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The neuro-ophthalmic examination.

A properly performed history and examination are essential to diagnosing correctly neuro-ophthalmic disorders. Aspects of the neuro-ophthalmic history and examination, particularly those dealing with evaluation of visual loss, are reviewed.

Color Perception Tests↗

Functional evaluation using magnetic resonance imaging of the visual cortex in patients with retrochiasmatic lesions.

OBJECT: The goal of this study was to evaluate the clinical potential of combining functional magnetic resonance (fMR) imaging with conventional morphological MR imaging and to assess its usefulness for objective evaluation of visual function as part of treatment planning in patients harboring space-occupying lesions involving the posterior afferent visual system. METHODS: It was hypothesized that regional activation of the visual cortex during visual stimulation would show an asymmetric response consistent with the well-known retinotopical organization of the human visual cortex. To test this hypothesis, the pattern of regional cortical activity detected by fMR imaging during binocular repetitive photic stimulation (10 Hz) was compared with the findings of conventional visual field testing. Functional mapping of the visual cortex was performed using a noninvasive blood oxygen level-dependent MR technique in 10 patients with intraaxial and two with extraaxial lesions. Experiments involving two of the patients were unsuccessful because of motion artifacts. In all the remaining patients functional activity was demonstrated in the primary visual area that corresponded to the anatomical location of the calcarine cortex. In nine patients, the identified patterns of activation in the visual cortex were consistent with the visual field deficits (seven homonymous hemianopsias, one homonymous central scotoma, and one inferior quadrantanopsia) and with the traditional teaching of retinotopical representation. Discordance between fMR imaging and perimetric findings was observed in one case. CONCLUSIONS: These results demonstrate that fMR imaging can be performed routinely and successfully in patients with visual abnormalities as part of a conventional neuroradiological evaluation. The technique provides essential information about the function-structure relationship specific to an individual patient and holds promise not only for diagnosis and therapy planning, but also for understanding the topography and functional specialization of the human visual cortex.

Adult↗

Measurement of Friedmann Visual Field Analyzer tests in primary open-angle glaucoma.

A method is described to allow an IBM PC/AT compatible microcomputer program to compute a score from the visual field data collected from a standard Friedmann Visual Field Analyzer (FVFA) Mk II testing procedure. The method could, in principle, be applied to any visual field data which provide quantitative information on the sensitivity of different retinal locations. The score takes into account the depth of any defects, their position in the visual field, and the clustering of defects into scotomata. Visual fields in 119 normal eyes, 82 ocular hypertensive (OH) eyes, and 75 glaucomatous eyes were assessed to provide the data necessary to implement this method. Analyzing the frequency of missed points against point luminance demonstrates that the most effective cut-off from which to regard points as defective is a miss at 0.8 log units or more above the working threshold. A statistical method of analyzing the distribution of defects following Bayesian principles is described and shows that points in the superior nasal, superior arcuate, and inferior arcuate areas provide the most information for detecting glaucoma. The program, written in Turbo Pascal v5.5, incorporates these findings in producing the score.

Aged↗

Mass visual field screening in a driving population.

With the cooperation of the California Department of Motor Vehicles and Synemed, Inc., visual field screening tests were administered to 1,027 eyes of driver's license applicants using the Fieldmaster Model 101-PR automated perimeter. Approximately 5% of the eyes tested demonstrated significant visual field loss. These results indicate that mass visual field screening in a driving population is feasible and may become a valuable technique for early detection of eye disease in large populations. It may also be an important factor in traffic safety.

Adolescent↗

Leber hereditary optic neuropathy associated with antiretroviral therapy for human immunodeficiency virus infection.

PURPOSE: Antiretroviral therapy has reduced the morbidity and mortality associated with human immunodeficiency virus (HIV) infection. However, side effects are increasingly recognized, including a commonly reported toxic mitochondrial myopathy. We report such a case of Leber hereditary optic neuropathy in a patient with antiretroviral therapy for HIV infection and speculate on a possible toxic etiologic role in the development of Leber hereditary optic neuropathy by a shared mitochondrial mechanism. METHODS: Case Report. Bilateral optic disk abnormalities observed in a 38-year-old HIV positive man with a family history of Leber hereditary optic neuropathy were documented with fundus photography, color vision testing, and visual field testing. Mitochondrial DNA testing was used to confirm the genetic predisposition to Leber hereditary optic neuropathy. RESULTS: Progressive bilateral optic nerve pallor temporally associated with the administration of antiretroviral medication was observed. Diagnostic testing revealed progressive visual field and color vision loss as well as a mitochondrial DNA mutation consistent with Leber hereditary optic neuropathy. CONCLUSION: Antiretroviral therapy may be associated with the onset of Leber hereditary optic neuropathy in genetically predisposed patients.

Adult↗

Inapparent visual field defects in multiple sclerosis patients.

To assess inapparent visual field defects in patients with multiple sclerosis free from optic neuritis. During 5 years period 120 patients with multiple sclerosis were examined at the University Department of Ophthalmology, Zagreb University Hospital Center. They were divided into three groups with 40 patients each: patients with acute unilateral optic neuritis, referred to ophthalmologist and treated with pulsed steroid therapy; patients with subjective feeling of blurred vision, normal visual acuity and no signs of acute optic neuritis; and patients free from subjective signs of visual impairment. Study patients underwent standard ophthalmologic examination and visual field testing in photopia by use of quantitative kinetic Goldmann perimetry. The initial and control examination by visual field testing were performed at least 6 months apart. Study results showed 65% of multiple sclerosis patients to have visual field defects without subjective signs of impaired vision. The most common defects were mild to moderate visual field narrowing with blind spot enlargement and depression from above. The following results were recorded: acute optic neuritis group: normal in 13/40 (32.5%) for the affected eyes and 27/40 (67.5%) for fellow eyes; mild visual field narrowing in 4/40 (10%) for the affected eyes and 10/40 (25%) for fellow eyes; moderate visual field narrowing with blind spot enlargement in 14/40 (35%) for the affected eyes and 1/40 (2.5%) for fellow eyes; and paracentral and arcuate scotomata in 9/40 (22.5%) for the affected eyes and 2/40 (5%) for fellow eyes; subjective symptom group: normal in 8/40 (20%) for the affected eyes and 11/40 (27.5%) for fellow eyes; mild visual field narrowing in 11/40 (27.5%) for the affected eyes and 16/40 (40%) for fellow eyes; moderate visual field narrowing with blind spot enlargement in 18/40 (45%) for the affected eyes and 10/40 (25%); andparacentral and arcuate scotomata in 3/40 (7.5%) for both affected and fellow eyes; and subjective symptom-free group: normal in 24/80 (30%), mild visual field narrowing in 22/80 (27.5%) moderate visual field narrowing with blind spot enlargement in 24/80 (30%); and paracentral and arcuate scotomata in 10/80 (12.5%). The presence of subclinical form of optic nerve involvement could be demonstrated in a very early stage of multiple sclerosis by the introduction of visual field testing in the standard examination protocol.

Adolescent↗

Evaluation of two Humphrey perimetry programs: full threshold and SITA standard testing strategy for learning effect.

PURPOSE: To compare learning effect of Swedish interactive threshold algorithm (SITA) standard strategy with full threshold testing. METHODS: Thirty-nine medical students with no experience in visual field testing had full threshold (FT) and SITA standard for either right or left eyes. They were chosen in such a way that 20 (Group I) had FT for right and SITA for left eyes and 19 (Group II) had SITA standard for right and FT for left eyes. It was designed to have both strategies on same person whereby eliminating inter-individual variability. Visual field testing was repeated in the same week of the first test on the same subject with the same strategy that was chosen for that eye. RESULTS: The authors found an improvement in mean deviation (MD) and pattern standard deviation (PSD) of first and second testings correspondingly for FT (MD from -3.04 to -2.55; PSD from -2.60 to -2.29) and SITA standard (MD from -2.86 to -2.20; PSD from 2.25 to 2.10) and changes were statistically significant (p<0.05). To analyze learning effect of visual field testings, we calculated percentage change in MD and PSD for full threshold and SITA standard strategy. The percentage changes in visual field parameters were significantly lower in SITA standard strategy testing for MD (p=0.02) and PSD (p=0.01). CONCLUSIONS: This study shows that a learning effect is present for both strategies and SITA standard may have a reduced learning effect compared to FT.

Adult↗

Evaluating a visual field screening test for glaucoma: how the choice of the gold standard affects the validity of the test.

OBJECTIVE: To examine the effects of the gold standard on sensitivity, specificity, and area under the ROC curve when determining the validity of a screening test. METHODS: Fifty-eight people were recruited from a Veterans' Administration eye clinic. Screening included testing with the 26-point oculokinetic perimeter (OKP). We used different gold standards to categorize patients as positive or negative for glaucoma. The glaucoma hemifield test (GHT) and corrected pattern standard deviation (CPSD) from the Humphrey visual field analyzer (HVF) were used as objective gold standards. Ophthalmologist review of the HVF, using two different methods for classifying visual field defects, was also used as a gold standard. RESULTS: Using the review of the HVF that included mild, moderate, or severe defects, the area under the ROC curve was not significantly different from 50%. However, for all the other gold standards, the area under the ROC curve was significantly different from 50%. CONCLUSIONS: In determining the ability of the OKP to differentiate between glaucoma and normal subjects, the choice of a gold standard led to different conclusions about its usefulness as a screening test for glaucoma. Furthermore, sensitivity and specificity varied widely depending on which gold standard was used, and what cutoff point was used for the OKP. Although clinician review of the HVF may be used when specific criteria are given to define visual field defects, using objective measures from the HVF is easier and will allow for better comparisons between studies.

Aged↗

The relation between intraocular pressure peak in the water drinking test and visual field progression in glaucoma.

AIM: To compare the results of the water drinking test between glaucomatous eyes with and without visual field progression. METHODS: Retrospective analysis of 76 eyes of 76 open angle glaucoma patients followed for a mean period of 26.0 (SD 13.8) months. Patients were submitted to the water drinking test at the beginning of the follow up period. Reliable achromatic automated perimetry tests performed during the studied period were used to characterise visual field progression. All subjects were under clinical therapy and had an intraocular pressure (IOP) lower than 17 mm Hg monitored by isolated measurements during the follow up period. The results of the water drinking test were compared between glaucomatous eyes with and without visual field progression. RESULTS: Twenty eight eyes reached definite visual field progression. There were no significant differences in the mean age, sex, race, basal IOP, number of antiglaucomatous drugs, initial mean deviation (MD), and corrected pattern standard deviation (CPSD) between eyes that showed visual field progression and the ones who did not progress. A significant difference of 1.9 (SD 0.6) mm Hg (p = 0.001, analysis of covariance; 95% CI 0.8 to 3.0) was observed between glaucomatous eyes that showed visual field deterioration and glaucomatous eyes that did not progress. A significant difference of 16.8% (SD 4.6%) in the mean percentage of IOP variation was also observed between the two groups (p<0.001, analysis of covariance; 95% CI 7.7 to 26.0). CONCLUSIONS: Mean IOP peak and percentage of IOP variation during water drinking test were significantly higher in patients with visual field progression compared with patients who did not progress.

Adult↗

Hemianopic offsets in the visual field of patients with glaucoma.

In 359 glaucoma patients (600 eyes), we measured the peripheral field with kinetic targets and the central 30 degrees field of vision with static testing, using the Field-master-5000 automated perimeter to evaluate the prevalence and significance of step-like visual-field defects along the vertical midline (a hemianopic offset). In all, 129 eyes of 121 patients had a hemianopic offset. In 108 eyes the offsets (involving either the peripheral or the central field or both) were associated with other glaucomatous field changes, whereas 21 eyes showed a peripheral offset as an isolated finding. Of the 8 cases of offsets found in both eyes, 7 were binasal and 1 occurred bitemporally but in separate hemifields. This study suggest that hemianopic offsets occur commonly in glaucoma patients but have limited diagnostic value because most are associated with other glaucomatous field changes. However, these findings help in distinguishing glaucomatous offsets from those caused by neurological lesions.

Glaucoma↗

[Detection of glaucomatous nerve fiber damage. Laser polarimetry in comparison with equivalent visual field loss].

PATIENTS AND METHODS: A total of 45 eyes of 45 patients were equally divided into three age-matched groups (controls, glaucoma, suspected glaucoma). All patients were examined using laser polarimetry and automated visual field testing. We compared the agreement of the location of visual field defects with changes in the nerve fiber layer. In addition we calculated the change in NFLT along a peripapillary circle at four different distances of the optic disc margin. RESULTS: The localization of nerve fiber defects corresponds to the localization of the visual field defect exactly in eight of 15 eyes and roughly in another five. Six of the eyes suspected of having glaucoma showed pathological results with the nerve fiber analyzer (NFA) although visual fields were normal. There was a significant difference for NFLT change towards the periphery between the three groups. CONCLUSION: While the NFA does not always allow a definite correlation between morphological defects and resulting functional impairment, a decrease in the NFLT with increasing distance from the optic disc margin seems helpful for clinical classification. The value of this new method has yet to be proven in longitudinal clinical studies.

Adult↗

Driving after epilepsy surgery: effects of visual field defects and epilepsy control.

The aim of this study was to assess the eligibility to drive in patients with mesial temporal sclerosis who undergo anterior temporal lobectomy. The two major determinants in a patient's ability to drive after such surgery are visual field defects and their seizure frequency. Thirteen patients were selected. The postoperative seizure frequency was assessed using Engel's criteria. Automated static perimetry was performed which consisted of a Humphrey Field Analyser (HFA) 30-2 Test, one for each eye and a Binocular Esterman 120 Test. Seven out of the 13 (54%) selected patients had no seizures post-operatively (Engel's 1); three (23%) patients had less than two seizures per year (Engel's 2) and three (23%) had more than 90% improvement in the frequency of seizures (Engel's 3). The seven patients with no seizures postoperatively were eligible to apply for a driving licence. Automated static perimetry performed on the same patients revealed three (23%) had normal visual field or non-specific loss, seven (54%) had partial homonymous quadrantanopia, one (8%) had complete homonymous quadrantanopia and two (15%) had bilateral concentric loss attributable to vigabatrin, which may have masked any loss occurring due to surgery. Of the 13 patients, only seven (54%) passed the standardised DVLA Esterman visual field test. Of the six (46%) who failed DVLA Esterman visual field test, one had complete homonymous quadrantanopia, three had incomplete homonymous quadrantanopia and two had concentric loss (due to vigabatrin). Although seven (54%) patients passed the visual field test and seven (54%) patients were seizure free only five of the seven seizure-free patients (i.e. 38% of the total number of patients) had visual fields that would make them eligible to drive. As driving is now stated by patients' as a major factor that improves their quality of life, it is important to stress the significance of surgically induced or other iatrogenic visual field defects that may prevent them from driving prior to the operation to avoid disappointments afterwards.

Adult↗

Comparative study of visual, auditory, and olfactory function in Usher syndrome.

BACKGROUND: Usher syndrome is a genotypically and phenotypically heterogeneous group of autosomal recessive diseases featuring retinitis pigmentosa (RP) and sensorineural hearing loss. A general ciliary dysfunction has been suspected following reports of a mutated cytoskeletal protein (myosin VIIA) in type IB, and preliminary data has suggested an olfactory deficit. The purpose of this study was to quantitatively assess olfactory function in Usher syndrome patients and to search for a correlation between the degree of impairment of the three sensory systems as indication of an underlying ciliary defect. METHODS: 39 patients with Usher syndrome (8 type I, 31 type II) were examined. The ophthalmologic protocol included patient history, visual acuity, eye morphology, Goldmann perimetry, and electroretinography. The ENT protocol included a thorough examination, speech-recognition test, pure-tone audiometry and an olfactory function test. RESULTS: In both groups, visual acuity was typically 20/40, the remaining visual field area was small, and the ERG responses were low to non-detectable. Average hearing loss was 100% in type I and 40% in type II. Olfactory thresholds were normal [median 9.7 (I) and 8.5 (II) vs. 8.5 in the control group]. There were multiple significant correlations between parameters of the same organ, but no relationship between parameters of different sensory systems. CONCLUSION: Almost all Usher syndrome patients in this study had an advanced form of RP. In contrast, auditory function differed considerably between type I and type II. An impairment of the olfactory system could not be detected, and there was no correlation between parameters representing visual function, hearing ability, and olfactory sense.

Adult↗

The incidence of irreversible retinal toxicity in patients treated with hydroxychloroquine: a reappraisal.

PURPOSE: To define the risk of hydroxychloroquine (HCQ)-related retinal toxicity in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) who are receiving recommended dosages of the drug (< or =6.5 mg/kg/day). DESIGN: Prospective cohort study, from 1985 to 2000. PARTICIPANTS: Greek patients with RA (n = 335) and SLE (n = 191) treated with HCQ, 400 of whom had completed at least 6 years of treatment. METHODS: Ophthalmologic evaluation was performed every 6 months from 1985 to 1995, and yearly thereafter. This consisted of best-corrected visual acuity, color vision testing, static central visual field testing, fundoscopy, electroretinography, and fluorescein angiography, when indicated. MAIN OUTCOME MEASURES: Fundus lesions attributed to HCQ. RESULTS: No HCQ retinal toxicity was noted in any of the 526 patients during the first 6 years of treatment. Two (3.4%) of the first 58 long-term (>6 years) treated patients developed HCQ-related maculopathy at 8 and 6.5 years of treatment, despite regular ophthalmologic evaluation. On follow-up 7 and 9 years after cessation of HCQ treatment, both patients had stable eye disease. No HCQ retinal toxicity was observed in the subsequent 342 patients who were treated for >6 years. Overall, the incidence of HCQ-related retinopathy in 400 patients who were treated with recommended dosages of the drug for a mean of 8.7 years was reduced to 0.5%. CONCLUSIONS: After a baseline ophthalmic examination to confirm the absence of preexisting fundus pathology, patients with normal renal function may receive HCQ at a maximal daily dosage of 6.5 mg/kg and continue safely for 6 years. However, annual screening is recommended in patients who have taken the drug, even in recommended doses, for >6 years.

Adolescent↗

The efficacy of the dicon screening field to detect eyes with glaucomatous field loss by Humphrey threshold testing.

PURPOSE: The authors compare the results of the Dicon suprathreshold, kinetic fixation perimeter with multiple stimulus presentation to automated threshold perimetry (Humphrey) in the same eye. METHODS: A Dicon screening visual field test and a Humphrey threshold visual field test were performed in 148 eyes of 148 persons with glaucoma or who were suspect for glaucoma. The number and pattern of missed points on the Dicon test were compared with Humphrey global indices in each eye. RESULTS: The median time to complete the 40-point, Dicon suprathreshold test was 2.7 minutes per eye. Regression analyses indicated that Dicon test parameters were modestly correlated with Humphrey corrected pattern standard deviation (CPSD) probability and mean deviation (R2 ranging from 0.21 to 0.46, p = 0.000). With glaucoma defined as a Humphrey Glaucoma Hemifield Test (GHT) result of outside normal limits, the best mix of sensitivity and specificity of Dicon results occurred at 2 or more missed points, with sensitivity of 64% and specificity of 83%. The specificity was maximum (90%) with a Dicon criterion of 3 or more adjacent missed points, but sensitivity at this level was 55%. With glaucoma defined by CPSD probability value less than 1%, sensitivity and specificity for two adjacent missed Dicon points were 69% and 87%, respectively. CONCLUSION: Dicon suprathreshold testing is a practical means to differentiate between some persons with glaucomatous damage and glaucoma suspects.

Adult↗

Unsupervised machine learning with independent component analysis to identify areas of progression in glaucomatous visual fields.

PURPOSE: To determine whether a variational Bayesian independent component analysis mixture model (vB-ICA-mm), a form of unsupervised machine learning, can be used to identify and quantify areas of progression in standard automated perimetry fields. METHODS: In an earlier study, it was shown that a model using vB-ICA-mm can separate normal fields from fields with six different patterns of visual field loss related to glaucomatous optic neuropathy (GON) along maximally independent axes. In the present study, an independent group of 191 patient eyes (66 with ocular hypertension (OHT), 12 with suspected glaucoma by field, 61 with suspected glaucoma by disc, and 52 with glaucoma) with five or more standard visual fields under observation for a mean of 6.24 +/- 2.65 years and 8.11 +/- 2.42 visual fields were evaluated with the vB-ICA-mm. In addition, eyes with progressive GON (PGON) were identified (n = 39). Each participant had a series of fields tested, with each field entered independently and placed along the axes of the previously developed model. This allowed change in one pattern of visual field defect (along one axis) to be assessed relative to results other areas of that same field (no change along other axes). Progression was based on a slope falling outside the 5th and the 95th percentile limits of all slopes, with at least two axes not showing such a deviation in a given individual's series of fields. Fields were also scored using Advanced Glaucoma Intervention Study (AGIS) and the Early Manifest Glaucoma Treatment Trial (EMGT) criteria. RESULTS: Thirty-two of 191 eyes progressed on vB-ICA-mm by this definition. Of the 32, 22 had field loss at baseline, 7 had only GON, 3 were OHTs and 12 were from the 39 eyes (31%) with PGON. The vB-ICA-mm identified a higher percentage of progressing eyes in each diagnostic category than did AGIS or and the EMGT. CONCLUSIONS: The vB-ICA-mm can quantitatively identify progression in eyes with glaucoma by evaluating change in one or more patterns of the visual field loss while other areas or patterns remain stable. This may enable each eye to contribute to the determination of whether change is caused by true progression or by variability.

Adult↗