Awareness of Usher Syndrome and the Need for Multidisciplinary Care: A Cross-Occupational Survey of Allied Health Clinicians

Lauren N Ayton; Karyn L Galvin; Lauren Johansen; Fleur O'Hare; Emily R Shepard

doi:10.2147/JMDH.S411306

Awareness of Usher Syndrome and the Need for Multidisciplinary Care: A Cross-Occupational Survey of Allied Health Clinicians

J Multidiscip Healthc. 2023 Jul 13:16:1927-1936. doi: 10.2147/JMDH.S411306. eCollection 2023.

Authors

Lauren N Ayton^#^{1

2

3}, Karyn L Galvin^#⁴, Lauren Johansen⁵, Fleur O'Hare^{1

3}, Emily R Shepard⁵

Affiliations

¹ Department of Optometry and Vision Sciences, University of Melbourne, Parkville, VIC, Australia.
² Department of Surgery (Ophthalmology), University of Melbourne, Parkville, VIC, Australia.
³ Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, East Melbourne, VIC, Australia.
⁴ Department of Audiology and Speech Pathology, University of Melbourne, Parkville, VIC, Australia.
⁵ UsherKids Australia, Mordialloc, VIC, Australia.

^# Contributed equally.

Abstract

Background: Usher syndrome is the most common cause of deaf-blindness, affecting up to 1 in 6000 people. Multidisciplinary care is required to maximize outcomes for individuals and families. This study assessed awareness of Usher Syndrome amongst allied health clinicians who provide care related to the primarily affected senses of hearing and vision, ie, optometry, orthoptics and audiology.

Methods: A prospective cross-sectional online survey of clinicians working in Australian university-affiliated clinics (7 optometry, 1 orthoptics and 4 audiology) was completed between September 2021 and January 2022. Questions were asked about the cause, common symptoms, and awareness of health professions who manage Usher syndrome.

Results: The 27 audiologists, 40 optometrists, and 7 orthoptists who completed the survey included 53 females (71.6%), had an average age of 37 years (range 24-70), and had an average duration of clinical experience of 13 years (range 1-45 years). The majority of respondents correctly identified Usher syndrome as a genetic condition (86%), identified at least two of the affected senses (97%), and identified the progressive nature of the vision and hearing losses (>90%). Awareness of vestibular dysfunction and its characteristics was low, as was knowledge of the key treatment roles that speech pathologists, genetic counsellors and geneticists play in the management of Usher Syndrome. The majority of respondents also did not identify important aspects of care within their own discipline.

Conclusion: This study has shown that there is a need for targeted education to be delivered to hearing and vision care allied health clinicians to raise awareness of the vestibular impacts and aspects of vision loss experienced by people with Usher syndrome. This education needs to target the broad range of clinicians who have a key role in providing multidisciplinary care (including speech pathologists, geneticists, and genetic counsellors) and to identify the key aspects of good-quality multidisciplinary care.

Keywords: audiology; deaf-blindness; genetics; optometry; orthoptics; physiotherapy.

Grants and funding

LA is funded by a National Health and Medical Research Council Investigator Grant (GNT#1195713) and a University of Melbourne Driving Research Momentum Fellowship. The funding bodies had no role in the study design, execution or writeup of this manuscript.