What’s hereditary deafness?

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Hereditary deafness is passed down through genes and can result in hearing loss, speech impairment, and blindness. It can be carried on dominant or recessive genes and affect different areas of the body. Genetic testing can determine if an individual carries the gene.

Hereditary deafness is a condition that is passed on to children from one or both parents through genetic makeup. The end result is hearing loss, which may or may not be present from birth. For some children, it simply means that they are predisposed to hearing loss later in life. Others may show signs of not just hearing loss but other related conditions such as blindness or speech impairment.

Genetic information can be carried on dominant or recessive genes. In cases where it is carried by a dominant gene, it only requires one parent to have the gene for it to affect the child. For recessive genes, the child must get the gene from both parents for it to show. One of the most common types of recessively inherited deafness is Usher syndrome, in which the individual exhibits not only hearing loss but varying degrees of blindness as well.

Symptoms of hereditary deafness can manifest themselves in several ways. People with syndromic hearing loss may show impairments in structures associated with hearing, such as difficulties with speech or balance. With nonsyndromic hearing loss, the only thing affected is the hearing. Onset varies with the individual and may be evident before or after the development of the ability to speak. Also, there is no fixed severity of hearing loss, as some individuals may experience only mild hearing loss while others may have profound or severe loss.

Hereditary deafness can affect different areas of the body. Favorable hearing loss occurs when there is an inherited abnormality in the formations and structures of the middle or external ear, whereas sensorineural hearing loss occurs due to abnormalities in the inner ear. There are other structures associated with a person’s ability to hear, including the cranial nerve, cerebral cortex, and brainstem. The name given to the hereditary malfunctions of these other structures is central auditory dysfunction.

There are more than 400 different hereditary conditions that include hearing loss as a component of their condition. Waardenburg syndrome is characterized by varying degrees of hearing loss accompanied by abnormal pigments in the skin, eyes and hair. Osteoarthritis is associated with hearing loss in Stickler syndrome, and branchiotorenal syndrome occurs in individuals who exhibit hearing loss along with external ear abnormalities.

Genetic testing can establish whether individuals carry the hereditary deafness gene. When a child exhibits hearing loss, it’s not always due to an inherited condition. It can be caused by environmental factors, prenatal infections, or even bacterial infections. The causes of hearing loss can be determined by parents planning to have more children.




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