspects of hearing loss treatment and guide care decisions. Level of Evidence: 3 [ABSTRACT FROM AUTHOR]

Objective: To identify sociodemographic factors associated with pediatric late‐identified hearing loss (LIHL) and classify novel subgroups within the LIHL population. Study Design: Retrospective cohort. Setting: Tertiary children's hospital. Methods: Our cohort included children with permanent hearing loss (HL) between 2012 and 2020 (n = 1087). Patients with early‐identified HL were compared to patients with LIHL (>6 months of age at diagnosis), and 3 subgroups: (1) late‐identified congenital HL: failed NHS but had a diagnostic audiogram >6 months old; (2) late‐onset HL: passed NHS and identified with HL after 6 months old; (3) late‐identified, unknown‐onset: unknown NHS results, identified after 6 months old. Geospatial analysis was performed using ArcGIS Pro. Results: Compared with early‐identified children, children with LIHL were more likely to have more comorbidities (odds ratio [OR] = 1.12, [1.01, 1.23]), be an under‐represented minority (URM) (OR = 1.92, [1.27, 2.93]) and have a higher social vulnerability index (SVI) (adjusted odds ratio [AOR] = 2.1, [1.14, 3.87]). However, subgroups in the LIHL cohort had variable associations. Children with late‐identified unknown onset hearing loss were uniquely associated with a primarily non‐English speaking household (AOR = 1.84, [1.04, 3.25]), whereas children with late‐onset hearing loss were less likely to have public insurance (AOR = 0.47, [0.27, 0.81]. There were no significant associations for children with late‐identified congenital hearing loss. Neighborhood disadvantage, as measured by SVI, had an increased association with late‐identified unknown onset HL (AOR = 4.08, [2.01, 8.28]) and a decreased association with late‐onset HL (AOR = 0.40, [0.22, 0.72]). Conclusion: Sociodemographic factors serve as proxies for health care access, and these factors vary across LIHL pathways. Understanding the risk factors associated with each LIHL subgroup may help address disparities in pediatric HL identification. [ABSTRACT FROM AUTHOR]

Background: Universal newborn hearing screening (UNHS) is effective in identifying newborns with possible hearing loss (HL). Outpatient follow-up for newborns referred after hospital-based screening remains a potential area of improvement. In this study, we evaluate the efficacy of a community health worker (CHW) intervention in promoting adherence to outpatient rescreening for newborns referred after initial UNHS. Methods: A mixed prospective-retrospective cohort study was performed to evaluate a CHW intervention at an academic medical center. Caregivers of referred newborns were contacted by CHWs prior to discharge and educated about HL and the importance of follow-up screening. The CHW outreach intervention was performed for 297 referred newborns between May 2020 and June 2021 and compared to a cohort of 238 newborns without the CHW intervention between March 2019 and June 2021. Statistical analyses were conducted using 2 × 2 Chi-square tests, two-tailed unpaired t -tests, multinomial logistic regression, and multiple linear regression. Results: In the intervention group, 236 of 297 newborns (79.5%) completed their outpatient follow-up rescreening; in the comparison group, 170 of 238 newborns (71.4%) completed their follow-up rescreening (P =.031, OR = 1.55 with regression P =.04). In the intervention group, the average time to follow-up was 13.4 days versus 12.5 days for the comparison group (P =.449, multiple R 2 =.02 with P =.78). Conclusions: CHW outreach intervention may increase adherence to outpatient follow-up rescreening for newborns referred after initial, hospital-based UNHS. Expansion of nursery teams to include CHWs may thus improve completion of recommended follow-up hearing screens. [ABSTRACT FROM AUTHOR]

IntroductionRapid advancements in Next Generation Sequencing (NGS) and bioinformatics tools have allowed physicians to obtain genetic testing results in a more rapid, cost-effective, and comprehensive manner than ever before. Around 50% of pediatric sensorineural hearing loss (SNHL) cases are due to a genetic etiology, thus physicians regularly utilize targeted sequencing panels that identify variants in genes related to SNHL. These panels allow for early detection of pathogenic variants which allows physicians to provide anticipatory guidance to families. Molecular testing does not always reveal a clear etiology due to the presence of multigenic variants with varying classifications, including the presence of Variants of Uncertain Significance (VUS). This study aims to perform a preliminary bioinformatics characterization of patients with variants associated with Type II Usher Syndrome in the presence of other multigenic variants. We also provide an interpretation algorithm for physicians reviewing molecular results with medical geneticists.MethodsReview of records for multigenic and/or VUS results identified several potential subjects of interest. For the purposes of this study, two ADGRV1 compound heterozygotes met inclusion criteria. Sequencing, data processing, and variant calling (the process by which variants are identified from sequence data) was performed at Invitae (San Francisco CA). The preliminary analysis followed the recommendations outlined by the American College of Medical Genetics and Association for Molecular Pathology (ACMG-AMP) in 2015 and 2019. The present study utilizes computational analysis, predictive data, and population data as well as clinical information from chart review and publicly available information in the ClinVar database.ResultsTwo subjects were identified as compound heterozygotes for variants in the gene ADGRV1. Subject 1's variants were predicted as deleterious, while Subject 2's variants were predicted as non-deleterious. These results were based on known information of the variants from ClinVar, multiple lines of computational data, population databases, as well as the clinical presentation.DiscussionEarly molecular diagnosis through NGS is ideal, as families are then able to access a wide range of resources that will ultimately support the child as their condition progresses. We recommend that physicians build strong relationships with medical geneticists and carefully review their interpretation before making recommendations to families, particularly when addressing the VUS. Reclassification efforts of VUS are supported by studies like ours that provide evidence of pathogenic or benign effects of variants.

After an infant hearing loss (HL) diagnosis, parents face a multitude of stressors as they try to make the best decisions for their children. For many parents with typical hearing, opting for cochlear implantation is part of the decision-making process. Findings from a sample of hearing parents who chose cochlear implantation for their children with HL reveal that they experience (a) five resilience triggers, (b) five resilience processes, and (c) multiple relationships between the triggers and processes. In this study, we also advance the communication theory of resilience to illustrate four ways parents enact resilience on behalf of their children and formalize a heuristic we call other resilience. By examining the resilience between and across the resilience triggers, parent processes, and children processes, we provide practical applications for clinicians, families, and networks. [ABSTRACT FROM AUTHOR]

Objectives: Enlarged vestibular aqueduct (EVA) is the most common anatomic abnormality contributing to permanent hearing loss (HL) in children. Although the association between EVA and HL is well‐documented, the pass rate for the newborn hearing screening (NBHS) for patients with EVA‐related HL is not. Our objective was to investigate the association between NBHS results and audiologic and clinical outcomes in a large cohort of pediatric patients with EVA. Methods: This was a retrospective chart review of patients seen in the Boston Children's Hospital (BCH) Department of Otolaryngology and Communication Enhancement with confirmed HL, known NBHS results, and confirmed EVA. Demographic, clinical, audiologic, and imaging data were collected from the medical record. Frequency‐specific data points from pure‐tone audiograms and/or automated auditory brainstem response tests were recorded, and four‐frequency pure tone average was calculated using air conduction thresholds at 500, 1000, 2000, and 4000 Hz. Results: Of the 183 patients included in the study, 84 (45.9%) passed their NBHS, whereas 99 (54.1%) did not pass. Compared with patients who did not pass, patients who passed were more likely to have unilateral EVA and unilateral HL, whereas they were less likely to undergo cochlear implantation and to have causative SLC26A4 variants. Conclusions: EVA‐associated HL may be identified at birth or during childhood, with nearly half the patients in this cohort passing their NBHS. Our results provide prognostic information for patients with EVA who pass their NBHS and highlight the importance of regular hearing monitoring for children not initially suspected of having HL. Level of Evidence: 4 Laryngoscope, 133:2786–2791, 2023 [ABSTRACT FROM AUTHOR]

Objectives: Genetic testing is the standard‐of‐care for diagnostic evaluation of bilateral, symmetric, sensorineural hearing loss (HL). We sought to determine the efficacy of a comprehensive genetic testing method, exome sequencing (ES), in a heterogeneous pediatric patient population with bilateral symmetric, bilateral asymmetric, and unilateral HL. Methods: Trio‐based ES was performed for pediatric patients with confirmed HL including those with symmetric, asymmetric, and unilateral HL. Results: ES was completed for 218 probands. A genetic cause was identified for 31.2% of probands (n = 68). The diagnostic rate was 40.7% for bilateral HL, 23.1% for asymmetric HL, and 18.3% for unilateral HL, with syndromic diagnoses made in 20.8%, 33.3%, and 54.5% of cases in each group, respectively. Secondary or incidental findings were identified in 10 families (5.52%). Conclusion: ES is an effective method for genetic diagnosis for HL including phenotypically diverse patients and allows the identification of secondary findings, discovery of deafness‐causing genes, and the potential for efficient data re‐analysis. Level of Evidence: 4 Laryngoscope, 133:2417–2424, 2023 [ABSTRACT FROM AUTHOR]

Objectives: Cochlear implantation (CI) is a safe and effective procedure for hearing rehabilitation, with few major complications. Device exposure or extrusion is a rare but major complication that often necessitates explantation due to wound dehiscence or infection. The objective of this report is to present a previously undescribed case in which the cochlear implant grounding wire extruded in 16-month-old patient 3 months post-operatively in the absence of trauma or infection. Methods: We reviewed the case report and the pertinent literature. Results: A 16-month old male suffered extrusion of his left cochlear implant grounding wire without known etiology 86 days post-operatively after bilateral cochlear implantation. The patient was taken for surgery, and the electrode was reimplanted without complication followed by 48 hours of prophylactic intravenous antibiotics. Nine month follow up revealed the implant functioning appropriately, with no further major complications encountered. Conclusion: We present this unique case to demonstrate that a cochlear implant grounding wire extrusion is not necessarily an indication for explantation in the absence of infection. [ABSTRACT FROM AUTHOR]

Objective: Clinical guidelines recommend genetic testing when evaluating congenital and late‐onset sensorineural hearing loss (SNHL). Genetic diagnoses can provide parents additional information regarding anticipated hearing loss progression, comorbid conditions, and family planning. Additionally, obtaining a genetic diagnosis may increase parental acceptance of hearing loss and subsequent pursuit of intervention. This study evaluates the association between genetic diagnoses and hearing loss intervention. Methods: We included children ages 0–18 years with SNHL who were hearing aid or cochlear implant candidates but non‐users and underwent hearing‐loss gene panel testing prior to initiating intervention. Univariate analyses were performed to identify predictors of hearing aid fitting or cochlear implantation. Multivariate logistic regression evaluated the impact of demographic and clinical factors on subsequent intervention. Results: Of the 385 children with SNHL who underwent hearing loss gene panel testing, 111 were included. Median age was 7.5 years. 56% were underrepresented minorities, 71% were non‐White, and 71% were publicly insured. Those found to have a genetic diagnosis were 4.6 times as likely to subsequently undergo intervention (p = 0.035). Additionally, bilateral hearing loss and earlier age of genetic testing were associated with increased likelihood of intervention. Conclusion: Up to half of children with SNHL are suspected to have an underlying genetic etiology. Children diagnosed with a genetic diagnosis are significantly more likely to subsequently utilize hearing aids or cochlear implantation. This provides additional support for clinical guidelines recommending genetic testing not only due to the impact of prognostication but also on treatment decision‐making. Level of Evidence: 4 Laryngoscope, 133:1982–1986, 2023 [ABSTRACT FROM AUTHOR]

BACKGROUND: We aimed to investigate the changes that may occur in the auditory neural network in pediatric congenital hearing loss cases. METHODS: Fifty-four cochlear implant candidates and 47 normal-hearing controls were included in this retrospective study. Fractional anisotropy, radial diffusivity, and apparent diffusion coefficient maps were generated. We placed region of interest on the cochlear nucleus, superior olivary nucleus, lateral lemniscus, medial geniculate body, auditory radiation, Heschl's gyrus, inferior fronto-occipital fasciculus, superior longitudinal fascicle, and corpus callosum splenium. The area of the cochlear nerve was measured. Diffusion tensor imaging metrics, children's ages, and cochlear nerve area were compared. RESULTS: Apparent diffusion coefficient and radial diffusivity values of patients were higher than the control group in all places except the radial diffusivity values of medial geniculate body. The fractional anisotropy values of the patients in lateral lemniscus, auditory radiation, Heschl's gyrus, inferior fronto-occipital fasciculus, superior longitudinal fascicle, and corpus callosum splenium were lower than the control group. There is a positive correlation between fractional anisotropy and age in both patient and control groups for all locations. The cochlear nerve area is lower in patients (0.88 ± 0.29) than in the control group (1.18 ± 0.14) (P = .000). The cochlear nerve area has a positive correlation with age in the patient group (P = .000) but has not in the control group. The cochlear nerve area positively correlates with fractional anisotropy values of all locations except fractional anisotropy values of medial geniculate body. CONCLUSION: The alterations of diffusion tensor imaging metrics on the auditory pathway reflect the microstructural changes of white matter tracts. [ABSTRACT FROM AUTHOR]

BackgroundChildhood hearing impairment affects language and cognitive development. Profound congenital sensorineural hearing impairment can be due to an abnormal cochleovestibular nerve (CVN) and cochleovestibular malformations, however, the etiology of these conditions remains unclear.MethodsWe used a trio-based exome sequencing approach to unravel the underlying molecular etiology of a child with a rare nonsyndromic CVN abnormality and cochlear hypoplasia. Clinical and imaging data were also reviewed.ResultsWe identified a de novo missense variant [p(Asn174Tyr)] in the DNA-binding Homeodomain of SIX1, a gene which previously has been associated with autosomal dominant hearing loss (ADHL) and branchio-oto-renal or Branchio-otic syndrome, a condition not seen in this patient.ConclusionsSIX1 has an important function in otic vesicle patterning during embryogenesis, and mice show several abnormalities to their inner ear including loss of inner ear innervation. Previous reports on patients with SIX1 variants lack imaging data and nonsyndromic AD cases were reported to have no inner ear malformations. In conclusion, we show that a de novo variant in SIX1 in a patient with sensorineural hearing loss leads to cochleovestibular malformations and abnormalities of the CVN, without any other abnormalities. Without proper interventions, severe to profound hearing loss is devastating to both education and social integration. Choosing the correct intervention can be challenging and a molecular diagnosis may adjust intervention and improve outcomes, especially for rare cases.

The article discusses key decision-making factors in pediatric microtia repair, a congenital ear malformation. Surgical options include autologous reconstruction using rib cartilage, porous polyethylene implants, and osseointegrated implants. The commentary emphasizes the need for additional research to improve shared decision-making between parents and children in microtia repair, highlighting the importance of age-appropriate information dissemination and balanced decision-making. The article also compares decision-making factors in microtia reconstruction with those in cleft palate surgery, emphasizing the need for shared decision-making among pediatric patients interested in microtia repair. [Extracted from the article]

Children with a history of temporary conductive hearing loss (CHL) during early development may show long-term impairments in auditory processes that persist after restoration of normal audiometric hearing thresholds. Tones in noise provide a simplified paradigm for studying hearing in noise. Prior research has shown that adults with sensorineural hearing loss may alter their listening strategy to use single-channel energy cues for tone-in-noise (TIN) detection rather than rove-resistant envelope or spectral profile cues. Our objective was to determine the effect of early CHL on TIN detection in healthy children compared to controls. Children ages 4–7 years, with and without a history of CHL due to otitis media with effusion (OME) before age 3 years, participated in a two-alternative forced choice TIN detection task. Audiometric thresholds were normal at the time of testing. Thresholds for detection of a 1000 Hz tone were measured in fixed-level noise and in roving-level noise that made single-channel energy cues unreliable. Participants included 23 controls and 23 with a history of OME-related CHL. TIN thresholds decreased with increasing age across participants. Children in both groups showed similar TIN sensitivity and little or no threshold elevation in the roving-level condition compared to fixed-level tracks, consistent with use of rove-resistant cues. In contrast to older listeners with sensorineural hearing loss, there was no detectable change in TIN sensitivity with roving level for children with a history of OME-related CHL. [ABSTRACT FROM AUTHOR]

Introduction: Pediatric hearing loss can significantly impact speech, language, social, and educational development. Providing access to speech and environmental sounds using amplification devices, such as hearing aids, can help improve developmental outcomes. However, timely rehabilitation and intervention may be delayed due to limited access to resources, further prolonging the adverse effects of childhood hearing loss. The aim of this study was to investigate socioeconomic barriers in time to dispensing hearing aids in a diverse pediatric patient population., Methods: Data from an existing internal database from a tertiary pediatric hospital were analyzed from January 2020 through August 2022 for barriers associated with hearing aid (HA) dispensing delays. Demographic and clinical characteristics were obtained. Multivariate regression and survival analysis statistics were used to identify factors associated with delayed time to dispensing hearing aids., Results: Of the 121 patients who had been appropriately diagnosed and fit, 108 (89.3%) had received hearings aids and 13 (10.7%) had not at the time of the study. Of those who had received HA, time to dispensing was not significantly impacted by sex, race, ethnicity, language, or income level. Insurance was found to be an influencing factor in time to receiving the HA., Conclusion: Factors such as insurance and hearing loss laterality contribute to delays in receiving hearing aids. Identifying these specific barriers and disparities in hearing rehabilitation services will prove vital in facilitating an expedited and equitable pathway to receiving hearing aids., Level of Evidence: 3 Laryngoscope, 135:923-928, 2025., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: This study aimed to assess how race, social vulnerability, and maternal age influence pediatric cochlear implant access and usage., Study Design: Retrospective cohort., Setting: Tertiary Pediatric University Hospital., Methods: This study included individuals aged 0 to 18 who received a cochlear implant at our center between the years 2000 and 2022. Social vulnerability data from 2020 was obtained from the Centers for Disease Control and Prevention., Results: Of the 302 patients included in our study, 43% were black and 50% were white. Patients from the highest to lowest social vulnerability quintiles comprised 31%, 25%, 18%, 10%, and 14% of our sample, respectively. Race was associated with social vulnerability index (SVI) (P < .001), with a mean score of 0.70 (±0.26) and 0.49 (±0.27) for black and white patients, respectively. Later age at hearing loss (HL) diagnosis and cochlear implantation (CI) were associated with more and most vulnerable SVI (P < .05). Delayed diagnosis was also associated with black and other racial groups (P = .041), and adolescent maternal age (P = .03). Greater SVI was associated with less daily cochlear implant usage (P = .004). The most vulnerable patients were more likely to be lost to follow-up (P = .03) despite no difference based on maternal age (P = .59) and insurance status (P = .47)., Conclusion: This study underscores the significance of mitigating disparities in timely diagnosis of HL, consistent CI usage, and appropriate follow-up care. This is a first step toward the formulation of novel strategies aimed at overcoming barriers and developing appropriate intervention programs., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Objective: Explore the experiences of stakeholders within hearing care pathways using a human-centered design process to design a patient navigator (PN) to improve hearing health equity for deaf or hard-of-hearing children., Study Design: A qualitative, prospective, observational study utilizing the Empathize, Define, and Ideate phases of Human-Centered Design., Setting: Academic tertiary children's hospital with interviews over Zoom., Methods: We interviewed 12 hearing care providers, diverse in role and education across the United States, and 10 parents of deaf or hard-of-hearing children, diverse in preferred language and delays in care (mean age at diagnosis of 4.7 months, mean age at intervention of 10.5 months). The main outcomes were themes and subsequent insights that arose from the thematic analysis of interviews, which were used within a design synthesis session., Results: Qualitative analysis revealed 3 themes: Intrinsic Barriers, Extrinsic Barriers, and Areas of Opportunity. Within Intrinsic Barriers, a majority of providers and parents described a grieving process that led to delays. Within Extrinsic Barriers, interviewees expressed challenges navigating multiple deaf or hard-of-hearing care pathways. Within Areas of Opportunity, providers and parents agreed on connecting parents of new hearing loss diagnoses with mentor families to improve the care-seeking experience. Intercoder reliability was achieved (Cohen's κ = 0.73). Findings from the qualitative analysis were incorporated in a synthesis session with diverse stakeholders to design a PN role., Conclusion: Providers and parents agree that the deaf or hard-of-hearing care pathway is difficult to navigate at baseline with delays in accessing care further exacerbated by barriers intrinsic to the family unit., (© 2025 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

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Auditory brainstem response (ABR) testing is the gold standard for diagnosis of hearing loss in children who cannot complete behavioral audiometry. Testing under general anesthesia is often recommended when natural sleep ABR and/or behavioral audiometry are unsuccessful. This study aims to determine which demographic and patient factors serve as barriers to receiving this diagnostic testing. A retrospective chart review from an internal database of patients who underwent ABR testing under anesthesia from 2017 to 2023 was completed. Patient demographics, clinical diagnoses, dates of initial recommendation, and dates of testing were recorded. A total of 395 patients met inclusion criteria, with a median time from initial evaluation to successful ABR under anesthesia of 5.1 months (range 0.1–209 months). This time was significantly higher in patients with public insurance compared to private insurance and in patients with the following medical complexities: cardiac disease, developmental delay, neurologic disease, eye disease, and genetic syndromes not associated with hearing loss. The interval was significantly shorter in patients with abnormal ear anatomy. Patient factors, such as insurance type and certain medical diagnoses, may lead to delayed ABR testing under anesthesia and thus delayed diagnosis and management of hearing loss. This has implications for the timely care and treatment of children with hearing loss. • Children with public insurance wait longer for ABR under anesthesia (70). • There is no difference in wait time based on sex, race, ethnicity, or state (63). • Medically complex children also experience delays in obtaining ABR under anesthesia (85). • Abnormal ear anatomy diagnosis leads to earlier ABR under anesthesia (71). [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To investigate patient‐specific characteristics that independently predict for progressive hearing loss in patients with enlarged vestibular aqueduct (EVA). Utilize multivariable predictive models to identify subgroups of patients with significantly different progression risks. Study Design: Retrospective analysis of patients evaluated at an academic tertiary care center. Cohort included 74 ears of patients with a diagnosis of EVA as defined by the Cincinnati criteria. Methods: Hearing trajectories were characterized, and a Kaplan–Meier estimator was utilized to determine progressive phenotype probabilities across the first 10 years after diagnosis. Cox proportional hazard regression was used to identify patient characteristics that independently altered this probability. Stratified risk groups were delineated from generated nomogram scores. Results: Male gender was associated with a 4.53 hazard ratio for progressive hearing loss (95% confidence interval [CI], 2.53 to 12.59). Each millimeter increase in operculum size was independently associated with an 80.40% increase in expected hazard (95% CI, 40.18 to 120.62). Each dB increase in air pure tone average at time of diagnosis decreased expected hazard by 1.59% (95% CI, −3.02 to −0.17). The presence of incomplete partition type II was associated with a 2.44 hazard ratio (95% CI, 1.04 to 5.72). Risk groups stratified by median nomogram score evidenced the discriminative ability of our model with the progression probability in the high‐risk group being six times higher at 1 year, nearly five times greater at 3 years, and three times greater at 9 years. Conclusions: EVA patient characteristics can be used to predict hearing loss probability with a high degree of accuracy (C‐index of 0.79). This can help clinicians make more proactive management decisions by identifying patients at high risk for hearing loss. Level of Evidence: 4 Laryngoscope, 132:1439–1445, 2022 [ABSTRACT FROM AUTHOR]

A case series with chart review of pediatric patients with immune-mediated sensorineural hearing loss, including sudden sensorineural hearing loss and autoimmune-mediated hearing loss, was performed. Ninety-eight patients with sensorineural hearing loss were reviewed, and 41 patients met the inclusion criteria, which included corticosteroid therapy for a decline in sensorineural hearing. The primary outcome was the corticosteroid response rate: 61% of patients responded. The secondary outcome was a sustained response following multiple courses of corticosteroids for additional hearing loss, which correlated with timely corticosteroid treatment: 57% showed sustained response with multiple treatments, but as anticipated, patients were more likely to respond when treated promptly. Sustained responses fell over time with multiple treatments. These data demonstrate pediatric immune-mediated sensorineural hearing loss is corticosteroid responsive in at a rate similar to their adult counterparts and should be considered for similar treatment approaches. [ABSTRACT FROM AUTHOR]

Children with hearing loss and their families represent a large variety with regard to their auditory, medical, psychological, and family resource characteristics. Despite recent advances, developmental outcomes are still below average, with a significant proportion of variety remaining unexplained. Furthermore, there is a lack of studies including the whole diversity of children with hearing loss. The AChild study (Austrian Children with Hearing Impairment—Longitudinal Databank) uses an epidemiological longitudinal design including all children living in Upper and Lower Austria with a permanent uni- or bilateral hearing loss below the age of 6 years, irrespective of additional disabilities, family language, and family resources. The demographic characteristics of the first 126 children enrolled in the study showed that about half of the children are either children with additional disabilities (31%) and/or children not growing up with the majority language (31.7%) that are usually excluded from comprehensive longitudinal studies. AChild aims for a characterization of the total population of young children with hearing loss including developmental outcomes. Another goal is the identification of early predictors of developmental trajectories and family outcomes. In addition to child-related predictors the examination of family–child transactions malleable by family-centred early intervention is of particular interest. The study is designed as participatory including parent representation atall stages. Measures have been chosen, following other large population-based studies in order to gain comparability and to ensure international data pooling. [ABSTRACT FROM AUTHOR]

Objective: To characterize the prevalence, imaging characteristics, and cochlear implant candidacy of pediatric patients with single-sided deafness (SSD). Methods: An audiometric database of patients evaluated at a large tertiary academic medical center was retrospectively queried to identify pediatric patients (<18 years old) with SSD, defined as severe to profound sensorineural hearing loss in one ear and normal hearing in the other. Medical records of identified patients were reviewed to characterize the prevalence, etiology, and cochlear implant candidacy of pediatric patients with SSD. Results: We reviewed audiometric data obtained from 1993 to 2018 for 52,878 children at our institution. 191 (0.36%) had the diagnosis of SSD. Cochlear nerve deficiency (either hypoplasia or aplasia) diagnosed on MRI and/or CT was the most common etiology of SSD and was present in 22 of 88 (25%) pediatric SSD patients with available imaging data. 70 of 106 (66%) pediatric SSD patients with available imaging had anatomy amenable to cochlear implantation. Conclusions: Pediatric SSD is a rare condition and the most common etiology based on radiology is cochlear nerve deficiency. High resolution imaging of the temporal bone is essential to determine cochlear nerve morphology prior to consideration of cochlear implantation. [ABSTRACT FROM AUTHOR]

Abstract Objectives This article reviews the current role of genetics in pediatric hearing loss (HL). Methods A review of the current literature regarding the genetic basis of HL in children was performed. Results To date, 119 nonsyndromic genes have been associated with HL. There are also hundreds of syndromic causes that have HL as part of the clinical phenotype. Conclusions Identifying HL genes coupled with clinical characteristics (“genotype‐phenotype”) yields a more accurate diagnosis and prognosis. Although the complexity of the auditory apparatus presents challenges, gene therapy is emerging and may be a viable management option in the future.

Objective: Expanded carrier screening (ECS) identified couples at-risk to have a baby with an autosomal recessive genetic condition. Several genes implicated in sensorineural hearing loss (SNHL) are included in prenatal or preconception genetics ECS testing. Early identification of SNHL risk may enable prognostication of hearing loss, early educational intervention, and minimization of unnecessary diagnostic testing. We sought to describe cases where ECS enabled early SNHL-risk identification., Study Design: Retrospective chart review., Setting: Maternal-Fetal Care Center and Otolaryngology department at an academic tertiary hospital., Methods: Medical records of parent-infant dyads with positive ECS results for variants in autosomal recessive SNHL genes were reviewed. Data regarding genetic diagnostic testing, newborn hearing screening, time to HL diagnosis, audiological evaluation, and clinical consultations were compiled., Results: Fifteen pregnant with positive ECS results for SNHL were referred for consultation with a pediatric otolaryngologist and genetic counselor. Generally, these couples were highly educated and adequately insured. 14 had pathogenic variants for GJB2 and 1 for USH2A. Four couples pursued prenatal genetic diagnosis via amniocentesis; 11 couples deferred genetic testing to the postnatal period or waited for initial hearing evaluation. Six babies inherited biallelic GJB2 mutations. Four were found to have SNHL on ABR by age 5 weeks and received follow-up management, 1 had a normal hearing evaluation despite being gene-positive, and 1 was lost to follow-up before hearing evaluation., Conclusions: Carrier screening and confirmatory prenatal or neonatal genetic testing provided considerable lead time for early audiometric testing and appropriate intervention services including hearing aid fitting., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

The purpose of the current study was to assess parent perceptions and experiences of genetic testing, as well as barriers for not undergoing testing in a sample of families of children with hearing loss. A 44-item questionnaire, The Parent Perception of Genetic Testing Questionnaire developed by the research study team was administered. Participants were recruited from a pediatric otolaryngology/audiology practice and social media. A total of 146 parents of children with hearing loss participated. Approximately 47.6% of the children in our sample underwent genetic testing, 44.8% did not, and 7.6% of parents were unsure. For those that did not undergo testing, reasons included: unaware (6.2%), not interested (8.9%), cost (8.9%), time (3.4%), fearful of results (2.7%), and never offered (15.1%). For those that did undergo testing, over half of the parents reported that they did not receive counseling before (55.1%) and 41.7% reported they received counseling after the testing. Furthermore, parents were confused about the results with 18.3% reporting they were Very Confused, 28.3% Somewhat Confused, 20% A Little Confused, and 33.3% Not Confused at All about the variant of uncertain or unknown significance. Notably, less than half of parents (43.4%) remembered what they were told about the mode of inheritance. Overall, our study highlighted the low adoption rate of genetic testing and lack of integration into standard of care for otology/audiology practices. Collaboration between hearing healthcare professionals and geneticists is warranted to determine how to reduce barriers to access while improving pre- and post-counseling., (© 2024 The Author(s). Journal of Genetic Counseling published by Wiley Periodicals LLC on behalf of National Society of Genetic Counselors.)

More than one decade after the introduction of newborn hearing screening in Upper Austria, most children were still older than 6 months at enrolment in early intervention. In this study, under the guidance of health authorities, a revised screening and tracking protocol was developed by a network of early intervention providers and representatives of ENT, obstetrics, and pediatrics, including screening professionals and parents of children with hearing loss. Critical process indicators following internationally recommended benchmarks were defined and collected annually by the health authorities. Due to data protection issues, the data collection system was not personalized. Regular network meetings, case-oriented meetings, and screener training sessions were held. As a result, even without additional costs and within the legal constraints related to data protection in Austria, the proportion of children enrolled in early intervention before 6 months of age was significantly increased from 26% to 81% in two representative birth cohorts before and after the introduction of the new protocol, respectively. The coverage for bilateral screening increased from 91.4 to 97.6% of the total number of births. [ABSTRACT FROM AUTHOR]

Background: The COVID-19 pandemic has affected the world in an unprecedented manner. It has aggravated psychological distress in parents of children with cochlear implants. Continuous use of a speech sound processor is critical for auditory stimulation in children with cochlear implants. However, movement restrictions imposed have affected access to hearing healthcare services. The current study explores the impact of the COVID-19 pandemic on hearing healthcare access for children with cochlear implants. Methods: An online questionnaire survey was conducted among parents of children with cochlear implants. Results: A total of 24 parents responded to the questionnaire. All the respondents reported that COVID-19 has a significant impact on access to hearing health services for their children. Speech processor breakdown and disconnection from the auditory mode of communication had a critical influence on behavioral changes in children. Conclusions: The current study highlights the hurdles faced by the parents in order to access hearing health services for their children. The use of innovative methods such as remote tele-audiology will be the way forward to tackle challenges faced by the parents.

Objectives: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity.Study Design: Meta-analysis.Methods: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis.Results: We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33.Conclusion: This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021. [ABSTRACT FROM AUTHOR]

The indications for cochlear implantation have gradually expanded as advancements in technology have evolved, resulting in improved audiologic outcomes for both adult and children. There remains a significant underutilization of cochlear implant technology in the United States, and recognition of the potential benefits of cochlear implantation for non-traditional indications is critical for encouraging the evolution of candidacy criteria. Adult cochlear implantation candidacy has progressed from patients with bilateral profound sensorineural hearing loss (SNHL) to include patients with greater degrees of residual hearing, single-sided deafness and asymmetric hearing, and atypical etiologies of hearing loss (eg, vestibular schwannoma, Ménière's disease, and otosclerosis). Indications for pediatric cochlear implantation have similarly evolved from children with bilateral severe to profound SNHL to implanting children at a younger age, including those with residual hearing, asymmetric hearing loss, inner ear malformations, as well as cochlear nerve deficiency. In this editorial, the literature investigating cochlear implantation for nontraditional indications is reviewed with an aim to use the best available evidence to encourage the evolution of candidacy criteria. [ABSTRACT FROM AUTHOR]

Objectives: This article reviews the current role of genetics in pediatric hearing loss (HL). Methods: A review of the current literature regarding the genetic basis of HL in children was performed. Results: To date, 119 nonsyndromic genes have been associated with HL. There are also hundreds of syndromic causes that have HL as part of the clinical phenotype. Conclusions: Identifying HL genes coupled with clinical characteristics ("genotype‐phenotype") yields a more accurate diagnosis and prognosis. Although the complexity of the auditory apparatus presents challenges, gene therapy is emerging and may be a viable management option in the future. [ABSTRACT FROM AUTHOR]

We quantified the intensity of early intervention (EI) services allocated to 1262 children who were deaf or hard of hearing (DHH) within a state program and identified factors associated with intervention intensity. Child specific data were collected on children born between 2008 and 2014. Data from Individualized Family Service Plans of children enrolled in Part C EI programming were evaluated for the type and duration of services during their EI enrollment. Associations between EI intensity and child/family variables were examined. Median age of EI enrollment was 5.3 months. The most frequently received services included primary service coordination, specialized DHH service, special instruction, language therapy, and family training; 60% of children received 4 or more different EI services. The median service intensity was 138.1 min per month across all EI years. The factors associated with higher EI intensity included severe hearing loss, bilateral hearing loss and presence of a disability. Children enrolled in EI at later ages received higher intensity of specialized DHH services, suggesting a need to “catch up” due to late acquisition of services. Evaluating EI service intensity broadens our understanding of effective components of state-based programs that support the developmental needs of children who are DHH.

Abstract Background Childhood hearing impairment affects language and cognitive development. Profound congenital sensorineural hearing impairment can be due to an abnormal cochleovestibular nerve (CVN) and cochleovestibular malformations, however, the etiology of these conditions remains unclear. Methods We used a trio‐based exome sequencing approach to unravel the underlying molecular etiology of a child with a rare nonsyndromic CVN abnormality and cochlear hypoplasia. Clinical and imaging data were also reviewed. Results We identified a de novo missense variant [p(Asn174Tyr)] in the DNA‐binding Homeodomain of SIX1, a gene which previously has been associated with autosomal dominant hearing loss (ADHL) and branchio‐oto‐renal or Branchio‐otic syndrome, a condition not seen in this patient. Conclusions SIX1 has an important function in otic vesicle patterning during embryogenesis, and mice show several abnormalities to their inner ear including loss of inner ear innervation. Previous reports on patients with SIX1 variants lack imaging data and nonsyndromic AD cases were reported to have no inner ear malformations. In conclusion, we show that a de novo variant in SIX1 in a patient with sensorineural hearing loss leads to cochleovestibular malformations and abnormalities of the CVN, without any other abnormalities. Without proper interventions, severe to profound hearing loss is devastating to both education and social integration. Choosing the correct intervention can be challenging and a molecular diagnosis may adjust intervention and improve outcomes, especially for rare cases.

Objective: This study aims to explore how genetics evaluation and testing for patients with pediatric onset hearing loss affects their diagnosis and management., Methods: Retrospective chart review was completed for patients with pediatric hearing loss that were evaluated by a genetic counselor from 2008 to 2022 with data entry into a REDCap database. Descriptive statistical analysis was completed., Results: Four hundred twenty-nine patients with pediatric onset hearing loss were evaluated by genetics. Majority of patients presented with bilateral (67 %) and sensorineural (83 %) hearing loss. Genetic testing was recommended for 76 % of patients with pediatric hearing loss evaluated by a genetic counselor with 70 % completing some or all recommended tests. Overall genetic testing diagnostic rate was 34 %, with 41 % of diagnoses syndromic. Yearly trends noted an increasing number of patients evaluated, tests ordered, and subsequently an increased number of diagnoses overtime. For diagnostic results, management recommendations were made for 45 % of patients (35/78) and for 92 % of family members (72/78). This compared to total management recommendations for all patients (82/429, 19 %) and family members (110/429, 26 %)., Conclusion: This study identified a genetic testing diagnostic rate for pediatric hearing loss of 34 % over 14 years. This study notes the beneficial outcomes of patients with hearing loss and their families meeting with a genetic counselor and the importance of collaboration with hearing loss management colleagues. It highlights the value a genetic counselor consult can add to a patient's diagnostic journey, in addition to how genetic testing impacts management for patients and their families., Competing Interests: Declaration of competing interest Sarah Mazzola and Allison Schreiber declare no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

More than one decade after the introduction of newborn hearing screening in Upper Austria, most children were still older than 6 months at enrolment in early intervention. In this study, under the guidance of health authorities, a revised screening and tracking protocol was developed by a network of early intervention providers and representatives of ENT, obstetrics, and pediatrics, including screening professionals and parents of children with hearing loss. Critical process indicators following internationally recommended benchmarks were defined and collected annually by the health authorities. Due to data protection issues, the data collection system was not personalized. Regular network meetings, case-oriented meetings, and screener training sessions were held. As a result, even without additional costs and within the legal constraints related to data protection in Austria, the proportion of children enrolled in early intervention before 6 months of age was significantly increased from 26% to 81% in two representative birth cohorts before and after the introduction of the new protocol, respectively. The coverage for bilateral screening increased from 91.4 to 97.6% of the total number of births.

Objectives: Despite the importance, impact, and prevalence of pediatric hearing loss (HL), there are very few published clinical practice guidelines (CPG) supporting the evaluation and management of pediatric patients with HL. Our objective was to appraise existing CPGs to ensure safe and effective practices.Methods: A literature search was conducted in PubMed, Google Scholar, EBSCO, as well as a manual Google search. Three independent assessors using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) instrument evaluated CPGs related to HL in children. Standardized domain scores were calculated for each guideline.Results: A total of four guidelines met the inclusion criteria and were appraised. Scope and purpose achieved a high median score of 83%. Stakeholder involvement, clarity of presentation, and editorial independence achieved intermediate scores of 67%, 54%, and 50%, respectively. The areas that required most improvement and achieved low scores were rigor of development and applicability, with scores of 22% and 38%, respectively. Based on the AGREE II measures, the four guidelines had domain scores less than 60% for each domain, and without modification no guideline could be recommended.Conclusions: Based on the AGREE II, the qualities of CPGs for pediatric HL have several shortcomings, and the need for a comprehensive CPG remains. Rigor of development and applicability present the greatest opportunities for improvement of these CPGs. Laryngoscope, 130:212-216, 2020. [ABSTRACT FROM AUTHOR]

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P <.001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted. [ABSTRACT FROM AUTHOR]

Abstract Introduction Non-genetic, congenital sensorineural hearing loss (cSNHL) is commonly caused by congenital CMV infection (cCMV). Hearing loss related to cCMV is variable in degree, often progressive, and can affect one or both ears. Objectives We sought to examine the outcomes of DBS testing in California, and the hearing outcomes of cCMV-positive children. Methods This is a retrospective study of patients with SNHL of unknown etiology aged 6 months to 17 years old presenting to a tertiary care pediatric center and evaluated for cCMV by DBS testing. Results 114 children (228 ears) with SNHL of unknown origin were included. 6/114 (5.3%) tested positive for cCMV versus 108/114 (94.7%), who tested negative. None of the cCMV-positive children had symmetric bilateral hearing loss, compared with 56.5% (61/108) of cCMV-negative children (p < 0.05). cCMV-positive children were more likely to have profound SNHL in the worse-hearing ear (5/6 (83%) vs 16/108 (14.9%) of cCMV-negative children, p < 0.001). 86% (5/6) exhibited progressive hearing loss, including progression or new-onset hearing loss in the previously better hearing ear. 3 of the 6 children with cCMV underwent CI. Conclusion A small proportion of patients presenting with SNHL tested positive on DBS. Of cCMV-positive children, most presented with profound hearing loss in the worse-hearing ear, and 50% of cCMV-positive children developed progressive hearing loss in the initially better-hearing ear. Prognostic information afforded by etiologic confirmation of cCMV infection informed decision-making concerning cochlear implantation in these cases. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical effect of comprehensive rehabilitation treatment on hearing impairment in children with secretory otitis media. Methods: 88 children with hearing loss caused by secretory otitis media diagnosed in our hospital from January 2016 to August 2017 were studied. All children were randomly divided into the comprehensive group and the control group, with 44 cases in each group. The comprehensive treatment group was given comprehensive rehabilitation, the control group was given ear massage.The curative effect, changes of air conduction and bone conduction threshold before and after treatment were observed and compared between two groups. Results: The effective rate of combined group(79.55 %) was significantly higher than that of the control group(56.82%, P<0.05). Compared with those before treatment, the air conduction threshold at 0.25-0.8 Hz and the bone conduction threshold at 2.0 and 4.0 kHz were significantly decreased after treatment in both groups(P<0.05). The above indicators were significantly lower in the comprehensive group than those in the control group(P<0.05). Conclusions: Comprehensive rehabilitation therapy, that is, joint sensory training, ear massage, music perception and exercise training, had a good effect on children with hearing loss caused by secretory otitis media, and could improve the children’s air conduction and bone conduction threshold levels. [ABSTRACT FROM AUTHOR]

This study examines how patient demographics impact pediatric sensorineural hearing loss (SNHL) prevalence using the 2016 Kids' Inpatient Database (KID). By analyzing age, gender, race, income, insurance, and region, the study provides insights for early intervention and diagnosis. Multivariate regression analysis reveals associations between these factors and SNHL occurrence., Objective: This study aims to assess the influence of patient demographics on pediatric SNHL prevalence, offering guidance for early detection and intervention strategies., Study Design: Using 2016 KID data, this retrospective analysis investigates how patient factors like age, gender, race, income, insurance, and region relate to SNHL prevalence. Multivariate regression is employed to control for potential confounders., Methods: Data from 6,266,285 patient discharges, including 9997 hearing loss cases, are analyzed. SNHL prevalence is calculated, and demographic variables are examined. Weighted odds ratios and multivariate regression are used to assess associations., Results: The study finds an overall SNHL prevalence of 101.67 cases per 100,000 discharges in 2016. Non-Hispanic White patients show the highest prevalence. Black patients are more likely to be diagnosed, while Hispanic patients are less likely. Multivariate analysis highlights race, income, insurance, region, age, and sex as significant confounders., Conclusion: This study underscores the impact of patient demographics on pediatric SNHL prevalence. Factors like race, income, insurance, and region play a role. These findings aid in risk assessment, early identification, and tailored interventions. Further research can uncover socioeconomic disparities and underlying mechanisms., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Otitis media with effusion (OME) is considered a form of relative sensory deprivation that often occurs during a critical period of language acquisition in children. Animal studies have demonstrated that hearing loss during early development can impair behavioral sensitivity to amplitude modulation (AM), critical for speech understanding, even after restoration of normal hearing thresholds. AM detection in humans with a history of OME-associated conductive hearing loss (CHL) has not been previously investigated. Our objective was to determine whether OME-associated CHL in children ages 6 months to 3 years results in deficits in AM detection in later childhood, after restoration of normal audiometric thresholds. Children ages 4 to 7 years with and without a history of OME-associated CHL participated in an AM detection two-alternative forced-choice task at 8 and 64 Hz modulation frequencies using a noise carrier signal and an interactive touch screen interface. Thirty-four subjects were studied (17 with a history of OME-related CHL and 17 without). Modulation detection thresholds improved with age and were slightly lower (more sensitive) for the 64 Hz modulation frequency for both groups. Modulation detection thresholds of children with a history of OME-associated CHL were higher than control thresholds at 5 years, but corrected to expected levels between ages 6-7. OME-associated CHL results in impaired AM detection, even when measured years after restoration of normal audiometric thresholds. Future studies may shed light on implications for speech and language development and academic success for children affected by OME and associated conductive hearing loss. [ABSTRACT FROM AUTHOR]

Abstract Introduction Family-centered healthcare demands that families provide input regarding the care of their children. Very little is known, however, about how families perceive their experience in different types of multidisciplinary team models, and specifically, in the multidisciplinary setting currently utilized in many pediatric hearing clinics. Methods Quantitative and qualitative parent survey responses were collected and analyzed in a tertiary care pediatric medical center after a one-day multidisciplinary assessment clinical appointment. Questions pertained to information across five domains, including overall experience, diagnosis, treatment plan formulation, additional testing, and resources. Quantitative responses were analyzed descriptively while qualitative responses were evaluated using content analysis to derive themes. Quantitative and qualitative data were evaluated separately and then compared to delineate themes for strengths and weaknesses. Results Overall, high satisfaction was evident in both quantitative and qualitative responses. Results suggested that a one-day multidisciplinary assessment appointment may contribute to parents feeling overwhelmed by information shared and not fully understanding which disciplines are providing care. Analysis revealed a specific area of weakness in our particular setting was inadequate provision of information about functional hearing (e.g., listening socially and academically). Results contributed to a change from a multidisciplinary team model to an interdisciplinary care coordination approach to pediatric hearing healthcare. Conclusions Understanding parent perspectives and expectations is the corner stone of family-centered care and may ultimately influence a child's developmental outcome. A systematic way of evaluating parent perspectives on the clinical process can influence service delivery and help children with hearing loss meet their potential. [ABSTRACT FROM AUTHOR]

Objective This study interviewed youth with unilateral hearing, utilizing their responses to generate candidate items for a condition-specific patient-reported instrument. Study Design Mixed methods, cross-sectional. Setting Tertiary care children's hospital. Participants and Methods Youth with unilateral hearing loss and normal hearing in the contralateral ear were identified and recruited for participation through query of an audiometric database and through hearing loss clinics. Interviews with the youth were qualitatively analyzed to identify common themes and generate items related to functional impact. A multi-institutional expert panel reviewed items with prespecified item selection criteria. Participants rated items for impact on daily life. For preliminary criterion validity assessment, statistical analyses explored correlations between functional scores and type and severity of hearing loss. Results Thirty-nine youth aged 9 to 18 years with unilateral hearing loss participated; 31% used a hearing device. Fifteen youth participated in interviews; thematic analysis, item crafting, and expert panel item review resulted in 41 items. Twenty-six youth responded to the items, reporting low functional scores in the domains of sound localization, ear positioning, and noise environment. They reported better levels of function in carrying out group conversations, focusing on schoolwork, and feeling safe during activities. Multivariate linear regression found that youth scored 0.4 points (or approximately 8%) lower on the functional impact scale with every 20–dB HL increase in pure tone average in the abnormal ear. Conclusion Youth with unilateral hearing loss report functional impact, particularly related to sound localization, ear positioning, and noise environment; therefore, they may benefit from a condition-specific functional assessment instrument. [ABSTRACT FROM AUTHOR]

Objectives: Evaluate usage trends of neural response telemetry (NRT) in cochlear implant centers across the nation and assess reported benefits of intraoperative NRT for pediatric cochlear implant recipients. Study Design: Survey. Study Participants: All US cochlear implant centers (n = 110). Methods: A 15-question multiple-choice survey was distributed electronically to all centers. The survey captured demographic information of all centers, practice patterns surrounding the use of NRT, and the extent to which intraoperative NRT is of benefit. Results: Thirty-two invited participants (29%) completed the survey. A majority of participants reported practicing in an academic center (66%), followed by a hospital setting (19%) and private practice (16%). Seventy-two percent of survey participants reported using NRT for pediatric cochlear implant recipients. Sixty-three percent felt it improved the ability to program at initial activation, and 50% of participants felt that NRT improves satisfaction at initial activation. Conclusion: This study suggests that a majority of surgeons use intraoperative NRT for pediatric cochlear implantation as an additional measure to ensure appropriate electrode placement and improve device activation. Larger studies are needed to better establish the relationship between intraoperative NRT and postoperative outcomes and justify the additional costs associated with intraoperative NRT. [ABSTRACT FROM AUTHOR]