MT-RNR1 1494C>T is assigned an increased risk of aminoglycoside-induced hearing loss based on strong evidence. Screening of individuals or probands of individuals with aminoglycoside-hearing looss identified 1494C>T in a number of cases (14681830, 16380089, 17085680, 17434445, 17698030, 17698299, 18830133, 19682603, 20100600, 21205314, 27397648, 29876232). LCLs and/or cybrind cell lines with 1494C>T mutation have reduced growth in the presence of aminoglycosides (14681830, 15722487). Paromomycin shows increased binding to rRNA constructs carrying the T allele (19687236). Increased Kd for neomycin binding to A-site constructs carrying the T allele compared to those carrying the C allele (28103015). Cybrid lines with the 1494C>T mutation grown in gentamicin have reduced ATP production and altered mitochondria morphology (25474306). Therefore, consensus among experts was this variant is associated with increased risk of aminoglycoside-induced hearing loss based on strong evidence.