GJB2gene mutation account for 50% autosomal recessive inheritance pattern and nonsyndromic hearing loss.Previous study considered thatGJB2gene deafness-causing always leads to congenital,bilaterally symmetric, non-progressive and profound hearing loss.Research in recent years indicates thatGJB2gene deafness-causing mutation also leads tolate-onset and progressive hearing loss.This paper reviewed the studies onGJB2-associated late-onset and progressive hearing loss and explored the characteristics of hearing change.
Key words:
GJB2; Gene; Mutation; Hearing