ESPE Abstracts (2015) 84 WG6.4

Management of Middle Ear and Hearing Problems in Turner Syndrome - How Can We Do Better?

David Lima, Malcolm Donaldsonb, Emma Jane Gaultc, Andrew Clementd, Haytham Kubbad & Avril Masone


aRoyal Hospital for Sick Children, Glasgow, UK; bGlasgow University School of Medicine, Glasgow, UK; cResearch Governance Office, Glasgow University, Glasgow, UK; dDepartment of Otolaryngology, Royal Hospital for Sick Children, Glasgow, UK; eEndocrine Department, Royal Hospital for Sick Children, Glasgow, UK


Background: Otological problems in Turner syndrome (TS) are notoriously common and troublesome, often requiring intervention with adeno-tonsillectomy, insertion of ventilation tubes and occasionally resulting in serious disease such as cholesteatoma.

Survey of otological problems in the West of Scotland: A case note review of 174 girls attending the Turner clinic in Glasgow, Scotland from 1989–2015 found that of 155 patients in whom data were available (current mean (range) age 26.9 (3.8–47.2) years), middle ear problems were documented in 85 (55%). Prevalence was higher with the 45, X (43/64 (67%)) and 45, X/46, XiXq (19/30 (63%)) karyotypes; lower with 45, X/46, XY (3/10(30%)) karyotype and zero in the seven patients with 45, X/46, XX. The type of middle ear problem encountered was age-related. Recurrent acute otitis media (AOM) and otitis media with effusion (OME) affected young girls – 31/60 girls with recurrent AOM were aged <5 years; while 42, 34 and 7 of 83 girls with OME were aged <5, 5–10 and >10 years. By contrast 22/32 patients with chronic perforation of tympanic membrane and 10/11 with retraction pocket were aged 5–15 years. Seven (4.5%) girls aged 11.9 (7.5–15.2) years developed cholesteatoma – a far higher prevalence than in the general population (3–6 per 100 000). Five (3.2%) girls aged <15 years were recorded as having sensori-neural hearing loss, with 10 (6.5%) receiving hearing aids. However, data are incomplete for the adult population and a questionnaire-based survey is about to be launched.

Suggested strategies to improve management of ear problems in TS: The UK TS Support Society reports that many girls present initially to the Ear Nose and Throat (ENT) clinic before TS is diagnosed, indicating the value of educating ENT specialists in early recognition. Otoscopy training should be offered to paediatricians caring for TS so that effusions, perforations and retraction pockets can be diagnosed promptly and accurately. Patients and families require written information, with clear guidelines as to when to seek medical advice (e.g. purulent/offensive ear discharge for >5 days). A prospective study examining the benefit of early antibiotic prophylaxis in girls at particular risk of middle ear sepsis (e.g. those with 45, X and 46, XiXq karotypes) should be discussed.

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