Research Article
Effects of CoQ10 Replacement Therapy on the Audiological Characteristics of Pediatric Patients with COQ6 Variants
Table 5
Systematic reviews of the audiological responses to oral CoQ10 administration.
| Nucleotide changes | Amino acid substitution | Hearing loss | References | Maintained normal hearing | Improved | Not improved | Newly developed hearing loss | Total |
| c.763G>A, homozygote | p.G255R | 0 | 1 | 0 | 1 | 2 | Heeringa et al. [4] | c.782C>T, homozygote | p.P261L | 1 | 0 | 0 | 0 | 1 | Gigante et al. [18] | c.1058C>A, homozygote | p.A353D | 0 | 0 | 1 | 0 | 1 | Heeringa et al. [4] | 0 | 0 | 2 | 0 | 2 | Perrin et al. [7] | 0 | 0 | 2 | 0 | 2 | Yildirim et al. [19] | c.1078C>T, homozygote | p.R360W | 0 | 0 | 0 | 1 | 1 | Cao et al. [9] | c.1078C>T/c.804delC | p.R360W/p.L269Wfs13 | 1 | 0 | 0 | 0 | 1 | Stańczyk et al. [8] | Total | | 2 | 1 | 5 | 2 | 10 | |
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