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Objective: This study aimed to determine if hypointense cochlear magnetic resonance imaging (MRI) constructive interference in steady-state (CISS) signal correlates with hearing outcomes in conservatively managed vestibular schwannoma (VS) patients., Study Design: Retrospective review of 657 cases from 1992 to 2020., Setting: Tertiary academic referral center., Patients: A retrospective review was performed to identify conservatively managed VS patients with appropriate baseline MRI, audiology, and at least 12-month audiological follow-up. Patients were excluded if they progressed to surgery or radiotherapy in less than 12 months, bilateral tumors, or surgery on the contralateral ear., Intervention: Conservatively managed patients with CISS imaging studies and audiology testing., Main Outcome Measures: Primary outcome measure change in pure-tone average (PTA) and word recognition score (WRS). Secondary outcome measures tumor size, presence of lateral fluid cap, or cystic changes., Results: A total of 92 individuals (47% male, 58 ± 11.6 yr) met the inclusion criteria, with 36 (39%) of patients demonstrating abnormal cochlear CISS signal. At baseline, abnormal cochlear CISS signal was associated with higher intracanalicular (IC) length (7.9 versus 6.6 mm, p = 0.0177) and lower WRS (55.7 versus 78.8 dBHL, p = 0.0054). During follow-up, individuals with abnormal cochlear CISS signal had significantly higher PTA (62.4 versus 46.4 dBHL, p = 0.0010). After adjusting for baseline covariates, abnormal cochlear CISS signal was consistently associated with a greater increase in PTA of 8.3 dBHL (95% confidence interval, 2.9-13.7; p = 0.0032) from baseline when compared with the normal group., Conclusions: Abnormal cochlear signal on MRI CISS sequences is associated with poorer hearing outcomes in conservatively managed VS patients., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: To examine the utility of telemedicine in a tertiary otologic practice., Study Design: Retrospective case series., Setting: Tertiary neurotology clinic., Patients: Consecutive adult patients presenting via video visit between January 2020 and January 2021., Interventions: Televideo modality to conduct visits with patients seeking evaluation for new concerns, second opinions, or routine follow-up for established conditions., Main Outcome Measures: Success of the televideo visit defined by the televideo visit being sufficient for determining a definitive plan and not requiring deferment of recommendations for a subsequent in-person visit., Results: A total of 102 televideo visits were performed among 100 unique patients. Of those, 92 (90.2%) visits were for second opinions or evaluation of new concerns, most commonly for vestibular schwannoma (n = 32, 31.4%), followed by sensorineural hearing loss (n = 20, 19.6%). Other visits were conducted for early postoperative follow-up and established general follow-up. In 91.2% of cases (n = 93), patients were successfully evaluated and provided recommendations from the initial video visit. All visits with patients having a diagnosis of meningioma (n = 7), and nearly all with vestibular Schwannoma (97%, n = 31) and sensorineural hearing loss (95%, n = 19) were successful. Of the 79 patients offered surgery as one potential treatment option, 31 patients underwent surgery at our institution by time of review. Patients with unsuccessful visits (n = 9, 8.8%) were advised to schedule additional in-person diagnostic imaging, vestibular testing, or cochlear implant candidacy evaluation to establish a more definitive care plan., Conclusion: Virtual televideo visits were successful for a high percentage of selected patients seen at a tertiary neurotology practice, particularly those seeking evaluation of vestibular schwannoma or sensorineural hearing loss., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To report on the results of intracanalicular vestibular schwannomas (ICVS) that were managed by wait and scan and to analyze the possible predictors of tumor growth and hearing deterioration throughout the observation period., Study Design: A retrospective case series., Setting: Quaternary referral center for skull base pathologies., Patients: Patients with sporadic ICVS managed by wait and scan., Intervention: Serial resonance imaging (MRI) with size measurement and serial audiological evaluation., Main Outcome Measure: Tumor growth defined as 2 mm increase of maximal tumor diameter, further treatment, and hearing preservation either maintain initial modified Sanna hearing class, or maintain initial serviceable hearing (class A/B)., Results: 339 patients were enrolled. The mean follow-up was 36.5±31.7 months with a median of 24 months. Tumor growth occurred in 141 patients (40.6%) either as slow growth (SG) in 26.3% of cases or fast growth (FG) in 15.3% of cases. Intervention was performed in only 64 cases (18.8%). Out of 271 patients who underwent hearing analysis, 86 patients (33.5%) showed hearing deterioration to a lower hearing class of the modified Sanna classification. Tumor growth and older age were predictors of hearing deterioration. Of the 125 cases with initial serviceable hearing (Class A/B), 91 cases (72.8%) maintained serviceable hearing at last follow-up. Tumor growth and a worse initial pure tone average (PTA) were predictors of hearing deterioration., Conclusions: Wait and scan management of ICVS is a viable option and only 18.8% of patients needed further treatment. Hearing tends to deteriorate over time., Competing Interests: The authors disclose no conflicts of interest. Sponsored by Mario Sanna Foundation., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: Management of small vestibular schwannomas has evolved to where observation with interval imaging is an accepted treatment strategy. Loss of residual hearing is a known complication of observation. Magnetic resonance imaging (MRI) may provide critical information to assist in determining which tumors are at highest risk of hearing loss. We wished to determine what effect fundal cap size and cochlear fluid-attenuated inversion recovery (FLAIR) signal had on the progression of hearing loss in a large cohort of observed subjects., Study Design: Retrospective chart review., Setting: Tertiary academic referral center., Patients: Three hundred ninety-three adults with a vestibular schwannoma who underwent expectant management with serial audiograms and MRI., Interventions: Audiogram and MRI., Main Outcome Measures: Hearing outcomes included pure-tone average and word discrimination score (WRS). Cochlear FLAIR signal was measured as a ratio between the affected and nonaffected cochlea. Cerebrospinal fluid fundal cap was measured from the most lateral aspect of the tumor to the fundus of the internal auditory canal., Results: An increased cochlear FLAIR ratio was associated with a worse initial WRS (p = 0.0001, β=-0.25). A multivariate regression analysis demonstrated the variables fundal cap and initial WRS to significantly predict change in WRS over time. The larger the fundal cap size, the smaller the change in the WRS (p = 0.047, β=-0.35)., Conclusions: Cerebrospinal fluid fundal cap size predicts the natural history of hearing in vestibular schwannoma patients. The presence of a smaller fundal cap is correlated with a greater risk of progression of hearing loss and should be a variable considered in the management of small vestibular schwannomas., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma., Study Design: Modified Delphi method., Methods: The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round., Results: Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus., Conclusion: This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

Objective: Dizziness is a major contributing factor to poor quality of life for patients with vestibular schwannoma (acoustic neuroma). We wished to review the literature on interventions for balance dysfunction in these patients., Data Sources: A systematic literature review was performed identifying studies that measured balance function before and after treatment for vestibular schwannoma. Data sources include Medline (1950-present), EMBASE (1974-present), Cochrane Library (issue 3, 2008), NHS Centre of reviews and dissemination, Clinical Evidence, Cochrane central register of controlled trial, and CINAHL., Study Selection: A minimum follow-up of 6 months was required, to estimate long-term balance function. Eight articles were identified, including five studies with surgical intervention, two studies with stereotactic radiotherapy, and one comparing the two. Study design was generally poor with a high risk of bias. These studies all utilized the Dizziness Handicap Inventory (DHI) as a measure of pre- and postintervention balance function., Data Extraction: Results showed that overall DHI scores are not statistically affected by intervention irrespective of modality (surgery or stereotactic radiotherapy). Patients selected with severe dizziness, who undergo surgery, improved postoperatively. No other studies for severe dizziness were noted for comparison., Conclusions: Age, sex, and tumor size have no statistically significant effect on DHI outcomes, and no evidence to suggest which treatment modality has better dizziness related outcomes.No specific treatment modality was superior in terms of long-term balance function. Patients with severe dizziness may benefit from surgery, although no comparator studies were identified.

Objective: To determine the current epidemiology and management trends for patients with vestibular schwannomas (VS)., Study Design: Retrospective cohort study., Setting: The Surveillance, Epidemiology, and End Results (SEER) tumor registry., Patients: The SEER database was queried to identify patients diagnosed with VS from 1973 to 2015. Demographics, patient and tumor characteristics, and treatment methods were analyzed., Results: A total of 14,507 patients with VS were identified. The mean age at diagnosis was 55 ± 14.9 years. Age-adjusted incidence from 2006 to 2015 was 1.4 per 100,000 per year and remained relatively stable. Incidence across age varied with sex, as younger women and older men had increased incidences comparatively. A higher percentage of patients underwent surgery alone (43%), followed by observation (32%), radiation alone (23%), and combined radiation and surgery (2%). Age 65 and older was associated with observation (odds ratio [OR] 1.417; p = 0.029) whereas age 20 to 39 and 40 to 49 were associated with surgery (OR 2.013 and 1.935; p < 0.001). Older age was associated with radiation. Larger tumor size was associated with surgery and combined treatment. African American patients and American Indian or Alaskan Native patients were more likely to undergo observation than surgery., Conclusions: The overall incidence of VS is 1.4 per 100,000 per year and has remained relatively stable. There is a trend toward more conservative management with observation, which may be secondary to earlier diagnosis given widespread use of magnetic resonance imaging. Further studies are necessary to investigate differences in disease patterns and disparities in management.

Objective: To examine quality of life changes for patients with vestibular schwannoma (VS) undergoing observation or stereotactic radiosurgery (SRS)., Study Design: Retrospective review., Setting: Academic medical center., Patients: Patients with VS who underwent observation or SRS and had at least two audiograms and Penn Acoustic Neuroma Quality of Life (PANQOL) surveys, a quality of life survey for patients with VS., Interventions: SRS or observation., Main Outcome Measures: Pure-tone average (PTA), speech discrimination score (SDS), PANQOL score; controlling for tumor size, baseline hearing, and other factors., Results: One hundred twenty-three patients met inclusion criteria: 89 underwent observation and 34 SRS. There was no significant difference in the rate of decline measured by PTA (PTA worsened at a rate of 0.25 dB/yr more in the observation group compared with the SRS group, p = 0.77) and SDS (SDS worsened at a rate of 2.1%/yr more in the SRS group compared with the observation group, p = 0.82). Kaplan-Meier analysis demonstrated the SRS group had a higher probability to progress to class D hearing over observation (hazard ratio 7.1, p = 0.005). The rate of change of the SRS PANQOL scores was significantly improved in the total (p = 0.005) and hearing (p = 0.04) domain score compared with observation. However, both groups regress to a similar PANQOL total and hearing domain score over time., Conclusion: PANQOL scores were higher at baseline in the observation group than in the SRS group. However, over time, PANQOL scores in the observation group decreased while PANQOL scores in the SRS group increased, resulting in PANQOL scores that were equivalent by the end of follow-up.

Objective: To review the demographics, treatment modalities, and survival of children with vestibular schwannomas., Study Design: Analysis using the Surveillance, Epidemiology, and End Results (SEER) database., Subjects and Methods: Pediatric patients from birth to 18 years in the SEER database were included from 2004 to 2014 based on a diagnosis of vestibular schwannoma using the primary site International Classification of Diseases (ICD) O-3 code of C72.4: acoustic nerve and the ICD O-3 histology codes of 9540/1: neurofibromatosis, Not Otherwise Specified (NOS); 9560/0: neurilemoma, NOS; or 9570/0: neuroma, NOS., Results: One hundred forty-eight pediatric vestibular schwannomas (VSs) cases were identified. The mean age at diagnosis was 13.9 years (range, 4.0-18.0). Eighty-five (57.4%) patients were women. Seventy-seven (52.0%) patients had isolated unilateral VSs while 71 (48.0%) patients had either bilateral VSs or unilateral VSs with other brain, spinal cord, or cranial nerve tumors. Eighty two (55.4%) patients received surgical resection only, 45 (30.4%) received no treatment, 6 (4.1%) received radiation only, and 12 (8.1%) received surgery and radiation. The median tumor size for patients who received no treatment was 9.5 mm (interquartile range [IQR]: 8.0) compared with 33.5 mm (IQR: 23.0) for patients who received surgical care and 41.0 mm (IQR: 1.5) for patients who received both surgery and radiation (p < 0.001). The 5-year overall survival rate was 97%., Conclusion: Pediatric VSs tend to be diagnosed in adolescence. No men or women predominance was appreciated. Treatment varied according to tumor size. Survival rates for children with vestibular schwannomas are excellent. These data may assist healthcare providers when counseling children with vestibular schwannomas and their families.

Objective: Characterize the morbidity associated with vestibular schwannoma (VS) as documented by treating providers., Study Design: Retrospective analysis of the MarketScan database., Setting: MarketScan database., Subjects and Methods: A study of morbidity in VS patients who underwent treatment between 2002 and 2012 was conducted. A detailed account of morbidity at multiple time points before and after treatment was described., Results: Of 3,355 patients treated for VS, 2,029 (60.5%) underwent surgery and 1,326 (39.5%) radiation. The average (SD) age of diagnosis was 52.5 (12.1) years. Patients were followed for a mean (SD) of 29.1 (24.2) months before and 39.2 (30.5) months after treatment. Hearing loss (65.4%), vertigo (28.0%), tinnitus (24.7%), and headache (15.2%) were the most documented symptoms at 6 months before treatment. At this juncture, higher rates of hearing loss (71.6 versus 58.6%, p < 0.0001), vertigo (32.3 versus 21.4%, p<0.0001), and tinnitus (26.9 versus 21.5%, p=0.0004) were reported for the surgery cohort. At 6 months posttreatment, surgery cohort were reported to have higher rates of hearing loss (OR 2.15, 95% CI: 1.82-2.52), vertigo (OR 1.56, 95% CI: 1.23-1.96), balance (OR 4.17, 95% CI: 3.03-5.56), facial weakness (OR 10.0, 95% CI: 6.67-14.29), headache (OR 1.56, 95% CI: 1.23-1.96), fatigue (OR 2.22, 95% CI: 1.19-4.17), and swallowing difficulties (OR 2.33, 95% CI: 1.43-3.70) compared with radiosurgery group. Surgery patients also experienced more treatment-related complications than the radiation (28.5 versus 2.6%, p<0.0001) cohort., Conclusions: Patients who eventually underwent surgery were reported by their providers to have higher morbidity before and after treatment.

Objective: To characterize the risk of progression to nonserviceable hearing in patients with sporadic vestibular schwannomas (VS) who elect initial observation., Study Design: Retrospective case series., Setting: Two tertiary care centers., Patients: VS patients with serviceable hearing who underwent at least two audiograms and two MRI studies before intervention or loss to follow-up., Main Outcome Measure(s): Serviceable hearing, defined as the pure tone average ≤ 50 dB HL and word recognition score ≥ 50%., Results: Four-hundred sixty-six patients (median age of 57 yr and median tumor diameter of 7.3 mm) had serviceable hearing at presentation and were followed for a median of 2.3 years (IQR 1.0 - 4.0). Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years following diagnosis were 94% (91-96; 357), 77% (73-82; 172), 66% (60-73; 81), 56% (49-65; 31), and 44% (33-59; 10), respectively. Each 10-dB increase in pure-tone averages at diagnosis was associated with a 2-fold increased likelihood of developing nonserviceable hearing (hazard ratio 2.07; p < 0.001). Each 10% decrease in word recognition score was associated with a 1.5-fold increased likelihood of developing nonserviceable hearing (hazard ratio 1.48; p < 0.001)., Conclusions: Among patients with sporadic VS, good baseline word recognition score and low pure-tone average are jointly associated with maintenance of serviceable hearing. These data may be used to guide patient counseling and optimize management.

Objective: Management of small vestibular schwannomas (VSs) consists of three options: serial observation, radiosurgery, and microsurgery. The authors reported the long-term hearing outcomes after retrosigmoid tumor removal in 110 patients and hearing follow-up outcomes in 160 serial observation patients with small VSs to explore the appropriate management strategy and predictive factors of hearing preservation for small VSs., Study Design: Retrospective study., Setting: Tertiary referral center., Patients: In this study, 110 patients with small VS (purely intracanalicular/cerebellopontine angle tumor ≤15 mm) during a 15-year period, from January 2001 to December 2015, were candidates for hearing preservation surgery through retrosigmoid approach, while 160 patients were candidates for serial observation. The main outcome measure was preservation of hearing under different hearing levels, assessed with the classification of American Academy of Otolaryngology-Head and Neck Surgery., Results: Preoperative hearing levels of the 110 study patients were Class A in 49 patients, Class B in 43 patients, and Class C in 18 patients. In all surgery patients (n = 110), 97.3% (107/110) patients maintained the same level during postoperative follow-up (mean follow-up time was 49.1 ± 28.2 mo) and 86 (78.2%) had complete radiologic and audiometric data at least 4 years follow-up for review. In the 4 years follow-up surgery group (n = 86), postoperative hearing levels were Class A, B, C, and D for 22, 11, 18, and 35 patients, and postoperative rates of preservation of serviceable and useful hearing were 59.3% (51/86) and 47.1% (33/70), respectively. In serial observation group, mean follow-up time was 35.2 ± 33.1 months; mean tumor size at presentation was 8.6 ± 4.3 mm; overall mean tumor growth rate was 1.08 ± 2.3 mm/yr; serviceable hearing preservation rate of 98 patients was 54.1% (53/98) at the 5-year end point and 48.7% (37/76) at the 7-year end point., Conclusion: Tumor removal should be the first treatment option for patients with small VSs and preserved hearing, especially for young patients with good hearing; retrosigmoid approach is an effective and safe approach for small VSs removal with excellent functional outcomes; better preoperative hearing predicted a higher rate of postoperative hearing preservation; patients without fundal extension were more likely to achieve hearing preservation than those with fundal extension, but no difference had been detected when retrosigmoid removal assisted with endoscope was performed; patients with small tumors originating from SVN were more likely to achieve hearing preservation compared with those with IVN-originating tumors.

Objective: To evaluate the impact of different management options on health-related quality of life (HRQoL) in vestibular schwannoma patients., Data Sources: A systematic search of the Cochrane Database, Database of Abstracts of Reviews of Effectiveness, and the Ovid Medline & EMBASE was performed. English and German language studies published between 1980 and 2015 were considered., Study Selection: This is a systematic review of HRQoL of patients managed for vestibular schwannoma. Studies in which HRQoL after one management option were evaluated or compared with other managements or with control populations using validated or reliable questionnaires, were included., Data Extraction: The included studies were independently evaluated by two reviewers. The quality of studies was assessed and graded as per Oxford Centre of Evidence Based Medicine System., Results: Ten prospective and 29 retrospective studies were identified: microsurgery initially exerted a negative effect on HRQoL but this tended to improve with follow up. Radiotherapy had a less negative effect but with minimal change over follow up. A significant limitation was that studies did not present results stratified by tumor size. Many patients will need active treatment despite the potential for negative effects on their QoL. The concept of a minimal clinically important difference has been introduced into this field and was compared with five studies., Conclusion: A number of prospective studies are available but none yet with a disease-specific questionnaire. Heterogeneity and the methodological weaknesses of the included studies constitute the principle limitation of this review. The introduction of the minimal clinically important difference should improve the relevance of studies and allow a sensitive comparison of treatments.

Objective: Recent studies have suggested good hearing preservation following stereotactic radiosurgery (STRS) in patients with vestibular schwannomas (VS). This study aims to assess audiological outcomes in patients with growing VS treated either with STRS, or managed conservatively., Study Design: Retrospective cohort study., Setting: Tertiary referral center., Patients: Out of 540 patients with VS, 69 patients with growing VS fulfilled the inclusion criteria; 24 treated conservatively and 45 with STRS. VS were considered growing if demonstrating more than 15% tumor volume increase during 1 year of watchful waiting., Intervention: American Association of Otolaryngology-Head and Neck Surgery (AAOHNS) hearing threshold (dB averaged over 500-3000 Hz) deterioration and Gardner-Robertson class deterioration over time were used as the primary outcome measures. Rate of progression to loss of functional hearing (Gardner-Robertson class I-II) was also determined between cohorts., Results: Mean follow-up was similar between treatment cohorts (STRS = 69.6 mo, conservative management = 71.7 mo). There was no significant difference in AAOHNS deterioration (t = 1.05, df = 53, p = 0.301) or Gardner-Robertson deterioration (χ = 0.47, df = 1, p = 0.492) between cohorts. Furthermore, rate of progression to loss of functional hearing was similar between cohorts (Hazard ratio = 0.704, 95% CI 0.287-1.728, p = 0.44). In STRS patients, AAOHNS deterioration was greater in those with lower AAOHNS thresholds at diagnosis (t = -2.683, df = 28, p = 0.0121). Similarly, Gardner-Robertson deterioration was significantly more likely in STRS patients with functional hearing (Gardner-Robertson class I-II) (Adjusted odds ratio = 32.14, 95% CI 3.15-328, p = 0.0034)., Conclusions: STRS results in similar audiological outcomes compared with patients managed conservatively; Consequently, STRS given to patients with VS to preserve hearing is not justified. In contrast to recent studies, patients with preserved hearing at diagnosis have significantly greater audiological deterioration when undergoing STRS.

Objective: To evaluate the influence of marital status on sporadic vestibular schwannoma (VS) in the United States., Study Design: Analysis of the Surveillance, Epidemiology, and End Results (SEER) database., Setting: National, population-based tumor registry., Subjects and Methods: The SEER database was queried to identify all patients with sporadic VS between 2004 and 2012. Univariable and multivariable analyses were used to identify differences in tumor size at presentation, management strategy, and mortality on the basis of patient marital status., Results: Eight thousand and eight hundred thirty eight patients met inclusion criteria. When comparing patient groups on the basis of marital status, significant differences emerged. Univariable and multivariable analyses revealed that married subjects were older, had smaller tumors at presentation, and had better overall survival compared with non-married patients. After adjusting for baseline differences between groups, non-married subjects were more likely to undergo observation (odds ratio [OR] 1.2, 95% confidence interval [CI] 1.08-1.35, p = 0.009), and were less likely to undergo surgery (OR 0.85, 95% CI 0.76-0.94, p = 0.002) than married subjects, while there was no difference between groups with regard to radiation treatment (OR 0.99, 95% CI 0.88-1.11, p = 0.828). Individual differences between non-married subgroups (i.e., single, separated/divorced, and widowed) are also reported., Conclusion: Marital status influences disease presentation, treatment, and outcome in patients with sporadic VS in the United States. The authors speculate that greater social support of married subjects may drive many of these differences between groups. Future research is needed to further elucidate the underlying causes for these findings as well as the influence of other important demographic variables such as socioeconomic status and general health status.

Objective: The aim of our study was to assess quality of life (QOL) among patients who underwent microsurgical excision of vestibular schwannoma (VS) compared with those managed conservatively., Study Design: Retrospective study., Setting: Tertiary care center., Patients: There was a total sample population of 376 patients diagnosed with a unilateral VS., Intervention: A total of 223 patients with unilateral VS returned the mailed questionnaires. These were then divided into two groups-78 that had undergone microsurgical excision and 145 that were managed conservatively. Subgroups within these primary groups were created for analysis., Main Outcome Measure: The primary outcome measure was the Medical Outcomes Study 36 Items Short Form (SF-36). The Dizziness Handicap Inventory test, Hearing Handicap Inventory test, and Tinnitus Handicap Inventory were also used., Results: The surgically managed group had a worse QOL when compared with the conservatively managed group using SF-36, significantly so in the domains of physical role limitation and social functioning. Trends were seen toward a better QOL in some domains in the subgroups of male patients and patients younger than 65 years. Worse QOL scores in the Tinnitus Handicap Inventory were seen in the subgroups with larger tumor size. Finally, on correlation analysis between all handicap inventories and SF-36, handicap due to disequilibrium had the strongest correlation with worsening of QOL. In SF-36, the vitality domain showed the greatest correlation with otologic handicap overall, whereas the role emotional domain showed the least., Conclusion: This study found that worse QOL scores for surgically managed versus conservatively managed VS patients are most significant in the areas of physical role limitation and social functioning. In some areas, patients who are male and younger report better QOL. Handicap due to disequilibrium seems to have the greatest negative impact on QOL. These factors should be considered when counseling patients regarding approach to VS, in the context of an experienced management program.

Introduction: The Patient Concerns Inventory-Acoustic Neuroma (PCI-AN) was developed to explore specifically the concerns that patients would like to discuss during their clinic consultation. The PCI covers a range of issues including hearing, intimacy, fatigue, financial/benefits, relationships, regret, and support for family. It also lists multidisciplinary team (MDT) members that patients would like to see or be referred on to., Method: The PCI-AN was emailed to members of the British Acoustic Neuroma Association., Results: A total of 465 complete (54.5%) responses were received. There were 284 female and 181 male subjects. Overall, the most common treatment modality was surgical excision (47%). A quarter of the study cohort had stereotactic radiosurgery, whereas 23% were conservatively managed with interval MRI scanning. The remaining 5% had both surgery and stereotactic radiosurgery. From the 55-item PCI-AN, the most commonly selected issues that patients wanted to talk about were related to the physical and functional well-being and treatment received. Tinnitus was the most frequently selected issue (46%), followed by fatigue/tiredness (43%), dizziness (33%), further investigation (39%), acoustic neuroma treatment (38%), and energy levels (32%). More than a quarter of the respondents had fears of their acoustic neuroma recurring (29%), had concerns about their facial appearance/ palsy (29%), or suffered pain in the head and neck region (26%). The 3 health-care professionals patients most wanted to talk with either in clinic or by referral were as follows: ENT/neurosurgeon (39%), vestibular (balance) physiotherapist (39%), and audiologist (39%). Although it was unknown how many respondents had facial palsy, 21% wanted to see a facial palsy physiotherapist, and another 10% sought referral to a plastic surgeon., Conclusion: The PCI-AN has shed light on an interesting array of issues, which may be overlooked by clinicians in busy skull base clinic. The PCI-AN allows for patient-directed consultation and ultimately empowers them to be actively involved in the management of their health.

Objective: Review the useful hearing preservation and tumor control outcomes of microsurgery (MS), stereotactic radiation (SR), conservative management (CM), and chemotherapy (ChT) for Neurofibromatosis type 2 vestibular schwannomas., Data Sources: Ovid MEDLINE was used to conduct a thorough search of English-language publications dating from 1948 to February 2013., Study Selection: Patients must have had useful hearing at diagnosis and treated with one of the 4 treatments as their primary therapy. All sporadic vestibular schwannoma cases were excluded., Data Extraction: A total of 19 articles were reviewed. Hearing preservation was defined as unchanged or improved useful hearing. Tumor control was defined as no change in size or tumor regression for SR, CM, and ChT-treated cases, and as no recurrence for MS treated cases., Conclusion: Microsurgery seems to have the worse overall results, while SR has very good tumor control with poor useful hearing preservation. If a patient qualifies for CM, he is likely to show the most satisfactory results with the least treatment available. A close follow-up on ChT clinical trials and possible side-effects is warranted as preliminary short-term results are quite favorable. Additional long-term studies are required for a better understanding of this disease.

Objective: Rehabilitation of hearing is complicated in patients with profound bilateral hearing loss in the presence of sporadic vestibular schwannoma (VS) or neurofibromatosis 2 (NF2), especially if the tumor does not need to be removed. We present the outcome of patients who have had a cochlear implant in the tumor affected ear without removal of the primary tumor., Design: This is a retrospective multicentre study investigating outcomes of cochlear implantation in profoundly deaf patients with vestibular schwannoma in the implanted ear., Materials and Methods: Out of 11 implanted patients, 5 required no treatment for their tumor, whereas 6 had previously undergone radiotherapy. Nine patients experienced NF2, and 2 had unilateral VS in the only hearing ear. Postoperative hearing was assessed with open and closed set speech discrimination, including City University of New York (CUNY) in noise and Bamford, Kowal and Bench (BKB) sentence scores., Results: Patients with untreated lesions experienced marked improvement in their BKB and CUNY scores in the implanted ear and were daily cochlear implant users. The improvement was less consistent in the patients who had radiotherapy where only 1 patient attained open set speech discrimination., Conclusion: Patients with unilateral VS (sporadic or those affected with NF2) whose tumor status was stable, benefited from cochlear implantation in their tumor-affected ear. Patients who had radiotherapy also benefited from CI, but their outcomes were variable.

Objective: To compare the long-term outcome of hearing and tumor control of small vestibular schwannomas treated with conservative management or radiation therapy., Data Sources: A thorough search for English-language publications and "in-process" articles dating from 1948 to January 2011 was performed using Ovid MEDLINE., Study Selection: The principal criteria were patients with no microsurgical intervention before their treatment, a follow-up of at least 5 years, and a useful hearing level at diagnosis., Data Extraction: Fourteen studies met our criteria. Hearing preservation outcome (worse, unchanged, and better) and tumor size outcome (growth, unchanged, and regression) data were collected from the articles, as well as all other significant observations. No studies using fractionated stereotactic radiotherapy met our criteria. Stereotactic radiation was the only radiation therapy analyzed., Data Synthesis: The data were analyzed using the Pearson χ2 test., Conclusion: Current data on conservative management and stereotactic radiation do not provide enough evidence to make a clear conclusion on the outcome of useful hearing preservation in a long-term manner. In this study, however, stereotactic radiation is shown to have better tumor control rates than conservative management. Additional studies reporting long-term follow-ups of patients are required so as to provide the medical field with a better understanding of vestibular schwannoma treatment therapies.

Objectives: To measure the health-related quality of life (QoL) of patients undergoing conservative management of a vestibular schwannoma and to identify audiovestibular factors that influence health-related QoL., Study Design: Cross-sectional case-control study., Intervention: Adult patients undergoing conservative management of a sporadic vestibular schwannoma were identified from a prospectively updated database. Each patient was asked to complete a series of questionnaires, including the Short Form 36 health-related QoL instrument, the Hearing Handicap Inventory, the Tinnitus Handicap Inventory, and the Dizziness Handicap Inventory. The QoL data obtained were compared with UK normal data. Multiple linear regression was performed to identify audiovestibular factors influencing QoL., Patients: Of 241 patients still undergoing conservative management, 165 completed the questionnaires. The mean age was 66.6 years. Mean duration of follow-up was 5.7 years., Results: Physical component summary scores were significantly lower than those of the normal population. Mental component summary scores were significantly above the normal population. Regression analysis showed that dizziness handicap score and age were strong predictors of physical component summary (both p < 0.0001). Dizziness handicap score and tinnitus handicap score were significant predictors of mental component summary (p = 0.0004 and p = 0.027 respectively). However, the model only explained a small amount of the data, suggesting that there may be other factors influencing QoL., Conclusion: Dizziness is the most significant audiovestibular predictor of QoL in patients with vestibular schwannomas. Tinnitus also has an impact on mental QoL. Hearing loss does not seem to influence QoL. Other factors such as illness perception may have an important role to play in determining QoL.

Objective: The aim of the present study was to evaluate the long-term hearing during "wait and scan" management of vestibular schwannomas., Subjects: During a 33-year period, from 1976 to 2008, 1,144 patients with vestibular schwannoma were allocated to observation by the wait and scan policy, with annual magnetic resonance imaging and audiologic examination. Two complete pure-tone and speech discrimination audiograms were available for 932 patients. In 900 patients (97%), the wait and scan period was at least 1 year. In 377 patients (40%), the observation time was at least 5 years, and in 102 patients (11%), at least 10 years., Results: At diagnosis, 491 patients (53%) presented with good hearing, that is, speech discrimination better than 70%. After a mean of 4.7 years of observation, 59% of these patients spontaneously preserved good hearing. Of patients with 100% speech discrimination at diagnosis, 69% maintained good hearing after more than 10 years of observation. Of patients with only a small discrimination loss at diagnosis, 38% maintained good hearing., Conclusion: Most vestibular schwannoma patients with 100% speech discrimination at diagnosis maintain good hearing even after many years of observation.

Objective: To determine the natural history and long-term quality-of-life (QOL) outcome after conservative treatment for vestibular schwannoma., Study Design: Prospective study conducted in a university-based tertiary referral center., Patients: A total of 70 vestibular schwannoma patients who were initially included in the wait and scan protocol between January 2002 and December 2003 were followed with a mean observation time of 43 months. All patients had small- or medium-sized tumors when they were included in the protocol. QOL was measured at diagnosis and at the end of follow-up in those patients who were still conservatively treated using the Short Form 36 Health Survey (SF-36). The study group was characterized by nongrowing small tumors and relatively stable symptoms over time., Main Outcome Measures: Clinical, audiometric, radiologic, and QOL results., Results: In 44 patients (63%), growth of the tumor was not observed, and 25 (36%) tumors did grow. Of the 70 included patients, 27 patients (39%) required treatment. Forty-one patients (59%) were still conservatively treated at the end of follow-up (mean 47 +/- 16 mo). Hearing was preserved in 16 (57%) of the 28 patients with useful hearing at diagnosis. At the end of follow-up, SF-36 scores were only slightly deteriorated for almost all subscales when compared with scores at diagnosis; however, differences were statistically not significant (p > 0.05). There was no significant correlation between the presence of cochleovestibular symptoms and QOL scores (p > 0.05)., Conclusion: Conservative observation of small vestibular schwannomas may be regarded as a reasonable management option because most of these tumors do not grow during an initial period of observation. Conservative treatment of this subset of patients with small, nongrowing tumors does not significantly affect life functioning, as reflected in SF-36 survey data. However, hearing loss did progress in this population. Thus, patients should be counseled regarding this risk and generic QOL measures such as the SF-36 should be used with caution in future assessments. This study emphasizes the importance of combining generic and disease-specific QOL measures in future studies of protocols of vestibular schwannoma management.

Objective: Vestibular schwannomas (VS) can be managed by observation. The goals were to examine clinical, radiographic, and audiometric variables at presentation and during observation that may predict which patients fail conservative management., Methods: A retrospective chart review was performed of 202 patients who elected observation primarily. Data collection included presenting symptoms, symptom progression, tumor size, audiologic measures, and global clinical outcomes. Univariate and multivariate analyses were performed., Results: Follow-up ranged from 1 month to 16 years (mean, 2.48 yr). Nineteen patients (9.4%) in the study group failed. Disequilibrium as a presenting symptom appeared more often in patients who failed observation (58% versus 32%; p = 0.039), as did new-onset disequilibrium. Presenting tumor size differed for patients who failed conservative management, with a mean of 14.0 versus 8.4 mm (p = 0.0006). Neurotologic complications compared favorably to those treated with primary surgery or radiotherapy., Conclusion: Patients with subjective disequilibrium at presentation and subjective disequilibrium developed during observation may be more likely to fail conservative management. Increased tumor size at presentation also may indicate the same, although no threshold could be achieved.

Objectives: To devise a protocol for the safe surveillance of vestibular schwannomas., Study Design: Retrospective review of case records., Setting: Tertiary referral neurotology clinic., Patients: Three hundred twenty patients have been managed conservatively with a mean follow-up of 43 months since 1997. Two hundred seventy-six patients with at least 1 follow-up scan have been included in the study., Intervention: Review of case records and radiologic data to devise management protocol., Main Outcomes Measured: Tumor growth rates, timing of detection of growth in growing tumors, and timing of detection of growth in tumors growing at different rates., Results: Of 276 patients, 62 (22%) demonstrated growth. The mean growth rate for growing tumors was 4 mm annually (range, 0.5-17 mm/yr). Of the growing tumors, 65% grew slowly (0.5-5 mm/yr) and 35% grew more rapidly (>5-17 mm/yr). Four tumors displayed genuine nonlinear growth. Of the rapidly growing tumors, 16 of 19 were detected at the first follow-up magnetic resonance imaging (6 mo). Of all growing tumors, 90% were detected within 3 years. The remaining 10% were detected within a further 3 years., Conclusion: Growth is usually manifest in the first 3 years after presentation. We recommend an initial magnetic resonance imaging scan at 6 months, with scans to take place at annual intervals for 2 years. A further scan 2 years later will identify any patient with indolent tumors. Thereafter, follow-up should be lifelong every 5 years. Cystic tumors represent a particular threat to patients and should only be treated conservatively with caution.

Objective: To evaluate illness perceptions, coping behavior, and quality of life (QOL) in patients with vestibular schwannoma (VS) at diagnosis., Study Design: Prospective patient analysis., Setting: University Teaching Hospital, tertiary care clinic., Patients: Consecutive patients with VS (n = 80) completed a set of questionnaires at diagnosis to assess psychological characteristics and QOL., Intervention: Diagnostic and rehabilitative., Main Outcome Measures: Psychological characteristics and QOL, measured via questionnaires focusing on illness perceptions (Illness Perception Questionnaire Revised), coping (Utrecht Coping List), and QOL (Medical Outcomes Study 36-Item Short Form Health Survey), were compared with normative data for the general Dutch population and to data concerning patients with other illnesses., Results: The 36-Item Short Form Health Survey scores of VS patients at diagnosis were significantly decreased when compared with healthy controls, patients with head and neck cancer, benign prostate hypertrophy, chronic obstructive pulmonary disease, and deaf patients. Scores for illness perceptions were in between those of patients with acute pain and chronic pain for most subscales except illness identity, emotional representations, and illness coherence. In their coping behavior, VS patients scored lower on the subscale active coping, sought less social support, and expressed their emotions less, but also showed less passive coping compared with reference values., Conclusion: Vestibular schwannoma patients experience impaired QOL compared with healthy controls and reference groups. Their illness perceptions are in between those of patients with acute and chronic pain, and their coping behavior is less active in general. This may have implications for clinical decision making and for optimizing interaction with patients. Changing illness perceptions and coping by means of an intervention and encouraging social support by means of patient support groups may improve QOL in VS patients.

Objective: To analyze an optimal management protocol for patients 65 years or older at the time of acoustic neuroma diagnosis., Study Design: Retrospective case review., Setting: Tertiary care hospital., Patients: Two hundred sixteen patients with acoustic neuroma 65 years or older at time of diagnosis., Intervention: Patients with smaller tumors (<2.5 cm) were followed with serial magnetic resonance imaging. If significant growth occurred, they were treated with surgery. Surgery was performed at initial diagnosis on patients with larger tumors or in selected patients for hearing preservation. Stereotactic radiotherapy was performed for poor surgical candidates and for patient choice., Outcome Measures: Measurement of acoustic neuroma growth and tabulation of complications., Results: One hundred fourteen patients were initially managed by observation, 80 with surgery, and 3 with radiation therapy, with an average follow-up of 35.4 months. For patients in the observation group, average tumor growth was 1.2 mm/yr. Thirty-two patients required crossover to surgery or radiotherapy due to tumor growth (average growth, 4.1 versus 0.3 mm/yr for those remaining in the observation group). One of the patients in the observation group had a complication (0.9%)., Conclusion: Management of acoustic neuromas in elderly patients can be based on size and "biological age" criteria. Surgical treatment can safely be reserved for the few patients who have significant tumor growth.

Objective: To compare the natural history of acoustic neuroma growth to the reported growth rate of acoustic neuromas after radiosurgical therapy, a retrospective review and meta-analysis of the literature was performed. The retrospective review was of one hundred eleven patients (average age, 71 yr) who chose to have their acoustic neuromas managed conservatively in our institution. These patients underwent serial magnetic resonance imaging for assessment of tumor growth for an average period of 38 months. Growth patterns if these untreated tumors were compared to that of radiosurgically treated acoustic neuromas reported in the literature., Data Sources: The English-language literature on the topic was searched systematically by Medline and Pubmed using the following key words: acoustic neuroma, vestibular schwannoma, conservative management, conservative treatment, nonsurgical, age, elderly, growth, observation, untreated, radio-surgery, gamma knife, 13 Gy and 12 Gy. There were no limits to the year of publication., Study Selection: Articles that fulfilled inclusion criteria (methods) were studied in detail., Data Extraction: All the articles described in the study selection were used in the review., Conclusion: The average growth rate of the untreated tumors was 0.7 +/- 1.4 mm/yr. Eighty-two percent grew less than 1 mm/yr, whereas 18% grew equal to or more than 1 mm/yr. Thirteen percent grew more than 2 mm/yr, with growth being noted at an average of 2.2 years after diagnosis. This represents an 87% control rate if tumor control rate is defined as less than 2-mm growth/yr. Meta-analysis indicates that tumor control rates range in the radiosurgical literature from 86% to 100%. The mean follow-up periods in the radiosurgical literature are generally not reported. Tumor control is not uniformly defined. Based on the results of this study, there is no discernable significant difference between growth patterns of untreated acoustic neuromas and those treated radiosurgically. To establish a significant difference, longer-term follow-up studies with larger sample sizes and tumor control rates are needed. Tumor control should be defined as zero growth.

Objective: The incidence of vestibular schwannomas (VSs) approaches 20 per million/yr. As treatment may depend on tumor growth, there is a demand of a treatment strategy based on hard data on the growth pattern of these tumors. This article reports growth data registered prospectively in 552 patients., Study Design: Of the 1,818 consecutive patients, diagnosed with VS during the period from 1975 to 2005, 729 patients were allocated to observation by repetitive magnetic resonance imaging. At least two scans had been performed in 552 patients at the time of data analysis. Two hundred thirty patients had a tumor confined to the internal acoustic meatus, whereas 322 patients had a tumor with an extrameatal extension. Growth to extrameatal extension was the definition for growth in intrameatal tumors, whereas a largest diameter change of more than 2 mm was the criteria for growth/shrinkage of extrameatal tumors. The mean observation time was 3.6 years (range, 1-15 yr)., Results: Seventeen percent of the intrameatal tumors grew, whereas significantly more of the extrameatal tumors displayed growth during the period (28.9%). Growth occurred within the first 5 years after diagnosis. No correlation could be demonstrated between tumor growth rate, sex, or age., Conclusion: VS growth occurs within the first 5 years after diagnosis in a limited number of tumors, primarily in tumors with an extrameatal extension. We found no relation between tumor growth and sex or age. These findings justify primary observation of small tumors. A treatment strategy is proposed for this disease, focusing on the patient group allocated to observation.

Objective: To review contemporary molecular biological literature related to skull base tumor biology and tumorigenesis., Data Sources: PUBMED and Ovid literature searches were performed using keyword search. Only English language articles published between 1965 and December 4, 2003 were chosen., Study Selection and Data Extraction: All relevant articles from the past 8 years, as well as landmark articles in years before 1995, were retrieved and reviewed., Conclusion: Consistent progress is being made toward the molecular genetic and biological basis of the most common skull base tumors. An understanding of these mechanisms will aid the neurotologist in future diagnosis and management of the lesions.