Academic literature on the topic 'Childhood deafness'

Permanent childhood sensorineural hearing loss, is one of the most common birth defects in developed countries. It is important to identify the aetiology of hearing loss for many reasons, as there may be important health surveillance implications particularly with syndromic causes. Non-syndromic sensorineural hearing loss is a highly heterogeneous genetic condition, meaning that it may be caused by any one of numerous genes, with very few phenotypic distinctions between the different genetic types. This has previously presented significant challenges for genetic testing. However, the introduction of new technologies should enable more comprehensive testing in the future, bringing significant benefits to more affected children and their families.

Background and Objectives: To examine the maturational status of the auditory cortex in adults with cochlear implants (CIs) using the latencies of the P1, N1, and P2 components of cortical auditory-evoked potentials (CAEPs). Subjects and Methods: A total of 25 adults with CIs and 25 age-matched, normal-hearing control subjects participated in this study. Specifically, patients with CIs were divided into three groups depending on their age of deafness onset: Group A comprised patients with prelingual deafness who had received CI during early childhood (n=7), Group B comprised patients with early childhood-onset, progressive deafness who had received CI during childhood (n=6), and Group C comprised patients with adult-onset deafness (n=12). The P1, N1, and P2 latencies of their CAEPs were then compared between CI patients and normal-hearing subjects. Results: All participants showed clear CAEP responses. P1 and N1 latencies in Group A and Group C patients were significantly longer than those in the control group. Meanwhile, Group B patients had significantly longer N1 and P2 latencies, as compared with those in the control group. Conclusions: Patients with prelingual deafness and those with early-childhood onset, progressive deafness who received CI developed primary and higher-order auditory areas postoperatively when they became adults. However, their auditory cortex maturational statuses seemed to be worse than that of normal-hearing individuals. Furthermore, adult patients with late-onset deafness might experience degenerative auditory cortex changes during the auditory deprivation period between deafness onset and cochlear implantation.

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O implante coclear, em nosso país, ainda é uma prática que se considera nova, em especial com os sujeitos surdos, por se tratar de um procedimento recente, que vem possibilitando, de certa forma, uma audição que a mídia vem chamando de ouvido biônico . Esta pesquisa tem o propósito de refletir sobre o assunto a fim conhecer mais sobre esse procedimento que vem sendo realizado com certa frequência no Brasil. Poucas são as pesquisas sobre esse tema na área da educação. A maioria dos dados apresentados vem da área clínica. A possibilidade do implante gera, na família de uma criança surda, a expectativa de que terá uma criança normal e que, a qualquer momento, o filho vai falar e ouvir. Para ter um bom resultado, segundo os especialistas da Fonoaudiologia, é necessário que a criança implantada evite contato com Libras e com outros surdos, pois pode comprometer o tratamento. Este estudo visa a analisar os impactos do implante coclear na vida de uma criança surda que frequenta uma escola de Ensino Fundamental com uma política bilíngue. O estudo toma como referencial a perspectiva histórico-cultural e considera que o sujeito surdo é constituído socialmente, por meio da linguagem, como aquisição cultural. Nesta pesquisa foi realizado o estudo de caso do tipo etnográfico de uma criança surda que passou por uma intervenção cirúrgica (implante coclear). A pesquisa de campo ocorreu por meio de observação e entrevistas semiestruturadas. Busca compreender as formas de relação estabelecidas entre a criança e os adultos e outras crianças em diferentes espaços, como a casa da criança, a clínica fonoaudiológica, a escola, observando as repercussões dessas relações na maneira como a criança se vê como implantada. O estudo apontou que há diferenças no modo como os profissionais compreendem a condição da criança com implante e se relacionam com ela de maneiras distintas. Para alguns, ela é surda e, portanto, privilegiam a Libras na interação com ela. Outros priorizam a oralidade, por considerar que o implante pode possibilitar à criança ouvir e falar. Outros, ainda, entendem que deve ser dada oportunidade a ela de aprendizado da Libras e da oralidade, de forma a ampliar suas possibilidades de contato e interação com os outros. Essa era também a opinião da mãe. A análise permite ver uma criança informada sobre a sua condição de criança implantada e que está aprendendo a conviver com o implante. Os depoimentos da criança fornecem indícios de que ela se reconhece como surda e que a língua de sinais é a que mais a completa como usuária de uma língua, contudo não nega as novas possibilidades que a oralidade lhe traz
The cochlear implant in our country is still considered a new practice, in particular with the deaf subject, because it is a recent procedure, which has been enabling, in a way, a hearing , often called by the media as bionic ear . The research aims at reflecting on the topic in order to understand more about that procedure that has been frequently carried out in Brazil. There has not been much research about this issue in the education field. Most of the data presented come from the clinical area. The implant possibility causes in the families of a deaf child the expectation of having a normal child and that anytime the child will be able to speak and hear. To achieve a good result, according to the speech specialists (audiologists), it is necessary that the child with the implant avoids contact with sign language and other people with hearing disability, which may compromise treatment. The study searches for analyzing the impacts of the cochlear implant in the life of a deaf child going to an Elementary School with a bilingual policy. The study uses as reference the historical-cultural perspective and considers the deaf is socially constituted, through the language, as cultural acquisition. It used the ethnographic study-case of a deaf child who had undergone surgery (cochlear implant). The field research was done through observation processes and semi-structures interviews. It attempts to comprehend the relation forms established between the child and the adults and other children in different spaces, as the child s home, the speech clinic, the school noting the repercussions from such relations the same way the child sees her/himself as the one with an implant. The study indicated that there are differences in the way the professionals understand the implanted children s condition and they relate with them in distinct ways. For some, the child is deaf and therefore, they prefer to use sign language when interacting. Others prioritize the orality, by considering that the implant can make the child able to hear and speak. Others even understand that an opportunity must be given to the child to learn the sign language and orality, in a way to broaden the child s possibilities of contact and interaction with others. That was also the mother s opinion. The analysis allows to see a child instructed regarding condition of an implanted child who is learning how to live with an implant. The child s testimonials provided evidence that the child recognizes her/himself as deaf and the sign language is the one that most fulfill her/his needs as a language user, however the opportunities brought by orality cannot be denied

CoordenaÃÃo de AperfeiÃoamento de Pessoal de NÃvel Superior
Esta dissertaÃÃo teve por objetivo verificar como as prÃticas docentes elaboradas e executadas nas instituiÃÃes de EducaÃÃo Infantil estÃo contribuindo para o progresso dos seus educandos surdos e como estÃo desenvolvendo estratÃgias e condiÃÃes adequadas à evoluÃÃo dessas crianÃas, sem esquecer as diferenÃas linguÃsticas entre esses agentes, analisando a lÃngua como possÃvel obstÃculo, em razÃo do desconhecimento, por parte de ambos, de suas lÃnguas naturais. Para alcanÃar esse objetivo, como percurso metodolÃgico, escolheu-se a anÃlise qualitativa dos dados, mediante estudo de caso. Optou-se por entrevistas, observaÃÃo e anÃlise de documentos, registrados em fotografias, alÃm das gravaÃÃes de Ãudio, do diÃrio de campo em caderno e lÃpis e, quando adequado e possÃvel, observaÃÃes digitadas em um notebook. As referÃncias teÃricas empregadas para a fundamentaÃÃo da anÃlise dos dados foram centradas, principalmente, mas nÃo exclusivamente, nas obras de Miguel Zabalza (1998), para a categoria que versa sobre a EducaÃÃo Infantil e suas caracterÃsticas tÃo peculiares a quem lida com os pequeninos; Carlos Skliar (1997) e Sanchez (1990), sobre surdez, sua linha histÃrica e suas vertentes educacionais, alÃm dos conceitos sobre Libras e correlatos; Priscila Lima (2010) e Rossana Ramos (2010), em EducaÃÃo Especial e Inclusiva, exibindo as variaÃÃes de terminologias que, com frequÃncia, mais nos confundem do que esclarecem; nos documentos do MEC, que normatizam os pilares deste trabalho: a EducaÃÃo Infantil, a EducaÃÃo Especial, a EducaÃÃo Inclusiva e temas relacionados à surdez, como a Libras, a regulamentaÃÃo da profissÃo dos intÃrpretes de Libras e o decreto sobre as Salas de Recurso Multifuncionais onde ocorre o Atendimento Educacional Especializado. Partindo das anÃlises destes, constata-se que, apesar dos esforÃos que estÃo sendo feitos, a ausÃncia da Libras como mediadora das relaÃÃes educador e educando e a superficialidade do conhecimento das particularidades da surdez dificultam o Ãxito das prÃticas docentes.

This thesis is focused on the ways that parents of deaf children and young adults with cochlear implants experience childhood deafness in an Australian setting. In the last three decades significant developments in deafness have resulted in greater access to earlier diagnosis and choices in rehabilitative interventions. These changes, brought about by universal newborn hearing screening and a capacity to identify deafness in the first few weeks of life, technological developments including the cochlear implant, and access to a range of education and language options, have been seen as advantageous by health and education professionals and by parents of deaf children. Furthermore, policies of informed choice and family centred practice have been developed as international benchmarks in service provision and to ameliorate the tensions and politics within the context of childhood deafness. Despite these changes, what is ‘best’ for a deaf child and their parents continues to be controversial and subject to debates that proliferate through the academy, public culture including social media, and in practices in deafness. This ethnographic thesis unpacks childhood deafness to contribute insights and knowledge about everyday experiences of deafness from the perspective of both parents of deaf children, and young adults with cochlear implants. It explores parents’ emotional reactions to childhood deafness and their interactions with health and education professionals, which were often contested and ambiguous. Additionally, I probe the experiences of young adults and the significance that technology had in their everyday lives to advance understandings about ways of being deaf and hearing through cochlear implants. Drawing on sixteen months of ethnographic data collected in an Australian city from 2017 to 2018 I critically explore the complex and socially situated relationships and structures in a deaf health and education context. I focus on parents’ encounters with clinical and education professionals as they experience a diagnostic referral process and undertake a ‘follow the baby’ approach to examine clinical and education encounters from first diagnosis through to a treatment seeking process. Drawing on phenomenology and an anthropology of the senses, emotions, and time and space, I show how parents’ experiences of deafness are historically and socially situated, which contributes knowledge and understandings about how parents and young adults experience deafness. I examine the role of emotion in clinical contexts including parents’ emotional reactions to diagnosis, to medical uncertainty and to the ways parents make time sensitive decisions for their children. I argue that in the clinical context women’s emotion is often characterised as problematic at the moment of diagnosis. Additionally, the experiences of young adults with cochlear implants are explored to understand the sensory significance of deafness and hearing through technology. The everyday experiences of young adults and parents are central to this thesis, which demonstrates the significance of technology, medicine, culture, and communication to diverse and creative experiences of deafness. In exploring the everyday practices of families with deaf children I illustrate how deafness continues to be framed by a binary medical or cultural lens. While some scholars frame deafness as a medical experience that embraces a social imaginary of ‘normal’, this thesis presents a more nuanced view to demonstrate the ways that cultural and creative production of difference and multiplicity are generated through diverse deaf experiences.
Thesis (Ph.D.) -- University of Adelaide, School of Social Science: Anthropology and Development Studies, 2022

The aetiological diagnosis of recessive non-syndromic hearing loss poses a challenge owing to marked heterogeneity and the lack of identifying clinical features. The finding that up to 50% of recessive non-syndromal genetic hearing loss among Caucasians was due to mutations in GJB2, the gene encoding Connexin 26 (Cx26) was a breakthrough, whose value as a diagnostic tool has been limited by the significant variation in the prevalence of deafness genes and loci among population groups. The significant association of the GJB6-D13S1830 deletion among individuals with one mutant GJB2 allele highlighted the need to explore population specific genetic mutations for NSHL. Although data from Sub-Saharan Africa is limited, reported studies found a high prevalence of R143W GJB2 mutation among Ghanaian, the 35delG mutation in 5 out of 139 Sudanese and a low prevalence of GJB2 variations among 385 Kenyan deaf children. The mutation spectrum of Waardenburg Syndrome (WS) in Africans has not been documented. During a visit to a School for the Deaf in the Limpopo Province of South Africa in 1997, it was noted that a high number of students came from Nzhelele sub-district. All had childhood onset hearing loss with no associated anomalies or disorders. The question arose as to whether there was a high-risk area for deafness in the Limpopo Province and what the aetiology of this hearing loss was.The main aim of this study was to investigate the role of GJB2, the GJB6-D13S1830 deletion, and the four common mitochondrial mutations, A1555G, A3243G, A7511C and A7445G, in the African hearing-impaired population of Limpopo province in South Africa, and to identify the mutation spectrum of the deafness genes found. The type and degree of hearing loss in this hearing impaired population would also be assessed. Secondly, this study sought to identify the mutations in a sibling pair with 2 clinical WS and to use the findings in a future study to establish the mutation spectrum of WS in the African population of the Limpopo province and of South Africa in general. The study was designed as a two phase study, in which phase 1 was used for hypothesis formulation and phase 2 was for hypothesis testing. While phase 1 was a descriptive retrospective case study, phase 2 was a combination of sample survey and prospective descriptive case study. In phase 1, demographic data of 361 students in two schools of the deaf in the Limpopo province was analyzed for evidence of areas of high risk populations for deafness in the province. In phase 2, a group of 182 individuals with genetic non-syndromic hearing loss (NSHL) and two siblings with clinical WS from two schools for the Deaf in the Limpopo Province of South Africa were investigated. A thorough clinical examination, audiological evaluation and urinalysis were done. Mutational screening was carried out in all 184 subjects using genomic DNA using single-strand conformation polymorphism (SSCP), multiplex polymerase chain reaction (PCR), and direct sequencing for GJB2, and Restriction Fragment-Length Polymorphism (PCR–RFLP) analysis for GJB6, and SSCP, hetero-duplex analysis, and direct sequencing of the first 8 exons of PAX3 and all of MITF for Waarenburg syndrome. Data analysis was by geographical mapping, frequency tables, tests of association with calculation of odds ratios, and binary logistic regression analysis using STATA and GIS mapping systems. The results indicate that there seem to be areas of genuine populations at risk for hearing loss in the Limpopo province of South Africa, namely Mutale and parts of Makhado and Thulamela municipalities. In Thulamela (NP343) wards 11-15, 26-30 and 31-35, and in Mutale (NP 344) wards 6-10, together accounted for 67 (18%) of participants in phase 1, and 33 (18%) of the participants in phase 2 of the study. Mutale municipality in the Vhembe 3 district gave with a projected prevalence of at least 13.14 deaf children per 100,000 African population attending the local school for the deaf. The observed hearing loss is a genetic, non-syndromic form, which is mainly severe and severe to profound, although without any clear defining configuration or shape. It is a stable, non-progressive and prelingual form of hearing loss, implying that this may be a recessive form of deafness. No identifiable environmental confounding factors or associations were identified. The deafness is not linked the common known auditory gene mutations in GJB2, the GJB6-D13S1830 deletion, or the common mitochondrial mutations A1555G, A3243G, A7511C and A7445G. Severe and profound levels of hearing loss were found in 22.8% and 75% of the cohort respectively, with the majority exhibiting flat (70.1%) or sloping (23.4%) audiograms that were commonly symmetrical (81.5%). However, as indicated, there was no clear pattern in the audiological findings overall. None of the 184 hearing impaired individuals exhibited any of the reported disease causing mutations of GJB2, including 35delG. There was, however, a high prevalence of two variants, the C>T variant at position g.3318-15 and the C>T variant at position g.3318-34, occurring in 21.4% and 46.2% of the deaf cohort respectively. The same variants were found to occur in 35% and 42.6% of a normal hearing control group (n = 63) respectively, indicating that these variations are polymorphisms. In three subjects (1.63% of the cohort), a T>A homozygous variation at position g.3318-6 was detected. Its significance in the causation of NSSNHL is yet to be determined. The GJB6-D13S1830 deletion was not detected in any of the participants. None of the four mitochondrial mutations screened for were found. 4 These results indicate that GJB2 is not a significant deafness gene in the African population of the Limpopo Province of South Africa and that significant genes for non-syndromic recessive hearing loss in this population are yet to be found. The geographical clustering of deafness found in this study, combined with the lack of identifiable common associated clinical features among the subjects of this study (excluding the WS sibling pair), suggests that these subjects have a genetic recessive non-syndromal type of hearing loss. In the context of historical and cultural evidence of consanguinity in this population, a founder effect cannot be ruled out. A rare mutation, R223X, previously identified only once out of 470 WS patients, was identified in the PAX3 gene among the WS sibling pair. A novel silent change GGG>GGT at amino acid 293, was also identified. These identical findings document, for the first time, a molecular defect in WS in an African sibling pair, and confirm WS Type I in this family, which could be found in other WS type I South Africans in the Limpopo Province of South Africa. The current study demonstrated that parents of genetically hearing impaired children in these areas are able to detect hearing loss at an early age, with over 60% suspecting their children’s hearing loss below 6 months of age. A child-centered management model encompassing all the areas relevant to childhood deafness/hearing impairment, which takes into consideration the prevailing logistical and financial constraints of the available healthcare system, is proposed. The implementation of this model requires a paradigm shift from the current fragmented model of service delivery to a cohesive patient-centered approach, based on concrete data from appropriate community based research, in which all the relevant parties communicate and share resources. 5 It would achieve the goals of early detection and intervention, as well as inclusive education for all. The relevant health and education policies are already in place and the posts funded. Equitable implementation of these policies would require appropriate community based research, as well as improved communication and consultation between the various stakeholders to ensure an efficient and affordable quality healthcare service for all hearing impaired South Africans.

Most deaf children in the United States are not educated in specialized schools for the deaf but in public schools. This has had a detrimental effect on these students because many public-school teachers misunderstand deafness and are unable to adjust their teaching strategies to address the needs of this population. The mission of this chapter is to educate teachers on deafness and how to better teach and relate to a child who is deaf or hard of hearing. Specifically, this chapter will provide pertinent information for helping teachers better understand deaf and hard-of-hearing students as well as provide evidence-based practices and teaching tips that can be utilized in the classroom with this group.

This chapter outlines ear, nose and throat diseases. It includes ENT examination, prevalence of ENT symptoms, the ear, audiology, painful ears, discharging ears, fluid in the middle ear, childhood deafness, cochlear implants, deafness in adults, tinnitus, acoustic neuroma, noise induced hearing loss, vertigo, rhinosinusitis and nasal polyps, the paranasal sinuses, nasal injury and foreign bodies, nosebleed (epistaxis), throat infections, stridor, acute airway obstruction, hoarseness, laryngeal nerve palsy, head and neck cancers, dysphagia, facial palsy, lumps in the neck, the salivary glands, dry mouth (xerostomia), and dentistry for doctors.

The prevalence of profound deafness in the United Kingdom is reported as 9 per 10,000 in 3-year-olds to 16 per 10,000 in 9- to 16-year-olds, although it is estimated that these figures may be higher because of possible under-diagnosing (Fortnum et al. 2001). In England and Scotland, approximately 13 in 10,000 children have a permanent hearing impairment of 40 dB or more (moderate to profound) across the frequencies 500–4,000 Hz (Fortnum and Davis, 1997; Fortnum, 2003; Kennedy and McCann, 2008; MacAndie et al. 2003) and in 11 in 10,000 this loss is congenital. The remainder are acquired or progressive. Profound impairment (≥95 dB loss) occurs in 2.4 per 10,000. Bilateral sensorineural deafness had a prevalence of 21 per 10,000 in a Finnish birth cohort (1974–1987) (Vartiainen, Kemppinen, and Karjalainen 1997). A survey in the general population in Sichuan, China, found a prevalence of 1.9 per 1,000 profound deafness (Liu et al. 1993). Table 44.1 shows the World Health Organization (WHO) and British Society of Audiology definitions of degrees of deafness. Sensorineural deafness involves problems either in hair cell function in the cochlear or in the nerve transmission of sound. For bilateral sensorineural deafness, the diagnosis is made at variable ages in health systems in which there is no universal neonatal screening. This varies across cultures, but can still be surprisingly late even in modern health systems. One study of 106 children with bilateral sensorineural deafness showed a mean age for first diagnosis at 42 months (median 33 months) of age, with a range from 4 months to 11 years of age (Walch 2003). In another study in Glasgow, Scotland (Chaurasia and Geddes, 2008), only 50% of early childhood deafness presented before 2 years of age. Conductive deafness involves problems with sound travelling from the outer ear, through the middle ear, to the cochlear. The commonest cause is otitis media (“glue ear”), with losses usually in the mild to moderate range. About half of children between 2 and 4 years will have at least one episode of otitis media with effusion (OME) (Zielhuis, Rach, and Van den Brock 1990).