Suzanne de Bruijn

126 Chapter 3.1 Table S3. Individual results of otoscopic examination, audiometry, imaging and progression of HL 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 W97-056 II:2 48 NT 81 72 63 70 72 87 65 0.6 53-82 II:4 43 NT Ab, NE, T 72 93 90 NT NT NT NT NA 63-72 II:5 70 NT NE 79 63 52 NT NT NT NT NA 0 Professional noise exposure III:10 21 NT NE 41 47 48 61 70 95 95 0.5 21-56 III.11 26 NT O, T 54 43 38 NT NT NT NT NA 0 III:12 20 NT T 41 40 43 43 42 90 95 NA 32-40 III:15 37 N NE,T 67 50 48 57 51 92 75 1.0 46-67 III:18 36 NT T 62 50 51 50 NT 95 90 0.9 41-62 III:19 14 N A 42 NA 73 NA NA NA 41 1.4 21-42 R ear profoundly deaf III:22 20 NT T 62 75 80 70 70 75 88 0.8 42-62 III:24 33 N 43 62 60 63 67 93 93 1.8 36-48 III:26 34 N O,T 45 50 52 NT NT NT NT NA 0 III:28 NOHL NT 52 7 10 NT NT NT NT NA 47-51 III:30 NR N A 64 53 35 42 25 100 100 1.2 48-64 Otosclerosis, infrequent balance complaints IV:20 7 N T 50 70 63 62 52 88 90 0.9 7-51 IV:22 NR N T 27 20 25 NT NT NT NT NA 0 IV:23 NR NT 36 7 8 NT NT NT NT 0.5 25-35 IV:25 24 NT 42 25 18 18 22 NT NT 0.4 22-42