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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: [email protected].
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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

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