Table 1.

Gene-specific reports of gene therapy in mouse models of deafness

Gene	Gene therapy method	Genotype	Age at intervention	Length of follow up	Age at best ABR results	Click-evoked best ABR results	Tone-burst best ABR results				Reference
							8kHz	16kHz	24kHz	32kHz
Tmc1	GR	Homo	P0–2	P84–90	P28–30	NR	65	65	85	100	Nist-Lund et al., 2019 (7)
		Homo	P0–2	P60	P25–30	NR	85	100	120	120	Askew et al., 2015 (8)
	RNAi	Hetero	P15–16	P140–146	P56–62	40	50	85	NR	90	Yoshimura et al., 2019 (9)
		Hetero	P0–2	P245–251	P28	40	20	30	NR	90	Shibata et al., 2016 (10)
	CRISPR/Cas9	Hetero	P1	P28–34	P28–34	NR	55	60	65	80	Gao et al., 2018 (11)
Clrn1	GR	Homo	P1	P28–34	P28–34	NR	85	90	95	95	Gyorgy et al., 2019 (12)
		Homo	P1–3	P60	P22–24	NR	30	30	35	50	Dulon et al., 2018 (13)
		Homo	P1–3	P70	P22	45	35	35	NR	40	Geng et al., 2017 (14)
Ush1c c.216G>A	GR	Homo	P1	P168–174	P42–48	NR	50	50	80	95	Pan et al., 2017 (15)
	ASOs	Homo	P5	P84–90	P28–34	45	45	45	NR	70	Lentz et al., 2013 (16)
Whrn	GR	Homo	P1–5	P90	P30–90	NR	95	95	NR	95	Chien et al., 2016 (17)
		Homo	P1–5	P120	P30	NR	60	80	NR	80	Isgrig et al., 2017 (18)
Otof	GR	Homo	P10	P210–216	P28–34	35	65	65	NR	60	Akil et al., 2019 (19)
		Homo	P6–7	P30	P23–30	50	70	90	90	90	Al-Moyed et al., 2019 (20)
Lhfpl5	GR	Homo	P0–1	P28–34	P28–29	NR	70	90	85	100	Gyorgy et al., 2017 (21)
MsrB3	GR	Homo	E12.5	P56–62	P28	25	25	30	40	40	Kim et al., 2016 (22)
Kcnq1	GR	Homo	P0–2	P210–216	NR	45	55	65	75	80	Chang et al., 2015 (23)
Gjb2	GR	Homo	P0	P84–90	P70–84	NR	NR	NR	65	NR	Iizuka et al., 2015 (24)
		Homo	P0–1	P28–34	P28–34	NR	85	85	85	85	Yu et al., 2014 (25)
Vglut3	GR	Homo	P1	P483–489	P50–52	40	65	55	NR	65	Akil et al., 2012 (26)

ABR, auditory brainstem response; GR; gene replacement; NR, not recorded/no report.