JAMA ophthalmology最新文献

Importance: Dual sensory impairment-that is, concurrent hearing and vision impairment-is common in aging populations. Individuals with dual sensory impairment face unique social and functional challenges, but the benefit of conventional rehabilitation in addressing these challenges is not well researched.

Objective: To determine the association between hearing impairment and the likelihood of achieving a clinically meaningful functional improvement following vision rehabilitation.

Design, setting, and participants: This was a cross-sectional analysis of data collected by the Low Vision Rehabilitation Outcomes Study, a prospective cohort study on clinical outcomes of vision rehabilitation. Data were included from outpatient low vision rehabilitation services provided at 28 clinical centers across the US between April 2008 and May 2011. The analyses included 611 adults with vision impairment and available self-reported hearing status. Of these, 407 had complete postrehabilitation follow-up data. Data were analyzed from July 2024 to March 2025.

Exposure: Self-reported hearing impairment status at baseline (normal hearing vs hearing impairment).

Main outcomes and measures: The primary outcome was the improvement in general capability as measured by the Activity Inventory before and after rehabilitation, computed using the method of successive dichotomizations. Rehabilitation was considered effective if the improvement reached a minimum clinically important difference (MCID). Multivariable logistic regression was used to evaluate the association between hearing impairment and achieving MCID. Covariates included age, sex, visual, psychological, physical, and cognitive status.

Results: Among the 611 participants (mean [SD; range] age, 73 [15.3; 19-101] years; 403 [66%] female), 358 had normal hearing and 253 had self-reported hearing impairment. Baseline ability did not differ by hearing status (β, -0.08; 95% CI, -0.56 to 0.41; P = .75). However, a smaller proportion of participants with hearing impairment achieved MCID following vision rehabilitation (39/169 [23%] vs 74/238 [31%]; odds ratio [OR], 0.58; 95% CI, 0.34-0.95; P = .03); this association was not attenuated by possession of hearing aid (17/74 [23%] vs 21/95 [22%]). In addition to better hearing status, participants with severe vision impairment (OR, 3.32; 95% CI, 1.2-11.86; P = .04) and higher depressive symptoms (OR, 1.38 per logit increase; 95% CI, 1.17-1.63; P < .001) were more likely to achieve MCID.

Conclusions and relevance: In this study, self-reported hearing impairment was associated with reduced likelihood of functional improvement following vision rehabilitation. These findings support the need for interdisciplinary efforts in rehabilitation programs to better serve individuals with dual sensory impairment.

Importance: Metachronous bilateral conversion in initially unilateral retinoblastoma is uncommon but clinically consequential, potentially requiring intensified treatment and carrying worse prognosis. Clarifying how age at diagnosis refines genetic-risk stratification could enable safer, more efficient surveillance protocols.

Objective: To estimate the incidence and timing of metachronous bilateral conversion in unilateral retinoblastoma and assess whether age at diagnosis and RB1 testing are associated with bilateral conversion risk.

Design, setting and participants: This was a retrospective cohort study at a tertiary center in Shanghai, China, including 1108 consecutive children with initially unilateral retinoblastoma diagnosed from July 2010 to October 2024 (after exclusions for short follow-up [n = 139], missing data [n = 53], or synchronous bilateral disease [n = 10]). The median (IQR) follow-up was 43.4 (24.2-67.6) months.

Exposures: Age at diagnosis and RB1 genetic status/subtypes assessed by next-generation sequencing and multiplex ligation-dependent probe amplification, including penetrance class (high vs low) and mosaic vs germline categorization.

Main outcomes and measures: Time to metachronous bilateral conversion; cumulative incidence functions with death as a competing risk; spatial distribution of fellow-eye tumors.

Results: Among 1108 patients (median [IQR] age at diagnosis, 22.2 [12.0-31.4] months; 591 [53.3%] male), 24 (2.2%) developed metachronous bilateral disease. At 24 months, cumulative incidence was 2.2% (95% CI, 1.3-3.1) overall. By genetic status, the 24-month cumulative incidence was 24.8% (95% CI, 13.8-35.9) in RB1 variant-positive vs 1.6% (95% CI, 0.0-3.1) in RB1 variant-negative patients. Among RB1 variant-positive patients, risk clustered among those diagnosed before 9 months, whereas no conversions were observed among those diagnosed at older than 9 months. Four RB1 variant-negative patients who were initially diagnosed at notably late ages (20.9, 42.7, 79.6, and 118 months) subsequently converted; these cases likely represent undetected low-level mosaicism, somatic variants below detection thresholds, or rare genomic events not captured by standard sequencing panels. Fellow-eye tumors did not involve macula and showed a nasal-predominant distribution.

Conclusions and relevance: The findings in this study suggest that age at diagnosis may refine genetic risk stratification for metachronous bilateral conversion. RB1 variant-positive patients diagnosed at 9 months or later represent a very low-risk subgroup that may warrant surveillance deescalation, while rare late conversions in RB1 variant-negative patients necessitate continued long-term monitoring.