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Updated: Dec 30, 2025
08:38
Transretinal ERG Recordings from Mouse Retina: Rod and Cone Photoresponses
Published on: March 14, 2012
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Analysis of retinal structure and function in cone dystrophy with supernormal rod response.
Ehab Abdelkader1, Z H Yasir2, Abdullah M Khan2
1Ophthalmology Department, Royal Alexandra Hospital, Corsebar Road, Paisley, PA2 9PN, UK. ehaboph@msn.com.
Documenta Ophthalmologica. Advances in Ophthalmology
|January 22, 2020
Summary
Cone dystrophy with supernormal rod response (CDSRR) is characterized by rod dysfunction and reduced flicker response. Supernormal rod-cone electroretinography (ERG) is common, suggesting a specific diagnostic pattern.
Area of Science:
- Ophthalmology
- Genetics
- Electrophysiology
Background:
- Cone dystrophy with supernormal rod response (CDSRR) is a rare inherited retinal disorder.
- Understanding its clinical and electrophysiological features is crucial for diagnosis and management.
Purpose of the Study:
- To characterize the clinical and electrophysiological features of CDSRR.
- To identify genetic variants associated with CDSRR in a cohort of patients.
Main Methods:
- Retrospective cohort study of 15 unrelated patients with CDSRR.
- Clinical examination, full-field electroretinography (ERG), multimodal retinal imaging, and next-generation sequencing for genetic testing.
Main Results:
- All patients exhibited rod dysfunction and prolonged rod b-wave latency.
- A supernormal mixed maximal response b-wave was observed in 63% of patients.
- Pathogenic variants in KCNV2 were identified in all patients, including novel and known mutations.
Conclusions:
- Rod dysfunction and reduced 30-Hz flicker response are consistent findings in CDSRR.
- Supernormal rod-cone ERG is more prevalent than previously reported.
- The combination of delayed rod b-wave and subnormal flicker response strongly suggests CDSRR diagnosis.
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