Table 1 Genes associated with ARHL

GRHL2

Deafness-causing single gene

The protein encoded by this gene is a transcription factor associated with deafness; associated with ARHL in European populations

[35]

Slc4a10

Mutant mice have elevated ABR thresholds; cochlear OHC loss is evident

[36]

SLC9A3R1

Hearing impairment in mutant zebrafish larvae

[39]

KCNQ4

May have effects on DFNA5

[40]

Ahl

Deletion of this gene results in disordered cochlear HCs, raising the ABR threshold

[42, 43]

GJB2

This gene encodes Cx26, and deficiency of Cx26 impairs active cochlear amplification

[44]

A430005L14Rik

Homozygous mutant mice exhibit asynchrony in auditory nerve firing during noise

[45]

Dclk1

Homozygous mutant mice had significantly higher hearing thresholds

[45]

GRM7

Neurotransmitter-related genes

The gene is able to increase the release of glutamate, bringing it to a toxic state that increases the risk of ARHL

[49]

NAT2

Oxidative stress-related genes

This gene is involved in the metabolism and detoxification of ROS, and the polymorphism of NAT2*6A is associated with ARHL in the population

[51]

GSTT1

GSTT1 null genotype was significantly associated with ARHL

[52]

SOD2 promoter variant (−38C > G)

May be associated with male ARHL

[53]

UCP2Ala55Val

UCP2 is able to control ROS, and the UCP2Ala55Val polymorphism was significantly associated with ARHL in the Japanese population

[55]

mtDNA 4977-bp

Mitochondrial function-related genes

Deletion of this gene may be associated with ARHL

[56]

mtDNA haplogroup A

Associated with increased hearing loss in Japanese men

[58]

mtDNA haplogroup N9

Associated with decreased hearing loss in Japanese women

[58]