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