Table 2.

Genes with mutations that cause progressive hearing loss in mice

Gene	Mutation	Proposed Gene Function	Reference
Atp1a1, Atp1a2, Slc12a2	targeted knockout (haploinsufficiency)	endocohlear potential, potassium homeostasis	(Diaz et al., 2007)
Barhl1	targeted knockout	maintenance of hair cells	(Li et al., 2002)
Cdh23	straiin variant	hair bundle integrity, tip link	(Noben-Trauth et al., 2003)
Cdh23, Pcdh15	v and av double heterozygotes	hair bundle integrity, tip link	(Zheng et al., 2005)
Cdkn2d	targeted knockout	hair cell re-entry into cell cycle	(Chen et al., 2003)
Chrnb2	targeted knockout	spiral ganglion cell maintenance	(Bao et al., 2005)
Coch	targeted knock-in	extracellular matrix?	(Robertson et al., 2008)
Col9a1	targeted knockout	integrity of tectorial membrane	(Suzuki et al., 2005)
Fbxo2, Skp1	targeted knockout	protein quality control in cochlea	(Nelson et al., 2007)
Gabr-a5,b2,b3	targeted knockout	hair cell and neuron maintenance	(Maison et al., 2006)
Kcnma1	targeted knockout	regulate OHC depolarization	(Ruttiger et al., 2004)
Kcnq4	targeted knockout	regulate OHC depolarization	(Kharkovets et al., 2006)
mt-Tr (mtDNA)	strain variant	energy metabolism, oxidative stress	(Johnson et al., 2001)
Nfrkb	targeted knockout	survival of spiral ganglion cells	(Lang et al., 2006)
Ngfr	targeted hypomorph	survival of spiral ganglion cells	(Sato et al., 2006)
Otos	targeted knockout	fibrocyte integrity	(Delprat et al., 2005)
Polg	targeted knock-in	accumulation of mtDNA mutations, oxidative stress	(Kujoth et al., 2005)
Smad5	targeted knockout (haploinsufficiency)	hair cell apoptosis	(Yang et al., 2009)
Sod1	targeted knockout	oxidative stress	(McFadden et al., 1999)
Vasp	targeted knockout	pillar cells actin cytoskeleton?	(Schick et al., 2004)