Acute Presentation of Vestibular Schwannoma Secondary to Intratumoral Hemorrhage: A Case Report and Literature Review.

BACKGROUND: Vestibular schwannomas (VS) are common slow-growing tumors that typically present with the insidious progression of unilateral hearing loss, tinnitus, vertigo, and gait imbalance. Clinically significant intratumoral acute hemorrhage is exceedingly rare and can present with the acute onset of symptoms, neurologic deterioration, and significant dysfunction of cranial nerves VII and VIII. We discuss a 40-year-old male who developed mild hearing loss and headaches over the course of a month prior to presenting with a large acutely hemorrhagic vestibular schwannoma. Additionally, we review the current literature pertaining to this pathology.

CASE PRESENTATION: A previously healthy 40-year-old male with a one-month history of mild headaches, dizziness, and left-sided hearing loss, tinnitus, and facial numbness presented with the acute onset of severe headache, vomiting, complete left-sided hearing loss, and left-sided facial weakness. Computed tomography and magnetic resonance imaging revealed a 4.1 x 2.7 cm hemorrhagic mass in the left cerebellopontine angle most consistent with VS. The patient subsequently underwent a retrosigmoid craniotomy and resection of the tumor. Pathology was consistent with hemorrhagic VS. Imaging at one-year follow-up demonstrated no residual or recurrent disease and facial motor function had completely recovered.

CONCLUSION: Histologically, vascular abnormalities and microhemorrhages are nearly ubiquitous across vestibular schwannomas and may contribute to cystic degeneration and rapid tumor growth. However, clinically significant hemorrhage is rarely encountered and is more commonly associated with more profound neurologic sequelae and cranial nerve VII dysfunction. Surgical resection at the time of presentation should be strongly considered to remove the risk of repeat hemorrhage and further deterioration. Our case represents a typical presentation and clinical course for a patient presenting with this rarely encountered pathology.