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Otitis media with effusion and hearing loss in children with Cornelia de Lange syndrome.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Marchisio, Paola Selicorni, Angelo Pignataro, Lorenzo Milani, Donatella Baggi, Elena Lambertini, Lara Dusi, Elisa Villa, Laura Capaccio, Pasquale Cerutti, Marta Esposito, Susanna Principi, Nicola
Spatial Coverage	Italy
Description	Country affiliation: Italy Author Affiliation: Marchisio P ( Institute of Pediatrics, University of Milan, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy.)
Abstract	The development of children who have syndromes with mental retardation and/or language delay can be worsened by sensorineural or conductive hearing loss (HL). Given the existing scarcity of data, we investigated the prevalence of otitis media with effusion and/or HL in 50 children with Cornelia de Lange syndrome (CdLS) aged 1-18 years, and its impact on the children's performance. The children underwent otological and audiological examinations in order to ascertain the relative frequencies of otitis media with effusion and/or hearing impairment; their demographic and clinical data were obtained by questionnaires and from information in their medical charts. Otitis media with effusion was diagnosed in 94%, and its prevalence was similar in all age groups; HL was detected in 40 children (80%). Conductive HL due to middle ear effusion was the main cause of hearing impairment alone (60%) or in combination with sensorineural deficit (20%). HL had a negative impact on performance regardless of the type. A history of routine audiological and/or otological assessments was reported by a minority of parents. Our findings indicate that otitis media with effusion and/or HL is an important feature of children with CdLS and may negatively affect their performance. Careful follow-up throughout childhood is necessary to detect and treat any hearing loss in children with CdLS in order to minimize its impact on performance.
File Format	HTM / HTML
ISSN	15524825
Issue Number	4
Volume Number	146A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2008-02-15
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics De Lange Syndrome Complications Epidemiology Hearing Loss Otitis Media With Effusion Adolescent Child Child Development Physiology Child, Preschool Cross-sectional Studies Physiopathology Female Humans Infant Italy Male Psychomotor Performance Journal Article
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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