Otologic disease in Turner syndrome

Objective: This study involved the assessment of the otologic and audiologic characteristics of a group of patients with Turner syndrome. Study Design: Prospective study consisting of a questionnaire (77 of 123 responders) and an otologic and audiologic evaluation in patients with Turner syndrome (41 participants). Setting: Tertiary academic medical setting. Patients: Children, adolescents, and adults with Turner syndrome (median age, 24 yr). Interventions: Otomicroscopy, audiometry, immittance measurements, and diagnostic imaging. Results: Otologic disease is an important characteristic in Turner syndrome. Sixty-six percent of the patients studied via the questionnaire reported a history of chronic or recurrent middle ear disease. Analysis of audiometric data in 40 patients tested reveals an equal amount of normal ears (38.8%) and pure sensorineural ears (38.8%), each constituting approximately one-third of the patient population. Pure conductive losses represent only one-fifth (21.3%) of auditory abnormality encountered. Conclusions: Careful follow-up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent sequelae. However, long-term periodic review is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.