Arcous Marine, Putois Olivier, Dalle-Nazébi Sophie, Kerbourch Sylvain, Cariou Anaelle, Ben Aissa Ines, Marlin Sandrine, Potier Rémy
Denis Diderot - Sorbonne Paris Cité, Université VII, Paris, France.
Faculty of Psychology, Université de Strasbourg, Strasbourg, France.
Disabil Rehabil. 2020 Sep;42(19):2809-2820. doi: 10.1080/09638288.2019.1571637. Epub 2019 Apr 12.
In Usher syndrome, deafness is congenital and blindness is acquired. Therefore, the progressive loss of one of the two senses forces individuals with this syndrome to reorganize their everyday tasks and relationships, creating new strategies to communicate, access information, and move within a given space. This reorganization can interfere with the subjects' capacity to build a good quality of life. We conducted a scoping review of both peer-reviewed and gray literature, to identify existing evidence of the role of psychosocial determinants on the quality of life of people with Usher syndrome. Twenty-one references met the inclusion criteria. Findings suggest that people with Usher syndrome seem to adjust their life habits to their condition, maintaining hope for the future, and believe in their capacities to accomplish their goals in spite of the various difficulties they encounter. However, this scoping review highlights a lack of research on adaptive strategies, as well as a lack of knowledge concerning the integration of the syndrome in one's identity, the relations to caregivers, and the specificities of the psychotherapeutic support. More information on these topics would enable better-adjusted social, psychotherapeutic, and medical responses.Implications for rehabilitationUsher syndrome, a rare genetic disease, leads to deafblindness, a cluster of related multiple sensory disabilities. People with Usher encounter several obstacles in their daily life. It is also difficult to adapt to the progressive loss of hearing and sight.This paper proposes a scoping review: we identify the main adaptation strategies used by people with Usher Syndrome in order to become autonomous in spite of these obstacles. In becoming more autonomous, they come to a better quality of life.We summarize the most frequent adaptation strategies (at school, work, leisure, interpersonal relationships, etc.) to help programs aimed at rehabilitation for people with Usher syndrome, and to find unexplored research perspectives (e.g., psychotherapies).
在乌舍尔综合征中，耳聋是先天性的，失明是后天获得的。因此，这两种感官之一的逐渐丧失迫使患有这种综合征的人重新组织他们的日常任务和人际关系，创造新的沟通、获取信息和在特定空间内移动的策略。这种重新组织可能会干扰受试者建立高质量生活的能力。我们对同行评审文献和灰色文献进行了一项范围综述，以确定社会心理决定因素对乌舍尔综合征患者生活质量影响的现有证据。21篇参考文献符合纳入标准。研究结果表明，乌舍尔综合征患者似乎会根据自身状况调整生活习惯，对未来保持希望，并且尽管会遇到各种困难，但仍相信自己有能力实现目标。然而，这项范围综述强调了对适应策略的研究不足，以及在将该综合征融入个人身份认同、与照顾者的关系以及心理治疗支持的特殊性方面缺乏了解。关于这些主题的更多信息将有助于做出更合适的社会、心理治疗和医疗应对措施。
对康复的启示
乌舍尔综合征是一种罕见的遗传病，会导致聋盲，即一系列相关的多重感官残疾。乌舍尔综合征患者在日常生活中会遇到诸多障碍。适应听力和视力的逐渐丧失也很困难。
我们确定了乌舍尔综合征患者为克服这些障碍实现自主而采用的主要适应策略。通过变得更加自主，他们的生活质量得到了提高。
我们总结了最常见的适应策略（在学校、工作、休闲、人际关系等方面），以帮助针对乌舍尔综合征患者的康复项目，并找到未被探索的研究视角（如心理治疗）。
