A Short Note on Hearing loss

Hearing loss is relatively common in the human population. Profound congenital hearing loss is estimated to occur in about 1 in 1000 births [1,2]. Early intervention has been shown to be effective in facilitating speech and language development in deaf and hard-of-hearing children [3,4]. Ninety-five percent of newborns with hearing loss identified by newborn hearing screening programs are born to hearing parents, obscuring the fact that the majority of newborns have a hereditary cause for their hearing loss. The majority of genetic hearing loss is inherited in an autosomal recessive pattern and often presents in the absence of a positive family history for hearing loss [5]. One gene, GJB2, which encodes the gap junction protein connexin 26, accounts for the largest proportion of autosomal recessive early childhood hearing loss in many populations [6-9]. If a specific etiology is known, descriptions of hearing loss may also include the etiologic diagnosis, such as Usher syndrome type 1–related hearing loss or GJB2-related hearing loss.

Nonsyndromic hearing loss may be transmitted as an autosomal recessive (~80%), autosomal dominant (~15%), or X-linked trait (~1%) [11]. More than 150 deafness-causing variants have been identified in GJB2, but a few common mutations account for a large percentage of alleles in several populations. GJB2-related hearing loss is sensorineural, usually present at birth, typically bilateral and nonprogressive, and can range from mild to profound in severity. Age-related hearing loss, or presbycusis, is a common neurosensory deficit. Presbycusis generally affects higher frequencies of sound disproportionately, making it difficult for those with presbycusis to understand speech. Presbycusis is a complex condition influenced by genetic and environmental factors. Certain environmental (nongenetic) factors play a major etiologic role in hearing loss. Congenital CMV infection is the most common nongenetic cause of hearing loss among children.

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Lisa M

Journal manager

Journal of phonetics and audiology

E-mail: jpay@scholarlypub.com