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Congenital hearing loss is a hearing loss present at birth. It can include hereditary hearing loss or hearing loss due to other factors present either in-utero (prenatal) or at the time of birth. It can include hereditary hearing loss or hearing loss due to other factors present either in-utero (prenatal) or at the time of birth.
Prelingual hearing loss can be considered congenital, present at birth, or acquired, occurring after birth before the age of one. Congenital hearing loss can be a result of maternal factors (rubella, cytomegalovirus, or herpes simplex virus, syphilis, diabetes), infections, toxicity (pharmaceutical drugs, alcohol, other drugs), asphyxia, trauma, low birth weight, prematurity, jaundice, and ...
Hearing loss in neonates is the most common congenital birth defect and sensory disorder, and can be caused by a variety of reasons. Research has placed the prevalence of significant permanent hearing loss in neonates at 1–2 per 1000 live births in the United States. [19] [20] With this screening, many forms of congenital hearing loss can be ...
Hearing loss is a partial or total inability to hear. [5] Hearing loss may be present at birth or acquired at any time afterwards. [6] [7] Hearing loss may occur in one or both ears. [2] In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. [8]
Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Nonsyndromic deafness constitutes 75% of all hearing loss cases, and an estimated 100 genes are thought to be linked to this condition.
Around 15-20% of newborns with Down syndrome are also diagnosed with some degree of congenital hearing loss. [13] Children with Down syndrome commonly have glue ear, which can lead to infections in the ear, and may cause hearing loss. [14] Glue ear is a condition in which the middle part of the ear behind the eardrum fills with fluid. [15]
They are used for congenital hearing loss when changes to the shape of the inner ear or nerve of hearing may help diagnosis of the cause of the hearing loss. They are also useful in cases where a tumour is suspected or to determine the degree of damage in a hearing loss caused by bacterial infection or auto-immune disease.
The most common type of congenital hearing loss in developed countries is DFNB1, also known as connexin 26 deafness or GJB2-related deafness. The most common dominant syndromic forms of hearing loss include Stickler syndrome and Waardenburg syndrome. The most common recessive syndromic forms of hearing loss are Pendred syndrome and Usher syndrome.