Foveation dynamics in congenital nystagmus IV: vergence.

PURPOSE: To evaluate foveation dynamics and characteristics of vergence eye movements during fixation of static targets at different distances and while tracking a target moving in depth in a subject with congenital nystagmus (CN). METHOD: Eye movements of a well-studied subject with CN were recorded using the magnetic search coil technique and analyzed using the OMtools software, including the eXpanded Nystagmus Acuity Function (NAFX). RESULTS: Both the phase planes and NAFX values during fixation of targets at various near distances were equivalent to those during fixation of a far target. When applied to vergence data, the NAFX values ("binocular" NAFX) were higher than for the individual eye data. Vergence tracking of targets moving in depth was demonstrated and was accurate for targets moving at speeds up to ~ 35°/sec. CONCLUSIONS: Target foveation qualities during fixation of targets at various near distances were equivalent to that during fixation of a far target. Stereo discrimination was limited by the foveation quality of the eye with the higher NAFX waveform. Foveation period slopes during vergence tracking demonstrated vergence movements despite the ongoing CN oscillation. Similar to what we found with fixation, pursuit, and the vestibulo-ocular systems, these findings establish that vergence in both static and dynamic viewing conditions functions normally in the presence of the CN oscillation.

Ocular Motor System Control Models and the Cerebellum: Hypothetical Mechanisms.

To review our studies and "top-down" models of saccadic intrusions and infantile nystagmus syndrome with the aim of hypothesizing areas of cerebellar connections controlling parts of the ocular motor subsystems involved in both types of function and dysfunction. The methods of eye-movement recording and modeling are described in detail in the cited references. Saccadic intrusions, such as square-wave jerks and square-wave oscillations, can be simulated by a single malfunction, whereas staircase saccadic intrusions required two independent malfunctions. The major infantile nystagmus syndrome waveforms are traceable to a failure to calibrate the damping ratio of the smooth pursuit system. The use of a behavioral ocular motor system model demonstrated how putative cerebellar dysfunctions could accurately simulate both the oscillations and the ocular motor responses seen in patients with both saccadic and pursuit disorders.

Effects of topical brinzolamide on infantile nystagmus syndrome waveforms: eyedrops for nystagmus.

BACKGROUND: Recent advances in infantile nystagmus syndrome (INS) surgery have uncovered the therapeutic importance of proprioception. In this report, we test the hypothesis that the topical carbonic anhydrase inhibitor (CAI) brinzolamide (Azopt) has beneficial effects on measures of nystagmus foveation quality in a subject with INS. METHODS: Eye movement data were taken, using a high-speed digital video recording system, before and after 3 days of the application of topical brinzolamide 3 times daily in each eye. Nystagmus waveforms were analyzed by applying the eXpanded Nystagmus Acuity Function (NAFX) at different gaze angles and determining the longest foveation domain (LFD) and compared to previously published data from the same subject after the use of a systemic CAI, contact lenses, and convergence and to other subjects before and after eye muscle surgery for INS. RESULTS: Topical brinzolamide improved foveation by both a 51.9% increase in the peak value of the NAFX function (from 0.395 to 0.600) and a 50% broadening of the NAFX vs Gaze Angle curve (the LFD increased from 20° to 30°). The improvements in NAFX after topical brinzolamide were equivalent to systemic acetazolamide or eye muscle surgery and were intermediate between those of soft contact lenses or convergence. Topical brinzolamide and contact lenses had equivalent LFD improvements and were less effective than convergence. CONCLUSIONS: In this subject with INS, topical brinzolamide resulted in improved-foveation INS waveforms over a broadened range of gaze angles. Its therapeutic effects were equivalent to systemic CAI. Although a prospective clinical trial is needed to prove efficacy or effectiveness in other subjects, an eyedrops-based therapy for INS may emerge as a viable addition to optical, surgical, behavioral, and systemic drug therapies.

Two Electrical Engineers, One Problem, and Evolution Produced the Same Solution: A Historical Note.

This note adds historical context into solving the problem of improving the speed of the step response of a low-order plant in two different types of control systems, a chemical mixing system and the human saccadic system. Two electrical engineers studied the above problem: one to understand and model how nature and evolution solved it and the other to design a control system to solve it in a man-made commercial system. David A. Robinson discovered that fast and accurate saccades were produced by a pulse-step of neural innervation applied to the extraocular plant. Leonidas M. Mantgiaris invented a method to achieve rapid and accurate chemical mixing by applying a large stimulus for a short period of time and then replacing it with the desired steady-state value (i.e., a "pulse-step" input). Thus, two humans used their brains to: 1) determine how the human brain produced human saccades; and 2) invent a control-system method to produce fast and accurate chemical mixing. That the second person came up with the same method by which his own brain was making saccades may shed light on the question of whether the human brain can fully understand itself.

A New Clinical Method for Visual Function Evaluation Including Estimation and Measurement of Therapeutic Effectiveness: Lessons From Infantile Nystagmus Research.

PURPOSE: To translate infantile nystagmus system (INS) research into easily understood, clinically relevant terminology and suggest modifications to research and clinical testing, data and clinical interpretation, and therapeutic choices and evaluation. METHODS: A clinical method is presented using only three best-corrected visual acuity measurements of patients with INS, whereby (1) a measure of the quality of visual acuity across the visual field is possible; (2) pre-therapy estimates of post-therapy improvements in peak acuity and the high-acuity range of gaze angles are possible; and (3) more realistic visual function outcome measures of therapy are available to the practitioner. RESULTS: The application of the high-acuity field quality spreadsheet to the analyses of patients with INS (before and after therapy) results in a quantitative measure of visual function based on three visual acuity measurements. CONCLUSIONS: The clinician can now duplicate adequate functional visual acuity descriptions in patients with INS along with their pre-therapy estimates and outcome measures. Previously, these have only been available to researchers or the rare clinicians who have access to both eye movement data and the expanded nystagmus acuity function analysis of INS waveforms. [J Pediatr Ophthalmol Strabismus. 2021;58(3):188-195.].

Topical lambda-cyhalothrin in reducing eye oscillations in a canine model of infantile nystagmus syndrome.

PURPOSE: To determine the ocular and systemic safety of using topical Lambda-Cyhalothrin (LCL) in a canine model of infantile nystagmus syndrome (INS). The rationale for this proposal is based on a case study of a patient whose INS improved after inadvertent ocular exposure to a pyrethroid pesticide containing LCL. METHODS: After in-vitro safety testing and IUCAC approval, we studied increasing concentrations of topical LCL drops (0.002% to 0.07%) in canines with a purposely bred defect in the RPE65 gene resulting in both retinal degeneration and INS. We collected data on ocular and systemic effects and performed eye-movement recordings (EMR). RESULTS: At the 0.07% concentration dose of LCL, there was minimal, reversible, conjunctival hyperemia. There was no other ocular or systemic toxicity. At the 0.06% dose, there was a visible decrease in the INS and EMR showed a 153%-240% increase in the nystagmus acuity function and a 30%-70% decrease in amplitude across gaze. There was also a 40%-60% decrease in intraocular pressure while on the drop in both eyes. CONCLUSION: This animal study suggests this new pharmacological agent has potential for topical treatment of both INS and diseases with raised intraocular pressure. Further, this new treatment approach confirms the importance of extraocular muscle proprioception in ocular motor diseases and their treatment.

Eye-movement-based assessment of visual function in patients with infantile nystagmus syndrome.

PURPOSE: Infantile Nystagmus Syndrome (INS) is an ocular motor system dysfunction characterized by the rhythmic to-and-fro oscillations of the eyes. Traditionally, the assessment of INS visual function solely focused on null- or primary-position visual acuity. Our purpose is to use the past four decades of INS research to introduce a more complete assessment of visual function in patients with INS. METHODS: All eye-movement data were obtained using high-speed digital video, infrared reflection, or scleral search coil systems. RESULTS: We have introduced four important aspects of a more complete INS visual function assessment: the eXpanded Nystagmus Acuity Function and visual acuity measurements in primary position; broadness of the eXpanded Nystagmus Acuity Function peak and high-acuity field; target acquisition time; and gaze-maintenance capability. CONCLUSIONS: Visual function in patients with INS is multifactorial and the simple assessment of primary position visual acuity is both inadequate and may not be the most important characteristic in overall visual function. A more complete visual function assessment should also include primary and lateral gaze eye-movement and visual acuity examinations, target acquisition time and gaze holding.

Two hypothetical nystagmus procedures: augmented tenotomy and reattachment and augmented tendon suture (Sans tenotomy).

PURPOSE: To review the hypothetical mechanism and therapeutic benefits of the four-muscle tenotomy and reattachment (T&R) procedure using knowledge accrued over the 10 years since its proposal; to describe an augmented tendon suture (ATS) technique to improve the procedure based on one of the originally suggested alternative methods (mechanical); and to hypothesize a new ATS procedure to achieve the same therapeutic benefits without extraocular muscle tenotomy or reattachment to the globe. METHODS: Standard surgical methods were used. RESULTS: The T&R procedure damps and improves infantile nystagmus syndrome (INS) waveforms, improves eXtended Nystagmus Acuity Function (NAFX) values, broadens the NAFX peak versus gaze angle, and damps slow eye movements but not saccades. The T&R procedure also damps acquired pendular and downbeat nystagmus, decreasing the patients' oscillopsia, and lowers the target acquisition time in INS. CONCLUSION: The T&R procedure directly affects only the enthesis of the tendon; there is idiosyncratic variation in the distribution of afferent fibers in the tendons. The ATS technique consists of placing several additional sutures in the tendon proximal to the tenotomy. Based on the hypothetical proprioceptive mechanism for the beneficial effects of the T&R procedure, the authors hypothesize that the ATS technique will maximize the therapeutic benefits and that an ATS procedure, using only tendon sutures without tenotomy, will duplicate the therapeutic effects of T&R. Eliminating the tenotomy component results in a simpler procedure more suitable for single-session, multi-muscle surgery that may be required for improving the waveforms of multiplanar nystagmus and less prone to cause complications.

Development and surgical removal of an epiretinal membrane in infantile nystagmus syndrome: a new type of oscillopsia.

PURPOSE: To report and discuss a focal oscillopsia in a small area of the visual field produced by, and after the removal of, an epiretinal membrane (ERM) in an individual with infantile nystagmus syndrome (INS) since birth with no associated afferent visual deficits. STUDY DESIGN: A retrospective case report. METHODS: A chart review, including clinical and electrophysiological data. A 74 y/o man with INS and an epiretinal membrane was studied. Detailed studies of the retina post-removal of an epiretinal membrane, with consequent changes in best-corrected visual acuity (BCVA), and subjective oscillopsia compared to INS waveforms. OCT measurements and eye-movement data from digital video and scleral search-coil systems were used. RESULTS: The monocular ERM produced an unexpected focal area of torsional/vertical oscillopsia (noted 1 year prior to the ERM surgery) in the portion of the visual field that corresponded with distortions from the ERM. The remainder of the visual field, corresponding with normal healthy retina was unaffected and stable in all planes. Post-removal, BCVA improved with redevelopment of the foveal pit and focal oscillopsia became less noticeable but remained due to the retinal distortion. CONCLUSIONS: In patients with INS, complete oscillopsia suppression across the visual field requires undistorted vision. If a retinal area of visual distortion develops or results from retinal surgery, a symptomatic island of oscillopsia in one or more planes may result.

Clinical and ocular motor complications of extraocular muscle extirpation for infantile nystagmus syndrome.

PURPOSE: To describe the effects of extraocular muscle extirpation performed after previous eye muscle surgery in a 20-year-old woman with infantile nystagmus syndrome (INS) for whom we have 19 years of follow-up data. METHODS: Clinical examinations were performed. Eye movement data analysis was carried out using the eXpanded Nystagmus Acuity Function (NAFX) and longest foveation domain (LFD). RESULTS: The patient re-presented to the authors at age 20, 2 years after bilateral anterior myectomy of the horizontal rectus muscles, bilateral anterior nasal transposition of the inferior oblique muscle, and bilateral superior oblique recessions. Evaluation revealed deterioration in nystagmus at lateral gaze angles, new incomitant strabismus with severe loss of convergence, limited ductions, saccadic hypometria, slow saccades, and hypo-accommodation. Also, there was a pre- to post-extirpation minimal change of 21% in her peak NAFX, a 50% decrease in LFD, plus a predominant, asymmetric, multiplanar oscillation. CONCLUSIONS: It appears that in this patient, horizontal extirpation failed to abolish the nystagmus and caused significant, new, symptomatic deficits interfering with many of the patient's visual functions.

Longitudinal Studies and Eye-Movement-Based Treatments of Infantile Nystagmus Syndrome: Estimated and Measured Therapeutic Improvements in Three Complex Cases.

INTRODUCTION AND PURPOSE: To demonstrate the utility of using eye-movement data to reveal the diagnostic characteristics of infantile nystagmus syndrome (INS), determine treatment, and both estimate and document therapeutic improvements in three patients with well-developed foveation periods, fairly broad, lateral gaze "nulls," head turns, strabismus, and complex, multiplanar nystagmus. PATIENTS AND METHODS: Infrared reflection, magnetic search coil, and high-speed digital video systems were used to record the eye movements of INS patients, pre- and post-Kestenbaum null-point correction surgery (horizontal or vertical). Data were analyzed and estimations made, using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools toolbox for MATLAB. RESULTS: In all three subjects (S1-S3), both peak NAFX and longest foveation domain (LFD) improved from their pre-Kestenbaum values. S1: 0.700-0.745 (6.4%) and 25-34° (36%), respectively. S2: 0.445-0.633 (42.4%) and >40° to >50° (10%), respectively. S3: 0.250-0.300 (20%) and 13° to ≫18° (see text), respectively. CONCLUSIONS: S1: Even at the high ends of the pre-therapy NAFX and LFD spectra, INS foveation (and therefore, visual-function) improvements may be adequate to justify nystagmus surgery and provide clinical improvements beneficial to the patient. S2: INS foveation improvements in the vertical plane are equal to those originally estimated using the horizontal data in prior patients. S3: Two apparent NAFX peaks can be converted into a very broad peak by surgery based on the preferred lower peak.

Combining recessions (nystagmus and strabismus) with tenotomy improved visual function and decreased oscillopsia and diplopia in acquired downbeat nystagmus and in horizontal infantile nystagmus syndrome.

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 hypertropia and rotated both eyes downward. Patient 2 had horizontal infantile nystagmus, a 20 degrees left-eye exotropia, and alternating (abducting-eye) fixation. Lateral rectus recessions and medial rectus tenotomies were performed. Horizontal and vertical eye movements were recorded pre- and postsurgically using high-speed digital video. The eXpanded Nystagmus Acuity Function (NAFX) and nystagmus amplitudes and frequencies were measured. RESULTS: Patient 1: The NAFX peak moved from 10 degrees up to primary position where NAFX values improved 17% and visual acuity increased 25%. Vertical NAFX increased across the -10 degrees to +5 degrees vertical range. Primary-position right hypertropia decreased approximately 50%; foveation time per cycle increased 102%; vertical amplitude, oscillopsia, and diplopia were reduced, and frequency was unchanged. Patient 2: Two lateral, narrow high-NAFX regions (due to alternating fixation) became one broad region with a 43% increase in primary position (acuity increased approximately 92.3%). Diplopia amplitude decreased; convergence and gaze holding were improved. Primary-position right exotropia was reduced; foveation time per cycle increased 257%; horizontal-component amplitude decreased 45.7%, and frequency remained unchanged. CONCLUSIONS: Combining tenotomy with nystagmus or strabismus recession procedures increased NAFX and visual acuities and reduced diplopia and oscillopsia in downbeat nystagmus and infantile nystagmus.

Combined gaze-angle and vergence variation in infantile nystagmus: two therapies that improve the high-visual-acuity field and methods to measure it.

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 gaze and convergence angles and the expanded nystagmus acuity function (NAFX) to evaluate the IN waveform's foveation quality at all fixation points. RESULTS: Recordings demonstrated that, at far, both subjects exhibited classic nulls (high NAFX values) with NAFX reduction at gaze angles lateral to the null. S1 was treated with prisms and S2 with surgery. When converged at near or at far with base-out prisms (S1) or after bimedial recession and bilateral tenotomy surgery (S2), NAFX was higher at both the null and lateral gaze angles; the null region was broadened. The longest foveation domain (gaze angles where the NAFX is within 10% of its peak) at near was three times wider than at far for S1 and two times wider after than before surgery for S2. The therapeutic improvement domain (gaze angles where the posttherapy NAFX is higher than pretherapy) was even broader. At fixed gaze angles in the central 20 degrees of gaze, S1's NAFX variation with vergence exhibited hysteresis, higher during divergence than convergence; S2 exhibited no hysteresis after surgery. CONCLUSIONS: Damping IN by means of convergence, induced either surgically or with prisms, broadened the range of gaze angles with higher foveation quality, mimicking the null-broadening effects of tenotomy. The discovery of vergence hysteresis may reflect pulley movement and might allow higher acuity, if a near point is transiently fixated just before a far target. The acuity domains provide new and more comprehensive evaluations of both pre- and posttherapy visual function than do primary-position acuity measurements, suggesting that high-visual-acuity fields should be included in clinical measures of visual function in nystagmus.

Eye movement recordings as an effectiveness indicator of gene therapy in RPE65-deficient canines: implications for the ocular motor system.

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 distant targets at gaze angles varying between +/-15 degrees horizontally and +/-10 degrees vertically. Ocular motility recordings were made, using two distinct methods-infrared reflection and high-speed video. The resultant recordings from three untreated, four treated, and three pre- and post-treatment dogs were analyzed for using the eXpanded Nystagmus Acuity Function (NAFX), which yields an objective assessment of best potential visual acuity, based on the duration and repeatable accuracy of foveation and centralisation. RESULTS: During fixation, the untreated dogs exhibited large-amplitude, classic IN waveforms, including pendular and jerk in both the horizontal and vertical planes, which prevented them from keeping the targets within the area centralis (the region of highest receptor density, spanning +/-3 degrees horizontally by +/-1.5 degrees vertically, analogous to the fovea). Some untreated dogs also had small-amplitude (0.5-1 degrees), high-frequency (6-9 Hz) oscillations. Under the same conditions, successfully treated canines no longer exhibited clinically detectable IN. Their IN was converted to waveforms with very low amplitudes that yielded higher NAFX values and allowed target images to remain well within the area centralis. Of note, uniocular treatment appeared to damp the IN in both eyes. Behaviorally, the treated dogs were able to successfully navigate through obstacles more easily without inadvertent contact, a task beyond the untreated dogs' ability. CONCLUSIONS: Gene therapy that successfully restored retinal function also reduced the accompanying IN to such a great extent that it was not clinically detectable approximately 90% of the time in many of the dogs. IN improvement, as quantified by the NAFX, is an objective motor indicator of visual improvement due to gene therapy.

"Staircase" saccadic intrusions plus transient yoking and neural integrator failure associated with cerebellar hypoplasia: a model simulation.

We present hypothesized ocular motor mechanisms of unique "staircase-like" sequences of saccadic intrusions in one direction that we have named, "staircase saccadic intrusions (SSI)," square-wave jerks/oscillations (SWJ/SWO), and transient failures of yoking and neural integrators in a patient with severe hypotonia, ataxic speech, motor and language developmental delays, and torticollis (Joubert syndrome). Brain magnetic resonance imaging showed hypoplasia of the cerebellar vermis and inferior cerebellar peduncles, abnormal superior cerebellar peduncles with deepening of the interpeduncular fossa, and enlargement of the fourth ventricle. During far and near fixation and smooth pursuit (rightward markedly better than leftward), the subject exhibited conjugate SSI (rightward more than leftward, with intersaccadic intervals equivalent to the normal 250 msec visual latency), SWJ, SWO, and uniocular, convergent and divergent saccades (including double saccades). Simulations using a behavioral ocular motor system model identified hypothetical mechanisms for SWJ, SWO, and SSI and ruled out the loss of efference copy as the cause. SSI may result from simultaneous dysfunctions: 1) a transient loss of accurate retinal-error information and/or sampled, reconstructed error; plus 2) a constant sampled, reconstructed retinal error that drives saccades.

Effects of tenotomy on patients with infantile nystagmus syndrome: foveation improvement over a broadened visual field.

PURPOSE: To investigate the effects of four-muscle tenotomy on visual function and gaze angle in patients with infantile nystagmus syndrome (INS). METHODS: Eye movements of nine patients with infantile nystagmus were recorded using infrared reflection or high-speed digital video techniques. Experimental protocols were designed to record the patients' eye-movement waveforms, pre- and post-tenotomy, at different gaze angles. We used the eXpanded Nystagmus Acuity Function (NAFX) to measure tenotomy-induced changes in the nystagmus at primary position and various gaze angles. The longest foveation domains (LFD) were measured from fitted curves. Peak-to-peak nystagmus amplitudes and foveation-period durations were also measured. All measurements were made unmasked. RESULTS: All seven patients with narrow, high-NAFX, gaze-angle regions showed broadening of these regions of higher visual function. Three patients showed moderate NAFX improvement (13.9-32.6%) at primary position, five showed large improvement (39.9-162.4%), and one showed no NAFX change (due to his high pretenotomy NAFX). Primary position measured acuities improved in six patients. All patients had reductions in nystagmus amplitudes ranging from 14.6 to 37%. The duration of the foveation period increased in all nine patients (11.2-200%). The percentage improvements in both the NAFX and the LFD decreased with higher pretenotomy values. CONCLUSIONS: In addition to elevating primary position NAFX, tenotomy also broadens the high-NAFX regions. This broadening effect is more prominent in patients who had sharp pretenotomy NAFX peaks. Four-muscle tenotomy produces higher primary position NAFX increases in infantile nystagmus patients whose pretenotomy values are relatively low, with the improvement decreasing at higher pretenotomy values. The tenotomy procedure improves visual function beyond primary position acuity. This extends the utility of surgical therapy to several different classes of patients with INS for whom other procedures are contraindicated. The pretenotomy NAFX can now be used to predict both primary position acuity improvements and broadening of a patient's high-NAFX range of gaze angles.

The diagnosis and treatment of infantile nystagmus syndrome (INS).

The successful treatment of infantile nystagmus syndrome (INS) depends primarily on accurate and repeatable diagnosis of the type(s) of nystagmus present as well as their variation with gaze and convergence angles or fixating eye. Research over the past 40 years has demonstrated that the only way to achieve both is by making and analyzing ocular motility recordings. Determination of the direct effects of peripheral and central INS therapies can only be made by pre- and post-therapy comparisons of the nystagmus characteristics, specifically of the quality of the foveation periods within each cycle. If one is only interested in cosmetic improvements, diminution of the nystagmus amplitude is all that need be measured. However, if improvement of visual function is the primary goal of therapy, then measurement of the pre- and post-therapy foveation quality must be made, both in primary position and over a broad range of gaze angles. The use of the eXpanded Nystagmus Acuity Function (NAFX) on nystagmus data yields both an accurate measure of foveation quality and a prediction of maximum potential acuity for the patient's waveform. When used with the patient's measured, pre-therapy visual acuity, the NAFX demonstrates the amount of visual acuity loss that is due to sensory abnormalities, demonstrates the amount due to the nystagmus waveform, and estimates the measured post-therapy acuity for all values of improved NAFX and gaze angles measured. The ability to predict visual acuity improvement was not possible before the use of the NAFX. The failure to incorporate accurate measures of nystagmus waveform and foveation quality into their diagnostic evaluation continues to deprive patients of the best possible standard of care and results in mistaken diagnoses as well as inappropriate and, in some cases, unneeded multiple surgeries.

Effects of augmented tenotomy and reattachment in the infantile nystagmus syndrome.

PURPOSE: To test the hypothesis that augmented tenotomy and reattachment surgery (AT-R), which involves placing an additional suture in each distal tendon during the 4-muscle tenotomy and reattachment (T-R) or other infantile nystagmus syndrome (INS) procedures, could increase the beneficial effects of many types of extraocular muscle (EOM) surgery to treat INS. METHODS: Both infrared reflection and high-speed digital video systems were used to record the eye movements in 4 patients with INS before and after AT-R surgery. Data were analyzed using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools software. RESULTS: Placement of the augmentation suture did not interfere with Kestenbaum, Anderson, bilateral medial rectus muscle recession, or T-R surgeries. The therapeutic effects of AT-R were similar to but not equal to those from the traditional single-suture surgeries (ie, broadening longest foveation domain [LFD] but no improvement of NAFX peak). The average of the NAFX percent improvements after AT-R was within 31% of those estimated from NAFX values before T-R; the average of the percent broadenings of the LFD values after AT-R was within 16%. CONCLUSIONS: The AT-R does not improve the foveation quality in INS above the traditional T-R surgery. It is not improved by an additional suture; indeed, some improvements may be diminished by the added suture. The hypothesized augmented-tendon suture technique (sans tenotomy) has been modified and remains to be tested.

Development of new treatments for congenital nystagmus.

The use of ocular motor data as the basis for the development of both nonsurgical and surgical therapies for congenital nystagmus (CN) has been underway since the mid-1960s. This paper presents three nonsurgical therapies (composite prisms, soft contact lenses, and afferent stimulation) and a new surgical therapy (four-muscle tenotomy) hypothesized from analysis of ocular motor data. The expanded nystagmus acuity function test was developed to both predict and measure the effectiveness of CN therapies and for intersubject comparisons. Base-out prisms may be used to damp CN during distance fixation in patients whose CN damps during near fixation and who are binocular (i.e., they have no strabismus). Soft contact lenses may be used in those whose CN damps with afferent stimulation of the ophthalmic division of the trigeminal nerve. Cutaneous afferent stimulation (rubbing, vibration, or electricity) of the forehead or neck damps CN in some individuals. Finally, as first demonstrated in an achiasmic Belgian sheepdog and later in humans, tenotomy of the four horizontal rectus muscles and reattachment at their original sites may also damp CN. Taken together, these findings suggest the existence of one or more proprioceptive feedback loops acting to change the small-signal gain of the extraocular plant. Four-muscle tenotomy provides a needed therapeutic option for the many individuals with CN for whom other surgical therapies are contraindicated. Tenotomy may also prove useful in see-saw nystagmus (it abolished it in the aforementioned canine) or other types of nystagmus; further studies of the latter are required.