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

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:

• deaf-blindness
• optometry
• audiology
• orthoptics
• genetics
• physiotherapy

Ethics Approval and Consent to Participate

Ethical approval was received from the University of Melbourne (ID 2021-22187). All participants provided informed consent through the online participant information and consent form, before the survey opened. Informed parental consent was provided for publication of the image of the child in Figure 1.

Acknowledgments

We thank the Melbourne Disability Institute at The University of Melbourne for its role in connecting researchers with the UsherKids Australia community group. We thank the Australian clinics that distributed the survey to their staff: Melbourne Audiology and Speech Pathology Clinic and Melbourne Eyecare Clinic (University of Melbourne); Deakin University School of Medicine, Optometry Clinic; University of New South Wales Optometry Clinic; Centre for Eye Health (University of New South Wales); Flinders Health 2GO (Flinders University); QUT Health Clinics – Optometry (Queensland University of Technology); University of Western Australia Audiology and Optometry clinics; Latrobe Communication Clinic (Latrobe University); and University of Queensland Audiology Clinic. Statistical assistance was provided by Dr Myra McGuiness. Feedback on the survey prior to data collection was provided by two audiologists, two speech pathologists, two orthoptists and three optometrists, otherwise not involved in the study. We also acknowledge the contribution of a University of Melbourne Master of Audiology student who contributed to the data collection.

Disclosure

Dr Karyn L Galvin is a Principal Investigator on several clinical studies sponsored by Cochlear Limited. These studies are entirely separate to the work included in the submitted paper. The authors declare that they have no other competing interests in this work.

Additional information

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.