| Title | Colour Vision in Stargardt Disease | 
| Purpose | To investigate the type and severity of colour vision deficiencies (CVDs) in Stargardt disease (STD). And, to establish how the degree of CVD relates to best-corrected visual acuity (BCVA), full field ERG (ffERG) and duration of disease. | 
| Methods | A retrospective, cross-sectional study of 97 patients with a clinical diagnosis of STD included a comprehensive medical history and a full clinical work-up, with extensive colour vision testing. Eight patients underwent anomaloscopy. ABCA4 was screened in 92 patients. | 
| Results | Patients were allocated to 5 BCVA groups and to 3 ffERG groups. Normal colour vision was found in almost 30% of patients. R/G CVDs increased as BCVA declined. More than 50% had a deutan type R/G CVD, although protan R/G CVDs became progressively apparent as BCVA decreased. A predominance of pseudoprotanomaly was evident only on anomaloscopy. Additional Blue/Yellow (B/Y) CVDs were noted in 25% of patients. B/Y CVDs and BCVA higher than 0.75 were seen in adult-onset STD. CVDs evolve to scotopization in patients with low BCVA and/or longstanding disease. Duration of disease did not correlate well with CVDs. Also, no statistically significant differences in ERG results were found between groups with or without a CVD. | 
| Conclusion | Since colour vision function is better correlated to BCVA than either disease duration or ffERG, it is a rather reliable indicator of disease severity. The presence of CVDs may help to establish an early diagnosis of STD. | 
| Last name | VANDENBROUCKE | 
| Initials | T | 
| Department | Dept Ophthalmol, Ghent University Hospital | 
| City | Ghent | 
| Last name | Buyl | 
| Initials | R | 
| Department | Dept Biostatistics, Vrije Universiteit Brussel | 
| City | Brussels | 
| Last name | De Zaeytijd | 
| Initials | J | 
| Department | Dept Ophthalmol, Ghent University Hospital | 
| City | Ghent | 
| Last name | Uvijls | 
| Initials | A | 
| Department | Dept Ophthalmol, Ghent University Hospital | 
| City | Ghent | 
| Last name | De Baere | 
| Initials | E | 
| Department | Ctr Medical Genetics, Ghent University Hospital | 
| City | Ghent | 
| Last name | Leroy | 
| Initials | BP | 
| Department | Dept Ophthalmol, Ghent University Hospital | 
| City | Ghent |