abbreviatioNs AAO-HNS = American Academy of Otolaryngology-Head and Neck Surgery; CPA = cerebellopontine angle; GBI = Glasgow Benefit Inventory; HRQOL = health-related quality of life; IQR = interquartile range; MCID = minimum clinically important difference; PANQOL = Penn Acoustic Neuroma Quality-of-Life; PROMIS-10 = 10-item Patient-Reported Outcomes Measurement Information System; SF-36 = 36-Item Short Form Health Survey; SRS = stereotactic radiosurgery; VS = vestibular schwannoma. submitted March 18, 2014. accepted November 11, 2014. iNclude wheN citiNg Published online January 2, 2015; DOI: 10.3171/2014.11.JNS14594. disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Internal departmental funding was used without commercial sponsorship or support. obJect The optimal treatment for sporadic vestibular schwannoma (VS) is highly controversial. To date, the majority of studies comparing treatment modalities have focused on a narrow scope of technical outcomes including facial function, hearing status, and tumor control. Very few publications have investigated health-related quality of life (HRQOL) differences between individual treatment groups, and none have used a disease-specific HRQOL instrument. methods All patients with sporadic small- to medium-sized VSs who underwent primary microsurgery, stereotactic radiosurgery (SRS), or observation between 1998 and 2008 were identified. Subjects were surveyed via postal questionnaire using the 36-Item Short Form Health Survey (SF-36), the 10-item Patient-Reported Outcomes Measurement Information System short form (PROMIS-10), the Glasgow Benefit Inventory (GBI), and the Penn Acoustic Neuroma Quality-of-Life (PANQOL) scale. Additionally, a pool of general population adults was surveyed, providing a nontumor control group for comparison. results A total of 642 respondents were analyzed. The overall response rate for patients with VS was 79%, and the mean time interval between treatment and survey was 7.7 years. Using multivariate regression, there were no statistically significant differences between management groups with respect to the PROMIS-10 physical or mental health dimensions, the SF-36 Physical or Mental Component Summary scores, or the PANQOL general, anxiety, hearing, or energy subdomains. Patients who underwent SRS or observation reported a better total PANQOL score and higher PANQOL facial, balance, and pain subdomain scores than the microsurgical cohort (p < 0.02). The differences in scores between the nontumor control group and patients with VS were greater than differences observed between individual treatment groups for the majority of measures. coNclusioNs The differences in HRQOL outcomes following SRS, observation, and microsurgery for VS are small. Notably, the diagnosis of VS rather than treatment strategy most significantly impacts quality of life. Understanding that a large number of VSs do not grow following discovery, and that intervention does not...

Sporadic vestibular schwannoma (VS) causes unilateral hearing loss, tinnitus, vertigo and unsteadiness. In many cases, the tumour size may remain unchanged for many years following diagnosis, which is typically made by MRI. In the majority of cases the tumour is small, leaving the clinician and patient with the options of either serial scanning or active treatment by gamma knife radiosurgery (GKR) or microneurosurgery. Despite the vast number of published treatment reports, comparative studies are few, and evidence is no better than class III (May, 2006). The predominant clinical endpoints of VS treatment include tumour control, facial nerve function and hearing preservation. Less focus has been put on symptom relief and health-related quality of life (QOL). It is uncertain if treating a small tumour leaves the patient with a better chance of obtaining relief from future hearing loss, vertigo or tinnitus than by observing it without treatment. Recent data indicate that QOL is reduced in untreated VS patients, and may differ between patients who have been operated and patients treated with GKR. In the present paper we review the natural course and complaints of untreated VS patients, and the treatment alternatives and results. Furthermore, we review the literature concerning quality of life in patients with VS. Finally, we present our experience with a management strategy applied to more than 300 cases since 2001.

Vertigo is the symptom causing the most pronounced negative effect on QOL in patients with VS. The more frequent VS symptoms, unilateral hearing loss and tinnitus, seem to be less important in the patients' perception of QOL as evaluated by the questionnaires used in this study. If vertigo could be relieved by treatment, this symptom should be a primary focus when discussing treatment options in small- to medium-sized VS.

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This is the second prospective study to demonstrate better facial nerve and hearing outcomes from GKRS than from open surgery for small- and medium-sized vestibular schwannomas.

Purpose Standard surgical treatment for symptomatic cervical disc disease has been discectomy and fusion, but the use of arthroplasty, designed to preserve motion, has increased, and most studies report clinical outcome in its favor. Few of these trials, however, blinded the patients. We, therefore, conducted the Norwegian Cervical Arthroplasty Trial, and present 2-year clinical outcome after arthroplasty or fusion. Methods This multicenter trial included 136 patients with single-level cervical disc disease. The patients were randomized to arthroplasty or fusion, and blinded to the treatment modality. The surgical team was blinded to randomization until nerve root decompression was completed. Primary outcome was the self-rated Neck Disability Index. Secondary outcomes were the numeric rating scale for pain and quality of life questionnaires Short Form-36 and EuroQol-5Dimension-3 Level. Results There was a significant improvement in the primary and all secondary outcomes from baseline to 2-year follow-up for both arthroplasty and fusion (P \ 0.001), and no observed significant between-group differences at any follow-up times. However, linear mixed model analyses, correcting for baseline values, dropouts and missing data, revealed a difference in Neck Disability Index (P = 0.049), and arm pain (P = 0.027) in favor of fusion at 2 years. The duration of surgery was longer (P \ 0.001), and the frequency of reoperations higher (P = 0.029) with arthroplasty. Conclusion The present study showed excellent clinical results and no significant difference between treatments at any scheduled follow-up. However, the rate of index level reoperations was higher and the duration of surgery longer with arthroplasty.

OBJECTIVE Vestibular schwannoma (VS) is a benign tumor with associated morbidities and reduced quality of life. Except for mutations in NF2, the genetic landscape of VS remains to be elucidated. Little is known about the effect of Gamma Knife radiosurgery (GKRS) on the VS genome. The aim of this study was to characterize mutations occurring in this tumor to identify new genes and signaling pathways important for the development of VS. In addition, the authors sought to evaluate whether GKRS resulted in an increase in the number of mutations. METHODS Forty-six sporadic VSs, including 8 GKRS-treated tumors and corresponding blood samples, were subjected to whole-exome sequencing and tumor-specific DNA variants were called. Pathway analysis was performed using the Ingenuity Pathway Analysis software. In addition, multiplex ligation-dependent probe amplification was performed to characterize copy number variations in the NF2 gene, and microsatellite instability testing was done to investigate for DNA replication error. RESULTS With the exception of a single sample with an aggressive phenotype that harbored a large number of mutations, most samples showed a relatively low number of mutations. A median of 14 tumor-specific mutations in each sample were identified. The GKRS-treated tumors harbored no more mutations than the rest of the group. A clustering of mutations in the cancer-related axonal guidance pathway was identified (25 patients), as well as mutations in the CDC27 (5 patients) and USP8 (3 patients) genes. Thirty-five tumors harbored mutations in NF2 and 16 tumors had 2 mutational hits. The samples without detectable NF2 mutations harbored mutations in genes that could be linked to NF2 or to NF2-related functions. None of the tumors showed microsatellite instability. CONCLUSIONS The genetic landscape of VS seems to be quite heterogeneous; however, most samples had mutations in NF2 or in genes that could be linked to NF2. The results of this study do not link GKRS to an increased number of mutations.

In VS patients, GKRS reduces the tumor growth rate and thereby the incidence rate of new treatment about tenfold. Hearing is lost at similar rates in both groups. Symptoms and QoL seem not to be significantly affected by GKRS.