Suzanne de Bruijn

127 A RIPOR2 deletion is a frequent cause of adult-onset hearing loss Table S3. Continued Family Subject Age of onset (y) Otoscopic examination Clinical remarks Imaging Audiometry Progression of HL General remarks CT MRI Subject age (y) PTA SRT Maximum SRS (%) Progres- sion rate (dB/y) YOF (y) R L R L R L IV:26 NOHL NT 41 7 7 NT NT NT NT NA NA IV:27 35 N 47 17 18 12 18 100 100 0.3 26-47 IV:28 30 N A, NE, T N N 45 32 52 37 55 100 92 1.8 25-46 Professional noise exposure IV:29 17 N NE 26 5 10 NT NT NT NT NA 22-26 IV:35 30 N NE, T 37 15 15 10 10 100 100 NA 0 Recreational noise exposure IV:38 8 N T 31 8 7 8 7 100 100 NA NA V:1 5 N NE, T 25 40 37 28 27 100 97 1.3 8-25 V:2 NOHL N 23 3 5 <10 <10 100 100 NA NA W02-016 II:3 29 NT T 82 68 68 65 70 100 93 0.9 82-92 II:6 47 N 89 80 83 80 80 52 67 2 76-86 II:9 40 N 73 NA 82 67 72 75 55 NA 69-73 R ear profoundly deaf above 2 kHz III:1 41 N A, V 73 40 67 35 77 88 62 NA 66-73 Infrequent vertigo attacks since the age of 65 years III:5 NR N 65 20 27 18 17 95 96 NA 0 III:9 35 N O, A, T, V N 39 48 42 47 38 80 100 3 24-47 Balance complaints after CI surgery III:10 32 N T 32 15 20 10 15 100 95 1.4 32-60 IV:1 53 NT V 42 12 10 NT NT NT NT NA 0 Benign paroxymal positional vertigo