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Purpose: To test the hypothesis that the topical carbonic anhydrase inhibitor brinzolamide (Azopt) has beneficial effects versus placebo on measures of nystagmus and visual acuity in adult subjects with infantile nystagmus syndrome (INS)., Design: Prospective, cross-over, double masked clinical trial., Setting: Single centre., Study Population: Five subjects ≥18 years old with typical INS and best-binocular visual acuity in their primary position null zone ETDRS 55 letters to 85 letters (20/200 to 20/50) and had no previous treatment for nystagmus., Intervention: In a randomised order, each subject received one drop of Azopt or placebo in both eyes three times a day separated by a washout period of at least a week followed by Azopt or placebo in both eyes three times a day; thus each subject got the drug and placebo, each acting as his or her own control., Outcome Measures: The nystagmus acuity function and INS waveforms obtained from eye movement recordings, binocular optotype visual acuity, using the ETDRS protocol analysed individually and as a group before and after Azopt and placebo., Results: Versus placebo and baseline measures, topical Azopt significantly improved; INS waveform characteristics in the primary position null zone, group mean values of the nystagmus acuity function across gaze (p<0.01) and group mean ETDRS binocular letter visual acuity (p<0.05). There was a predictable decrease in intraocular pressure (IOP) without any systemic or ocular adverse events., Conclusions: Although a prospective large-scale clinical trial is needed to prove effectiveness, an eye-drop-based therapy for INS may emerge as a viable addition to optical, surgical, behavioural and systemic drug therapies for INS., Trial Registration Number: NCT01312402., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Aims: We developed a new data analysis algorithm called the automated nystagmus acuity function (ANAF) to automatically assess nystagmus acuity function. We compared results from the ANAF with those of the well-known expanded nystagmus acuity function (NAFX)., Methods: Using the ANAF and NAFX, we analysed 60 segments of nystagmus data collected with a video-based eye tracking system (EyeLink 1000) from 30 patients with infantile or mal-development fusional nystagmus. The ANAF algorithm used the best-foveation positions (not true foveation positions) and all data points in each nystagmus cycle to calculate a nystagmus acuity function., Results: The ANAF automatically produced a nystagmus acuity function in a few seconds because manual identification of foveation eye positions is not required. A structural equation model was used to compare the ANAF and NAFX. Both ANAF and NAFX have similar measurement imprecision and relatively little bias. The estimated bias was not statistically significant for either methods or replicates., Conclusions: We conclude that the ANAF is a valid and efficient algorithm for determining a nystagmus acuity function.

Background/aims: The infantile nystagmus syndrome (INS) usually begins in infancy and may or may not be associated with visual sensory system abnormalities. Little is known about its specific waveforms in the first 6 months of life or their relation to the developing visual system. This study identifies the clinical and ocular motility characteristics of the INS and establishes the range of waveforms present in the first 6 months of life., Methods: 27 infants with involuntary ocular oscillations typical of INS are included in this analysis. They were evaluated both clinically and with motility recordings. Eye movement analysis was performed off line from computer analysis of digitised data. Variables analysed included age, sex, vision, ocular abnormalities, head position, and null zone, neutral zone characteristics, symmetry, conjugacy, waveforms, frequencies, and foveation times., Results: Ages ranged from 3 to 6.5 months (average 4.9 months). 15 patients (56%) had abnormal vision for age, nine (33%) had strabismus, five (19%) had an anomalous head posture, 13 (48%) had oculographic null and neutral positions, nine (33%) had binocular asymmetry, and only two showed consistent dysconjugacy. Average binocular frequency was 3.3 Hz, monocular frequency 6.6 Hz. Average foveation periods were longer and more "jerk" wave forms were observed in those patients with normal vision., Conclusions: Common clinical characteristics and eye movement waveforms of INS begin in the first few months of infancy and waveform analysis at this time may help with both diagnosis and visual status.

Background: Penetrating keratoplasty in infancy and childhood has traditionally met with limited visual success due to a combination of unique physiology and technical problems in this patient population. With the advances in microsurgical instrumentation, corneal preservation, and visual developmental physiology ophthalmologists are finding increasing indications for penetrating keratoplasty in the childhood population. The long term results of neonatal penetrating keratoplasty in two patients with unilateral congenital corneal opacification are reported., Methods: Penetrating keratoplasty was performed on one eye in each of two infants within the first 3 weeks of life. Amblyopia treatment and optical therapy have been continued since surgery., Results: After 6 years both grafts have remained clear. One patient developed the infantile esotropia syndrome. Visual development using Snellen optotypes is age normal for both transplanted eyes., Conclusions: Penetrating keratoplasty when combined with optical correction and amblyopia therapy may restore and preserve vision in selected patients with congenital corneal opacification if performed in the neonatal period.

The ocular findings and visual prognosis were reviewed in 24 patients with the Treacher-Collins syndrome who were evaluated in the craniofacial clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between 1980 and 1991. All patients had some abnormality. Vision loss was present in 37% of patients. Amblyopia was present in 33%, significant refractive errors were present in 58%, and anisometropia was documented in 17%. Strabismus was present in 37% and significant lid and adnexal abnormalities were seen in 96%. The prognosis for normal vision in at least one eye is good but vision loss secondary to amblyopia is more resistant to treatment owing to other medical problems and social concerns.