RESUMO Introdução:É necessário analisar a eficácia da protetização, considerando a necessidade de amplificação e dificuldade na comunicação. Objetivo:Analisar a satisfação de adultos e idosos, usuários de próteses auditivas, com perda auditiva de graus severo e profundo, usando um questionário e pesquisar fatores que possam dificultar a adaptação. Método:Estudo 58,9% mulheres e 41,1% homens, cerca de 55% dos pacientes apresentaram perda auditiva de grau severo e 45% profundo. 87,5% referiu uso maior que 8 horas diárias. Os usuários acreditam que a aquisição da prótese auditiva foi a melhor opção e que esta auxilia muito no entendimento da fala. Sentem-se mais confiantes e capazes com a amplificação sonora e referem bom atendimento pelos profissionais do serviço. O desempenho ao telefone foi o problema encontrado. Conclusão:Foi demonstrado elevado grau de satisfação na população estudada. O grau da perda auditiva, assim como a incompatibilidade de sistemas entre telefone e bobina telefônica e a dificuldade no posicionamento do telefone são fatores que dificultam o bom desempenho ao telefone. A acentuada perda auditiva colabora para que a prótese auditiva seja parte integrante do dia-a-dia e essencial no auxílio à comunicação. ACTRN12610000424000 Palavras-chave:saúde pública, perda auditiva, auxiliares de audição, satisfação do paciente, adulto, idoso. SUMMARY Introduction:It is necessary to analyze the efficiency of the hearing aids use, considering the necessity of enlargement and communication difficulty. Objective:To analyze the satisfaction of adults and elders, users of hearing aids with hearing loss of severe and deep degree, using a questionnaire and searching factors that can difficult the adaptation. Method:A retrospective study of 56 patients, with age group between 18 and 86 years old, hearing loss of severe and deep degree and users of HA by a program of concession of hearing aids, submitted to the questionnaire: "Satisfaction with Amplification in Daily Life". The global average was calculated by sub scale and by question and it was given emphasis to the questions that resulted in best and worse averages. Results:58,9% were women and 41,1% men, about of 55% of the patients presented hearing loss of severe degree and 45% deep degree. 87,5% referred use major than 8 hours daily. The users believe that the acquisition of the hearing aid was the best option and this aid very much in the speech understanding. They feel themselves trustful and able with the sounding amplification and refer a good service by professionals of the service. The performance to the telephone was the problem found. Conclusion:It was demonstrated high degree of satisfaction in the studied population. The hearing loss degree, as well as the incompatibility of systems between telephone and telecoils and the difficulty in the placement of telephone ate factors that difficult the good performance to the telephone. The accentuated hearing loss collaborate for the hearing aid be an integral part of the daily life and essential in the ai...

To describe the value of integrating phenotype/genotype data, disease staging, and evaluation of functional vision in patient-centered management of retinal dystrophies. • METHODS: (1) Cross-sectional structure-function and retrospective longitudinal studies to assess the correlations between standard fundus autofluorescence (FAF), optical coherence tomography, visual acuity (VA), and perimetry (visual field [VF]) examinations to evaluate photoreceptor functional loss in a cohort of patients with rod-cone dystrophy (RCD); (2) flood-illumination adaptive optics (FIAO) imaging focusing on photoreceptor misalignment and orientation of outer segments; and (3) evaluation of the impact of visual impairment in daily life activities, based on functional (visual and mobility) vision assessment in a naturalistic environment in visually impaired subjects with RCD and subjects treated with Luxturna R for RPE65 -related Leber congenital amaurosis before and after therapy. • RESULTS: The results of the cross-sectional transversal study showed that (1) VA and macular sensitivity were weakly correlated with the structural variables; and (2) functional impairment (VF) was correlated with reduction of anatomical markers of photoreceptor structure and increased width of autofluorescent ring. The dimensions of the ring of increased FAF evolved faster. Other criteria that differed among groups were the lengths of the ellipsoid zone, the external limiting membrane, and