Stylohyoid Ligament Calcification: A Greater-Than-Expected Cause of Otalgia in Turner Syndrome.

Abstract

ContextOtitis is common in patients with Turner syndrome (TS) and may be misdiagnosed in the presence of other causes of otalgia.ObjectiveWe hypothesized that stylohyoid ligament calcification (SLC), named Eagle syndrome (ES), is a common cause of otalgia in TS.DesignCohort of 1-year data collection.SettingWe analyzed all consecutive women with Turner syndrome (TW).PatientsNinety-six TW and 55 age-paired normal control women (CW).InterventionParticipants were asked about current or past otalgia and had bilateral tonsillar palatine palpated by the same physician.Main Outcome MeasuresWhen otalgia or cervicalgia plus painful palatine tonsil palpation was positive, participants underwent facial X-ray or three-dimensional cranial CT. If SLC was >25 mm, ES was confirmed.ResultsThirty-four TW (35%) had clinical signs and 27/34 (79%) had radiologically confirmed ES. Of the TW with confirmed ES (27/96; 28%), 14 (51.9%) were inadvertently treated for recurrent otitis as a presumed cause of otalgia. Eleven of the TW with ES (26.1%) were below age 21. There was no association with karyotype, age, body mass index, or growth hormone use. Ten CW (18.2%) complained of symptoms of ES, but only 4 (7.3%) were radiologically confirmed (CW vs TW, P < 0.01), and none were <21 years old. ES occurred more at younger ages in TW (P < 0.002).ConclusionES is more prevalent in TW than in controls and occurs at younger ages. ES must be assessed as a common comorbidity of TS at any age, especially during childhood, as a differential diagnosis of otalgia.