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Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear, sensory organ (cochlea and associated structures), or the vestibulocochlear nerve (cranial nerve VIII). SNHL accounts for about 90% of reported hearing loss.
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Deafness can be broken down into four different types of hearing loss: conductive hearing loss, sensorineural hearing loss, mixed hearing loss, and auditory neuropathy spectrum disorder. All of these forms of hearing loss cause an impairment in a person's hearing where they are not able to hear sounds correctly.
This condition was first discovered in 1995 by Melberg et al. when they described 5 members of a 4-generation Swedish family where cerebellar ataxia and sensorineural deafness presented as an autosomal dominant trait, 4 of them had narcolepsy and 2 had diabetes mellitus. The oldest members had psychiatric symptoms, neurological anomalies, and ...
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]
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.
Pages in category "Syndromes with sensorineural hearing loss" The following 10 pages are in this category, out of 10 total. This list may not reflect recent changes .
Ocular albinism late onset sensorineural deafness (OASD) is a rare, X-linked recessive disease characterized by intense visual impairments, reduced retinal pigments, translucent pale-blue irises and moderately severe hearing loss from adolescence to middle-age. [1] It is a subtype of Ocular Albinism (OA) that is linked to Ocular albinism type I ...