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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 .
This type of hearing loss allows hearing at low and high frequencies, with most of the hearing loss occurring at the mid-frequencies. #5. Unilateral Hearing Loss Audiogram
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
An audiologist conducting an audiometric hearing test in a sound-proof testing booth. Identification of a hearing loss is usually conducted by a general practitioner medical doctor, otolaryngologist, certified and licensed audiologist, school or industrial audiometrist, or other audiometric technician.
The Rinne test (/ ˈ r ɪ n ə / RIN-ə) is used primarily to evaluate loss of hearing in one ear. [1] It compares perception of sounds transmitted by air conduction to those transmitted by bone conduction through the mastoid. Thus, one can quickly screen for the presence of conductive hearing loss. [2]
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.
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 is a partial or total inability to hear. [5] Hearing loss may be present at birth or acquired at any time afterwards. [6] [7] Hearing loss may occur in one or both ears. [2] In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. [8]
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