Auditory Brainstem Response Abnormalities and Hearing Loss in Children With Craniosynostosis

Abstract

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.

Hearing loss in syndromic craniosynostoses: Otologic manifestations and clinical findings

“…Additionally, children with syndromic craniosynostosis may benefit from Auditory Brainstem Response (ABR) testing as part of their audiologic evaluation [99]. A study of ABR results in patients with FGFR2 related craniosynostosis, namely patients with Apert, Crouzon and Jackson-Weiss syndromes, suggest that these patients may suffer from auditory nerve compression as it passes through the posterior cranial fossa and internal acoustic meatus [99].…”

“…A study of ABR results in patients with FGFR2 related craniosynostosis, namely patients with Apert, Crouzon and Jackson-Weiss syndromes, suggest that these patients may suffer from auditory nerve compression as it passes through the posterior cranial fossa and internal acoustic meatus [99]. This was hypothesized based on the results of prolonged interpeak latencies in this subset of patients.…”

Impairment of perinatal hypoxia–ischemia to the preterm brainstem

“…The brainstem auditory evoked responses (BAERs), reflect electrophysiological activity of auditory neurons along this pathway. The responses provide important information regarding functional integrity of the brainstem in clinical problems that affect the brainstem auditory pathway, including perinatal HI [21][22][23][24][25][26][27][28]. In experimental animals with HI, BAER has been successfully used to assess neuroprotective effects of hypothermia on hypoxic-ischemic brain damage [29][30][31].…”

Hearing disorders in brainstem lesions

“…BAEPs are useful in assessing the auditory system in young children because they do not require their cooperation. Church et al (2007) reported that, in cases of craniostenosis, BAEPs showed prolongation of I-III interpeak interval in 65% of children with normal hearing. They recommend that "auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression."…”