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Published on: February 29, 2020
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
Insights
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.
Objectives:
Craniosynostosis is a devastating disorder characterized by premature closure of the cranial plates before or shortly after birth. This results in an abnormally shaped skull, face, and brain. Little is known about hearing disorders in such patients, and nothing has been published about their auditory brainstem responses. Our objective was to evaluate such patients for auditory brainstem response and hearing disorders with the long-term goal of improving patient evaluation and management.
Patients And Methods:
We evaluated the auditory brainstem responses, hearing, and brain images of children with fibroblast growth factor receptor 2 craniosynostosis (n = 11).
Results:
Prolongation of the auditory brainstem response I-to-III interpeak latency was a frequent characteristic of fibroblast growth factor receptor 2 craniosynostosis, occurring in 91% of our patients. Prolongation of the III-to-V interpeak latency was an occasional characteristic, occurring in 27% of our patients. Whenever the I-to-III interpeak latency was prolonged, wave II was always abnormal. Associated morbidities included sensorineural hearing loss (27%), recurrent otitis media (100%), and Arnold-Chiari malformation (27%). Cranial decompression improved the interpeak latencies of 2 children.
Conclusions:
These previously undocumented auditory brainstem response abnormalities reflect abnormal neural transmission, which could cause peripheral and central auditory processing disorders. We speculate that the major pathogenic basis of the I-to-III interpeak latency and wave II abnormalities is compression of the auditory nerve as it passes through the internal auditory meatus and posterior fossa, which would explain the auditory nerve hearing loss, tinnitus, and vertigo that affect these children. Awareness of these abnormalities could lead to important advancements in the auditory and neurosurgical assessment and management of this overlooked patient group. We provide recommendations for the improved assessment and management of these patients. In particular, we recommend that auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression.
