Torticollis in children with enlarged vestibular aqueducts. (April 2020)

• Record Type: Journal Article
• Authors: Brodsky, Jacob R.; Kaur, Karampreet; Shoshany, Talia; Manganella, Juliana; Barrett, Devon; Kawai, Kosuke; Murray, Makenzie; Licameli, Greg; Albano, Victoria; Stolzer, Amanda; Kenna, Margaret
• Is Part Of: International journal of pediatric otorhinolaryngology. Volume 131(2020:Apr.)
• Publication Date: 2020-04
• Subjects: Torticollis -- Paroxysmal -- EVA -- Enlarged vestibular aqueduct -- Pendred; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Oto-rhino-laryngologie -- Périodiques; Pédiatrie -- Périodiques; 618.9209751
• DOI: 10.1016/j.ijporl.2020.109862
• Languages: English
• ISSNs: 0165-5876

Abstract

Abstract: 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.