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

Context: Otitis is common in patients with Turner syndrome (TS) and may be misdiagnosed in the presence of other causes of otalgia.

Objective: We hypothesized that stylohyoid ligament calcification (SLC), named Eagle syndrome (ES), is a common cause of otalgia in TS.

Design: Cohort of 1-year data collection.

Setting: We analyzed all consecutive women with Turner syndrome (TW).

Patients: Ninety-six TW and 55 age-paired normal control women (CW).

Intervention: Participants were asked about current or past otalgia and had bilateral tonsillar palatine palpated by the same physician.

Main outcome measures: When 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.

Results: Thirty-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).

Conclusion: ES 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.