Survey of hearing disorders in an urban population in Rondonia, Northern Brazil

OBJECTIVE: To identify the prevalence of hearing loss for the population in the urban area. METHODS: A cross-sectional household survey based on the World Health Organization Ear and Hearing Disorders Survey Protocol was conducted in 298 households in the urban area of Monte Negro, Rondonia, Northern Brazil, from 2005 to 2007. Ear examinations, behavioral audiometry and pure tone audiometry were conducted on 577 individuals. RESULTS: The results showed that 3.8% (95%CI 2.17;5.45) of population were classified in the disabling hearing impairment category. The prevalence of moderate hearing impairment was 3.4%; severe impairment was 0.4%; and profound hearing impairment was not found. CONCLUSIONS: The impairing hearing loss prevalence found in this study is within of the international prevalence for this level of hearing loss and smaller than observed in a previous study in the South region of Brazil.

Contribution of the evaluation strategy and the use of hearing protection for the uncertainty associated with occupational exposure to noise

Doctoral Thesis for PhD degree in Industrial and Systems Engineering

Análise e identificação dos termos matemáticos utilizados no 1º e 2º ciclo do ensino básico e sua correspondência na língua gestual portuguesa

Dissertação de mestrado em Educação Especial (área de especialização em Dificuldades de Aprendizagem Específicas)

A New Large Deletion in the DFNB1 Locus Causes Nonsyndromic Hearing Loss.

Mutations in the GJB2 gene encoding the gap junction protein connexin 26 are responsible for up to 30% of all cases of autosomal recessive nonsyndromic hearing impairment (HI) with prelingual onset in most populations. The corresponding locus DFNB1, located on chromosome 13q11-q12, is also affected by three distinct deletions. These deletions extended distally to GJB2, which remains intact. We report a novel large deletion in DFNB1 observed in a patient presenting profound prelingual HI. This deletion was observed in trans to a GJB2 mutated allele carrying the p.Val84Met (V84M) mutation and was shown to be associated with hearing loss. The deletion caused a false homozygosity of V84M in the proband. Quantification of alleles by quantitative fluorescent multiplex PCR (QFM-PCR) enabled us to study the breakpoints of the deletion. The deleted segment extended through at least 920kb and removed the three connexin genes GJA3, GJB2 and GJB6. The distal breakpoint inside intron 2 of CRYL1 gene differed from the breakpoints of the known DFNB1 deletions. This case highlights the importance of screening for large deletions in molecular studies of GJB2.

Hearing loss in Adult Women with Turner Syndrome

L'objectiu d'aquest estudi és definir els patrons d’hipoacúsia en dones amb Síndrome de Turner i els possibles factors que poden afavorir el desenvolupament d’hipoacúsia neurosensorial en dones adultes amb Síndrome de Turner. Es va trobar que més de la meitat de les dones amb Sindrome de Turner presenten hipoacúsia a l’audiometria, confirmat pels potencials evocats auditius de tronc; la hipoacúsia neurosensorial és el tipus de pèrdua d'audició més freqüent entre dones de mitjana edat amb síndrome de Turner i l'edat, el cariotip i la història prèvia d'otitis mitja recurrent són possibles factors de risc per l’aparició d’hipoacúsia en aquestes pacients.

Prevalence of the A1555G (12S rRNA) and tRNA Ser(UCN) mitochondrial mutations in hearing-impaired Brazilian patients

Mitochondrial mutations are responsible for at least 1% of the cases of hereditary deafness, but the contribution of each mutation has not yet been defined in African-derived or native American genetic backgrounds. A total of 203 unselected hearing-impaired patients were screened for the presence of the mitochondrial mutation A1555G in the 12S rRNA gene and mutations in the tRNA Ser(UCN) gene in order to assess their frequency in the ethnically admixed Brazilian population. We found four individuals with A1555G mutation (2%), which is a frequency similar to those reported for European-derived populations in unselected samples. On the other hand, complete sequencing of the tRNA Ser(UCN) did not reveal reported pathogenic substitutions, namely A7445G, 7472insC, T7510C, or T7511C. Instead, other rare substitutions were found such as T1291C, A7569G, and G7444A. To evaluate the significance of these findings, 110 "European-Brazilians" and 190 "African-Brazilians" unrelated hearing controls were screened. The T1291C, A7569G and G7444A substitutions were each found in about 1% (2/190) of individuals of African ancestry, suggesting that they are probably polymorphic. Our results indicate that screening for the A1555G mutation is recommended among all Brazilian deaf patients, while testing for mutations in the tRNA Ser(UCN) gene should be considered only when other frequent deafness-causing mutations have been excluded or in the presence of a maternal transmission pattern.

Classification and hearing evolution of patients with sudden sensorineural hearing loss

The aim of this study was to analyze clinical aspects, hearing evolution and efficacy of clinical treatment of patients with sudden sensorineural hearing loss (SSNHL). This was a prospective clinical study of 136 consecutive patients with SSNHL divided into three groups after diagnostic evaluation: patients with defined etiology (DE, N = 13, 10%), concurrent diseases (CD, N = 63, 46.04%) and idiopathic sudden sensorineural hearing loss (ISSHL, N = 60, 43.9%). Initial treatment consisted of prednisone and pentoxifylline. Clinical aspects and hearing evolution for up to 6 months were evaluated. Group CD comprised 73% of patients with metabolic decompensation in the initial evaluation and was significantly older (53.80 years) than groups DE (41.93 years) and ISSHL (39.13 years). Comparison of the mean initial and final hearing loss of the three groups revealed a significant hearing improvement for group CD (P = 0.001) and group ISSHL (P = 0.001). Group DE did not present a significant difference in thresholds. The clinical classification for SSNHL allows the identification of significant differences regarding age, initial and final hearing impairment and likelihood of response to therapy. Elevated age and presence of coexisting disease were associated with a greater initial hearing impact and poorer hearing recovery after 6 months. Patients with defined etiology presented a much more limited response to therapy. The occurrence of decompensated metabolic and cardiovascular diseases and the possibility of first manifestation of auto-immune disease and cerebello-pontine angle tumors justify an adequate protocol for investigation of SSNHL.

Développement des habiletés linguistiques chez les enfants porteurs d'un implant cochléaire

Cette thèse examine le développement du langage des enfants sourds qui ont reçu un implant cochléaire (IC) en bas âge. Une première étude rapporte une revue systématique qui avait pour but d’évaluer les connaissances actuelles concernant le développement du vocabulaire et de la grammaire chez les enfants qui ont reçu un IC avant l’âge de trois ans. Vingt-huit études ont été sélectionnées; une analyse descriptive de même qu’une méta-analyse ont été effectuées séparément pour chaque aspect du langage évalué (vocabulaire et grammaire, aspect réceptif et expressif). Au résultat, en dépit de la variabilité observée dans les études, il appert que l’implant cochléaire influence positivement le développement langagier; toutefois, seule une minorité de participants aux études a atteint des niveaux de langage comparables à ceux d’enfants entendants de même âge chronologique. La majorité des enfants continuent de présenter divers degrés de retard de langage, tant au plan réceptif qu’expressif, et ce, après jusqu’à cinq années de port de l’appareil. Les résultats suggèrent aussi, malgré la variabilité observée dans les études, que les bénéfices langagiers sont influencés par le fait de recevoir l’implant à deux ans plutôt qu’à trois ans. À partir des tendances retrouvées dans la littérature, les habiletés de vocabulaire et de grammaire chez 27 enfants qui ont reçu l’implant cochléaire en bas âge (entre 8 et 28 mois) ont été comparées avec celles d’un groupe d’enfants entendants, en utilisant des outils d’évaluation standardisés. Alors que les résultats de groupe montrent que les enfants qui reçoivent un IC autour de l’âge de deux ans atteignent des niveaux de langage dans les limites de la normale, les résultats individuels d’un sous-groupe formé de enfants les plus âgés font état de quatre profils de développement, soit des niveaux de langage dans les limites de la normale pour l’ensemble des composantes, un retard généralisé à l’ensemble des composantes, des habiletés lexicales dans la norme assorti d’un retard morphosyntaxique et enfin un profil atypique montrant des disparités importantes à travers les composantes du langage. Dans trois des quatre profils, la compréhension des phrases était particulièrement faible. Ces résultats suggèrent que le fait de recevoir un implant cochléaire entre l’âge d’un et deux ans ne garantit pas l’atteinte de niveaux de langage dans les limites de la normale malgré une expérience de port de l’appareil d’une durée appréciable. Une étape antérieure du développement linguistique a été examinée de plus près dans la troisième étude. La taille et la composition du vocabulaire expressif de onze enfants ayant reçu un IC à un âge moyen de 15 mois ont été comparées à celles de l’échantillon d’enfants entendants ayant servi à établir les normes en français québécois pour le questionnaire Mots et énoncés des Inventaires MacArthur-Bates du développement de la communication (IMBDC). Les scores d’âge équivalent selon la taille totale du vocabulaire des enfants avec IC étaient supérieurs à l’âge auditif (correspondant à la durée de port de l’appareil) mais inférieurs à l’âge chronologique. La représentation grammaticale en fonction de la taille du vocabulaire des enfants avec IC suit la tendance observée dans la norme. Ces résultats suggèrent que le profil lexical des enfants avec implant est très similaire à celui des enfants entendants lorsque le nombre total de mots acquis est le même. Les résultats de cette thèse suggèrent que l’implant peut, de manière générale, avoir un effet « normalisant » sur le langage ; toutefois, il semble que l’amélioration de l’accès auditif ne suffise pas pour rattraper à coup sûr le niveau de langage des pairs entendants dans l’ensemble des composantes du langage. Alors que les habiletés lexicales se rapprochent du profil typique, les habiletés de compréhension morphosyntaxique sont fortement atteintes chez une majorité d’enfants, suggérant un profil apparenté à un trouble de langage.

Évaluation de la production morphosyntaxique chez un enfant québécois francophone porteur d'implant cochléaire.

Cette étude décrit le développement des structures morphosyntaxiques et morphophonologiques chez les enfants francophones âgés entre 3 et 6 ans. Ces données pourront contribuer à particulariser certaines difficultés morphosyntaxiques et morphophonologiques retrouvées chez l’enfant déficient auditif québécois porteur d’implant cochléaire. Le premier objectif de notre projet pilote vise à comparer les habiletés morphosyntaxiques et morphophonologiques au niveau expressif de l’enfant porteur d’IC à celles des enfants entendants de même âge auditif et chronologique. L’étude évalue spécifiquement l’accord intra-nominal en genre, et les processus de fusion, d’élision et de liaison. Nous prédisons qu’une entrée auditive inférieure à la norme aura un impact sur l’acquisition des règles morpho(phonolo)giques en français. Le deuxième objectif consiste à observer si la maîtrise de ces structures est liée à la maîtrise de la production phonémique chez l’enfant franco-québécois porteur d’IC. L’élaboration de deux tâches expérimentales et la passation de tâches évaluatives et expérimentales ont permis d’étudier les difficultés morphologiques et phonologiques de l’enfant porteur d'IC. Le groupe témoin a inclus 14 enfants à développement typique. Ils ont été comparés au cas de Vincent, âgé de 59 mois, porteur d’implant cochléaire. Ce dernier présente des étapes de développement linguistique décalées qui correspondent à celles d’enfants appariés sur l’âge auditif (date d’IC) plutôt qu’à l’âge chronologique (AC). Nous avons observé des similitudes et des différences, sur le plan phonologique et morphosyntaxique, entre Vincent et les enfants entendants : il présente des performances significativement moins bonnes que la norme pour certaines de structures morphosyntaxiques et processus morphophonologiques (accord du genre, élision, fusion) mais meilleures que les témoins dans la tâche de liaison. Nous pensons que le gain prothétique n’est pas le seul facteur qui a un impact sur le développement linguistique précoce et que d’autres facteurs l’influenceraient, tels l’âge d’implantation, le mode de communication, l’implantation bilatérale, l’investissement des parents et les effets d’apprentissage. Enfin, notre étude de cas n’a pu étayer si la maîtrise de la production phonémique est en relation avec la maîtrise de certaines structures et processus morphologiques.

As atitudes dos professores face à inclusão de alunos com deficiência : o contacto com a deficiência

RESUMO: Actualmente as práticas de exclusão evoluíram para uma perspectiva de inclusão, assim como para a consciencialização dos direitos e deveres de cada um, como forma de dar resposta à sociedade heterogénea existente. A visão baseada nos sistemas de identificação e classificação dos sujeitos em várias categorias de deficiências era algo muito usual, mas que foi abolida, dando assim lugar ao conceito de Necessidades Educativas Especiais, com uma óptica mais abrangente, tendo em conta o contexto em que o sujeito está envolvido (Nunes, 2000). As atitudes dos professores face aos alunos com deficiência têm melhorado significativamente (Ribeiro, 1999), no entanto o processo de inclusão destas crianças no ensino regular não está isento de problemas. Neste sentido, e para que este desafio seja ultrapassado com sucesso, torna-se essencial que os professores modifiquem as suas atitudes e passem a desempenhar um papel mais activo nas suas funções, devendo para isso, começar por adaptar o currículo, e posteriormente repensar as suas estratégias e métodos de trabalho, como forma a responder às necessidades de todos os alunos (Ainscow, 1997). O objectivo principal deste estudo é verificar se o contacto com a deficiência (a nível da experiência no ensino, formação inicial e contacto na infância/juventude), por parte dos professores, influencia as suas atitudes em relação à formação necessária para a inclusão de alunos com deficiência, bem como às vantagens que esta representa para esses mesmos alunos. A amostra foi constituída por 672 professores do ensino regular, todos estão actualmente no activo e leccionam níveis de ensino do Pré-Escolar ao Ensino Secundário, de Norte a Sul do país. (N = 482 do género feminino e N =190 do género masculino). O instrumento de avaliação aplicado foi o questionário APIAD – Atitude dos Professores face à Inclusão de Alunos com Deficiência (Leitão, 2011). Concluiu-se que a experiência no ensino de alunos com deficiência influencia significativamente a atitude dos professores face à formação necessária (deficiência motora: p<0,001; deficiência auditiva: p<0,001; deficiência visual: p<0,001; deficiência mental: p=0,004) e face às vantagens da inclusão para os alunos com deficiência (deficiência motora: p=0,005; deficiência auditiva: p<0,001; deficiência visual: p<0,001; deficiência mental: p=0,022). No que se refere ao contacto com pessoas com deficiência durante a formação inicial, concluiu-se que existem diferenças significativas na atitude dos professores face às vantagens da inclusão para os alunos com deficiência (deficiência motora: p<0,001; deficiência auditiva: p<0,001; deficiência visual: p<0,001; deficiência mental: p<0,001). No entanto, no que respeita à formação, a atitude dos professores não difere, independentemente de terem tido esse contacto (deficiência motora: p=0,393; deficiência auditiva: p=0,456; deficiência visual: p=0,055; deficiência mental: p=0,342). Relativamente ao contacto com pessoas com deficiência durante a infância/juventude conclui-se que não existem diferenças na atitude dos professores em relação à formação necessária (deficiência motora: p=0,893; deficiência auditiva: p=0,667; deficiência visual: p=0,459; deficiência mental: p=0,918). Por sua vez, no que respeita às vantagens da inclusão para os alunos com deficiência, esta variável só influencia significativamente a atitude dos professores no caso da deficiência visual (deficiência motora: p=0,154; deficiência auditiva: p=0,100; deficiência visual: p=0,045; deficiência mental: p=0,149). ABSTRACT: Currently the exclusionary practices evolved to an inclusion perspective, as well as the awareness of rights and duties of each one as a way to reply to the existing heterogeneous society. The vision-based systems for identification and classification of subjects into various categories of disabilities was very unusual, but it was abolished, giving way to the concept of Special Educational Needs, with a broader perspective, considering the context in which the subject is involved (Nunes, 2000). The teachers attitude face to the students with disabilities have improved significantly (Ribeiro, 1999), however the process of inclusion of these children in regular education isn't exempt of problems. In this direction and so this challenge is exceeded successfully, it is essential that teachers change their attitudes and start to perform a more active role in their functions, and to do so, start by adapting the curriculum and then rethink their strategies and working methods, in order to meet the needs of all students (Ainscow, 1997). The main purpose of this study is to verify that the contact with the disability (educational level of experience, initial formation and contact in childhood/youth), among teachers, influences their attitudes towards the needed formation for the inclusion of students with disabilities as well as the benefits that this represents for them. The sample consisted by 672 regular educational teachers, all currently in employment and teaching from Preschool to High school, from North to South. (N = 482 females and N = 190 males). The evaluation instrument used was the survey APIAD - Teachers attitude towards the inclusion of students with disabilities (Leitão, 2011). It was concluded that the experience in teaching students with disabilities influences significantly the teachers attitude faced to the necessary formation (motor disability: p<0,001; hearing impairment: p<0,001; visual impairment: p<0,001; mental disability: p=0,004) and faced to the inclusion benefits for students with disabilities (motor disability: p=0,005; hearing impairment: p<0,001; visual impairment: p<0,001; mental disability: p=0,022).Concerning to the contact with people with disabilities during the initial formation, it was concluded that there are significant differences in the teachers attitude face to the inclusion benefits for students with disabilities (motor disability: p<0,001; hearing impairment: p<0,001; visual impairment: p<0,001; mental disability: p<0,001). In relation to the formation, the teachers attitude is the same, regardless of whether or not they have had such contact (motor disability: p=0,393; hearing impairment: p=0,456; visual impairment: p=0,055; mental disability: p=0,342). Regarding to the contact with people with disabilities during childhood/youth, it was concluded that there is no difference in the teachers attitude in relation to the formation needed (motor disability: p=0,893; hearing impairment: p=0,667; visual impairment: p=0,459; mental disability: p=0,918). On the other way, regarding to the inclusion benefits for students with disabilities, this influences significantly the teachers attitude just in the visual impairment. (motor disability: p=0,154; hearing impairment: p=0,100; visual impairment: p=0,045; mental disability: p=0,149).

Communication strategies used by children at a school for the deaf

This paper discusses a study to determine the communication strategies used by hearing impaired children and their effectiveness.

Social skills training in deaf and hard of hearing preschoolers

This paper addresses the importance of the development of social skills for deaf and hard of hearing preschool children. The author presents social skills lessons and activities for teachers to use with preschool children.

Vacation studies for deaf children

This paper discusses means of rehabilitation for hearing impaired children during the summer.

Assessing phonemic awareness skills in hearing impaired students

This paper discusses phonemic awareness and its role in providing appropriate literacy instruction to both hearing and hearing impaired children. The ability of hearing-impaired students to gain phonological awareness skills is examined and instructional models for teaching phonological and phonemic awareness skills are presented. Also included is a checklist to be used to assess phonological and phonemic awareness skills in hearing-impaired students.