Noise induces Ca2+ signaling waves and Chop/S-Xbp1 expression 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 have been limited due to limited ability to measure calcium dynamics in mature-hearing, noise-exposed mice.

Modulating the unfolded protein response with ISRIB mitigates cisplatin ototoxicity.

Cisplatin is a commonly used chemotherapy agent with a nearly universal side effect of sensorineural hearing loss. 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.

Medical Home Care for Children with Deafness and Hearing Problems.

Background And Objectives: Since 2001, the American Academy of Pediatrics (AAP) has advocated for improving the effectiveness of early hearing screening, diagnosis, and intervention (EHDI) and reducing hearing health disparities through the medical home model of care (MHMOC). However, the scope of the MHMOC and its components and potential disparities among US children with deafness or hearing problems (DHH) have not been identified.

Methods: Data from the 2016 to 2020 National Survey of Children's Health (N = 174 551) were analyzed, including 12 121 children with DHH, 171 523 children without DHH, and 38 722 with other special health needs (SHCN).

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.

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.

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.

Noise induces intercellular Ca 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.

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.

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.

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.

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.

TAD evolutionary and functional characterization reveals diversity in mammalian TAD boundary properties and function.

Topological associating domains (TADs) are self-interacting genomic units crucial for shaping gene regulation patterns. Despite their importance, the extent of their evolutionary conservation and its functional implications remain largely unknown. In this study, we generate Hi-C and ChIP-seq data and compare TAD organization across four primate and four rodent species and characterize the genetic and epigenetic properties of TAD boundaries in correspondence to their evolutionary conservation.

TMTC4 is a hair cell-specific human deafness gene.

Transmembrane and tetratricopeptide repeat 4 (Tmtc4) is a deafness gene in mice. Tmtc4-KO mice have rapidly progressive postnatal hearing loss due to overactivation of the unfolded protein response (UPR); however, the cellular basis and human relevance of Tmtc4-associated hearing loss in the cochlea was not heretofore appreciated. We created a hair cell-specific conditional KO mouse that phenocopies the constitutive KO with postnatal onset deafness, demonstrating that Tmtc4 is a hair cell-specific deafness gene.

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.

Modulating the Unfolded Protein Response with ISRIB Mitigates Cisplatin Ototoxicity.

Cisplatin is a commonly used chemotherapy agent with a nearly universal side effect of sensorineural hearing loss. 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.

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.

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.

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.

TAD Evolutionary and functional characterization reveals diversity in mammalian TAD boundary properties and function.

Topological associating domains (TADs) are self-interacting genomic units crucial for shaping gene regulation patterns. Despite their importance, the extent of their evolutionary conservation and its functional implications remain largely unknown. In this study, we generate Hi-C and ChIP-seq data and compare TAD organization across four primate and four rodent species, and characterize the genetic and epigenetic properties of TAD boundaries in correspondence to their evolutionary conservation.

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.

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.

Factors associated with congenital cytomegalovirus infection detected by dried blood spot testing in children with hearing loss.

Objective: Congenital cytomegalovirus (cCMV) infection in infants leads to an increased risk of developing sensorineural hearing loss (SNHL), even if they are asymptomatic at birth. There are currently no national guidelines for universal screening for CMV, placing children with cCMV at a high risk for unidentified and untreated HL, which in turn places them at greater risk for lasting impacts on quality of life and cognitive and behavioral abilities. We sought to describe the sociodemographic and hearing loss characteristics of children with HL due to cCMV.