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Other causes of congenital hearing loss that are not hereditary in nature include prenatal infections, illnesses, toxins consumed by the mother during pregnancy or other conditions occurring at the time of birth or shortly thereafter. These conditions typically cause sensorineural hearing loss ranging from mild to profound in degree. [3]
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 ...
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.
Waardenburg syndrome has multiple different types with some variations in symptoms, and symptoms can vary among those with the same type. The two features consistent across all types of Waardenburg syndrome are some degree of congenital sensorineural hearing loss and some degree of pigmentation deficiencies, most consistently in the eyes. [11]
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.
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.
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 ...
Björnstad syndrome is an autosomal recessive congenital condition involving pili torti, sensorineural deafness, and hair abnormalities. [2] It was first characterized in 1965, in Oslo, by prof. Roar Theodor Bjørnstad after he observed an association between pili torti and hearing loss. [3]
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