Table 1.

Phenotypic comparisons of models of mitochondrial neurosensory hearing loss

Mouse Model	mtDNA mutation PolgD257A/D257A	oxidative stress MCAT TG	oxidative stress Gpx1−/−	apoptosis Bak−/−	apoptosis Bcl-2 TG
Hearing	↓	↑	↓	↑	na
Sensory Hair cells	↓	↑	↓	↑	↑
SG neurons	↓	↑	na	↑	na
Life span	↓	↑	na	na	na
Oxidative Damage	na	↓	na	↓	na
Apoptosis	↑	na	na	↓	↓
Reference	Kujoth et al., 2005 Niu et al., 2007 Someya et al., 2008	Schriner et al., 2005 Someya et al., 2009	Cheng et al., 1997 Ohlemiller et al., 2000	Lindsten et al., 2000 Someya et al., 2009	Martinou et al., 1994 Cunningham et al., 2004

na: no available data.

↑: indicates elevated levels compared to age-matched WT.

↓: indicates reduced levels compared to age-matched WT.