[Clinical findings of a group of children with auditory neuropathy spectrum disorder].
OBJECTIVE
In order to extend the knowledge of auditory neuropathy spectrum disorder (ANSD), the clinical data of a group of infants and young children with this disorder was reported.


METHODS
This retrospective study involved 84 cases (151 ears). The subjects aged from 2 months to 6 years old. Descriptive analysis was carried out on case history, audiological and imaging results and the outcome of cochlear implantation.


RESULTS
There were a variety of case history, including 11 of hyperbilirubinemia, 2 of family history of deafness, 3 of hypoxia, 1 of prematurity, 1 of nuclei basales pathology, 2 of congenital atelencephalia,and 13 of cochlear nerve deficiency (CND). The audiological results varied too. Click ABR ranged from no response at the maximum output level to Wave V thresholds as low as 70 dB nHL. Behavioral thresholds were mild in 1, moderate in 2 and profound in 20. The inner ear MRI were normal in 16 cases, CND in 12 and CND accompanied by periventricular leukomalacia in 1. 5 cases were implanted in the affected ear. The results in 3 were comparable with that of cochlear loss, 1 were less effective than that of cochlear loss, 1 became none user of the device.


CONCLUSIONS
ANSD is a heterogeneous group with a wide variety on case history, audiological and imaging results and the outcome of cochlear implant. Therefore, comprehensive clinical examinations are necessary so that individualized intervention program can be made.
Department of Otolaryngology, Beijing Tongren Hospital of Capital Medical University, Beijing Institute of Otolaryngology, Beijing, 100005, China. lingyanmo@gmail.com
Lingyan
L
Mo
Fei
F
Yan
Hui
H
Liu
Jing
J
Chen
Xueqin
X
Chen
Lihui
L
Huang
D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D005260:Female; D006313:Hearing Loss, Central / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies