Surgical decompression of Chiari I malformation for isolated progressive sensorineural hearing loss

Progressive sensorineural hearing loss has been associated with Chiari type I malformation. Retrocochlear features on auditory brainstem response testing have been reported; however, debate exists over the exact location of the defect. Surgical decompression may be beneficial if brainstem or vascular compression have played a significant role in the pathophysiology of the hearing loss. Lack of specific audiologic data before and after surgical decompression has kept surgical decision making theoretic. This report presents the case of a 10-year-old boy with a progressive, asymmetric sensorineural hearing loss without other neurologic abnormalities. He underwent a posterior fossa decompression that resulted in stable hearing over the 2 years he was followed postoperatively. Preoperative and postoperative audiologic and evoked response features are described, and the rationale for operating with symptoms limited to hearing loss is discussed.