2021 Volume 31 Issue 2 Pages 137-141
Among patients with idiopathic bilateral sensorineural hearing loss, seven causative genes that develop late-onset hearing loss under the age of 40 were identified and defined as juvenile onset bilateral sensorineural hearing loss; this hearing loss is clearly different from age-related hearing loss. The genes are ACTG1, CDH23, COCH, KCNQ4, TECTA, TEMPRSS3, and WFS1. If the patients have severe to profound hearing loss (70 dB or more), they can be extrapolated as intractable diseases. For patients with juvenile onset bilateral sensorineural hearing loss, genetic testing with next-generation sequencers and genetic counseling are accompanied with autonomous options ranging from hearing aids to hearing implants, which has become very beneficial.
