Table 1.

Patient characteristics

Characteristic	TMPRSS3-Group, N = 33 ears (100%)	Control-group, N = 67 ears (100%)	P value
Gender, % female	15 (45.5)	43 (64.2)	0.074
Age at implantation (mean ± SD)	24 ± 19	27 ± 26	0.584
Duration of hearing loss prior to implantation (mean ± SD)	16 ± 14	17 ± 18	0.908
Learning difficulties	1 (3.0)		0.165
EVA on CT or MRI	0 (0.0)	15 (22.4)	0.045
Affected gene
ACTB	0 (0.0) (0 (0.0).0 (0.0))	1 (1.5)
ACTG1	0 (0.0) (0 (0.0).0 (0.0))	1 (1.5)
ADGRV1	0 (0.0)	1 (1.5)
CEP95	0 (0.0)	1 (1.5)
CLRN1	0 (0.0)	3 (4.5)
COCH	0 (0.0)	10 (14.9)
GJB2	0 (0.0)	8 (11.9)
GJB6	0 (0.0)	1 (1.5)
LARS2	0 (0.0)	1 (1.5)
MITF	0 (0.0)	2 (3.0)
MITO	0 (0.0)	1 (1.5)
MYO15A	0 (0.0)	5(7.5)
MYO7A	0 (0.0)	4 (6.0)
POU4F3	0 (0.0)	1 (1.5)
PRPS1	0 (0.0)	1 (1.5)
PTPN11	0 (0.0)	1 (1.5)
SLC26A4	0 (0.0)	15 (22.4)
SOX10	0 (0.0)	1 (1.5)
TMPRSS3	33 (100)	0(0.0)
TPRN	0 (0.0)	2 (3.0)
TUBB4B	0 (0.0)	2 (2.5)
USH2A	0 (0.0)	3 (4.5)
WFS1	0 (0.0)	2 (3.0)
CI side
Left	15 (45.5)	30 (44.8)	0.173
Right	14 (42.4)	35(52.2)
Bilateral (simultaneously)	4 (21.1)	2 (3.0)
Hearing aid in ear to be implanted	21 (63.6)	46 (68.7)	0.616
Surgical technique
Cochleostomy	15 (45.5)	54 (80.6)	0.002
Round window	16 (48.5)	13 (19.4)
Extended round window	1 (3.0)	0 (0.0)
Not reported	1 (3.0)	0 (0.0)

SD standard deviation, EVA enlarged vestibular aqueduct, CT computer tomography, MRI magnetic resonance imaging, CI cochlear implant