Psychosocial determinants associated with quality of life in people with usher syndrome. A scoping review

Abstract

Background: 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.

Methods: 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.

Results: 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 rehabilitation

• Usher 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).

Keywords:

• Usher Syndrome
• deafblindness
• psychosocial determinants
• quality of life
• self-perception

Disclosure statement

On behalf of all coauthors, I testify that they approve its publication in the journal, that the manuscript is not being considered by another journal, and that no substantial part or findings have been published elsewhere. No potential conflict of interest was reported by the authors.

Funding

This work was supported by two grants, one awarded by the Rare Disease Foundation (“Fondation Maladies Rares”) to the DéPsySurdi project, led by author RP (http://depsysurdi.fr), and another awarded by the French National Research Agency (ANR) to the Light4Deaf project - Project ANR-15-RHU-001Light4deaf (http://www.ushersocio.org), the 8th Work Package of which is co-led by authors SM and RP.