Abstract
The inner ear cytoarchitecture forms one of the most intricate and delicate organs in the human body and is vulnerable to the effects of genetic disorders, aging, and environmental damage. Owing to the inability of the mammalian cochlea to regenerate sensory hair cells, the loss of hair cells is a leading cause of deafness in humans. Millions of individuals worldwide are affected by the emotionally and financially devastating effects of hearing impairment (HI). This paper provides a brief introduction into the key role of genes regulating inner ear development and function. Potential future therapies that leverage on an improved understanding of these molecular pathways are also described in detail.
Highlights
The human ear is a highly complex instrument that is comprised of three main sections: the outer ear, the middle ear, and the inner ear
While many surgical remedies exist for the treatment of hearing loss stemming from dysfunction of the outer and middle ear, few effective remedies have been developed for the treatment of hearing impairment resulting from inner ear disorders
The inner ear is comprised of two main components, the auditory system which receives the amplified mechanical vibrations transmitted from the middle ear and the vestibular system which is responsible for maintaining balance
Summary
The human ear is a highly complex instrument that is comprised of three main sections: the outer ear, the middle ear, and the inner ear. While many surgical remedies exist for the treatment of hearing loss stemming from dysfunction of the outer and middle ear, few effective remedies have been developed for the treatment of hearing impairment resulting from inner ear disorders. The inner ear is comprised of two main components, the auditory system which receives the amplified mechanical vibrations transmitted from the middle ear and the vestibular system which is responsible for maintaining balance. Hair cells located in the organ of Corti generate action potentials in response to perturbations which are transmitted to the auditory cortex via the cochlear nerve [2]. Conductive hearing loss is usually diagnosed as defects in the outer and middle ear that prevent sound from being transmitted to the cochlea while sensorineural hearing loss is usually diagnosed as dysfunction of the inner ear, cochlear nerve, or auditory cortex. Understanding the link between genetics, HI, Genetics Research International hair cells, and the highly differentiated supporting cells surrounding them, remains a prerequisite to developing effective regenerative therapies
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