![]() ![]() The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. A wide range of prenatal and postnatal etiologies have been proposed. The exact prevalence of ANSD remains unknown clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). Hearing loss is present at birth (congenital) or appears sometime later in life (acquired or delayed onset).Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Hearing loss gets either better or worse over time (fluctuating) or stays the same over time (stable). Hearing loss worsens over time (progressive) or happens quickly (sudden). Hearing loss is the same in both ears (symmetrical) or is different in each ear (asymmetrical). Hearing loss happened before a person learned to talk (pre-lingual) or after a person learned to talk (post-lingual). Hearing loss is in one ear (unilateral) or both ears (bilateral). ![]() The degree of hearing loss can range from mild to profound:Ī person with a mild hearing loss may hear some speech sounds but soft sounds are hard to hear.Ī person with a moderate hearing loss may hear almost no speech when another person is talking at a normal level.Ī person with severe hearing loss will hear no speech when a person is talking at a normal level and only some loud sounds.Ī person with a profound hearing loss will not hear any speech and only very loud sounds. For more information, visit the National Institute of Deafness and Other Communication Disorders. Hearing loss that occurs when sound enters the ear normally, but because of damage to the inner ear or the hearing nerve, sound isn’t organized in a way that the brain can understand. Hearing loss that includes both a conductive and a sensorineural hearing loss. Hearing loss that occurs when there is a problem in the way the inner ear or hearing nerve works. This type of hearing loss can often be treated with medicine or surgery. Hearing loss caused by something that stops sounds from getting through the outer or middle ear. The auditory pathway processes sound information as it travels from the ear to the brain so that our brain pathways are part of our hearing. This nerve sends sound information from the ear to the brain. the semicircular canals that help with balance.the snail shaped organ for hearing known as the cochlea.three small bones called ossicles that send the movement of the eardrum to the inner ear.the eardrum, sometimes called the tympanic membrane, which separates the outer and middle ear.the part we see on the sides of our heads, known as pinna.A hearing loss can happen when any part of the ear or auditory (hearing) system is not working in the usual way. ![]()
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