Sensorineural Hearing Loss

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چکیده

1. Review the patterns of hearing loss in hereditary hearing impairment. AL First, we must understand that genetic hearing loss seems to breach all categories of hearing loss, including the following: congenital, progressive, and adult onset; conductive, sensory, and neural; syndromic and nonsyndromic; high-frequency, low-frequency, or mixed frequency; and mild or profound. Genetic hearing loss may show patterns of recessive, dominant, or sex-linked inheritance and may be a result in mutation of both cellular or mitochondrial DNA (and RNA, in the case of mitochondrial genes). Genetic hearing loss may be subject to environment and aging, such as noiseinduced or age-induced hearing loss. New genetic mutations are linked to hearing loss every year. More than 100 loci have been identified involving genes that code for proteins involved in the structure and function of hair cells, supporting cells, spiral ligament, stria vascularis, basilar membrane, spiral ganglion cells, auditory nerve, and virtually every structural element of the inner ear. Dysfunctional proteins have been identified in the impaired molecular-physiologic processes of potassium and calcium homeostasis, apoptotic signaling, stereocilia linkage, mechanicoelectric transduction, electromotility, and other processes.

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تاریخ انتشار 2012