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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 .
Alport syndrome is a genetic disorder [1] affecting around 1 in 5,000–10,000 children, [2] characterized by glomerulonephritis, end-stage kidney disease, and hearing loss. [3] Alport syndrome can also affect the eyes, though the changes do not usually affect vision, except when changes to the lens occur in later life.
A Rinne test should always be accompanied by a Weber test to also detect sensorineural hearing loss and thus confirm the nature of hearing loss. The Rinne test was named after German otologist Heinrich Adolf Rinne (1819–1868); [ 3 ] [ 4 ] the Weber test was named after Ernst Heinrich Weber (1795–1878).
Relapsing polychondritis is a systemic disease characterized by repeated episodes of inflammation and in some cases deterioration of cartilage.The disease can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected.
Hearing loss completely recovers in around 35–39% of patients with SSNHL, usually within one to two weeks from onset. [34] Steroid treatment within seven days, a lower initial severity of hearing loss, the absence of vertigo, younger patient age, and a history of cardiovascular disease are all associated with complete hearing recovery. [35]
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.
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