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Purpose: The purpose of this study was to compare changes in phoria adaptation between young adult binocularly normal controls (BNCs) and participants with symptomatic convergence insufficiency (CI), who were randomized to either office-based vergence accommodative therapy (OBVAT) or office-based placebo therapy (OBPT)., Methods: In the double-masked randomized clinical trial, 50 BNC and 50 CI participants were randomized to the following therapeutic interventions: OBVAT or OBPT with home reinforcement for 12 one-hour office sessions. A 6∆ base-out and 6∆ base-in phoria adaptation experiment at near (40 cm) was conducted using the flashed Maddox rod technique at baseline and at outcome. Measurements included the rate and the magnitude of phoria adaptation., Results: At baseline, BNC and CI participants had significantly different rates and magnitudes of base-in and base-out phoria adaptation (P < 0.001). When comparing the outcome to baseline measurements, significant main effect differences in longitudinal measurements were observed for the magnitude and the rate of phoria adaptation for both base-out and base-in experiments (P < 0.05). For the magnitude and rate of phoria adaptation, post hoc analyses using paired t-tests revealed that the CI group administered the OBVAT intervention exhibited a significant increase in the magnitude and rate of phoria adaptation compared to baseline for both base-in and base-out phoria adaptation (P < 0.01) but not for those administered OBPT., Conclusions: Phoria adaptation is significantly different at baseline between those with normal binocular vision and symptomatic CI participants. OBVAT significantly improves the rate and magnitude of both base-out and base-in phoria adaptation at near compared to OBPT. Results have clinical implications for new therapeutic interventions.

Purpose: Convergence insufficiency (CI) is characterized by abnormal vergence eye movement frequently accompanied by abnormal accommodation and subjective symptoms, such as headache, blurred vision, and diplopia. CI is treated with vergence and accommodation exercises that are integrated so that the relative contributions of vergence and accommodation exercises to the outcome are concealed. The purpose of the present study was to determine the individual contributions of vergence and accommodation exercises for the treatment of CI in school children., Methods: In a prospective crossover study 44 children aged 9 to 13 years with CI were randomized to perform either vergence exercises followed by accommodation exercises each for 6 weeks or the 2 treatment regimes in the reverse order. The outcome measures were recovery from CI and the parameters vergence facility, positive fusional vergence, near point of convergence, monocular amplitude, and facility of accommodation., Results: After the first 6-week period, full recovery from CI was significantly more frequent in the group commencing vergence exercises than in the group commencing monocular accommodation exercises (p = 0.01), whereas there was no significant difference between these proportions after the second 6-week period (p = 0.45). Vergence facility and positive fusional vergence improved significantly more after the period with vergence exercises than after the accommodation exercises, whereas there was no significant difference between the effects of the two types of exercises on the other studied parameters., Conclusions: Vergence treatment induces a faster recovery of CI than accommodation treatment in school children. This may be used to improve compliance and success rate of the treatment.

Purpose: We compared the adaptive capacities of reflexive fusional convergence and divergence in 10 participants with untreated convergence insufficiency (CI) to 10 age-matched binocularly normal controls (BNCs) in an effort to elucidate the functional basis of CI., Methods: Vergence responses were monitored binocularly at 250 Hz using video-based infrared oculography, while single and double-step disparity stimuli were viewed dichoptically. The double-step stimuli were designed to induce an adaptive increase in the convergence or divergence reflexive fusional response dynamics., Results: As expected, convergence responses in the CI population were significantly slower at baseline (BNC 12.0 ± 1.8°/s vs. CI 7.4 ± 2.5°/s; P < 0.001), but divergence response velocities were similar between groups (P = 0.38). Critically, we observed an impaired adaptive change in convergence peak velocities in the CI group when compared to BNCs (-18.2% ± 27.3% vs. 25.4% ± 9.8%; P < 0.001). Adaptive changes in reflexive fusional divergence responses were similar between groups (P > 0.5) and significantly less robust when compared to BNC convergence., Conclusions: The results support the hypothesis that the adaptive capacities of vergence are related to the strength of the underlying reflexive fusional response. Combined, the evidence suggests that the clinical condition of convergence insufficiency is underpinned by an underdeveloped or perturbated reflexive fusional vergence response mechanism. We relate these observations to different clinical guidelines for the management and treatment of this condition.

Purpose: Pattern strabismus is characterized by a cross-axis pattern of horizontal and vertical misalignments. In A-pattern strabismus, for example, a divergent change in the horizontal misalignment occurs on downgaze. Work with nonhuman primate models has provided evidence that this disorder is associated with abnormal cross-talk between brainstem pathways that normally encode horizontal and vertical eye position and velocity. Neurons in the interstitial nucleus of Cajal (INC) are normally sensitive to vertical eye position; in the present study, we test the hypothesis that, in monkeys with pattern strabismus, some INC neurons will show an abnormal sensitivity to horizontal eye position., Methods: Monkeys were rewarded for fixating a visual target that stepped to various locations on a tangent screen. Single neurons were recorded from INC in one normal monkey, and two with A-pattern strabismus. Multiple linear regression analysis was used to estimate the preferred direction for each neuron., Results: In the normal monkey, all INC neurons had preferred directions within 20° of pure vertical (either up or down). The preferred directions were significantly more variable in the monkeys with pattern strabismus, with a minority being more sensitive to horizontal eye position than vertical eye position. In addition, the vertical eye position sensitivity was significantly less in the monkeys with strabismus., Conclusions: In pattern strabismus, neurons in INC show neurophysiological abnormalities consistent with a failure to develop normal tuning properties. Results were consistent with the hypothesis that, in pattern strabismus, INC receives an abnormally strong signal related to horizontal eye position.

Purpose: Human children with disorders affecting vergence eye movements have difficulty during close work, such as reading. Patients with convergence insufficiency show a receded near point and an exophoria that is greater at near than at far. Neurologic abnormalities may underlie these symptoms, but it is difficult to test this idea directly because there is no animal model for this disorder. In the present case report, we describe behavioral testing in a rhesus monkey with a naturally occurring impairment of vergence eye movements (monkey CI)., Methods: Three monkeys were trained to perform a variety of oculomotor tasks that required saccades, vergence, and/or smooth tracking of a visual target moving in depth., Results: Two of the monkeys (N1 and N2) were able to perform these tasks correctly. The third, monkey CI, was able to correctly perform these tasks when the required vergence angle was ≤5° but had difficulty when the task required larger convergence. This animal showed a consistent exodeviation that worsened as the target drew closer. When a variable prism was used to test disparity vergence in monkey CI, the animal showed an unstable convergence response (maximum 6°) that increased with prism correction, up to 12 prism diopters. By comparison, monkey N1 was able to achieve stable, appropriate convergence up to 26 prism diopters. Monkey CI's performance on vergence tasks improved when a large-field random checkerboard pattern was used to provide additional depth cues., Conclusions: Monkey CI appears to have a naturally occurring disorder of vergence eye movements.

Purpose: Convergence insufficiency is a very common disorder that can have significant adverse effects on school performance. When reading, children with this disorder often experience diplopia and headaches. We have recently obtained a rhesus monkey with a naturally occurring impairment of vergence eye movements. In the companion paper, we report behavioral testing that shows a pattern of impairments similar to what clinicians observe in human children with convergence insufficiency, including a receded near point, an exophoria that increases as target distance decreases, and difficulty maintaining an appropriate vergence angle when presented with a large field stimulus at near. For the present case report, we wondered whether these behavioral deficits would be associated with abnormal discharge patterns in brainstem neurons related to vergence eye movements., Methods: Single unit activity was recorded from near and far response cells in the supraoculomotor area in the vergence-impaired monkey, while he performed a smooth vergence tracking task or fixated visual targets at different distances., Results: We found an abnormally weak sensitivity to both vergence angle and vergence velocity. Nonetheless, these neurons modulated in association with contextually inappropriate slow vergence movements that occurred in the absence of saccades but not for slow divergence drifts that immediately followed converging saccades. Modulation of activity was more robust when additional depth cues were available., Conclusions: These data suggest that disorders affecting vergence eye movements may be associated with impoverished sensory input to the near and far response cells and, perhaps, aberrant tuning in vergence-related neurons.

Purpose: Microsaccades shift the image on the fovea and counteract visual fading. They are also thought to serve as an optimal sampling strategy while viewing complex visual scenes. The goal of our study was to assess visual search in amblyopic children., Methods: Twenty-one amblyopic children with varying severity of amblyopia and 10 healthy controls were recruited. Eye movements were recorded using infrared video-oculography during amblyopic and fellow eye viewing while the subjects performed (1) visual fixation, (2) exploration of a blank scene, and (3) visual search task (spot the difference between two images). The number of correctly identified picture differences and reaction time were recorded. Microsaccade, saccades, and intersaccadic drifts were analyzed in patients without latent nystagmus (LN). Slow phase velocities were computed for patients with LN., Results: Both patients with and without LN were able to spot the same number of differences but took longer during fellow eye viewing compared to controls. The ability to identify differences was diminished during amblyopic eye viewing particularly those with LN and severe amblyopia. We found reduced frequencies of microsaccades and saccades in both amblyopic and fellow eyes during fixation and visual search but not during exploration of blank scene. Across all tasks, amblyopes with LN had increased intersaccadic drifts., Conclusions: Our findings suggest that deficient microsaccade and saccadic activity contributes to poorer sampling strategy in amblyopia, which is seen in both amblyopic and fellow eye. These deficits are more notable among subjects who experienced binocular decorrelation earlier in life, with subsequent development of LN.

Purpose: Precise measurements of visual fixation and its instability were recorded during optical coherence tomography (OCT) as a marker of neural network dysfunction in multiple sclerosis (MS), which could be used to monitor disease progression or response to treatment., Methods: A total of 16 MS patients and 26 normal subjects underwent 30 seconds of scanning laser ophthalmoscope (SLO)-based eye tracking during OCT scanning of retinal layer thickness. Study groups consisted of normal eyes, MS eyes without prior optic neuritis (MS wo ON), and MS eyes with prior optic neuritis (MS + ON). Kernel density estimation quantified fixation instability from the distribution of fixation points on the retina. In MS wo ON eyes, fixation instability was compared to other measures of visual and neurologic function., Results: Fixation instability was increased in MS wo ON eyes (0.062 deg2) compared to normal eyes (0.030 deg2, P = 0.015). A further increase was seen for MS + ON eyes (0.11 deg2) compared to MS wo ON (P = 0.04) and normal (P = 0.006) eyes. Fixation instability correlated weakly with ganglion cell layer (GCL) volume and showed no correlation with low-contrast letter acuity, EDSS score, or SDMT score., Conclusions: Fixation instability reflects the integrity of a widespread neural network germane to visual processing and ocular motor control, and is disturbed in MS. Further study of visual fixation, including the contribution of microsaccades to fixation instability, may provide insight into the localization of fixation abnormalities in MS and introduce innovative and easily measured outcomes for monitoring progression and treatment response.

Purpose: To investigate the relationship between objective ocular torsion and the presence or absence of the trochlear nerve in subjects with unilateral superior oblique palsy (SOP)., Methods: A total of 159 subjects with congenital and acquired unilateral SOP were reviewed. Eighty-four subjects who had a normal trochlear nerve (present group) and 75 subjects without a trochlear nerve (absent group) were included. Cyclovertical motility parameters and objective ocular torsion were compared between groups, and factors related to ocular torsion were evaluated., Results: The degree of "net" excyclotorsion in the paretic eye was larger in the absent group compared to the present group (P = 0.002). The proportion of net excyclotorsion in the paretic eye was greater in the absent group (11% vs. 37%), while net incyclotorsion was greater in the present group (41% vs. 23%) (P < 0.001). Net excyclotorsion of the paretic eye was associated with absence of the trochlear nerve (P < 0.001) and smaller size of the paretic SO (P < 0.001). Net incyclotorsion of the paretic eye was related with a normal trochlear nerve (P = 0.005), larger size of the paretic SO (P = 0.002), and greater hypertropia during ipsilateral gaze (P = 0.024)., Conclusions: The status of the trochlear nerve, paretic SO size, and hypertropia during ipsilateral gaze which reflects the tensile strength of the ipsilateral superior rectus, significantly contribute to ocular torsion in unilateral SOP.

Purpose: Spinocerebellar ataxias type 2 and 28 (SCA2, SCA28) are autosomal dominant disorders characterized by progressive cerebellar and oculomotor abnormalities. We aimed to investigate cerebellar, brainstem, and extraocular muscle involvement in the mitochondrial SCA28 disease compared with SCA2., Methods: We obtained orbital and brain 1.5 T-magnetic resonance images (MRI) in eight SCA28 subjects, nine SCA2, and nine age-matched healthy subjects. Automated segmentation of cerebellum and frontal lobe was performed using Freesurfer software. Manual segmentations for midbrain, pons, and extraocular muscles were performed using OsiriX., Results: Eye movement abnormalities in SCA2 subjects were characterized by slow horizontal saccades. Subjects with SCA28 variably presented hypometric saccades, saccadic horizontal pursuit, impaired horizontal gaze holding, and superior eyelid ptosis. Quantitative brain MRI demonstrated that cerebellar and pons volumes were significantly reduced in both SCA2 and SCA28 subjects compared with controls (P < 0.03), and in SCA2 subjects compared with SCA28 (P < 0.01). Midbrain and frontal lobe volumes were also significantly reduced in SCA2 compared to controls (P < 0.03), whereas these volumes did not differ between SCA2 and SCA28 and between SCA28 and control subjects. The extraocular muscle areas were 37% to 48% smaller in SCA28 subjects compared with controls (P < 0.002), and 14% to 36% smaller compared with SCA2 subjects (P < 0.03). Extraocular muscle areas did not differ between SCA2 and controls., Conclusions: Our MRI findings support the hypothesis of different cerebellar and extraocular myopathic contributions in the eye movement abnormalities in SCA2 and SCA28 diseases. In SCA28, a myopathic defect selectively involving the extraocular muscles supports a specific impairment of mitochondrial energy metabolism.

Purpose: This study employed magnetic resonance imaging (MRI) to investigate possible size and contractility changes in the superior oblique (SO) muscle, and possible isometric hypertrophy in the inferior oblique (IO) muscle, resulting from abnormal mechanical loading in Brown syndrome (BrS)., Methods: High resolution orbital MRI was obtained in 4 congenital and 11 acquired cases of BrS, and compared with 44 normal subjects. Maximal cross-section areas and posterior partial volumes (PPVs) of the SO were analyzed in central gaze, supraduction, and infraduction [corrected] for the SO, and in central gaze only for the IO., Results: In congenital BrS, mean maximum SO cross-sectional areas were 24% and 20% less than normal in affected and unaffected eyes, respectively (P = 0.0002). Mean PPV in congenital BrS was also significantly subnormal bilaterally (29% and 34% less in affected and unaffected eyes, respectively, P = 0.001). However, SO muscle size and volume were normal in acquired cases. The SO muscle did not relax in supraduction in BrS, although there was normal contractile thickening in infraduction. The IO muscle had normal size bilaterally in BrS., Conclusions: Congenital BrS may be associated with SO hypoplasia that could reflect hypoinnervation. However, unique isometric loading of oblique extraocular muscles due to restrictive hypotropia in adduction in BrS is generally not associated with changes in muscle bulk or in SO contractility. Unlike skeletal muscles, the bulk and contractility of extraocular muscles can therefore be regarded as independent of isometric exercise history. Restriction to elevation in BrS typically arises in the trochlea-tendon complex.

Purpose: To determine the mechanisms of vertical fusional vergence in patients with "congenital unilateral superior oblique paresis" (SOP) and to discuss the implications of these mechanisms., Methods: Eleven patients were examined with our eye-tracking haploscope., Results: Three different fusion mechanisms were found, producing significantly different cyclovergence to vertical vergence ratios (P < 0.05): primary use of the vertical rectus muscles in seven patients (ratio: 0.36 ± 1.6), primary use of the oblique muscles in one patient (0.04), and use of the superior oblique muscle in the higher eye and the superior rectus muscle in the lower eye in three patients (1.15 ± 0.32). Lancaster red-green testing showed alignment differences among these groups, primarily differences in amount of subjective extorsion between the two eyes in straight-ahead gaze: The patient with oblique-muscle-mediated fusion showed essentially no subjective extorsion (0.5°), the patients with vertical-rectus-muscle-mediated vertical fusion showed a mean ± SD subjective extorsion of 3.6° ± 1.4°, and the patients with the mixed (oblique/rectus) fusion mechanism showed 7.0° ± 1.7° (P < 0.05)., Conclusions: The choice of fusion mechanism may be a function of how much intorting effect is needed. Use of the oblique muscles bilaterally causes the least intorting effect, use of the vertical rectus muscles bilaterally adds more intorting effect, and activation of the "paretic" superior oblique muscle in the higher eye and the superior rectus muscle in the lower eye provides the greatest intorting effect. Subclassifying "congenital SOP" in this way (in which the "paretic" muscle may remain functional in many cases) may help guide its optimal surgical correction.

Purpose: To determine the prevalence of binocular vision (BV) and eye movement disorders in a clinic population of older adults., Methods: Retrospective clinic data were abstracted from files of 500 older patients seen at the University of Waterloo Optometry Clinic over a 1-year period. Stratified sampling gave equal numbers of patients in the 60 to 69, 70 to 79, and 80+ age groups. Data included age, general and ocular history and symptoms, use of antidepressants, a habit of smoking, refraction, visual acuity, BV and eye movement status for the most recent full oculo-visual assessment, and an assessment 10 years prior. The prevalence of any BV or eye movement abnormal test (AT) result, defined as a test result outside the normal range, was determined. This included strabismus (any) or phoria; incomitancy; poor pursuits; and remote near point of convergence (NPC). The prevalence of significant BV disorders (diagnostic entities, i.e., a clinical condition that may need treatment and may have functional implications) was also determined., Results: The prevalence of any BV or eye movement at was 41%, 44%, and 51% in the 60 to 69, 70 to 79, and 80+ age groups, respectively. These figures were lower for 10 years earlier: 31%, 36%, and 40% for ages 50 to 59, 60 to 69, and 70+, respectively. The prevalence of any BV or eye movement disorder was 27%, 30%, and 38% for the three age groups and 17%, 19%, and 24% for 10 years prior. Age and use of antidepressants most commonly predicted BV or eye movement AT or disorder., Conclusions: BV disorders are common among older adults.

Purpose: The purpose of the current research is to understand if the different eye movement abnormalities in patients with the same neurologic disease are related to varied disease processes or, alternately, do different patients adopt different strategies to overcome a singular brain deficiency., Methods: Using a magnetic search coil, we measured saccade dynamics, that is position and velocity waveforms, for patients diagnosed with spinocerebellar ataxia type 3 (SCA-3), also known as Machado-Joseph disease (MJD)., Results: We observed that the saccadic waveform of the majority of the SCA-3 patients (7 of 10) exhibited dynamic overshoot, with the eye passing the desired endpoint and making a rapid correction before coming to rest. Patients with normal waveforms, that is with no dynamic overshoot, had saccades with relatively low peak velocity., Conclusions: Velocity feedback in a closed loop control system is essential for providing a fast response without overshoot. Lack of a velocity feedback or an imbalance between position and velocity gains yields a tradeoff between response time and overshoot. While the goal of a saccade is to get to the desired position, models based on animal research suggest that the saccadic control also incorporates a velocity feedback. Results presented here indicated that all SCA-3 patients had deviations in the saccadic waveform, albeit of two types, either slow saccades or dynamic overshooting saccades. Using saccadic models based on animal research can explain how a single deficit, that is a mismatched velocity control of the motor error due to the disease, can yield these two different abnormalities in human patients.

Purpose: To assess the effect on visual acuity of compensating fixation instability by controlling retinal image motion in people with macular disease., Methods: Ten patients with macular disease participated in this study. Crowded and noncrowded visual acuity were measured using an eye tracking system to compensate for fixation instability. Four conditions, corresponding to four levels of retinal image motion, were tested: no compensation (normal motion), partial compensation (reduced motion), total compensation (no motion), and overcompensation (increased motion). Fixation stability and the number of preferred retinal loci were also measured., Results: Modulating retinal image motion had the same effect on crowded and noncrowded visual acuity (P = 0.601). When fixation instability was overcompensated, acuity worsened by 0.1 logMAR units (P < 0.001) compared with baseline (no compensation) and remained equal to baseline for all other conditions., Conclusions: In people with macular disease, retinal image motion caused by fixation instability does not reduce either crowded or noncrowded visual acuity. Acuity declines when fixation instability is overcompensated, showing limited tolerance to increased retinal image motion. The results provide evidence that fixation instability does not improve visual acuity and may be a consequence of poor oculomotor control.

Purpose: Joubert syndrome is a genetic disorder characterized by hypoplasia of the midline cerebellum and deficiency of crossed connections between neural structures in the brain stem that control eye movements. The goal of the study was to quantify the eye movement abnormalities that occur in Joubert syndrome., Methods: Eye movements were recorded in response to stationary stimuli and stimuli designed to elicit smooth pursuit, saccades, optokinetic nystagmus (OKN), vestibulo-ocular reflex (VOR), and vergence using video-oculography or Skalar search coils in 8 patients with Joubert syndrome. All patients underwent high-resolution magnetic resonance imaging (MRI)., Results: All patients had the highly characteristic molar tooth sign on brain MRI. Six patients had conjugate pendular (n = 4) or see-saw nystagmus (n = 2); gaze holding was stable in four patients. Smooth-pursuit gains were 0.28 to 1.19, 0.11 to 0.68, and 0.33 to 0.73 at peak stimulus velocities of 10, 20, and 30 deg/s in six patients; smooth pursuit could not be elicited in four patients. Saccade gains in five patients ranged from 0.35 to 0.91 and velocities ranged from 60.9 to 259.5 deg/s. Targeted saccades could not be elicited in five patients. Horizontal OKN gain was uniformly reduced across gratings drifted at velocities of 15, 30, and 45 deg/s. VOR gain was 0.8 or higher and phase appropriate in three of seven subjects; VOR gain was 0.3 or less and phase was indeterminate in four subjects., Conclusions: The abnormalities in gaze-holding and eye movements are consistent with the distributed abnormalities of midline cerebellum and brain stem regions associated with Joubert syndrome.

Purpose: An earlier study suggested that deficits of vergence can influence postural control via the efferent and afferent proprioceptive signals. In this study, postural control in 28 children with vertigo with normal vestibular function but with vergence abnormalities and in 19 normal children of comparable age was assessed with orthoptic tests., Methods: A posturography platform was used to examine posture in quiet stance. The child was asked to fixate a target at 40 cm or at 200 cm, either with eyes open (vision condition) or with eyes covered by a black mask (no vision condition). In a complementary test in 15 of 28 children with vertigo and in all 19 normal children, postural control was evaluated during monocular viewing (dominant and nondominant eye viewing)., Results: For all children examined, postural stability was better when fixating a target at near than at far distance and with both eyes than with one eye or with eyes covered. In all conditions, the children with vertigo were more unstable than were the control children., Conclusions: Binocular visual information, such as vergence disparity, is essential in stabilizing posture at near distance. Postural instability reported in children with vertigo and vergence abnormalities could be due to poor vergence inputs and/or to immature compensatory mechanisms controlling postural stability (vestibular, somatosensory inputs and/or cerebellar processes).

Purpose: Photosensitivity, or photoparoxysmal response (PPR), is an abnormal EEG reaction to intermittent photic stimulation (IPS), consisting of spikes, spike-waves, and intermittent slow waves. Depending on the spread of the abnormal activity, PPR subgroups have been defined as having either propagating PPR or localized, occipital spikes (OS) only. Recent research suggests an enhanced excitability in the occipital cortex may underlie this reaction during IPS, but it remains unclear whether changes in excitability affect the function of the occipital cortex to other, less provocative visual stimuli. In this study, cortical function in photosensitivity was assessed using two visual aftereffects that occur after prolonged adaptation., Methods: Motion and tilt aftereffects were compared in healthy subjects with (n = 14, seven with propagating PPR, seven with OS) or without (n = 14) PPR., Results: The duration of the motion aftereffect was shorter in the PPR group than in the control group. The size of the tilt aftereffect did not differ between the groups. Thirteen from each group had participated in an earlier study in which occipital transcranial magnetic stimulation (TMS) was used to elicit phosphenes and to suppress the perception of briefly presented letters. The TMS intensity required to elicit phosphenes correlated with the size of the tilt aftereffect (TAE) in the PPR group only., Conclusions: This study provides further evidence of enhanced cortical excitability in subjects with photosensitivity, which is likely to reflect changes in excitatory neurotransmission.

Purpose: The linear vestibulo-ocular reflex (LVOR) is mediated primarily by the otolith organs in the inner ear. Skew deviation is a vertical strabismus believed to be caused by imbalance of otolithic projections to ocular motor neurons (disynaptically through the medial longitudinal fasciculus in the brain stem or polysynaptically through the cerebellum). The authors postulated that if skew deviation is indeed caused by damage to these projections, patients with skew deviation would show abnormal LVOR responses., Methods: Six patients with skew deviation caused by brain stem or cerebellar lesions and 10 healthy subjects were recruited. All subjects underwent brief, sudden, interaural translations of the head (head heaves) using a head-sled device at an average peak acceleration of 0.42g (range, 0.1-1.1g) while continuously viewing an earth-fixed target monocularly at 15 and 20 cm. LVOR sensitivity (peak rotational eye velocity to peak linear head velocity) and velocity gain (peak actual-to-ideal rotational eye velocities) were calculated for the responses within the first 100 ms after onset of head movements., Results: LVOR sensitivities and velocity gains in patients were decreased by 56% to 62% in both eyes compared with healthy subjects. This binocular reduction in LVOR responses was asymmetric--the magnitude of reduction differed between eyes by 37% to 143% for sensitivities and by 36% to 94% for velocity gains. There were no differences in response between right and left heaves., Conclusions: The binocular, asymmetric reduction in LVOR sensitivity and velocity gain provides support that imbalance in the otolith-ocular pathway is a mechanism of skew deviation.

Purpose: Dyslexia is a language-based learning disability characterized by difficulties with reading, spelling, and writing. Persons with dyslexia often have deficits in processing rapid temporal sensory information. There is also evidence of sensorimotor deficits in persons with dyslexia. Whether these deficits include ocular motor problems is still an open question. Some previous studies have shown an increased saccadic latency in dyslexics, whereas others have not reproduced this finding. The purpose of the present study was to investigate saccadic latency in young adults with dyslexia during the double-step paradigm, a task that requires rapid sequential visual information processing and saccade generation. The study hypothesis was that dyslexics have a longer saccadic latency in the second orthogonal saccade, a task that nondyslexics parallel process and perform rapidly., Methods: Eight students with dyslexia and eight age-matched control subjects participated in the study. Their eye movements were monitored with the scleral search coil technique in simple saccade trials and in the double-step paradigm. The second saccade was either orthogonal or colinear to the first. Intersaccadic interval and latency were calculated for the second saccade., Results: No difference in saccadic latency was found for colinear second saccades; however, dyslexics had significantly longer latencies for orthogonal second saccades. This included a subset of subjects who had longer latencies for orthogonal than for colinear saccades., Conclusions: The findings indicate that under certain conditions, when the demand for rapid visual information processing is high and a rapid saccade sequence is required, some persons with dyslexia show ocular motor deficits manifested by longer saccadic latencies.

Purpose: To evaluate the effect of orthoptic treatment on the AC/A (A, accommodation; C, convergence) and CA/C ratios in subjects with convergence insufficiency (CI)., Methods: The change in AC/A and CA/C ratios after a 12-week period of home-based orthoptic treatment was examined in 10 subjects (mean age, 25.4+/-4.1 years [SD]). Both the AC/A and CA/C ratios were measured by using gradient response methods. For the AC/A ratio, the gradient phoria method was used, and for the CA/C ratio the prism-induced change in accommodation was measured with a refractometer., Results: No change in the AC/A and CA/C ratios (P>0.05) were found after orthoptic treatment. However, improvements were found (P<0.05) in the fast and slow vergence mechanisms., Conclusions: Despite improvements in the fast and slow vergence mechanisms no change was found in the AC/A and CA/C ratios after orthoptic treatment in CI subjects. This finding is unexpected in light of the present understanding of CI, and an alternative theory is proposed.

Purpose: To describe the eye movement abnormalities in patients with chorea-acanthocytosis (ChAc), a form of neuroacanthocytosis. This autosomal recessive, neurodegenerative disorder with aberrant erythrocyte morphology (acanthocytosis) is caused by mutations in the VPS13A gene. In contrast to Huntington's disease (for which ChAc has occasionally been mistaken), ocular involvement in ChAc has not been systematically studied., Methods: Three patients aged 26, 30, and 44 years with ChAc and six normal volunteers aged 31 to 48 years were included. Patients had dystonia, chorea, parkinsonism, dysarthria, dysphagia, seizures, cognitive abnormalities, and acanthocytosis. All had heterozygous VPS13A mutations and degeneration of the basal ganglia on magnetic resonance imaging (MRI) typical of ChAc. Patients had an extensive clinical and laboratory work-up. Neuro-ophthalmic examinations and eye movement recordings made with the magnetic search coil technique assessed patients' fixation characteristics, horizontal and vertical saccades, pursuit, and antisaccades. These were compared to the data of control subjects., Results: Patients exhibited more than 30 square-wave jerks (small saccadic intrusions) per minute, versus 0 to 8 in the control subjects, as well as fractionated (multistep) and hypometric horizontal and vertical saccades. Decreased saccadic peak-velocity and reduced saccadic range were more pronounced for vertical saccades. Pursuit testing performed in two patients showed low gain. Results of antisaccade testing done in one patient were abnormal., Conclusions: The findings suggest brain stem involvement as an additional site of neurodegeneration outside the basal ganglia in ChAc. Patients with this progressive, intractable disease have pronounced ocular motor abnormalities. Eye movement recordings could assist in diagnosing ChAc, monitoring its progression and possible treatment evaluation.

Purpose: To describe symmetric convergence eye movements evoked by disparity and/or accommodative cues in esotropic macaque monkeys, with the goal of determining whether these animals have the vergence deficits found in humans with esotropia., Methods: Physical far and near targets were used to evoke large (approximately 8 degrees) symmetric convergence eye movements in four adult macaque monkeys (two with strabismus, two normal), using positive-feedback rewards. One strabismic monkey had infantile-onset, small-angle esotropia (small-eso approximately 2 degrees) induced by alternating occlusion from birth to age 9 months. The other strabismic monkey had naturally occurring, large-angle (approximately 25 degrees) infantile-onset esotropia (large-eso). Visual acuity was normal in each eye as measured by spatial sweep visually evoked potentials (VEPs). Eye movements were recorded using magnetic search coils., Results: When viewing binocularly, both normal monkeys exhibited accurate, stereotyped symmetric convergence movements that achieved 87% to 96% of the required change in vergence angle by the end of the initial movement. In contrast, the small-eso monkey's convergence response when viewing binocularly was variable, strikingly asymmetric, usually accompanied by a disjunctive saccade, and subnormal, achieving only 56% of required vergence. The convergence response of the large-eso monkey was also asymmetric and weak, achieving 18% of the required vergence and employing conjugate saccades to refixate the near target. Monocular viewing (i.e., accommodative vergence) caused substantial reductions in both convergence amplitudes and velocities in the normal monkeys, but had a minor effect on the vergence behavior of the strabismic animals., Conclusions: Monkeys with small- and large-angle infantile esotropia have striking maldevelopments of binocular (disparity-driven) convergence and use accommodative vergence and saccades to refixate near targets. Their vergence behavior resembles that in esotropic humans. The maldevelopment may be explained in large part by the paucity of binocular connections recently described in the visual cortex of esotropic macaques.

Purpose: To study possible causes of motility disturbances that may result from orbital decompression surgery in patients with Graves orbitopathy and especially the role of rectus extraocular muscle paths., Methods: Sixteen patients with Graves orbitopathy were studied before and 3 to 6 months after translid (6 patients) and coronal (10 patients) orbital decompression surgery for disfiguring proptosis. Ocular motility changes were measured by comparing maximum ductions and severity of diplopia, and the positions and the displacements of the anterior rectus muscle paths were objectively measured using cine magnetic resonance imaging (MRI)., Results: Averaged preoperative rectus muscle path positions were not different from those in normal subjects. Averaged postoperative muscle path positions were generally the same as preoperative paths. The only significant exceptions were centrifugal (outward from the orbital axis) displacements of the inferior rectus (IR) muscle path after translid surgery, and of the medial rectus (MR) muscle path after coronal surgery. The amount of IR path displacement with translid surgery was directly correlated with range of depression and with severity of vertical diplopia. The amount of MR path displacement with coronal surgery was inversely correlated with range of abduction and directly correlated with severity of horizontal diplopia., Conclusions: The anterior orbital connective tissue seems to form a "functional skeleton" that is usually (except as noted for IR and MR) capable of keeping the rectus muscle paths aligned after decompression surgery and preserving the normal functions of rectus muscle pulleys. The centrifugal displacement of the IR and MR may increase the elastic component of the muscle force, leading to the specific patterns of motility disturbance that may occur in some patients after translid and coronal surgery. These findings suggest that standard surgical management of Graves orbitopathy should be supplemented.

Purpose: To describe the saccades made by patients with Graves' disease (GD) and to attempt to distinguish these objectively and quantitatively from the saccades in control subjects., Methods: In 12 euthyroid patients with GD, the saccades of both eyes were recorded simultaneously with electromagnetic search coils. Subjects were asked to alternate their gazes between two fixed targets that were 10 degrees, 20 degrees, 30 degrees, or 40 degrees apart along each horizontal or vertical meridian. The data from the patients with GD and those from the similarly recorded group of 12 control subjects were examined in two ways. First, the difference in saccadic sizes between the two eyes was assessed. Second, the saccadic dynamics--that is, the maximum velocity and the saturation constant of the main sequence--were determined for each eye. Repeated measurement analysis of variance was used to test observed differences between the two groups. Finally, through exact logistic regression analysis, classification of the saccades as those of a patient with GD or of a control subject was carried out., Results: The saccades of patients with GD were generally less conjugate than those of control subjects (P < 0.05). On average, the maximum main sequence velocities in patients with GD were lower than in control subjects (P < 0.05). The saccades of patients with GD were well differentiated from those of control subjects., Conclusions: The saccades in GD may differ markedly from normal saccades, and the two can be reliably distinguished.

Visual evoked potentials (VEPs) were recorded under binocular and monocular viewing conditions in 19 2- to 10-month-old infants, 19 stereodeficient adults, and 12 normal adults. VEPs were elicited by medium contrast, phase-alternated checkerboards with check sizes ranging from 10-52 min of arc. Binocular VEP summation was defined as the percentage by which the binocular VEP amplitude exceeded the mean of the two monocular VEP amplitudes. In the stereonormal adults, binocular VEP summation was significantly greater than zero and significantly less than 100%. In the stereodeficient adults, binocular VEP summation was not significantly greater than zero. These findings support the hypothesis that the magnitude of binocular VEP summation reflects the activation of binocular cortical neurons. However, binocular VEP summation in the infants averaged approximately 95%. Moreover, infants under 5 months of age, many younger than the reported age of onset for stereopsis, showed binocular VEP summation of nearly 145%. The significantly higher level of binocular VEP summation in the infants was the result of much larger binocular VEP amplitudes, while monocular VEP amplitudes were equivalent to those of stereonormal and stereodeficient adults. These results support the hypothesis that VEP amplitude is mediated by two independent pools of monocular cortical neurons and that binocular VEP amplitude in stereonormal and stereodeficient adults saturates at a lower level than in infants. Thus, it is hypothesized that binocular VEP summation is not representative of the activation of binocular cortical neurons.