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The hearing loss associated with congenital aural atresia is a conductive hearing loss—hearing loss caused by inefficient conduction of sound to the inner ear. Essentially, children with aural atresia have hearing loss because the sound cannot travel into the (usually) healthy inner ear—there is no ear canal, no eardrum, and the small ear ...
Molecular genetic Testing determines that FGF3 is the only gene, whose mutation can cause congenital deafness with Michel's Aplasia, microdontia and microtia Carrier testing for at-risk relatives requires identification of mutations which are responsible for occurrence of disease in the family. Prevention (of secondary complications)
Noise. Noise exposure is the cause of approximately half of all cases of hearing loss, causing some degree of problems in 5% of the population globally. [5] The National Institute for Occupational Safety and Health (NIOSH) recognizes that the majority of hearing loss is not due to age, but due to noise exposure.
Sensorineural hearing loss ( SNHL) is a type of hearing loss in which the root cause lies in the inner ear, sensory organ ( cochlea and associated structures), or the vestibulocochlear nerve ( cranial nerve VIII). SNHL accounts for about 90% of reported hearing loss. [citation needed] SNHL is usually permanent and can be mild, moderate, severe ...
Branchio-oto-renal syndrome. Branchio-oto-renal syndrome has an autosomal dominant pattern of inheritance. Branchio-oto-renal syndrome (BOR) [4] [5] is an autosomal dominant genetic disorder involving the kidneys, ears, and neck. It is also known as Melnick-Fraser syndrome. [2] [3]
Pendred syndrome is a genetic disorder leading to congenital bilateral (both sides) sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism (decreased thyroid gland function). There is no specific treatment, other than supportive measures for the hearing loss and thyroid hormone supplementation in case of hypothyroidism.
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