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

Mieke M C Bijveld, Maria M van Genderen, Frank P Hoeben, Amir A Katzin, Ruth M A van Nispen, Frans C C Riemslag, Astrid M L Kappers

    Research output: Contribution to journalArticleResearchpeer-review

    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.

    Original languageEnglish
    Pages (from-to)e62927
    JournalPLoS ONE
    Volume8
    Issue number5
    DOIs
    Publication statusPublished - 2013

    Keywords

    • 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

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