OBJECTIVES: To determine syndrome-specific type, severity, and prevalence of 
hearing loss to facilitate follow-up and treatment.
DESIGN: Tertiary pediatric hospital craniofacial clinic survey study. If 
insufficient or no data were available for a child, he or she was referred to an 
audiologist for pure-tone audiometry.
SETTING: Academic research facility.
PATIENTS: Information was gathered regarding 132 children and young adults with 
craniosynostosis.
MAIN OUTCOME MEASURES: The primary outcome was hearing assessment of children 
and young adults with various types of craniosynostosis. A secondary outcome was 
inference regarding the incidence of otitis media among children and young 
adults with craniosynostosis.
RESULTS: We found mild or moderate hearing loss in 44.0% of patients with Apert 
syndrome, in 28.5% with Crouzon syndrome, in 62.1% with Muenke syndrome, in 
28.6% with Saethre-Chotzen syndrome, and in 6.7% with complex craniosynostosis. 
Hearing loss was conductive in most patients with Apert, Crouzon, and 
Saethre-Chotzen syndromes and it was predominantly sensorineural in patients 
with Muenke syndrome. Sensorineural hearing loss at lower frequencies was found 
only in patients with Muenke syndrome.
CONCLUSIONS: 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.