BSPED2013 Poster Presentations (1) (89 abstracts)
Middle ear disease in Turner syndrome: prevalence and risk factors
Kenneth Lupton 1 , Emma-Jane Gault 1 , Sarah Al-Hassani 1 , Haytham Kubba 2 & Malcolm Donaldson 1
1University of Glasgow, Glasgow, UK; 2Royal Hospital for Sick Children, Glasgow, UK.
Introduction: Middle ear disease in Turner syndrome (TS) is common, often resulting in troublesome temporary hearing loss, and more rarely to serious suppurative disease with cholesteatoma formation. We have examined the prevalence and pattern of middle ear disease in our TS clinic in relation to age and karyotype.
Methods: Case note review of all girls with TS attending clinic 1989–2012, scoring the most serious middle ear problem for each as: none (0), otitis media (OM) ± effusion (OME) only (1), OME requiring intervention (e.g. grommets) (2), suppurative OM/perforation/retraction pocket (3) and cholesteatoma (4).
Results: Of 173 subjects with TS, current median (range) age 25.6 (1.8–45.3) years, data were available in 155 as shown.
Median age for girls with category 3 and 4 involvement was 9.25 (1.7–15.90) and 12.05 (7.1–15.2) years respectively, significantly older than the usual age of grommet insertion for OME (4–6 years).
| 45,X (n=70) | 45,X/46,XiXq & 46,XiXq (n=35) | 45,X/ 46,XY (n=10) | 45,X/ 46XX (n=9) | 45,X/ 47,XXX (n=11) | 45,X/ 46,XrX (n=14) | Other (n=22) | |
| 0 | 23 | 13 | 6 | 6 | 6 | 7 | 11 |
| 1 | 13 | 5 | 2 | 0 | 3 | 3 | 2 |
| 2 | 17 | 8 | 2 | 0 | 0 | 3 | 2 |
| 3 | 6 | 3 | 0 | 0 | 2 | 0 | 5 |
| 4 | 4 | 3 | 0 | 0 | 0 | 0 | 0 |
Conclusion
The prevalence of middle ear disease in our TS population is at least 54%. Girls with 45,X monosomy and isochromosome of Xq and those who experience problems (e.g. ear discharge) beyond the age of 9 years are at high risk of serious disease.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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