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Useful residual hearing despite radiological findings suggestive of anacusis

Bamiou, DE; Mahoney, CO; Sirimanna, T; (1999) Useful residual hearing despite radiological findings suggestive of anacusis. Journal of Laryngology and Otology , 113 (8) 714 - 716. 10.1017/S0022215100145001. Green open access

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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 labyrinthine dysplasia as shown by computed tomography (CT) scans and, in the first case, an absent VIIIth nerve bilaterally shown by magnetic resonance imaging (MRT). 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.

Type: Article
Title: Useful residual hearing despite radiological findings suggestive of anacusis
Open access status: An open access version is available from UCL Discovery
DOI: 10.1017/S0022215100145001
Publisher version: http://dx.doi.org/10.1017/S0022215100145001
Language: English
Additional information: Copyright Cambridge University Press 1999
Keywords: Labyrinth, Dysplasia, Cochlear implant, Inner-ear, Cochlear implantation, Malformations, Meningitis, Children, Deafness, Mondini, Dysplasia
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > The Ear Institute
URI: https://discovery.ucl.ac.uk/id/eprint/99434
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