Novel Therapeutic Interventions in Hereditary Hearing Loss

Sensorineural hearing loss is the most common defect in humans that is very heterogeneous and genetic factors, environmental or both are involved in its development. More than 50 percent of the hearing loss causes have been attributed to genetic factors. Several studies have shown ongoing negative impact of hearing loss on communication and quality of life. So the attempt to optimize performance and maintain or improve hearing seems necessary. In this regard, after hearing assessment interventions are done. These interventions can be behavioral which include genetic screening and then doing a series of do's and donts in daily life or some hearing devices such as hearing aid or cochlear implant, depending on the individual's disorder pathophysiology could be used. Other interventions in this field are done by medications such as glucocorticoid steroids and antioxidants which a number of them are in the stage of clinical trials now. Currently, the most important intervention is genetherapy. Genetherapy is a promising treatment for many inherited disorders being investigated. Genetherapy is capable to restore hearing by overcome functional deficits caused by genetic mutations. Moreover, genetherapy can potentially be used to induce the regeneration of hair cells by transferring genes which are essential for differentiation of hair cells in the cochlea. In this context, successful testing have done on following genes GJB2, VGLUT3, SLC26A4, TMC1, USH1C.