Surgical management of otologic disease in pediatric patients with Turner syndrome

Abstract

Previous reports suggest that patients with Turner syndrome have a predisposition for acute and chronic otitis media. However, the role of early or aggressive surgical management of otologic disease has not been explored in the pediatric population. With respect to otitis media in pediatric Turner syndrome patients, we examined (1) the impact of timing and (2) the type of surgical intervention in the treatment of disease. Retrospective 10-year review of patients with Turner syndrome and chronic otitis and its surgical management at a single pediatric tertiary institution. One hundred and seventy-eight patients with Turner syndrome were evaluated at our institution from 1997 to 2007. Thirty-two (18.0%) were diagnosed with middle ear disease. Eighteen (10.1%) were referred to otolaryngology for evaluation. Average age at presentation was 4.7 years (range: 1 month to 12 years). The 18 patients referred to otolaryngology required a mean of 16 clinic visits each for otologic symptoms. A mean of 6.7 pressure equalization tubes (PET) were required per patient (range: 0-25). Middle ear effusions (n=14, 78%) along with tympanic membrane retractions and/or perforations (n=10, 55.6%) were the most common otoscopic findings. Patients with tympanic membrane retractions (8/18) required a higher average number of PET (9.1) and cumulatively underwent a total of five tympanoplasty-type procedures. Six ears had evidence of cholesteatoma. Two patients underwent myringoplasty, 6 patients underwent tympanoplasty (33.3%, mean age 11.6 years), and 3 patients (16.7%, mean age 9.4 years) underwent tympanomastoidectomy. Revision procedures were common. Older age at first PET placement was significantly correlated with the need for later tympanoplasty and/or tympanomastoidectomy operations (p<0.036). Tympanoplasty or tympanomastoidectomy patients had their first PET placed on average at 5.2 years as compared to 2.6 years in those not requiring tympanoplasty or tympanomastoidectomy operations. Recurrent and chronic otitis media is common in patients with Turner syndrome. Once established, disease is recalcitrant and leads to multiple surgical procedures. Early PET insertion is advocated to offset the future necessity of more extensive tympanic procedures.