Suzanne de Bruijn

131 A RIPOR2 deletion is a frequent cause of adult-onset hearing loss Table S5. Individual results of age of onset, otoscopy, audiometry and progression of HL of the phenocopies 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 (%) Progression rate (dB/y) YOF (y) R L R L R L W97-056 III:14 52 N V 69 42 40 48 42 85 92 0.8 39-70 Ménière-like phenotype III:20 46 N Ab, T 70 45 40 37 37 100 100 0.7 50-71 Smoking, COPD Gold III, often antibiotics III:21 NR NT 68 38 37 20 25 NT NT 0.8 45-68 W04-262 III:10 55 N T, V 60 33 38 33 41 100 95 NA 0 Family members with HL that do not carry the RIPOR2 variant are considered a phenocopy. Age of onset (AoO) is the age of onset in years as reported by the subjects. Subject age is the age at which the audiometric data of column 9 to 14 were obtained. If no speech audiometry was performed during the latest audiometric testing, the penultimate audiogram was selected. Progression rate of HL was calculated 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; NR, age of onset of HL not reported; NT, not tested; N, no abnormalities; V, vestibular complaints; Ab, subject reported long-term antibiotics usage, but no details about duration and which antibiotics; T, Tinnitus; NA, not applicable.