Background: Previously, we discovered a strain of Kunming mice, referred to as the KMush/ush strain, that exhibited notably abnormal electroretinogram (ERG) readings and elevated thresholds for auditory brainstem responses (ABRs), which resembled the characteristics of Usher Syndrome (USH). We successfully identified the pathogenic genes, Pde6b and Adgrv1, after KMush/ush crossbred with CBA/CaJ mice, referred to as CBA-1ush/ush, CBA-2ush/ush or CBA-2ush/ush. In this investigation, we crossbred KMush/ush and CBA/J mice to establish novel recombinant inbred lines and analysed their phenotypic and genotypic characteristics. Methods: ERG readings, ABR testing, fundus morphology, histological examination of the retina and inner ear, reverse transcription-quantitative polymerase chain reaction (RT-qPCR) analysis, western blotting, DNA sequence analysis and behavioural experiments were performed to assess the phenotypes and genotypes of the progeny lines. Results: No obvious waveforms in the ERG were detected in F1 hybrid mice while normal ABR results were recorded. The F2 hybrids, which were called J1ush/ush or J2ush/ush, exhibited segregated hearing-loss phenotypes. J1ush/ush mice had a retinitis pigmentosa (RP) phenotype with elevated ABR thresholds, whereas J2ush/ush mice exhibited only the RP phenotype. Interestingly, J1ush/ush mice showed significantly higher ABR thresholds than wild-type mice at 28 days post born (P28), and RT-qPCR and DNA-sequencing analysis showed that Adgrv1 gene expression was significantly altered in J1ush/ush mice, but histological analysis showed no significant structural changes in the organ of Corti or spiral ganglia. Further elevation of ABR-related hearing thresholds by P56 manifested only as a reduced density of spiral ganglion cells, which differed significantly from the previous pattern of cochlear alterations in CBA-2ush/ush mice. Conclusions: We successfully introduced the hearing-loss phenotype of inbred mice with USH into CBA/J mice, which provides a good animal model for future studies on the important physiological roles of the Adgrv1 gene in inner-ear structure and for therapeutic studies targeting Adgrv1-mutated USH.

目的 研究不同模拟飞行光环境下,不同视力受试者动态视力(DVA)的差异,为保障飞行安全提供试验依据.方法 招募24名受试者,其中6名视力正常者为1.0/1.0视力组(小数视力),18名近视受试者随机分为0.8/0.8视力组、0.6/0.6视力组和0.4/0.4视力组,每组6人.在模拟日光、迎光、背光、曙暮光和云上光环境下,通过观察3.0、4.5、6.0、7.5 km/h运动速度的视标检测DVA.采用具有2个重复测量因素的三因素重复测量方差分析,比较不同光环境对不同视力者DVA的影响.结果 光环境、静态视力和运动速度3个因素间不存在交互作用(P>0.05).通过进一步两因素交互作用检验,分析发现光环境和静态视力存在交互作用(P<0.05),运动速度和静态视力存在交互作用(P<0.05),光环境和运动速度不存在交互作用(P>0.05).与日光环境相比,所有视力组受试者在背光下的DVA显著提高(P<0.001),在曙暮光、迎光下的DVA均显著下降(P<0.001).除0.4/0.4视力组外,其余视力组在云上光环境下的DVA显著低于日光(P<0.05).在相同的模拟光环境下,1.0/1.0视力组DVA最高,0.8/0.8视力组高于0.6/0.6视力组,0.4/0.4视力组最低,差异有统计学意义(P<0.05).对于3.0、4.5、6.0 km/h运动速度的视标,1.0/1.0视力组DVA最高,0.8/0.8视力组高于0.6/0.6视力组,0.4/0.4视力组最低,差异有统计学意义(P<0.05).1.0/1.0视力组观察7.5 km/h运动速度视标的DVA显著高于低视力组(P<0.05),0.4/0.4视力组低于0.8/0.8和0.6/0.6视力组,差异有统计学意义(P<0.05).所有视力组受试者DVA均随目标运动速度的增加而显著下降(P<0.05).结论 光环境和静态视力水平、运动速度和静态视力水平对DVA水平的作用相互影响.近视者在不利光环境(如迎光、云上光和曙暮光)下观察快速运动目标时,DVA严重下降,应引起飞行人员和航空医学工作者的重视.

目的 比较同一体质量区间不同品系大鼠、小鼠眼轴的差别.方法 选用野生型(SD)大鼠、实验室现有的不同品系大鼠和小鼠:先天性静止性夜盲(CSNB)大鼠、卷毛(HL)大鼠、视网膜锥细胞失功能(RCD)大鼠、C57BL/6小鼠、锥体光感受器功能丧失 1(cpfl1)小鼠、锥体光感受器功能丧失 3(cpfl3)小鼠、先天性静止性夜盲(Nob2)小鼠,按照体质量划分为不同组别(大鼠以 50 g为一区间分为 10 组,小鼠以 10 g为一区间共分为两组),测量其眼轴,在同一体质量区间比较不同品系大鼠和小鼠之间的眼轴区别.结果 大鼠与小鼠眼轴长度随体质量增加而增长,在不同品系之间存在差异.其中 100～150 g体质量的 CSNB大鼠眼轴长度大于 SD大鼠(P<0.05).150～200 g体质量的 RCD大鼠眼轴长度大于 SD 大鼠(P<0.01),但 SD 与 CSNB 大鼠之间不具有统计学差异;200～250 g、450～500 g、500～550 g体质量的 HL与 RCD大鼠眼轴较 SD大鼠长(P<0.05),但 SD 与 CSNB 大鼠之间没有统计差异;250～300 g与 300～350 g体质量的三种品系大鼠眼轴均长于 SD 大鼠(P<0.01);350～400 g 体质量的 CSNB与 RCD大鼠眼轴长于 SD大鼠(P<0.01),但 SD大鼠与 HL 大鼠之间无统计学差异;400～450 g 体质量的四种品系大鼠眼轴均无统计学差异;550～600 g体质量的 HL大鼠眼轴较 SD大鼠长(P<0.05),CSNB与 SD 大鼠之间无统计差异.全部体质量的 C57BL/6 小鼠眼轴长度短于其余 3 个品系的小鼠(P<0.01);结论 不同体质量区间,各基因突变大鼠或小鼠眼轴长于对照鼠(SD大鼠 C57BL/6 小鼠)眼轴,且在大鼠中,此差异在发育期更为明显.

Background To develop machine learning models for objectively evaluating visual acuity (VA) based on pattern-reversal visual evoked potentials (PRVEPs) and other related visual parameters. Methods Twenty-four volunteers were recruited and forty-eight eyes were divided into four groups of 1.0, 0.8, 0.6, and 0.4 (decimal vision). The relationship between VA, peak time, or amplitude of P100 recorded at 5.7°, 2.6°, 1°, 34′, 15′, and 7′ check sizes were analyzed using repeated-measures analysis of variance. Correlations between VA and P100, contrast sensitivity (CS), refractive error, wavefront aberrations, and visual field were analyzed by rank correlation. Based on meaningful P100 peak time, P100 amplitude, and other related visual parameters, four machine learning algorithms and an ensemble classification algorithm were used to construct objective assessment models for VA. Receiver operating characteristic (ROC) curves were used to compare the efficacy of different models by repeated sampling comparisons and ten-fold cross-validation. Results The main effects of P100 peak time and amplitude between different VA and check sizes were statistically significant (all P < 0.05). Except amplitude at 2.6° and 5.7°, VA was negatively correlated with peak time and positively correlated with amplitude. The peak time initially shortened with increasing check size and gradually lengthened after the minimum value was reached at 1°. At the 1° check size, there were statistically significant differences when comparing the peak times between the vision groups with each other (all P < 0.05), and the amplitudes of the vision reduction groups were significantly lower than that of the 1.0 vision group (all P < 0.01). The correlations between peak time, amplitude, and visual acuity were all highest at 1° ( r s = − 0.740, 0.438). VA positively correlated with CS and spherical equivalent (all P < 0.001). There was a negative correlation between VA and coma aberrations ( P < 0.05). For different binarization classifications of VA, the classifier models with the best assessment efficacy all had the mean area under the ROC curves (AUC) above 0.95 for 500 replicate samples and above 0.84 for ten-fold cross-validation. Conclusions Machine learning models established by meaning visual parameters related to visual acuity can assist in the objective evaluation of VA.

The traditional methods for the control of myopia development are optical correction, orthokeratology lens, defocus glasses and other methods, however, these approaches are poor. At present, it has been demonstrated that outdoor activities and the application of low concentration atropine had a positive effect on preventing the occurrence of myopia and controlling the growth of myopia diopter. The occurrence and development of axial myopia was related to scleral remodeling toward myopic direction, but the mechanism remains unclear. In addition, true myopia caused by the axial growth was irreversible. After long-term spaceflight, astronauts had posterior eyeball flattening, axial shortening, and hyperopic displacement of refractive state, indicating that stress environment could cause sclera remodeling. Therefore, the combination of appropriate exercise to promote the transfer of blood head and increase the hydrostatic pressure gradient of the head and eye tissue might affect the local scleral remodeling and shorten the axial direction of the eye, or delay the trend of axial growth, which could provide a new idea for the prevention and control of myopia.

AIM: To study the effects of the specific simulated luminous environment on the visual performance of people with different vision, so as to provide an experimental basis for revising pilots' vision standards. METHODS: A controlled randomized trial was conducted. Twenty-four volunteers were recruited and divided into four groups(1.0/1.0, 0.8/0.8, 0.6/0.6 and 0.4/0.4, decimal vision)according to right/left eye visual acuity, with six subjects in each group. Each subject was tested for static distant vision, kinetic visual acuity, color vision, depth perception error and visual search time under the simulated luminous environments of sunlight, twilight, and on-cloud, respectively, to compare changes in the impact of distinctive luminous surroundings on the visual performance indicators of human beings with different vision.RESULTS: There were main effect differences in static distant vision, kinetic visual acuity, color error, depth perception error and visual search time under different light environments(all P<0.01). The binocular static distant visual acuity, abilities of color discrimination, depth perception and visual search in simulated sunlight environment were higher than those in simulated twilight and on-cloud environments. In the 0.4/0.4 vision group, kinetic vision in simulated twilight and on-cloud environments were significantly lower than that in simulated sunlight environment(P<0.01). There were main effect differences in binocular static distant vision, kinetic visual acuity, depth perception error and visual search time among subjects with different vision(all P<0.05). Compared with 1.0/1.0 vision group, those with 0.6/0.6 and 0.4/0.4 vision had significant decrease in kinetic visual acuity, depth perception ability and visual search ability(all P<0.05). CONCLUSION: Different luminous environments have a great impact on the visual performance of people with low vision, which poses a potential threat to flight safety.

目的 调查现役飞行人员视网膜周边退行性变性的患病情况,为招飞体检和飞行人员医学鉴定标准提供数据支持.方法 采用Daytona P200T设备,对2019年10月～12月在某疗养院疗养的562名飞行人员进行眼底图像采集,每眼采集正前方、鼻侧、颞侧、上方、下方5个方位4个眼底周边视网膜图像,检查其视网膜周边退行性变性患病情况,并对检查结果按照年龄、飞行时间、机型进行分组统计分析.结果 562名受检飞行人员中有93人存在不同类型视网膜周边退行性变性,其中视网膜周边非压迫性白为8.01％;视网膜周边囊样变性为3.74％,视网膜周边干性裂孔为1.96％、视网膜周边格子样变性为1.96％,视网膜周边蜗牛足迹变性为0.53％,视网膜周边马蹄形裂孔为0.18％,视网膜铺路石样变性为0.18％,总患病率为16.55％;视网膜周边退行性变性与年龄、飞行时间和机型之间没有相关关系.结论 年度体检合格的飞行人员人群中视网膜周边退行性变性仍有一定的患病率,视网膜周边退行性变性及其类型对飞行安全的潜在影响仍需要进一步评估.