Understanding the impacts of the COVID-19 response measures on deaf adults in Cape Town

Background: International literature has evidenced that Deaf people have been disadvantaged during the COVID-19 pandemic; however, there is currently little research published within the South African context. Objectives: This study investigated the ways in which the COVID-19 pandemic and its consequent response measures impacted Deaf adults in Cape Town. Method: Using a descriptive approach, semi-structured, qualitative interviews were held with 15 Deaf adults in Cape Town, South Africa. Participants were purposively selected through a local Deaf organisation. Data were analysed using thematic analysis. Results: Data revealed the challenges experienced when accessing information, the impact of communication barriers on daily life, and how the response measures impacted access to healthcare. Conclusion: The findings of this study demonstrate how the needs of the Deaf community were overlooked and their voices disregarded during the planning of the national pandemic response, ultimately having detrimental consequences. Therefore, the authors argue for greater inclusion of Deaf representatives to ensure equal access to information and resources, especially during a crisis. Contribution: This study contributes to the growing body of knowledge on the consequences of the COVID-19 pandemic in the field of disability and insights can inform both future research and interventions to promote equity and inclusion for Deaf people.

Em Defesa de Escolas Bilíngues de Surdos: Interlocuções dos Movimentos de Resistência em Portugal e Brasil / In Defense of Bilingual Schools for the Deaf: Interlocutions of Resistance Movements in Portugal and Brazil

RESUMO: Este trabalho parte de uma aproximação entre estudos realizados no Brasil e em Portugal, cenários em que as lutas pelo reconhecimento das pessoas surdas como participantes de uma comunidade linguística minorizada vêm pautando as discussões em defesa de uma escola bilíngue. Olha-se para os dois contextos a partir do entendimento da escola como lugar do encontro com a diferença, dando atenção no que se aproximam e, também, nas suas singularidades, dadas suas condições históricas, políticas e sociais. Realizaram-se interlocuções entre os movimentos de resistência pela manutenção dos espaços "Escola" - Escolas de Surdos (Brasil) e Escolas de Referência para Educação Bilíngue de Alunos Surdos - EREBAS (Portugal) - como também aproximação aos discursos de estudantes surdos, com o objetivo de olhar as potencialidades desses espaços possibilitarem experiências de ser Escola. Os estudantes — sujeitos da experiência — perceberam que a identificação linguística proporcionada pela Escola de Surdos/ EREBAS está relacionada ao acesso ao conhecimento, que vem sendo negado — ou não garantido — nos espaços da "escola inclusiva". A defesa desses espaços precisa estar atrelada à busca de um lugar que dê significado às coisas do mundo, que abra o mundo e possibilite experiências que só acontecem nesses espaços de encontro com a diferença.

ABSTRACT: This work starts from an approximation between studies carried out in Brazil and Portugal, scenarios in which the fights for recognition of deaf people as participants of a minoritized linguistic community has been the topic of discussions defending a bilingual school. Both contexts are seen from the understanding of school as a place to meet with the difference, with an attentive look into what approximates them and also their singularities, given their historical, political, and social conditions. Interlocutions were held between resistance movements for the maintenance of "School" spaces - Schools for the Deaf (Brazil) and Reference Schools for Bilingual Education for Deaf Students - EREBAS (Portugal) -, as well as the approximation approaching the speeches of deaf students, with the aim of looking at the potentiality of these spaces to enable experiences of being a School. The students - subjects of experience - noticed that the linguistic identification offered by the School for the Deaf/EREBAS is related to access to knowledge, which has been denied - or not guaranteed - in the spaces of the "inclusive school". The defense of these spaces needs to be linked to the search for a place that gives meaning to the things in the world, that opens up the world and enables experiences that only happen in these spaces of encounter with difference.

O Homem Que Calculava em Libras: Tradução, Contação de História e Matemática / The Man Who Counted in Libras: Translation, Storytelling and Mathematics

RESUMO: Esta pesquisa teve por objetivo compreender os desafios e as possibilidades de tradução e contação em Língua Brasileira de Sinais (Libras) das histórias presentes no livro O homem que Calculava, do escritor modernista brasileiro Malba Tahan. Tratase de uma investigação qualitativa que se debruçou sobre o processo de tradução audiovisual das histórias presentes no livro, as adaptações feitas em suas narrativas, os recursos de preparação para a produção e edição dos vídeos, a implementação de recursos gráficos e visuais e, por fim, sua divulgação como produto da pesquisa. As análises indicam o potencial didático que emerge na interação entre os envolvidos no processo de tradução das histórias: professores, intérpretes e discentes (surdo e ouvinte), possibilitando formas de expressão artísticas que exploram a visualidade inerente da língua de sinais aliada a recursos gráficos, tomando as narrativas como elemento central para o desenvolvimento criativo ancorado no ensino da Matemática por meio da leitura e da interpretação de histórias.

ABSTRACT: This research aimed to understand the challenges and possibilities of translation and storytelling in the Brazilian Sign Language (known by the acronym LIBRAS) of the stories present in the book O homem que calculava [The man who counted] by the Brazilian modernist writer Malba Tahan. This is a qualitative investigation that focused on the audiovisual translation process of the stories present in the book, on the adaptations made in its narratives, the preparation resources for the production and editing of the videos, the implementation of graphic and visual resources and, finally, its dissemination as a research product. The analyzes indicate the didactic potential that emerges in the interaction between those involved in the process of translating the stories: teachers, interpreters and students (deaf and hearing), enabling forms of artistic expression that explore the inherent visuality of sign language combined with graphic resources, taking the narratives as a central element for the creative development anchored in the teaching of Mathematics through the reading and interpretation of stories.

La Figura del Intérprete en Lengua de Señas Chilena: Estado Actual y Desafíos en el Contexto Escolar / The Figure of the Interpreter in Chilean Sign Language: Current State and Challenges in the School Context

RESUMEN: Los intérpretes en Lengua de Señas Chilena (ILSCh) constituyen un recurso para la inclusión, eliminando barreras de estudiantes sordos que manejan la modalidad visual-gestual para comunicarse. Chile tiene normativa que favorece la participación del ILSCh en distintos ámbitos, y este estudio se centró en los intérpretes en contexto escolar, considerando que existe un desconocimiento de los procesos, implementación, resultados, logros y situación laboral. Las interrogantes del estudio fueron: ¿cuál es el estado actual del ILSCh, las trayectorias formativas, el rol y los desafíos de su quehacer? El objetivo general fue describir la figura del ILSCh y los desafíos que enfrenta en su desempeño laboral en contexto escolar, situados en las regiones Metropolitana y Valparaíso, Chile. El enfoque fue cualitativo, interpretativo, diseño fenomenográfico, descriptivo y transeccional, la muestra intencionada por criterios de 24 intérpretes de nivel escolar. Las técnicas de recogida de información fueron: formulario de identificación, entrevista y grupo focal. Se analizaron las transcripciones con el software Atlas TI. Los resultados muestran la diversidad en la formación, centrada en el vocabulario de la lengua, con mínimas líneas del contexto educativo, desconocimiento del rol, solicitudes de tareas fuera de sus funciones y situaciones laborares sin regulación y diversidad de jefaturas.

ABSTRACT: The interpreters in Chilean Sign Language (ILSCh) constitute a resource for inclusion, eliminating the barriers of deaf students who use the visual-gestural modality to communicate. Chile has regulations that favor the participation of the ILSCh in different áreas, and this study focused on interpreters in the school context, considering that there is a lack of knowledge of the processes, implementation, results, achievements and employment situation. The questions of the study were: What is the current state of the ILSCh, its formative trajectories, its role and the challenges of its work? The general objective was to describe the figure of the ILSCh and the challenges it faces in its work performance in the school context, located in the Metropolitan and Valparaíso regions, Chile. The approach was qualitative, interpretative, phenomenographic, descriptive and transactional design, the sample intentional by criteria of 24 school level interpreters. The information collection techniques were: identification form, interview and focus group. The transcripts were analyzed with the Atlas TI software. The results show the diversity in the training, focused on the vocabulary of the language, with minimal lines of the educational context, lack of knowlegde of the role, requests for tasks outside their functions and work situations without regulation and diversity of leadership.

Sistema Único de Saúde: acessibilidade das pessoas surdas no interior da Bahia / Unified Health System: accessibility of deaf people in the countryside of Bahia state / Sistema Único de Salud: accesibilidad de las personas sordas en el interior de Bahia

A acessibilidade das pessoas surdasno Sistema Único de Saúde ainda é precária devido às barreiras de comunicação. O objetivo do estudo foi analisar o acesso e o atendimento ofertado à comunidade surda nos serviços de saúde públicos, no município de Vitória da Conquista, Bahia, por meio de uma pesquisa qualitativa. Participaram oito profissionais de saúde e treze usuários surdos, cujos dados foram coletados com aplicação de um questionário e um roteiro de entrevista semiestruturada. Os resultados, com base na análise do conteúdo, apontaram que não há capacitação, as Unidades Básicas de Saúde não apresentam estratégias de acessibilidade nos atendimentos, têm rara comunicação em Libras e um número reduzido de intérpretes ­ o que dificulta a comunicação entre o profissional de saúde e o usuário surdo. A formação em Libras e a presença de um intérprete são apontadas como estratégias para um atendimento de qualidade às pessoas surdas

The accessibility of deaf people in the Unified Health System is still precarious due to communication barriers. The objective of the study was to analyze the access and care offered to the deaf community in public health services, in the city of Vitória da Conquista, Bahia, through qualitative research. Eight health professionals and thirteen deaf users took part in the research, whose data were collected using a questionnaire and a semi-structured interview script. Based on the content analysis, the results showed that there is no training, the Basic Health Units do not have accessibility strategies in care, rarely communicate in Libras and a have a small number of interpreters in their ranks ­ hindering the communication between health professional and deaf user. Training in Libras and the presence of an interpreter are identified as strategies for quality care for deaf people

La accesibilidad para los sordos en el Sistema Único de Salud todavía es precaria debido a las barreras de comunicación. El objetivo del estudio fue analizar el acceso y la atención ofrecida a este público en los servicios de salud públicos, en el municipio de Vitória da Conquista, Bahia, con una investigación cualitativa. Participaron ocho profesionales de la salud y trece usuarios sordos, cuyos datos fueron recolectados a través de cuestionario y guión de entrevista semiestructurada. Los resultados, basados en el análisis de contenido, mostraron que no hay capacitación, las Unidades Básicas de Salud no tienen estrategias de accesibilidad, y tienen poca comunicación en Libras y reducido número de intérpretes ­ lo que dificulta la comunicación entre el profesional de la salud y el usuario sordo. La formación en Libras y la presencia de un intérprete se identifican como estrategias para la atención de calidad a los sordos

Current status and influencing factors of chronic disease self-management behavior of middle-aged and elderly deaf people aged 50 years and over in Shanghai / 上海预防医学

ObjectiveTo investigate the current status and influencing factors of chronic disease self-management behavior of middle-aged and elderly deaf people aged 50 years and over in Shanghai， and to provide reference for improving their chronic disease self-management behavior. MethodsDuring September 2021 to February 2022， 271 middle-aged and elderly deaf people aged 50 years and over in Shanghai were investigated face-to-face by general questionnaire， chronic disease self-management study measures， self-efficacy scale， social capital questionnaire， hospital anxiety and depression scale， health disturbance scale. ResultsThe mean score of chronic disease self-management behavior was 32.107±7.527. Gender， frequency of searching health knowledge by mobile phone， whether to eat fruit， self-efficacy and social capital were influencing factors of chronic disease self-management behaviors， which could explain 37.04% of the variance. ConclusionThe chronic disease self-management behavior of the middle-aged and elderly deaf people aged 50 years and over is low and needs to be improved. We can promote the middle-aged and elderly deaf people to form the good self-management behavior by improving their self-efficacy， improving their level of social capital and forming good living habits.

Discursos sobre o Papel do Tradutor-Intérprete Educacional de Libras/Português / Discourses about the Role of the Educational Translater-Interpreter of Libras/Portuguese

RESUMO: O tradutor-intérprete educacional de Língua Brasileira de Sinais (Libras)/Português é fundamental em um espaço educacional que busca ser inclusivo a surdos, porém sua função vem sendo debatida frente à pluralidade existente quanto às disciplinas e aos contextos nos quais esses profissionais atuam. Diante disso, este trabalho promove uma discussão a fim de somar a um constructo que, aos poucos, tece uma perspectiva mais clara acerca dos aspectos que caracterizam a prática de um intérprete educacional. Para isso, analisaram-se respostas de 53 intérpretes de diferentes regiões do Brasil, obtidas por meio de um questionário enviado via Google Forms, que indagava sobre qual seria a sua função no âmbito educacional. A partir disso, aplicando o método de análise de conteúdo, identificaram-se quatro categorias que apontavam o intérprete educacional como: colaborador no processo de inclusão, mediador cultural, coformador do discente surdo e mediador linguístico. Compreende-se que, em sua complexidade, a função do intérprete educacional possui alguns desdobramentos que não podem ser ignorados, atribuindo a esses profissionais um lugar de indiferença ou assistencialismo excessivo nos processos de ensino e de aprendizagem. Ao contrário, eles devem participar ativamente, compreendendo o que caracteriza sua função, também a partir do diálogo com a instituição, discente surdo e comunidade surda.

ABSTRACT: The educational translator-interpreter of Brazilian Sign Language (LIBRAS)/Portuguese is essential in an educational space that seeks to be inclusive for the deaf, however his/her role has been debated in view of the existing plurality regarding the disciplines and contexts in which these professionals work. Due to that, this work promotes a discussion in order to add to a construct that gradually makes a clearer perspective about the aspects that characterize the practice of an educational interpreter. For this, answers from 53 interpreters from different regions of Brazil, obtained through a questionnaire sent via Google Forms, which asked what their role in the educational field would be, were analyzed. From this, applying the method of content analysis, four categories were identified that pointed to the educational interpreter as: collaborator in the inclusion process, cultural mediator, co-trainer of the deaf student and linguistic mediator. It is understood that, in its complexity, the role of the educational interpreter has some ramifications that cannot be ignored, attributing to these professionals a place of indifference or excessive assistance in the teaching and learning processes. On the contrary, they must actively participate, understanding what characterizes their role, also based on dialogue with the institution, deaf students and the deaf community.

A Criança Surda como Sujeito do Olhar: Infância e Surdez nas Pesquisas em Educação / Deaf Child as a Subject of the Gaze: Childhood and Deafness in Education Research

RESUMO: O objetivo deste texto é debater como infância, criança, surdez e experiência visual têm se configurado como temas investigativos na Educação, detendo-se em uma análise sobre o olhar das crianças surdas e como sua experiência visual produz e é produzida pelo olhar. Realizou-se um levantamento bibliográfico no Banco de Theses e Dissertações da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (Capes) e nos trabalhos aprovados nas reuniões nacionais da Associação Nacional de Pós-Graduação e Pesquisa em Educação (ANPEd) - GT Educação Especial, tendo como vetor decisivo pesquisas que tinham a infância e/ou a criança surda como temas centrais. Na análise das 23 pesquisas encontradas, verificaram-se três recorrências: a criança ou a infância surda como conceitos abrangentes, pouco abordados em suas singularidades; a experiência visual da criança surda assumida como secundária à aquisição, seja de outras linguagens (como, por exemplo, a escrita), seja da Língua Brasileira de Sinais (Libras), portanto, como experiência suplementar; o conceito de aprendizagem da criança surda vinculado à imagem como recurso didático. Com base nisso, percorreram-se pesquisas e trabalhos que rompem com essas recorrências e, assim, indicam caminhos para a construção de uma agenda de pesquisa sobre criança e infância surda.

ABSTRACT: The purpose of this text is to discuss how childhood, children, deafness and visual experience have been configured as research themes in Education, focusing on an analysis of the gaze of deaf children and how their visual experience produces and is produced by the gaze. A bibliographic review was carried out in the Theses and Dissertations Database of the Coordination for the Improvement of Higher Education Personnel (CAPES) and in the approved papers for the national meetings of the National Association of Research and Graduate Studies on Education (ANPEd) -Special Education Work Group), having as a decisive vector research that had childhood and/or deaf child as central themes. In the analysis of the 23 studies founded, three recurrences were verified: deaf child or childhood as general concepts, not assumed in their singularities; the visual experience of the deaf child assumed to be secondary to the acquisition, whether of other languages (such as writing), or the Brazilian Sign Language (LIBRAS), therefore, as a supplementary experience; the concept of learning for the deaf child linked to image as a didactic resource. Based on that, research and work that break with these recurrences have been investigated and thus indicate ways for the construction of a research agenda on children and deaf childhood.

Estágio Curricular na EPT: Proposta pa ra Potencializar a Inclusão de Estudantes Surdos do Instituto Federal de Roraima - Campus Novo Paraíso / Curricular Internship at EPT: Proposal to Enhance the Inclusion of Deaf Students from the Federal Institute of Roraima - Novo Paraíso Campus

RESUMO: Este estudo, como projeto da linha de pesquisa Práticas Educativas em Educação Profissional e Tecnológica, objetivou analisar o processo de acompanhamento e orientação durante o período de estágio curricular de estudantes surdos dos cursos técnicos no âmbito do Instituto Federal de Educação, Ciência e Tecnologia de Roraima - campus Novo Paraíso (IFRR/CNP), a fim de desenvolver e avaliar um Manual Orientador de Estágio. A pesquisa foi desenvolvida em quatro etapas processuais: processo de caracterização do cenário de inclusão dos estudantes surdos no âmbito do IFRR/CNP; processo de identificação dos aspectos positivos e dificuldades referentes ao período de estágio dos estudantes surdos do IFRR/CNP; processo de elaboração do produto educacional - Manual Orientador de Estágio para alunos surdos do IFRR/CNP; e processo de aplicação e avaliação do produto educacional. Os resultados apontaram a relevância do Núcleo de Atendimento às Pessoas com Necessidades Educacionais Específicas (NAPNE) em sua atuação no IFRR/CNP como um meio de promover a inclusão dos alunos surdos na instituição, destacando o papel primordial do intérprete de Língua Brasileira de Sinais (Libras) nesse processo. O produto educacional desenvolvido foi considerado, mediante avaliação, um instrumento inclusivo, proporcionando ao estudante surdo informações úteis ao período de estágio em Libras, sendo um apoio importante para outros estudantes surdos que ingressarem na instituição.

ABSTRACT: This study, as a project of the research line Educational Practices in Vocational and Technological Education, aimed to analyze the process of monitoring and guidance during the period of curricular internship of deaf students of technical courses within the scope of the Federal Institute of Education, Science and Technology of Roraima - Novo Paraíso campus (IFRR/ CNP), in order to develop and evaluate an Internship Guidance Manual. The research was developed in four procedural steps: process of characterization of the scenario of inclusion of deaf students in the scope of the IFRR/CNP; process of identifying the positive aspects and difficulties related to the internship period of deaf students at the IFRR/CNP; process of elaboration of the educational product - Internship Guidance Manual for deaf students of the IFRR/CNP and process of application and evaluation of the educational product. The results showed the relevance of the Center of Assistance for People with Specific Educational Needs (NAPNE) in its performance at the IFRR/CNP as a way of promoting the inclusion of deaf students in the institution, highlighting the primary role of the Brazilian Sign Language (LIBRAS) interpreter in this process. The educational product developed was considered, through evaluation, an inclusive instrument, providing the deaf student with useful information during the internship period in LIBRAS, being an important support for other deaf students who enter the institution.

Libras no contexto escolar: instrumento ilustrado de avaliação de narrativas sinalizadas1 / Libras in the school context: illustrated instrument for evaluation of signed narratives

RESUMO O contato inicial com uma língua acessível ocorre, para os estudantes surdos brasileiros, frequentemente no ambiente escolar, ao entrar em contato com outras crianças surdas e com interlocutores adultos fluentes em Língua Brasileira de Sinais (Libras). Esse acesso tardio pode ocasionar um certo atraso na aquisição e no desenvolvimento da linguagem. Assim, a educação bilíngue de surdos coloca os professores frente ao desafio de como avaliar a Libras no contexto escolar. Diante das propostas educacionais, ainda há poucos instrumentos que contribuam para avaliar os conhecimentos em Libras dos estudantes surdos. Portanto, o objetivo deste estudo é, por meio de um instrumento ilustrado de investigação de narrativas sinalizadas, contribuir com a avaliação da Libras e com o trabalho pedagógico de professores que atuam na educação bilíngue de surdos. O instrumento de avaliação de textos narrativos mostra-se bastante sensível para detectar o desenvolvimento linguístico dos discursos narrativos sinalizados, contribuindo intrinsicamente para a observação de funções/necessidades comunicativas.

ABSTRACT The initial contact with an accessible language occurs, for Brazilian deaf students, often in the school environment, when they encounter other deaf children and with adult interlocutors who are fluent in Brazilian Sign Language (LIBRAS). This late access can cause a certain delay in language acquisition and development. Thus, bilingual education for the deaf place's teachers in the face of the challenge of how to evaluate LIBRAS in the school context. In view of the educational proposals, there are still few instruments that contribute to assessing deaf students' knowledge in LIBRAS. Therefore, the objective of this study is, through an illustrated instrument for investigating signaled narratives, to contribute to the evaluation of LIBRAS and to the pedagogical work of teachers who work in bilingual education for the deaf. The instrument for evaluating narrative texts proves to be quite sensitive to detect the linguistic development of signaled narrative discourses, intrinsically contributing to the observation of communicative functions/needs.

Las epistemologías sordas pensadas desde las experiencias con la otredad / Deaf epistemologies from the experiences with the otherhood

Objetivo: El presente artículo tiene como finalidad reflexionar, desde una perspectiva crítica, el cómo se han pensado los procesos de subjetivación de lo que se ha denominado sordedad, a partir de la construcción del otro en el contexto de la modernidad. Estructura: Se plantea la alteridad y alteridad sorda explicitando que la sordedad es una forma más de otredad y que en este sentido, obedece a una misma matriz de inteligibilidad que opera sobre la idea de cuerpos normativizados. Posteriormente, se plantean las miradas de la sordera, se analizan los dispositivos de saber-poder que están a la base de cada modelo de conceptualización de la sordedad, con el propósito de conocer los presupuestos que los sustentan. Conclusión: En este contexto, se concluye el riesgo que implica perpetuar los discursos referidos a la sordedad desde el dispositivo de normalidad de la cultura oyente y que, en consecuencia, la sitúan en un "estatus especial de inferioridad" que termina discriminando y excluyendo; como propuesta se presenta la consideración de las epistemologías sordas.

Aim: The purpose of this article is to propose, from a critical perspective, that the processes of subjectivation of what has been called deafhood have been thought, of from the construction of the other in the context of modernity. Structure: Alterity and deaf alterity are considered, explaining that deafhood is another form of otherhood and that, in this sense; it obeys the same matrix of intelligibility which operates on the idea of normativised bodies. Subsequently, the views of deafness are raised; the knowledge-power devices that are at the base of each model of conceptualization of deafhood are analyzed, in order to understand the assumptions that support them. Conclusion: In this context, the discussion is based on the risk involved in perpetuating the discourse regarding deafness from the normality device of the hearing culture. And conclusions are places it in a "special status of inferiority" that ends up discriminating and excluding. As a proposal, deaf epistemologies are introduced.Dra Karina Andrea Muñoz VilugrónORCID: 0000-0003-3938-2758Source | Filiacion:Universidad Austral de Chile. Sede Puerto MonttBIO:Profesora de Educación Diferencial/Magister en Educación /Doctor en Ciencias de la EducaciónCity | Ciudad:Puerto Monnt (Chile)e-mail:karina.munoz@uach.clDr Santiago Leonardo Rodríguez PonceORCID: 0000-0002-6644-6676 Source | Filiacion:Universidad Austral de Chile. Sede Puerto MonttBIO:Magister en Cultura y Educación / Doctorado en Inclusión y DiscapacidadCity | Ciudad:Puerto Monnt (Chile)e-mail:santiago.rodriguez@uach.clDra Daniela Andrea Bachmann VeraORCID: 0000-0003-3757-3817Source | Filiacion:Universidad Austral de Chile. Sede Puerto MonttBIO:Psicóloga / Magister en Educación / Doctora en Ciencias de la EducaciónCity | Ciudad:Puerto Monnt (Chile)e-mail:daniela.vera@uach.clCitar como:Muñoz Vilugrón, K. A., Rodríguez Ponce, S. L., y Bachmann Vera, D. A. (2023). Las epistemologías sordas pensadas desde las experiencias con la otredad. Areté, 23 (2), 1-7. Obtenido de: https://arete.ibero.edu.co/article/view/26922Para su más amplia difusión, esta obra y sus contenidos se distribuyen bajo licencia: Creative Commons Atribución-NoComercial-SinDerivadas 4.0 InternationalSe autoriza cualquier tipo de reproducción y sus diferentes usos, siempre y cuando se preserve la integridad del contenido y se cite la fuente.ARETÉARETÉFonoaudiologíaRevistaISSN-l: 1657-2513 | e-ISSN:2463-2252

Adaptación inicial y pilotaje de la Evaluación Neuropsicológica Infantil para jóvenes Sordos colombianos / Initial adaptation and piloting of the Neuropsychological Assessment of Children for Colombian Deaf youth

El 90% de los niños sordos nacen en hogares de padres oyentes y pueden experimentar retrasos en la adquisición del lenguaje debido a la falta de exposición temprana a la lengua de señas. La Evaluación Neuropsicológica Infantil 2 (ENI-2) puede ser utilizada para evaluar las habilidades lingüísticas y cognitivas de niños y jóvenes, para lo cual dispone de algunas tareas que es necesario adaptar para ser aplicada en el caso de las personas Sordas. Esta investigación de enfoque cuantitativo, alcance descriptivo y tipo transversal consta de una adaptación inicial a la prueba ENI-2 y su respectivo pilotaje, con el fin de brindar una herramienta y datos preliminares, que pueden contribuir a generar una caracterización cognitiva y lingüística de niños y jóvenes Sordos. La adaptación realizada implicó aspectos como seleccionar las pruebas, modificar la modalidad de la respuesta de algunas tareas, postular un equivalente acorde al contexto bilingüe de las personas Sordas, ajustar estímulos e indicaciones, interpretar en lengua de señas colombiana las indicaciones, entre otras. Se debe tener en cuenta que este es solo un primer acercamiento al proceso de adaptación de la prueba, dado que se evidenció luego del pilotaje la necesidad de realizar más ajustes. Se contó con una muestra a conveniencia no probabilística, con la participación de 7 estudiantes (5 hombres y 2 mujeres) de un colegio para Sordos en la ciudad de Bogotá. Además de aportar la propuesta inicial de adaptación, los resultados de este estudio serán útiles para postular un perfil inicial de habilidades lingüísticas y cognitivas de jóvenes sordos, lo que puede facilitar el enfocar acciones de intervención fonoaudiológica, en torno a las habilidades de los escolares Sordos inmersos en un modelo educativo bilingüe-bicultural.

90% of deaf children are born into hearing parents' households and may experience delays in language acquisition due to a lack Neuropsychological Assessment 2 (INAP-2) can be used to assess the linguistic and cognitive abilities of children and youth, for which some tasks need to be adapted for application in the case of Deaf individuals. This quantitative research with a descriptive scope and cross-sectional design consists of an initial adaptation of the INAP-2 test and its respective pilot study, in order to provide a tool and preliminary data that can contribute to generating a cognitive and linguistic characterization of Deaf children and youth. The adaptation process involved aspects such as selecting the tests, modifying the response mode for some tasks, proposing an equivalent suitable for the bilingual context of Deaf individuals, adjusting stimuli and instructions, interpreting instructions in Colombian Sign Language, among others. It should be noted that this is just an initial approach to the test adaptation process, as further adjustments were identified after the pilot study. A non-probabilistic convenience sample was used, involving the participation of 7 students (5 males and 2 females) from a school for the Deaf in the city of Bogotá. In addition to providing the initial adaptation proposal, the results of this study will be useful for establishing an initial profile of linguistic and cognitive skills in Deaf youth, which can facilitate the focus of speech therapy intervention actions regarding the skills of Deaf students immersed in a bilingual-bicultural educational model.

Fonoaudiólogos y educadores diferenciales en la educación superior inclusiva con la población sorda / Speech language pathology and differential educators in the higher education inclusive with the deaf population

La Educación Superior es un derecho fundamental de todo ser humano, desde esta premisa la universidad para los estudiantes sordos es un espacio intercultural que brinda la oportunidad de intercambios de experiencias, costumbres, aprendizajes que se dan durante la inclusión en educación, convirtiéndose para la población en un reto que les permite la independencia, autonomía e igualdad de oportunidades, por esta razón el fonoaudiólogo y el educador diferencial se convierten en profesionales que deben participar en los procesos para la toman decisiones, eliminando barreras, generando facilitadores. El objetivo de este estudio fue diseñar los lineamientos de promoción para la participación de fonoaudiólogos y educadores diferenciales en educación superior de la persona sorda. La metodología de investigación fue cualitativa, descriptiva, en el cual se realizaron entrevistas semiestructuradas a profesionales hombres y mujeres de Colombia y Chile, mayores de 18 años con experiencia en educación superior. Los resultados indican que el diseño de lineamientos permite el trabajo interdisciplinar tanto del fonoaudiólogo como del educador diferencial en procesos de inclusión de estudiantes sordos en educación superior respondiendo a las necesidades, mitigando barreras y evitando el fracaso y la deserción académica, como conclusión se establece la importancia de tener en cuenta la participación de los profesionales, resaltando elementos cruciales como: Conocimiento, profesional, sensibilización, reflexivo, comunicación, contexto, apoyo y ajustes, soportando la base principal de la formación, el desempeño para la comprensión y el reconocimiento de sus roles en los equipos de inclusión. Dichos elementos son la base fundamental para la construcción de los lineamientos

Higher education is a fundamental right of every human being. From this premise, university becomes an intercultural space for deaf students that provides the opportunity for the exchange of experiences, customs, and learning that occur during their inclusion in education. It becomes a challenge for the deaf population, enabling their independence, autonomy, and equal opportunities. For this reason, speech therapists and special education teachers become professionals who must participate in the decision-making processes, eliminating barriers and creating facilitators. The objective of this study was to design guidelines for the involvement of speech therapists and special education teachers in the higher education of deaf individuals. The research methodology was qualitative and descriptive, involving semistructured interviews with male and female professionals from Colombia and Chile, aged 18 and above, with experience in higher education. The results indicate that the design of guidelines allows for interdisciplinary work by both speech therapists and special education teachers in the inclusion processes of deaf students in higher education, addressing their needs, mitigating barriers, and preventing academic failure and dropout. In conclusion, the importance of considering the participation of professionals is established, highlighting crucial elements such as knowledge, professionalism, sensitization, reflection, communication, context, support, and adjustments. These elements support the foundation of training and performance for understanding and recognizing their roles in inclusion teams. These elements form the fundamental basis for the construction of the guidelines.

Validación de contenido de un protocolo de evaluación del lenguaje en sordos / Content validation of a language assessment protocol for the deaf

Para la evaluación del lenguaje en escolares Sordos, son muy escasos los instrumentos disponibles que permitan hacer un seguimiento integral a su evolución en sus diferentes aspectos: fonético ­ fonológico, morfosintáctico, semántico y pragmático, en esta población. Por ello, el presente estudio cuantitativo, descriptivo transversal, se orientó a validar el contenido de un protocolo para su valoración dirigido a estudiantes sordos de 6 a 12 años, que utilizan la Lengua de Señas Colombiana y se encuentran escolarizados en básica primaria. Para la validación, participaron 3 jueces expertos con amplia experiencia en el abordaje fonoaudiológico de estudiantes sordos, utilizando para emitir su juicio cuatro categorías: suficiencia, relevancia, coherencia y claridad. Para el procesamiento de la información, se aplicó el método de Análisis de Varianza (ANOVA) y el gráfico de la prueba de Less Significant Diffence (LSD) de Fisher. Estas técnicas fueron utilizadas con una confianza del 95%. En los resultados, en cuanto al nivel fonético ­ fonológico, se obtuvo como puntuación promedio el puntaje máximo 4.0, lo que equivale a un cumplimiento del 100%. En los niveles morfosintáctico y pragmático, se obtuvo un promedio de 3,75 sobre 4 con un 94% de cumplimiento, respectivamente. Finalmente, en el nivel semántico, el puntaje promedio global fue de 3,5 el cual corresponde a un cumplimiento del 88%, encontrándose dentro del umbral de aceptación establecido (80%). Por tanto, los resultados muestran que todas las dimensiones e ítems del protocolo fueron validados en cuanto a contenido, cumpliendo con el propósito para el cual fue diseñado

For the evaluation of language in deaf schoolchildren, there are very few instruments available that allow a comprehensive monitoring of its evolution in its different aspects: phoneticphonological, morphosyntactic, semantic and pragmatic, in this population. Therefore, this quantitative, descriptive, cross-sectional study was oriented to validate the content of a protocol for its evaluation directed to deaf students from 6 to 12 years old, who use Colombian Sign Language and are in elementary school. For the validation, 3 expert judges with ample experience in the phonoaudiological approach to deaf students participated, using four categories for their evaluation: sufficiency, relevance, coherence, and clarity. For data processing, the Analysis of Variance method (ANOVA) and Fisher's Less Significant Diffence (LSD) test graph were applied. These techniques were used with 95% confidence. In the results, regarding the phonetic-phonological level, the average score obtained was the maximum score (4.0), which is equivalent to 100% compliance. In the morphosyntactic and pragmatic levels, an average score of 3.75 out of 4 was obtained, with 94% compliance, respectively. Finally, on the semantic level, the overall average score was 3.5, which corresponds to a compliance of 88%, being within the established acceptance threshold (80%). Therefore, the results show that all dimensions and items of the protocol were validated in terms of content, fulfilling the purpose for which it was designed

Formación bilingüe en niños y niñas sordas a través de la literatura infantil / Initial approaches towards bilingual education in deaf children through children's literature

El presente artículo investigativo evidencia los resultados parciales de la etapa de pilotaje de la propuesta de investigación observación acción en el abordaje fonoaudiológico y pedagógico, en niñas y niños sordos inmersos en un contexto bilingüe, por medio del uso de la literatura infantil acompañada de estrategias/ recursos lingüísticos y cognitivos. A fin de evidenciar el impacto que tiene el trabajo con la literatura infantil en el aprendizaje del español escrito como segunda lengua. Se contó con la participación de diez niños y niñas sordos que cursaban los niveles de prescolar y ciclo uno en una serie de talleres durante quince semanas que involucraban actividades relacionadas con la literatura infantil y los momentos de antes, durante y después de la lectura. Entre los resultados se evidencia fortalecimiento en la adquisición y consolidación de conceptos, pertinencia del uso de clave de color en el procesamiento gramatical, uso de herramientas metacognitivas en los procesos de comprensión lectora. Tras los resultados obtenidos se puede concluir que esta iniciativa permitió la interiorización de manera efectiva la macro estructura de la historia, asociado a la capacidad de evocarla y descrniños el uso de la tabla cognitiva como estrategia relevante para la representación escrita con su correspondiente en LSC, progreso evidente en la precisión en el rastreo en la tabla cognitiva, y una disminución de la latencia de la respuesta tras los tiempos de implementación de las sesiones. Finalmente se destaca como la mediación de la literatura infantil, como eje transversal que favorece desde todas sus dimensiones, la formación del sujeto Sordo bilingüe.

This research article shows the partial results of the piloting stage of the action observation research proposal in the speech therapy and pedagogical approach, in deaf girls and boys immersed in a bilingual context, through the use of children's literature accompanied by strategies/ linguistic and cognitive resources. In order to demonstrate the impact that working with children's literature has on learning written Spanish as a second language. Ten deaf boys and girls attending preschool and cycle one levels participated in a series of workshops for fifteen weeks that involved activities related to children's literature and the moments before, during and after reading. Among the results, there is evidence of strengthening in the acquisition and consolidation of concepts, relevance of the use of color key in grammatical processing, use of metacognitive tools in reading comprehension processes. After the results obtained, it can be concluded that this initiative allowed the effective internalization of the macro structure of the story, associated with the ability to evoke and describe it in L1, the use of the cognitive table as a relevant strategy for written representation with its corresponding in LSC, evident progress in tracking accuracy on the cognitive table, and a decrease in response latency after session implementation times. Finally, it stands out as the mediation of children's literature, as a transversal axis that favors, from all its dimensions, the formation of the bilingual Deaf subject.

Retos para la promoción y garantía de derechos en primera infancia sorda / Challenges for the promotion and guarantee of rights in deaf early childhood

Desde un enfoque de derechos, diferencial y dual, la población sorda es reconocida como sujetos sociales y de especial protección, quienes tienen unas particularidades sociolingüistas y culturales. Sin embargo, la evidencia señala que sus atenciones en el marco de la Ruta Integral de Atenciones se ven limitadas, porque no se implementan políticas que promueven la garantía de derechos, o se desconoce su cultura, sus necesidades y los ajustes razonables/ acciones afirmativas que requieren, para garantizar su inclusión como sujetos plenos de derechos en Colombia. Es por lo que, mediante el presente artículo, se exponen retos que tiene la gestión del INSOR y la responsabilidad de los diferentes sectores, principalmente salud, y/o entidades que trabajan la protección integral de los niños de 0 a 5 años, en la promoción y garantía de derechos para la atención y desarrollo feliz e integral, inclusión familiar y social, en el marco de la Ruta Integral de Atenciones ­ RIA, desde los periodos de preconcepción y gestación de las madres sordas, hasta los niños de 0 meses a 5 años. Se plantea una metodología de enfoque cualitativo y de tipo reflexivo, basada en la analítica e interpretación del autor, recurriendo a fuentes originales e investigaciones fundamentadas por el INSOR en el tema de Primera Infancia Sorda, utilizando la metodología aplicada. Como resultado se pretende hacer evidente la necesidad de una atención diferencial con la población sorda, en los periodos de preconcepción y gestación de mujeres sordas, y niños sordos en la Primera Infancia, que parte de reconocer sus características y particularidades, requiriéndose un acompañamiento para la disminución de barreras por parte de quienes brindan atención en los diferentes entornos, principalmente en el entorno de la salud.

From a rights-based, differential, and dual perspective, the deaf population is recognized as social subjects deserving of special protection, characterized by sociolinguistic and cultural particularities. However, evidence indicates that their care within the framework of the Comprehensive Care Route is limited because policies promoting the guarantee of their rights are not implemented, or their culture, needs, and reasonable adjustments/affirmative actions required for their full inclusion as rights-bearing individuals in Colombia are not well understood. Therefore, through this article, challenges in the management of INSOR (National Institute for the Deaf) and the responsibility of different sectors, primarily healthcare, and entities working on the comprehensive protection of children aged 0 to 5, in promoting and ensuring their rights in care and holistic development, family and social inclusion, within the framework of the Comprehensive Care Route - RIA, from the preconception and gestation periods of deaf mothers to children aged 0 months to 5 years, are outlined. A qualitative and reflective approach methodology is proposed, based on the author's analysis and interpretation, drawing from original sources and research conducted by INSOR on the topic of Deaf Early Childhood, using the applied methodology. The aim is to highlight the need for differential care for the deaf population during the preconception and gestation periods of deaf women and deaf children in early childhood. This approach acknowledges their characteristics and particularities, requiring support to reduce barriers from those providing care in different environments, especially in healthcare settings.

Early numerical cognition in deaf and hearing children: Closer than expected? / Cognición numérica temprana en niños sordos y oyentes: ¿más cercana de lo esperado?

Abstract Deaf students show a significant delay in their understanding of numeracy and measurement concepts as well as verbal problem solving. There is still no consensus about the origin of this delay but several studies have shown that deaf people show differences in basic numerical skills and executive function (EF), which could underlie the differences in the way they learn and develop their cognitive abilities. Children have the innate ability to estimate and compare numerosities without using language or numerical symbols. The ability to discriminate large numerosities depends on the approximate number system (ANS), a cognitive system believed to be governed by a neural circuit within the intraparietal sulcus. Researchers hypothesize that the ANS underlies the development of arithmetic and there is data supporting the contribution of the ANS for math achievements. Little is known about the approximate number system of deaf children at early ages. Deaf and hearing preschool children were compared in terms of specific cognitive functions shown to be important for success in mathematics. Executive functions and symbolic and nonsymbolic magnitude comparison abilities of 7 deaf children and 14 hearing children aged 4-7 years (M = 69.90 months, SD = 11.42), were compared. To do so, neuropsychological assessments for school-aged children were adapted into Portuguese Sign Language. Significant group differences were found in abstract counting as well as in symbolic and nonsymbolic magnitude comparisons. These findings suggest that deaf children are less competent in these early numeracy skills than are their hearing peers.

Resumen La cognición de los sordos ha sido objeto de numerosos estudios que buscan comprender cómo los niños y adultos sordos procesan la información. Dichos estudios han demostrado que las personas sordas muestran diferencias en las habilidades numéricas y la función ejecutiva (FE), lo que podría ser la base de las diferencias conocidas en la forma en que las personas sordas aprenden y desarrollan sus habilidades cognitivas. Se han encontrado diferencias entre estudiantes sordos y oyentes en varias áreas de razonamiento numérico, en matemática y en la eficiencia en el procesamiento de representaciones numéricas como la comparación de magnitud. En las tareas de comparación de magnitud, los resultados dependían de si se estaban haciendo comparaciones simbólicas (números arábigos) o no simbólicas (puntos). En un estudio, los niños sordos fueron más lentos que sus compañeros oyentes en las tareas de comparación de magnitud simbólica, pero no en las tareas no simbólicas. Sin embargo, en un estudio más reciente, también se encontraron diferencias en las tareas no simbólicas. Se considera que la capacidad para comparar y discriminar grandes numerosidades depende del sistema numérico aproximado (ANS, Approximate Number System), un sistema cognitivo que se cree está gobernado por un circuito neuronal dentro del surco intraparietal. Los investigadores plantean la hipótesis de que el ANS subyace en cierta medida al desarrollo de la aritmética. Hay algunos datos que apoyan esta hipótesis: por ejemplo, las diferencias individuales en la agudeza del ANS se correlacionan positivamente con las habilidades numéricas y los logros futuros en matemática. Por otro lado, se ha encontrado un deterioro en la agudeza del ANS en niños con discapacidades de aprendizaje matemático. En consecuencia, los investigadores han propuesto que el ANS contribuye a la aparición de conceptos numéricos que los niños requieren para la competencia básica en el conteo y las comparaciones de magnitud simbólica. Otros han sugerido que la asociación entre la agudeza en la comparación de magnitud no simbólica y el rendimiento en matemática está moderada por factores de dominio general como las funciones ejecutivas (FE), en particular el control inhibitorio. En general, no está claro si existen diferencias en la agudeza de comparación de magnitud simbólica y no simbólica en niños sordos más pequeños y en qué medida se relacionan con las FE. El estudio actual examina la agudeza de las representaciones numéricas simbólicas y no simbólicas en niños sordos en edad preescolar e investiga la posible influencia del funcionamiento ejecutivo en estas habilidades matemáticas básicas. Se recolectaron datos de 21 niños portugueses del área de Lisboa, siete de los cuales eran sordos congénitamente y 14 tenían audición normal; los niños tenían entre 4 y 7 años de edad (M = 69.9 meses, DT = 11.42). Se seleccionaron tareas para medir lo siguiente: (a) FE, (b) memoria de trabajo, (c) lenguaje y (d) habilidades numéricas tempranas. Se empleó la tarea Shape School Task para evaluar FE. Se administró la versión portuguesa de la tarea de tapping de bloques de Corsi para evaluar la amplitud visuoespacial. Se desarrolló una tarea de comparación de puntos para examinar la capacidad de los niños de decidir instantáneamente cuál de las dos matrices de puntos es más grande utilizando el software Panamath. Se utilizaron dos tareas para evaluar la capacidad de los niños para producir palabras numéricas en un contexto cardinal y el Numeracy Screener para medir su capacidad para comprender la magnitud numérica simbólica. Los resultados indicaron que los niños sordos mostraron retrasos en las capacidades de comparación de magnitud simbólica y no simbólica. En las FE solo se encontraron diferencias en una tarea que implicaba una combinación de conmutación e inhibición; por lo demás, su función ejecutiva era comparable a la de los niños no sordos.

Exploiting Genetic Counseling as a Tool for the Analysis of Dissociative Behavior and Sub-normality from Conception to Birth among Maladjusted Blind, Deaf and Dumb Infants

This paper analyzes the dissociation behavior of children from pregnancy to birth and resultant sub-normality using genetic counseling in an attempt to mainstream children with birth disabilities and the challenges of the genetic counselor. Genetic counseling is a process of communication to provide information about a genetic condition, or inheritance, and support decision making and adjustment in families with the inheritance gene. It uses children that need special needs, the blind, deaf and dumb. Genetic counseling approach, re-affirmation, care and integration, and confidence-building institutionalized in school and family life remains some of the vital tools needed to support these children using genetic guidance and counseling as the best approach to adjusting the different maladaptive behavior of children. The genetic counseling approach includes information about the implications of testing positive for the genetic disorder, including the psychological impact and other consequences, whether to inform relatives of your intention to test, or not to test and the usual pattern of progression of the sub-normality identified tested for and its potential treatments. Thus, the paper seems to analyze sub-normality as classified within the adequacy of social adaptation. Analysis of dissociation behavior associated with sub mentality shows discouragement, contempt, and neglect from an early age, emotionally and maladjusted social growth. Therefore, the counselor must experience sub-normality by getting involved and clarifying their feeling toward the children with these disabilities.

Representação social sobre a surdez: uma revisão integrativa / Social representation on deafness: an integrative review / Representación social sobre la sordera: una revisíon integradora

O presente estudo teve como objetivo realizar uma revisão integrativa da lite-ratura referente aos estudos científicos que envolvessem a representação social e a surdez. Para isso, realizou-se uma busca nas bases de dados SciELO.org, PePSIC, LILACS, PsycINFO (APA) e Scopus (Elsevier), por meio da plataforma Periódicos CAPES no mês de setembro do ano 2020. Os descritores foram: representação social, surdez, surdo e deficiente auditivo, na língua portuguesa, inglesa e espanhola. Foram analisados 11 artigos e com resultados acerca das populações surda e ouvinte. Nos artigos com pessoas surdas observam--se temáticas sobre identidade, cultura e direitos enquanto cidadãos para dirimirem e modificarem os estereótipos acerca da surdez. As pesquisas com os ouvintes foram realizadas com os familiares e a sociedade de modo geral, tendo como destaque representações baseadas em estereótipos negativos e aspectos limitantes. Ademais, sugerem-se novas produções cientificas que deem continuidade à teoria das representações sociais associadas com outros aspectos que envolvam a surdez e tenham como amostra, a comunidade surda.

The present study aimed to carry out an integrative review of the literature regarding scientific studies involving social representation and deafness. For this, a search was carried out in the databases SciELO.org, PePSIC, LILACS, PsycINFO (APA) and Scopus (Elsevier), through the platform Periodicals CAPES, in September 2020. The descriptors were: representation social, deaf, deaf and hearing impaired, in Portuguese, English and Spanish. Eleven articles were analyzed with abouth the deaf, and hearing populations. In the articles with deaf people, themes about identity, culture and rights as citizens are observed to resolve and modify stereotypes about deafness. Surveys with listeners were carried out with family members and society in general, highli-ghting representations based on negative stereotypes and limiting aspects. Furthermore, new scientific productions are suggested that give continuity to the theory of social representations associated with other aspects that involve deafness and have the deaf community as a sample.

El presente estudio tuvo como objetivo realizar una revisión integradora de la literatura sobre estudios científicos que involucran la representación social y la sordera. Para ello, se realizó una búsqueda en las bases de datos SciELO.org, PePSIC, LILACS, PsycINFO (APA) y Scopus (Elsevier), a través de la plataforma de Publicaciones CAPES, en septiembre de 2020. Los descriptores fueron: representación social, sordo, sordo y con discapacidad auditiva, en portugués, inglés y español. Se analizaron once artículos con resultados sobre la población sorda y auditiva. En los artículos con personas sordas se observan temas sobre identidad, cultura y derechos como ciudadanos para resolver y modificar estereotipos sobre la sordera. Se realizaron encuestas con los oyentes a familiares y sociedad en general, destacando representaciones basadas en estereotipos negativos y aspectos limitantes. Además, se sugieren nuevas producciones científicas que dan continuidad a la teoría de las representaciones sociales asociadas a otros aspectos que involucran la sordera y tienen como muestra a la comunidad sorda.

Caracterización fenotípica de la retinitis pigmentaria asociada a sordera / Phenotypic characterization of retinitis pigmentosa associated with deafness

Introducción. El síndrome de Usher es una alteración genética caracterizada por la asociación de retinitis pigmentaria y sordera. Sin embargo, hay casos con familias en las cuales, a pesar de presentarse dicha asociación, no se puede diagnosticar un síndrome de Usher ni ninguno otro. Objetivo. Reevaluar fenotípicamente a 103 familias con diagnóstico previo de posible síndrome de Usher o retinitis pigmentaria asociada con sordera. Materiales y métodos. Se revisaron las historias clínicas de 103 familias con un posible diagnóstico clínico de síndrome de Usher o retinitis pigmentaria asociada con sordera. Se seleccionaron las familias cuyo diagnóstico clínico no correspondía a un síndrome de Usher típico. Los afectados fueron valorados oftalmológica y audiológicamente. Se analizaron variables demográficas y clínicas. Resultados. Se reevaluaron 14 familias cuyo diagnóstico clínico no correspondía al de síndrome de Usher. De las familias con diagnóstico inicial de síndrome de Usher típico, el 13,6 % recibieron uno posterior de "retinitis pigmentaria asociada con sordera" de "otro síntoma ocular asociado con hipoacusia',' o en forma aislada en una misma familia, de "retinitis pigmentaria" o "hipoacusia'.' Conclusiones. Es fundamental el estudio familiar en los casos en que la clínica no concuerda con el diagnóstico de síndrome de Usher típico. En los pacientes con retinitis pigmentaria asociada con sordera, el diagnóstico clínico acertado permite enfocar los análisis moleculares y, así, establecer un diagnóstico diferencial. Es necesario elaborar guías de nomenclatura en los casos con estos hallazgos atípicos para orientar a médicos e investigadores en cuanto a su correcto manejo.

Introduction: There are several syndromes that associate retinitis pigmentosa with deafness or hearing loss. The most frequent is Usher syndrome, a genetic disorder of autosomal recessive inheritance, which, in some cases, is accompanied by vestibular dysfunction. However, there are cases of families that despite having retinitis pigmentosa associated with deafness, cannot be classified as Usher or other syndromes due to additional findings. Objective: To reassess the phenotypes of 103 families previously diagnosed as possible Usher syndrome and/or retinitis pigmentosa associated with deafness. Materials and methods: We conducted a descriptive and retrospective study by reviewing the medical records of 103 families with a probable clinical diagnosis of Usher syndrome and/or retinitis pigmentosa associated with deafness. Families whose clinical diagnosis did not correspond to the typical Usher syndrome were selected and evaluated ophthalmologically and audiologically. Demographic and clinical variables were analyzed. Results: We selected and then reevaluated 14 families and 55 individuals as they did not correspond to a clinical diagnosis of Usher syndrome; 13.6% of the families initially considered to have typical Usher syndrome were later diagnosed with retinitis pigmentosa associated with deafness, another ocular symptom associated with hearing loss, retinitis pigmentosa, or isolated hearing loss in the same family. Conclusions: Family studies are essential in cases where the symptoms do not match the typical Usher' syndrome. In the cases of retinitis pigmentosa associated with deafness, a correct clinical diagnosis allows for focusing on the molecular analyses to establish a differential diagnosis. The need for nomenclature guidelines on these atypical findings is relevant to aid physicians and researchers in the best approach to these cases.