A Preliminary Study of Hearing Loss in Children With Craniosynostosis

<i>Objective</i>To describe the frequency and types of hearing loss in children with syndromic and non-syndromic craniosynostosis.<i>Design</i>Retrospective cohort study.<i>Setting</i>Large tertiary pediatric hospital.<i>Patients</i>Children with craniosynostosis that underwent at least one audiological evaluation between the years of 2010 and 2021 at a single institution. Hearing loss was defined as conductive or permanent (sensorineural or mixed).<i>Results</i>Of 130 total children examined, 107 (82.3%) had non-syndromic craniosynostosis and 23 (17.7%) had syndromic craniosynostosis. Within the non-syndromic cohort, 77 (72%) had normal hearing and 30 (28%) had hearing loss, of which 21/30 (70%) had conductive hearing loss and 9/30 (30%) had permanent hearing loss. Within the syndromic cohort, two (9.5%) had normal hearing and 21 (90.5%) had hearing loss, of which 16/21 (76.2%) had conductive hearing loss and 5/21 (23.8%) had permanent hearing loss. Multivariable analysis involving syndromic status, anatomical type of craniosynostosis, and medical complexity revealed that patients with syndromic craniosynostosis had higher odds for conductive and permanent hearing loss (49.80 OR, <i>P</i> = .002 and 34.91 OR, <i>P</i> < .05, respectively). Patients with unicoronal craniosynostosis and those with significant medical comorbidities had higher odds for permanent hearing loss (19.50 OR, <i>P</i> = .03 and 23.36 OR, <i>P</i> < .05, respectively).<i>Conclusions</i>Children with craniosynostosis had high rates of hearing loss, especially those with syndromic or unicoronal craniosynostosis and those with significant medical comorbidities. Twenty-eight percent of patients with non-syndromic craniosynostosis also had hearing loss. Larger prospective studies are necessary to more precisely estimate hearing loss associated with non-syndromic craniosynostosis.