ROC curve analysis demonstrated that the residual EZ area at initial diagnosis could predict the degree of BCVA deterioration within 2 years (area under the curve [AUC] = 0.70).<h4>Conclusions</h4>Our findings indicate that the foveal sparing phenotype is associated with better visual prognosis and is more frequently observed in IRDs associated with EYS and USH2A mutations. Here, USH2A is linked to respiratory distress syndrome in premature infants.