|
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
|
- *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.
