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The audiograms revealed that in the first brother a mild to moderate SNHL was present since birth, and the auditory threshold was reduced by 50 dB in the high-frequency range at the age of 19 years. The second brother had a severe SNHL since birth, and the auditory threshold was reduced by 100 dB in the high-frequency range. Both brothers had a tinnitus from early childhood. With age, both brothers developed a mild hyperacusis. Otitis media was observed from early childhood. Both brothers have a monozygotic twin sister who did not have hearing loss and showed normal auditory thresholds. The audiometric pattern was different between the brothers. The frequency of the first brother was higher than that of the second. 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