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A therapeutic trial of vitamin A in patients with pigmentary retinal degenerations: a negative study.

A prospective therapeutic trial was designed to test the hypothesis that measurable improvement of retinal functions might occur in some patients with pigmentary retinal degeneration when placed on high doses of solubilized vitamin A (Aquasol A, 50,000 I.U. per day by mouth for 28 days). After a standard ophthalmic history and examination pre-trial examinations consisting of visual acuity, visual fields, color vision tests, dark adaptations and cone thresholds were obtained on two separate occasions. Electroretinography was usually performed only once. Forty-seven patients were entered into the study of which 27 had typical retinitis pigmentosa. The patients showed no significant change in visual function from pre-trial results when tested after taking the vitamin A for 28 days. Post-trial electroretinography performed on 10 patients with recordable pre-trial electroretinograms showed no change. The 10 patients retested at six and 12 months after the trial showed no significant change in visual function.

Adolescent↗

Colour vision in diabetic and normal pseudophakes is worse than expected.

Automated colour vision testing in pseudophakes showed unexpected results. Chromatic discrimination sensitivity was measured in 22 diabetic pseudophakes with no retinopathy, 23 diabetic pseudophakes with background retinopathy and 34 non-diabetic pseudophakes. These results were compared with those in age-matched normal and diabetic phakic subjects, all of whom had good vision. The diabetics were also matched for retinopathy grading and duration of diabetes. In all three groups, red-green discrimination sensitivity was worse in the pseudophakes when compared with the corresponding phakic subjects (normals, p < 0.001; no retinopathy, p = 0.467; background retinopathy, p = 0.057). However, tritan vision was marginally worse in the normal pseudophake group but was better in the two diabetic pseudophake groups, when compared with phakic controls. This may be due to a reduction in tritan sensitivity in age-matched phakic controls from the effects of increased lens yellowing with age.

Aged↗

[A retrospective study of patients with epidemic optic neuropathy in a health area].

The sample of 104 patients who were diagnosed optic epidemic neuropathy at the health area of the "Rampa" Polyclinic in 1993 was reviewed. 50 of them were studied again. Visual acuity as well as the sensitivity to contrast by the Pelli Robson's method were explored. Ishihara's color-vision test was applied and an ophthalmological examination including fundus of the eye was made. All this was done by a group of experts who were searching temporary papillary paleness or lost of the bundles of papillomacular fibres, which are the diagnostic elements of the disease even in those cases that recover vision. 21 (42%) of the 50 studied patients fulfilled the criteria established for these cases by the Ministry of Public Health concerning visual acuity and color vision to diagnose optic epidemic neuropathy, although only 14 (28%) were ratified as cases. The typical alterations of the fundus of the eye were described, confirming the diagnosis. Cases were classified according to the initial state of visual acuity and to evolution. 3 had had an affectation of the visual acuity of 0.1 or worse (severe), and only one patient had a mild affectation with 0.8 of vision. The rest ranged between 0.2 and 0.6. Only one patient had a serious sequela. The low percentage of cases ratified as optic epidemic neuropathy does not represent what happened in the whole country, but it may be considered as a pattern of what took place at those units with the same conditions during the peak of the epidemic.

Color Perception↗

[Progressive cone dystrophy: electrophysiological changes in female carriers].

The authors evaluated a family with X-linked progressive cone dystrophy and special attention was paid to female carriers. Twenty-four members of the family were examined. One generation II--male and five generation III--males were affected. Two generation II--females who, in each case had affected children, but who were asymptomatic, underwent electrophysiological evaluations. The electroretinograms were found to be subnormal in both patients with alterations of cone-mediated responses and color vision. The discovery of abnormalities in female carriers emphasized the necessity of systematically performing electroretinography, together with color vision testing and pedigree examination, when assessing so called sporadic cone dystrophy or in cases where the modes of inheritance are not clear.

Adolescent↗

Subjective perceptual distortions and visual dysfunction in children with autism.

Case reports and sensory inventories suggest that autism involves sensory processing anomalies. Behavioral tests indicate impaired motion and normal form perception in autism. The present study used first-person accounts to investigate perceptual anomalies and related subjective to psychophysical measures. Nine high-functioning children with autism and nine typically-developing children were given a questionnaire to assess the frequency of sensory anomalies, as well as psychophysical tests of visual perception. Results indicated that children with autism experience increased perceptual anomalies, deficits in trajectory discrimination consistent with dysfunction in the cortical dorsal pathway or in cerebellar midsagittal vermis, and high spatial frequency contrast impairments consistent with dysfunctional parvocellular processing. Subjective visual hypersensitivity was significantly related to greater deficits across vision tests.

Adolescent↗

L-Dopa improves colour vision in Parkinson's disease.

In recent studies disorders of colour vision in Parkinsonian patients have been demonstrated. Up to now, the influence of dopaminergic treatment on those phenomena remains unclear. We therefore performed a colour vision test (Farnsworth-Munsell 100 Hue Test) in 19 patients with Parkinson's disease before and after the oral application of the morning dose of L-Dopa. The colour discrimination was significantly improved after the ingestion of L-Dopa. There was no different effect of L-Dopa on the blue-yellow or red-green axis of colour vision. The morphological structures responsible for these colour vision disturbances are unknown, but it can be concluded that the dopamine deficiency in Parkinson's disease is not restricted to the basal ganglia but may involve the visual system as well.

Adult↗

[Physiologic principles of studying visual functions].

The retinal coding system for visual information comprises three important steps: 1) hyper- and depolarization of the sensory cells as the active basis for perception of light and dark; 2) reaction solely to changes in the bipolar cell on/off system, as a consequence of the action of the horizontal cells with the receptive fields, as a basis for adaptation to brightness and glare; and 3), under the influence of the amacrine cells, important time-space functions as a basis for visual perception of movement. The signal form, which changes over from analog to digital in the ganglion cell system, also results from this last step. therefore, both latencies and amplitudes (VEP) are important criteria for long signal response paths. Taking the examples of "statistical perimetry", visual acuity testing, and examination of contrast sensitivity, clinically relevant methodological problems from signal detection theory are illustrated: there are no "certain" measured values. This uncertainty and the age-dependence of normal values make it difficult to identify pathologic conditions, though trend analyses can help. As the sizes of the retinal areas examined by these methods differ so widely, correlations between the various examination methods are theoretically possible; but one method can never be substituted by another. In routine practice, to detect pathology early, one will sometimes deviate from the prescribed examination method (vision tests with low contrast for visual acuity).

Dark Adaptation↗

Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system.

PURPOSE: To determine the phenotype of 3 patients from 2 pedigrees with an unusual form of cone dystrophy in which the peripheral cone system is more affected than the central cone system, and whose rod system is relatively normal. DESIGN: Three observational case reports with electrophysiologic and psychophysical studies. METHODS: Three patients underwent fundus photography, fluorescein angiography, color vision testing, Goldmann visual field testing, full-field electroretinograms (ERGs), focal macular cone ERGs, and rod-cone perimetry, in addition to routine ophthalmologic examinations. Multifocal ERGs also were recorded from 2 patients. RESULTS: The fundus examination and fluorescein angiogram results essentially were normal except for a mild temporal pallor of the optic disc in 2 patients. The corrected visual acuity ranged from 20/16 to 20/100. Color vision was normal in 1 patient, but was abnormal in 2 patients. A relative paracentral scotoma was detected in 2 patients. Full-field ERG cone responses were reduced significantly, but rod responses were normal in all patients, as in patients with typical cone dystrophy. However, the focal macular cone ERGs were well preserved in all patients. Psychophysical rod-cone perimetry demonstrated that the peripheral cone system was impaired, whereas the rod sensitivity was completely normal. The results of the multifocal ERG in 2 patients supported the findings made by the full-field and focal macular ERGs. CONCLUSIONS: These findings demonstrate that there is a subgroup of patients with cone dystrophy where the peripheral cone system is more affected than the central cone system.

Adult↗

The ability of color defectives to judge signal lights at sea.

Measures were made of the ability of color-defective men to judge correctly the colors of navigation lights (red, green, or white) presented to them at night under realistic sea conditions. Eighty-one color-defective men were employed; they were categorized as to type and degree of defect using a battery of five color-vision tests. While the average performance of the color-defective men was considerably poorer than that of 24 color normals, there were large individual differences within each category of defect. Attempts to account for these differences in performance by variations in acuity, intelligence, and motivation failed. The extent to which the data can be accounted for by modern color-vision theory is discussed.

Color Vision Defects↗

Bilateral simultaneous orbital decompression for Graves' orbitopathy with a combined endoscopic and Caldwell-Luc approach.

Graves' orbitopathy can lead to cosmetic deformity, orbital pain, and visual impairment. Surgical intervention can improve proptosis, cosmetic appearance of the eyelids, vision, and orbital pain with minimal morbidity. Ten patients with dysthyroid orbitopathy underwent concurrent bilateral orbital decompressions. Of these, 9 underwent simultaneous bilateral endoscopic and transantral decompressions, and 1 had only bilateral endoscopic decompressions. Medical management of keratopathy was attempted before surgery. All patients were previously treated with radioiodine and high-dose corticosteroids, and 2 patients had prior low-dose orbital irradiation. Preoperative and postoperative visual acuity, color vision testing, and measurement of proptosis were recorded for all patients. In addition, photographs and CT scans of the orbit and sinuses were done. After surgery, visual acuity improved in 8 patients and remained unchanged in 2 patients. Diplopia in the primary and downward gaze improved in 1 patient and remained unchanged in the 3 others who had it before surgery. Color vision deficits in the blue/yellow range were present in 8 patients before surgery and all reversed within 2 weeks after surgery. Proptosis decreased by an average of 4.83 mm (range 4-7 mm) in patients undergoing the combined decompression and decreased by 4 mm in the 1 patient who had the endoscopic decompression only. Ethmoid sinusitis developed in 1 patient but resolved with oral antibiotics, and another patient had an asymmetric result requiring additional unilateral surgery, which corrected the asymmetry. Overall, this approach avoids external incisions and allows excellent visualization in the regions of the orbital apex and ethmoid roof, facilitating maximal decompression without the increased risk of bleeding or visual disturbances.

Adult↗

Colour vision and occupational toluene exposure: results of repeated examinations.

Potential effects of human occupational exposures to toluene on colour vision were investigated in a follow-up study over 4 years with three repeated examinations. Colour vision was measured with the Lanthony desaturated colour vision test D-15d, and the colour confusion index (CCI) was calculated. The mean current exposures were 26+/-21 ppm for printers (high toluene level) and 3+/-4 ppm for end-processors (low toluene level). The mean exposure durations were 23+/-6 years for "long-time exposed" and 7+/-2 years for "short-time exposed" subjects. Repeated analyses (n=162) and multiple regressions (maximum available n=267) did not reveal significant effects of toluene with respect to intensity or duration of current or long-term exposure. Age and occupational qualification were significantly associated with CCI in both kinds of analysis, whereas alcohol consumption (carbohydrate-deficient transferrin, CDT) and smoking habits (cigarettes per day) were not. It is concluded that current industrial exposure limits of toluene provide sufficient protection against possible disturbance of colour vision.

Adult↗

Unilateral or asymmetric congenital ptosis, head posturing, and amblyopia.

PURPOSE: To determine the prognostic implication of compensatory head posturing in patients with unilateral or asymmetric congenital ptosis. METHODS: A retrospective review of 80 consecutive patients with unilateral or asymmetric congenital ptosis was performed. The presence of documented compensatory head posturing, age of onset, age of presentation, visual acuity, refraction, and amblyopia were recorded, and binocularity was tested. RESULTS: Five of seven (71%), patients with unilateral or asymmetric congenital ptosis and compensatory head posturing had amblyopia. All of these patients had straight eyes and four of the five amblyopic patients had anisometropia of less than 2 diopters (D). CONCLUSION: The high incidence of amblyopia in this group can occur in the absence of significant anisometropia and strabismus. This unusually high incidence of amblyopia in this subgroup of patients with unilateral or asymmetric congenital ptosis and compensatory head posturing warrants compulsive examination and prophylactic part-time occlusion therapy of the nonptotic eye until reliable vision testing can be performed.

Amblyopia↗

Autosomal recessive vitreoretinopathy and encephaloceles.

We conducted a ten-year follow-up of an unusual pedigree with an autosomal recessive vitreoretinal degeneration, severe myopia, and congenital encephalocele. All five affected members (four girls and one boy) also had early, recurrent bilateral detachments. Color vision testing disclosed an acquired tritan dyschromatopsia and electroretinography showed subnormal photopic and scotopic amplitudes, delayed b-wave implicit times and 30-Hz flicker-phase relations, and absent scotopic b-wave oscillations.

Child↗

Performance of red-green color deficient subjects on the Farnsworth Lantern (FALANT).

BACKGROUND: The Farnsworth Lantern (Falant) is an occupational color vision test intended to identify people with significant red-green color deficiency who are unable to name aviation, marine or railway signal lights correctly. The colors shown are white, green and red selected to be within protan and deutan isochromatic zones. HYPOTHESIS: The Falant grades the severity of color deficiency and identifies subjects with different types of deficiency. METHOD: 270 color deficiency subjects (diagnosed with the Neitz anomaloscope) were examined. A subset of 108 subjects also completed the Farnsworth D15 and the Farnsworth-Munsell 100 hue test. RESULTS: All dichromats and 75% of anomalous trichromats failed the Falant. The mean error score of dichromats was greater than that anomalous trichromats, but errors were made in a similar number of qualitative color naming categories. The range of Falant error scores was continuous with no demarkation between the criteria for pass and fail. It was not possible to identify anomalous trichromats likely to pass the Falant from the size of the anomaloscope matching range or from the results of Farnsworth-Munsell tests. CONCLUSIONS: People with severe red-green color deficiency fail the Falant, but neither the type nor the severity of color deficiency can be determined either from the qualitative results or from the error score.

Aviation↗

Peripheral neuropathy in styrene-exposed workers.

BACKGROUND: The toxicity of styrene on the peripheral nervous system is still debated. CASES: The paper presents two cases of peripheral sensorimotor neuropathy in styrene-exposed workers. Exposure, evaluated by biological monitoring, ranged between 100 and 150% of the current limits proposed by the American Conference of Governmental Industrial Hygienists (ACGIH). The subjects complained of leg weakness and numbness, cramps, and paresthesia. Electrophysiology revealed a moderate peripheral sensorimotor neuropathy of a demyelinating type. Color-vision testing showed a subclinical deficit. Common inherited and acquired causes of peripheral neuropathy and dyschromatopsia other than styrene were ruled out by personal history, medical examination, laboratory data, and chest X-ray. CONCLUSIONS: The results suggest that long-term occupational exposure to environmental levels of styrene that are equal, or slightly above, the ACGIH limits can induce a clinical form of peripheral neuropathy and a subclinical impairment of color vision. As a consequence, a careful reappraisal of the real preventive meaning of the current ACGIH occupational limit for styrene, at least on an individual basis, is needed.

Adult↗

Neurological deficits in solvent-exposed painters: a syndrome including impaired colour vision, cognitive defects, tremor and loss of vibration sensation.

Five individuals are described who had participated in a study of former dockyard painters. All had worked between 16 years and 45 years as industrial painters, much of the time inside ships. All underwent structured neurological examination, colour vision testing (allowing calculation of a colour confusion index corrected for age and alcohol), and detailed psychometric testing. An occupational history sufficient to allow estimation of past exposure to solvents was taken. All gave a history of exposure to high concentrations of solvents at work, and several described episodes of acute narcosis. All showed neurological deficits and some had overt neurological disease, although in no case had this previously been linked to their work. The most striking features, sufficient to constitute a syndrome, were acquired blue-yellow colour vision deficits, coarse tremor, impaired vibration sensation in the legs and cognitive impairment. Their estimated cumulative exposures to solvents ranged between the equivalent of 13 and 37 calendar years working at the Occupational Exposure Standard concentration (OES years). This study for the first time gives an indication of the concentrations of solvents likely to lead to serious neurological disease in humans. It serves as a reminder to physicians to take an occupational history from patients with obscure neurological or psychological impairment.

Aged↗