Audiological Profile of Children and Young Adults With Syndromic and Complex Craniosynostosis

Abstract

Most patients with syndromic and complex craniosynostosis have recurrent otitis media with effusion, causing episodes of conductive hearing loss throughout their lives. Sensorineural hearing loss can occur in all 4 syndromes studied but is the primary cause of hearing loss in children and young adults with Muenke syndrome. For patients with these syndromes, we recommend routine visits to the general practitioner or otolaryngologist, depending on national standards of care, to screen for otitis media with effusion throughout life. We also advise early screening for sensorineural hearing loss among children and young adults with these syndromes.

A Preliminary Study of Hearing Loss in Children With Craniosynostosis

“…27,28 Lastly, for Crouzon, Pfeiffer, and Saethre-Chotzen syndromes, hearing loss has been described as a result of changes in skull structure and recurrent otitis media leading to CHL. 15,29,30 Overall, in our study, patients with syndromic craniosynostosis had significant odds for conductive and permanent hearing loss.…”

Hearing Loss in Sagittal Versus Metopic Non-syndromic Craniosynostosis

“…For non-syndromic changes, prior research has indicated that ranges of hearing loss are often attributed to conductive changes, likely secondary to chronic serous otitis media as well as bony changes, with some quoted rates being between 20% and 30% for isolated sagittal pattern. 4,[9][10][11]18,22 The results found herein were somewhat more optimistic, with a final hearing loss percentage of 7.9%. Regardless, this is elevated to the general population, and demands adequate index of suspicion, particularly as these can relate to cognitive, speech, and behavioral abnormalities.…”

“…The relationship between craniosynostosis, both in the syndromic and non-syndromic forms, and hearing deficits have long been identified and examined. 1,4,[9][10][11] Previous research has indicated that this can be both due to primary dystosis of aberrant bone osteosynthesis as well as secondarily though increased infections and effusions. 4,[9][10][11] This study indicates that non-syndromic sagittal and metopic craniosynostosis may have similar rates of hearing loss, and no clear identifiable clinical features are necessarily associated between hearing loss.…”

Otitis Media and Hearing Loss in Patients With Nonsyndromic Craniosynostosis: A Multicenter Study

“…They noted a predominance of CHL in this population, except in Muenke syndrome where sensorineural loss predominated (Agochukwu et al, 2014). In another study, these results were redemonstrated, however, some patients with Muenke syndrome had mixed hearing loss (De Jong, 2011). A smaller case series demonstrated that 43% of syndromic craniosynostosis children had CHL, 57% mixed hearing loss, and zero patients had pure SNHL.…”