The Usher lifestyle survey: maintaining independence: a multi-centre study

Abstract

Patients with Usher syndrome face a special set of challenges in order to maintain their independence when their sight and hearing worsen. Three different types of Usher (I, II and III) are distinguished by differences in onset, progression and severity of hearing loss, and by the presence or absence of balance problems. In this study 93 Usher patients from seven European countries filled out a questionnaire on maintaining independence (60 patients type I, 25 patients type II, four patients type III and four patients type unknown). Results of Usher type I and II patients are presented. Following the Nordic definition of maintaining independence in deaf-blindness, three domains are investigated: access to information, communication and mobility. Research variables in this study are: age and type of Usher, considered hearing loss- and the number of retinitis pigmentosa-related sight problems. Usher type I patients tend to need more help than Usher type II patients and the amount of help that they need grows when patients get older or when considered hearing loss worsens. No patterns in results were seen for the number of retinitis pigmentosa related sight problems.

Sensory and demographic characteristics of deafblindness rehabilitation clients in Montréal, Canada

“…Not surprisingly, the second‐largest diagnostic group, after age‐related sensory impairment, was Usher Syndrome. This group of clients has history and a more established identity within the DSI rehabilitation community and their needs have previously been discussed in more detail 39,40 . What emerged from the diagnostic review in Appendix S1 was the large number and variety of other diagnostic combinations that can cause dual impairment, many of which are rarely discussed in the research context.…”

Quality of Life and Cochlear Implantation in Usher Syndrome Type I

“…The Usher Lifestyle Survey is a descriptive questionnaire consisting of nine main questions divided into several subquestions on the topic of maintaining independence. This questionnaire was developed in the United Kingdom by Sense 12,13 . In 1980, the Nordic countries agreed to a common definition of domains of independence that are adversely affected in deaf–blindness.…”

Psychosocial well-being and health-related quality of life in a UK population with Usher syndrome

“…25 Other studies have also shown that Usher syndrome in adults can be associated with stress, anxiety and depression 26–28. There is also evidence that Usher syndrome is linked to decreased social trust24 and increased feelings of social isolation and loneliness 28.…”