RESUMO
INTRODUCTION: Chiari type I malformations can present in different ways, but the most frequent symptom is an occipitocervical headache. Hearing loss as the main presenting symptom is rare. CASE: A young woman with progressive left-sided unilateral hearing loss was diagnosed with a Chiari type I malformation. She underwent a suboccipital craniectomy with C1 laminectomy and duraplasty. The hearing loss had resolved postoperatively with normalization of the audiometry. CONCLUSION: Chiari type I malformation can present solely with hearing loss. Improvement after surgical decompression is possible. This phenomenon is not emphasized well enough within the neurologic community. In this report, we present a summary of the pathophysiology and management in Chiari type I malformations.