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Surviving cochlear function in the presence of auditory nerve agenesis

Published online by Cambridge University Press:  29 June 2007

S. J. O'Leary  and
W. P. Gibson
S. J. O'Leary*
Affiliation:
Departments of Otolaryngology, The Royal Victorian Eye and Ear Hospital, East Melbourne 3002, Australia.
W. P. Gibson
Affiliation:
Departments of Otolaryngology, University of Sydney, Sydney, Australia.
*
Address for correspondence: Stephen O'Leary, Department of Otolaryngology, Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne, Victoria 3002, Australia.
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Abstract

This case reports electrophysiological evidence for cochlear function in a child with radiological evidence of bilateral auditory nerve agenesis or severe hypoplasia. The diagnosis of auditory nerve agenesis was supported by a bilateral atresia of internal auditory canals on computed tomography (CT) scan and magnetic resonance imaging (MRI) absent auditory brainstem responses and absent behavioural responses to sound. Despite the apparent absence of an auditory nerve or spiral ganglion, electrocochleography revealed surviving cochlear function at 70–80 db HL and an abnormal electrocochleographic waveform. This case demonstrates that cochlear function may develop without afferent, or efferent innervation. It also emphasizes that cochlear function may occur in the presence of profound deafness.

Keywords

Evoked potentials, auditoryDeafnessAcoustic nerveAcoustic nerve diseases
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 11 , November 1999 , pp. 1008 - 1010
Copyright
Copyright © JLO (1984) Limited 1999

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