[Clinical and audiological findings in children with auditory neuropathy].
BACKGROUND
Auditory neuropathy is a disorder characterised by preservation of outer hair cells function with normal otoacoustic emissions (OAEs), but with absent auditory brainstem responses (ABR). Perisynaptic synchronisation disorder is one of the possible pathogenesis underlying auditory neuropathy. In this paper we describe the clinical presentation and audiological findings in pediatric auditory neuropathy and its management.


METHODS
9 children with auditory neuropathy could be included in the study. An audiological evaluation was performed in all children including behavioural audiometry, measurement of the OAEs as well as electrocochleography (ECoG) and ABR recordings. Children who failed to get any benefit from conventional amplification received a cochlear implant. Prior to implantation the responses to electrical stimuli were examined with the promontory test and with the electrically evoked ABR.


RESULTS
One child showed auditory neuropathy only on one side with normal hearing thresholds on the contralateral ear. Another child had normal hearing thresholds after the follow up period. Four children received a hearing aid. But variable hearing reactions were observed. Thus in three cases a CI is planned. In three children cochlea implantation was done. Following implantation a remarkable improvement in hearing/speech capabilities with the CI compared to conventional hearing aids were observed in all three cases. Beside, these three children developed open set speech discrimination and are using now oral language for communication.


CONCLUSIONS
Auditory neuropathy is a disorder which presents with different clinical and audiological findings. Thus the management of this disorder must be an individual one. In light of our findings we support the use of cochlear implants as an option for children with auditory neuropathy in cases where conventional amplification does not work sufficiently.
Shehata-Dieler
W
W
Universitätsklinikum Würzburg, Klinik und Poliklinik für Hals-, Nasen- und Ohrenkranke, Würzburg. dieler_w@klinik.uni-wuerz-burg.de
Völter
C
C
Hildmann
A
A
Hildmann
H
H
Helms
J
J
D001300:Audiometry, Evoked Response; D001302:Audiometry, Speech; D001309:Auditory Threshold / Q000502:physiology; D001933:Brain Stem / Q000503:physiopathology; D002675:Child, Preschool; D019929:Cochlear Implantation; D003056:Cochlear Nerve / Q000503:physiopathology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D018072:Hair Cells, Auditory, Outer / Q000503:physiopathology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000503:physiopathology / Q000534:rehabilitation*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology; D013063:Speech Discrimination Tests; D000160:Vestibulocochlear Nerve Diseases / Q000175:diagnosis / Q000503:physiopathology / Q000534:rehabilitation*