A prediction model for recurrence after translabyrinthine surgery for vestibular schwannoma: toward personalized postoperative surveillance.

PURPOSE: The aim of this study is to compute and validate a statistical predictive model for the risk of recurrence, defined as regrowth of tumor necessitating salvage treatment, after translabyrinthine removal of vestibular schwannomas to individualize postoperative surveillance. METHODS: The multivariable predictive model for risk of recurrence was based on retrospectively collected patient data between 1995 and 2017 at a tertiary referral center. To assess for internal validity of the prediction model tenfold cross-validation was performed. A 'low' calculated risk of recurrence in this study was set at < 1%, based on clinical criteria and expert opinion. RESULTS: A total of 596 patients with 33 recurrences (5.5%) were included for analysis. The final prediction model consisted of the predictors 'age at time of surgery', 'preoperative tumor growth' and 'first postoperative MRI outcome'. The area under the receiver operating curve of the prediction model was 89%, with a C-index of 0.686 (95% CI 0.614-0.796) after cross-validation. The predicted probability for risk of recurrence was low (< 1%) in 373 patients (63%). The earliest recurrence in these low-risk patients was detected at 46 months after surgery. CONCLUSION: This study presents a well-performing prediction model for the risk of recurrence after translabyrinthine surgery for vestibular schwannoma. The prediction model can be used to tailor the postoperative surveillance to the estimated risk of recurrence of individual patients. It seems that especially in patients with an estimated low risk of recurrence, the interval between the first and second postoperative MRI can be safely prolonged.

The Course of Hearing Loss in Patients With a Progressive Vestibular Schwannoma.

OBJECTIVE: This study evaluates the natural course of hearing loss (HL) prior to treatment in patients with progressive tumors and an indication for active intervention. Evaluating this patient group specifically can put hearing outcomes after vestibular schwannoma therapy into an adequate context. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. METHODS: Inclusion criteria comprised unilateral vestibular schwannomas prior to active treatment, with ≥2 mm extracanalicular (EC) tumor growth and ≥2 audiograms. We performed a comprehensive assessment of hearing using multiple outcome parameters including (the annual decrease in) pure-tone averages (PTAs; an average of 0.5, 1, 2, and 3 kHz). Predictors for HL were evaluated (patient age, tumor size/progression, follow-up duration, baseline hearing). RESULTS: At presentation, 86% of patients suffered from sensorineural HL on the affected side (≥20 dB PTA) with a median of 39 dB (interquartile rate [IQR]: 27-51 dB). The median follow-up duration was 21 months (IQR: 13-34 months), after which 58% (187/322) of patients experienced progressive HL (≥10 dB), with a median increase of 6.4 dB/year. At the last follow-up, the median PTA was 56 dB (IQR: 37-73). Median speech discrimination scores deteriorated from 90% (IQR: 70%-100%) to 65% (IQR: 35%-100%). Tumor progression (maximal EC diameter) was significantly correlated to the progression of sensorineural HL, corrected for follow-up (F(2,228) = 10.4, p < .001, R2 = 8%). CONCLUSION: The majority of patients (58%) with radiologically confirmed progressive vestibular schwannomas experience progressive sensorineural HL during observation. Tumor progression rate, EC tumor extension, and longer follow-up are factors associated with more sensorineural HL.

Prognostic Factors for the Outcome of Translabyrinthine Surgery for Vestibular Schwannomas.

OBJECTIVE: To identify predictors of tumor recurrence and postoperative facial nerve function after translabyrinthine surgery for unilateral vestibular schwannomas. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: Between 1996 and 2017 a total of 596 patients with unilateral vestibular schwannoma underwent translabyrinthine surgery. Pre- and postoperative clinical status, radiological, and surgical findings were evaluated. INTERVENTIONS: Translabyrinthine surgery. MAIN OUTCOME MEASURES: Potential predictors for tumor recurrence and facial nerve outcome were analyzed using Cox regression and ordinal logistic regression, respectively. RESULTS: The extent of tumor removal was total in 32%, near-total in 58%, and subtotal in 10%. In 5.5% (33/596) of patients the tumor recurred. Subtotal tumor resection (p = 0.004, hazard ratios [HR] = 10.66), a young age (p = 0.008, HR = 0.96), and tumor progression preoperatively (p = 0.042, HR = 2.32) significantly increased the risk of recurrence, whereas tumor size or histologic composition did not. A good postoperative facial nerve function (House-Brackmann grade 1-2) was achieved in 85%. The risk of postoperative facial nerve paresis or paralysis increased with tumor size (p < 0.001, OR = 1.52), but was not associated with the extent of tumor removal, histologic composition, or patient demographics. CONCLUSIONS: Translabyrinthine surgery is an effective treatment for vestibular schwannoma, with a good local control rate and facial nerve outcome. The extent of tumor removal is a clinically relevant predictor for tumor recurrence, as are young patient age and preoperative tumor progression. A large preoperative tumor size is associated with a higher risk of postoperative facial nerve paresis or paralysis.

Pediatric Auditory Brainstem Implant Users Compared With Cochlear Implant Users With Additional Disabilities.

OBJECTIVES: To evaluate long-term language development in children with prelingual deafness who received auditory brainstem implants (ABIs) compared with children who received cochlear implants (CIs) at the same hospital. Additional non-auditory disabilities were taken into account. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Ten children with bilateral malformations of the cochlea and/or cochlear nerve who received ABIs, including seven with additional disabilities, and 147 children with CIs as a reference group, including 22 children with additional disabilities. INTERVENTION: ABIs were implanted at 1.3 to 6.2 years of age. Follow-up ranged from 1.1 to 7.7 years. MAIN OUTCOME MEASURES: Receptive and expressive language abilities were assessed using the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Categories of Auditory Performance (CAP), the Meaningful Use of Speech Scale (MUSS), and the Speech Intelligibility Rate (SIR). RESULTS: Of the 10 children with ABIs, seven had long-term follow-up data. Within 1 year, six of the seven children with ABIs could identify sounds, respond to speech, and use their voice to attract attention. Language skills developed at a slower rate than in children with CIs and reached the same competence level when additional disabilities were absent. These language skills matched, on average, those of children with CIs with additional disabilities. CONCLUSION: For deaf children with bilateral inner ear malformations, ABIs provide satisfactory auditory input. Children with ABIs are able to develop receptive and expressive language skills comparable to those of children with CIs with additional disabilities. Using this knowledge, preoperative parent counselling can be refined.

Mathematical Models for Tumor Growth and the Reduction of Overtreatment.

Background To improve our understanding of the natural course of head and neck paragangliomas (HNPGL) and ultimately differentiate between cases that benefit from early treatment and those that are best left untreated, we studied the growth dynamics of 77 HNPGL managed with primary observation. Methods Using digitally available magnetic resonance images, tumor volume was estimated at three time points. Subsequently, nonlinear least squares regression was used to fit seven mathematical models to the observed growth data. Goodness of fit was assessed with the coefficient of determination ( R 2 ) and root-mean-squared error. The models were compared with Kruskal-Wallis one-way analysis of variance and subsequent post-hoc tests. In addition, the credibility of predictions (age at onset of neoplastic growth and estimated volume at age 90) was evaluated. Results Equations generating sigmoidal-shaped growth curves (Gompertz, logistic, Spratt and Bertalanffy) provided a good fit (median R 2 : 0.996-1.00) and better described the observed data compared with the linear, exponential, and Mendelsohn equations ( p < 0.001). Although there was no statistically significant difference between the sigmoidal-shaped growth curves regarding the goodness of fit, a realistic age at onset and estimated volume at age 90 were most often predicted by the Bertalanffy model. Conclusions Growth of HNPGL is best described by decelerating tumor growth laws, with a preference for the Bertalanffy model. To the best of our knowledge, this is the first time that this often-neglected model has been successfully fitted to clinically obtained growth data.

Basic fibroblast growth factor and fibroblastic growth factor receptor-1 may contribute to head and neck paraganglioma development by an autocrine or paracrine mechanism.

Paragangliomas are hypervascular tumors arising from neural crest-derived paraganglia that are associated with the autonomic nerve system. Mutations in genes coding for subunits of mitochondrial complex II are associated with hereditary paragangliomas, and it has been suggested that these mutations result in a pseudohypoxic signal triggering tumorigenesis. Fibroblastic growth factors are hypoxia-inducible angiogenic stimuli that are involved in the angiogenesis and tumorigenesis of several neoplasms. It has been demonstrated that basic fibroblastic growth factor (bFGF) is a survival factor for cultured chief cells of the carotid body, capable of inducing proliferation. To examine the role of this growth factor in paragangliomas, we studied the immunohistochemical expression of bFGF and its high affinity receptor fibroblastic growth factor receptor 1 (FGFR1) in 7 normal carotid bodies and in 33 head and neck paragangliomas, including 2 malignant cases and their metastases. Immunohistochemical expression of bFGF and FGFR1 in tumors was confirmed by real-time polymerase chain reaction. FGFR1 was moderately present in carotid bodies, and there was strong and significantly enhanced cytoplasmatic staining of FGFR1 in all paragangliomas. Chief cells in carotid bodies and tumors showed strong cytoplasmatic staining for bFGF. The results indicate that FGFR1 and bFGF may contribute to the development of head and neck paragangliomas.

Intratemporal facial nerve transfer with direct coaptation to the hypoglossal nerve.

OBJECTIVE: To evaluate functional recovery after facial-hypoglossal nerve transfer with direct coaptation of the intratemporal part of the facial nerve. STUDY DESIGN: Retrospective study. SETTING: University-based tertiary referral center. PATIENTS: Nine patients who underwent facial-hypoglossal transfer surgery between 2001 and 2006 to treat a unilateral complete facial nerve palsy. INTERVENTION: The facial nerve is mobilized in the temporal bone, transsected at the second genu, transferred and directly coaptated to a partially incised hypoglossal nerve. MAIN OUTCOME MEASURES: The House-Brackmann grading system was used to evaluate facial nerve reinnervation. Tongue atrophy and movements were documented. Quality of life related to facial function was assessed using the validated Facial Disability Index. RESULTS: A House-Brackmann Grade III (86%) was achieved in six patients, and Grade IV (14%) in one patient with an average follow-up of 22 months (range, 12-48 mo). Two patients had a follow-up of less than 12 months after surgery, and reinnervation was still in progress. In none of the patients who were operated on was tongue atrophy or impaired movement observed. Postoperative Facial Disability Index scores (mean, 71.8 +/- standard deviation [SD] 10.6) for physical functioning and social functioning (mean, 85.7 +/- SD 9.8) were increased for all patients when compared with preoperative scores (mean, 28.6 +/- SD 9.0; mean, 37.7 +/- SD 14.4, respectively). CONCLUSION: The facial-hypoglossal nerve transfer with direct coaptation of the intratemporal part of the facial nerve offers good functional results with low lingual morbidity and improved quality of life. The technique is straightforward, relatively simple, and should be considered as first option for reanimation of traumatic facial nerve lesions.

BCRP expression in schwannoma, plexiform neurofibroma and MPNST.

BACKGROUND: peripheral nerve sheath tumors comprise a broad spectrum of neoplasms. Vestibular schwannomas and plexiform neurofibromas are symptomatic albeit benign, but a subset of the latter pre-malignant lesions will transform to malignant peripheral nerve sheath tumors (MPNST). Surgery and radiotherapy are the primary strategies to treat these tumors. Intrinsic resistance to drug therapy characterizes all three tumor subtypes. The breast cancer resistance protein BCRP is a transmembrane efflux transporter considered to play a key role in various biological barriers such as the blood brain barrier. At the same time it is associated with drug resistance in various tumors. Its potential role in drug resistant tumors of the peripheral nervous system is largely unknown. OBJECTIVE: to assess if BCRP is expressed in vestibular schwannomas, plexiform neurofibromas and MPNST. MATERIAL AND METHODS: immunohistochemical staining for BCRP was performed on a tissue microarray composed out of 22 vestibular schwannomas, 10 plexiform neurofibromas and 18 MPNSTs. RESULTS: sixteen out of twenty-two vestibular schwannomas (73%), nine out of ten plexiform neurofibromas (90%) and six out of eighteen MPNST (33%) expressed BCRP in the vasculature. Tumor cells were negative. CONCLUSION: BCRP is present in the vasculature of vestibular schwannomas, plexiform neurofibromas and MPSNT. Therefore, it may reduce the drug exposure of underlying tumor tissues and potentially cause failure of drug therapy.

Age and Tumor Volume Predict Growth of Carotid and Vagal Body Paragangliomas.

Objective Treatment for head and neck paragangliomas (HNGPL) can be more harmful than the disease. After diagnosis, an initial period of surveillance is often indicated, and surgery or radiotherapy is reserved for progressive disease. With the aim to optimize this "wait and scan" strategy, we studied growth and possible predictors. Design A retrospective cohort study was conducted. Setting This study was conducted at a tertiary referral center for patients with HNGPL. Methods Tumor volume was estimated for 184 SDHD -related carotid and vagal body paragangliomas using sequential magnetic resonance imaging. Cox regression was used to study predictors of tumor growth. Results The estimated fraction of growing tumors ranged from 0.42 after 1 year of follow-up to 0.85 after 11 years. A median growth rate of 10.4 and 12.0% per year was observed for carotid and vagal body tumors, respectively. Tumor location, initial volume, and age ( p < 0.05) were included in our prediction model. The probability of growth decreased with increasing age and volume, indicating a decelerating growth pattern. Conclusions We created a prediction model (available online), enabling a more individualized "wait and scan" strategy. The favorable natural course of carotid and vagal body paragangliomas was confirmed; although with long follow-up growth will be observed in most cases.

Quality of Life in 807 Patients with Vestibular Schwannoma: Comparing Treatment Modalities.

Objective In vestibular schwannoma treatment, the choice among treatment modalities is controversial. The first aim of this study was to examine the quality of life of patients with vestibular schwannoma having undergone observation, radiation therapy, or microsurgical resection. The second aim was to examine the relationship between perceived symptoms and quality of life. Last, the association between quality of life and time since treatment was studied. Study Design Cross-sectional study. Setting Tertiary referral center. Subjects and Methods A total of 1208 patients treated for sporadic vestibular schwannoma between 2004 and 2014 were mailed the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire and additional questions on symptoms associated with vestibular schwannoma. Total and domain scores were calculated and compared among treatment groups. Propensity scores were used, and results were stratified according to tumor size to control for potential confounders. Correlations were calculated to examine the relationship between self-reported symptoms and quality of life, as well as between quality of life and time since treatment. Results Patients with small tumors (≤10 mm) under observation showed a higher PANQOL score when compared with the radiation therapy and microsurgical resection groups. A strong negative correlation was found between self-reported symptoms and quality of life, with balance problems and vertigo having the largest impact. No correlation was found between PANQOL score and time since treatment. Conclusion This study suggests that patients with small vestibular schwannomas experience better quality of life when managed with observation than do patients who have undergone active treatment.

Volumetrical measurements in vestibular schwannoma, the influence of slice thickness and patient's repositioning.

OBJECTIVE: Conservative treatment in vestibular schwannomas is mainly dependent on optimal tumor size determination. The first objective of this study was to establish interobserver and intraobserver variability and the accuracy and reproducibility of three different measurement methods: one bidimensional and two volumetrical. The second objective was to evaluate the influence of the use of different magnetic resonance imaging (MRI) slice thickness and the influence of patient's repositioning on the measurements' outcome. STUDY DESIGN: Two consecutive studies have been prospectively performed, both mainly concerning volumetrical measurements. SETTING: Both studies were performed in a tertiary academic, multidisciplinary center. PATIENTS: In the first study, 19 patients were included between March 1996 and May 2002, with a total of 52 scans. The second study comprised 14 patients. All patients in the first study had at least two MRI examinations performed according to a standard protocol (T1-weighted gadolinium-enhanced, slice thickness of 3 mm, and interslice gap of 0.3 mm). The population in the second study underwent a conservative wait and scan (W&S) treatment. METHODS: Both studies are discussed separately. In the first study, all scans were measured by four investigators, two of whom performed the measurements twice using three different methods. The first method concerns a manually performed bidimensional surface measurement along the petrous pyramid. The second method concerns a semiautomatic volumetrical measurement on a computer, relying on contour detection, and the last method concerns a fully automatic volume reconstruction also performed on a computer using different gray shade scales. All 14 patients included in the second study underwent three magnetic examinations. Three different T1-weighted gadolinium-enhanced sequences were used: the first using a slice thickness of 1 mm, the second again with 1-mm slice thickness but after having repositioned the patient. In the third sequence, a slice thickness of 3 mm was used. All scans were measured by two investigators using the three different methods, as described previously. RESULTS: The manual surface method shows large intraobserver variability, and its reproducibility is significantly lower compared with volume measurements. Because of a relatively large systematic error in small tumors, sensitivity of growth detection is low. Both volumetrical methods are hardly interobserver- and intraobserver-dependent, and the gray shade method turned out to be the most accurate. Radiologic progression is only significant at a volume increase of at least 50%. The influence of patient repositioning is negligible, whereas the use of 1-mm slice thickness seems to be superior to a 3-mm slice thickness. CONCLUSION: The volumetrical gray shade method is the most accurate method to detect early tumor progression. As tumor increase of at least 50% is needed to be able to speak of statistically significant tumor growth, the absence of radiologic progression does not mean that there is no tumor growth. Repositioning of the patient has no influence on the measurements' outcome, whereas for optimal magnetic resonance imaging examinations, a 1-mm slice thickness protocol seems to be superior.

Somatic loss of maternal chromosome 11 causes parent-of-origin-dependent inheritance in SDHD-linked paraganglioma and phaeochromocytoma families.

Germline mutations in succinate dehydrogenase subunits B, C and D (SDHB, SDHC and SDHD), genes encoding subunits of mitochondrial complex II, cause hereditary paragangliomas and phaeochromocytomas. In SDHB (1p36)- and SDHC (1q21)-linked families, disease inheritance is autosomal dominant. In SDHD (11q23)-linked families, the disease phenotype is expressed only upon paternal transmission of the mutation, consistent with maternal imprinting. However, SDHD shows biallelic expression in brain, kidney and lymphoid tissues (Baysal et al., 2000). Moreover, consistent loss of the wild-type (wt) maternal allele in SDHD-linked tumours suggests expression of the maternal SDHD allele in normal paraganglia. Here we demonstrate exclusive loss of the entire maternal chromosome 11 in SDHD-linked paragangliomas and phaeochromocytomas, suggesting that combined loss of the wt SDHD allele and maternal 11p region is essential for tumorigenesis. We hypothesize that this is driven by selective loss of one or more imprinted genes in the 11p15 region. In paternally, but not in maternally derived SDHD mutation carriers, this can be achieved by a single event, that is, non-disjunctional loss of the maternal chromosome 11. Thus, the exclusive paternal transmission of the disease can be explained by a somatic genetic mechanism targeting both the SDHD gene on 11q23 and a paternally imprinted gene on 11p15.5, rather than imprinting of SDHD.

Tumor Biology of Vestibular Schwannoma: A Review of Experimental Data on the Determinants of Tumor Genesis and Growth Characteristics.

OBJECTIVE: Provide an overview of the literature on vestibular schwannoma biology with special attention to tumor behavior and targeted therapy. BACKGROUND: Vestibular schwannomas are benign tumors originating from the eighth cranial nerve and arise due to inactivation of the NF2 gene and its product merlin. Unraveling the biology of these tumors helps to clarify their growth pattern and is essential in identifying therapeutic targets. METHODS: PubMed search for English-language articles on vestibular schwannoma biology from 1994 to 2014. RESULTS: Activation of merlin and its role in cell signaling seem as key aspects of vestibular schwannoma biology. Merlin is regulated by proteins such as CD44, Rac, and myosin phosphatase-targeting subunit 1. The tumor-suppressive functions of merlin are related to receptor tyrosine kinases, such as the platelet-derived growth factor receptor and vascular endothelial growth factor receptor. Merlin mediates the Hippo pathway and acts within the nucleus by binding E3 ubiquiting ligase CRL4. Angiogenesis is an important mechanism responsible for the progression of these tumors and is affected by processes such as hypoxia and inflammation. Inhibiting angiogenesis by targeting vascular endothelial growth factor receptor seems to be the most successful pharmacologic strategy, but additional therapeutic options are emerging. CONCLUSION: Over the years, the knowledge on vestibular schwannoma biology has significantly increased. Future research should focus on identifying new therapeutic targets by investigating vestibular schwannoma (epi)genetics, merlin function, and tumor behavior. Besides identifying novel targets, testing new combinations of existing treatment strategies can further improve vestibular schwannoma therapy.

The art of perception: Patients drawing their vestibular schwannoma.

OBJECTIVES/HYPOTHESIS: Drawings made by patients are an innovative way to assess the perceptions of patients on their illness. The objective of this study, at a university tertiary referral center, on patients who have recently been diagnosed with vestibular schwannoma, was to examine whether patients' illness perceptions can be assessed by drawings and are related to their quality of life. STUDY DESIGN: Cross-sectional study. METHODS: Patients diagnosed with vestibular schwannoma (mean age [range], 55.4 [17-85] years) between April 2011 and October 2012 were included (N = 253). Sociodemographics, illness perceptions (Brief Illness Perception Questionnaire [B-IPQ]), and disease-specific quality of life (Penn Acoustic Neuroma Quality of Life [PANQOL] scale) were assessed to evaluate the impact of being diagnosed with vestibular schwannoma. Furthermore, patients' drawings of their tumor were analyzed to explore the association between illness perceptions, drawings, and quality of life. RESULTS: Comparison of the B-IPQ scores of the current sample (N = 139; response rate 54.9%) with other disease samples shows a significantly lower score for patients with vestibular schwannoma on the Coherence dimension, indicating a low understanding of the illness. Illustration of emotions (N = 12) in the drawings gave a negative association with quality of life. Intercorrelations indicate a positive association between a low amount of physical and emotional consequences of the illness and a higher score on the Balance, Hearing, and Energy dimensions of the PANQOL. CONCLUSIONS: Patients' drawings give an insight into their perception of the tumor inside their head. Use of drawings may be helpful when developing and offering self-management programs. Quality of life appears to be significantly affected by the diagnosis. LEVEL OF EVIDENCE: 4.

G2M arrest, blocked apoptosis, and low growth fraction may explain indolent behavior of head and neck paragangliomas.

Head and neck paragangliomas are characterized by unusually slow growth and a strong hereditary component, which is associated with inactivating mutations in subunits of complex II of the mitochondrial respiratory chain. It is unclear how mutations induce tumorigenesis and lead to the indolent clinical behavior that often plays a prominent role in treatment strategies. To better understand the natural course of the tumors, we studied a number of growth-related parameters in 42 hereditary and sporadic paragangliomas. Computerized image analysis showed that the fraction of Ki-67-positive cells was generally below 1%, in accordance with the slow growth. Weak or negative immunohistochemical staining indicated wild-type TP53 status, whereas p-21(waf) expression was heterogeneous. Most tumors showed strong expression of Bcl-x(L), and no apoptotic cells could be detected with the terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick-end labeling assay. Flow cytometry showed abnormal DNA content profiles in 52% of the tumors, including overt aneuploidy as well as G(2)/M arrest or tetraploidization. These results fit into a model in which a stress-activated cell cycle checkpoint at the G(2) to M transition and inhibition of apoptosis permit the expansion of only a minor fraction of cycling cells with high likelihood of polyploidization.

Head and neck paragangliomas: improved tumor detection using contrast-enhanced 3D time-of-flight MR angiography as compared with fat-suppressed MR imaging techniques.

BACKGROUND AND PURPOSE: MR imaging techniques have proved their efficacy in imaging the head and neck region. In this study, we compared T1-weighted, dual T2-weighted, and fat-suppressed MR imaging and unenhanced and contrast-enhanced 3D time-of-flight MR angiography sequences for detection of head and neck paragangliomas. METHODS: Thirty-one patients with 70 paragangliomas were examined. Four combinations of MR images were reviewed by two neuroradiologists: T1-weighted and dual T2-weighted fast spin-echo images, T1- and T2-weighted fat-suppressed fast spin-echo images, T1-weighted and contrast-enhanced T1-weighted fat-suppressed spin-echo images, and unenhanced and contrast-enhanced 3D time-of-flight MR angiograms. The randomized examinations were independently evaluated for image quality, presence of tumor, tumor size, and intratumoral flow signal intensity. The standard of reference for presence of tumor was digital subtraction angiography. Data were analyzed by using the logistic regression method. RESULTS: Mean sensitivity, specificity, and negative predictive values, respectively, were assessed by the two observers to be as follows: for dual T2-weighted fast spin-echo, 74%/99%/86%; for T2-weighted fat-suppressed fast spin-echo, 70%/100%/85%; for contrast-enhanced T1-weighted fat-suppressed spin-echo, 73%/100%/86%; and for unenhanced and contrast-enhanced 3D time-of-flight MR angiography, 89%/99%/93%. Sensitivity was significantly better for unenhanced and contrast-enhanced 3D time-of-flight MR angiography (P =.000028). More intratumoral flow signal intensity was depicted with unenhanced and contrast-enhanced 3D time-of-flight MR angiography. CONCLUSION: A combination of unenhanced and contrast-enhanced 3D time-of-flight MR angiography is superior for detecting paragangliomas and should be added to a standard imaging protocol, especially for patients with familial paragangliomas because they are more susceptible to multicentric disease.

The value of MR angiography techniques in the detection of head and neck paragangliomas.

OBJECTIVE: The objective of this study was to compare three-dimensional phase-contrast angiography (3D PCA), 2D time-of-flight (2D TOF), and 3D TOF magnetic resonance (MR) angiography and a proton density weighted technique in terms of their ability to detect head and neck paragangliomas. MATERIALS AND METHODS: 14 patients with 29 paragangliomas were examined at 1.5 T. Three MR angiography sequences (3D PCA, 2D TOF, and multi-slab 3D TOF) and a proton density (PD) weighted sequence were reviewed by four neuroradiologists. The gold standard was digital subtraction angiography. Presence of tumor was assessed in five grades of confidence. Sensitivity and specificity were calculated after dichotomizing the results. Data was analyzed using the logistic regression method. RESULTS: Mean sensitivity and specificity for the four observers were for PD: 72%/97%, for 3D PCA: 75%/90%, for 2D TOF: 66%/93%, and for 3D TOF: 90%/92%. Sensitivity was significantly better for 3D TOF MRA (P < 0.001). No substantial between-observer variation for tumor detection was present. CONCLUSION: Our results demonstrate that, using 3D TOF MRA, paragangliomas in the head and neck region can be detected with high sensitivity and specificity. Further investigation is necessary to judge the value of 3D TOF MR angiography against fat suppressed contrast enhanced T1 weighted and fat suppressed T2 weighted MR sequences to find the optimal imaging sequence for paragangliomas.

Emotional intelligence in association with quality of life in patients recently diagnosed with vestibular schwannoma.

OBJECTIVE: The objective of this study was two-fold. First, to examine the levels of emotional intelligence in patients recently diagnosed with vestibular schwannoma, in comparison to those of healthy individuals and patients with other physical illness. Second, to evaluate the correlation between Emotional Intelligence and quality of life. STUDY DESIGN: Cross sectional study in a university tertiary referral center. METHODS: Consecutive patients (mean age [range], 56.4 [17-85] yr) diagnosed with vestibular schwannoma between April 2011 and October 2012 (N = 254). Sociodemographic characteristics, clinical characteristics, disease-specific quality of life (PANQOL), and Emotional Intelligence (TEIQue-SF) were assessed by questionnaire before the start of medical treatment. RESULTS: Levels of Emotional Intelligence in patients with vestibular schwannoma (N = 178; response rate 70.1%) were significantly lower compared with healthy individuals and patients with cancer. Emotional Intelligence was highly positively correlated to disease-specific quality of life. Balance disorders and cranial nerve dysfunction made a significant negative contribution to the quality of life. For educational level, a significant positive contribution was found as well. CONCLUSION: The substantial impact of a vestibular schwannoma-diagnosis on a psychological measure (i.e., Emotional Intelligence) in the affected patients as demonstrated in our study has important clinical and research implications when developing guidelines about counselling of these patients. This also has to be taken into account when making clinical decisions about the proposed treatment. Addressing Emotional Intelligence may be helpful in the development of a self-management program for patients with vestibular schwannoma.

Validating the Penn Acoustic Neuroma Quality Of Life Scale in a sample of Dutch patients recently diagnosed with vestibular schwannoma.

OBJECTIVE: To examine the validity of the Penn Acoustic Neuroma Quality-of-Life Scale (PANQOL) in a sample of Dutch patients recently diagnosed with vestibular schwannoma. STUDY DESIGN AND SETTING: Cross-sectional study in a university tertiary referral center. METHODS: Between April 2011 and March 2012 consecutive patients (mean age, 56.4; range, 17-85 yr) diagnosed with vestibular schwannoma (n = 155) were included. The PANQOL was translated into Dutch according to the accepted rules of forward-backward translation. Quality of life at diagnosis was measured with the generic SF-36 and the disease-specific PANQOL. Factor analysis was used to explore the factor structure of the PANQOL. The scores of the patients in the current study were compared with those of patients from the United States of America. Correlations between SF-36 and PANQOL were examined to study psychometric characteristics of the PANQOL. RESULTS: One hundred nineteen patients (76.8%) completed the questionnaires. SF-36 scores are comparable to previously published studies measuring Quality of Life at diagnosis. Factor analysis on our data has confirmed the original 7-dimensional structure of the PANQOL. The PANQOL scores from the Dutch and the USA patients are comparable. Correlations between PANQOL and SF-36 dimensions corroborate the validity of the Dutch PANQOL version. CONCLUSION: Vestibular schwannoma patients experience a reduced quality of life immediately after the diagnostic process. The PANQOL seems to be a valid disease-specific measure of quality of life in Dutch patients who have recently been diagnosed with vestibular schwannoma.