[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.

Method: 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.

Result: 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.

Conclusion: 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.