Newborn hearing

Congenital cytomegalovirus (cCMV) infection is a leading cause of sensorineural hearing loss (SNHL) and neurodevelopmental disabilities in children worldwide. Some regions in the United States and Canada have implemented universal newborn screening for cCMV, which requires molecular diagnostic technologies for identifying cCMV, such as PCR testing of newborn dried blood spots (DBS). This study aimed to evaluate the sensitivity of droplet digital PCR (ddPCR) compared to quantitative real-time PCR to detect CMV DNA in newborn DBS...

GJB2 was originally identified in severe, non-syndromic sensorineural hearing loss (SNHL), but was subsequently associated with mild and moderate SNHL. Given the increasing utilisation of genetic testing pre-conceptually, prenatally, and neonatally, it is crucial to understand genotype-phenotype correlations. This study evaluated the nature and frequency of GJB2 variants in an Australian paediatric population with varying degrees of SNHL ascertained through newborn hearing screening. Audiograms from individuals with GJB2 variants and/or a GJB6 deletion (GJB6-D13S11830) were retrospectively reviewed (n = 127)...

PURPOSE: To analyze hyperbilirubinemia as an indicator for the definition of risk protocol in newborn hearing screening (NHS) and in auditory monitoring in full-term and preterm neonates. METHODS: This is an observational, cross-sectional and retrospective study. A total of 554 children born in a public maternity hospital were included and divided into two groups: (G1) with 373 full-terms neonates; (G2) with 181 preterm neonates. Data were collected from the participant's medical records to obtain information regarding the result of the NHS, performed by recording the automated auditory brainstem response (AABR), birth conditions, clinical characteristics, interventions performed, and results of the first test of total bilirubin (TB) and indirect bilirubin (IB) as well as the peak of TB and IB...

BACKGROUND: Cytomegalovirus (CMV) is one of the most important pathogens associated with congenital infection worldwide. Most congenital CMV-infected infants are asymptomatic at birth; however, some can develop delayed sequelae, especially hearing loss. METHODS: This study aimed to investigate the prevalence of congenital CMV infection in a neonatal intensive care unit in a low-income region of Brazil. The objectives extended to identifying associated factors, assessing the clinical status of infected newborns, and undertaking a two-year follow-up to discern potential long-term consequences in the affected infants...

BACKGROUND AND OBJECTIVES: Niemann-Pick type C (NPC) is a rare lysosomal storage disease characterized by hepatosplenomegaly and progressive neurological deterioration due to abnormal intracellular cholesterol transport. Cyclic oligosaccharide 2-hydroxypropyl-β-cyclodextrin (HPBCD) is an effective treatment for NPC; however, few reports have shown its long-term efficacy and safety. To demonstrate long-term efficacy and safety of intrathecal HPBCD (IT-HPBCD) treatment for NPC, we herein reports five patients with NPC treated using IT-HPBCD for 4-11 years...

The prevalence of hearing loss is 0.09-2.3% in low risk neonates, and 0.3-14.1% in the high-risk population. The treatment requires early identification by neonatal hearing screening and early rehabilitation. OAE (oto-acoustic emission) and ABR (Auditory Brain Response) are the two objective tests used to evaluate hearing loss in neonates. OAE tests the biological response of the cochlea to auditory stimuli. ABR tests the auditory pathway. The aim is to estimate hearing loss in high-risk neonates using the Distortion Product Oto- acoustic emission (DP OAE) and to correlate the associated high-risk factors...

OBJECTIVE: The aim of this study is to assess the neonatal click Auditory Brainstem Response (ABR) results in relation to the subsequently determined mean hearing loss (HL) over 1, 2 and 4 kHz, as well as over 2 and 4 kHz. METHODS: Between 2004-2009, follow-up data were collected from Visual Reinforcement Audiometry (VRA) at 1 and 2 years and playaudiometry at 4 and 8 years of newborns who had failed neonatal hearing screening in the well-baby clinics and who had been referred to a single Speech and Hearing center...

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...

INTRODUCTION: At least 60% of cases of severe hearing loss result from genetic factors. In this study genetic screening was carried out for common genetic deafness in women of childbearing age to prevent deafness and birth defects via providing genetic counseling and follow-up services for high-risk families. MATERIAL AND METHODS: In total 60,391 pre-pregnancy/early-gestation women who received treatment in second-level or above hospitals in Weihai from February 2017 to December 2019 were selected...

PURPOSE: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. METHODS: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL...

Under the current meat sheep breeding system, newborn lambs usually live with their mothers until weaning, and in daily management, they often need to be separated from their ewes for a short period due to dehorning, disease treatment, etc. Such short-term separation was considered to be a high-intensity stress for the lambs. This study aimed to explore the effects of 1 h sensory separations on behaviors and the concentration of stress-related indicators of small-tailed Han lambs. Lambs were assigned to four groups: auditory, visual, and tactile separation (AVT) group; visual and tactile separation (VT) group; tactile separation (T) group; and control (C) group...

Introduction Congenital toxoplasmosis (CT), despite being mostly subclinical at birth, can cause disabling disease in the fetus and lead to long-term sequelae. It is an important cause of chorioretinitis in infants and adolescents. Data on postnatal treatment are controversial, and there is a lack of universal guidelines. Methods A cross-sectional study of newborns with suspected CT was conducted between January 2007 and December 2021. Results Seventy-one patients with suspected CT were included. During pregnancy, 64 (90...

During the last decades, a growing number of studies have shown that infections during pregnancy have an important impact on both pregnant women and their fetuses. Our goal was to include newborns from pregnancies with SARS-CoV-2 infection and to investigate the extension of neonatal complications using cardiac, abdominal, and cerebral ultrasonography; hearing testing; and indirect ophthalmoscopy. Likewise, neonates whose mothers were vaccinated against COVID-19 during pregnancy and those from pathology-free pregnancies were examined...

Human cytomegalovirus (CMV) is the leading cause of congenital infection worldwide and the most common cause of non-genetic sensorineural hearing loss. As there is no vaccine or other specific intervention to prevent congenital CMV infection, there is a need to identify maternal and congenital infections with sensitive and specific testing as early as possible. There is no widely accepted practice for screening during pregnancy or in all newborns for identification of possible cases of congenital CMV. Currently, screening during pregnancy is limited to those identified as at risk followed by fetal and/or neonatal testing when congenital infection is suspected...

Cytomegalovirus (CMV) infections exert a substantial impact on the practice of pediatric infectious diseases. Although most infections in children are minimally symptomatic, several populations are at risk for CMV-associated disease, including immunosuppressed children, children with HIV infection, and, most significantly, children with congenital CMV (cCMV) infection. In spite of the ubiquitous nature of CMV infection, few studies have quantified the impact of CMV-associated care in a pediatric outpatient clinic setting...

BACKGROUND: In recent years, neonatal hearing screening (NHS) has gained rapid traction in both developed and developing nations. However, the efficacy of these efforts depends on comprehensive standardization across all screening facets. This study aimed to assess the status and quality of NHS by investigating the knowledge, attitudes, beliefs, and practices of hearing screening practitioners regarding NHS. METHODS: A cross-sectional survey was conducted, and an online questionnaire based on the knowledge-attitude/belief ( A / B )-practice model was distributed to all NHS practitioners in Luzhou, western China...

OBJECTIVE: To assess the efficacy of valganciclovir in infants with hearing loss and clinically inapparent congenital cytomegalovirus infection (cCMV), as there is no consensus on treatment of this group. STUDY DESIGN: A nationwide, non-randomized controlled trial, comparing six weeks of oral valganciclovir to no treatment in infants with cCMV, recruited after newborn hearing screening resulted in referral. The choice whether to treat was left to parents of subjects...

Long-term outcomes of persistent pulmonary hypertension of newborn (PPHN) depend on disease severity, duration of ventilation, and associated anomalies. Congenital diaphragmatic hernia survivors may have respiratory morbidities and developmental delay. The presence of PPHN is associated with increased mortality in hypoxic-ischemic encephalopathy, though the effects on neurodevelopment are less clear. Preterm infants can develop pulmonary hypertension (PH) early in the postnatal course or later in the setting of bronchopulmonary dysplasia (BPD)...

The aim of this study is to comprehensively investigate the prevalence and distribution patterns of three common genetic variants associated with hearing loss (HL) in Chinese neonatal population. Methods : Prior to June 30, 2023, an extensive search and screening process was conducted across multiple literature databases. R software was utilized for conducting meta-analyses, cartography, and correlation analyses. Results: Firstly, our study identified a total of 99 studies meeting the inclusion criteria. Notably, provinces such as Qinghai, Tibet, Jilin, and Heilongjiang lack large-scale genetic screening data for neonatal deafness...

Hearing impairment is the most common sensory deficit. It affects 2 to 3 of 1000 newborns and nearly 50% of adults 75 years of age and older in the United States.1 Idiopathic sudden sensorineural hearing loss (ISSNHL) is characterized by an abrupt hearing loss requiring immediate diagnosis and treatment. Systemic glucocorticoids are widely used as the primary treatment for ISSNHL,2 but no head-to-head comparisons of the effectiveness and risk profiles of high doses over a more commonly used lower dose of glucocorticoids have been conducted to inform standard-of-care practice...