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The Rate of Progression of <jats:italic>USH2A<\/jats:italic>\u2010related Retinal Degeneration (RUSH2A) is a natural history study that enrolled 127 participants, 80 with USH2 and 47 with ARRP. Hearing was measured by pure\u2010tone thresholds and word recognition scores, and olfaction by the University of Pennsylvania Smell Identification Test (UPSIT). SNHL was moderate in 72% of USH2 participants and severe or profound in 25%, while 9% of ARRP participants had moderate adult\u2010onset SNHL. Pure\u2010tone thresholds worsened with age in ARRP but not in USH2 participants. The degree of SNHL was not associated with other participant characteristics in either USH2 or ARRP. Median pure\u2010tone thresholds in ARRP participants were significantly higher than the normative population (<jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001). Among 14 USH2 participants reporting newborn hearing screening results, 7 reported passing. Among RUSH2A participants, 7% had mild microsmia and 5% had moderate or severe microsmia. Their mean (\u00b1<jats:italic>SD<\/jats:italic>) UPSIT score was 35 (\u00b13), similar to healthy controls (34 [\u00b13]; <jats:italic>p<\/jats:italic>\u00a0=\u20090.39). Olfaction differed by country (<jats:italic>p<\/jats:italic>\u00a0=\u20090.02), but was not significantly associated with clinical diagnosis, age, gender, race\/ethnicity, smoking status, visual measures, or hearing. Hearing loss in <jats:italic>USH2A<\/jats:italic>\u2010related USH2 did not progress with age. ARRP patients had higher pure\u2010tone thresholds than normal. Newborn hearing screening did not identify all <jats:italic>USH2A<\/jats:italic>\u2010related hearing loss. Olfaction was not significantly worse than normal in participants with <jats:italic>USH2A\u2010<\/jats:italic>related retinal degeneration.<\/jats:p>","DOI":"10.1002\/ajmg.a.62437","type":"journal-article","created":{"date-parts":[[2021,7,31]],"date-time":"2021-07-31T04:59:41Z","timestamp":1627707581000},"page":"3717-3727","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Auditory and olfactory findings in patients with <scp><i>USH2A<\/i><\/scp>\u2010related retinal degeneration\u2014Findings at baseline from the rate of progression in <scp><i>USH2A<\/i><\/scp>\u2010related retinal degeneration natural history study (<scp>RUSH2A<\/scp>)"],"prefix":"10.1002","volume":"185","author":[{"given":"Alessandro","family":"Iannaccone","sequence":"first","affiliation":[{"name":"Duke Eye Center, Department of Ophthalmology Duke University Medical School  Durham North Carolina 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