Genetic Update on Auditory Neuropathy

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Genetic Update on Auditory Neuropathy

Auditory neuropathy (AN) is defined as a sensorineural hearing impairment (HI) characterized by preserved cochlear outer hair cell function with normal otoacoustic emissions but abnormal or absent brainstem evoked potentials. The prevalence of AN in HIs remains to be established and its pathophysiology has not been determined. Several environmental and genetic causes have been identified, but t...

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An Update on Auditory Neuropathy Spectrum Disorder in Children

Auditory neuropathy spectrum disorder (ANSD) is a form of hearing impairment in which cochlear (outer hair cell) function appears normal but afferent neural activity in the auditory pathway is disordered. It is now over 20 years since the first paediatric ANSD cases appeared in the literature and over 15 years since the disorder was identified in infant populations. In the ensuing period our un...

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Auditory steady state response in auditory neuropathy.

REVIEW Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response. PURPOSE This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response. MATERIALS AND METHODS Thirt...

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Cochlear implantation of auditory neuropathy.

Auditory neuropathy (AN) is a hearing disorder that presents with a grossly abnormal or absent neural response as measured by evoked potentials in the presence of normal outer hair cell function evidenced by present otoacoustic emissions or cochlear microphonics. Rehabilitation for patients with AN is challenging due to abnormal temporal encoding at the auditory nerve leading to severely impair...

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ژورنال

عنوان ژورنال: Audiology and Neurotology Extra

سال: 2011

ISSN: 1664-5537

DOI: 10.1159/000329545