Hostname: page-component-cd9895bd7-jkksz Total loading time: 0 Render date: 2024-12-28T20:45:14.994Z Has data issue: false hasContentIssue false
  • English
  • Français

Useful residual hearing despite radiological findings suggestive of anacusis

Published online by Cambridge University Press:  29 June 2007

Doris Eva Bamiou ,
Cliodna O Mahoney  and
Tony Sirimanna
Doris Eva Bamiou*
Affiliation:
Audiology Department, Great Ormond Street Hospital, London, UK.
Cliodna O Mahoney
Affiliation:
Audiology Department, Great Ormond Street Hospital, London, UK.
Tony Sirimanna
Affiliation:
Audiology Department, Great Ormond Street Hospital, London, UK.
*
Address for correspondence: Dr D. E. Bamiou, Audiology Departmeny, Great Ormond Street Hospital, London WC1N 3JH. e-mail: doriseva@ndirect.co.uk.
Get access Rights & Permissions [Opens in a new window]

Abstract

A severe malformation of the inner ear, often referred to as severe labyrinthine dysplasia or common cavity deformity, consists of an absent or dilated cochlear basal coil, wide communication with the vestibule and a tapered internal acoustic meatus and can be associated with absent hearing. We discuss two children with severe labyringthine dysplasia as shown by computed tomography (CT) scans and, in the first case, an absent VIIIth nerve bilaterally shown by magnetic resonance imaging (MRI). In 1995, both cases were precluded from cochlear implantation, on the basis of the absent VIIIth nerve (first case) and increased risk of CSF leak during operation (second case). However, audiometric results and vocalization patterns of both children suggested the presence of some residual hearing function, while recently reported specific surgical techniques have been found to be safe and effective in the cochlear implantation of the common cavity deformity. The management of such cases should be decided on the grounds of a full audiological assessment in conjunction with the radiological features, in the light of current surgical trends shown to be safe and effective.

Keywords

LabyrinthDysplasiaCochlear implant
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 8 , August 1999 , pp. 714 - 716
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

Boothroyd, A. (1984) Auditory perception of speech contrasts by subjects with sensorineural hearing loss. Journal of Speech and Hearing Research 27: 134–144.CrossRefGoogle ScholarPubMed
Boothroyd, A., Cawkwell, S. (1970) Vibrotactile thresholds in pure tone audiometry. Acta Otolaryngologica 69: 381–387.CrossRefGoogle ScholarPubMed
Hoffman, R. A., Downey, L. L., Waltzman, S. B., Cohen, N. L. (1997) Cochlear implantation in children with cochlear malformations. American Journal of Otology 18: 184–187.Google ScholarPubMed
Jackler, R. K., Luxford, W. M., House, W. F. (1987) Congenital malformations of the middle ear: A classification based on embryogenesis. Laryngoscope 97: 2–14.CrossRefGoogle Scholar
Kent, R. D., Osberger, M. J., Netshell, R., Hustedde, S. G. (1987) Phonetic development in identical twins who differ in auditory function. Journal of Speech and Hearing Disorders 52: 64–75.CrossRefGoogle ScholarPubMed
Luntz, M., Balkany, T., Hodges, A. V., Telischi, F. F. (1997) Cochlear implants in children with congenital inner ear malformations. Archives of Otolaryngology – Head and Neck Surgery 123(9): 974–977.CrossRefGoogle ScholarPubMed
Lynch, M. P., Oiler, D. K., Steffens, M. (1989) Development of speech-like vocalizations in a child with congenital absence of cochleas: the case of total deafness. Applied Psycholinguistics 10: 315–333.CrossRefGoogle Scholar
McElveen, J. T., Carasco, V. N., Miyamoto, R. T., Linthreum, F. H. (1997) Cochlear implantation in common cavity malformations using a transmastoid labyrinthotomy approach. Laryngoscope 107: 1032–1036.CrossRefGoogle ScholarPubMed
Ormerod, F. C. (1960) The pathology of congenital deafness. Journal of Laryngology and Otology 74: 919–950.CrossRefGoogle ScholarPubMed
Page, E. L., Eby, T. L. (1997) Meningitis after cochlear implantation in Mondini malformation. Otolaryngology – Head and Neck Surgery 116(1): 104–106.Google ScholarPubMed
Paparella, M. M. (1980) Mondini's deafness. A review of histopathology.Annals of Otology, Rhinology Laryngology 89 (Suppl 69): 1–10.CrossRefGoogle ScholarPubMed
Phelps, P. D. (1990) Mondini and ‘Pseudo Mondini’. Clinical Otolaryngology 15: 99–101.CrossRefGoogle ScholarPubMed
Phelps, P. D. (1992) The basal turn of the cochlea. British Journal of Radiology 65: 370–374.CrossRefGoogle ScholarPubMed
Phelps, P. D. (1994) Ear dysplasia after Mondini. Journal of Laryngology and Otology 108: 461–465.CrossRefGoogle ScholarPubMed
Phelps, P. D., King, A., Michaels, L. (1994) Cochlear dysplasia and meningitis. American Journal of Otology 15: 551–557.Google ScholarPubMed
Phelps, P. D., Michaels, L. S. (1995) The common cavity congenital deformity of the inner ear. ORL 57: 228–231.CrossRefGoogle ScholarPubMed
Phelps, P. D., Proops, D., Sellars, S., Evans, J., Michaels, L. (1993)Congenital cerebrospinal fluid fistula through the middle ear and meningitis. Journal of Laryngology and Otology 107: 492–495.CrossRefGoogle ScholarPubMed
Steel, K. P. (1995) Inherited hearing defects in mice. Annual Review of Genetics 29: 675–701.CrossRefGoogle ScholarPubMed
Steel, K. P., Brown, S. D. M. (1996) Genetics of deafness. Current Opinionin Neurobiology 6: 520–525.CrossRefGoogle ScholarPubMed
Steffens, M. L., Eilers, R. E., Fishman, L., Oiler, D. K., Urbano, R. C. (1994) Early vocal development in tactually aided children with severe- profound hearing loss. Journal of Speech and Hearing Research 37: 700–711.CrossRefGoogle ScholarPubMed
Woolford, T. J., Roberts, G. R., Hartley, C., Ramsden, R. T. (1995) Etiology of hearing loss and cochlear computed tomography: findings in preimplant assessment. Annals of Otology – Rhinology and Laryngology (Suppl 166): 201–206.Google ScholarPubMed
Zalzal, G. H., Shott, S. R., Towbin, R., Cotton, R. T. (1986) CT scan in the diagnosis of temporal bone diseases in children. Laryngoscope 96: 27–32.CrossRefGoogle ScholarPubMed