A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy.
A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy.
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遗传性视网膜变性的虚拟现实定向和流动性测试：基因治疗后的概念验证测试。
DOI：
10.2147/opth.s292527
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Bennett J
中科院分区：
文献类型：
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作者：
Aleman TS;Miller AJ;Maguire KH;Aleman EM;Serrano LW;O'Connor KB;Bedoukian EC;Leroy BP;Maguire AM;Bennett J
测试虚拟现实（VR）定向和移动性（O&M）方案用于测量遗传性视网膜变性（IRD）患者的功能性视力的能力。使用市售VR硬件设计的VR-O&M方案在正常视力对照受试者（n=7;年龄10- 35岁;平均22.5岁）和患有RPE 65相关的Leber先天性黑蒙的患者（n=3;年龄7- 18岁;平均12.7岁）中进行测试，其中两人在基因治疗前后进行测试。患者接受视野检查和全视野敏感性测试。VR-O&M参数与视功能障碍相关。RPE 65患者的视力平均低于20/200，暗适应敏感度损失>5 log单位，视野收缩在20°和40°之间。在治疗前，患者需要约1000倍的明亮环境来导航，至少有4倍以上的碰撞，并且与对照受试者相比，定向和导航都较慢。锥细胞的改善（1-2 L.u.）和杆状病毒介导的（>4 L. u.）处理后的灵敏度导致在~100倍的较暗亮度下较少的碰撞（至少一半），并且导致快4倍的导航时间。这项研究提供了概念验证数据，支持使用VR-O&M系统来量化视觉功能障碍和治疗后视力改善对IRD功能性视力的影响。VR-O&M在潜在的挑战性场景中非常有用，例如患有严重IRD的儿科患者。VR-O&M测试将提供急需的灵活性，无论是在其部署方面，还是在转化研究环境中测试可能受基因治疗影响的各种视觉属性的可能性方面。这项研究提供了概念验证数据，支持使用虚拟现实定向和移动性测试来量化疾病及其治疗对遗传性视网膜变性中功能性视力的影响。
To test the ability of a virtual reality (VR) orientation and mobility (O&M) protocol to serve a measure of functional vision for patients with inherited retinal degenerations (IRDs). A VR-O&M protocol designed using a commercially available VR hardware was tested in normally sighted control subjects (n=7; ages 10–35yo; Average 22.5yo) and patients with RPE65-associated Leber Congenital Amaurosis (n=3; ages 7–18yo; Average 12.7yo), in two of them before and after gene therapy. Patients underwent perimetry and full-field sensitivity testing. VR-O&M parameters correlated with the visual dysfunction. Visual acuities in RPE65 patients were on average worse than 20/200, dark-adapted sensitivity losses >5 log units, and fields constricted between 20° and 40°. Before treatment, patients required ~1000-fold brighter environment to navigate, had at least x4 more collisions, and were slower both to orient and navigate compared to control subjects. Improvements in cone- (by 1–2 L.u.) and rod-mediated (by >4 L.u.) sensitivities post-treatment led to fewer collisions (at least by half) at ~100-fold dimmer luminances, and to x4 times faster navigation times. This study provides proof-of-concept data in support for the use of VR-O&M systems to quantify the impact that the visual dysfunction and improvement of vision following treatments has on functional vision in IRDs. The VR-O&M was useful in potentially challenging scenarios such as in pediatric patients with severe IRDs. A VR-O&M test will provide much needed flexibility, both in its deployment as well as in the possibility to test various attributes of vision that may be impacted by gene therapy in the setting of translational studies. This study provides proof-of-concept data in support for the use of a virtual reality orientation and mobility test to quantify the impact of the disease and of treatments thereof on functional vision in inherited retinal degenerations.