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KMID : 0858620060100020122
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Korean Journal of Audiology
2006 Volume.10 No. 2 p.122 ~ p.128
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Clinical Manifestations in the Children with Auditory Neuropathy
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Choi Seong-Jun
Mun Hyoung-Ah
Kang Young-Sook
Park Kee-Hyun
Park Hun-Yi
Choung Yun-Hoon
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Abstract
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Background and Objectives : The use of otoacoustic emissions (OAE) and auditory brainstem response (ABR) testing in children with hearing difficulty has led to the recognition of this recently described disorder called auditory neuropathy/auditory dys-synchrony (AN/AD). This diagnosis indicates that the infant has significant hearing loss despite having normal outer hair cells in the cochlea. We retrospectively reviewed the clinical manifestations and natural history of eight infants diagnosed as AN/AS.
Subjects and Methods : 938 children with hearing difficulties, who visited the Department of Otolaryngology, Ajou University Hospital, Suwon, Korea between January 2003 and September 2006 were enrolled in this study. All children took pure-tone audiometries (PTA), impedance measurements, transient evoked otoacoustic emissions (TEOAE), click-evoked auditory brainstem responses (ABR), and radiologic tests. AN criteria was abnormal ABR, normal TEOAE, and normal impedance.
Results: Eight (0.85%) of 938 children with hearing difficulty were diagnosed as AN/AD. The mean age was 36 months old (3 months - 8 years). Six (75%) of 8 children is bilateral AN/AD and 2 (25%) is unilateral. We treated 6 children with cochlear implants and hearing aids in the bilateral AN/AD and have performed the regular audiological tests on 2 children with unilateral AN/AD. One child’s hearing was recovered in the behavior observation audiometry over time without any ABR recovery, and so he does not use HAs now.
Conclusion: Neonatal hearing loss in newborn hearing screenings should be confirmed with ABR as well as OAE not to skip the children with AN/AD. Managements for children with AN/AD requires serial clinical and audiometric evaluations with a prominent role for subjective tests, because there is possibility that behavioral hearing can be recovered without the change of ABR.
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KEYWORD
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Auditory neuropathy, Otoacoustic emission, Auditory brainstem response, Clinical manifestation, Diagnosis, Treatment
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