Prevalence, risk factors and management strategies for otological problems in girls with Turner syndrome
Abstract
AIM: To determine the prevalence and risk factors of ear disease in Turner syndrome (TS), propose an algorithm for future surveillance and recommend preventative strategies. METHODS: Review of TS patients seen in the West of Scotland between 1989 and 2015, with questionnaire follow-up in 2015. RESULTS: Of 168 girls, median age 27.3 (3.8-47.2) years, ear problems occurred more frequently with 45,X and 45,X/46,XiXq than other karyotypes: 71/103 (69%) versus 23/65 (35%). Recurrent acute otitis media (AOM) first developed at 0-5 years in 23 (40%) girls, persisting in 16 (10%) at 5-10 years; and first developing at 5-10 years in 11 (7%). Persistent otitis media with effusion (OME) first developed at 0-5 and 5-10 years in 23 (40%) and 14 (8%) girls. Recurrent AOM was significantly linked with cholesteatoma in 8 (4.9%) girls (7 aged >10 years). Permanent hearing loss was documented in 28 girls (16.7%), with 16 (9.5%) receiving hearing aids (bone-anchored in 3). CONCLUSION: Acute otitis media and OME occur commonly in preschool TS girls and may persist or newly develop in later childhood. Recurrent AOM predisposes to cholesteatoma. Strategies to reduce otological morbidity include: intensive patient education, annual audiology, vaccinations and a randomised trial of antibiotic prophylaxis in high-risk groups.
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Recurrent otitis media in early childhood has been shown to be a strong predictor of future middle ear pathologies, including tympanic membrane perforations and scarring, retractions, and cholesteatoma.
“Recurrent otitis media in early childhood has been shown to be a strong predictor of future middle ear pathologies, including tympanic membrane perforations and scarring, retractions, and cholesteatoma.655”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [655]
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- Authors
- Lim, D., Hassani, S., Lupton, K., Gault, E. J., Wynne, D., Clement, W., Kubba, H., Mason, A., & Donaldson, M.
- Affiliations
- St Peter's Hospital (GB)
- NHS Grampian (GB)
- NHS Greater Glasgow and Clyde (GB)
- University of Glasgow (GB)
- Corresponding author
- Donaldson, Malcolm
- Venue
- Acta Paediatrica
- Publisher
- Wiley
- Published
- 2019-12-29
- Volume
- 109
- Issue
- 10
- Pages
- 2075-2083
- ISSN
- 0365-1436 (ISSN-L)
- 0803-5253
- 1651-2227
- OpenAlex
- W2993632425
- Cited by
- 11
- References
- 25
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 3 · 2023: 2 · 2022: 2 · 2021: 2
- FWCI
- 0.84
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Acta Paediatrica (publishedVersion)
- PubMed (publishedVersion)
- Minerva Access (University of Melbourne) (submittedVersion)
- Topics
- Health Sciences › Medicine › Otorhinolaryngology › Ear Surgery and Otitis Media
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Life Sciences › Neuroscience › Sensory Systems › Hearing, Cochlea, Tinnitus, Genetics
- Keywords
- Turner syndrome, acute otitis media, otitis media with effusion, cholesteatoma, hearing loss, risk factors, prevalence, surveillance algorithms, antibiotic prophylaxis, vaccination, patient education
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Middle Aged, Otitis Media, Otitis Media with Effusion, Risk Factors, Turner Syndrome, Prevalence, Young Adult
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