[The diagnosis of auditory neuropathy in young children].
OBJECTIVE
To present the clinical data of a group of children with audiological profile of auditory neuropathy.


METHODS
Forty-eight infants and young children who had severely abnormal click ABRs along with present CMs and/or DPOAEs were included in this retrospective study. Click ABRs, CMs, DPOAEs, tympanograms, behavioral thresholds and inner ear MRI were analysed.


RESULTS
Fourty children present with bilateral audiological profile of AN, 8 were unilateral. Most of the ears (68.2%) were of absent click ABRs at the maximum presentation level of 100 dB nHL. However, some of them had repeatable Wave V at very high presentation levels. All ears showed present CMs while only 41.7% showed preserved DPOAEs. Four children were diagnosed as CND by further investigation of inner ear MRI.


CONCLUSIONS
It is recommended that CM testing be carried out routinely in children with severely abnormal click ABRs so as not to miss out AN; Behavioral hearing can not be predicted by auditory evoked responses; if audiological profile of AN is detected, further MRI exploration of well-developed cochlear nerve is necessary.
Mo
Lingyan
L
Key Laboratory of Otolaryngology Head and Neck Surgery, Capital Medical University, Ministry of Education, Beijing Tongren Hospital of Capital Medical University, Beijing Institute of Otolaryngology, Beijing 100005, China. moly@trhos.com
Yan
Fei
F
Liu
Hui
H
Chen
Jing
J
Zhang
Fan
F
D001301:Audiometry, Pure-Tone; D002675:Child, Preschool; D003951:Diagnostic Errors; D005072:Evoked Potentials, Auditory; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D008297:Male; D009421:Nervous System Malformations / Q000175:diagnosis; D012189:Retrospective Studies; D000160:Vestibulocochlear Nerve Diseases / Q000175:diagnosis* / Q000503:physiopathology