Management strategies for bilateral vestibular schwannomas

Abstract

Bilateral vestibular schwannomas (VS) are rare. Most patients in India present late in the course of illness with large tumors and disabling deafness. Clinical presentation and management goals are different from that of unilateral VS. To highlight the differences in clinical presentations and surgical results of bilateral VS compared to unilateral VS; and, to propose a management strategy for these tumors with reference to tumor size, extent of growth and the presence or absence of hearing impairment. This is a retrospective study of 16 patients with bilateral VS treated over 10 years in a tertiary referral hospital. Assessment of VIIth and VIIIth cranial nerve function, tumor size, volume and extent of growth was performed in all patients. The management strategy was based on Samii's classification of tumor extent. All patients were operated using a retromastoid suboccipital approach. Postoperative results were analyzed and compared with those of unilateral VS. The mean age of presentation was 25.7 years. Hearing impairment was the commonest symptom. Headache with features of raised intracranial pressure were present in 10 (62.5%) patients. Giant tumors were present in seven (43.7%) patients; large tumors in eight (50%) and a medium-sized tumor in one (6.3%). Total tumor resection was achieved in 13 patients and subtotal resection in two. One patient was managed conservatively and followed up with serial CT scans. On the contralateral side, one large tumor required total excision. One medium sized tumor underwent sub-capsular excision in an attempt to preserve hearing. The facial nerve was anatomically preserved in seven (46.7%) patients and in one, the cochlear nerve was anatomically preserved. There was no peri-operative mortality. Patients with bilateral schwannomas are younger, have larger lesions, poorer preoperative hearing and are more likely to lose either auditory and/or facial nerve function during attempted total resection of the tumor. Classifying the tumors into two groups by extent, that is, tumors extending to the cerebellopontine angle cistern (T1-T3a) and, tumors extending to or compressing the brainstem (T3b to T4b), allows the surgical strategy to be defined.