신생아 청각선별검사로 진단된 선천성 난청환아의 임상적 추적
Clinical Follow-up of Hearing-Impaired Infants Detected by Newborn Hearing Screening
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초록
Abstract
Background and Objectives：When screned using cord blood, congenital hearing los are detected more frequently than other congenital metabolic diseases such as phenylketonuria or congenital hypothyroidism. Newborn hearing screning is im-portant because the early identification and intervention of neonatal hearing los is beneficial for the language development. We aimed to analyze clinical characteristics including associated diseases and present hearing state, and the efects of speech rehabi-Subjects and Method：Seventy nine hundred twelve neonates (6915 wel babies and 997 NICU babies) were screned by transient evoked otoacoustic emision (TEOAE) and auditory brainstem response (ABR). Medical records of infants with bilateral hea-ring los of more than 60 dB were evaluated, and they were further studied with temporal bone CT scan and folow-up hearing tests using ABR. The exon2 of the connexin26 gene was sequenced to detect the mutation. Results：Fourteen of 7912 infants birth weight, chromosomal anomaly, cleft palate, congenital nevus, and congenital aural atresia. Three of 14 infants were reve-aled to have normal hearing after follow-up hearing test, which were asociated with cleft palate, hyperbilirubinemia or prema-turity. One of them had 235delC mutation of the connexin26, and the temporal bone CT scan demonstrated the finding of enlarged vestibular aqueduct syndrome (EVAS) in one infant. Two infants participated in the conected spech rehabilitation program and showed significant development of language. Conclusion：newborn hearing screning test. The establishment of auditory and spech rehabilitation program conected with newborn hearing screening is esential in treating hearing-impaired neonates.
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