Values of electroretinogram responses according to axial length.

Accurate interpretation of electroretinograms (ERGs) requires knowledge of effects of axial myopia on ERG responses. Our purpose was to derive expected changes of ERG responses according to axial length, to stimulus conditions that conform to the International Society for Clinical Electrophysiology of Vision (ISCEV) Standard for Electroretinography. ERGs from 60 subjects were recorded. The subjects were assigned to one of three groups according to the level of myopia. Thirty-three subjects had high myopia (-6.00 D to -14.50 D; mean age, 31 years), eight had mild myopia (-3.00 D to -5.00; mean age, 28 years), and 19 had a small refractive error (+0.75 D to -2.75 D; mean age, 27 years). No subjects had myopic retinopathy. Stimulus-response curves were fitted to dark-adapted b-wave amplitudes and maximum amplitude and semi-saturation constants derived. Axial lengths, measured with A scan ultrasound, ranged from 22.2 mm to 30.0 mm. Analysis of variance and post hoc t-tests revealed significant difference between subjects with high myopia and subjects with small refractive error for ERG amplitude data. There were no significant differences between the three groups for implicit times, the ratio of b- to a-wave and semi-saturation constant. There is linear reduction in the logarithmic transform of ERG amplitude with increasing axial length, related more to axial length than refractive error. We provide relative slope and intercept values, allowing labs to derive expected ERG amplitudes according to axial length. These derivations are valid for persons with no retinopathy.

Electrophysiological evidence of cortical fusion in children with early-onset esotropia.

PURPOSE: To investigate sensory fusion responses in infants and children with early-onset esotropia to gain insights into the sequence of events that leads to strabismus. METHODS: Sensory fusion was tested by measuring visual evoked potential (VEP) responses to dynamic random dot correlograms (DRDCs) in a group of children (n = 23) with early-onset esotropia. Thirteen children were tested before surgical alignment, and 13 children were tested after surgical alignment (three children were tested before and after surgery). If the angle of strabismus was larger than 5 prism diopters, it was corrected with Fresnel prisms (Fresnel Prism and Lens, Scottsdale, AZ). RESULTS: Five (38%) of the 13 children who were tested before surgery showed detectable VEP responses to correlogram stimuli compared with 11 (85%) of the 13 children who were tested after surgical alignment. There were no significant statistical differences between VEP responses to DRDCs from the postsurgery group and VEP responses from an age-matched control group with normal binocular vision. CONCLUSIONS: The presence of cortical sensory fusion in children with early-onset esotropia suggests that a congenital defect of sensory fusion cannot be the root cause of esotropia in most children. The data suggest that sensory fusion, when measured by VEP responses to DRDCs, is more robust than stereopsis to abnormal binocular experience and support the notion that pathways processing correlated/anticorrelated stimuli may not completely overlap with pathways processing disparity information.

Cortical binocularity and monocular optokinetic asymmetry in early-onset esotropia.

PURPOSE: To investigate the correlation between directional asymmetry in ocular responses to monocularly viewed optokinetic stimuli (monocular optokinetic nystagmus, MOKN) and sensory fusion in infants and toddlers with early-onset esotropia. METHODS: Subjects were 14 infants and toddlers with early-onset esotropia (7-26 months old; median, 10 months), and 16 with no esotropia (6-22 months; median, 11 months) who provided control data. Monocular optokinetic nystagmus in response to a 30 degrees/sec square-wave grating (0.25 cycles/degree) was measured by electro-oculogram. Sensory fusion was assessed with visual evoked potentials (VEPs) to random-dot correlograms after correction of the strabismus angle with Fresnel prisms. RESULTS: All subjects with early-onset esotropia had MOKN with a faster slow-phase component for temporal-to-nasalward (TN) than nasal-to-temporalward (NT) motion. Ninety-three percent of subjects had MOKN asymmetry higher than the 95th percentile of the control group. Of subjects who cooperated with VEP fusion testing, 5 subjects with early-onset esotropia (45%) and 11 control subjects (92%) showed evidence of sensory fusion. CONCLUSIONS: Symmetrical MOKN did not develop in infants and toddlers with early-onset esotropia. This deficit existed in most infants who showed sensory- cortical fusion. These results are consistent with the belief that optokinetic nystagmus asymmetry may not be associated with a deficit in the cortical fusion facility, but rather with deficits in binocular pathways projecting to MOKN control centers. These deficits may be associated with abnormal processing subsequent to sensory fusion or with abnormal processing in motion pathways, which run parallel to sensory fusion pathways.

Time courses for maturation of electroretinogram responses from infancy to adulthood.

The purpose of this study was to determine how responses in the normal human electroretinogram (ERG) change with subject age. We studied 62 children, 10 days to 15 years old, and 30 subjects 15-37 years old, using the standard protocol established by the International Society for Clinical Electrophysiology of Vision, with Burian-Allen bipolar contact-lens electrodes. We measured rod response, maximal response, oscillatory potentials (OPs), cone response, flicker response, and b-wave amplitude/log intensity (V/log I) curve. A logistic growth curve was used to describe the developmental changes. Dark- and light-adapted ERG a- and b-wave amplitudes reached adult levels by three to five years of age. although b-wave amplitudes of scotopic rod-mediated responses were slower to reach maturity than mixed rod-cone mediated responses. In early infancy OPs were the most immature of the ERG responses, although the rate of development thereafter exceeded that of the other responses such that OP amplitudes were within adult levels by two years of age. Amplitudes of the ERG responses in 21 children sedated with chloral hydrate did not differ significantly from 21 who had not been sedated. ERG responses developed at varying rates, reflecting different developmental stages in photoreceptors, middle retinal layers and more proximal retina.

Characterization of the ocular phenotype of Duchenne and Becker muscular dystrophy.

PURPOSE: Dystrophin, the Duchenne muscular dystrophy gene product, has been localized to the outer plexiform layer of normal human retina. The purpose of this study is to define completely the ocular phenotype associated with mutations at Xp21, the Duchenne muscular dystrophy gene locus. METHODS: Twenty-one patients with a diagnosis of Duchenne muscular dystrophy and five patients with Becker muscular dystrophy had ophthalmologic examinations, including electroretinograms (ERGs). Electroretinogram results were correlated with respect to patient DNA analysis. RESULTS: Twenty-three (88%) patients had reduced scotopic b-wave amplitudes to bright-white flash stimulus, including nine with negative-shaped ERGs. Rod-isolated responses were reduced or not recordable above noise in 14 (67%) patients. Most isolated cone responses (92%) were normal. Flicker amplitudes were reduced in seven patients. Two of these patients with proximal (5' end) deletions had normal scotopic b-waves to dim blue and bright-white flash stimulus. Patients with deletions toward the middle of the gene had greater reductions in their scotopic b-wave amplitudes than patients with deletions located toward the 5' end. Most patients had normal color vision, extraocular muscle function, and Snellen visual acuity. Increased macular pigmentation was seen in 16 patients with Duchenne muscular dystrophy. CONCLUSION: Most patients with Duchenne or Becker muscular dystrophy have evidence of abnormal scotopic ERGs. Patients with deletions in the central region of the gene had the most severe ERG changes. This study supports previous suggestions that dystrophin may play a role in retinal neurotransmission. The presence of increased macular pigmentation and normal photopic ERGs distinguishes patients with Duchenne muscular dystrophy mutations from other X-linked retinal disorders with negative-shaped ERGs.