Hearing restoration after resection of an intracanalicular vestibular schwannoma: a role for emergency surgery?

Patients with vestibular schwannomas (VSs) most commonly present with sensorineural hearing loss, which is often insidious or gradual. Up to 26% of patients may present with sudden hearing loss, however, which poses an important surgical challenge. Sudden hearing loss has been attributed to spasm or occlusion of the labyrinthine artery resulting from tumor compression, and it is usually treated with corticosteroids. Hearing preservation surgery is not usually attempted in patients who have poor or nonserviceable hearing preoperatively. The authors describe a 68-year-old man with complete deafness of the left ear since childhood, who developed sudden, profound sensorineural hearing loss in the right ear. Magnetic resonance imaging revealed a small right-sided intracanalicular tumor. Treatment with high-dose corticosteroids produced only minimal improvement in hearing. Subsequent emergency decompression and resection of a VS resulted in rapid improvement and restoration of hearing, with facial nerve preservation. Although most neurotologic lesions in patients with hearing in only one ear are managed nonsurgically, resection of small tumors in the setting of sudden hearing loss should be considered in selected cases. This finding indicates that a therapeutic window may exist during which sudden hearing loss caused by intracanalicular tumors is reversible.