Document Detail

Useful residual hearing despite radiological findings suggestive of anacusis.
MedLine Citation:
PMID:  10748844     Owner:  NLM     Status:  MEDLINE    
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 (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.
D E Bamiou; C O Mahoney; T Sirimanna
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of laryngology and otology     Volume:  113     ISSN:  0022-2151     ISO Abbreviation:  J Laryngol Otol     Publication Date:  1999 Aug 
Date Detail:
Created Date:  2000-04-25     Completed Date:  2000-04-25     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  8706896     Medline TA:  J Laryngol Otol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  714-6     Citation Subset:  AIM; IM    
Audiology Department, Great Ormond Street Hospital, London, UK.
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MeSH Terms
Audiometry, Evoked Response
Audiometry, Pure-Tone
Cochlear Implantation / contraindications*
Ear, Inner / abnormalities*,  radiography
Hearing Disorders / etiology
Hearing Loss, Bilateral / congenital*,  rehabilitation
Magnetic Resonance Imaging
Tomography, X-Ray Computed
Vestibulocochlear Nerve / abnormalities*

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