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The Nystagmus Acuity Function (NAF) provides an objective measurement of the foveation characteristics of nystagmus waveforms and an assessment of potential visual acuity for subjects with congenital (CN) or latent/manifest latent (LMLN) nystagmus. It is based on the subjects' ability to maintain fixation within a physiologically based 'foveation window' of(More)
Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epithelium-specific 65-kDa protein gene (RPE65). We investigated the safety of subretinal delivery of a recombinant adeno-associated virus (AAV) carrying RPE65 complementary(More)
PURPOSE To investigate the convergence-induced waveform and high-acuity-field improvements resulting from different therapies in two subjects with infantile nystagmus (IN) that was damped by convergence and to report a new finding in one of the subjects. METHODS Infrared reflection was used to measure eye movements during fixation of targets at different(More)
Attempts to simulate dysfunction within ocular motor system (OMS) models capable of exhibiting known ocular motor behavior have provided valuable insight into the structure of the OMS required for normal visual function. The pendular waveforms of congenital nystagmus (CN) appear to be quite complex, composed of a sustained sinusoidal oscillation punctuated(More)
We investigated the effects of four-muscle tenotomy on saccadic characteristics in infantile nystagmus syndrome (INS) and acquired pendular nystagmus (APN). Eye movements of 10 subjects with INS and one with APN were recorded using infrared reflection, magnetic search coil, or high-speed digital video. The expanded nystagmus acuity function (NAFX)(More)
PURPOSE To investigate the effects of combined tenotomy and recession procedures on both acquired downbeat nystagmus and horizontal infantile nystagmus. METHODS Patient 1 had downbeat nystagmus with a chin-down (upgaze) position, oscillopsia, strabismus, and diplopia. Asymmetric superior rectus recessions and inferior rectus tenotomies reduced right(More)
PURPOSE To perform ocular motility recordings of infantile nystagmus (IN) in RPE65-deficient canines and determine whether they can be used as a motor indicator of restored retinal function to investigate the effects of gene therapy. METHODS Treated and untreated canines were comfortably suspended in a custom-built sling and encouraged to fixate on(More)
PURPOSE Human eye-movement recordings have documented that surgical treatment of congenital nystagmus (CN) also produces a broadening of the null zone and changes in foveation that allow increased acuity. We used the achiasmatic Belgian sheepdog, a spontaneously occurring animal model of human CN and see-saw nystagmus (SSN), to test the hypothesis that(More)
We applied new methods for canine eye-movement recording to the study of achiasmatic mutant Belgian Sheepdogs, documenting their nystagmus waveforms and comparing them to humans with either congenital nystagmus (CN) alone or in conjunction with achiasma. A sling apparatus with head restraints and infrared reflection with either earth- or head-mounted(More)