Hostname: page-component-78c5997874-94fs2 Total loading time: 0 Render date: 2024-11-18T16:37:37.417Z Has data issue: false hasContentIssue false
  • English
  • Français

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.
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Aso, S., Gibson, W. P. (1994) Electrocochleography in profoundly deaf children: comparison of promontory and round window techniques. American Journal of Otology 15: 376–379.Google ScholarPubMed
Chisin, R., Perlman, M., Sohmer, H. (1979) Cochlear and brainstem responses in hearing loss following neonatal hyperbilirubinemia. Annals of Otology 88: 352–357.Google ScholarPubMed
Corwin, J. T., Cotanche, D. A. (1989) Development of location-specific hair cell stereocilia in denervated embryonic ears. Journal of Comparative Neurology 288: 529–537.CrossRefGoogle ScholarPubMed
Deltenre, P., Mansbach, A. L., Bozer, C., Clercx, A., Hecox, K. E. (1997) Auditory neuropathy: a report on three cases with early onsets and major neonatal illnesses. Electroencephalography and ClinicalNeurophysiology 104: 17–22.CrossRefGoogle ScholarPubMed
Guirado, C. R. (1992) Internal auditory meatal malformations. Revue de Laryngologie 113: 419–421.Google Scholar
Konradsson, K. S. (1996) Bilaterally presented otoacoustic emissions in four children with profound idiopathic unilateral sensorineural hearing loss. Audiology 35: 217–227.CrossRefGoogle Scholar
Mansbach, A. L, Deltenre, P., Pelc, P., Marquet, T. (1998) Auditory neuropathy. Abstracts, 7th International Congress Paediatric OtolaryngologyHelsinkiJune 1998, p 280.Google Scholar
Moore, D. R. (1990) Auditory brainstem of the ferret: early cessation of developmental sensitivity of neurons in the cochlear nucleus to removal of the cochlea. Journal of Comparative Neurology 302: 810–823.CrossRefGoogle ScholarPubMed
Starr, A., Picton, T. W., Sininger, Y., Hood, L. J., Berlin, C. I. (1996) Auditory neuropathy. Brain 119: 741–753.CrossRefGoogle ScholarPubMed
Van De Water, T. R. (1986) Determinants of neurons-sensory receptor cell interaction during development of the inner ear. Hearing Research 22: 265–277.CrossRefGoogle ScholarPubMed
Van De Water, T. R. (1988) Tissue interactions and cell differentiation: neurone-sensory cell interaction during otic development. Development (Suppl 103): 185–193.CrossRefGoogle ScholarPubMed
Van De Water, T. R., Galinovic-Schwartz, V., Ruben, R. J.(1989) Neuron-receptor cell interaction during development ofthe inner ear. A heterochronic ganglion study. Acta Oto-Laryngologica 107: 412–412.CrossRefGoogle Scholar
Vivnaud, J., Jardin, C., Rosen, L. (1986) The Ear, Masson Pub. USA: pp 115–116.Google Scholar
Wong, S.H.-W., Gibson, W. P., Sanli, H. (1997) Use of transtympanic round window electrocochleography for threshold estimations in children. American Journal of Otology 18: 632–636.Google ScholarPubMed