Table 1. Primary cohort characteristics (n = 49 probands).

	Non-syndromic group		Syndromic group
Probands	All (N = 32)	Probands with PV or VUS (N = 10)	All (N = 17)	Probands with PV or VUS (N = 9)
Female	17	5	9	5
Male	15	5	8	4
Family history
Familial	10	5	4	2
Sporadic	22	5	13	7
Level of hearing loss
Mild	2	0	2	2
Moderate	2	2	5	2
Moderate-Severe	10	3	5	3
Severe	9	2	1	1
Profound	9	3	4	1

PV: pathogenic or likely pathogenic variant; VUS: variant of uncertain significance.