Life Expectancy After Diagnosis of a Vestibular Schwannoma in Patients 70 Years and Older.

Importance: Over the past decades, the number of patients, especially in the older adult patient group, diagnosed with vestibular schwannoma (VS) has increased. Assuming that older adult patients have more comorbidities, a longer recovery period after surgery, a higher rate of surgical complications, and a higher mortality rate after VS surgery, a treatment strategy for this group of patients is warranted, based on clinical evidence on postsurgical survival. Objective: To evaluate the survival after diagnosis of a VS in patients 70 years and older, treated with either observation or surgery, and to compare these findings with the life span of an age-matched background population in Denmark. Design, Setting, and Participants: This was a retrospective cohort study of 624 patients 70 years and older diagnosed with VS in Denmark from 1976 to 2016. Since 1976, all patients with a VS have been registered in a national database, which contains 3637 patients. Of the included patients in this study, 477 were treated conservatively with the "wait-and-scan" strategy, and 147 were treated surgically with removal of the tumor. The survival of the patients was compared with a matched background population in Denmark. Data analysis was performed from January 1976 to January 2017. Exposures: Surgery, radiotherapy, or none. Main Outcomes and Measures: The main outcome was survival among the patients and compared with the matched background population. Results: A total of 624 patients were included (317 female patients [50.8%] and 307 male patients [49.2%]). The mean (SD) survival in the observed patients was 9.2 (4.7) years after diagnosis, whereas for the background population, the expected survival was 11 years from the mean age at diagnosis. For the surgically treated patients, the mean (SD) survival was 11.8 (6.6) years, and expected survival was 11 years for the matched background population. The mean (SD) survival was 10.7 (5.5) years in female patients and 8.9 (5.0) years in male patients. There was no significant difference in survival between treatment modalities, irrespective of tumor size. Conclusions and Relevance: In this cohort study, survival after diagnosis of a VS in patients 70 years and older was similar in the surgical group compared with the age-matched background population. In the wait-and-scan group, the survival after diagnosis was marginally shorter, which may be associated with increased comorbidity.

Natural History of Growing Sporadic Vestibular Schwannomas During Observation: An International Multi-Institutional Study.

OBJECTIVE: Active treatment of small- or medium-sized vestibular schwannoma during wait-and-scan management is currently recommended at most centers globally once growth is detected. The primary aim of the current study was to characterize the natural history of growing sporadic vestibular schwannoma during observation. STUDY DESIGN: Cohort study. SETTING: Four tertiary referral centers across the United States and Denmark. PATIENTS: Patients with two prior MRI scans demonstrating ≥2 mm of linear growth who continued observational management. INTERVENTION: Observation with serial imaging. MAIN OUTCOME MEASURE: Subsequent linear growth-free survival (i.e., an additional ≥2 mm of growth) following initial growth of ≥2 mm from tumor size at diagnosis. RESULTS: Among 3,402 patients undergoing observation, 592 met inclusion criteria. Median age at initial growth was 66 years (IQR 59-73) for intracanalicular tumors (N = 65) and 62 years (IQR 54-70) for tumors with cerebellopontine angle extension (N = 527). The median duration of MRI surveillance following initial detection of tumor growth was 5.2 years (IQR 2.4-6.9) for intracanalicular tumors and 1.0 year (IQR 1.0-3.3) for cerebellopontine angle tumors. For intracanalicular tumors, subsequent growth-free survival rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years following the initial MRI that demonstrated growth were 77% (67-88; 49), 53% (42-67; 31), 46% (35-60; 23), 34% (24-49; 17), and 32% (22-47; 13), respectively. For cerebellopontine angle tumors, subsequent growth-free survival rates were 72% (68-76; 450), 47% (42-52; 258), 32% (28-38; 139), 26% (21-31; 82), and 22% (18-28; 57), respectively. For every 1 mm increase in magnitude of growth from diagnosis to tumor size at detection of initial growth, the HRs associated with subsequent growth were 1.64 (95% CI 1.25-2.15; p < 0.001) for intracanalicular tumors and 1.08 (95% CI 1.01-1.15; p = 0.02) for cerebellopontine angle tumors. CONCLUSIONS: Growth detected during observation does not necessarily portend future growth, especially for slowly growing tumors. Because early treatment does not confer improved long-term quality of life outcomes, toleration of some growth during observation is justifiable in appropriately selected cases.

The natural history of vestibular schwannoma growth-prospective 40-year data from an unselected national cohort.

BACKGROUND: Optimal management of vestibular schwannoma (VS) is still debated and thus international consensus has not been achieved. Treatment options are observation, radiotherapy, and surgery. Knowledge on the natural history of tumor growth is essential for choice of treatment modality. The aim is to present intra-/extrameatal tumor growth and management data from a prospective, unselected national cohort of patients diagnosed with VS during the period 1976-2015. METHODS: Since 1976, all data from patients diagnosed with sporadic VS in Denmark have been referred to our national treatment center, where they have been entered prospectively into the national database. Data on tumor localization, growth, and treatment were retrieved. Growth definition: >2 mm by linear measurement, in accordance with the Tokyo 2001 consensus-meeting recommendations. RESULTS: 3637 cases of VS were diagnosed, in which 1304 patients had surgery and 21 received radiotherapy post diagnosis. 2312 patients were observed with mean follow-up of 7.33 years. Of these, 434(19%; 102 intra-and 332 extrameatal tumors) changed to active treatment during the observation period due to tumor growth. 5 years after diagnosis, 21% of the intrameatal tumors exhibited growth during observation, whereas 37% of extrameatal tumors had grown, increasing to 25% intrameatal and 42% extrameatal after 10 years. Following growth, the intrameatal tumors were mostly observed further and the extrameatal mostly underwent surgery. Tumor growth occurred mainly within the first 5 years post diagnosis. CONCLUSION: This natural history study documents the growth occurrence of both intra-and extrameatal VS during the first 12 years after diagnosis and should be used in patient counseling, management, and treatment decision making.

Epidemiology and Diagnostic Characteristics of Vestibular Schwannomas-Does Gender Matter?

INTRODUCTION: A number of epidemiological studies have reported data on, e.g., tumor size and hearing at diagnosis for patients with a vestibular schwannoma (VS), whereas only a few have touched upon the potential significance of sex. The aim of this report is thus to present gender-specific data on incidence and age, tumor localization, tumor size, and hearing loss at diagnosis. MATERIAL AND METHODS: Since 1976, various data for all patients diagnosed with a sporadic VS in Denmark have been entered prospectively into a national database. Data on sex, incidence, age, tumor localization, tumor size, and hearing (discrimination) were extracted for the period 1976 to 2015. RESULTS: Over the 40 years, 3,637 cases were diagnosed, of which 1,804 were women (50%) and 1,833 men (50%). For both sexes, an increasing incidence of tumors with a steadily decreasing size was found. Age was increasing and hearing at diagnosis was increasingly better.Previously, women had more extrameatal and thus larger tumors. During the most recent decade, more tumors were found in men and hearing at diagnosis was better for women, whereas age and tumor size were comparable. CONCLUSION: The data on gender-specific epidemiology and diagnostic characteristics show varying trends over the 40-year-period spanned. For both sexes, an increasing incidence of tumors with a steadily decreasing size was found. Age is increasing and hearing at diagnosis is increasingly better. More extrameatal and thus larger tumors were previously found in women. Contemporarily, more vestibular schwannomas are found in men and diagnostic hearing is better in women.

Epidemiology Of Vestibular Schwannomas - Prospective 40-Year Data From An Unselected National Cohort.

OBJECTIVE: Reports on the epidemiology of vestibular schwannoma (VS) indicate an increase in diagnosed cases, often based on selected materials over a limited period of time. This report presents prospective 40-year epidemiological data from an unselected national cohort of all patients diagnosed with a VS in Denmark since 1976. STUDY-DESIGN: Data on gender, age, tumor localization and size registered during the period 1976-2015 were retrieved. RESULTS: 3637 new cases of VS were diagnosed during the 40-year period. The annual number of diagnosed VS increased from 14 in 1976 to 193 in 2015. Mean extrameatal tumor size decreased from 26mm in 1976 to 13.4mm in 2015. Large and giant tumors were more frequent during the first decades, whereas predominantly smaller tumors were diagnosed during the recent years. Median age at diagnosis increased gradually from 49.2 years in 1976 to 60 years in 2015. CONCLUSION: Over the past 40 years, the incidence rate of vestibular schwannomas has increased steadily from 3 VS/million/year to 34 VS/million/year, primarily due to easier access to improved diagnostics and the finding of more tumors in older people. Concurrently, the diagnostic tumor size has decreased from 26mm to 7mm, and the age at diagnosis has increased from 49 to 60 years.

Systematic Review of Hearing Preservation in Observed Vestibular Schwannoma.

Objective This is a systematic review of the literature on the spontaneous course of hearing in patients observed with a vestibular schwannoma. Included studies are appraised using the Grading of Recommendations Assessment, Development and Evaluation system. Design PubMed, Embase, Medline, Cochrane library, and CINAHL (Cumulative Index to Nursing and Allied Health Literature) were searched for literature on hearing in patients observed with a vestibular schwannoma. Participants Of 217 evaluated papers, 15 were included, representing a total of 2,142 patients. Main Outcome Measures Hearing according to the AAO-HNS (American Academy of Otorhinolaryngology- Head and Neck Surgery) classification system. Weighted average of the proportion of patients preserving good hearing (>70% discrimination score and pure tone audiometry [PTA] < 30dB) and serviceable hearing (>50% discrimination score and PTA < 50dB) was determined. Results Fifty percent of patients presenting with good hearing at diagnosis had preserved this after a mean of 5 years of observation, whereas serviceable hearing was preserved in 54%. Patients with normal discrimination at diagnosis preserve their hearing very well. Very few studies exist on long-term hearing preservation. Conclusions After 5 years of observation, around half of patients will have preserved good or serviceable hearing. Patients with normal discrimination at diagnosis are more likely to preserve good hearing.

Wide diameter bone-anchored hearing system implants: a comparison of long-term follow-up data between tissue reduction and tissue preservation techniques.

OBJECTIVE: To present long-term data on the Wide Ponto implant bone-anchored hearing system (BAHS) in regards to implant stability, soft tissue reaction and implant loss for two case series undergone either the tissue reduction- or the tissue preservation surgical technique. METHODS: Comparison of two consecutive, prospective case series. Each case series enrolled 24 patients. The case series underwent one-stage implantation of the Wide Ponto implant BAHS using either a linear incision technique with subcutaneous reduction or a linear incision technique without subcutaneous reduction. Implant stability quotient (ISQ) values were measured using resonance frequency analysis and soft tissue reactions were graded according to Holgers' classification system. Follow-up visits were performed at 10 days, 6 weeks, 6 months, 12 months and annually up to 4 years (tissue preservation) or 5 years (tissue reduction) postoperatively. RESULTS: The two case series had homogenous patient populations and followed an identical postoperative scheme. The ISQ values increased consistently the first 12 months for both groups (p ≤ 0.001), and were higher in the tissue preservation case series, (p = 0.04, 9 mm abutment). More than 91% of the soft tissue observations were assessed as Holgers' grade 0 or 1. One implant (2.1%) was lost due to trauma. CONCLUSION: In both case series, the Wide Ponto implant showed increasing implant stability during the follow-up period from the time of surgery, irrespective of surgical technique, indicating good osseointegration. Soft tissue reactions were rare and of minor severity. Implant survival was high.

Two rare cases of laryngeal intralymphatic histiocytosis.

We report two rare cases of intralymphatic histiocytosis causing, respectively, recurrent and persistent episodes of upper airway swelling and breathing difficulties. Case 1 was a 39-year-old man who was referred with recurrent upper airway swelling causing difficulty in breathing. A direct laryngoscopy was performed under general anesthesia due to minimal effect from treatment with antibiotics and anti-oedema medication. On examination, the larynx was found to be swollen and oedematous but not inflamed. Biopsies from the aryepiglottic folds showed intralymphatic histiocytosis. The patient was extensively examined but the only abnormal finding was a low CD4 count. The breathing difficulties fluctuated during the diagnostic process and settled after a year. Case 2 was a 35-year-old man who presented with persistent laryngeal swelling. Biopsies from the epiglottis showed intralymphatic histiocytosis. Extensive investigations were performed but discovered no abnormal findings. He received CO2 laser treatment twice and the swelling decreased. Intralymphatic histiocytosis is extremely rare in upper airway pathology. It is an important differential diagnosis in patients with recurrent and chronic laryngeal swelling and dyspnoea.

Serotonergic projections from the raphe nuclei to the subthalamic nucleus; a retrograde- and anterograde neuronal tracing study.

The objective of this study is to establish which subdivision of the dorsal raphe nucleus (DRN) supplies serotonergic projections to the subthalamic nucleus (STN) in the rat brain. Several studies in recent years have shown that serotonin (5-HT) might have a therapeutic role in the most prevalent basal ganglia (BG) movement disorder, Parkinson's disease (PD), but, because of the depletion of dopaminergic input to the BG, l-DOPA has been the main treatment for PD patients. Autoradiography showed that serotonin receptors 5-HT1B and 5-HT2C and the serotonin transporter were present in STN, whereas the 5-HT1A and 5-HT2A not were present. Retrograde tracer FluoroGold or Choleratoxin subunit B were iontophoretically delivered in the STN and combined with immunohistochemistry for 5-HT in order to map the topographic organization in the dorsal raphe system. The study showed that approximately 320+/-137 neurons were retrogradely traced from each STN to the DRN, located mainly in the dorsal- and ventrolateral DRN, and of these 108+/-42 or 34% co-localized 5-HT. Additionally anterograde tracer PHA-L was injected in the DRN to confirm projections to STN and accordingly only a sparse number of axon terminals were observed in the STN.