Edwards A, Fishman G A, Anderson R J, Grover S, Derlacki D J
Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, 60612, USA.
Arch Ophthalmol. 1998 Feb;116(2):165-8. doi: 10.1001/archopht.116.2.165.
To determine the extent of visual acuity and visual field impairment in patients with types 1 and 2 Usher syndrome.
The records of 53 patients with type 1 and 120 patients with type 2 Usher syndrome were reviewed for visual acuity and visual field area at their most recent visit. Visual field areas were determined by planimetry of the II4e and V4e isopters obtained with a Goldmann perimeter. Both ordinary and logistic regression models were used to evaluate differences in visual acuity and visual field impairment between patients with type 1 and type 2 Usher syndrome.
The difference in visual acuity of the better eye between patients with type 1 and type 2 varied by patient age (P=.01, based on a multiple regression model). The maximum difference in visual acuity between the 2 groups occurred during the third and fourth decades of life (with the type 1 patients being more impaired), while more similar acuities were seen in both younger and older patients. Fifty-one percent (n=27) of the type 1 patients had a visual acuity of 20/40 or better in at least 1 eye compared with 72% (n=87) of the type 2 patients (age-adjusted odds ratio, 3.9). Visual field area to both the II4e (P=.001) and V4e (P<.001) targets was more impaired in the better eye of type 1 patients than type 2 patients. A concentric central visual field greater than 20 degrees in at least 1 eye was present in 20 (59%) of the available 34 visual fields of type 1 patients compared with 70 (67%) of the available 104 visual fields of type 2 patients (age-adjusted odds ratio, 2.9) with the V4e target and in 6 (21%) of the available 29 visual fields of type 1 patients compared with 36 (38%) of the available 94 visual fields of type 2 patients (age-adjusted odds ratio, 4.9) with the II4e target. The fraction of patients who had a visual acuity of 20/40 or better and a concentric central visual field greater than 20 degrees to the II4e target in at least 1 eye was 17% (n=5) in the type 1 patients and 35% (n=33) in the type 2 patients (age-adjusted odds ratio, 3.9).
Visual acuity and visual field area were more impaired in patients with type 1 than type 2 Usher syndrome. Of note, 27 of 53 type 1 (51%) and 87 of 120 type 2 (72%) patients had a visual acuity of 20/40 or better in at least 1 eye. These data are useful for overall counseling of patients with Usher syndrome.
确定1型和2型Usher综合征患者的视力和视野损害程度。
回顾了53例1型和120例2型Usher综合征患者最近一次就诊时的视力和视野面积记录。视野面积通过使用Goldmann视野计测量II4e和V4e等视线的平面测量法来确定。使用普通回归模型和逻辑回归模型评估1型和2型Usher综合征患者在视力和视野损害方面的差异。
1型和2型患者较好眼的视力差异因患者年龄而异（基于多元回归模型，P = 0.01）。两组之间视力的最大差异出现在生命的第三个和第四个十年（1型患者受损更严重），而在年轻和老年患者中视力更相似。51%（n = 27）的1型患者至少一只眼睛的视力为20/40或更好，而2型患者为72%（n = 87）（年龄调整后的优势比为3.9）。1型患者较好眼相对于2型患者，对II4e（P = 0.001）和V4e（P < 0.001）目标的视野面积受损更严重。在1型患者的34个可用视野中，20个（59%）至少一只眼睛存在大于20度的同心中心视野，而2型患者的104个可用视野中有70个（67%）（年龄调整后的优势比为2.9）；对于II4e目标，1型患者的29个可用视野中有6个（21%），而2型患者的94个可用视野中有36个（38%）（年龄调整后的优势比为4.9）。至少一只眼睛视力为20/40或更好且对II4e目标有大于20度同心中心视野的患者比例，1型患者为17%（n = 5），2型患者为35%（n = 33）（年龄调整后的优势比为3.9）。
1型Usher综合征患者的视力和视野面积损害比2型患者更严重。值得注意的是，53例1型患者中有27例（51%）和120例2型患者中有87例（72%）至少一只眼睛的视力为20/40或更好。这些数据有助于对Usher综合征患者进行全面的咨询。
