Related Experiment Video
Updated: May 30, 2026
Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
Published on: November 4, 2025
Audiological profile of children and young adults with syndromic and complex craniosynostosis
Tim de Jong1, Martijn S Toll, Henriëtte H W de Gier
1Department of Plastic, Reconstructive, and Hand Surgery, Erasmus Medical Center-Sophia, Rotterdam, the Netherlands. t.dejong@erasmusmc.nl
Insights
Hearing loss is common in craniosynostosis syndromes, varying in type and severity. Early screening for conductive and sensorineural hearing loss is recommended for better management.
Area of Science:
- Genetics and Developmental Biology
- Otolaryngology
- Pediatric Medicine
Background:
- Craniosynostosis, a condition involving premature fusion of skull sutures, is associated with various genetic syndromes.
- Hearing loss is a significant comorbidity in children with craniosynostosis, impacting development and quality of life.
- Understanding syndrome-specific hearing loss patterns is crucial for timely diagnosis and intervention.
Purpose of the Study:
- To characterize the type, severity, and prevalence of hearing loss in different craniosynostosis syndromes.
- To identify specific audiometric profiles for conditions like Apert, Crouzon, Muenke, and Saethre-Chotzen syndromes.
- To inform targeted follow-up and treatment strategies for hearing impairment in this population.
Main Methods:
- A survey study was conducted at a tertiary pediatric hospital's craniofacial clinic.
- Pure-tone audiometry was performed for children lacking sufficient audiological data.
- Data were collected from 132 children and young adults diagnosed with craniosynostosis.
Main Results:
- Mild to moderate hearing loss was observed in 44.0% of Apert, 28.5% of Crouzon, 62.1% of Muenke, and 28.6% of Saethre-Chotzen syndrome patients.
- Conductive hearing loss predominated in Apert, Crouzon, and Saethre-Chotzen syndromes.
- Sensorineural hearing loss, particularly at lower frequencies, was primarily identified in Muenke syndrome patients.
Conclusions:
- Recurrent otitis media with effusion is common, leading to lifelong conductive hearing loss in syndromic craniosynostosis.
- Sensorineural hearing loss occurs across studied syndromes but is the main type in Muenke syndrome.
- Routine screening for otitis media and early detection of sensorineural hearing loss are recommended for affected individuals.
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.
Related Concept Videos
01:22Sutures of the Skull
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
01:14Cranial Bones: Superior and Posterior View
The frontal bone is the single bone that forms the forehead. At its anterior midline, between the eyebrows, there is a slight depression called the glabella. The frontal bone also forms the supraorbital margin of the orbit. Near the middle of this margin is the supraorbital foramen, the opening that provides passage for a sensory nerve to the forehead. The frontal bone is thickened just above each supraorbital margin,...
01:27Cranial Bones: Lateral View
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...
01:08Overview of the Skull
The cranial vault surrounds and protects the brain and houses the middle and inner ear structures. This cavity is bounded superiorly by the rounded top of the skull, which...
01:11The Auditory Ossicles
The aptly named stapes look very much like a stirrup. The three ossicles are unique to mammals, and each plays a role in...
01:19Cranial Nerves: Overview and Anatomy