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. 2016 Oct 28;11(10):e0165650. doi: 10.1371/journal.pone.0165650

Fig 3. Patients with characteristic audiograms.

Fig 3

(a) One case (female, 4 years old, onset at birth) with bi-allelic pathogenic variants in GJB2 had a cochlear implant in the left ear and had profound hearing loss in the right ear. (b) One case (male, 3 years old, onset at 1 year old) with bi-allelic pathogenic variants in SLC26A4 had a cochlear implant in the left ear and had severe hearing loss in the right ear. (c)One case (male, 3 years old, onset at 2 yrs) with homoplasmic MT-RNR1 m.1555A > G had a cochlear implant in the left ear and had severe hearing loss in the right ear with a down-sloping shaped audiogram, which is characteristic of aminoglycoside-induced HL.