Stickler Syndrome (SS): Laser Prophylaxis for Retinal Detachment (Modified Ora Secunda Cerclage, OSC/SS).

Purpose: To introduce a novel technique of encircling laser prophylaxis (ora secunda cerclage Stickler syndrome, OSC/SS) to prevent rhegmatogenous retinal detachment (RRD) in Stickler syndrome eyes.

Patients And Methods: After first eye RRD at age 50 and at age 18, respectively, a 53-year-old father and his 22-year-old son with type 2 SS (STL2) gave informed consent and underwent OSC/SS prophylaxis, performed in each fellow eye. A 26-year-old STL2 daughter then suffered first eye retinal detachment and similarly chose fellow eye OSC/SS prophylaxis.

Reliability of Semiautomated Kinetic Perimetry (SKP) and Goldmann Kinetic Perimetry in Children and Adults With Retinal Dystrophies.

Purpose: To investigate the precision of visual fields (VFs) from semiautomated kinetic perimetry (SKP) on Octopus 900 perimeters, for children and adults with inherited retinal degenerations (IRDs). Goldmann manual kinetic perimetry has long been used in the diagnosis and follow-up of these patients, but SKP is becoming increasingly common. Octopus VFs (OVFs) and Goldmann VFs (GVFs) were both mapped on two occasions.

Evaluating New Ophthalmic Digital Devices for Safety and Effectiveness in the Context of Rapid Technological Development.

Importance: The US Food and Drug Administration's medical device regulatory pathway was initially conceived with hardware devices in mind. The emerging market for ophthalmic digital devices necessitates an evolution of this paradigm.

Objectives: To facilitate innovation in ophthalmic digital health with attention to safety and effectiveness.

Fixational eye movements in Tourette syndrome.

Studies of saccadic eye movements in subjects with Tourette syndrome (TS) have provided additional evidence that there is a link between TS symptoms and deficits in fronto-striato-thalamic networks. These studies revealed impaired timing and inhibition of saccades. We compared fixational eye movements, such as microsaccades and ocular drifts, in subjects with TS and healthy controls.

Standing Balance and Spatiotemporal Aspects of Gait Are Impaired Upon Nocturnal Awakening in Healthy Late Middle-Aged and Older Adults.

Study Objectives: Nocturnal awakenings may constitute a unique risk for falls among older adults. We describe differences in gait and balance between presleep and midsleep testing, and whether changes in the lighting environment during the midsleep testing further affect gait and balance.

Methods: Twenty-one healthy, late middle-aged and older (64.

Safety and Proof-of-Concept Study of Oral QLT091001 in Retinitis Pigmentosa Due to Inherited Deficiencies of Retinal Pigment Epithelial 65 Protein (RPE65) or Lecithin:Retinol Acyltransferase (LRAT).

Unlabelled: Restoring vision in inherited retinal degenerations remains an unmet medical need. In mice exhibiting a genetically engineered block of the visual cycle, vision was recently successfully restored by oral administration of 9-cis-retinyl acetate (QLT091001). Safety and visual outcomes of a once-daily oral dose of 40 mg/m2/day QLT091001 for 7 consecutive days was investigated in an international, multi-center, open-label, proof-of-concept study in 18 patients with RPE65- or LRAT-related retinitis pigmentosa.

Oral 9-cis retinoid for childhood blindness due to Leber congenital amaurosis caused by RPE65 or LRAT mutations: an open-label phase 1b trial.

Background: Leber congenital amaurosis, caused by mutations in RPE65 and LRAT, is a severe form of inherited retinal degeneration leading to blindness. We aimed to assess replacement of the missing chromophore 11-cis retinal with oral QLT091001 (synthetic 9-cis-retinyl acetate) in these patients.

Methods: In our open-label, prospective, phase 1b trial, we enrolled patients (aged ≥6 years) with Leber congenital amaurosis and RPE65 or LRAT mutations at McGill University's Montreal Children's Hospital.

Evaluating Barten image metric for predicting character recognition in people with low vision.

Electronic devices with small visual displays (SVDs) are often inaccessible to the millions of Americans with vision loss. The Barten square root integral (SQRI) is an image quality metric that has been shown to predict whether people with normal vision can see images on a cathode ray tube monitor. The present proof-of-concept study begins to explore whether the same metric could predict the ability of users with low vision to see images on SVDs.

Patient awareness of binocular central scotoma in age-related macular degeneration.

Purpose: To assess whether age-related macular degeneration (AMD) patients are aware of binocular central visual field defects.

Methods: One hundred fifty-three consecutive AMD patients in their initial low-vision rehabilitation evaluation were immediately asked at the beginning of their visit (1) whether they were able to see any blind spots or defects in their field of vision and (2) whether they had any evidence or experiences that led them to believe that they had defects in their field of vision. They then had their vision assessed by binocular central visual field testing using the California Central Visual Field Test, binocular reading performance evaluated using the Smith-Kettlewell Reading Test (SK Read) and MN Read charts, and visual acuity measured using the ETDRS chart at 1 meter.

A test of face discrimination ability in aging and vision loss.

Purpose: The ability to recognize faces is fundamental to social interactions but has not been studied extensively in visual disorders such as age-related macular degeneration (AMD). We report here the development of a face discrimination test, in which both response times (RTs) and accuracies are measured. Results are compared for young and older control subjects and older adults with AMD to determine the factors underlying performance on this test.

Incomplete cortical reorganization in macular degeneration.

Purpose: Activity in regions of the visual cortex corresponding to central scotomas in subjects with macular degeneration (MD) is considered evidence for functional reorganization in the brain. Three unresolved issues related to cortical activity in subjects with MD were addressed: Is the cortical response to stimuli presented to the preferred retinal locus (PRL) different from other retinal loci at the same eccentricity? What effect does the role of age of onset and etiology of MD have on cortical responses? How do functional responses in an MD subject's visual cortex vary for task and stimulus conditions?

Methods: Eight MD subjects-four with age-related onset (AMD) and four with juvenile onset (JMD)-and two age-matched normal vision controls, participated in three testing conditions while undergoing functional magnetic resonance imaging (fMRI). First, subjects viewed a small stimulus presented at the PRL compared with a non-PRL control location to investigate the role of the PRL.

Eye and visual function in traumatic brain injury.

Combat blast is an important cause of traumatic brain injury (TBI) in the Department of Veterans Affairs polytrauma population, whereas common causes of TBI in the civilian sector include motor vehicle accidents and falls. Known visual consequences of civilian TBI include compromised visual acuity, visual fields, and oculomotor function. The visual consequences of TBI related to blast remain largely unknown.

Age effects on visual-perceptual processing and confrontation naming.

The impact of age-related changes in visual-perceptual processing on naming ability has not been reported. The present study investigated the effects of 6 levels of spatial frequency and 6 levels of contrast on accuracy and latency to name objects in 14 young and 13 older neurologically normal adults with intact lexical-semantic functioning. Spatial frequency and contrast manipulations were made independently.

Estimating cerebral blood volume with expanded vascular space occupancy slice coverage.

A model for quantifying cerebral blood volume (CBV) based on the vascular space occupancy (VASO) technique and varying the extent of blood nulling yielding task-related signal changes with various amounts of blood oxygenation level-dependent (BOLD) and VASO weightings was previously described. Challenges associated with VASO include limited slice coverage and the confounding inflow of fresh blood. In this work, an approach that extends the previous model to multiple slices and accounts for the inflow effect is described and applied to data from a multiecho sequence simultaneously acquiring VASO, cerebral blood flow (CBF), and BOLD images.

Models of reading performance in older adults with normal age-related vision.

Reading rate with print size (reading performance) was studied in a group of 132 older readers with normal age-related vision using the Minnesota Low Vision Reading Acuity (MNREAD) test. Regression by a monotonic Weibull model had greater convergence success with lower residual error than either Logistic or Gompertz models. Reading performance by Weibull model regression was characterized by inflexion slope, critical print size, and maximum reading rate.

Effects of preferred retinal locus placement on text navigation and development of advantageous trained retinal locus.

Sixty readers were evaluated for visual function and text-navigation ability. The visual field and preferred retinal locus (PRL) were measured with a scanning laser ophthalmoscope (SLO). We found significant differences in text-navigation ability based on scotoma and PRL placement.

Visual function in patients with choroidal neovascularization resulting from age-related macular degeneration: the importance of looking beyond visual acuity.

Purpose: A detailed understanding of overall quality of vision may help primary care physicians, optometrists, and general ophthalmologists to improve the care of patients with choroidal neovascularization (CNV) resulting from age-related macular degeneration (AMD).

Methods: Published literature was reviewed using Medline searches and the authors' knowledge of the field.

Results: Both visual acuity and contrast sensitivity are strongly associated with the ability to perform vision-related activities of daily living.

Tracking retinal motion with a scanning laser ophthalmoscope.

The vast majority of people with low vision retain some functional vision to perform everyday tasks. To study the effectiveness and efficiency of the visual tasks performed by people with low vision, knowing the movement patterns of their preferred retinal locus (PRL) used for fixation, saccade, and pursuit is critical. The scanning laser ophthalmoscope (SLO) has been used to acquire retinal images while a subject is performing a visual tracking exercise.

Preferred retinal loci and macular scotoma characteristics in patients with age-related macular degeneration.

Many patients with macular scotomas due to age-related macular degeneration do not perceive black spots in the visual field where the scotomas are located. Rather, they describe objects as "vanishing," "jumping out of nowhere" or "having blurry parts," or a combination of features. In addition, when the macular scotoma affects the fovea, the visual system uses 1 or more preferred retinal loci (PRLs) as a "pseudofovea" to perform visual tasks.

The artificial silicon retina microchip for the treatment of vision loss from retinitis pigmentosa.

Objective: To determine the safety and efficacy of the artificial silicon retina (ASR) microchip implanted in the subretinal space to treat vision loss from retinitis pigmentosa.

Methods: The ASR microchip is a 2-mm-diameter silicon-based device that contains approximately 5000 microelectrode-tipped microphotodiodes and is powered by incident light. The right eyes of 6 patients with retinitis pigmentosa were implanted with the ASR microchip while the left eyes served as controls.