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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]
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.
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]
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.
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.
Profound unilateral hearing loss is a specific type of hearing loss when one ear has no functional hearing ability (91 dB or greater hearing loss). People with profound unilateral hearing loss can only hear in monaural (mono). Profound unilateral hearing loss or single-sided deafness, SSD, makes hearing comprehension very difficult.
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 hearing loss typically worsens over the years, and progression can be step-wise and related to minor head trauma. In some cases, language development worsens after head injury , demonstrating that the inner ear is sensitive to trauma in Pendred syndrome; this is as a consequence of the widened vestibular aqueducts usual in this syndrome. [ 4 ]
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