Table 1. Details of Phenotype of the 46 patients.

Patient number	Gender	Agea	Pre/Postlingual	Degree of hearing lossb	Evolution of hearing loss	IEM
01	Male	6	Prelingual	Profound	Progressive	EVA
02	Male	7	Prelingual	Profound	Fluctuating	EVA
03	Male	2	Prelingual	Profound	Stable	EVA
04	Male	13	Prelingual	Severe	Fluctuating	EVA
05	Female	10	Prelingual	Severe	Progressive	EVA
06	Female	5	Prelingual	Profound	Fluctuating	EVA
07	Female	6	Prelingual	Severe	Stable	EVA
08	Female	11	Postlingual	Profound	Fluctuating	EVA
09	Female	2	Prelingual	Profound	Stable	EVA
10	Female	5	Prelingual	Profound	Progressive	EVA
11	Female	1	Prelingual	Severe	Stable	EVA
12	Female	15	Postlingual	Severe	Progressive	EVA
13	Female	1	Prelingual	Profound	Stable	EVA
14	Female	2	Prelingual	Profound	Stable	EVA
15	Female	2	Prelingual	Profound	Stable	EVA
16	Female	6	Postlingual	Profound	Progressive	EVA
17	Female	13	Prelingual	Profound	Fluctuating	EVA
18	Female	7	Postlingual	Severe	Fluctuating	EVA
19	Female	6	Prelingual	Profound	Progressive	EVA
20	Female	3	Prelingual	Profound	Stable	EVA
21	Female	2	Prelingual	Severe	Stable	EVA
22	Female	4	Prelingual	Profound	Fluctuating	EVA
23	Female	2	Prelingual	Profound	Stable	EVA
24	Female	1	Prelingual	Profound	Stable	EVA
25	Female	6	Prelingual	Profound	Fluctuating	EVA
26	Female	6	Prelingual	Severe	Progressive	EVA
27	Female	4	Prelingual	Profound	Fluctuating	EVA
28	Female	4	Prelingual	Profound	Stable	EVA
29	Female	4	Prelingual	Profound	Stable	EVA
30	Female	6	Prelingual	Severe	Progressive	EVA
31	Female	10	Prelingual	Profound	Progressive	EVA
32	Female	11	Prelingual	Severe	Fluctuating	EVA
33	Female	1	Prelingual	Profound	Stable	EVA
34	Female	3	Prelingual	Profound	Stable	EVA
35	male	13	Prelingual	Profound	Progressive	EVA
36	male	4	Prelingual	Profound	Stable	EVA
37	Female	15	Prelingual	Severe	Fluctuating	EVA
38	male	10	Prelingual	Profound	Fluctuating	EVA
39	Female	12	Prelingual	Profound	Progressive	EVA
40	male	2	Prelingual	Profound	Stable	EVA
41	male	26	Postlingual	Severe	Fluctuating	EVA
42	Female	4	Prelingual	Profound	Stable	EVA
43	male	5	Prelingual	Profound	Stable	EVA
44	Female	6	Prelingual	Severe	Fluctuating	EVA
45	male	6	Prelingual	Profound	Progressive	EVA
46	male	5	Prelingual	Severe	Stable	EVA

^a Age (in years) at genetic consultation for study inclusion.

^b Profound means hearing threshold consultation foeans hearing threshold 71-90dB, Moderate means hearing threshold 41-55dB, Mild means hearing threshold 26-40dB.

IEM: inner ear malformation