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Auditory brainstem response abnormalities and hearing loss in children with craniosynostosis
Michael W Church1, Leslie Parent-Jenkins, Arlene A Rozzelle
1Department of Obstetrics and Gynecology, Wayne State University School of Medicine, Detroit, Michigan, USA. mchurch@med.wayne.edu
Pediatrics
|May 23, 2007
Summary
Craniosynostosis patients often show prolonged auditory brainstem response latencies, indicating neural transmission issues. Early auditory brainstem response testing is recommended for managing hearing disorders in these children.
Area of Science:
- Neuroscience
- Otolaryngology
- Genetics
Background:
- Craniosynostosis, premature cranial suture fusion, causes abnormal skull development.
- Hearing disorders and auditory brainstem response (ABR) characteristics in craniosynostosis are poorly understood.
- Fibroblast growth factor receptor 2 (FGFR2) mutations are a common genetic cause of craniosynostosis.
Observation:
- This study evaluated ABR, hearing, and brain imaging in 11 children with FGFR2 craniosynostosis.
- Prolonged I-to-III interpeak latency was observed in 91% of patients.
- Abnormal wave II was consistently associated with prolonged I-to-III latency.
Findings:
- 91% of patients exhibited prolonged auditory brainstem response I-to-III interpeak latency.
- 27% of patients had prolonged III-to-V interpeak latency.
- Associated conditions included sensorineural hearing loss (27%) and recurrent otitis media (100%).
Implications:
- ABR abnormalities suggest abnormal neural transmission and potential auditory processing disorders.
- Auditory nerve compression in the posterior fossa is a suspected cause of ABR abnormalities.
- Standardizing ABR diagnostics can improve auditory and neurosurgical management for craniosynostosis patients.
