To facilitate participation in hearing situations at work sites persons with hearing loss may use assistive listening devices (ALDs). Compared to personal hearing aids ALDs have received little research attention. The aim of this study was dual; firstly to describe ALD-users in Sweden and the usefulness of various equipment in relation to specified hearing situations at work. Secondly, to describe favorable and non favorable conditions for using ADL at everyday situations at work. Seventy ADL-users answered a questionnaire and eleven of these persons were subsequently interviewed. The concept of stigma has been identified in research on hearing loss as a strong factor of denial and resistance for the use of both hearing aids and ALDs, depending on the negative connotations related to visible means for hearing. In this study overcoming stigmatization was further analyzed using social recognition as a theoretical approach. In a concluding discussion, where both categorizations and themes from the interviews were used, it issuggested that the use of visible ALDs is a way of overcoming stigmatization and that their visibility function as a mechanism in developing mutual social recognition in the work group.Though the results show a fairly high degree of usefulness from ALDs, work place adaptations should to a greater extent pay attention to environmental factors (e.g. noise and bad acoustics) influencing the use of this technology. More information on such factors is needed from studies of realistic situations at concrete work sites. Conclusions from this study are mainly valid for this sample; knowledge of the population is small due to lack of registers kept by the providers of this technology. To facilitate further studies on employed persons with hearing loss available statistics could be significantly improved by keeping records on both medical data and all kinds of rehabilitative adaptations measures.

No description available.

Abstract Finding the genetic causes leading to phenotypes of mitochondrial diseases is challenging because of heterogeneity of the disorders and variety of the underlying biochemical defects. In adults, many of the manifestations of mitochondrial diseases cannot be distinguished from the neurodegenerative processes associated with old age. A single mutation or mutations within the same gene can result in a broad range of disorders. Conversely, clinically similar, monogenic disorders may be caused by genes which are governing entirely different cellular pathways. This study investigated the genetic etiology underlying certain symptoms which are characteristic for mitochondrial syndromes, or mimics of the mitochondrial ones. In the first project, we presented the contribution of genetic variation in the Wolfram Syndrome 1 gene to the risk of diabetes mellitus and sensorineural hearing impairment. We also estimated the frequency of a rare pathogenic variation in WFS1. The second project detected a link between the complex phenotype of age-related hearing impairment and the WFS1 gene. Monogenic forms of ARHI are extremely rare and we succeeded in recognizing one Mendelian form of the trait. The third project confirmed the Mitofusin 2 gene causality in the outlier phenotype of Charcot-Marie-Tooth disease. The fourth project described a Finnish family with two affected siblings with adult-onset ataxia, diabetes mellitus, and hypergonadotropic hypogonadism. The found novel mutation in mtDNA, m.8561C>G, was located in the overlapping region of two mitochondrial genes and resulted in an impaired assembly and dysfunctional energy production of mitochondrial ATP synthase. This thesis expands our knowledge about complex neurological phenotypes and identifies not only some causative genes but also outlier phenotypes, which should be noted in clinical practice. / Tiivistelmä Perintötekijät mitokondriaalisten ja niiden kaltaisten tautien taustalla ovat vaikeasti tunnistettavissa. Tautien kirjo on valtava, ja niihin johtavat biokemialliset syyt ovat moninaisia. Aikuisten mitokondriotaudit voivat jäädä diagnosoimatta, koska oireet voivat peittyä vanhenemiseen liittyviin neurodegeneratiivisiin prosesseihin. Sama mutaatio tai eri mutaatiot samassa geenissä voivat johtaa kliinisesti täysin erilaisiin ilmiasuihin. Toisaalta, kliinisesti samankaltaiset taudit voivat olla geneettisesti ja solubiologiallisesti kirjavia. Tässä tutkimuksessa selvitetään geneettistä etiologiaa tiettyjen mitokondriaalisille ja niiden kaltaisille taudeille tyypillisten oireiden taustalla. Ensimmäisessä osajulkaisussa tunnistetaan geneettisiä riskivariantteja Wolfram Syndrome 1 -geenissä diabeteksen ja kuulonaleneman taustalta. Lisäksi tutkimuksessa estimoidaan harvinaisen tautia aiheuttavan variaation määrää kyseisessä geenissä. Toinen projekti esittelee suomalaisen perheen, jossa myöhään alkaneen kuulonaleneman, ikäkuulon, geneettinen syy paljastuu WFS1-geenistä, jota ei aiemmin ole liitetty kyseiseen ilmiasuun. Yhden geenin aiheuttamat ikäkuulotapaukset ovat todella harvinaisia, koska ikäkuulo on monimutkainen kokonaisuus, johon ympäristötekijöillä on suuri vaikutus. Kolmas osajulkaisu kuvaa potilastapauksia, joiden ilmiasu on epätyypillinen Charcot-Marie-Toothin neuropatia. Tautigeeni on tunnettu Mitofusin 2, mutta sen aiheuttaman taudinkuvat ovat yleensä vakavampia ja varhain alkaneita. Viimeinen osajulkaisu kuvaa suomalaisen perheen, jonka kahden oireisen sisaruksen taustalta löytyy mitokondriaalisen DNA:n uusi mutaatio, joka sijaitsee kahden geenin alueella muuttaen niiden molempien lopputuotetta. Mutaation, m.8561C>G, osoitetaan vaikuttavan mitokondriaalisen ATP-syntaasin rakentumiseen ja energiatuotantoon. Tämä väitöskirja laajentaa geneettistä tietoisuutta neurologisten tautien taustalla ja esittelee uusia geneettisiä syitä ja ilmiasuja, jotka tulisi huomioida kliinisessä työssä terveydenhuollossa.

The purpose of this investigation was to determine the prevalence of specific learning disabilities in school-aged hearing impaired children based on the proposed theoretical definition of the National Joint Committee for Learning Disabilities (1981) and the theoretical definition constructed by the Canadian Association for Children and Adults with Learning Disabilities (1981). The operationalization of these theoretical definitions, coupled with the current operational definition issued by the Texas Education Agency (1983), formulated the investigative framework.

No description available.

Antalet elever med hörselnedsättning som placeras i hörande skolor istället för i hörselklass eller specialskola ökar. Skillnaderna mellan skolformerna är att specialskolan och hörselklasserna erbjuder anpassade lärmiljöer och skolpersonal med särskild kompetens i frågor som rör hörselnedsättning och dess konsekvenser för individen. Denna kompetens är inte självklar i de hörande skolorna och inget nationellt övergripande ansvar finns för att se över denna elevgrupps förutsättningar till lärande och delaktighet i skolorna, vilket i praktiken innebär att skolgången för dessa elever inte alls är jämlik eller likvärdig. Syftet med denna studie är att bidra med kunskaper om hur lärare i hörande skolor uppfattar arbetet med att undervisa en elev med hörselnedsättning i sin klass och vilka utvecklingsområden samt framgångsfaktorer som finns. Datainsamlingen har skett med kvalitativ semistrukturerad intervjumetod av sex högstadielärare och en gymnasielärare från spridda delar av Sverige. Resultaten och analysen av intervjuerna visar att det finns ett flertal utvecklingsområden för skolorna att arbeta med för att denna elevgrupp ska få en utbildning som ger dem lika förutsättningar till kunskapsutveckling och identitetsutveckling som sina klasskamrater i enlighet med vad skollagen förskriver. Resultaten visar också på ett antal framgångsfaktorer som när de tillämpas möjliggör till delaktighet och lärande i undervisningen oavsett hörselfunktion. / The number of hard of hearing students with a school placement in mainstream schools instead of a placement in special schools or in a class adapted for hard of hearing students are increasing. The differences between the school forms depend on how the auditive environment is adapted to improve the opportunities to hear and how. The special schools and the classes adapted for hard of hearing students do also offer teachers with specialist competence regarding hearing loss and its consequences for the individual. This competence is not given in the mainstream schools and furthermore, there is no national overall responsibility with an aim to review how schools provide this group of students with opportunities for learning and participation, which indicates that the schooling of these students is not equal or equivalent at all. The purpose of this study is to contribute with knowledge in how teachers in mainstream schools perceive the work of teaching these students and make visible developmental areas but also factors that enhance the learning and participation in the classrooms. The method used for this study is qualitative through semistructured interviews with six junior high school teachers and one high school teacher from different parts of Sweden. The results and the analysis of the interviews show that there are a number of development areas for the schools to work with in order for this group of students to receive an education that gives them the same conditions for knowledge development and identity development as their classmates in accordance with the School Education Act. The results also show a number of success factors that, when applied, enable participation and learning in teaching, regardless of hearing function.

Orientador: Ana Cláudia Moreira Almeida-Verdu / Resumo: A deficiência auditiva sensorioneural pré-lingual pode comprometer o desenvolvimento da linguagem oral. O implante coclear (IC) é um dispositivo auditivo que permite a essa população a detecção de sons na frequência da fala humana. A detecção de sons pode não ser suficiente para o desenvolvimento da linguagem e requer procedimentos de ensino para que se promova a aprendizagem do uso do dispositivo e o desenvolvimento das habilidades auditivas e expressivas. Pesquisas em interface entre Fonoaudiologia e Análise do Comportamento mostraram que o ensino baseado em equivalência (Equivalence-Based Instruction, EBI) envolvendo a rede de leitura pode aumentar a precisão da fala de crianças com deficiência auditiva pré-lingual que usam IC, de palavras às sentenças. Ainda, a produtividade verbal tem sido demostrada nos estudos prévios por meio da combinação do EBI que inclui o constructed response matching to sample (CRMTS) e ensino por matrizes, o que pode aumentar o controle por unidades menores e o potencial recombinativo. Nessa direção, o presente estudo avaliou os efeitos de dois procedimentos de ensino, baseado em seleção (MTS) e em construção de respostas (CRMTS), sobre a aprendizagem ou fortalecimento das relações condicionais entre sentenças ditadas e impressas, em crianças com IC; ainda, visou verificar se relações derivadas e consistentes com as relações de equivalência são observadas, e avaliar se esse EBI poderia promover o aumento da precisão da fala em tarefas de nomeaçã... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: Pre-lingual sensorineural hearing loss can compromise the development of oral language. The cochlear implant (CI) is an auditory device that allows this population to detect sounds in the frequency of human speech. Sound detection may not be sufficient for language development and require teaching procedures to promote learning how to use the device and develop auditory and expressive skills. Research on the interface between Speech-Language Pathology and behavior analysis has shown that Equivalence-Based Instruction (EBI) involving the reading network can increase the speech accuracy of children with pre-lingual hearing loss who use CI, from words to sentences. Still, verbal productivity has been demonstrated in previous studies through the combination of EBI that includes constructed response matching to sample (CMRTS) and training by matrices, which can increase control by smaller units and the recombinative potential. In this sense, the present study evaluated the effects of two teaching procedures, based on selection (MTS) and the constructed response (CRMTS), on learning or strengthining the conditional relations between dictated and printed sentences, in children with CI, still, it aimed to verify if derived and consistent relations with the equivalence relations are observed, and to evaluate if this EBI could promote the increase of speech accuracy in tasks of naming action figures and recombinative performances. Two children participated, with sensorineural hearing l... (Complete abstract click electronic access below) / Mestre

The presented work deals with the question of achieving financial literacy in pupils with hearing impairment, as one of the important areas of development of civic competencies for life in today's society. The main part of the work is the presentation of the results of a research survey aimed at determining the level of financial literacy in the target group of 15-18-year-old students with hearing impairments who study at secondary schools established for students with hearing impairments in the Czech Republic and their comparison with the results of non-disabled students who were included in PISA financial literacy testing. The research was conducted on three levels. Proven tools from international testing for the quantitative part of the survey and standardized psychological tests were selected, along with school registry data for the part that addresses the qualitative characteristics of pupils. Subsequent analysis of the obtained data served to verify the hypotheses what affects the achieving financial literacy in pupils with hearing impairment. Furthermore, data obtained through the ICT saturation questionnaire, which monitors safety techniques and pupils' relationships and attitudes towards ICT and compares them with participants in PISA testing, were processed and commented on. The aim of...

Syftet med föreliggande studie var att undersöka det sociala samspelet under raster hos grundskoleelever med hörselnedsättning där övriga skolkamrater är hörande. För att besvara studiens syfte och frågeställningar använde vi oss av strukturerad icke-deltagande observation. Vi utgick från ett observationsschema där vi förde anteckningar kring vad som observerades under rasten. Som teoretisk utgångspunkt använde vi oss av det sociokulturella perspektivet samt delaktighetsmodellen. Sammanlagt åtta elever i låg- och mellanstadiet på skolor i olika kommuner observerades. Tidigare forskning inom området har pekat mot olika resultat gällande om eleverna är socialt inkluderade eller inte. Vår studies resultat visar att av de åtta observerade eleverna, har sex av eleverna gott socialt samspel med kamrater medan två elever intog en mer iakttagande roll. / The purpose of this study was to investigate the social interaction during breaks among elementary school students with hearing loss where the other schoolmates are hearing. To answer the study's purpose and questions, we used structured non-participant observation. We started from an observation schedule where we kept notes on what was observed during the breaks. As a theoretical starting point, we used the socio-cultural perspective and the participation model. A total of eight students in primary and middle school at schools in different municipalities were observed. Previous research in the area has pointed to different results regarding whether students are socially included or not. The result of our study shows that of the eight students observed, six of the students have good social interaction with peers, while two students took a more observant role.

The theoretical part of the presented work focuses on the description of the characteristics of a secondary school pupil, hearing impairment and possibilities of education of pupils with hearing impairment in the Czech Republic. Finally, it touches on the issue of distance education, a theme that has currently been very topical. The aim of the practical part of the work was to describe the course of distance education of pupils with hearing impairment in the secondary school for the hearing-impaired during the closure of schools in the academic year 2020/2021. For the purposes of the work, three Czech secondary schools for the hearing-impaired were selected. To achieve the research goal, a qualitative research design was used. Eleven teachers from the selected schools were interviewed. The interviews were then analyzed by open coding. The results of the research showed that from the teachers' point of view, distance learning appears to be demanding, ineffective, and has a number of negative effects on students with hearing impairment such as social separation from the Deaf community they identify with at school. KEYWORDS distance learning, pubescence, interwiew, hearing impairment, secondary school for the hearing-impaired