Objective: Vestibular schwannomas (VSs) affect young patients with Neurofibromatosis 2 (NF-2) and cause very serious problems for hearing, facial expression, and brain stem function. Our objective was to determine a therapy concept for the right timing and indication of neurosurgical therapy.
Methods: In 1000 consecutive VS resections, 120 tumors in 82 patients with NF-2 were surgically treated by the same surgeon (MS) at the Department of Neurosurgery at Nordstadt Hospital from 1978 to 1993. The mean age of the patients was 27.5 years. Sixty tumors were surgically treated in 41 male patients, and 60 tumors were surgically treated in 41 female patients. Bilateral tumor resection was performed in 38 patients (76 operations, after previous partial surgery in 15 cases elsewhere), and unilateral operations were performed in 44 patients, 5 of whom had undergone ipsi- or contralateral surgery that was performed elsewhere. The operative and clinical findings are evaluated and compared with the data of patients without NF-2.
Results: In 105 cases, complete tumor resections were achieved. In 15 cases, deliberate subtotal resections were performed. These were for brain stem decompression in 4 cases and for hearing preservation in the last hearing ear in 11 cases, with successful preservation in 8 of the 11. Pre- and postoperative hearing rates were higher in male than in female patients (70% in male versus 65% in female patients before surgery and 40.5 versus 31%, respectively, after surgery). Hearing was preserved in 29 of 81 ears (36%). The rate of preservation was 24% in cases of large tumors and 57% in cases of small tumors (<30 mm). Twenty-one of 82 patients (26%) were bilaterally deaf before surgery. Twenty-five patients had uni- or bilateral hearing after surgery (i.e., 41 % of those with preoperative hearing or 30.5% of the whole group). Anatomic facial nerve preservation was achieved in 85%. The facial nerve was reconstructed intracranially at the cerebellopontine angle by sural grafting in 17 cases and by hypoglossal-facial reanimation in 5. Two deaths occurred 1 and 3 months postsurgically as a result of malignant tumor growth with brain stem dysfunction and respiratory problems. In summary, for patients with NF-2, the presentation ages are lower, tumor progression is faster, the chances of anatomic and functional nerve preservation are lower, the chances of good outcomes are best when surgery is performed early and when there is good preoperative hearing function, and the danger of sudden hearing loss is higher. The chances and danger often differ from side to side among individual patients.
Conclusion: The indication and the timing of tumor resections are in some respects different from normal VS handling and are dependent on the tumor extension and related necessity of brain stem decompression and on the auditory function. As an optimal goal, completeness of resection with functional cochlear nerve preservation is formulated, and as an acceptable compromise, subtotal microsurgical resection with functional cochlear nerve preservation in the last hearing ear is suggested.