The Developmental Eye Movement Test Does Not Detect Oculomotor Problems: Evidence from Children with Nystagmus.

SIGNIFICANCE: The Developmental Eye Movement (DEM) test, a test purported to assess oculomotor skills, does not detect eye movement disorder in nystagmus syndromes. The test should not be used for the clinical evaluation of oculomotor disorders. PURPOSE: The DEM test ratio compares a horizontal number naming subtest with a vertical one to identify oculomotor problems independent of a child's visual-verbal naming skills. Here, we tested the construct validity of this method by comparing scores of children with and without pathologic nystagmus. Such a nystagmus disturbs normal fixation and saccadic behavior because of the presence of involuntary rhythmic oscillations of the eyes. Therefore, if the ratio is indeed a comprehensive measure of oculomotor problems, children with nystagmus should show an increased ratio score. METHODS: The DEM test performances of normally sighted children (n = 94), children with ocular visual impairments (VI o ; n = 33), and children with cerebral visual impairment (n = 30) were analyzed using linear regression. Part of the children with VI o and cerebral visual impairment had either fusion maldevelopment nystagmus syndrome (n = 8) or infantile nystagmus syndrome (n = 20), whereas the others showed no pathologic nystagmus. RESULTS: The times needed for the horizontal and vertical subtests were significantly different between children with normal vision, VI o , and cerebral visual impairment ( P < .001). However, the presence of nystagmus did not add significantly to the horizontal and vertical times ( P > .20), nor did it have an effect on the ratio ( P > .10). CONCLUSIONS: The DEM test ratio is not sensitive to fixation and saccade abnormalities associated with nystagmus, indicating that it does not have general construct validity to detect true eye movement disorders. Although not suitable for the evaluation of oculomotor disorders, the subtests do have clinical relevance in the diagnosis of cerebral visual impairment.

The speed acuity test as a diagnostic aid in cerebral visual impairment.

One of the characteristics of children with cerebral visual impairments (CVI) is that they need more time to process visual information. However, currently, few tests are available that can reliably measure visual processing speed. The speed acuity test, a discrimination reaction-time test in which participants indicate the orientation of Landolt-C symbols as quickly and accurately as possible, was specifically developed to determine the time a child needs to discern visual details. The test measures both the accuracy and the latency of the responses for nine different optotype sizes in order to control for decreased visual acuity. The results show that children with CVI need significantly more time to respond to the largest optotype sizes than age-matched normally sighted children and children with visual impairments due to an ocular disorder (VIo). This effect is independent of the time it takes to make a motor response. However, the reaction-time difference between the children with CVI and VIo is not seen for optotype sizes at the acuity threshold. Together with reaction times on visual and auditory detection tasks as controls, reaction times measured in the speed-acuity test allow for acceptable discrimination (AUC in ROC analysis: 0.81) between CVI and VIo.

The Developmental Eye Movement Test as a Diagnostic Aid in Cerebral Visual Impairment.

The symptoms that characterize children with cerebral visual impairments (CVI) are diverse, ranging from extensive behavioral or physical disabilities to subtle changes that can easily be missed. A correct diagnosis of CVI is therefore difficult to make, but having a wide variety of tests available can be helpful. This study aims to determine if the developmental eye movement test (DEM) can be one of those tests. In this test, a fixed set of numbers has to be read aloud, first in vertical columns and then in horizontal lines. In order to measure differences between children with CVI compared to normally sighted age-matched controls and children with a visual impairment (VI), we determined DEM times, crowding intensities and the reaction time to a large visual stimulus for all three groups. We found that children with CVI or VI need significantly more time to read the DEM numbers than age-matched controls. Additionally, children with CVI need more time than children with VI to read the horizontal DEM, but not the vertical DEM. We also found a significant difference between the children with CVI and the other two groups in the relationship between horizontal DEM performance and crowding intensity. However, for the relationship between DEM performance and visual detection time, no group-differences were found. We conclude that the DEM can be a useful addition in the diagnosis of CVI, especially in combination with information about crowding.

One-year effects of bifocal and unifocal glasses on executive functions in children with Down syndrome in a randomized controlled trial.

Appropriate glasses can improve visual functioning of children with Down syndrome (DS), but it is unknown if such interventions influence their cognitive impairments. In a randomized controlled trial with 1-year follow-up. Children with DS (2-16 years) were provided either bifocal glasses (add +2.5 Dioptres; n = 50) or unifocal glasses (n = 52). Executive functions were assessed pre- and post-intervention with the task-based Minnesota Executive Function Scale (MEFS) and with questionnaires, BRIEF-P and BRIEF, parents' and teachers' version. Intervention effects and associations between executive functions, (near) vision and ocular alignment were analysed. Intervention improved MEFS-Total-scores in the bifocal group (p = 0.002; Cohen's d = 0.60) but not in the unifocal group (p = 0.191; Cohen's d = 0.24). Post-intervention, there was no intergroup difference (p = 0.120; Cohen's d = 0.34). Post-intervention, higher MEFS-scores were associated with better visual acuities (crowded near p = 0.025; uncrowded near p = 0.019; distant p = 0.045). Pre-post changes in MEFS-scores correlated significantly with improved ocular alignment (p = 0.040). Exploratory analysis of the questionnaires showed improved teacher-rated BRIEF-scores in both groups (bifocals: p = 0.014, Cohen's d = 1.91; unifocals: p = 0.022, Cohen's d = 1.46), with no intergroup difference (p = 0.594; Cohen's d = 0.23). These results demonstrate positive effects of wearing better-correcting glasses on executive functioning in children with DS, suggesting a link between their visual and executive functioning. However, the relative contributions of distant and near vision need further study.

Differences between children with Down syndrome and typically developing children in adaptive behaviour, executive functions and visual acuity.

In children with Down syndrome (DS) development of visual, motor and cognitive functions is atypical. It is unknown whether the visual impairments in children with DS aggravate their lag in cognitive development. Visual impairment and developmental lags in adaptive behaviour and executive functions were assessed in 104 children with DS, 2-16 years, by comparing their adaptive behaviour, executive functions and visual acuity (distant and near) scores against published age-matched norm scores of typically developing children. Associations between these lags were explored. Mean (± SEM) differences to age-matched norms indicated reduced performance in DS: Vineland Screener questionnaire, - 63 ± 3.8 months; task-based Minnesota Executive Function Scale (MEFS), - 46.09 ± 2.07 points; BRIEF-P questionnaire, 25.29 ± 4.66 points; BRIEF parents' and teachers' questionnaire, 17.89 ± 3.92 points and 40.10 ± 3.81 points; distant and near visual acuity, 0.51 ± 0.03 LogMAR and 0.63 ± 0.03 LogMAR (near - 0.11 ± 0.04 LogMAR poorer than distant). Adaptive behaviour (Vineland-S) correlated with the severity of visual impairment (r = - 0.396). Children with DS are severely impaired in adaptive behaviour, executive functions and visual acuities (near visual acuity more severely impaired than distant visual acuity). Larger impairment in adaptive behaviour is found in children with larger visual impairment. This supports the idea that visual acuity plays a role in adaptive development.

Visual fixations rather than saccades dominate the developmental eye movement test.

When children have visual and/or oculomotor deficits, early diagnosis is critical for rehabilitation. The developmental eye movement (DEM) test is a visual-verbal number naming test that aims to measure oculomotor dysfunction in children by comparing scores on a horizontal and vertical subtest. However, empirical comparison of oculomotor behavior during the two subtests is missing. Here, we measured eye movements of healthy children while they performed a digital version of the DEM. In addition, we measured visual processing speed using the Speed Acuity test. We found that parameters of saccade behavior, such as the number, amplitude, and direction of saccades, correlated with performance on the horizontal, but not the vertical subtest. However, the time spent on making saccades was very short compared to the time spent on number fixations and the total time needed for either subtest. Fixation durations correlated positively with performance on both subtests and co-varied tightly with visual processing speed. Accordingly, horizontal and vertical DEM scores showed a strong positive correlation with visual processing speed. We therefore conclude that the DEM is not suitable to measure saccade behavior, but can be a useful indicator of visual-verbal naming skills, visual processing speed, and other cognitive factors of clinical relevance.

LONGITUDINAL STUDY OF RPE65-ASSOCIATED INHERITED RETINAL DEGENERATIONS.

PURPOSE: To study the disease course of RPE65-associated inherited retinal degenerations (IRDs) as a function of the genotype, define a critical age for blindness, and identify potential modifiers. METHODS: Forty-five patients with IRD from 33 families with biallelic RPE65 mutations, 28 stemming from a genetic isolate. We collected retrospective data from medical charts. Coexisting variants in 108 IRD-associated genes were identified with Molecular Inversion Probe analysis. RESULTS: Most patients were diagnosed within the first years of life. Daytime visual function ranged from near-normal to blindness in the first four decades and met WHO criteria for blindness for visual acuity and visual field in the fifth decade. p.(Thr368His) was the most common variant (54%). Intrafamilial variability and interfamilial variability in disease severity and progression were observed. Molecular Inversion Probe analysis confirmed all RPE65 variants and identified one additional variant in LRAT and one in EYS in two separate patients. CONCLUSION: All patients with RPE65-associated IRDs developed symptoms within the first year of life. Visual function in childhood and adolescence varied but deteriorated inevitably toward blindness after age 40. In this study, genotype was not predictive of clinical course. The variance in severity of disease could not be explained by double hits in other IRD genes.

Saccade latencies during a preferential looking task and objective scoring of grating acuity in children with and without visual impairments.

PURPOSE: We determined the latencies of orienting responses during a preferential looking task in children with normal vision and in children with visual impairments between 6 and 12 years old, and assessed the feasibility of scoring grating detection in these populations with video-based eye tracking. METHODS: Children performed a computerized preferential looking test, while a remote eye tracker measured the children's eye movements. The stimuli consisted of a 2 × 2 grid, with three uniform grey fields and one target field consisting of a black-and-white square wave grating. The grating was presented randomly at one of the four locations. The spatial frequencies (1.05, 2.11 and 7.02 cyc/deg) were randomly interleaved, with 10 trials per spatial frequency. Three different methods were used to score the accuracy of the responses: (1) primary saccade ends on target, (1) gaze 50% of the presentation time on target, and (3) a combination of method 1 and 2 (i.e. primary saccade ends on target, and/or gaze 50% of the presentation time on target). RESULTS: The combined scoring method was most reliable to determine whether children could resolve the gratings. Children with visual impairments had significantly lower accuracies than children with normal vision with all three scoring methods. In addition, saccade latencies decreased with age and were significantly longer (62 ± 15 ms) in children with visual impairments. CONCLUSION: The use of eye tracking to assess grating detection with a preferential looking task in clinical populations provides valuable additional information, including objective detection measures and developmental delays in saccade latencies.

Symbol Discrimination Speed in Children With Visual Impairments.

Purpose: We measured visual acuity and visual discrimination speed simultaneously in children with visual impairments to determine whether they are slower than children with normal vision. Methods: Five- to twelve-year-old children with visual impairments due to ocular dysfunction (VIo; n = 30) or cerebral visual impairment (CVI; n = 17) performed a speed-acuity test in which they indicated the orientation of Landolt-C symbols as quickly and accurately as possible. The reaction times for symbols ranging between -0.3 and 1.2 logMAR relative to acuity threshold were compared with normative data. To test whether children were already slow in merely detecting symbols, we also compared their reaction times on a simple visual detection task (VDT) to normative data. An auditory detection task (ADT) was used to probe for other, more general deficits. Results: Of the children with visual impairments, 88% had abnormally long reaction times in the speed-acuity test. This deficit was partly explained by their reduced acuity, but 40% still needed more time to discriminate acuity-matched optotypes. Children responded late in the VDT too, especially those with CVI, but this impairment could not fully account for their slow symbol discrimination. In children with CVI, reaction times in the ADT were affected as much as those in the VDT, suggesting more general sensorimotor problems in CVI. Conclusions: The speed-acuity test offers additional insight in visual impairment. Children with VIo and CVI are abnormally slow in discerning foveal details. Magnification of materials is often insufficient to compensate for this deficit, partly because stimulus detection is already hampered.

Development of Symbol Discrimination Speed in Children With Normal Vision.

Purpose: Many visually guided tasks require rapid perception of visual details, but how fast children can discern foveal stimuli and how this ability improves with age are still unknown. To fill this gap, we tested normally sighted children between 5 and 12 years of age with a combined symbol-discrimination reaction-time test. Methods: Children (n = 94) had to indicate, as fast and accurately as possible, the orientation of a Landolt C symbol (90 trials). Task difficulty was manipulated by varying symbol size (-0.43 to 1.09 logMAR at 5 m). The resulting reaction times were analyzed with a drift-diffusion model. Reaction times on a visual and auditory detection task were measured to assess the contribution of other factors, such as delays in stimulus detection and executing the motor response. Results: Detection and discrimination were significantly faster in older children. Five-year-olds needed ∼440 ms for visual detection and ∼980 ms for discrimination of the largest symbols while 12-year-olds needed only ∼250 ms and ∼500 ms for this. The extra time needed for discrimination compared with detection decreased with age. The decrease in reaction time with increasing optotype size was also age-dependent and indicated an increase in sensitivity with age. Despite the time pressure, acuity thresholds were normal (within the EN ISO-8597 standard). Conclusions: Our data revealed substantial developmental improvements in visual discrimination speed, which suggests that an important optimization takes place in the developing visual system of 5- to 12-year-old children. Since the speed-acuity test allows for quick and reliable assessment of visual recognition acuity and speed, it may be useful in clinical testing too.

Useful field of view test performance throughout adulthood in subjects without ocular disorders.

Previous research has shown an age-related decline in Useful Field of View (UFOV) test performance, which measures the duration required to extract relevant information from a scene in three subtasks. However, these results are mostly based on data that may have been confounded by (age-related) ocular diseases. We examined UFOV performance in subjects aged 19.5 to 70.3 years to investigate how UFOV performance changes throughout adulthood. All subjects underwent a thorough ophthalmological examination to exclude ocular disorders. We also examined some elementary visual functions, i.e., near and far visual acuity, crowding and contrast sensitivity. We investigated whether these functions were related to age and whether they could explain a possible age-related decline in UFOV performance. The subjects (n = 41) performed very well on almost every measure and reached far better UFOV and visual acuity scores than those reported by other studies that relied on self-reported absence of ocular pathology. We did not find significant relationships between age and any of the elementary visual functions or the first two UFOV subtasks (R2UFOV1 = 0.03, p = 0.25; R2UFOV2 = 0.07, p = 0.10). However, we found an age-related decline in performance on the third UFOV subtask (R2UFOV3 = 0.36, p < 0.001), which was unrelated to performance on the elementary visual function tasks. Our results show that performance on the first two UFOV subtasks as well as central elementary visual functions may remain high in the absence of obvious ophthalmological pathology.

Development and validation of a high-speed stereoscopic eyetracker.

Traditional video-based eyetrackers require participants to perform an individual calibration procedure, which involves the fixation of multiple points on a screen. However, certain participants (e.g., people with oculomotor and/or visual problems or infants) are unable to perform this task reliably. Previous work has shown that with two cameras one can estimate the orientation of the eyes' optical axis directly. Consequently, only one calibration point is needed to determine the deviation between an eye's optical and visual axes. We developed a stereo eyetracker with two USB 3.0 cameras and two infrared light sources that can track both eyes at ~ 350 Hz for eccentricities of up to 20°. A user interface allows for online monitoring and threshold adjustments of the pupil and corneal reflections. We validated this tracker by collecting eye movement data from nine healthy participants and compared these data to eye movement records obtained simultaneously with an established eyetracking system (EyeLink 1000 Plus). The results demonstrated that the two-dimensional accuracy of our portable system is better than 1°, allowing for at least ± 5-cm head motion. Its resolution is better than 0.2° (SD), and its sample-to-sample noise is less than 0.05° (RMS). We concluded that our stereo eyetracker is a valid instrument, especially in settings in which individual calibration is challenging.

A meta-analysis of perceptual and cognitive functions involved in useful-field-of-view test performance.

The useful-field-of-view (UFOV) test measures the amount of information someone can extract from a visual scene in one glance. Its scores show relatively strong relationships with everyday activities. The UFOV test consists of three computer tests, suggested to measure processing speed and central vision, divided attention, and selective attention. However, other functions seem to be involved as well. In order to investigate the contribution of these suggested and other perceptual and cognitive functions, we performed a meta-analysis of 116 Pearson's correlation coefficients between UFOV scores and other test scores reported in 18 peer-reviewed articles. We divided these correlations into nine domains: attention, executive functioning, general cognition, memory, spatial ability, visual closure, contrast sensitivity, visual processing speed, and visual acuity. A multivariate mixed-effects model analysis revealed that each domain correlated significantly with each of the UFOV subtest scores. These correlations were stronger for Subtests 2 and 3 than for Subtest 1. Furthermore, some domains were more strongly correlated to the UFOV than others across subtests. We did not find interaction effects between subtest and domain, indicating that none of the UFOV subtests is more selectively sensitive to a particular domain than the others. Thus, none of the three UFOV subtests seem to measure one clear construct. Instead, a range of visual and cognitive functions is involved. Perhaps this is the reason for the UFOV's high ecological validity, as it involves many functions at once, making it harder to compensate if one of them fails.

Predictors of Sensitivity to Perceptual Learning in Children With Infantile Nystagmus.

Purpose: To identify predictors of sensitivity to perceptual learning on a computerized, near-threshold letter discrimination task in children with infantile nystagmus (idiopathic IN: n = 18; oculocutaneous albinism accompanied by IN: n = 18). Methods: Children were divided into two age-, acuity-, and diagnosis-matched training groups: a crowded (n = 18) and an uncrowded training group (n = 18). Training consisted of 10 sessions spread out over 5 weeks (grand total of 3500 trials). Baseline performance, age, diagnosis, training condition, and perceived pleasantness of training (training joy) were entered as linear regression predictors of training-induced changes on a single- and a crowded-letter task. Results: An impressive 57% of the variability in improvements of single-letter visual acuity was explained by age, training condition, and training joy. Being older and training with uncrowded letters were associated with larger single-letter visual acuity improvements. More training joy was associated with a larger gain from the uncrowded training and a smaller gain from the crowded training. Fifty-six percent of the variability in crowded-letter task improvements was explained by baseline performance, age, diagnosis, and training condition. After regressing out the variability induced by training condition, baseline performance, and age, perceptual learning proved more effective for children with idiopathic IN than for children with albinism accompanied by IN. Training gains increased with poorer baseline performance in idiopaths, but not in children with albinism accompanied by IN. Conclusions: Age and baseline performance, but not training joy, are important prognostic factors for the effect of perceptual learning in children with IN. However, their predictive value for achieving improvements in single-letter acuity and crowded letter acuity, respectively, differs between diagnostic subgroups and training condition. These findings may help with personalized treatment of individuals likely to benefit from perceptual learning.

CORRIGENDUM: The expanding clinical phenotype of Bosch-Boonstra-Schaaf optic atrophy syndrome: 20 new cases and possible genotype-phenotype correlations.

This corrects the article DOI: 10.1038/gim.2016.18.

Putative digenic inheritance of heterozygous RP1L1 and C2orf71 null mutations in syndromic retinal dystrophy.

BACKGROUND: Retinitis pigmentosa (RP) is the most common cause of inherited retinal degeneration and can occur in non-syndromic and syndromic forms. Syndromic RP is accompanied by other symptoms such as intellectual disability, hearing loss, or congenital abnormalities. Both forms are known to exhibit complex genetic interactions that can modulate the penetrance and expressivity of the phenotype. MATERIALS AND METHODS: In an individual with atypical RP, hearing loss, ataxia and cerebellar atrophy, whole exome sequencing was performed. The candidate pathogenic variants were tested by developing an in vivo zebrafish model and assaying for retinal and cerebellar integrity. RESULTS: Exome sequencing revealed a complex heterozygous protein-truncating mutation in RP1L1, p.[(Lys111Glnfs*27; Gln2373*)], and a heterozygous nonsense mutation in C2orf71, p.(Ser512*). Mutations in both genes have previously been implicated in autosomal recessive non-syndromic RP, raising the possibility of a digenic model in this family. Functional testing in a zebrafish model for two key phenotypes of the affected person showed that the combinatorial suppression of rp1l1 and c2orf71l induced discrete pathology in terms of reduction of eye size with concomitant loss of rhodopsin in the photoreceptors, and disorganization of the cerebellum. CONCLUSIONS: We propose that the combination of heterozygous loss-of-function mutations in these genes drives syndromic retinal dystrophy, likely through the genetic interaction of at least two loci. Haploinsufficiency at each of these loci is insufficient to induce overt pathology.

Assessment of near visual acuity in 0-13 year olds with normal and low vision: a systematic review.

BACKGROUND: The inclusion for rehabilitation of visually impaired children is partly based on the measurement of near vision, but guidelines for near visual acuity assessment are currently lacking. The twofold purpose of this systematic review was to: (i) provide an overview of the impact of the chart design on near visual acuity measured, and (ii) determine the method of choice for near vision assessments in children of different developmental ages. METHODS: A literature search was conducted by using the following electronic databases: PubMed, Cochrane Library, and EMBASE. The last search was run on March 26th 2016. Additional studies were identified by contacting experts and searching for relevant articles in reference lists of included studies. Search terms were: vision test(s), vision assessment(s), visual acuity, chart(s) and near. RESULTS: For children aged 0-3 years the golden standard is still the preferential looking procedure. Norms are available for this procedure for 6-36 month old children. For 4-7 year olds, we recommend using the LEA symbols, because these symbols have been properly validated and can be used in preliterate children. Responses can be verbal or by matching the target symbol. In children aged 8-13 years, the recommended method is the ETDRS letter chart, because letter acuity is more predictive for functional vision and reading than symbol acuity. In 8-13 year olds, letter acuity is 0.1-0.2 logMAR poorer than symbol acuity. CONCLUSIONS: Chart design, viewing distance, and threshold choice have a serious impact on near visual acuity measurements. Near visual acuity measured with symbols is lower than near visual acuity measured with gratings, and near visual acuity measured with letters is lower than near visual acuity measured with symbols. Viewing distance, chart used, and letter spacing should be adapted to the child's development and reported in order to allow comparisons between measurements.

Perceptual Learning in Children With Infantile Nystagmus: Effects on Visual Performance.

PURPOSE: To evaluate whether computerized training with a crowded or uncrowded letter-discrimination task reduces visual impairment (VI) in 6- to 11-year-old children with infantile nystagmus (IN) who suffer from increased foveal crowding, reduced visual acuity, and reduced stereopsis. METHODS: Thirty-six children with IN were included. Eighteen had idiopathic IN and 18 had oculocutaneous albinism. These children were divided in two training groups matched on age and diagnosis: a crowded training group (n = 18) and an uncrowded training group (n = 18). Training occurred two times per week during 5 weeks (3500 trials per training). Eleven age-matched children with normal vision were included to assess baseline differences in task performance and test-retest learning. Main outcome measures were task-specific performance, distance and near visual acuity (DVA and NVA), intensity and extent of (foveal) crowding at 5 m and 40 cm, and stereopsis. RESULTS: Training resulted in task-specific improvements. Both training groups also showed uncrowded and crowded DVA improvements (0.10 ± 0.02 and 0.11 ± 0.02 logMAR) and improved stereopsis (670 ± 249″). Crowded NVA improved only in the crowded training group (0.15 ± 0.02 logMAR), which was also the only group showing a reduction in near crowding intensity (0.08 ± 0.03 logMAR). Effects were not due to test-retest learning. CONCLUSIONS: Perceptual learning with or without distractors reduces the extent of crowding and improves visual acuity in children with IN. Training with distractors improves near vision more than training with single optotypes. Perceptual learning also transfers to DVA and NVA under uncrowded and crowded conditions and even stereopsis. Learning curves indicated that improvements may be larger after longer training.

Perceptual Learning in Children With Infantile Nystagmus: Effects on 2D Oculomotor Behavior.

PURPOSE: To determine changes in oculomotor behavior after 10 sessions of perceptual learning on a letter discrimination task in children with infantile nystagmus (IN). METHODS: Children with IN (18 children with idiopathic IN and 18 with oculocutaneous albinism accompanied by IN) aged 6 to 11 years were divided into two training groups matched on diagnosis: an uncrowded training group (n = 18) and a crowded training group (n = 18). Target letters always appeared briefly (500 ms) at an eccentric location, forcing subjects to quickly redirect their gaze. Training occurred twice per week for 5 consecutive weeks (3500 trials total). Norm data and test-retest values were collected from children with normal vision (n = 11). Outcome measures were: nystagmus characteristics (amplitude, frequency, intensity, and the expanded nystagmus acuity function); fixation stability (the bivariate contour ellipse area and foveation time); and saccadic eye movements (latencies and accuracy) made during a simple saccade task and a crowded letter-identification task. RESULTS: After training, saccadic responses of children with IN improved on the saccade task (latencies decreased by 14 ± 4 ms and gains increased by 0.03 ± 0.01), but not on the crowded letter task. There were also no training-induced changes in nystagmus characteristics and fixation stability. Although children with normal vision had shorter latencies in the saccade task (47 ± 14 ms at baseline), test-retest changes in their saccade gains and latencies were almost equal to the training effects observed in children with IN. CONCLUSIONS: Our results suggest that the improvement in visual performance after perceptual learning in children with IN is primarily due to improved sensory processing rather than improved two-dimensional oculomotor behavior.

Perceptual Learning in Children With Infantile Nystagmus: Effects on Reading Performance.

PURPOSE: Perceptual learning improves visual acuity and reduces crowding in children with infantile nystagmus (IN). Here, we compare reading performance of 6- to 11-year-old children with IN with normal controls, and evaluate whether perceptual learning improves their reading. METHODS: Children with IN were divided in two training groups: a crowded training group (n = 18; albinism: n = 8; idiopathic IN: n = 10) and an uncrowded training group (n = 17; albinism: n = 9; idiopathic IN: n = 8). Also 11 children with normal vision participated. Outcome measures were: reading acuity (the smallest readable font size), maximum reading speed, critical print size (font size below which reading is suboptimal), and acuity reserve (difference between reading acuity and critical print size). We used multiple regression analyses to test if these reading parameters were related to the children's uncrowded distance acuity and/or crowding scores. RESULTS: Reading acuity and critical print size were 0.65 ± 0.04 and 0.69 ± 0.08 log units larger for children with IN than for children with normal vision. Maximum reading speed and acuity reserve did not differ between these groups. After training, reading acuity improved by 0.12 ± 0.02 logMAR and critical print size improved by 0.11 ± 0.04 logMAR in both IN training groups. The changes in reading acuity, critical print size, and acuity reserve of children with IN were tightly related to changes in their uncrowded distance acuity and the changes in magnitude and extent of crowding. CONCLUSIONS: Our findings are the first to show that visual acuity is not the only factor that restricts reading in children with IN, but that crowding also limits their reading performance. By targeting both of these spatial bottlenecks in children with IN, our perceptual learning paradigms significantly improved their reading acuity and critical print size. This shows that perceptual learning can effectively transfer to reading.