Ion channel-related hereditary hearing loss: a narrative review

Abstract

Abstract Sensorineural hearing loss is the most common sensory deficit in humans, with an estimated prevalence of 1 in 500 newborns. Approximately half of childhood hearing loss is attributed to genetic factors and can be classified as syndromic or non-syndromic based on the inheritance pattern. The ion channel genes KCNQ1, KCNE1, KCNQ4, P2RX2, TMC1, KCNJ10, and CACNA1D have frequently been associated with genetic hearing loss. Because of the important roles these genes play in cochlear hair cell function and the auditory pathways, mutations in these genes that result in impaired ion channel function can lead to hereditary hearing loss. The main purpose of this review was to examine the latest research progress on the functional roles, inheritance pattern, gene expression, protein structure, clinical phenotypes, mouse models, and possible treatments of the most commonly studied ion channels associated with inherited deafness. A comprehensive summary could help highlight ion channels that should be investigated as potential drug targets for the treatment of inherited deafness.