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.

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.

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.

Modulating the Unfolded Protein Response with ISRIB Mitigates Cisplatin Ototoxicity.

Cisplatin is a common chemotherapy drug with a nearly universal side effect of ototoxicity. The cellular mechanisms underlying cisplatin ototoxicity are poorly understood. Efforts in drug development to prevent or reverse cisplatin ototoxicity have largely focused on pathways of oxidative stress and apoptosis. An effective treatment for cisplatin ototoxicity, sodium thiosulfate, is associated with reduced survival in disseminated hepatoblastoma, highlighting the need for more specific drugs. The unfolded protein response (UPR) and endoplasmic reticulum (ER) stress pathways have been shown to be involved in the pathogenesis of noise-induced hearing loss and cochlear synaptopathy in vivo , and these pathways have been implicated broadly in cisplatin cytotoxicity. This study sought to determine whether the UPR can be targeted to prevent cisplatin ototoxicity. Neonatal cochlear cultures and HEK cells were exposed to cisplatin and UPR-modulating drugs, and UPR marker gene expression and cell death measured. Treatment with ISRIB, a drug that activates eif2B and downregulates the pro-apoptotic PERK/CHOP pathway of the UPR, was tested in an in vivo mouse model of cisplatin ototoxicity and well as a head and neck squamous cell carcinoma (HNSCC) cell-based assay of cisplatin cytotoxicity. Cisplatin exhibited a biphasic, non-linear dose-response of cell death and apoptosis that correlated with different patterns of UPR marker gene expression in HEK cells and cochlear cultures. ISRIB treatment protected against cisplatin-induced hearing loss and hair-cell death, but did not impact cisplatin's cytotoxic effects on HNSCC cell viability. These findings demonstrate that targeting the pro-apoptotic PERK/CHOP pathway with ISRIB can mitigate cisplatin ototoxicity without reducing anti-cancer cell effects, suggesting that this may be a viable strategy for drug development.

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.

International Pediatric Otolaryngology Group (IPOG) consensus on scoring of pediatric Drug Induced Sleep Endoscopy (DISE).

OBJECTIVES: To develop consensus statements for the scoring of pediatric drug induced sleep endoscopy in the diagnosis and management of pediatric obstructive sleep apnea. METHODS: The leadership group identified experts based on defined criteria and invited 18 panelists to participate in the consensus statement development group. A modified Delphi process was used to formally quantify consensus from opinion. A modified Delphi priori process was established, which included a literature review, submission of statements by panelists, and an iterative process of voting to determine consensus. Voting was based on a 9-point Likert scale. Statements achieving a mean score greater than 7 with one or fewer outliers were defined as reaching consensus. Statements achieving a mean score greater than 6.5 with two or fewer outliers were defined as near consensus. Statements with lower scores or more outliers were defined as no consensus. RESULTS: A total of 78 consensus statements were evaluated by the panelists at the first survey - 49 achieved consensus, 18 achieved near consensus, and 11 did not achieve consensus. In the second survey, 16 statements reached consensus and 5 reached near consensus. Regarding scoring, consensus was achieved on the utilization of a 3-point Likert scale for each anatomic site for maximal observed obstructions of <50% (Score 0, no-obstruction), ≥ 50% but <90% (Score 2, partial obstruction), and ≥ 90% (Score 3, complete obstruction). Anatomic sites to be scored during DISE that reached consensus or near-consensus were the nasal passages, adenoid pad, velum, lateral pharyngeal walls, tonsils (if present), tongue base, epiglottis, and arytenoids. CONCLUSION: This study developed consensus statements on the scoring of DISE in pediatric otolaryngology using a modified Delphi process. The use of a priori process, literature review, and iterative voting method allowed for the formal quantification of consensus from expert opinion. The results of this study may provide guidance for standardizing scoring of DISE in pediatric patients.

Understanding Barriers to Timely Diagnosis and Intervention Among Immigrant Children With Hearing Loss.

OBJECTIVE: Identify the age at diagnosis and intervention of immigrant and/or non-English-speaking children with hearing loss (HL) and risk factors associated with delays. Identify barriers for non-English-speaking caregivers of deaf/hard-of-hearing children. STUDY DESIGN: Sequential mixed methods. SETTING: Tertiary care center in an urban city. METHODS: The analysis includes descriptive statistics, and 1-way and 2-way analysis of variance of the retrospective chart review. The quantitative study demonstrated foreign-born experienced disparities, so we conducted semistructured interviews on a subset of non-English-speaking families in the cohort that was then thematically analyzed using a human-centered design strategy. RESULTS: We divided 532 children into 3 groups: US-born with English as the preferred language (N = 294), US-born and non-English language preferred (N = 173), and foreign-born (N = 67). The laterality of HL and pure-tone averages were similar among the groups (p = .972 and .071, respectively). Age at diagnosis and time to the intervention were significantly different (39.7, 31.5, 75.8 months, p < .001 and 24.6, 29.2, 48.9 months, p = .001, respectively). Ages at diagnosis and intervention were associated with birthplace (p = .005, p = .0005, respectively) but not preferred language (p = .667, p = .343, respectively). Included in the qualitative interviews were Mandarin- (n = 5), Arabic- (n = 4), and Spanish-speaking families (n = 3). Insights revealed participants' quest for anticipatory guidance and social support, the consequences of cultural stigma, and the complexity of caring for a child with HL in an immigrant family. CONCLUSION: Foreign-born children with HL have significant delays in diagnosis and intervention compared to US-born children. For non-English-speaking parents, the diagnosis of HL presents challenges beyond that of the immigrant experience.

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.

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.

Congenital aural atresia and first branchial cleft anomalies: Cholesteatoma and surgical management.

Objectives: To describe the prevalence and significance of first branchial cleft anomalies in children with congenital aural atresia. Methods: Retrospective cohort study and case series. Patients were included if they had ICD-10 code Q16.0, Q16.1, Q16.9, Q17.2, or Q17.9 in their medical record and were seen at UCSF Benioff Children's Hospital from 2012 to 2020 for aural atresia. Children were categorized as having aural atresia and first branchial cleft anomalies if the presence of a first branchial cleft anomaly was noted in otolaryngology provider notes; otherwise, they were categorized as aural atresia alone. Patients with aural atresia and first branchial cleft anomalies were included in the case series. Results: Among 125 children with congenital aural atresia, 5 (4%) were identified with first branchial cleft anomalies. In all cases, an epithelialized tract was noted to originate from the inferior aspect of the middle ear cleft, exiting the temporal bone in an inferomedial position adjacent to the stylomastoid foramen, with a cutaneous exit point inferior to the expected location of the native ear canal. There was no association with sex, microtia grade, or laterality of atresia; however, children with aural atresia and first branchial cleft anomalies were significantly more likely to have syndromes such as Goldenhar and Treacher Collins (p = .04) than those with aural atresia alone.3/5 (60%) children with aural atresia and first branchial cleft anomalies presented with cholesteatoma compared with 1/120 (0.8%) children with aural atresia alone, a significant difference (p < .001). All four children over the age of two have undergone surgical management. In two of these, excision of the branchial cleft anomaly could be combined with atresiaplasty, with normal hearing results in both cases. Conclusions: Aural atresia can be associated with comorbid anomalies of the head and neck. First branchial cleft anomalies can be suspected based on characteristic clinical appearance and confirmed with computed tomography showing a typical course through the temporal bone. When present in the context of congenital aural atresia, first branchial cleft anomalies are associated with a significantly increased risk for cholesteatoma, often necessitating surgical management with favorable hearing outcomes. Level of evidence: 4.

Outcomes of an Early Childhood Hearing Screening Program in a Low-Income Setting.

IMPORTANCE: Early identification of childhood hearing loss through newborn hearing screening mitigates permanent speech, language, and developmental delays, but many children are lost to follow-up or develop postnatal hearing loss. Early childhood hearing screening programs may help identify these children, but evidence on their outcomes is limited. OBJECTIVE: To assess outcomes from a low-income, preschool-based hearing screening program and risk factors for hearing loss in this population. DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort study of 6820 children aged 2 to 6 years from urban, low-income public preschools who received hearing screening from July 1, 2015, to June 30, 2019, was performed using San Francisco Department of Public Health records. A multivariate logistic regression analyzed risk factors for hearing loss. Data analysis was conducted from January 14, 2020, to April 20, 2021. EXPOSURES: Annual single-visit, 2-tiered screening was implemented with conditioned play pure-tone audiometry (CPA) and distortion product otoacoustic emissions (OAEs). MAIN OUTCOMES AND MEASURES: Rates of successful screening, referred screening, loss to follow-up, and hearing loss. RESULTS: Of 6820 children (age, 2-6 years) screened, 3425 (50.2%) were boys, 15% were White/non-Hispanic, and 48% had English as the primary home language. A total of 403 (5.9%) children were referred for full medical or audiologic evaluation after 2-tiered CPA/OAE screening. Only 24 children were unable to complete both CPA and OAE testing for a screening completion rate of 99.6%. After medical evaluation, 114 of 403 children (28.3%) passed hearing rescreening and 55 (13.6%) were lost to follow-up. The prevalence of conductive hearing loss was 2.9% (n = 195), and the prevalence of sensorineural hearing loss was 0.2% (n = 13). Primary language, race and ethnicity, and sex were not associated with rates of referral or hearing loss. CONCLUSIONS AND RELEVANCE: The findings of this cohort study suggest that preschool-based screening programs can be a useful method to identify early childhood hearing loss and that teacher concerns are associated with final diagnostic hearing status.

Access Challenge Index: A Novel Disparity Measure Predictive of Language Outcomes in Children Who Are Deaf/Hard of Hearing.

OBJECTIVE: To evaluate the effect of demographic disparities on language outcomes in a diverse group of children who are deaf or hard of hearing. STUDY DESIGN: Retrospective cohort study. SETTING: UCSF Benioff Children's Hospital (a tertiary care center). METHODS: Forty-four patients aged <18 years were identified with sensorineural hearing loss managed with a behind-the-ear hearing aid or cochlear implant. Demographic and clinical data were extracted from the medical record. The primary outcome measure was the Preschool Language Scales-5 at least 6 months after intervention. Predictors of language outcome were assessed: hearing level at the time of hearing intervention, cochlear implant status, age of identification and intervention, travel time to site of hearing care, home language, race/ethnicity, insurance type, and Access Challenge Index-a novel measure of educational environment and family support based on the Child Cochlear Implant Profile. Multivariate and univariate analysis assessed predictors for association with intervention and receptive, expressive, and total language scores. RESULTS: Overall 82% of patients had cochlear implants. The median age at hearing intervention was 12 months. The sample was 59% female, 52% non-White, and 61% publicly insured, and 20% had a non-English primary home language. Accounting for multiple demographic and clinical predictors, a high Access Challenge Index score was independently associated with longer time to intervention (P = .01) and poorer language outcomes (P < .001). CONCLUSION: Access Challenge Index-a novel comprehensive measure of educational and family environment-is a strong independent predictor of language outcomes in children who are deaf or hard of hearing.

Tracking Home Language Production and Environment in Children Who Are Deaf or Hard of Hearing.

OBJECTIVE: To use an automated speech-processing technology to identify patterns in sound environments and language output for deaf or hard-of-hearing infants and toddlers. STUDY DESIGN: Observational study based on a convenience sample. SETTING: Home observation conducted by tertiary children's hospital. METHODS: The system analyzed 115 naturalistic recordings of 28 children <3.5 years old. Hearing ability was stratified into groups by access to sound. Outcomes were compared across hearing groups, and multivariable linear regression was used to test associations. RESULTS: There was a significant difference in age-adjusted child vocalizations (P = .042), conversational turns (P = .022), and language development scores (P = .05) between hearing groups but no significant difference in adult words (P = .11). Conversational turns were positively associated with each language development measure, while adult words were not. For each hour of electronic media, there were significant reductions in child vocalizations (ß = -0.47; 95% CI, -0.71 to -0.19), conversational turns (ß = -0.45; 95% CI, -0.65 to -0.22), and language development (ß = -0.37; 95% CI, -0.61 to -0.15). CONCLUSIONS: Conversational turn scores differ among hearing groups and are positively associated with language development outcomes. Electronic media is associated with reduced discernible adult speech, child vocalizations, conversational turns, and language development scores. This effect was larger in children who are deaf or hard of hearing as compared with other reports in typically hearing populations. These findings underscore the need to optimize early language environments and limit electronic noise exposure in children who are deaf or hard of hearing.

Validation of a Hearing-Related Quality-of-Life Questionnaire for Parents and Deaf or Hard-of-Hearing Infants and Toddlers.

OBJECTIVE: To design and validate a hearing-related quality-of-life questionnaire targeted toward parents and deaf or hard-of-hearing infants and toddlers: the Hearing-Related Infant/Toddler and Parent Quality of Life (HIP-QL) questionnaire. STUDY DESIGN: Cross-sectional questionnaire and prospective instrument validation. SETTING: Academic pediatric otolaryngology clinic. METHODS: A 67-question questionnaire developed from constructs of a grounded theory analysis was administered to parents of 31 deaf or hard-of-hearing children and 14 typically hearing children. Questionnaire construct validity, reliability, and discriminant validity were tested. RESULTS: Based on exploratory factor analysis, a 32-item construct composed of developmentally appropriate questions was reduced to a 17-item construct with 4 domains addressing quality of life for both child (auditory/communication behavior, temperament) and parent (management, parent-directed factors). Internal consistency measures were appropriate (Cronbach's alpha = 0.65), and test-retest reliability was high (intraclass correlation coefficient = 0.73). Total HIP-QL scores correlated significantly with related total PedsQL scores (r = 0.57, P < .001). As predicted, parents of children who are deaf or hard of hearing reported significantly lower mean HIP-QL scores but not mean PedsQL scores. HIP-QL was more sensitive than PedsQL for predicting case versus control membership (86.7% vs 76.9%). Multivariable regression confirmed a negative relationship between severity of hearing loss and HIP-QL score after controlling for age, sex, income, and maternal education. CONCLUSIONS: This context-specific questionnaire is the first validated quality-of-life instrument for parents and deaf or hard-of-hearing infants and toddlers. Previously, parental stress and functional disability questionnaires were used as proxies; therefore, this questionnaire has the potential to serve as an important tool for patient- and caregiver-centered outcomes research.

Awake bedside nasal endoscopy for primary management of neonatal dacryocystoceles with intranasal cysts.

OBJECTIVE: To present outcomes of neonatal dacryocystoceles treated through endoscopic intranasal cyst marsupialization (EICM) without general anesthesia and nasolacrimal duct (NLD) probing or irrigation. METHODS: Records of eleven consecutive infants diagnosed with unilateral or bilateral congenital cysts associated with the nasolacrimal system between January 2016 and February 2019 at our institution were retrospectively reviewed. RESULTS: Age at diagnosis ranged from 0 to 56 days, and 45.5% were male. 54.5% had dacryocystitis before surgical intervention. Patients were initially treated with a combination of massage and topical antibiotics; some received parenteral antibiotics if infected. Nearly all patients (90.9%) had one or more intranasal cysts. Of these patients, two underwent lacrimal probing and EICM in the operating room. One underwent EICM only in the operating room. Another patient initially failed bedside NLD probing and subsequently underwent bedside nasal endoscopy and awake EICM. The final six patients underwent EICM without general anesthesia or NLD probing. No complications were noted. Follow-up ranged from 7 to 906 days. Complete resolution was observed in all ten patients who underwent EICM, regardless of anesthesia. CONCLUSIONS: Neonatal dacryocystoceles and/or intranasal cysts are successfully treated at the bedside through nasal endoscopy with simple awake endoscopic intranasal cyst marsupialization. Avoidance of general anesthesia and NLD probing or irrigation would greatly simplify and decrease the cost of dacryocystocele management.

Use of otoacoustic emissions to improve outcomes and reduce disparities in a community preschool hearing screening program.

INTRODUCTION: Hearing loss substantially impacts pediatric development, and early identification improves outcomes. While intervening before school-entry is critical to optimize learning, early-childhood hearing screening practices are highly variable. Conditioned play audiometry (CPA) is the gold standard for preschool hearing screening, but otoacoustic emission (OAE) testing provides objective data that may improve screening outcomes. OBJECTIVES: To compare outcomes of a community-based low-income preschool hearing program before and after implementation of OAE in a single-visit, two-tiered paradigm. We hypothesized that this intervention would reduce referral rates and improve follow-up while maintaining stable rates of diagnosed sensorineural hearing loss. METHODS: We performed a cohort study of 3257 children screened from July 2014-June 2016. Department of Public Health data were analyzed pre- and post-implementation of second-line OAE testing for children referred on CPA screening with targeted follow-up by DPH staff. Primary outcomes included referral rates, follow-up rates, and diagnosis of sensorineural hearing loss. RESULTS: Demographics, pure-tone pass rates, and incidence of newly-diagnosed permanent hearing loss were similar across years. After intervention, overall pass rates increased from 92% to 95% (P = 0.0014), while only 0.7% remained unable to be tested (P<0.0001). 5% of children were unable to be tested by CPA screening but passed OAE testing, obviating further evaluation. Referral rate decreased from 8% to 5% (P = 0.0014), and follow-up improved from 36% to 91% (P<0.0001). Identification of pathology in children with follow-up increased from 19% to over 50%. Further, disparities in pass rates and ability to test seen in Year 1 were eliminated in Year 2. CONCLUSION AND RELEVANCE: In a community setting, implementation of second-line OAE screening for CPA referrals reduced referral rates, increased identification of hearing loss, reduced outcome disparities, and improved follow-up rates. This study provides lessons in how to improve outcomes and reduce disparities in early-childhood hearing screening.

Unilateral Hearing Loss in Youth: Development of Candidate Items for a Condition-Specific Validated Instrument.

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.