Differences in Hearing Devices and Speech Therapy Utilization Between Children With Permanent Unilateral Versus Bilateral Hearing Loss.

OBJECTIVES: In this study, we aimed to describe differences in diagnosis and both auditory and speech/language intervention utilization between children with permanent unilateral hearing loss as compared with bilateral hearing loss. DESIGN: A retrospective cohort study was performed of children evaluated in a multidisciplinary hearing loss clinic at a tertiary care pediatric hospital. Children aged 0 to 18 years with either permanent unilateral or bilateral hearing loss were included. RESULTS: One hundred fourteen children with unilateral hearing loss and 268 children with bilateral hearing loss were studied for a total of 382 children. There were no demographic differences between children with permanent unilateral versus bilateral hearing loss. Rates of newborn hearing screening and referred screening results were similar between those with unilateral and bilateral hearing loss. Despite similar rates of referred newborn hearing screening, those with bilateral hearing loss were diagnosed at a younger age (mean 3.6 years, SD 3.8 years) as compared with those with unilateral hearing loss (mean 5.0 years, SD 4.2 years). Children with unilateral hearing loss had similar severity of hearing loss in their poorer hearing ear as compared with children with bilateral hearing loss, yet they were significantly less likely to be fitted with hearing devices (53% versus 78%) or receive speech/language therapy (36% versus 54%) as compared with children with bilateral hearing loss. Multivariate analysis found that bilateral hearing loss and earlier age of hearing loss diagnosis were associated with hearing device use. CONCLUSIONS: Early diagnosis and intervention for childhood hearing loss have a significant impact on a child's educational success and social relationships. However, little is known about differences in diagnosis and resource utilization between children with permanent unilateral hearing loss versus bilateral hearing loss. Children with unilateral hearing loss were diagnosed at a later age and were less likely to utilize hearing devices or speech/language therapy compared with those with bilateral hearing loss, despite having similar severity of hearing loss in the poorer hearing ear. There is a strong body of evidence that children with unilateral hearing loss have improved hearing outcomes with hearing devices, which suggests there is room for improvement in identifying unilateral hearing loss and providing adequate services to optimize educational success. However, speech therapy is generally implemented in response to language delays. Therefore, children with unilateral loss may have lower rates of language delays as compared with those with bilateral hearing loss, thereby explaining differences in speech therapy utilization.

Barriers to Meeting National Early Hearing Detection and Intervention Guidelines in a Diverse Patient Cohort.

OBJECTIVES: To determine our audiology clinics status in meeting the Joint Committee on Infant Hearing recommended 1-3-6 benchmarks for identification and intervention for congenital sensorineural hearing loss and identify those factors contributing to delay in identification and intervention. DESIGN: This is a retrospective case series. Children with sensorineural hearing loss who underwent auditory brainstem response (ABR) testing, hearing aid evaluation, or cochlear implant mapping at our tertiary pediatric medical center between January 2018 and December 2021 were included. Simple and multiple linear regression analyses were used to identify social, demographic, and health factors associated with primary outcomes, defined as age at hearing loss identification, age at intervention (here defined as amplification start), and interval between identification and intervention. RESULTS: Of 132 patients included, mean age was 2.4 years, 48% were male, and 51% were Hispanic. There was significant association between each Hispanic ethnicity (p = 0.005, p = 0.04, respectively), insurance type (p = 0.02, p = 0.001, respectively), and later age at identification and intervention. In multivariable analyses, Hispanic ethnicity was significantly associated with both delays in identification and intervention (p = 0.03 and p = 0.03, respectively), and public insurance was associated with delays in intervention (p = 0.01). In addition, the total number of ABRs was significantly associated with both older age of identification and intervention (p < 0.001, p < 0.001, respectively). Mediator analysis demonstrated that the effect of ethnicity on age at identification is mediated by the total number of ABRs performed. CONCLUSIONS: A significant association between total number of ABRs and age at identification and intervention for children with hearing loss exists. Hispanic ethnicity was associated with delays in meeting milestones, further mediated by the number of ABRs, providing a potential avenue for intervention in addressing this disparity.

A systematic review of the prevalence of late identified hearing loss in childhood.

OBJECTIVES: The objective of this systematic review was to assess the evidence about the prevalence of permanent hearing loss for children not identified from newborn hearing screening (NHS). DESIGN: Articles were grouped into three categories based on the methodological approach: (1) all participants received diagnostic testing, (2) otoacoustic emission (OAE) or pure tone screening was completed and those not passing were referred for a diagnostic test, and (3) data were retrieved from archival records. Study characteristics, prevalence, and contextual factors were synthesised and narratively described. STUDY SAMPLE: 30 peer-reviewed articles. RESULTS: Prevalence of permanent hearing loss per 1,000 children ranged from 0.32 to 77.87 (M = 7.30; SD = 16.87). Variations in the criteria for inclusion contributed to prevalence differences. Prevalence was higher when unilateral and milder degrees of hearing loss were included, and older children had higher prevalence (M = 13.71; SD = 23.21) than younger children (M = 1.57; SD = 0.86). CONCLUSION: There is scant research on prevalence of childhood hearing loss after NHS that utilised methods to accurately differentiate between permanent and temporary hearing loss. Rigorous research is needed on the prevalence of permanent childhood hearing loss to inform strategies for monitoring, identification, intervention, and management.

Racial and ethnic disparities in genetic testing for hearing loss: a systematic review and synthesis.

Racial/ethnic disparities in the diagnostic efficacy of genetic testing for hearing loss have been described. These disparities may relate to differences in variant classification between different racial/ethnic groups, which may, in turn, derive from disparate representation of these groups in the published literature. We sought to quantify racial/ethnic disparities in the published literature on the human genetics of hearing loss. We conducted a search of PubMed for articles describing single-gene, multiple-gene, or whole-exome sequencing for individuals with sensorineural hearing loss. Data on the included subjects, including race/ethnicity and/or region of origin, a number of subjects tested, and method of testing, were extracted. 1355 populations representing 311,092 subjects from 1165 studies were included. Overall, subjects of European and Asian ancestry were equivalently represented, but those of Latino American, African, and indigenous North American ancestry were significantly underrepresented; over 96% of all subjects in the published literature were European or Asian. Within populations, the majority of subjects derived from a small subset of countries. The observed disparity was greater for multiple-gene and whole-exome sequencing than for single-gene sequencing. These findings illustrate the large disparity in the published literature on the genetics of hearing loss, and demonstrate the need for increased representation of Latino American, African, and indigenous North American populations.

Racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing for sensorineural hearing loss.

Understanding racial and ethnic disparities in diagnostic rates of genetic testing is critical for health equity. We sought to understand the extent and cause of racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing (CGT) for sensorineural hearing loss (SNHL). We performed a retrospective cohort study at two tertiary children's hospitals on a diverse cohort of 240 consecutive pediatric patients (76% publicly insured, 82% non-White) with SNHL of unknown etiology who underwent CGT. Definite and possible genetic diagnoses were assigned for each patient, representing the likelihood of a genetic cause of hearing loss. Associations between diagnostic rates were examined. 3.8 ± 2.1 variants were detected per patient; this frequency did not vary between White/Asian and Hispanic/Black cohorts. Overall, 82% of variants were variants of uncertain significance (VUS). Compared with White and Asian subjects, variants identified among Hispanic and Black children were less likely to be classified as pathogenic/likely pathogenic (15% vs. 24%, p < 0.001), and Hispanic and Black children were less likely to have a definite genetic diagnosis (10% vs. 37%, p < 0.001). The adjusted odds ratio for definite genetic diagnosis in Black and Hispanic children compared with White and Asian children was 0.19. Expanding genetic diagnostic criteria to include predicted deleterious VUSs reduced these disparities between White/Asian and Hispanic/Black children, with comparable molecular diagnostic rates (41% vs. 38%, p = 0.72). However, in silico predictions are insufficiently valid for clinical use. Increased inclusion of underrepresented groups in genetic hearing-loss studies to clinically validate these variants is necessary to reduce racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing.

Prevention and management of hearing loss in patients receiving ototoxic medications.

Following the efforts of patient advocates, the World Health Organization published updated guidelines for management of multidrug-resistant tuberculosis in 2018 that advised against the routine use of ototoxic second-line injectable drugs (amikacin, capreomycin and kanamycin). Although hearing loss is no longer considered an unavoidable harm for patients with multidrug-resistant tuberculosis, ototoxic medications continue to be used for several infectious and oncological disorders around the world. These drugs contribute to more than a half a million cases of hearing loss worldwide annually. Currently, there are no international standards for preventing and managing hearing loss associated with ototoxic medications. We present recent data on the prevention and management of hearing loss related to these drugs and highlight the variability in care across settings. More importantly, we aim to provide an evidence-based framework for evaluating, screening and preventing ototoxicity. Finally, we identify avenues for future research so that patients no longer have to choose between hearing loss and a disease cure. There remain significant gaps in our understanding about optimal screening and treatment of ototoxic hearing loss. Here we aim to inspire future international guidelines to address gaps in ototoxicity care and establish research agendas for eliminating ototoxic medications.

Sous l'impulsion des défenseurs des droits des patients, l'Organisation mondiale de la Santé a publié une version actualisée des lignes directrices relatives à la prise en charge de la tuberculose multirésistante en 2018, qui déconseille l'usage systématique de médicaments ototoxiques injectables de deuxième intention (amikacine, capréomycine et kanamycine). Bien que la perte auditive ne soit plus considérée comme un risque inévitable chez les patients atteints de tuberculose multirésistante, les médicaments ototoxiques continuent à être largement employés pour traiter de nombreuses maladies infectieuses et oncologiques à travers le monde. Ces médicaments sont impliqués chaque année dans plus de la moitié des cas de déficience auditive dans le monde. Il n'existe actuellement aucune norme internationale consacrée à la prévention et à la prise en charge de la perte auditive causée par des médicaments ototoxiques. Dans le présent document, nous exposons les données récentes à ce propos et soulignons la variabilité des soins prodigués d'une région à l'autre. Nous tentons surtout d'établir, à partir d'éléments concrets, un cadre dédié à l'évaluation, au dépistage et à la prévention de l'ototoxicité. Enfin, nous dégageons des pistes pour de futures études, afin que les patients n'aient plus à choisir entre une perte auditive et un remède. D'importantes lacunes subsistent dans notre compréhension du dépistage et du traitement de la perte auditive d'origine ototoxique. Nous espérons inspirer de futures lignes directrices internationales afin d'y remédier et de développer des programmes de recherche pour supprimer les médicaments ototoxiques.

Tras los esfuerzos de los defensores de pacientes, la Organización Mundial de la Salud publicó en 2018 unas directrices actualizadas para el tratamiento de la tuberculosis multirresistente en las que se desaconsejaba el uso rutinario de medicamentos inyectables de segunda línea ototóxicos (amikacina, capreomicina y kanamicina). Aunque la pérdida de audición ya no se considera un daño inevitable para los pacientes con tuberculosis multirresistente, los medicamentos ototóxicos se siguen administrando para varios trastornos infecciosos y oncológicos en todo el mundo. Estos fármacos contribuyen a más de medio millón de casos de pérdida de audición en todo el mundo cada año. En la actualidad, no existen estándares internacionales para prevenir y tratar la pérdida de audición asociada a los medicamentos ototóxicos. En este documento, se presentan datos recientes sobre la prevención y el tratamiento de la pérdida de audición relacionada con estos fármacos y se destaca la variabilidad de la atención en los distintos entornos. Además, se pretende ofrecer un marco basado en la evidencia para evaluar, detectar y prevenir la ototoxicidad. Por último, se identifican las vías de investigación futura para que los pacientes no tengan que elegir entre la pérdida de audición y la cura de la enfermedad. Siguen existiendo importantes deficiencias en el conocimiento del cribado y el tratamiento óptimos de la pérdida de audición ototóxica. En este sentido, se pretende inspirar futuras directrices internacionales para abordar las deficiencias en la atención a la ototoxicidad y establecer programas de investigación para eliminar los medicamentos ototóxicos.

Disparities in Speech and Language Delay Among Children With Aural Atresia.

OBJECTIVE: To describe risk factors for speech and language delay in a diverse population of children with aural atresia. METHODS: Retrospective chart review was performed from 2012 to 2020 at UCSF Benioff Children's Hospital to identify children with aural atresia evaluated for speech, language, or auditory skills delays. Ninety-five children with aural atresia, conductive hearing loss, and assessment of speech, language, or auditory skills delay were included. Demographic and clinical data were analyzed to identify predictors of speech and language delay. Cohort and case-control analyses were performed to determine risk factors for delayed intervention, and for ultimate delays in speech, language, or auditory skills. RESULTS: Children exhibited a wide diversity of race/ethnicity, primary home language, geography, and insurance status. Eighty-nine percent had unilateral aural atresia. Forty-eight percent had delays in speech, language, or auditory skills. Most children used hearing aids (84%), had educational accommodations (84%), and received speech therapy (63%). In a univariate retrospective cohort analysis, public-insured ( p = 0.004), non-English speaking ( p = 0.002) and non-white/non-Hispanic children ( p = 0.007) were found to be significantly less likely to be fit with hearing aids in infancy. Children with delays were fit with hearing aids at later ages. In a multivariate case-control analysis, primary home language was a significant predictor for presence of delays [OR, 3.9 (95% CI: 1.2-13.2), p = 0.03]. CONCLUSIONS: Disparities due to insurance type, primary language, and race/ethnicity are associated with delays in hearing aid fitting for children with aural atresia. Earlier, hearing amplification is correlated with reduced risk for speech, language, and auditory skills delay. These findings can help identify at-risk children for targeted support.

Tongue surgeries for pediatric obstructive sleep apnea: a systematic review and meta-analysis.

OBJECTIVES: To evaluate the international literature for studies reporting outcomes for obstructive sleep apnea (OSA) in children undergoing isolated tongue surgeries. METHODS: Two authors searched from inception through November 14, 2016 in four databases including PubMed/MEDLINE. RESULTS: 351 studies were screened. Eleven studies (116 children) met criteria. Most children were syndromic and had craniofacial disorders, co-morbidities, or other serious medical issues. Surgeries included base-of-tongue (BOT) reduction (n = 114), tongue suspension (n = 1), and hypoglossal nerve stimulation (n = 1). The pre- and post-BOT reduction surgeries decreased apnea-hypopnea index (AHI) from a mean (M) and standard deviation (SD) of 16.9 ± 12.2/h to 8.7 ± 10.6/h (48.5% reduction) in 114 patients. Random effects modeling (109 patients) demonstrated a standardized mean difference for AHI of -0.78 (large magnitude of effect) [95% CI -1.06, -0.51], p value <0.00001. For BOT surgery in 53 non-syndromic children, the AHI decreased 59.2% from 14.0 ± 11.4 to 5.7 ± 6.7/h, while in 55 syndromic children, the AHI decreased 40.0% from 20.5 ± 19.1 to 12.3 ± 18.2/h. BOT reduction improved lowest oxygen saturation from M ± SD of 84.7 ± 7.4-87.9 ± 6.5% in 113 patients. Hypoglossal nerve stimulation and tongue-base suspension are limited to case reports. CONCLUSIONS: Most children undergoing tongue surgeries in the literature were syndromic and had craniofacial disorders, co-morbidities, or other serious medical issues. Children with a body mass index <25 kg/m2 and non-syndromic children have had the most improvement in AHI. The specific type of surgery must be tailored to the patient. Patients with co-morbidities should undergo treatment in centers that are equipped to provide appropriate perioperative care.

Sociodemographic Disparities in Educational Services in Children who are Deaf or Hard of Hearing.

OBJECTIVE: Examine the association between sociodemographic factors and Individualized Education Program (IEP) establishment. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. METHODS: Participants included deaf or hard-of-hearing children who were eligible for an IEP with "deafness" or "hard of hearing" as a primary or secondary disability. Primary outcome measures were time intervals between initial referral for services and parental consent; parental consent to determination of eligibility; and initial referral to eligibility (the sum of the previous 2 intervals). Student's t tests and linear regression were used to examine the association between sociodemographic factors and the primary outcome variables. RESULTS: Of the 88 participants, 51 (58%) were male, 45 (51%) were from underrepresented minority (URM) groups, 35 (40%) spoke a primary language other than English, and 53 (60%) utilized public insurance. IEP establishment was significantly delayed in participants who required an English-language interpreter. Most of the delay occurred in the time between the initial referral and parental consent (mean: 115 vs 37 days, P = .02). There were also significant delays from the time of referral for services to eligibility in URM participants (mean: 159 vs 85 days, P = .04). Significant delays were also associated with Minority Status and Language within social vulnerability index percentile rankings. CONCLUSION: This study found that IEP establishment was delayed in both URM participants and those who required an English-language interpreter. These results highlight the importance of clear communication between the school system and caregivers in the IEP establishment process, particularly with families who require an English-language interpreter or identify as URM.

Risk Factors Associated with Delays in Hearing Loss Identification in Pediatric Patients.

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.

Disparities in Newborn Hearing Screening Outcomes in the United States From 2007 to 2017.

OBJECTIVE: Establishing timely language intervention for children who are deaf or hard of hearing is crucial for their cognitive and language development. Newborn hearing screening (NBHS) programs are now commonplace, but disparities in receipt of support may exist. This study seeks to investigate if states with more diverse populations, less educated mothers, fewer resources, and no legislative mandate of screening have lower rates of milestone completion. STUDY DESIGN: This is a cross-sectional study. SETTING: Data describing screening, identification, and intervention rates from individual state NBHS programs were aggregated by the Centers for Disease Control and Prevention from 2007 to 2017. METHODS: Regression models were fitted to assess associations between these outcomes and state demographic and policy variables. Forest plots from meta-analyses were used to obtain nationwide pooled estimates of the relative risk (RR) of maternal predictors from individual state data. RESULTS: State averages of maternal education level, age, and race/ethnicity were found to be significantly associated with various outcomes. The presence of program funding and legislative state mandate were associated with multiple improved outcomes. Meta-analyses identified increased RRs for most outcomes based on maternal education less than high school, age 19 and below, and non-White race/ethnicity. CONCLUSION: There is evidence of disparities in access to and timing of screening, identification testing, and intervention by various demographic and policy factors at the state level. More research is needed to further explore these relationships and determine how to address existing disparities in order to provide more equitable care.

Teletherapy to address language disparities in deaf and hard-of-hearing children: study protocol for an inclusive multicentre clinical trial.

INTRODUCTION: Children who are deaf or hard-of-hearing (DHH) are at risk for speech and language delay. Language outcomes are worse in DHH children from lower socioeconomic backgrounds, due in part to disparities in access to specialised speech-language therapy. Teletherapy may help improve access to this specialised care and close this language gap. Inclusion of diverse DHH children in prospective randomised clinical trials has been challenging but is necessary to address disparities and pursue hearing health equity. Stakeholder input regarding decisions on study design elements, including comparator groups, masking, assessments and compensation, is necessary to design inclusive studies. We have designed an inclusive, equitable comparativeness effectiveness trial to address disparities in paediatric hearing health. The specific aims of the study are to determine the effect of access to and utilisation of speech-language teletherapy in addressing language disparities in low-income children who are DHH. METHODS AND ANALYSIS: After stakeholder input and pilot data collection, we designed a randomised clinical trial and concurrent longitudinal cohort trial to be conducted at four tertiary children's hospitals in the USA. Participants will include 210 DHH children aged 0-27 months. 140 of these children will be from lower income households, who will be randomised 1:1 to receive usual care versus usual care plus access to supplemental speech-language teletherapy. 70 children from higher income households will be simultaneously recruited as a comparison cohort. Primary outcome measure will be the Preschool Language Scales Auditory Comprehension subscale standard score, with additional speech, language, hearing and quality of life validated measures as secondary outcomes. ETHICS AND DISSEMINATION: This study was approved by the Institutional Review Boards of the participating sites: the University of California, San Francisco (19-28356), Rady Children's Hospital (804651) and Seattle Children's Hospital (STUDY00003750). Parents of enrolled children will provide written informed consent for their child's participation. Professional and parent stakeholder groups that have been involved throughout the study design will facilitate dissemination and implementation of study findings via publication and through national and regional organisations. TRIAL REGISTRATION NUMBER: NCT04928209.

Geographic, Sociodemographic, and Clinical Factors Associated With Parental Self-Efficacy in Pediatric Patients With Hearing Loss.

OBJECTIVE: To identify geographic, sociodemographic, and clinical factors associated with parental self-efficacy in a diverse cohort of deaf or hard-of-hearing (DHH) children. STUDY DESIGN: Cross-sectional study. SETTING: Tertiary children's hospital. METHODS: Four hundred forty parents of DHH children aged 0 to 17 completed the 25-item Scale of Parental Involvement and Self-Efficacy (SPISE) survey from 2014 to 2022. Residential addresses were geocoded and assigned Area Deprivation Index and Social Vulnerability Index rankings, and univariable and multivariable analyses were conducted using sociodemographic and clinical variables, including sex, race/ethnicity, insurance type, survey language, age at the survey, comorbidities, newborn hearing screening results, and hearing loss laterality and severity. RESULTS: Compared to English and Spanish-speaking parents, Chinese-speaking parents were associated with overall lower parental self-efficacy and involvement (regression coefficient = -0.518, [-0.929, -0.106]), Cohen's d = 0.606) and lower scores on items related to their ability to affect multiple aspects of their child's development and expression of thoughts as well as competency in checking and putting on their child's sensory device. Across univariable and multivariable analyses, besides Chinese language, all other sociodemographic, clinical, and geographic variables were not associated with SPISE score. CONCLUSION: To achieve the best patient outcomes, care teams can use the SPISE to evaluate parental self-efficacy and provide targeted support to parents at risk for having lower knowledge and confidence scores about critical skills necessary to facilitate their child's auditory access and language development. Notably, this study found similar reports of parental efficacy across various sociodemographic, clinical, and geographic variables but significantly lower SPISE scores in Chinese-speaking families.

Synaptic ribbon dynamics after noise exposure in the hearing cochlea.

Moderate noise exposure induces cochlear synaptopathy, the loss of afferent ribbon synapses between cochlear hair cells and spiral ganglion neurons, which is associated with functional hearing decline. Prior studies have demonstrated noise-induced changes in the distribution and number of synaptic components, but the dynamic changes that occur after noise exposure have not been directly visualized. Here, we describe a live imaging model using RIBEYE-tagRFP to enable direct observation of pre-synaptic ribbons in mature hearing mouse cochleae after synaptopathic noise exposure. Ribbon number does not change, but noise induces an increase in ribbon volume as well as movement suggesting unanchoring from synaptic tethers. A subgroup of basal ribbons displays concerted motion towards the cochlear nucleus with subsequent migration back to the cell membrane after noise cessation. Understanding the immediate dynamics of synaptic damage after noise exposure may facilitate identification of specific target pathways to treat cochlear synaptopathy.

Noise induces intercellular Ca 2+ signaling waves and the unfolded protein response in the hearing cochlea.

Exposure to loud noise is a common cause of acquired hearing loss. Disruption of subcellular calcium homeostasis and downstream stress pathways in the endoplasmic reticulum and mitochondria, including the unfolded protein response, have been implicated in the pathophysiology of noise-induced hearing loss. However, studies on the association between calcium homeostasis and stress pathways has been limited due to limited ability to measure calcium dynamics in mature-hearing, noise-exposed mice. We used a genetically encoded calcium indicator mouse model in which GcAMP is expressed specifically in hair cells or supporting cells under control of Myo15Cre or Sox2Cre, respectively. We performed live calcium imaging and UPR gene expression analysis in 8-week-old mice exposed to levels of noise that cause cochlear synaptopathy (98 db SPL) or permanent hearing loss (106 dB SPL). UPR activation occurred immediately after noise exposure and was noise dose-dependent, with the pro-apoptotic pathway upregulated only after 106 dB noise exposure. Spontaneous calcium transients in hair cells and intercellular calcium waves in supporting cells, which are present in neonatal cochleae, were quiescent in mature-hearing cochleae, but re-activated upon noise exposure. 106 dB noise exposure was associated with more persistent and expansive ICS wave activity. These findings demonstrate a strong and dose-dependent association between noise exposure, UPR activation, and changes in calcium homeostasis in hair cells and supporting cells, suggesting that targeting these pathways may be effective to develop treatments for noise-induced hearing loss.

Probing versus primary nasal endoscopy for the treatment of congenital dacryocystoceles.

PURPOSE: To compare outcomes and complications of three surgical techniques for the treatment of congenital dacryocystoceles: nasolacrimal probing and irrigation (P+I), P+I plus nasal endoscopy (NE) with intranasal cyst marsupialization, and primary NE with intranasal cyst marsupialization. METHODS: The medical records of children ≤2 years of age at a single academic center with a diagnosis of dacryocystocele from 2012 to 2022 were retrospectively identified and reviewed. The primary outcome was resolution of the dacryocystocele (ie, elimination of the medial canthal mass and resolution of tearing or discharge) after a single procedure ("primary success"). Surgical techniques were compared using exact logistic regression. RESULTS: Of 54 patients, 21 (39%) underwent P+I, 23 (43%) underwent P+I plus nasal endoscopy, and 10 (18%) underwent primary NE. Primary success was 76% for P+I and 100% for the other two cohorts. Most patients (89%) who underwent P+I received general anesthesia compared with none who underwent primary nasal endoscopy. Most complications were related to the use of general anesthesia, with a complication rate of 10% for P+I, 48% for P+I plus NE, and 0% for primary NE. Most P+I procedures required hospital admission compared to half of primary NE procedures. CONCLUSIONS: In our study cohort, primary NE provided good outcomes and was associated with a lower complication rate than P+I with or without NE.

Congenital Cytomegalovirus Testing Outcomes From the ValEAR Trial.

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.

Hearing Impairment and School Engagement Outcomes in US Children.

Importance: Ensuring appropriate school engagement for deaf or hard of hearing (DHH) children in the US is important for improving their long-term outcomes as they grow into adults. Objective: To examine the associations between hearing loss (HL), its sequelae (speech and/or language disorders, behavioral and/or conduct problems, and neuropsychiatric and/or learning disorders), and various school engagement measures among school-aged children in the US. Design, Setting, and Participants: This cross-sectional study combined data from the nationally representative 2016-2021 National Survey of Children's Health. A total of 155 178 randomly selected children (weighted, approximately 49 340 700 children) aged 6 to 17 years with a parent or caregiver who responded to an address-based survey by mail or online were included. All analyses were weighted to account for the probability of selection and nonresponse and to reflect population-based estimates representative of all noninstitutionalized school-aged US children and adolescents residing in housing units. Main Outcomes and Measures: Diverse school engagement measures, including extracurricular participation in sports, clubs, paid work, volunteer work, and organized lessons, as well as educational performance variables, including missed school days, not caring about doing well in school, not doing required homework, grade repetition, and parent or guardian contacted by school. Results: Of the estimated 49 340 700 children aged 6 to 17 years (41.1% aged 6-10 years; 51.1% male; 54.7% without underrepresented minority status), an estimated 1.4% (95% CI, 1.2%-1.5%) were reported by their parent or guardian to have deafness or hearing problems. Having DHH status was associated with significantly worse outcomes on 8 of 10 school engagement measures (eg, participation in sports: adjusted odds ratio [AOR], 0.75 [95% CI, 0.60-0.93]; missed school days: AOR, 2.98 [95% CI, 2.21-4.00]), even after adjustment for age, sex, underrepresented minority status, highest educational level of parent or guardian, federal poverty level of the household, and primary language in the household. Moreover, although subgroup analyses of DHH children with and without HL sequelae revealed significant differences (speech and/or language disorder: AOR, 5.83 [95% CI, 4.31-7.89]; behavioral and/or conduct problem: AOR, 2.75 [95% CI, 2.10-3.60]; neuropsychiatric and/or learning disorder: AOR, 3.06 [95% CI, 2.39-3.91]), HL sequelae only partially mediated the associations between these disparities. Conclusions and Relevance: In this cross-sectional study, DHH status itself may have been the primary factor directly associated with school engagement disparities. These findings suggest the need for greater emphasis on educational accommodations and support for hearing status itself, independent of the presence or absence of HL sequelae.

Impact of Genetic Testing on Hearing Interventions.

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.

Impact of Sociodemographic Disparities on Language Outcomes After Cochlear Implantation in a Diverse Pediatric Cohort.

OBJECTIVE: We examined how sociodemographic and audiologic factors affect receptive and expressive language outcomes in children with cochlear implantation. STUDY DESIGN: Retrospective cohort study. SETTING: A hearing loss (HL) clinic at a tertiary center. METHODS: Sociodemographic variables, HL characteristics, age at implantation, and receptive language scores (Preschool Language Scale and the Clinical Evaluation of Language Fundamentals) were collected from patients with congenital HL who received their first implant by 4 years old after January 1, 2007. t Tests, linear regression, Mann-Whitney, Cohen's d, and mediation analysis were used for descriptive statistics and hypothesis testing. RESULTS: Among 79 patients, 42 (53%) were females, 44 (56%) under-represented minorities, and 56 (71%) had public insurance. At least 1 year after implantation, the median receptive language score was 69 (range 50-117). Females (p = .005), having private insurance (p = .00001), having a Cochlear Implant Profile score below 4 (p = .0001), and receiving their implant at or before 12 months of age (p = .0009) were significantly associated with improved receptive language outcomes. Insurance type had a significant effect on receptive language outcomes, independent from age at first implantation (total effect: coef = -13.00, p = .02; direct effect: coef = -12.26, p = .03; indirect effect: coef = -0.75, p = .47). Sociodemographic variables had large effect sizes, with the Cochlear Implant Profile score having the largest effect size (d = 1.3). CONCLUSION: Sociodemographic factors have a large impact on receptive language outcomes. Public insurance is associated with worse receptive language, not mediated by later age at implantation, suggesting that other factors primarily impact language outcomes in publicly insured children with cochlear implants.