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. 2022 Oct 5;16:998507. doi: 10.3389/fnins.2022.998507

TABLE 1.

Mitochondrial dynamics dysfunction induced hearing loss.

Research model Target gene Disease Hearing loss type Fusion/Fission Hearing loss/Mitochondrial mechanism References
Patients with ADOA disease OPA1 Autosomal dominant optic atrophy Auditory neuropathy Pro-fusion Neuronal mitochondria fragmented Ham et al., 2019
Patients with CMT disease MFN2 Charcot-Marie-Tooth disease Auditory neuropathy Pro-fusion Unmyelinated auditory nerve dysfunction Choi et al., 2020
C57BL/6J mice Drp1 Age related hearing loss Age related hearing loss Pro-fission Inhibit Drp1, promote the progression of presbycusis Lin et al., 2019
Fibroblasts from FRDA patient FXN Friedreich’s ataxia Auditory neuropathy Pro-fusion Iron accumulation and oxidative damage in mitochondria Rojas et al., 2021
Gene knockout mice WFS1 Wolfram syndrome Auditory neuropathy Pro-fusion Upregulation mitochondrial dysfunction genes Cagalinec et al., 2016