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Update on Auditory Neuropathy/Dyssynchrony in Children

  • Hearing Loss in Children (D Horn and H Ou, Section Editors)
  • Published:
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Abstract

Purpose of Review

Auditory neuropathy/dyssynchrony (AN/AD) is a form of sensorineural hearing loss characterized by the presence of cochlear outer hair cell (OHC) function and absent or abnormal auditory neural function. This article is meant to update clinicians on best practices for diagnosis and management of AN/AD.

Recent Findings

Exciting advances in genetics present opportunities for additional evidence to classify AN/AD based on site of lesion, and may lead to additional understanding of the pathophysiology as well as prognosis. Cochlear implantation continues to be a highly effective intervention for managing AN/AD in pediatric patients.

Summary

AN/AD can be a challenging condition to manage given the heterogeneity of its presentation and variety of options for management. Ultimately, clinicians must tailor treatment to the individual child which requires frequent follow-up and communication with families, educators, and other providers. Further research is needed to fully understand this disorder and advance evidence-based care for these children.

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Funding

Christina L. Runge reports grant funding from the National Institute on Deafness and Communication Disorders, MED-EL Corp., and Ascending Hearing Technologies; and personal fees from Novartis Corp., Frequency Therapeutics Corp., and Pipeline Therapeutics. She also reports a pending patent to the Medical College of Wisconsin/University of Wisconsin Milwaukee/Ascending Hearing Technologies for “Personal Sound Amplification Products with Self-Fitting.”

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  1. Medical College of Wisconsin, Milwaukee, WI, 53226, USA

    Alexandra N. Roman

  2. Department of Otolaryngology & Communication Sciences and Physiology, Medical College of Wisconsin, Milwaukee, WI, 53226, USA

    Christina L. Runge

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Roman, A.N., Runge, C.L. Update on Auditory Neuropathy/Dyssynchrony in Children. Curr Otorhinolaryngol Rep 8, 276–284 (2020). https://doi.org/10.1007/s40136-020-00297-4

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