It is well accepted that the accommodation system is characterized by steady-state errors in focus. The purpose of this study was to correlate these errors with changes in ocular wavefront aberration and corresponding image quality when accommodating. A wavefront analyzing system, the Complete Ophthalmic Analysis System (COAS), was used in conjunction with a Badal optometer to allow continuous recording of the aberration structure of the eye for a range of accommodative demands (up to 8 D). Fifty consecutive recordings from seven subjects were taken. Monocular accommodative response was calculated as (i) the equivalent refraction minimizing wavefront error and (ii) the defocus needed to optimize the modulation transfer function at high spatial frequencies. Previously reported changes in ocular aberrations with accommodation (e.g., the shift of spherical aberration to negative values) were confirmed. Increased accommodation errors for near targets (lags) were evident for all subjects, although their magnitude showed a significant intersubject variability. It is concluded that the one-to-one stimulus/response slope in accommodation function should not always be considered as ideal, because higher order aberrations, especially changes of spherical aberration, may influence the actual accommodative demand. Fluctuations may serve to preserve image quality when errors of accommodation are moderate, by temporarily searching for the best focus.
Reaction times (RTs) are obtained for a wide range of contrasts of vertical sinusoidal gratings. The data are plotted as a function of the reciprocal of contrast. In some conditions, a single linear function accounts for the data. In others a clear bi-linear function is obtained. The low and high contrast regions of the function are interpreted as representing magno and parvo activity, respectively. RT-based supra-threshold sensitivity functions are obtained for different luminances, stimulus durations and eccentricities and these are compared with conventional threshold-based sensitivities to establish the extent to which RTs and contrast sensitivity are constrained by the same sensory processes.
Cone-rod degeneration (CRD) belongs to the disease spectrum of retinal degenerations, a group of hereditary disorders characterized by an extreme clinical and genetic heterogeneity. It mainly differentiates from other retinal dystrophies, and in particular from the more frequent disease retinitis pigmentosa, because cone photoreceptors degenerate at a higher rate than rod photoreceptors, causing severe deficiency of central vision. After exome analysis of a cohort of individuals with CRD, we identified biallelic mutations in the orphan gene CEP78 in three subjects from two families: one from Greece and another from Sweden. The Greek subject, from the island of Crete, was homozygous for the c.499+1G>T (IVS3+1G>T) mutation in intron 3. The Swedish subjects, two siblings, were compound heterozygotes for the nearby mutation c.499+5G>A (IVS3+5G>A) and for the frameshift-causing variant c.633delC (p.Trp212Glyfs(∗)18). In addition to CRD, these three individuals had hearing loss or hearing deficit. Immunostaining highlighted the presence of CEP78 in the inner segments of retinal photoreceptors, predominantly of cones, and at the base of the primary cilium of fibroblasts. Interaction studies also showed that CEP78 binds to FAM161A, another ciliary protein associated with retinal degeneration. Finally, analysis of skin fibroblasts derived from affected individuals revealed abnormal ciliary morphology, as compared to that of control cells. Altogether, our data strongly suggest that mutations in CEP78 cause a previously undescribed clinical entity of a ciliary nature characterized by blindness and deafness but clearly distinct from Usher syndrome, a condition for which visual impairment is due to retinitis pigmentosa.
The variation in power across the simultaneous-image lenses produces enhanced depth of focus. The through-focus nature of the image, which influences the "best focus" (distance correction) and the reading addition, will vary with several factors, including lens centration, the wearer's pupil diameter, and ocular aberrations, particularly spherical aberration; visual performance with some designs may show greater sensitivity to these factors.
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