Document Detail

Management of hearing loss in Apert syndrome.
MedLine Citation:
PMID:  15949104     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Apert syndrome is one of the craniosynostosis syndromes, with a birth prevalence estimated to be between 9.9 and 15.5/million, and accounts for 4.5 per cent of craniosynostoses. Although conductive hearing loss is common in Apert syndrome there are contradicting reports regarding the cause of this hearing loss. There is also no detailed information available on the management of hearing loss in Apert syndrome. MATERIALS AND METHODS: A retrospective analysis of case notes of Apert syndrome patients seen between 1970 and 2003 at Great Ormond Street Children's Hospital, London, was undertaken. RESULTS: Seventy case notes were obtained. The incidence of congenital hearing impairment was between 3 and 6 per cent. Almost all patients had otitis media with effusion (glue ear), which tended to persist into adult life. More than 56 per cent of cases developed permanent conductive hearing loss by 10-20 years. Repeated grommet insertion was common; even though 35 per cent had trouble with ear discharge and persistent conductive hearing loss. Statistically, grommets made no difference to the risk of developing permanent hearing loss. CONCLUSION: This study, of the largest number of Apert syndrome cases assembled to date, showed that early optimization of hearing with possible hearing aids needs to be considered. Repeated grommet insertion does not help in optimizing hearing, especially if ear discharge complicates the picture.
D Rajenderkumar; D Bamiou; T Sirimanna
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of laryngology and otology     Volume:  119     ISSN:  0022-2151     ISO Abbreviation:  J Laryngol Otol     Publication Date:  2005 May 
Date Detail:
Created Date:  2005-06-13     Completed Date:  2005-07-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8706896     Medline TA:  J Laryngol Otol     Country:  England    
Other Details:
Languages:  eng     Pagination:  385-90     Citation Subset:  AIM; IM    
Department of Audiological Medicine, Great Ormond Street Children's Hospital, London, UK.
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MeSH Terms
Acrocephalosyndactylia / complications*,  surgery,  therapy
Analysis of Variance
Hearing Aids
Hearing Loss, Conductive / congenital*,  surgery,  therapy
Middle Ear Ventilation / instrumentation,  methods
Otitis Media with Effusion / complications,  therapy
Retrospective Studies
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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