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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 ...
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
A negative Stenger result indicates that the patient responded to the signal presented to the better ear, indicating an organic asymmetric hearing loss. A positive Stenger result indicates that the patient did not respond even though the signal was 10 to 20 dB above the better ear, indicating a non-organic hearing loss. [4]
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.
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.
The tone decay test (also known as the threshold tone decay test or TTDT) is used in audiology to detect and measure auditory fatigue. It was developed by Raymond Carhart in 1957. In people with normal hearing, a tone whose intensity is only slightly above their absolute threshold of hearing can be heard continuously for 60 seconds.
The most common type of congenital hearing loss in developed countries is DFNB1, also known as connexin 26 deafness or GJB2-related deafness. The most common dominant syndromic forms of hearing loss include Stickler syndrome and Waardenburg syndrome. The most common recessive syndromic forms of hearing loss are Pendred syndrome and Usher syndrome.