auditory neuropathy


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auditory neuropathy

a disorder of hearing in children characterized by sensorineural hearing loss for pure tones, reduced word discrimination disproportionate to the pure tone loss, normal outer hair cell function as determined by measurement of otoacoustic emissions, and absent or abnormal auditory brainstem response.

au·di·to·ry neu·rop·a·thy

(aw'di-tōr-ē nūr-op'ă-thē)
A distinctive type of hearing deficit that seemingly is due to a malfunction of the eighth cranial nerve. Previously referred to as auditory neural synchrony disorder. Speech comprehension in quiet surroundings is out of proportion to the pure tone threshold elevation.

auditory neuropathy

Abbreviation: AN
Impaired hearing in children due to an absence of auditory evoked potentials, despite the presence of normal cochlear hair cell structure and function.
Synonym: auditory dyssynchrony
See also: neuropathy
References in periodicals archive ?
Cochlear implantation in patients with auditory neuropathy of varied etiologies.
Cochlear implants in five cases of auditory neuropathy: postoperative findings and progress.
Auditory neuropathy. In: Handbook of clinical neurology, 2015, pp.
Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy. Brain.
A novel missense mutation in a C2 domain of oToF results in autosomal recessive auditory neuropathy. Am J Med Genet A.
Therefore, there is sufficient evidence that the abnormal hearing thresholds can be improved or restored in some HR infants within a few months and this is especially true for infants suffering from hearing loss of auditory neuropathy type.
Particularly, the HR infants with an auditory neuropathy profile should be treated less aggressively than the other HR infants, in view of the fact that partial or complete ABR recovery is more likely to take place in this group during the following months.
"Since we previously knew of only two genes associated with auditory neuropathy, finding this gene mutation is significant.
DISCUSSION: Auditory neuropathy is a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired.
CONCLUSION: The child presenting with deaf mute and perinatal insult along with abnormal brainstem evoked response and normal otoacoustic emission suspect auditory neuropathy. The features of auditory neuropathy along with bilateral bat ears, hyperactive attention deficit, and hypertelorism are rarest clinical findings which are not reported in any ENT syndromes till date.
Auditory neuropathy spectrum disorder would be an extreme example of dyssynchronous neural firing, affecting even the response to the click [32-34].