Wolfram Sendromlu Hastalarda Optik Koherans Tomografi Bulguları

Objectives: To report the optical coherence tomography (OCT) findings in patients with Wolfram syndrome.\udMaterials and Methods: Four patients who fulfilled the criteria for Wolfram syndrome were recruited to the study. In all patients,\udOCT was performed with Stratus OCT (OCT-3, Carl Zeiss Meditec, Inc. Germany). The fast retinal nerve fiber layer (RNFL) and fast\udmacular thickness protocols were used to measure the RNFL and macular thickness, respectively. The fast optic disc protocol was used to\uddetermine the cup-to-disc ratios of the optic disc. All patients were examined with VEP (Retimax, CSO Strumenti Oftalmici, Florence,\udItaly).\udResults: In eight eyes of four patients (3 male and 1 female) with a mean age of 18.5±2.08 years (range 16-21 years), RNFL, macular\udthickness, and cup-to-disc ratios were determined. The mean RNFL was 42.2±5.6 µm (range 34.1-49.5 µm), while the mean macular\udthickness and cup-to-disc ratios were 145±15 µm (range 125-160 µm) and 0.79±0.07 (range 0.7-0.92), respectively. There was a\udmoderate negative correlation between VEP latencies and macular and RNFL thicknesses (Spearman correlation coefficient was -0.23\udand -0.34, respectively).\udConclusions: RNFL loss and secondary optical atrophy are severe complications that may affect the visual acuity in patients with\udWolfram syndrome. Retinal changes in these patients may be quantified and can be observed using OCT. (Turk J Ophthalmol 2014;\ud44: 212-5