Sensorineural hearing impairment in children with Chiari I malformation.

OBJECTIVES

We describe the prevalance of Chiari I malformation in children presenting to a pediatric tertiary care hearing loss clinic, characterize the phenotype of hearing loss in children with Chiari I malformation, and discuss the potential pathophysiology of sensorineural hearing impairment (SNHI) related to Chiari I malformation.

METHODS

This study was a retrospective case series of patients seen in a pediatric tertiary care hearing loss clinic affiliated with an academic medical center. We considered 481 patients with SNHI who had radiographic imaging as part of the evaluation for the cause of their hearing impairment. Hearing impairment was determined by behavioral audiogram and/or auditory brain stem response testing. Radiographic imaging was performed with computed tomography and/or magnetic resonance imaging.

RESULTS

Six children with SNHI also had a diagnosis of Chiari I malformation. Four of these 6 children had unilateral hearing impairment. One child had asymmetric bilateral impairment; 1 child had symmetric bilateral impairment. The severity of hearing impairment varied from mild to profound. All children were referred to the neurosurgery department. None underwent surgical decompression.

CONCLUSIONS

Chiari I malformation may be associated with SNHI. Central nervous system imaging for the evaluation of children with SNHI may be indicated, particularly in individuals with negative results on temporal bone computed tomography and genetic testing.