If mitochondrial mishandling of nucleic acid/protein complexes triggers innate immune activation and if the inflammatory process in the nervous tissue is responsible for deafness, ataxia, and leukodystrophy in the later course of Perrault syndrome, then the therapeutic injection of antisense oligonucleotides to deplete STING as an integrator of inflammatory signaling might have neuroprotective value. Here, STING1 is linked to cerebellar ataxia.