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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.)

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., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

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., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Stephen Leong, BA: None. Kristy Medina, MPH: None. Patricia J. Peretz, MPH: None. Maria I. Olmeda-Jenkins, MA, CCC-A: None. Melissa A. Oliver, MED: None. Megan Kuhlmey, AuD, CCC-A: None. Stacey A. Bernstein, MS, CCC-SLP: None. Fajah Ferrer: None. Luz Adriana Matiz, MD: None. Anil K. Lalwani, MD: Medical Advisory Board of Spiral Therapeutics.

Objective: To evaluate factors influencing the diagnostic yield of comprehensive gene panel testing (CGPT) for hearing loss (HL) in children and to understand the characteristics of undiagnosed probands., Study Design: This was a retrospective cohort study of 474 probands with childhood-onset HL who underwent CGPT between 2016 and 2020 at a single center. Main outcomes and measures included the association between clinical variables and diagnostic yield and the genetic and clinical characteristics of undiagnosed probands., Results: The overall diagnostic yield was 44% (209/474) with causative variants involving 41 genes. While the diagnostic yield was high in the probands with congenital, bilateral, and severe HL, it was low in those with unilateral, noncongenital, or mild HL; cochlear nerve deficiency; preterm birth; neonatal intensive care unit admittance; certain ancestry; and developmental delay. Follow-up studies on 49 probands with initially inconclusive CGPT results changed the diagnostic status to likely positive or negative outcomes in 39 of them (80%). Reflex to exome sequencing on 128 undiagnosed probands by CGPT revealed diagnostic findings in 8 individuals, 5 of whom had developmental delays. The remaining 255 probands were undiagnosed, with 173 (173/255) having only a single variant in the gene(s) associated with autosomal recessive HL and 28% (48/173) having a matched phenotype., Conclusion: CGPT efficiently identifies the genetic etiologies of HL in children. CGPT-undiagnosed probands may benefit from follow-up studies or expanded testing., Competing Interests: Declaration of Competing Interest This work was partially supported by endowed funds in the Roberts Individualized Medical Genetics Center (RIMGC). The authors declare no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

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

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., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Hearing loss is associated with reduced quality and quantity of auditory input, and difficulty in cognitive and language skills. This study aimed to investigate the relationship between language, working memory, and rapid naming skills in children with mild to moderate sensorineural hearing loss (MMHL)., Methods: Twenty children with MMHL with the same auditory experience and demographical conditions using bilateral hearing aids were included. Verbal memory subscale of the Working Memory Scale (WMS), consisting of verbal short-term memory (V-STM) and verbal working memory (V-WM) subtests, was administered to all participants. They also completed rapid automatized naming tasks and standardized language measures., Results: The language score showed a moderate and significant correlation with verbal memory (VM) score (p = 0.03, r = 0.48) and a moderate and negative correlation with rapid automatized naming (RAN) duration (p = 0.06, r = -0.61). The VM score showed a moderate and significant negative correlation with RAN duration (p = 0.01, r = -0.67). The language level has a strong and significant positive correlation with V-STM (p = 0.007, r = 0.60), V-WM (p = 0.009, r = 0.58), and VM level (p = 0.003, r = 0.65). VM subtests levels have a strong and significant positive correlation with each other (p = 0.017, r = 0.53). RAN level has a strong and significant negative correlation with VM (p = 0.001, r = -0.70), V-WM (p = 0.001, r = -0.76), V-STM (p = 0.001, r = -0.69), and language level (p = 0.001, r = -0.77)., Conclusion: The results suggest that the language, verbal working memory, and rapid naming skills of children with MMHL are closely related. It is recommended that the relationship between verbal short-term memory, verbal working memory, rapid naming skills, and language skills should be considered in therapeutic and educational settings. To the best of our knowledge, this is the first study to examine the relationships between verbal-short-term -working memory, duration of rapid automatized naming, and language skills in children with MMHL., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objectives: To reveal hearing loss patterns in patients with enlarged vestibular aqueduct (EVA) syndrome according to demographic and clinical characteristics., Methods: A retrospective, longitudinal study design was utilized to identify patients with EVA. Ears of patients were categorized into one of four cohorts: progressive fluctuating, progressive non-fluctuating, stable fluctuating, and stable non-fluctuating patterns. Pairwise and group comparisons were performed with non-parametric tests to assess vestibular aqueduct (VA) morphology, clinical, and demographic variables between hearing loss pattern cohorts. Rates of hearing loss in the subgroups were determined utilizing a mixed linear effects model., Results: 44 patients (25 female, 19 male, median diagnosis age: 8.06 years) met inclusion criteria. 16 individuals demonstrated unilateral EVA and 29 had bilateral EVA, resulting in 74 total ears with EVA. Amongst the four cohorts, differences in operculum widths amongst groups were statistically significant (p = 0.049) while VA midpoint widths were not (p = 0.522). Progressive hearing loss ears without fluctuations demonstrated a 3.20 dB per year (p < 0.001) progression while progressive hearing loss ears with fluctuations reported a rate of 3.52 dB loss per year (p < 0.001)., Conclusion: Hearing fluctuations occur similarly in EVA patients with stable and progressive hearing loss. With the exception of increased rates of hearing loss progression for fluctuating progressive hearing loss patients, vestibular aqueduct morphology, demographic, and clinical characteristics commonly reported are likely not strong predictors for whether patients will or will not experience fluctuating patterns of hearing loss., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Introduction: As the most common neonatal sensory disorder in the USA, infant hearing loss has an incidence of 1.7 per 1000 births. The consequences of delayed diagnosis and failure to obtain timely intervention include significant communication impairment and negative socioeconomic effects. Early Hearing Detection and Intervention (EHDI) national standards dictate that all infants should be screened and diagnosed by 3 months of age and there is a need for interventions that promote adherence to timely diagnosis. Patient navigation (PN) has been shown to be efficacious to decrease non-adherence with infant hearing diagnostic care; however, PN has yet to be tested in diverse communities or implemented into real-world settings., Methods and Analysis: The proposed research is a community-engaged, type 1 hybrid effectiveness-implementation trial of a PN intervention aimed at decreasing infant hearing diagnosis non-adherence after failed newborn hearing screening, delivered in state-funded EHDI clinics. Guided by our community advisory board and partners, we aim to (1) test the effectiveness of PN to decrease non-adherence to receipt of infant hearing diagnosis within 3 months after birth using a stepped-wedge trial design, (2) investigate implementation outcomes and factors influencing implementation and (3) determine the cost-effectiveness of PN from the perspective of third-party payers. The study will be conducted from April 2019 until March 2024., Ethics and Dissemination: This protocol was approved by the University of Kentucky Institutional Review Board. Although all research involving human subjects contains some risk, there are no known serious risks anticipated from participating in this study. We will seek to disseminate our results in a systematic fashion to patients, key stakeholder, policymakers and the scientific community. Our results will impact the field by partnering with communities to inform the scale-up of this innovative patient supportive intervention to create efficient and effective EHDI programmes and maximise public health impact., Trial Registration Number: Clinicaltrials.gov (Pre-results phase): NCT03875339., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

There is mounting evidence to support the premise that children with hearing loss (CHL) are at increased risk for listening-related fatigue and its associated sequelae. This article provides an overview of the construct of listening-related fatigue in CHL, its importance, possible academic and psychosocial consequences, and recommendations for the identification and management of fatigue associated with pediatric hearing loss., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective To systematically review and quantify current evidence regarding the association of GJB2 mutation status with outcomes of pediatric cochlear implantation. Data Sources PubMed, Embase, and the Cochrane Library were searched for "GJB2,""pediatric hearing loss," and "cochlear implantation" and their synonyms, with no language restrictions, until December 2, 2015. Review Methods Studies were included that investigated the status of GJB2 mutation and its predictive value for outcomes of pediatric cochlear implantation. Speech recognition scores, Infant-Toddler Meaningful Auditory Integration Scale, Speech Intelligibility Rating, and Categorized Auditory Performance were pooled using weighted mean differences, and a 95% confidence interval. Results Eighteen studies met the inclusion criteria. The differences between GJB2-related deafness and non- GJB2-related deafness due to unidentified causes and other types of genetic deafness without additional disabilities were not statistically significant ( P = .15 and P = .30, respectively); however, the difference between GJB2-related deafness and acquired hearing loss due to environmental etiologies was statistically significant and favored GJB2-related deafness ( P = .03). Conclusion GJB2-related deafness leads to significantly better cochlear implantation outcomes when compared with acquired deafness caused by environmental etiologies. However, GJB2 mutation is not associated with a significantly better prognosis when compared with those whose deafness results from either nonsyndromic hearing loss of unknown origin or other types of genetic mutations in the absence of other neurologic deficits.

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.

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]

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]

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 FROM AUTHOR]

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation. [ABSTRACT FROM AUTHOR]