Disease mechanisms have become better understood in previously incurable forms of early-onset severe retinal dystrophy, such as Leber congenital amaurosis (LCA). This has led to novel treatments and clinical trials that have shown some success. Standard methods to measure vision were difficult if not impossible to perform in severely affected patients with low vision and nystagmus. To meet the need for visual assays, we devised a psychophysical method, which we named full-field stimulus testing (FST). From early versions based on an automated perimeter, we advanced FST to a more available light-emitting diode platform. The journey from invention to use of such a technique in our inherited retinal degeneration clinic is reviewed and many of the lessons learned over the 15 years of application of FST are explained. Although the original purpose and application of FST was to quantify visual thresholds in LCA, there are rare opportunities for FST also to be used beyond LCA to measure aspects of vision in other inherited retinal degenerations; examples are given. The main goal of the current review, however, remains to enable investigators studying and treating LCA to understand how to best use FST and how to reduce artefact and confounding complexities so the test results become more valuable to the understanding of LCA diseases and results of novel interventions.

在以前无法治愈的早发性严重视网膜营养不良形式中，疾病机制已经得到了更好的理解，例如 Leber 先天性黑蒙 (LCA)。这导致了新的治疗方法和临床试验，并取得了一些成功。在视力低下和眼球震颤的严重患者中，测量视力的标准方法即使不是不可能也很困难。为了满足视觉分析的需要，我们设计了一种心理物理方法，我们将其命名为全场刺激测试 (FST)。从基于自动边界的早期版本，我们将 FST 提升到更可用的发光二极管平台。回顾了从发明到在我们的遗传性视网膜变性诊所中使用这种技术的过程，并解释了 15 年来应用 FST 的许多经验教训。尽管 FST 的最初目的和应用是量化 LCA 中的视觉阈值，但 FST 也很少有机会在 LCA 之外用于测量其他遗传性视网膜变性的视力方面。给出了例子。然而，当前审查的主要目标仍然是使研究和治疗 LCA 的研究人员能够了解如何最好地使用 FST 以及如何减少伪影和混杂复杂性，从而使测试结果对理解 LCA 疾病和新型结果更有价值干预。