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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.
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 ...
Autosomal dominant cerebellar ataxia (ADCA) is a form of spinocerebellar ataxia inherited in an autosomal dominant manner. ADCA is a genetically inherited condition that causes deterioration of the nervous system leading to disorder and a decrease or loss of function to regions of the body.
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 ...
Strokes – Depending on what blood vessels are affected by the stroke, one of the symptoms can be deafness; Superior semicircular canal dehiscence, a gap in the bone cover above the inner ear, can lead to low-frequency conductive hearing loss, autophony, and vertigo. Syndromic hearing loss can be either conductive or sensorineural. It occurs ...
Treatment of childhood dementia focuses on managing symptoms and improving quality of life. This can include: Medications: Anti-seizure medications, behaviour-modifying drugs, and muscle relaxants. [1] Therapies: Physiotherapy, occupational therapy, and speech therapy are used to maintain physical function for as long as possible. [1]
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