Related Experiment Video
Updated: Jul 9, 2026
Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation
Published on: February 29, 2020
Hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis
Jeremy D Prager1, Eric W Wang, David W Molter
1Department of Otolaryngology - Head & Neck Surgery, Washington University School of Medicine, Saint Louis, MO 63110, United States. pragerj@ent.wustl.edu
Insights
Children with isolated nonsyndromic sagittal synostosis (INSS) do not have a higher risk of hearing loss compared to the general population. Conductive hearing loss, often due to middle ear effusion, is the most common type but does not appear to cause developmental issues.
Area of Science:
- Pediatric craniofacial surgery
- Audiology
- Developmental pediatrics
Background:
- Patients with isolated nonsyndromic sagittal synostosis (INSS) exhibit higher rates of speech, language, cognitive, and behavioral issues.
- The prevalence and nature of hearing abnormalities in children with craniosynostosis are not well-understood.
Purpose of the Study:
- To characterize the prevalence, severity, and type of hearing loss in pediatric patients diagnosed with isolated nonsyndromic sagittal synostosis.
Main Methods:
- A retrospective chart review was conducted on patients with isolated nonsyndromic sagittal synostosis treated at a pediatric craniofacial clinic.
- The review aimed to identify and categorize hearing loss based on audiometric data.
Main Results:
- Hearing loss was identified in 15 out of 57 patients (26%) with isolated nonsyndromic sagittal synostosis.
- The majority of hearing loss cases (12 patients) were conductive, with mild to moderate severity.
- The prevalence of conductive hearing loss (21%) in this cohort was comparable to the general pediatric population.
Conclusions:
- Conductive hearing loss, likely from middle ear effusion, is the most common auditory impairment in children with isolated nonsyndromic sagittal synostosis.
- There is no increased risk of hearing loss in this population compared to age-matched controls.
- Hearing loss does not appear to be a causative factor for the observed speech, language, cognitive, and behavioral impairments in INSS patients.
Objective:
Recent studies have shown increased rates of speech, language, cognitive and behavioral abnormalities in patients with isolated nonsyndromic sagittal synostosis. Little is known regarding the prevalence and type of hearing abnormalities in children with craniosynotosis. This study characterizes hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis.
Methods:
Retrospective chart review of isolated nonsyndromic sagittal synostosis patients in a pediatric craniofacial clinic to determine the prevalence, severity, and type of hearing loss.
Results:
Fifteen of 57 patients with isolated nonsyndromic sagittal synostosis had hearing loss (26% with a 95% confidence interval of 14.9 to 37.7%). Twelve patients demonstrated a conductive loss and three an unspecified type of loss (no bone conduction thresholds were recorded for these patients). The prevalence of conductive hearing loss in our population was 21% with a 95% confidence interval of 10.4 to 31.6%. All cases of hearing loss were mild or moderate in severity.
Conclusions:
The most common hearing impairment in patients with isolated nonsyndromic sagittal synostosis is conductive hearing loss likely secondary to middle ear effusion. These patients do not appear to have a higher frequency of middle ear effusion and conductive hearing loss than the normal population of comparable age. We conclude that there is no evidence of increased risk of hearing loss in our study population. We infer that hearing loss does not play a causative role in the elevation of risk for speech, language, behavioral and cognitive impairments which these patients experience. Health care professionals are obligated to ensure that conductive loss from middle ear effusion does not exacerbate these impairments. This obligation can safely be performed by managing hearing loss in the INSS population similar to hearing loss in comparably aged normal children.
