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Torticollis in children with enlarged vestibular aqueducts
Jacob R Brodsky1, Karampreet Kaur2, Talia Shoshany2
1Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA; Harvard Medical School, 243 Charles Street, Boston, MA, 02114, USA.
Objectives:
To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA).
Methods:
An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss.
Results:
Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA.
Conclusion:
Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.
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