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The hearing loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth. [1] Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early adult life. Some children may pass newborn hearing screen to lose hearing in infancy but others present with a hearing loss at birth.
The symptoms of hearing loss in babies and children, however, are slightly different, and can be difficult to catch. For instance, a baby with hearing loss may not startle at loud noises.
Enlarged vestibular aqueducts are commonly picked up after newborn hearing screen when a child is identified as having a hearing loss. The hearing loss is commonly mixed and can be of any degree when first identified. The conductive component is due to a third window effect caused by the widened vestibular aqueduct.
Many infants with CRS may be born with sensorineural deafness and thus should undergo a newborn hearing evaluation. Hearing loss may not be apparent at birth and thus requires close auditory follow up. Infants with confirmed hearing impairment may require hearing aids and may benefit from an early intervention program. [4]
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 ...
In X-linked hearing loss, the mother carries the recessive trait for hearing loss on the sex chromosome. She can pass on the trait to male and female children, but usually only male children are affected. There are some genetic syndromes, in which hearing loss is one of the known characteristics.
People with Pendred syndrome present with a hearing loss either at birth or during childhood. The hearing loss is commonly progressive. In early stages it is usually a mixed hearing loss (both conductive and sensorineural hearing loss) because of a third window effect due to the inner ear malformation (widened vestibular aqueducts).
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.