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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. [citation needed] SNHL is usually permanent and can be mild, moderate, severe ...
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 ...
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.
Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the structures of the ear (see auditory system ). It has been argued to be as the combination of auditory verbal agnosia and auditory agnosia. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of ...
Autoimmune inner ear disease. 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.
Waardenburg syndrome is a group of rare genetic conditions characterised by at least some degree of congenital hearing loss and pigmentation deficiencies, which can include bright blue eyes (or one blue eye and one brown eye ), a white forelock or patches of light skin. These basic features constitute type 2 of the condition; in type 1, there ...
Auditory agnosia is a form of agnosia that manifests itself primarily in the inability to recognize or differentiate between sounds. It is not a defect of the ear or "hearing", but rather a neurological inability of the brain to process sound meaning. While auditory agnosia impairs the understanding of sounds, other abilities such as reading ...
none. Prognosis. medium. Frequency. rare, about 24 cases have been described in medical literature. Deaths. -. Corneal dystrophy-perceptive deafness syndrome, also known as Harboyan syndrome, is a rare genetic disorder characterized by congenital hereditary corneal dystrophy that occurs alongside progressive hearing loss of post-lingual onset. [2]
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