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Table 1 Summary of subjects with hearing loss

From: Diverse spectrum of rare deafness genes underlies early-childhood hearing loss in Japanese patients: a cross-sectional, multi-center next-generation sequencing study

Family Subject Age at onset (years) Age at the time of the study (years) Hearing loss severity (left/right)* Progression
1 III:3 45 53 Moderate/Moderate Yes
IV:2 10 16 Mild/Normal No
2 III:1 unknown no data Profound/Profound Unknown
III:2 unknown no data Moderate/Severe Unknown
IV:3 0 1 Severe** Yes
3 III:1 0 9 Severe** Unknown
III:2 0 6 Moderate/Moderate Unknown
4 I:2 0 30s Profound/Profound No
II:1 0 2 Profound/Profound No
5 III:1 0 2 Severe** No
III:2 0 2 Profound** No
6 II:1 5 14 Profound/Severe Yes
II:2 0 12 Profound/Profound Yes
7 II:1 0 3 Moderate (ASSR***) Unknown
II:2 0 0 Severe (ASSR) Unknown
  1. *Hearing loss severity was evaluated based on average hearing level at 500, 1,000, 2,000, and 4,000 Hz (mild, 20–40 dB; moderate, 41–70 dB; severe, 71-95dB; profound, >95 dB) according to recommendations [3]. **Binaural hearing level. ***ASSR, auditory steady state responses.