Assessment of night vision problems in patients with congenital stationary night blindness

PLoS One. 2013 May 3;8(5):e62927. doi: 10.1371/journal.pone.0062927. Print 2013.

Abstract

Congenital Stationary Night Blindness (CSNB) is a retinal disorder caused by a signal transmission defect between photoreceptors and bipolar cells. CSNB can be subdivided in CSNB2 (rod signal transmission reduced) and CSNB1 (rod signal transmission absent). The present study is the first in which night vision problems are assessed in CSNB patients in a systematic way, with the purpose of improving rehabilitation for these patients. We assessed the night vision problems of 13 CSNB2 patients and 9 CSNB1 patients by means of a questionnaire on low luminance situations. We furthermore investigated their dark adapted visual functions by the Goldmann Weekers dark adaptation curve, a dark adapted static visual field, and a two-dimensional version of the "Light Lab". In the latter test, a digital image of a living room with objects was projected on a screen. While increasing the luminance of the image, we asked the patients to report on detection and recognition of objects. The questionnaire showed that the CSNB2 patients hardly experienced any night vision problems, while all CSNB1 patients experienced some problems although they generally did not describe them as severe. The three scotopic tests showed minimally to moderately decreased dark adapted visual functions in the CSNB2 patients, with differences between patients. In contrast, the dark adapted visual functions of the CSNB1 patients were more severely affected, but showed almost no differences between patients. The results from the "2D Light Lab" showed that all CSNB1 patients were blind at low intensities (equal to starlight), but quickly regained vision at higher intensities (full moonlight). Just above their dark adapted thresholds both CSNB1 and CSNB2 patients had normal visual fields. From the results we conclude that night vision problems in CSNB, in contrast to what the name suggests, are not conspicuous and generally not disabling.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Case-Control Studies
  • Child
  • Dark Adaptation*
  • Electroretinography
  • Eye Diseases, Hereditary / physiopathology*
  • Female
  • Genetic Diseases, X-Linked / physiopathology*
  • Humans
  • Light
  • Male
  • Middle Aged
  • Myopia / physiopathology*
  • Night Blindness / physiopathology*
  • Night Vision*
  • Pattern Recognition, Visual*
  • Surveys and Questionnaires
  • Visual Acuity*
  • Visual Fields

Supplementary concepts

  • Night blindness, congenital stationary

Grants and funding

This study is partially funded by ODAS Stichting: http://www.odasstichting.nl/. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. No additional external funding was received for this study.