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Audiological phenotypes of affected subjects with Usher syndrome type II (USH2) variants. (A) SB237-461, bilateral severe down-sloping sensorineural hearing loss (SNHL). (B) SB354-692, profound down-sloping SNHL on the right ear and severe down-sloping SNHL on the left ear. Red and blue lines represent the right and left ear hearing thresholds, respectively. Numbers with a percentage demonstrate the speech audiometry score in each evaluated ear. SDS, speech discrimination score.
