Documenta Ophthalmologica

, Volume 133, Issue 1, pp 61–70 | Cite as

Abnormal 8-Hz flicker electroretinograms in carriers of X-linked retinoschisis

  • J. Jason McAnany
  • Jason C. Park
  • Frederick T. Collison
  • Gerald A. Fishman
  • Edwin M. Stone
Original Research Article



To evaluate rod-isolated, cone-isolated, and combined rod and cone flicker electroretinograms (ERGs) as a possible means to identify electrophysiological abnormalities in carriers of X-linked retinoschisis (XLRS).


Full-field ERGs were recorded from six carriers of XLRS (aged 34–66 years) and eight normally sighted subjects (aged 27–59 years) under rod-isolated (ERGR), cone-isolated (ERGC), and combined rod and cone (ERGR+C) conditions. ERGs were obtained using a four-primary LED-based ganzfeld photostimulator and standard recording techniques. The four primaries were modulated sinusoidally in phase to achieve combined rod and cone activation (ERGR+C) or in different phases to achieve ERGR and ERGC by means of triple silent substitution. After 30 min of dark adaptation, 8- and 15-Hz ERGR, ERGC, and ERGR+C responses were obtained at a mean luminance level of 24 scot. cd/m2. Standard ISCEV ERGs were also obtained from each subject.


The ISCEV and 15-Hz flicker ERGs were generally within the normal range for the carriers. The 8-Hz ERGR, ERGC, and ERGR+C amplitudes were also generally normal. In contrast, the carriers had ERGR, ERGC, and ERGR+C timing abnormalities, with phase advances beyond the range of normal for the ERGR (four carriers), ERGC (four carriers), and ERGR+C (three carriers). Only one carrier had normal 8-Hz responses under all conditions.


The 8-Hz ERG timing abnormalities in five of six carriers indicate that retinal function is not necessarily normal in carriers of XLRS. The 8-Hz flicker ERG may be useful for studying retinal function in these individuals.


Electroretinogram (ERG) Rod Cone Flicker X-linked retinoschisis 



This research was supported by National Institutes of Health Research Grants R00EY019510 (JM), P30EY001792 (UIC core Grant), an unrestricted departmental Grant from Research to Prevent Blindness, the Pangere Family Foundation, and the Grousbeck Family Foundation (GAF and EMS).


The National Institutes of Health, Research to Prevent Blindness, the Pangere Family Foundation, and the Grousbeck Family Foundation provided financial support in the form of funding. The sponsors had no role in the design or conduct of this research.

Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  1. 1.Department of Ophthalmology and Visual SciencesUniversity of Illinois at ChicagoChicagoUSA
  2. 2.Department of PsychologyUniversity of Illinois at ChicagoChicagoUSA
  3. 3.Department of BioengineeringUniversity of Illinois at ChicagoChicagoUSA
  4. 4.The Pangere Center for Inherited Retinal DiseasesThe Chicago LighthouseChicagoUSA
  5. 5.Stephen A. Wynn Institute for Vision Research, Department Ophthalmology and Visual SciencesThe University of IowaIowa CityUSA

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