Update on Auditory Neuropathy/Dyssynchrony in Children
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HEARING LOSS IN CHILDREN (D HORN AND H OU, SECTION EDITORS)
Update on Auditory Neuropathy/Dyssynchrony in Children Alexandra N. Roman 1 & Christina L. Runge 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
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. Keywords Auditory neuropathy . Auditory dyssynchrony . Cochlear implant
Introduction Auditory neuropathy/dyssynchrony (AN/AD) is a disorder in which hearing impairment results from a lesion within the cochlea and/or auditory nerve. AN/AD is a distinct form of sensorineural hearing loss (SNHL) with a unique clinical presentation and diagnosis. Typical presentation of SNHL includes loss of outer hair cell function, and in severe cases inner hair cell function, while in AN/AD outer hair cell function is maintained with a site of lesion located within the inner hair cells and/or auditory nerve [1]. This is apparent in the diagnosis of AN/AD, which is characterized by present cochlear microphonic (CM) with absent or abnormal auditory brainstem response (ABR) [2]. Otoacoustic emissions This article is part of the Topical collection on Hearing Loss in Children * Christina L. Runge [email protected] Alexandra N. Roman [email protected] 1
Medical College of Wisconsin, Milwaukee, WI 53226, USA
2
Department of Otolaryngology & Communication Sciences and Physiology, Medical College of Wisconsin, Milwaukee, WI 53226, USA
(OAE) may also be present in AN/AD but are absent in approximately 15% of cases and therefore not solely relied upon for diagnosis [3•, 4, 5, 6, 7]. Additional clinical features of auditory neuropathy include speech perception deficits beyond what is expected from a behavioral audiogram, absent middle-ear reflexes, and varying degrees of stable or fluctuating hearing loss [8]. For this auditory disorder, including the term “dyssynchrony” has been proposed versus solely using “neuropathy’ given that the auditory nerve may not always be directly implicated in all patients, and to ensure clinicians do not overlook cochlear imp
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