The TMEM43 gene encodes a transmembrane protein that is associated with connexin-linked function in cochlear glia-like supporting cells (GLSs), and mutations in this gene have been recently associated with hearing loss (HL).

To restore hearing function in patients with hearing loss or deafness, cochlear implantation is seen as the only method that works, but insertion of the electrode array may cause direct mechanical injury to the cochlea, with the subsequent loss attributed to an inflammatory response driven by proinflammatory cytokines, reactive oxygen species and apoptotic signals.

Otoferlin is a calcium sensor protein critical for the transmission of the signal from inner hair cells (IHCs) to the spiral ganglion neurons (SGNs), and it is encoded by the OTOF gene. Pathogenic biallelic loss of function variations in OTOF result in failure of synaptic transmission, causing autosomal recessive deafness 9 (DFNB9), which is a congenital severe-to-profound auditory neuropathy.

Hearing loss related to aging is mostly predicted by the degree of loss of outer hair cells. Investigators from Acousia Therapeutics GmbH have tested the novel Kv7.4 channel activator ACOU-082 in mice.

Otosclerosis affects about 0.3% of population and it is among the most common cause of conductive hearing loss. Otosclerosis is highly familial, with positive family history reported in about 50% to 60% of cases. The disease is characterized by pathologic remodeling of the bone encasing the inner ear (otic capsule).

Decibel Therapeutics Inc. has received clearance of its clinical trial application (CTA) by the U.K.'s Medicines and Healthcare products Regulatory Agency (MHRA) to initiate a phase I/II trial of gene therapy DB-OTO in pediatric patients with congenital hearing loss due to an otoferlin deficiency.

Acousia Therapeutics GmbH, and its partners at the Tübingen Hearing Research Center at University of Tübingen, have generated new preclinical data in support of hearing loss drug candidates ACOU-085 and ACOU-082.

Hyloris Pharmaceuticals SA has announced the development of HY-083, a new proprietary intranasal formulation of a TRPV1 agonist to treat idiopathic rhinitis. Hyloris' treatment approach is to activate and depolarize TRPV1 receptors leading to restoration of a normal function of the nasal mucosa.