Suzanne de Bruijn

129 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 III:11 33 N NE, T 38 38 37 40 40 100 95 NA 36-38 W08-1421 III:2 cong. N T 67 42 43 30 40 87 65 1.8 50-67 IV:2 PS N A, Ns, O, T 34 27 28 27 32 95 93 NA 6 V:1 0 R eardrum perforation A, Ab, Ns, O, T 9 73 67 58 60 NT NT NA NA Neonatal intensive care, surgeries, ototoxic antibiotics W18-0470 II:2 6 N 66 42 38 27 27 100 95 NA 0 III:1 30 N O 31 19 18 16 14 100 100 NA 0 III:3 11 NT 23 23 22 15 13 100 100 NA 5 W18-1004 I:1 12 N 54 17 23 NT NT NT NT NA 0 II:1 4 N 20 38 35 30 27 100 95 NA 5 W18-0471 I:1 20 N A, T 47 72 117 64 NA 83 0 NA 0 W18-0472 I:1 27 N T, V 31 35 37 18 22 100 100 NA 0 Migrainous vertigo W18-0473 I:1 39 NT N N 48 35 42 NT NT NT NT NA 0 W18-1005 I:1 25 N N 26 42 45 32 37 100 97 NA 0 W18-1160 I:1 12 N T 50 47 48 42 43 100 100 NA 7 Age of onset (AoO), age of onset in years as reported by the subjects. Subject age, the age at which the audiometric data of column 9 to 14 were obtained, in general the last audiogram. If no speech audiometry was performed during the latest pure tone audiometry, the latest audiogram in which both were measured, was selected. Progression rate of HL, calculated as described in the methods section, if there was at least a follow-up duration of 10 years. Y, years; PTA, pure tone average, mean of 0.5, 1 and 2 kHz air conduction thresholds; R, right; L, left; SRT, speech reception threshold; SRS, speech recognition score in %; YOF, years of follow up; NOHL, unaffected subject; NR, age of onset of HL not reported; PS, subject reported onset of HL during primary school; NT, not tested; N, no abnormalities; T, Tinnitus; NE, extensive exposure to noise; O, recurrent otitis; A, asymmetric HL; Ab, subject reported long-termantibiotics usage, but no details about duration andwhich antibiotics; V, vestibular complaints; NA, not applicable.