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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.
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]
Patulous Eustachian tube is a physical disorder. The exact causes may vary depending on the person and are often unknown. [5] Weight loss is a commonly cited cause of the disorder due to the nature of the Eustachian tube itself and is associated with approximately one-third of reported cases. [6]
The shape of the audiogram resulting from pure-tone audiometry gives an indication of the type of hearing loss as well as possible causes. Conductive hearing loss due to disorders of the middle ear shows as a flat increase in thresholds across the frequency range. Sensorineural hearing loss will have a contoured shape depending on the cause.
[3] [14] Additionally, hearing loss is not as common as in type 2. [3] Rarely, cleft lip has been reported in this form of Waardenburg syndrome. [15] Type 4 can also be caused by a mutation in SOX10 (the same gene as in type 2E), in which it is known as type 4C; hearing loss is very common and severe in this type. [16]
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