Clinical Characteristics of Pediatric Auditory Neuropathy / 대한이비인후과학회지

ABSTRACT

BACKGROUND AND OBJECTIVES: Auditory neuropathy (AN) is a hearing disorder caused by desynchronous neural discharge of auditory nerve. The purpose of this study is to describe in detail various clinical manifestations of children with AN. SUBJECTS AND METHOD: The medical record of 18 children with AN were retrospectively reviewed. Demographic information, the findings of various examination including audiologic, radiologic, neurologic, and vestibular tests, and the outcomes of cochlear implantation were analyzed. RESULTS: There were 14 boys and 4 girls. The onset of hearing loss was ranged from birth to 2 years of age. Most children were diagnosed with bilateral AN based on positive otoacoustic emission (OAE) with absent ABR and they showed no changes in hearing thresholds. They had showed persistently preserved OAE and showed abnormal vestibular test results, and were not accompanied by peripheral neuropathy. In contrast, a small number of children had unilateral AN and had cochear microphonic instead of OAE. They showed changes in hearing threshold and showed disappearance of OAE, and were accompanied by peripheral neuropathy. Most children who received cochlear implants showed significant improvement in speech perception abilities. CONCLUSION: AN is a heterogeneous disorder that has some common characteristics in auditory function. Children with AN who are not benefited from appropriate auditory rehabilitation using a hearing aid can obtain significant improvement from cochlear implantation.