Inner ear sensory hair cells and auditory neurons (IHC/OHC and SGN)

Inner ear sensory hair cells and auditory neurons are the fundamental cellular units of the mammalian auditory system located within the cochlea. Sensory hair cells, categorized into inner hair cells (IHCs) and outer hair cells (OHCs), are responsible for mechanotransduction, converting mechanical sound vibrations into electrochemical signals (NIH, 2023). Auditory neurons, specifically spiral ganglion neurons (SGNs), receive these signals and transmit them to the central nervous system via the auditory nerve. Damage or loss of these cells due to genetic mutations, noise trauma, aging, or ototoxic medications like cisplatin is the primary cause of permanent sensorineural hearing loss, as these cells lack the innate capacity for regeneration in humans (StatPearls, 2023). Current therapeutic research focuses on regenerative strategies, such as Notch signaling inhibition to trigger progenitor cell differentiation, and gene therapies designed to replace defective proteins like otoferlin (PubMed, 2022). Additionally, pharmacological efforts aim to protect these cells from apoptosis induced by aminoglycoside antibiotics or chemotherapeutic agents.

Other names

Cochlear hair cellsOrgan of Corti cellsSpiral ganglion neuronsAuditory nerve fibersAcoustic neurons

Notch signaling inhibition for progenitor cell activation, AAV-mediated gene replacement for protein deficiency, and otoprotection via antioxidant or anti-apoptotic pathways.