Related Experiment Video
Updated: Aug 29, 2026
How to Obtain Reliable Visual Event-related Potentials in Newborns
Published on: October 24, 2019
Visual electrophysiological screening in diagnosing infants with congenital nystagmus
Jelka Brecelj1, Branka Stirn-Kranjc
1Eye Clinic, University Medical Centre, Zaloska c. 29a, 1525 Ljubljana, Slovenia. jelka.brecelj@kclj.si
Insights
Visual electrophysiological screening using electroretinogram (ERG) and visual evoked potentials (VEP) helps classify congenital nystagmus in infants. This non-invasive method identifies retinal and postretinal pathway dysfunction, aiding diagnosis.
Area of Science:
- Ophthalmology
- Neuroscience
- Pediatrics
Background:
- Congenital nystagmus (CN) is an involuntary eye movement condition often associated with visual pathway dysfunction.
- Accurate diagnosis of CN requires evaluating both retinal and postretinal pathways.
- Early identification of visual pathway abnormalities is crucial for timely intervention in infants.
Purpose of the Study:
- To assess the diagnostic value of visual electrophysiological screening in infants with congenital nystagmus.
- To differentiate between retinal and postretinal visual pathway dysfunction in this population.
- To establish the utility of simultaneous electroretinogram (ERG) and visual evoked potentials (VEP) for CN evaluation.
Main Methods:
- Simultaneous electroretinogram (ERG) and visual evoked potentials (VEP) were recorded in 28 infants with congenital nystagmus.
- Skin electrodes were used for ERG, and VEP was recorded using flash and/or pattern-reversal stimuli.
- Recordings were performed in infants under one year of age, with follow-up assessments conducted up to age six years.
Main Results:
- Initial screening identified retinal dysfunction in 36% and postretinal dysfunction in 32% of infants.
- At follow-up, retinal dysfunction was present in 36% and postretinal dysfunction in 50% of children.
- Normal retinal and postretinal function was observed in 14% of children, all diagnosed with congenital idiopathic nystagmus.
Conclusions:
- Visual electrophysiological screening, including simultaneous ERG and VEP, is effective in evaluating infants with congenital nystagmus.
- This non-invasive method objectively identifies retinal and postretinal pathway dysfunction, aiding in the classification of CN.
- The findings support the use of ERG and VEP as essential tools for diagnosing the underlying causes of congenital nystagmus in infants.
Objective:
To determine the value of visual electrophysiological screening in evaluating retinal and postretinal visual pathway function in infants with congenital nystagmus.
Methods:
In 28 infants with congenital nystagmus electroretinogram (ERG) was recorded with skin electrodes and, since the infants were alert, visual evoked potentials (VEP) were simultaneously recorded. The first recording was performed before the age of 1 year (age 2-11 months, mean age 6.8 months). Follow-up was performed between the ages of 7 months and 6 years (mean age 2.7 years). ERG was detected to flash stimulus and VEP to flash and/or pattern-reversal stimulus.
Results:
In infants less than 1 year of age, retinal dysfunction was recognised in 36% of infants who had been diagnosed as Leber's congenital amaurosis, achromatopsia and retinal dystrophy; postretinal dysfunction was recognised in 32% of infants who had been diagnosed with ocular albinism, optic nerve hypoplasia and neurological nystagmus; retinal or postretinal function was normal in 7% of infants that had been diagnosed as congenital idiopathic nystagmus, while retinal function was normal and postretinal function was undefined in 25% of infants that had been diagnosed as optic nerve hypoplasia, neurological nystagmus and congenital idiopathic nystagmus. At follow-up the findings were: retinal dysfunction in 36%, postretinal dysfunction in 50% and normal retinal and postretinal function in 14% of children. All those children with normal retinal and postretinal function had been diagnosed as congenital idiopathic nystagmus.
Conclusions:
Visual electrophysiological screening of infants with congenital nystagmus can establish or exclude retinal and postretinal pathway dysfunction. Therefore simultaneous skin ERG and VEP which is a non-invasive approach for infants enables an objective means for identifying the basis of congenital nystagmus, thereby assisting in its classification.
