Table 1.

The contribution of Manf deficiency and Cdh23^ahl mutation to the hearing phenotype in the mouse strains studied.

Mouse	Manf status	Cdh23ahl locus sequence	Cdh23 status	Hearing phenotype (P56)
C57BL/6J wild type	Manf +/+		Cdh23753G→A	Normal
C57BL/6J Manf cKO	Manf fl/fl; Pax2-cre		Cdh23753G→A	Severe impairment
CBA/Ca wild type	Manf +/+		Cdh23753G	Normal
CBA/Ca Manf KO	Manf −/−		Cdh23753G	Normal
CD-1 wild type	Manf +/+		Cdh23753G→A	Severe impairment
CBAxCD-1 F2 Manf KO	Manf −/−		Cdh23753G→A	Severe impairment (see also Herranen et al [2020])
CBAxCD-1 F2 Manf KO	Manf −/−		Heterozygous for: Cdh23753G→A	Normal (see also Herranen et al [2020])

Each row presents one representative mouse together with its background strain, Manf genotype, a snippet of the sequencing chromatogram containing the Cdh23^753G➔A point mutation locus, Cdh23 genotype, and a basic hearing status at P56. ABRs and outer hair cell loss of CBA x CD-1 hybrid (F2) mice were presented in Herranen et al (2020). Manf deficiency and the homozygous Cdh23^ahl mutation are together required for the severe sensorineural hearing loss phenotype (= highly elevated ABR thresholds at all frequencies). Abbreviations: ABR, auditory brainstem response; cKO, conditional knock out; F2, F2 generation; Het, heterozygote; KO, knock out.