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Deaf child*

Sigrid Bosteels, Michel Vandenbroeck, Geert Van Hove
New-born screening programs for congenital disorders and chronic disease are expanding worldwide and children "at risk" are identified by nationwide tracking systems at the earliest possible stage. These practices are never neutral and raise important social and ethical questions. An emergent concern is that a reflexive professionalism should interrogate the ever earlier interference in children's lives. The Flemish community of Belgium was among the first to generalize the screening for hearing loss in young children and is an interesting case to study the public justification of early interventions for families with deaf children...
October 19, 2016: Journal of Bioethical Inquiry
Stephanie Kletke, Vaishnavi Batmanabane, Tianyang Dai, Ajoy Vincent, Shuning Li, Karen A Gordon, Blake C Papsin, Sharon L Cushing, Elise Héon
The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having Usher syndrome. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was done to determine the ocular phenotype...
October 15, 2016: Clinical Genetics
Lindsey Edwards, Lynne Aitkenhead, Dawn Langdon
OBJECTIVE: This study aimed to establish the relationship between short-term memory capacity and reading skills in adolescents with cochlear implants. METHODS AND MATERIALS: A between-groups design compared a group of young people with cochlear implants with a group of hearing peers on measures of reading, and auditory and visual short-term memory capacity. The groups were matched for non-verbal IQ and age. The adolescents with cochlear implants were recruited from the Cochlear Implant Programme at a specialist children's hospital...
November 2016: International Journal of Pediatric Otorhinolaryngology
Mo Chen, Zhaoyan Wang, Zhiwen Zhang, Xun Li, Weijing Wu, Dinghua Xie, Zi-An Xiao
OBJECTIVE: The present study aims to test whether deaf children with unilateral cochlear implantation (CI) have higher intelligence quotients (IQ). We also try to find out the predictive factors of intelligence development in deaf children with CI. METHODS: Totally, 186 children were enrolled into this study. They were divided into 3 groups: CI group (N = 66), hearing loss group (N = 54) and normal hearing group (N = 66). All children took the Hiskey-Nebraska Test of Learning Aptitude to assess the IQ...
November 2016: International Journal of Pediatric Otorhinolaryngology
Mingming Wang, Fengguo Zhang, Lei Xu, Yun Xiao, Jianfeng Li, Zhaomin Fan, Qian Sun, Xiaohui Bai, Haibo Wang
OBJECTIVE: To identify the disease-related SLC26A4 mutants in a Chinese Han pedigree associated with Enlarged vestibular aqueduct (EVA). METHODS: EVA diagnosis was based on the family history, clinical examinations, systematically audiometric evaluations, high-resolution computed tomography (HRCT) of the temporal bone, and magnetic resonance imaging (MRI) of inner ear. Sanger sequencing and mutation analysis of the SLC26A4 gene were performed in all members of this family to identify the disease-related SLC26A4 mutants...
November 2016: International Journal of Pediatric Otorhinolaryngology
Katrien Vermeire, Leslie Wexler, Andrea Vambutas
We present a 3-year old boy with Leopard syndrome. His clinical manifestations included a congenital bilateral sensorineural hearing loss. He underwent cochlear implantation on the right side at age 1 year and on the left side at age 1.5 years. The patient is doing very well and mainstreamed in a regular pre-school program with a teacher of the deaf and home based speech therapy. Bilateral cochlear implantation in the case of a child with Leopard syndrome can be successful.
November 2016: International Journal of Pediatric Otorhinolaryngology
Yongzhi Liu, Liying Ao, Haitao Ding, Dongli Zhang
The aim was to study the frequencies of common deafness-related mutations and their contribution to hearing loss in different regions of Inner Mongolia. A total of 738 deaf children were recruited from five different ethnic groups of Inner Mongolia, including Han Chinese (n=486), Mongolian (n=216), Manchurian (n=24), Hui (n=6) and Daur (n=6). Nine common mutations in four genes (GJB2, SLC26A4, GJB3 and mitochondrial MT-RNR1 gene) were detected by allele-specific PCR and universal array. At least one mutated allele was detected in 282 patients...
October 10, 2016: Genetics and Molecular Biology
Soren Gantt, Francois Dionne, Fred K Kozak, Oran Goshen, David M Goldfarb, Albert H Park, Suresh B Boppana, Karen Fowler
Importance: Congenital cytomegalovirus (cCMV) infection is a major cause of childhood deafness. Most cCMV infections are not diagnosed without newborn screening, resulting in missed opportunities for directed care. Objective: To estimate the cost-effectiveness of universal and targeted newborn cCMV screening programs compared with no cCMV screening. Design, Setting, and Participants: Models were constructed using rates and outcomes from prospective cohort studies of newborn cCMV screening in US postpartum care and early hearing programs...
October 10, 2016: JAMA Pediatrics
Sanjay Chawla, Girija Natarajan, Seetha Shankaran, Athina Pappas, Barbara J Stoll, Waldemar A Carlo, Shampa Saha, Abhik Das, Abbot R Laptook, Rosemary D Higgins
Importance: Many premature infants are born without exposure to antenatal steroids (ANS) or with incomplete courses. This study evaluates the dose-dependent effect of ANS on rates of neonatal morbidities and early childhood neurodevelopmental outcomes of extremely premature infants. Objective: To compare rates of neonatal morbidities and 18- to 22-month neurodevelopmental outcomes of extremely premature infants exposed to no ANS or partial or complete courses of ANS...
October 10, 2016: JAMA Pediatrics
Marc S Schwartz, Eric P Wilkinson
OBJECTIVE: Auditory brainstem implants (ABIs), which have previously been used to restore auditory perception to deaf patients with neurofibromatosis type 2 (NF2), are now being utilized in other situations, including treatment of congenitally deaf children with cochlear malformations or cochlear nerve deficiencies. Concurrent with this expansion of indications, the number of centers placing and expressing interest in placing ABIs has proliferated. Because ABI placement involves posterior fossa craniotomy in order to access the site of implantation on the cochlear nucleus complex of the brainstem and is not without significant risk, we aim to highlight issues important in developing and maintaining successful ABI programs that would be in the best interests of patients...
September 26, 2016: Laryngoscope
Takehiko Ueyama, Yuzuru Ninoyu, Shin-Ya Nishio, Takushi Miyoshi, Hiroko Torii, Koji Nishimura, Kazuma Sugahara, Hideaki Sakata, Dean Thumkeo, Hirofumi Sakaguchi, Naoki Watanabe, Shin-Ichi Usami, Naoaki Saito, Shin-Ichiro Kitajiri
DIAPH1 encodes human DIA1, a formin protein that elongates unbranched actin. The c.3634+1G>T DIAPH1 mutation causes autosomal dominant nonsyndromic sensorineural hearing loss, DFNA1, characterized by progressive deafness starting in childhood. The mutation occurs near the C-terminus of the diaphanous autoregulatory domain (DAD) of DIA1, which interacts with its N-terminal diaphanous inhibitory domain (DID), and may engender constitutive activation of DIA1. However, the underlying pathogenesis that causes DFNA1 is unclear...
October 5, 2016: EMBO Molecular Medicine
Christine Yoshinaga-Itano, Mallene Wiggin
Hearing is essential for the development of speech, spoken language, and listening skills. Children previously went undiagnosed with hearing loss until they were 2.5 or 3 years of age. The auditory deprivation during this critical period of development significantly impacted long-term listening and spoken language outcomes. Due to the advent of universal newborn hearing screening, the average age of diagnosis has dropped to the first few months of life, which sets the stage for outcomes that include children with speech, spoken language, and auditory skill testing in the normal range...
November 2016: Seminars in Speech and Language
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
Jenessa L Seymour, Kathy A Low, Edward L Maclin, Antonio M Chiarelli, Kyle E Mathewson, Monica Fabiani, Gabriele Gratton, Matthew W G Dye
Theories of brain plasticity propose that, in the absence of input from the preferred sensory modality, some specialized brain areas may be recruited when processing information from other modalities, which may result in improved performance. The Useful Field of View task has previously been used to demonstrate that early deafness positively impacts peripheral visual attention. The current study sought to determine the neural changes associated with those deafness-related enhancements in visual performance...
September 23, 2016: Hearing Research
Lian van Berkel-van Hoof, Daan Hermans, Harry Knoors, Ludo Verhoeven
BACKGROUND: Augmentative signs may facilitate word learning in children with vocabulary difficulties, for example, children who are Deaf/Hard of Hearing (DHH) and children with Specific Language Impairment (SLI). Despite the fact that augmentative signs may aid second language learning in populations with a typical language development, empirical evidence in favor of this claim is lacking. AIMS: We aim to investigate whether augmentative signs facilitate word learning for DHH children, children with SLI, and typically developing (TD) children...
September 23, 2016: Research in Developmental Disabilities
A C Jones, E Toscano, N Botting, C R Marshall, J R Atkinson, T Denmark, R Herman, G Morgan
Previous research has highlighted that deaf children acquiring spoken English have difficulties in narrative development relative to their hearing peers both in terms of macro-structure and with micro-structural devices. The majority of previous research focused on narrative tasks designed for hearing children that depend on good receptive language skills. The current study compared narratives of 6 to 11-year-old deaf children who use spoken English (N=59) with matched for age and non-verbal intelligence hearing peers...
September 21, 2016: Research in Developmental Disabilities
Caterina Garone, Juliana Gurgel-Giannetti, Simone Sanna-Cherchi, Sindu Krishna, Ali Naini, Catarina M Quinzii, Michio Hirano
SUCLA2 defects have been associated with mitochondrial DNA (mtDNA) depletion and the triad of hypotonia, dystonia/Leigh-like syndrome, and deafness. A 9-year-old Brazilian boy of consanguineous parents presented with psychomotor delay, deafness, myopathy, ataxia, and chorea. Despite the prominent movement disorder, brain magnetic resonance imaging (MRI) was normal while (1)H-magnetic resonance spectroscopy (MRS) showed lactate peaks in the cerebral cortex and lateral ventricles. Decreased biochemical activities of mitochondrial respiratory chain enzymes containing mtDNA-encoded subunits and mtDNA depletion were observed in muscle and fibroblasts...
September 20, 2016: Journal of Child Neurology
Faustino Núñez-Batalla, Carmen Jáudenes-Casaubón, Jose Miguel Sequí-Canet, Ana Vivanco-Allende, Jose Zubicaray-Ugarteche, Rubén Cabanillas-Farpón
Important progress in the fields of molecular genetics (principally) and diagnostic imaging, together with the lack of a consensus protocol for guiding the diagnostic process after confirming deafness by neonatal screening, have led to this new work document drafted by the Spanish Commission for the Early Detection of Child Deafness (Spanish acronym: CODEPEH). This 2015 Recommendations Document, which is based on the most recent scientific evidence, provides guidance to professionals to support them in making decisions regarding aetiological diagnosis...
September 16, 2016: Acta Otorrinolaringológica Española
Marek Meristo, Karin Strid, Erland Hjelmquist
Previous research suggests that deaf children who grow up with hearing parents display considerable difficulties in understanding mental states of others, up to their teenage years when explicitly asked in a verbal test situation (Meristo et al., 2007). On the other hand, typically developing pre-verbal infants display evidence of spontaneous false belief attribution when tested in looking-time tasks, although verbal tests are typically not passed before the age of 4years (Onishi & Baillargeon, 2005). The purpose of the present study was to examine whether deaf children of hearing parents are able to demonstrate spontaneous belief attribution in a non-verbal eye-tracking task...
September 13, 2016: Cognition
Somayeh Reiisi, Mohammad Amin Tabatabaiefar, Mohammad Hosein Sanati, Morteza Hashemzadeh Chaleshtori
OBJECTIVES: Non-syndromic sensorineural hearing loss (NSHL) is a common disorder affecting approximately 1 in 500 newborns. This type of hearing loss is extremely heterogeneous and includes over 100 loci. Mutations in the GJB2 gene have been implicated in about half of autosomal recessive non-syndromic hearing loss (ARNSHL) cases, making this the most common cause of ARNSHL. For the latter form of deafness, most frequent genes proposed include GJB2, SLC26A4, MYO15A, OTOF, and CDH23 worldwide...
July 2016: Iranian Journal of Basic Medical Sciences
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