Indeed, this paradox has been reported in motor neuron degeneration (such as SOD1 mutant rodent models of ALS) (Brahimi et al., 2016), in the death of retinal ganglion cell neurons in a model of RP (Galán et al., 2017b), and may be germane to the death of spiral ganglion neurons in a noise-induced hearing loss model (NIHL). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.