Ayton Lauren N, Galvin Karyn L, Johansen Lauren, O'Hare Fleur, Shepard Emily R
Department of Optometry and Vision Sciences, University of Melbourne, Parkville, VIC, Australia.
Department of Surgery (Ophthalmology), University of Melbourne, Parkville, VIC, Australia.
J Multidiscip Healthc. 2023 Jul 13;16:1927-1936. doi: 10.2147/JMDH.S411306. eCollection 2023.
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.
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.
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.
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.
尤塞氏综合征是导致失聪失明的最常见原因，每6000人中就有1人受其影响。需要多学科护理，以使个人和家庭的治疗效果最大化。本研究评估了在与主要受影响的听觉和视觉相关护理领域工作的专职医疗临床医生对尤塞氏综合征的认知情况，即验光、斜视矫正和听力学领域。
2021年9月至2022年1月期间，对在澳大利亚大学附属医院工作的临床医生（7名验光师、1名斜视矫正师和4名听力学家）进行了一项前瞻性横断面在线调查。调查询问了关于尤塞氏综合征的病因、常见症状以及对管理该综合征的医疗专业人员的认知情况。
完成调查的27名听力学家、40名验光师和7名斜视矫正师中，有53名女性（71.6%），平均年龄37岁（范围24 - 70岁），平均临床经验为13年（范围1 - 45年）。大多数受访者正确将尤塞氏综合征识别为一种遗传性疾病（86%），识别出至少两种受影响的感官（97%），并认识到视力和听力损失的渐进性（>90%）。对前庭功能障碍及其特征的认知较低，对言语病理学家、遗传咨询师和遗传学家在尤塞氏综合征管理中所起的关键治疗作用的了解也较少。大多数受访者也未识别出其所在学科护理的重要方面。
本研究表明，需要对听力和视力护理领域的专职医疗临床医生进行有针对性的教育，以提高他们对尤塞氏综合征患者所经历的前庭影响和视力丧失方面的认识。这种教育需要针对在提供多学科护理中起关键作用的广泛临床医生（包括言语病理学家、遗传学家和遗传咨询师），并确定优质多学科护理的关键方面。
