Effects of Acetazolamide on Infantile Nystagmus Syndrome Waveforms: Comparisons to Contact Lenses and Convergence in a Well-Studied Subject

M.J Thurtell1, 2, L.F Dell’Osso*, 1, 2, 3, R.J Leigh1, 2, 3, M Matta1, 4, J.B Jacobs1, 2, R.L Tomsak1, 2
1 Daroff-Dell’Osso Ocular Motility Laboratory, Louis Stokes Cleveland Department of Veterans Affairs Medical Center and CASE Medical School, USA
2 Department of Neurology, University Hospitals Case Medical Center; Cleveland, OH, USA
3 Department of Biomedical Engineering, Case Western Reserve University and University Hospitals Case Medical Center; Cleveland, OH, USA
4 Clinical Neurology, Department of Neuroscience, the University of Sassari, Sassari, Italy

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© Thurtell et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Daroff-Dell’Osso Ocular Motility Laboratory, Louis Stokes Department of Cleveland Veterans Affairs Medical Center, 10701 East Boulevard, Cleveland, OH 44106 USA; Tel: (216) 421-3224; Fax: (216) 231-3461; E-mail:



To determine if acetazolamide, an effective treatment for certain inherited channelopathies, has therapeutic effects on infantile nystagmus syndrome (INS) in a well-studied subject, compare them to other therapies in the same subject and to tenotomy and reattachment (T&R) in other subjects.


Eye-movement data were taken using a high-speed digital video recording system. Nystagmus waveforms were analyzed by applying an eXpanded Nystagmus Acuity Function (NAFX) at different gaze angles and determining the Longest Foveation Domain (LFD).


Acetazolamide improved foveation by both a 59.7% increase in the peak value of the NAFX function (from 0.395 to 0.580) and a 70% broadening of the NAFX vs Gaze Angle curve (the LFD increased from 20° to 34°). The resulting U-shaped improvement in the percent NAFX vs Gaze Angle curve, varied from ~60% near the NAFX peak to over 1000% laterally. The therapeutic improvements in NAFX from acetazolamide (similar to T&R) were intermediate between those of soft contact lenses and convergence, the latter was best; for LFD improvements, acetazolamide and contact lenses were equivalent and less effective than convergence. Computer simulations suggested that damping the central oscillation driving INS was insufficient to produce the foveation improvements and increased NAFX values.


Acetazolamide resulted in improved-foveation INS waveforms over a broadened range of gaze angles, probably acting at more than one site. This raises the question of whether hereditary INS involves an inherited channelopathy, and whether other agents with known effects on ion channels should be investigated as therapy for this condition.

Keywords: Infantile nystagmus syndrome, waveforms, foveation, drug.