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Autoimmune inner ear disease (AIED) was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing. [2] The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system .
Sensorineural hearing loss: Conductive hearing loss: Anatomical site: Inner ear, cranial nerve VIII, or central processing centers Middle ear (ossicular chain), tympanic membrane, or external ear: Weber test: Sound localizes to normal ear in unilateral SNHL Sound localizes to affected ear (ear with conductive loss) in unilateral cases Rinne test
Cogan syndrome (also Cogan's syndrome) is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and hearing loss. It can lead to deafness or blindness if untreated.
In case of a severe sensorineural hearing loss caused due to a dead labyrinth, a false negative Rinne test may occur. It is caused by the fact that even though one ear is unable to respond to the test, the other ear can still be stimulated by the bone conduction test (via conducting sound through skull bones to the opposite ear), causing the ...
Affected children show a marked decline in learning between ages 2 and 6, followed by eventual loss of language skills and loss of some or all hearing. Some children may never learn to speak. In the syndrome's second stage, aggressive behavior, hyperactivity, profound dementia, and irregular sleep may make children difficult to manage ...
Ashton Kutcher said he lost his ability to walk and see for nearly a year after being diagnosed with vasculitis. Vasculitis is an autoimmune disorder caused by inflammation to blood vessels.
Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Nonsyndromic deafness constitutes 75% of all hearing loss cases, and an estimated 100 genes are thought to be linked to this condition.
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