Chiari Type I Malformation Presenting with Unilateral Hearing Loss

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.

Interscapular pain with Chiari Type I malformation attributed to atypical spinal accessory neuralgia

Background: The spinal accessory nerve (XI) is traditionally considered a motor nerve. However, as some studies have documented the presence of nociceptive fibers in XI, vascular XI neural compression may lead to an atypical neuralgia. Case Description: A 27-year-old male presented with a Chiari Type I malformation contributing to interscapular pain. Following a posterior fossa and microvascular decompression of XI, the patient improved, thus confirming the underlying diagnosis of a XI atypical neuralgia. Conclusion: Unilateral, posterior-interscapular deep pain may be due to an atypical spinal accessory nerve (XI) neuralgia rather than a Chiari Type I malformation or syrinx. Posterior fossa decompression, subpial tonsillar resection, and XI nerve microvascular decompression resolved this patient’s complaints. In the future, CTA or MRA vascular studies along with a balanced steady-state gradient echo MRI sequence would be better to document the presence of XI nerve neurovascular compromise.