Refinement of Molecular Diagnostic Protocol of Auditory Neuropathy Spectrum Disorder

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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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Children With Auditory Neuropathy Spectrum Disorder (ANSD)

In 1991, Arnold Starr and colleagues described an 11-year-old child with the paradoxic findings of absent auditory brainstem responses in the presence of normal cochlear microphonics (CMs) and otoacoustic emissions (OAEs). The child demonstrated severely impaired speech understanding despite a mild loss of hearing sensitivity. Further evaluation showed impaired temporal processing as exhibited ...

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Management of children with auditory neuropathy spectrum disorder (ANSD).

INTRODUCTION ANSD is a challenging problem. OBJECTIVE To present our experience on management of the children with ANSD with respect to clinical data. METHODS This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAE...

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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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ژورنال

عنوان ژورنال: Medicine

سال: 2015

ISSN: 0025-7974

DOI: 10.1097/md.0000000000001996