To determine the extent of visual acuity and visual field impairment in patients with types 1 and 2 Usher syndrome.
Methods
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.
Results
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° 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° 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).
Conclusions
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.
BackgroundVision related quality of life (VRQOL) measures one's satisfaction with their visual ability to perform daily activities. Aging leads to vision impairment that can negatively affect VRQOL and physical, psychological, and social well-being.ObjectiveTo assess vision-related quality of life (VR-QOL) and its determinants among elderly residents from rural blocks of Haryana, India.MethodThis cross-sectional study was performed across 52 Indian villages. The vision-related quality of life scores was assessed using National Eye Institute Vision Function Questionnaire-25(VFQ-25). Social Connectedness, Geriatric Depression Scale, Mini-Mental Status Examination, and the Katz Index of Independence were collected using validated tools. Continuous data was represented as Mean (±SD) or median (IQR) depending upon their distribution. Categorical data as frequency (%). Bivariate analysis was done using Student t-test or Rank Sum test for continuous variables (for up to 2 groups) and One-Way ANOVA was run for continuous variables (for more than 2 groups). Chi-Sq. test or Fisher's for categorical variables and multivariable linear regression model was run to calculate the determinants of the VFQ composite score.ResultAmongst the 420 participants, 58% were females and 74% were illiterates. The mean VR-QOL was 42.30 ± 11.19 in the study population. The study found higher VR-QOL scores in General Health, Distance activities, and Near activities, while lower scores were observed in Mental health, Dependency, Social functioning, Ocular pain, General vision, and Role difficulties. Both the mean VR-QOL composite score and the VR-QOL domains of General Health, General Vision, Ocular Pain, Role Difficulties, Mental Health, and Dependence were all greater for women than for men. Among the reported morbidities 26% had hearing loss, 23% were hypertensives, 9% were diabetics, 85% were dependent, 60% had depression, 14% had moderate dementia, 10% had lower tendency to connect, 8% had low vision and 35% had vertigo. VFQ scores were lower among males, non-Hindus, hypertensives and among person with history of vertigo and those living in dim light than their counterparts.ConclusionThe current study shows that VR-QOL is significantly lower in male, participants with history of vertigo, hypertension, diabetes and who live in dim light. There is a need to develop strategies to address these determinants through multidisciplinary approach including chronic disease management and environmental modification and promote healthy aging of elderly population.
Purpose. The purpose of this study was to gain information about the employment status of legally blind patients. Methods. Fifty-two patients with one of four juvenile-onset macular dystrophies or achromatopsia responded to questions about their employment histories and their psychological well-being. Results from the questionnaire were analyzed using z-tests for differences in proportions or t-tests for differences in means. Results. Forty-eight percent of the patients reported that they were employed and 52% that they were not employed. The subgroup that was not employed had a significantly higher proportion of women than men, whereas the employed group had approximately equivalent proportions of men and women. The employed subgroup reported that their success at work was due to social support. This subgroup had significantly higher household incomes, was significantly less likely to collect disability-income benefits, had significantly higher educational levels, had significantly higher positive affect, and had significantly lower negative affect than the subgroup that was not employed. A logistic regression analysis indicated that education was the primary predictor of employment. Conclusion. Analysis supports the conclusion that it is beneficial for legally blind individuals to obtain an optimal level of education and receive suitable social support to facilitate their successful employment.
Objective . To prospectively evaluate the effect of intravitreal bevacizumab on aqueous levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF) in patients with exudative age-related macular degeneration (AMD) and correlate clinical outcomes with cytokine levels. Methods . 30 eyes of 30 patients with exudative AMD underwent intravitreal injection of bevacizumab three times at monthly intervals. The aqueous samples prior to the 1st injection (baseline) and 3rd injection were analyzed for VEGF and IL-6 levels. Subjects were subgrouped based upon change in the central subfield (CSF) macular thickness on SD-OCT at 8 weeks. Group 1 included patients (n=14) with a decrease in CSF thickness greater than 10% from the baseline (improved group). Group 2 included patients (n=16) who had a decrease in CSF thickness 10% or less (treatment-resistant). Results . In subgroup analysis, in both groups 1 and 2 patients, compared to aqueous VEGF, aqueous IL-6 levels showed a better correlation with CSF thickness on SD-OCT (r=0.72and 0.71, resp.). Conclusions . Aqueous IL-6 may be an important marker of treatment response or resistance in wet macular degeneration. Future therapeutic strategies may include targeted treatment against both VEGF and IL-6, in patients who do not respond to anti-VEGF treatment alone.
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