Deaf

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OBJECTIVES: (i) To identify peer reviewed publications reporting the mental and/or physical health outcomes of Deaf adults who are sign language users and to synthesise evidence; (ii) If data available, to analyse how the health of the adult Deaf population compares to that of the general population; (iii) to evaluate the quality of evidence in the identified publications; (iv) to identify limitations of the current evidence base and suggest directions for future research. DESIGN: Systematic review...

PURPOSE: To examine effects of aerobic exercise interventions on brain via the structural Magnetic Resonance Imaging (MRI), as well as functional change during working memory (WM) task using fMRI in deaf children. METHOD: The study applied a cluster randomized controlled design. Twelve deaf children in the intervention group were required to complete an eleven-week aerobic exercise intervention, while other twelve age and gender matched deaf children in the control group were required to keep their normal daily life...

Introduction  Cochlear implant (CI) activation usually takes place at ∼ 30 days postoperative (PO). In our service, CI surgery is performed with local anesthesia and sedation, so activation is possible with the patient's cooperation, immediately after the CI surgery, still in the operating room (OR). Objective  The objective of the present study was to provide the patient with hearing experience with the CI and to assess auditory perception immediately after surgery while still in the OR, as well as to compare impedance telemetry (IT), neural response telemetry (NRT), and comfort (C) level at two moments: in the OR and at the definitive activation, ∼ 30 days PO...

OBJECTIVES: Postlingually deaf adults with cochlear implants (CIs) have difficulties with perceiving differences in speakers' voice characteristics and benefit little from voice differences for the perception of speech in competing speech. However, not much is known yet about the perception and use of voice characteristics in prelingually deaf implanted children with CIs. Unlike CI adults, most CI children became deaf during the acquisition of language. Extensive neuroplastic changes during childhood could make CI children better at using the available acoustic cues than CI adults, or the lack of exposure to a normal acoustic speech signal could make it more difficult for them to learn which acoustic cues they should attend to...

Congenital deafness critically challenges the ability of affected children to acquire spoken language and normal social interactions. The clinical study by Lv et al. recently published in The Lancet1 presents a major breakthrough as the first successful causative treatment aiming to restore natural hearing in deaf children.

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...

Background: The vestibular phenotypes of patients with genetic hearing loss are poorly understood. Methods: we performed genetic testing including exome sequencing and vestibular function tests to investigate vestibular phenotypes and functions in patients with genetic hearing loss. Results: Among 627 patients, 143 (22.8%) had vestibular symptoms. Genetic variations were confirmed in 45 (31.5%) of the 143 patients. Nineteen deafness genes were linked with vestibular symptoms; the most frequent genes in autosomal dominant and recessive individuals were COCH and SLC26A4 , respectively...

BACKGROUND: Congenital hearing loss (HL), one of the most common paediatric chronic conditions, significantly affects speech and language development. Its early diagnosis and medical intervention can be achieved via newborn hearing screening. However, data on the prevalence and aetiology of congenital HL in infants who fail newborn hearing screening are limited. METHODS: The sample population included 153 913 infants who underwent newborn hearing screening, and the prevalence of congenital HL, defined as moderate to profound bilateral HL (BHL) or unilateral HL (UHL) (≥40 dB HL), in one prefecture of Japan was measured to minimize the loss-to-follow-up rate, a common factor affecting the screening procedure...

OBJECTIVES: This study aimed to investigate the effects of an adhesive bone conduction device (aBCD) in children with congenital single-sided deafness (SSD). Specifically, we examined whether the aBCD elicits improvement in the speech perception ability of children with congenital SSD and whether using this device would adversely affect the horizontal localisation abilities of these children. METHODS: Thirteen school-aged children with SSD and seven children with Normal Hearing (NH) were included in this study...

Sign languages (SLs) are expressed through different bodily actions, ranging from re-enactment of physical events (constructed action, CA) to sequences of lexical signs with internal structure (plain telling, PT). Despite the prevalence of CA in signed interactions and its significance for SL comprehension, its neural dynamics remain unexplored. We examined the processing of different types of CA (subtle, reduced, and overt) and PT in 35 adult deaf or hearing native signers. The electroencephalographic-based processing of signed sentences with incongruent targets was recorded...

Despite the proven effectiveness of cochlear implant (CI) in the hearing restoration of deaf or hard-of-hearing (DHH) children, to date, extreme variability in verbal working memory (VWM) abilities is observed in both unilateral and bilateral CI user children (CIs). Although clinical experience has long observed deficits in this fundamental executive function in CIs, the cause to date is still unknown. Here, we have set out to investigate differences in brain functioning regarding the impact of monaural and binaural listening in CIs compared with normal hearing (NH) peers during a three-level difficulty n-back task undertaken in two sensory modalities (auditory and visual)...

OBJECTIVE: Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS...

PURPOSE: While the impact of paediatric cochlear implantation on parents and siblings are recognised, limited evidence exists regarding the effect of paediatric cochlear implantation on the entire family life (e.g., routine interactions, family activities). This study aimed to describe the impact of paediatric cochlear implantation on family life as perceived by parents, using concept mapping. MATERIALS AND METHODS: Parents of paediatric cochlear implant (CI) recipients ( n  = 29) participated in this concept mapping study to generate, group, and rate statements regarding the impact of paediatric cochlear implantation on family life...

Johanson-Blizzard syndrome (JBS) is a rare hereditary autosomal recessive disorder caused by a mutation in the ubiquitin protein ligase E3 component n-recognin 1 (UBR1) gene. This syndrome is characterized by the following typical clinical features: hypoplasia or aplasia of the alae nasi, congenital scalp defects, sensorineural hearing loss, hypothyroidism, growth retardation, psychomotor retardation, imperforate anus, genitourinary anomalies, and atypical hair patterns. Here, we describe a case of a 12-year-old girl with JBS of consanguineous parents...

INTRODUCTION: Hearing loss is a global issue of hearing disability and early detection and rehabilitation of hearing loss are important for the development of speech and language skills in hearing-impaired infants. There are multiple risk factors that aid in hearing loss but some are potential factors that contribute toward hearing loss in infants. The aim of this study was to assess the burden of hearing loss and its correlation with risk factors among high-risk infants at a teaching institution in Jaipur, Rajasthan...

BACKGROUND: ALL_EARS@UoS is a patient and public involvement and engagement (PPIE) group for people with lived experience of hearing loss. The purpose of the group is to share experiences of hearing loss and hearing healthcare, inform research and improve services for patients at University of Southampton Auditory Implant Service. A year after inception, we wanted to critically reflect on the value and challenges of the group. Four members of ALL_EARS@UoS were recruited to an evaluation steering group...

OBJECTIVE: To describe strategies used to maximise follow-up after a neonatal randomised trial, how these differed for families of different ethnicity, socioeconomic status and urban versus rural residence and investigate relationships between the difficulty of follow-up and rate of neurosensory impairment. METHOD: hPOD was a multicentre randomised trial assessing oral dextrose gel prophylaxis for neonatal hypoglycaemia. Follow-up at 2 years was conducted from 2017 to 2021...

Critical to managing a crisis such as COVID-19 is the propagation of information to all vulnerable populations. Despite guidelines regarding communicating with people with differing accessibility needs during crises, some often find their needs unmet. Following a lack of assisted communications for d/Deaf people during the COVID-19 pandemic, a Twitter hashtag campaign, #WhereIsTheInterpreter, was launched in the UK, protesting the lack of accessibility during official press briefings around the epidemic. The campaign received support from across the globe...

A common cause of deafness in humans is dysregulation of the endocochlear potential generated by the stria vascularis (SV). Thus, proper formation of the SV is critical for hearing. Using single-cell transcriptomics and a series of Shh signaling mutants, we discovered that the Shh receptor Patched1 (Ptch1) is essential for marginal cell (MC) differentiation and SV formation. Single-cell RNA sequencing analyses revealed that the cochlear roof epithelium is already specified into discrete domains with distinctive gene expression profiles at embryonic day 14, with Gsc as a marker gene of the MC lineage...