To investigate the clinical characteristics of patients with acute zonal occult outer retinopathy (AZOOR).

Medical records of 13 patients who visited Seoul National University Bundang Hospital from May, 2003 to May, 2015 and diagnosed with AZOOR were retrospectively reviewed.

Thirteen patients (11 women and 2 men), with a mean age of 28.5 ± 11.4 years were followed for 42.8 ± 30.3 months. Visual field loss, photopsia, and blurred vision were common complaints. Initially, four patients had bilateral disease and seven patients showed bilateral involvement at the last visit. Mean best corrected visual acuity of involved eyes (BCVA) was 0.75 ± 0.32 (log MAR). Among 20 eyes with AZOOR, BCVA was 20/40 or better in 17 eyes (85.0%). The mean spherical equivalent was -4.59 ± 3.23 diopters (D), and 15 eyes (75.0%) had myopia less than -2.00 D. Nine eyes of seven patients (34.6%) had abnormal fundus findings. All patients underwent full field electroretinogram (ERG) or multifocal ERG and a visual field test. Thirteen patients (100.0%) showed a decreased response in ERG and visual field defects presented in every patient. With respect to the visual field test, 10 eyes (50.0%) showed improvement, 6 eyes (30.0%) had stationary status, and the progression of the visual field defect was observed in 4 eyes (20.0%). Among 13 patients, 4 (30.8%) patients showed flu-like symptom, 4 (30.8%) patients had fatigue, 2 (15.4%) patients had nausea, and 2 (15.4%) patients showed headache.

AZOOR should be considered as one of the differential diagnoses, especially in female patients with myopia who show photopsia or visual field defects. ERG and visual field tests are necessary to confirm a decrease in retinal function and visual field loss. Central vision is preserved in most cases and recovery of visual field defect occurs often.