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Usually, people with this disorder have ataxia, mild–moderate sensorineural hearing loss, narcolepsy, and cataplexy. These symptoms start happening when an affected person is about 30 years old. [6] [7] A bit later in life, people with ADCADN start showing a decline in executive function known as dementia.
Many genes related to deafness are involved in the development and function of the inner ear. Gene mutations interfere with critical steps in processing sound, resulting in hearing loss. Different mutations in the same gene can cause different types of hearing loss, and some genes are associated with both syndromic and nonsyndromic deafness.
The association between age-related hearing loss and Alzheimer's disease was found to be nonsignificant, and this finding supports the hypothesis that hearing loss is associated with dementia independent of Alzheimer pathology. [32] There are several hypotheses about the underlying causal mechanism for age-related hearing loss and cognitive ...
Congenital hearing loss is a hearing loss present at birth. It can include hereditary hearing loss or hearing loss due to other factors present either in-utero (prenatal) or at the time of birth. It can include hereditary hearing loss or hearing loss due to other factors present either in-utero (prenatal) or at the time of birth.
Familial Danish dementia is an extremely rare, neurodegenerative disease characterized by progressive cataracts, loss of hearing, cerebellar ataxia, paranoid psychosis, and dementia. Neuropathological hallmarks include extensive atrophy of all areas of the brain, chronic diffuse encephalopathy , and the presence of exceedingly thin and nearly ...
Hearing loss can also be attributed to genetic mutations, noise exposure, exposure to therapeutic drugs that have ototoxic side effects and chronic other conditions.
Children who inherit genetic mutations from parents with early-onset Alzheimer’s typically show signs of dementia at the same age their parents did. Meet the 24-year-old helping Lilly test a ...
Hearing loss due to noise has been described as primarily a condition of modern society. [17] In preindustrial times, humans had far less exposure to loud sounds. Studies of primitive peoples indicate that much of what has been attributed to age-related hearing loss may be long-term cumulative damage from all sources, especially noise.
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