Disease - Deafness, autosomal recessive, 59
Definition
A form of sensorineural hearing impairment with absent or severely abnormal auditory brainstem response but normal otoacoustic emissions (auditory neuropathy or auditory dys-synchrony). Auditory neuropathies result from a lesion in the area including the inner hair cells, connections between the inner hair cells and the cochlear branch of the auditory nerve, the auditory nerve itself and auditory pathways of the brainstem.
Acronym
DFNB59
Synonyms
DFNB59 auditory neuropathy
Keywords
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