BACKGROUND: Hearing loss is the third most prevalent chronic condition faced by older adults and has been linked to difficulties in speech perception, activities of daily living, and social interaction. Recent studies have suggested a correlation between severity of hearing loss and an individual's cognitive function; however, a causative link has yet to be established. One intervention option for management of the most severe to profound hearing loss in older adults is cochlear implantation. We performed a review to determine the status of the literature on the potential influence of cochlear implantation on cognition in the older adult population. METHODS: Over 3800 articles related to cochlear implants, cognition, and older adults were reviewed. Inclusion criteria were as follows: (1) study population including adults > 65 years, (2) intervention with cochlear implantation, and (3) cognition as the primary outcome measure of implantation. RESULTS: Out of 3,886 studies selected, 3 met inclusion criteria for the review. CONCLUSIONS: While many publications have shown that cochlear implants improve speech perception, social functioning, and overall quality of life, we found no studies in the English literature that have prospectively evaluated changes in cognitive function after implantation with modern cochlear implants in older adults. The state of the current literature reveals a need for further clinical research on the impact of cochlear implantation on cognition in older adults.

Introduction: This study investigated hearing aid (HA) satisfaction among adult with hearing impairment (HI) in New Zealand. This study aimed to answer three questions: 1) What are the current HA satisfaction levels amongst adult HA users in New Zealand? 2) How do the satisfaction findings of this study compare with other HA satisfaction data? 3) What client factors are related to HA satisfaction? Method: Participants were recruited prospectively. They completed a questionnaire prior to HA fitting and a questionnaire three months post-fitting. Information was collected on: age, gender, HA experience, HI severity, hearing ability, change in hearing ability, hearing handicap, communication self-efficacy, change in communication self-efficacy, HA self-efficacy, HA usage, and number of appointments. HA satisfaction was measured via the Satisfaction with Amplification in Daily Life questionnaire (SADL; Cox & Alexander, 1999). Results: Data were collected for 47 participants. Of these, 91.5% fell within or above the normative range for global satisfaction established by Cox & Alexander (1999). The mean SADL scores were predominantly high compared to previous research. Satisfaction with negative features of HAs was especially high in this study. However satisfaction with the service and cost of HAs was low compared to other research. SADL scores were found to significantly relate to age, gender, change in hearing ability, hearing handicap, communication self-efficacy, change in communication self-efficacy, and HA self-efficacy. Conclusions: Results differed from previous research indicating that HA satisfaction may differ over time and across countries. Assessing HA satisfaction in a comprehensive standardised way, as opposed to with a single-item measure, can help identify important related factors. Targeting identified variables such as communication and HA self-efficacy may lead to improved treatment efficacy.

This thesis examined phonological processing in adults with postlingually acquired moderate-to-severe hearing impairment (HI) and whether explicit working memory processing of phonology and individual working memory capacity (WMC) can compensate for degraded phonological representations in this group (papers I-III). A second aim was to provide reference data for a test of WMC, the reading span test, and to examine the relation between reading span test performance and speech recognition in noise in a larger sample of 50-89 year olds with HI (paper IV). Non-auditory tasks of phonological processing, episodic long-term memory and WMC were used in papers I-III, and both behavioral and electrophysiological measures were collected. Results showed that phonological processing was impaired in the group with HI but that WMC and explicit working memory processing of phonology could be employed to compensate for degraded phonological representations. However, this compensation may come at the cost of interfering with episodic memory encoding. An  electrophysiological marker of HI in text-based rhyme judgments was found. Paper IV presented reference data for reading span test performance in two versions of the test in individuals with HI, and results suggesting that WMC may be differentially predictive of speech recognition in noise in different age groups of older adults with HI. The clinical implications of the present results concerns the double disadvantage of individuals with lower WMC and HI. A structured assessment of WMC in rehabilitative settings would help to identify these individuals and tailor treatment to their needs. The reading span test is suggested as a suitable future candidate for clinical WMC assessment. / I denna avhandling undersöktes fonologiska processer hos personer med postlingualt förvärvad, måttlig till mycket grav hörselnedsättning (HNS) och om inkoppling av explicit bearbetning av fonologi eller individuell arbetsminneskapacitet kan kompensera för försämrade ljudminnen i denna grupp (studie I-III). Ett andra syfte var att sammanställa referensdata för ett test av arbetsminneskapacitet, lässpann, och undersöka relationen mellan prestation på lässpann ochigenkänning av tal-i-brus i en större grupp av 50-89 åriga personer  med HNS (studie IV). Icke-auditiva test av fonologisk förmåga, episodiskt långtidsminne och arbetsminneskapacitet användes i studie I-III och både beteendedata och elektrofysiologiska data samlades in. Resultaten visade på försämrad fonologisk förmåga i gruppen med HNS men också att explicit bearbetning av fonologi och arbetsminneskapacitet kunde användas för att kompensera för försämrade fonologiska representationer i denna grupp. Denna kompensation kan dock ske på bekostnad av att inkodning i episodiska långtidsminnet störs. Vidare identifierades en elektrofysiologisk markör för HNS i en textbaserad rimuppgift. I studie IV presenterades referensdata i två versioner av lässpanntestet för personer med mild till mycket grav HN och resultat som pekar mot möjliga skillnader mellan olika åldersgrupper i hur väl arbetsminneskapacitet predicerar igenkänning av tal-i-brus. Kliniska implikationer rör den dubbla belastning det innebär att ha både en HN och relativt sänkt arbetsminneskapacitet. En strukturerad bedömning av arbetsminneskapacitet i samband med rehabilitering skulle kunna bidra till att synliggöra dessa personer och anpassa insatser efter deras behov. Lässpanntestet är ett test som i framtiden skulle kunna vara användbart i det sammanhanget.

1 Summary The thesis deals with the quality of everyday life of the hearing impaired. Part of the text defines the concept of the quality of life and points out the present common problems concerning the life of hearing impaired people. The issues of education, job opportunities, identity and personal realization of a hearing impaired individual in the context of the current view of the quality of life are highlighted. The text also focuses on the heterogeneity of the hearing impaired population and emphasizes their different status in mainstream society relating to the communication code they use. The goal of this thesis is to bring closer the problems of the quality of life of the hearing impaired, with the emphasis on the current problem areas which need further improvement. The practical part contains an analysis and evaluation of a questionnaire survay focused on specific areas of the quality of life quality of the hearing impaired - the qualification, employability, job opportunities and ways of communication. The final part of the practical part contains the commentaries on these reasearch results. Key definitions: Quality of life Hearing impairment Communication codes Minority

Tourism has become a major part of today's society and has been seen as a human privilege. The expansion of tourism makes travel more accessible to more people. Still, people with a disability can face difficulties when traveling for the purpose of tourism. Information becomes important to accomplish accessible tourism for everyone, but the problem in various tourism disciplines occurs with the lack of knowledge about people with disabilities.  Tourism has different disciplines. Transportation is one of the basics of making tourism accessible around the world. Transportation has a major impact on tourism development but also in tourist experiences. If the transportation mode is not accessible, it can be a negative experience for people with a disability and can affect the entire journey and their willingness to attempt further trips. Disability is a broad definition and it is important to understand that different disabilities require different needs and that everyone is individual. This study analyzes how hearing loss affects travelers and the understanding of; (1) What may be needed to assist people with hearing impairments when traveling; (2) How does traveling affect people with hearing impairment when they do not have accessibility? The method is a qualitative method with in-depth interviews because this study wants to understand and reflect participants’ experiences when traveling with a hearing impairment.

The diploma thesis deals with the problem of phonemic differentiation in preschool children. The introductory part is focused on the specifics of preschool age, ontogenesis of speech and characterization of phonemic hearing. The aim of this thesis is to determine the level of phonemic differentiation in children with cochlear implant and children without hearing impairment. The results were obtained using a standardized test battery Evaluation of Phonemic Hearing in Preschool Children by authors Škodová, Mischek and Moravcová from 1995, which is created for this issue. Subsequently, the results of both groups were compared and individual differences in performance between children with cochlear implant and children without hearing impairment were examined. Research has shown that better results in phonemic distinction amounted children without hearing impairment, although the difference in success rate between the two groups was not significant. The main benefit of this work is the fact that the ability of phonemic differentiation is important in children with cochlear implant and its development should be part of rehabilitation care. KEYWORDS phonemic hearing, preschool age, cochlear implant, hearing impairment

O objetivo do presente estudo foi avaliar a efetividade da capacitação dos agentes comunitários de saúde, por meio da videoconferência, na área de saúde auditiva infantil. Participaram da pesquisa 50 profissionais divididos aleatoriamente em dois grupos de acordo com a metodologia de ensino: 31 agentes comunitários de saúde participaram da capacitação de forma presencial e 19 por meio de videoconferência. A atividade ocorreu em um encontro de oito horas, por meio de aulas expositivas sobre o conteúdo ministrado e a utilização do material adaptado Primary ear and hearing care training resource basic, intermediate and advanced levels (WHO, 2006) proposto pela Organização Mundial da Saúde, de forma simultânea para ambos os grupos. A capacitação contemplou os temas: Audição e deficiência auditiva; Tipos, prevenção e causas da deficiência auditiva; Técnicas de identificação e diagnóstico da deficiência auditiva e Aspectos gerais da deficiência auditiva. A efetividade da capacitação por meio da videoconferência foi avaliada por meio de questionário pré e pós-capacitação aplicado aos agentes comunitários de saúde (WHO, 2006). Adicionalmente, outro questionário foi aplicado a fim de verificar a qualidade da capacitação e satisfação dos participantes. Os resultados demonstraram que ambos os grupos apresentaram diferença estatisticamente significante quando considerado o desempenho apresentado no instante pré e póscapacitação, de forma mais expressiva para o grupo que participou da capacitação de forma presencial, tanto na análise do escore total como quando considerado os temas abordados separadamente. A análise qualitativa da videoconferência como metodologia de ensino comparada a capacitação presencial, de acordo com a visão dos ACS foi muito semelhante, sendo considerada de fácil entendimento permitindo a interação com o ministrante. A videoconferência como metodologia de ensino é efetiva para a capacitação dos agentes comunitários de saúde na área de saúde auditiva, porém deve ser utilizada de forma complementar à capacitação realizada de forma presencial. / The present work aimed to verify the effectiveness of a training program for health care professionals, by videoconference, in infantile hearing health Program. The 50 professionals, divided randomly in two groups, constituted the sample: 31 health care professionals to face-to-face instruction and 19, by videoconference. The training was provided in eight hours, through classes and a textbook adapted from the material Primary ear and hearing care training resource basic, intermediate and advanced levels (WHO, 2006), by World Health Organization in both groups, simultaneously. The topics focused were as follows: normal and impaired hearing; types, prevention and causes of the hearing impairment; procedures to identify and diagnose the hearing impairment and global aspects of hearing impairment. To investigate the effectiveness of a training program, was conduced an application to the pre and post-questionnaire in the health care professionals (WHO, 2006). Another questionnaire was applied to evaluate the training quality and participants satisfaction. The results obtained showed, in both groups, an increase in the score in the post questionnaire, as compared to the one initially applied. However, the performance was more expressive in the group trained face-to-face, considering total and topics scores isolated. The quality evaluation of videoconference, compared by face-to-face instruction, in the opinion of health care professionals was similar, and was considered easy and interactive. This study allowed us to conclude that the videoconference, as learning tool, is effective for health care professionals training in infantile hearing Program, but must be utilized complementary a face-to-face instruction.

A herança autossômica recessiva pode ser responsável por aproximadamente 77% dos casos de surdez hereditária. Em 1996, Chaib e col. mapearam o loco responsável por surdez profunda neurossensorial de herança recessiva na região cromossômica 2p22-23 (DFNB9). Em 1999, Yasunaga e col. identificaram esse gene como o que codifica a proteína OTOFerlina (OTOF) nessa região. Até hoje, já foram descritas 31 mutações patogênicas diferentes no gene OTOF em populações de várias origens, com destaque a mutação Q829X que foi encontrada em ~3% dos casos de surdez na Espanha (Migliosi e col., 2002; Rodríguez-Ballesteros e col., 2003). Alguns pacientes com mutações no gene OTOF apresentavam neuropatia auditiva, um tipo de deficiência auditiva neurossensorial caracterizada pela ausência ou anomalia das ondas no exame dos Potenciais Evocados Auditivos do Tronco Encefálico ou BERA com a presença das emissões otoacústicas e/ou microfonismo coclear. O objetivo desse projeto foi investigar a contribuição relativa das mutações no gene OTOF ao casos de neuropatia auditiva e de outros tipos surdez em famílias brasileiras. Uma amostra de 343 propósitos portadores de deficiência auditiva foi submetida ao estudo da mutação Q829X. Não foi identificada em nenhum caso. Dessa casuística foram selecionados 48 propósitos de famílias com consangüinidade ou com 2 ou mais afetados na irmandade e quatro pacientes com neuropatia auditiva e com consangüinidade parental ou com dois ou mais afetados na irmandade. Além disso, foram também selecionados 7 casos isolados com neuropatia auditiva e 5 casos de portadores de alterações no tronco encefálico. Essa amostra totalizou 64 propósitos. Propósitos dessas 64 famílias foram genotipados em relação a cinco marcadores de microssatélites ligados ao gene OTOF. A análise dos haplótipos excluiu ligação ao gene OTOF em 34 casos, 19 não eram conclusivos e 11 indicaram possibilidade de ligação ao gene OTOF (incluindo uma família com pais consangüíneos e neuropatia auditiva e três propósitos com neuropatia auditiva). Simultaneamente, os 64 propósitos foram triados para mutações em oito exons do gene OTOF, nos quais mutações já haviam sido descritas, por meio de SSCP seguido de seqüenciamento. Os 11 casos com resultados compatíveis com ligação ao gene OTOF (4 com neuropatia auditiva) e os sete casos de neuropatia auditiva foram selecionados para o seqüenciamento de todos os exons (total de 18 propósitos). Identificamos no total 58 alterações diferentes. Onze variantes eram potencialmentes patogênicas, encontradas em sete dos propósitos, todos pertencentes ao grupo dos 18 selecionados. Quatro casos eram heterozigotos compostos [98G>A (R33Q) e 2401G>T e 2402A>T (E801L)]; [1841G>A (G614E) e 3239G>C (R1080P)], [3751T>G (C1251G) e 5431A>T (K1811X)] e [2348delG (G783fs) e 5800-5801insC (L1934fs)], dois eram heterozigotos [1552-1567del16 (R518fs); 2905- 2923del19ins11 (A969fs)] sem que uma segunda mutação fosse detectada e um apresentava a mutação em homozigose [3400C>T (R1134X)]. Desses sete propósitos com mutações patogênicas, somente um paciente com mutação em heterozigose não apresentava neuropatia auditiva. Dentre os 11 casos com neuropatia auditiva, seis tinham pelo menos uma mutação no gene OTOF que poderia ser a causa de surdez. Esse achado reforça a associação entre o fenótipo da neuropatia auditiva e mutações no gene OTOF. A variante Q829X não foi encontrada nenhuma vez em nossa amostra, portanto, não deve ser causa importante de surdez na nossa população. Porém, nosso estudo mostra que mutações no gene OTOF são causas freqüentes de neuropatia auditiva no Brasil (mais de 50% dos casos). Nossos resultados reforçam a hipótese que pacientes com neuropatia auditiva devem ser selecionados para pesquisa de mutações no gene OTOF e que talvez mais de 50% dos casos de neuropatia auditiva tenham causa genética. / 77% of nonsyndromic prelingual deafness have an autosomal recessive inheritance. In 1996, Chaib et al. mapped a locus associated with sensorineural nonsyndromic recessive deafness to chromosome region 2p22-23 (DFNB9) by linkage studies. In 1999, Yasunaga et al. identified the OTOF gene encoding OTOFerlin, in this region. To date, there are 31 different pathogenic mutations described in the OTOF gene, from populations of variable origins. A Q829X mutation was found at a frequency of ~3% of deafness in Spain (Migliosi e col., 2002; Rodríguez-Ballesteros e col., 2003). Some affected individuals with mutations in the OTOF gene were reported to present auditory neuropathy, a type of deafness characterized by an absent or severely abnormal auditory brainstem response, with preservation of otoacoustic emissions and/or cochlear microphonics. The main purpose of this project was to investigate the relative contribution of OTOF mutations to auditory neuropathy and other type of deafness, amongst Brazilian families. We enrolled 343 Brazilian unrelated subjects with nonsyndromic hearing loss. A specific test for the Q829X mutation was performed first. We failed to find any subjects carrying this mutation. From this group, we selected 48 probands from families with consanguinity or with two or more affected sibs and four probands with diagnosis of auditory neuropathy and from consanguineous unions or with two or more affected sibs. In addition, we selected 7 isolated subjects with auditory neuropathy and 5 cases with diagnosis of brainstem alteration. This gave a total of 64 probands. Subjects from the 64 families were genotyped for five microsatellites markers, linked to the OTOF gene. The analysis of the haplotype excluded linkage to the OTOF gene in 34 families, it was inconclusive in 19 families and it showed compatibility with linkage in the remaining 11 families (including one with consanguineous parents and auditory neuropathy and three with diagnosis of auditory neuropathy). Simultaneously, the 64 subjects were screened for mutations in 8 exons previously identified to other mutations using the SSCP technique. In positive cases, DNA sequencing was carried out. In the 11 subjects consistent with putative linkage to OTOF gene and the 7 isolated cases of auditory neuropathy, an exon by exon screening for mutations in the OTOF gene was performed using DNA sequencing (Total of 18 subjects). We found a total of 58 different variants. Eleven were possibly causative mutations and were found in seven of the 18 subjects. Amongst them, four cases were compound heterozygotes R33Q with E801L, G614E with E1080P, 2348delG with 5800-5801insC and K1811X with C1251G, two cases were heterozygotes [1552- 1567del16 and 2905-2923del19in11] without a second mutation and one presented a mutation in homozygous form [3400C>T (R1134X)]. Among these seven probands, only one patient with a heterozygote mutation did not have a diagnosis of auditory neuropathy. In the 11 cases of auditory neuropathy, six had at least one mutation in the OTOF gene that is the probable cause of their deafness. These findings support the association between auditory neuropathy and mutations in the OTOF gene. While we failed to confirm the high frequency of Q829X mutation found in Spain, our study shows that mutations in the OTOF gene are frequent causes of auditory neuropathy in Brazil (more than 50%). Our results reinforced that patients with auditory neuropathy must be selected for mutation detection in the OTOF gene and that more than 50% of cases of auditory neuropathy have a defined genetic etiology.

A educação em saúde odontológica depende da comunicação, que garanta de fato o conhecimento e adoção das medidas preventivas no cotidiano das crianças, sendo de extrema importância quando se deseja mudar atitudes em relação á doença, priorizando a promoção de saúde. Os programas educativos estão previstos na Política Nacional de Saúde Bucal e devem contemplar o acesso universal a todos os cidadãos. Neste contexto é necessário reconhecer o preparo dos profissionais de odontologia quanto às práticas de comunicação e informação utilizadas para desenvolver hábitos adequados de manutenção da saúde bucal com pacientes com perda de audição.A perda auditiva é um déficit sensorial muito comum e causa grande desvantagem de comunicação. A falta de comunicação leva á desigualdades no acesso á saúde e o desconhecimento sobre cuidados com a saúde bucal representa um fator a ser considerado. Este estudo se propôs a avaliar o conhecimento sobre saúde bucal de pais e professores em escolas especiais de educação para crianças surdas que se comunicam através da Língua Brasileira de Sinais (Libras), e o conhecimento de cirurgiões-dentistas sobre as questões relacionadas ás estratégias de comunicação com pacientes surdos. A conclusão deste estudo enfatiza a necessidade de programas educativos que contemplem ás especificidades de uma criança surda através de parcerias estabelecidas entre a escola, pais e o cirurgião-dentista, para garantir uma comunicação efetiva e a aquisição do conhecimento sobre saúde bucal. / Oral health education depends on communication that can indeed ensure understanding and adoption of preventive measures on the day-to-day of children, and it is extremely important to change attitudes in relation to diseases, prioritizing the promotion of health. Educational programs are included in the National Oral Health Policy and should focus on universal access to all citizens. In this context, it is necessary to recognize how dental professionals are prepared in the practices of communication and information used to develop suitable habits to maintain the oral health of patients with hearing loss. Hearing loss is a very common sensorial deficit and causes major impairment to communication. Lack of communication leads to inequalities in access to health and unawareness about oral health can be a factor to be considered. This study aimed at assessing the knowledge of parents and teachers about oral health in special schools of education for deaf children that communicate in Libras (Brazilian Sign Language), and dentists understanding of issues related to communication strategies with deaf patients. The conclusion of this study emphasizes the need for educational programs that can contemplate the specificities of a deaf child by means of partnerships established between the school, parents and dentists, so as to ensure effective communication and the acquisition of knowledge on oral health.

A família é sempre um fator de grande importância no processo de habilitação da criança com implante coclear. O desenvolvimento da linguagem depende do envolvimento dos pais no processo de habilitação e exige um compromisso contínuo, para que a criança com deficiência auditiva tenha benefícios com o seu implante coclear. A família tem assumido uma sobrecarga significativa ao longo do processo, trazendo repercussões na vida familiar e nas próprias necessidades e interesses. Nesse contexto, o objetivo deste estudo é compreender a vivência de mães, na trajetória da infância à adolescência de seus filhos com implante coclear, buscando apreender os significados e sentidos atribuídos por elas nessa experiência. O estudo foi de natureza qualitativa e o método utilizado foi a investigação fenomenológica, que consiste na apreensão do fenômeno tal como ele se manifesta para a pessoa que o vivencia. Foram entrevistadas 12 mães das primeiras crianças que realizaram a cirurgia, no programa de implante coclear do Centro de Pesquisas Audiológicas do Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo. Os depoimentos, mediados por uma questão norteadora e submetidos ao processo de análise fenomenológica, revelaram as seguintes categorias temáticas: A ressonância subjetiva do diagnóstico; Conhecendo o implante coclear e tomando decisões; A vivência da angústia na cirurgia; O nascimento da audição; Vivenciando o cotidiano no processo de habilitação; A experiência de momentos críticos no percurso da habilitação; Relações familiares; A fase escolar: uma nova etapa de enfrentamento; O processo do adolescer vivenciado com superproteção e com novos desafios; O apoio do psicólogo, da equipe e outros; Temporalidade e projetos de vida. Foi utilizado o referencial teórico de Romero, que nos levou à compreensão da vivência dessas mães, a partir da afetividade, que é o modo como elas são afetadas subjetivamente na sua relação com o mundo e, nessa relação, mostraram como se vinculam e o significado que elas dão ao que vem ao seu encontro, revelando o sentido de sua existência com um filho com implante coclear. / The family is always a major important factor in the habilitation process of the child with cochlear implant. The development of language depends on the parental involvement in the habilitation process and requires a continuous commitment, so that the child with hearing impairment may be benefited by the cochlear implant. The family has been assuming a significant overload during the process, causing repercussions in the family life and the needs and interests. Within this context, this study aimed to understand the experience of mothers in the trajectory of infancy to adolescence of their children with cochlear implants, aiming to identify the meanings and feelings assigned by them in this experience. The study was qualitative and the method comprised phenomenological investigation, which consists of apprehension of the phenomenon as it is manifested to the person experiencing it. The interviews were applied to 12 mothers of the first children submitted to surgery in the cochlear implant program of the Center for Audiological Research of the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo. The reports, mediated by a guiding question and submitted to the phenomenological analysis process, revealed the following thematic categories: the subjective resonance of diagnosis; knowing the cochlear implants and making decisions; the experience of anxiety in the surgery; the birth of hearing; experience the daily life in the habilitation process; the experience of critical moments in the habilitation process; family relationships; the school age: a new stage of coping; the process of adolescing experienced with overprotection and new challenges; the support of the psychologist, staff and others; temporality and life projects. The theoretical reference of Romero was used, which led us to understand the experience of these mothers, based on the affectivity, which is the way through which they are subjectively affected in their relationship with the world and, in that relationship, they showed how they link themselves and the meaning they assign to what comes to them, revealing the meaning of their existence with a child with cochlear implant.