Abstract
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° 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.
In some individuals with infantile nystagmus syndrome (INS),
1 the nystagmus damps at a specific gaze angle (null angle) or with convergence (convergence null) on a near target.
2 3 Because the vergence angle damps the INS, the same result is obtained regardless of the stimulus inducing the convergence.
4 5 This fact has been widely exploited therapeutically, either optically (e.g., prisms
6 7 8 ) or surgically, to allow better visual acuity.
9 10 11 12 13 14 15 16 17 18 19 It has been our experience that in binocular patients with both types of nulls, the vergence null is usually stronger than the gaze-angle null.
20 We may take advantage of that by the use of base-out prisms and −1.00 S (to converge the eyes and negate the induced accommodation) while viewing distant targets in primary position.
2 Alternatively, surgical recession of both medial rectus muscles (artificial divergence) requires convergence to realign the eyes, thereby damping the nystagmus.
13 21 22 23
The visual acuities of individuals with INS, or fusion maldevelopment nystagmus syndrome (FMNS) is directly related to the foveation characteristics of nystagmus waveforms (Guo S, et al.
IOVS 1990;31:ARVO Abstract 83).
24 25 26 27 28 29 30 31 32 33 34 35 36 Using these waveform characteristics (i.e., foveation-period duration, standard deviations of both mean foveation position and velocity, and number of cycles in an interval of fixation), we defined a mathematical function, the expanded nystagmus acuity function (NAFX), and incorporated it into a computer program to automate its application (Jacobs JB, et al.
IOVS 1998;39:ARVO Abstract 697).
37 The NAFX is a repeatable, numerical measure of waveform foveation quality and estimation of the best-predicted visual acuity that a subject with nystagmus can achieve under benign real-world conditions. Its prior use in human (including a masked-data clinical trial) and animal studies has established its value as both a waveform quality measure and acuity estimator.
18 34 38 39 40 We previously defined the
longest foveation domain (LFD; the range of gaze angles in which the NAFX is within 10% of its peak value—that is, within 1 Snellen line of the best acuity).
37 The LFD identifies the range of gaze angles where high acuity is possible and provides a new and potentially important measure of visual function (a
high-visual-acuity field) for patients with INS. To this, we add the therapeutic improvement domain (TID, range of gaze angles in which the NAFX is greater than the baseline). The TID is a therapeutic measure of
all gaze angles with increased acuity.
One objective of this study was to investigate the observation that once IN is damped by convergence in a binocular subject, it remains damped over a broader range of gaze angles than when the subject is not converged (i.e., the damping is not confined to primary position).
41 42 We wanted to investigate whether (1) the damping of IN by convergence also improves the waveform, yielding a higher potential visual acuity; (2) the beneficial effects of convergence apply across a wide range of gaze angles; and (3) either base-out prisms or bimedial recession surgery can produce similar therapeutic effects. We used the NAFX as a quantitative measure of waveform quality. If the NAFX values increase under the above-imposed conditions or therapies, the hypotheses are supported; if they do not, the hypotheses are rejected. We present detailed data analyses from two representative INS subjects: One (S1) was treated with prisms and the other (S2) with surgery. Both demonstrated the same effects of vergence on potential visual function. We also present the unexpected finding that
hysteresis exists in the effects of vergence on INS.
Both subjects were treated with therapies that induced convergence on far targets; S1 using prisms and S2, a combination of bimedial rectus muscle recessions and bilateral muscle tenotomies. These therapies are contraindicated by strabismus and lack of stereopsis.
S1 was a 61-year-old man with INS and no other visual system deficits. The beneficial effects of base-out prisms on both the INS waveform and visual acuity have been documented.
2 32 His observations of improved acuity at lateral gaze angles when using base-out prisms prompted this study. S1’s horizontal–torsional waveforms were pendular with foveating saccades (P
fs) and pseudopendular with foveating saccades (PP
fs), with well-developed foveation. In addition to a gaze-angle null at 2° left gaze, his IN damped with convergence; he is an experienced observer. He had no strabismus and normal stereopsis. His best corrected visual acuity improved from 20/40 OU to 20/25 OU with 7-D base-out prism therapy added to his refraction (presbyopia negated the need for −1.00 S to be added OU).
S2 was a 16-year-old girl with INS and no other visual system deficits. She was a naïve observer (unaware of the purposes of this study) and the characteristics of her IN included jerk (J), jerk with extended foveation (Jef), pseudocycloid (PC), and PPfs waveforms. Clinically, there was no gaze-angle null but convergence damped the IN. She had no strabismus and normal stereopsis. Her best corrected visual acuity was 20/40 OU. Therapeutically, minimal bimedial rectus muscle recessions (2 mm each) and bilateral rectus muscle tenotomies were performed, after which the eye-movement measures were repeated. After surgery, her visual acuity improved to 20/25 OU. No strabismus was induced by the surgery.
An interesting, unexpected, and potentially important finding of our study was the hysteresis (i.e., system output is dependent on both the current and previous inputs) exhibited by S1’s NAFX. In the central 20° of gaze (0°, ±5°, ±10°) the NAFX measured for fixation on most targets was higher during divergence than during convergence—that is, the NAFX at a specific vergence angle was higher if the previous target was closer rather than farther away. Therefore, the mechanism underlying the convergence effects in raising the potential visual acuity (NAFX) of S1 was more effective during divergence at central gaze angles than during convergence. To investigate whether hysteresis was uncommon or dependent on fixation of stationary targets (e.g., the stepped-target paradigm), we examined the data of randomly selected prior subjects with INS with convergence damping. Four of five (80%) subjects exhibited hysteresis while fixating the slowly moving vergence target. Two subjects who fixated (5 seconds) stationary primary-position targets at different distances during convergence and divergence between far and near (20 D) targets did not. The effects of greater convergence (e.g., 60 D) were not measured; this, and any differences between vergence pursuit and vergence steps are currently being assessed as part of a study focused on hysteresis.
IN Convergence Damping.
NAFX-Vergence Hysteresis.