Abstract
Neurosensory responses of hearing and balance are mediated by receptors in specialized neuroepithelial sensory cells. Any disruption of the biochemical and molecular pathways that facilitate these responses can result in severe deficits, including hearing loss and vestibular dysfunction. Hearing is affected by both environmental and genetic factors, with impairment of auditory function being the most common neurosensory disorder affecting 1 in 500 newborns, as well as having an impact on the majority of elderly population. Damage to auditory sensory cells is not reversible, and if sufficient damage and cell death have taken place, the resultant deficit may lead to permanent deafness. Cochlear implants are considered to be one of the most successful and consistent treatments for deaf patients, but only offer limited recovery at the expense of loss of residual hearing. Recently there has been an increased interest in the auditory research community to explore the regeneration of mammalian auditory hair cells and restoration of their function. In this review article, we examine a variety of recent therapies, including genetic, stem cell and molecular therapies as well as discussing progress being made in genome editing strategies as applied to the restoration of hearing function.
Highlights
Hearing loss is a serious global morbidity that affects 360 million people worldwide (WHO, 2013)
The results of this study have demonstrated that this therapy can rescue the inner ear structure and function against damage caused by exposure to an ototoxic level of gentamicin (Suzuki et al, 2000)
The results of this study suggest that brain derived neurotrophic factor (BDNF) exerts protective effects on spiral ganglions in response to cochlear insults such as noise leading to rescue of hearing
Summary
Hearing loss is a serious global morbidity that affects 360 million people worldwide (WHO, 2013). The organ of Corti converts the mechanical energy of sound waves into electrical signals that are transduced to the auditory pathway in the brain (Kazmierczak and Muller, 2012) It does so through the use of hair cells, which contain stereocilia that move in response to specific soundwave initiated fluid waves (Goutman et al, 2015; Figure 1B). In addition to the essential elements of the conduction pathway, the tensor tympani and the stapedius muscle are responsible for damping the movement of the middle ear bones in response to high levels of sound The contraction of these muscles is vital for preventing mechanical damage and stimulus-induced hearing loss.
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