Clinical Considerations in the Differential Diagnosis of Children with Auditory Neuropathy Spectrum Disorder from Sensorineural Hearing Loss; Case Report

Abstract

Background: Auditory neuropathy spectrum disorder (ANSD) is a type of hearing impairment distinct from sensorineural hearing loss in the peripheral auditory system. The purpose of this study was to emphasize the importance of using the test battery approach and the role of the family in the differential diagnosis of ANSD from common sensorineural hearing loss in the form of reports of two children with hearing impairment. Case Report: The first child, a 19-month-old boy with cerebral palsy with a history of kernicterus and twice of blood exchange. He was first diagnosed with bilateral profound sensorineural hearing loss using electrophysiological tests. Due to the rejection of the hearing aid and the parent' doubts about the disappropriate reactions of the child, in re-evaluation via test battery approach, ANSD was obtained. The second child was a 6-year-old girl with no specific medical history in school entrance screening. In the first session of hearing evaluation using audiometry and acoustic immittance, bilateral mild to moderate sensorineural hearing loss was achieved with more loss at low frequencies. Due to the low word recognition score and parental denial, electrophysiological tests were performed, and ANSD was confirmed. Conclusion: Accurate case history, hearing assessment of children with sensorineural hearing loss with ANSD protocols, which is an appropriate combination of behavioral and electrophysiological hearing tests, as well as increased family awareness, are important factors in the differential diagnosis of ANSD and selection of appropriate intervention. © 2021 Isfahan University of Medical Sciences(IUMS). All rights reserved.