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We often come across children and young adults brought in for consultation for suspected hearing loss and having hearing difficulty in noisy backgrounds but who often have normal audiograms. Such patients are suspected to have auditory neuropathy. The term auditory neuropathy (AN) is used to describe a group of patients demonstrating evidence of present cochlear (inner ear) activity accompanied by absent or highly abnormal neural responses at the level of the auditory nerve and brainstem. These patients with AN/AS typically present with difficulty in understanding speech, particularly in noisy situations, and can have significant impact on speech perception ability and delayed speech and language development. A high index of suspicion must be maintained to make the correct diagnosis. Otoacoustic emissions (OAE), cochlear microphonics (CM) and ABR form the most sensitive combination of measures to assess AN. Pure-tone thresholds may range from normal sensitivity to severe or profound hearing loss. Speech recognition is typically poorer with AN/AS than expected, based on pure-tone thresholds. Management of these patients can be quite challenging. Trial of amplification is recommended. If a child is not progressing, particularly after several months of hearing aid use, then cochlear implantation may be recommended. This paper covers management of such patients. It details the investigations and treatment options that should be considered in their management.

Auditory Neuropathy/Auditory Synaptopathy.
Hood LJ.
OTOLARYNGOL CLIN NORTH AM
2021;54(6):1093-100.
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CONTRIBUTOR
Sangeeta Maini

FRCS ORL-HNS, Aberdeen Royal Infirmary, Forresterhill, Aberdeen, AB25 2ZN.

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CONTRIBUTOR
Bhaskar Ram

FRCS (EDIN) , FRCS ORL-HNS, Department of Otolaryngology Head & Neck Surgery, Aberdeen Royal Infirmary, Forresterhill, Aberdeen AB25 2ZN, UK.

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