Lim, D., Gault, E., Kubba, H., Morrissey, M., Wynne, D. and Donaldson, M. (2014) Cholesteatoma has a high prevalence in Turner syndrome, highlighting the need for earlier diagnosis and the potential benefits of otoscopy training for paediatricians. Acta Paediatrica, 103(7), e282-e287. (doi: 10.1111/apa.12622)
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Publisher's URL: http://dx.doi.org/10.1111/apa.12622
Abstract
<b>Aim</b><p></p> Girls with Turner syndrome are prone to cholesteatoma, a serious suppurative middle ear disease. We aimed to confirm its high prevalence in Turner syndrome, identify risk factors and suggest possible strategies for earlier detection.<p></p> <b>Methods</b><p></p> We reviewed 179 girls with Turner syndrome between 1989 and 2012 to identify cases of cholesteatoma.<p></p> <b>Results</b><p></p> Seven girls (3.9%) had cholesteatoma (index girls) and each was compared with three age-matched girls without cholesteatoma (comparison girls). All the index girls had either the 45,X or 45,X/46X,i(Xq) karyotypes. Nine ears were initially affected, with three recurrences in two girls. Median age at first cholesteatoma presentation was 11.9 years (range: 7.5–15.2), with otorrhoea for three (range: one to seven) months in all 12 affected ears. Index girls had a significantly higher proportion of previous recurrent acute (p = 0.007) and chronic otitis media (p = 0.008), chronic perforation (p = 0.038) aural polyps (p < 0.0001) and tympanic membrane retraction (p = 0.0001) than comparison girls.<p></p> <b>Conclusion</b><p></p> Cholesteatoma has a high prevalence in Turner syndrome. Risk factors include 45,X and 46,XiXq karyotypes; a history of chronic otitis media, tympanic membrane retraction and persistent otorrhoea; and older age. Earlier recognition of ear disease is needed and otoscopy training for paediatricians caring for Turner syndrome patients may be beneficial.
Item Type: | Articles |
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Status: | Published |
Refereed: | Yes |
Glasgow Author(s) Enlighten ID: | Wynne, Dr David and Donaldson, Dr Malcolm and Gault, Miss Emma-Jane and Kubba, Mr Haytham |
Authors: | Lim, D., Gault, E., Kubba, H., Morrissey, M., Wynne, D., and Donaldson, M. |
College/School: | College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing |
Journal Name: | Acta Paediatrica |
Journal Abbr.: | Acta Paediatr |
Publisher: | Wiley |
ISSN: | 1651-2227 |
ISSN (Online): | 1651-2227 |
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