Molecular approach of auditory neuropathy

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Refinement of Molecular Diagnostic Protocol of Auditory Neuropathy Spectrum Disorder

Auditory neuropathy spectrum disorder (ANSD) is a sensorineural hearing disorder caused by dysfunction of auditory neural conduction. ANSD has a heterogeneous etiology, including genetic factors; the response to cochlear implantation significantly varies depending on the etiology. The results of timely cochlear implantation for OTOF-related ANSD (DFNB9) have been reported to be good. Therefore,...

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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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Current understanding of auditory neuropathy.

Auditory neuropathy is defined by the presence of normal evoked otoacoustic emissions (OAE) and absent or abnormal auditory brainstem responses (ABR). The sites of lesion could be at the cochlear inner hair cells, spiral ganglion cells of the cochlea, synapse between the inner hair cells and auditory nerve, or the auditory nerve itself. Genetic, infectious or neonatal/perinatal insults are the ...

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ژورنال

عنوان ژورنال: Brazilian Journal of Otorhinolaryngology

سال: 2015

ISSN: 1808-8694

DOI: 10.1016/j.bjorl.2015.03.005