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Purpose: For patients with vestibular schwannomas (VS), a conservative observational approach is increasingly used. Therefore, the need for accurate and reliable volumetric tumor monitoring is important. Currently, a volumetric cutoff of 20% increase in tumor volume is widely used to define tumor growth in VS. The study investigates the tumor volume dependency on the limits of agreement (LoA) for volumetric measurements of VS by means of an inter-observer study., Methods: This retrospective study included 100 VS patients who underwent contrast-enhanced T1-weighted MRI. Five observers volumetrically annotated the images. Observer agreement and reliability was measured using the LoA, estimated using the limits of agreement with the mean (LOAM) method, and the intraclass correlation coefficient (ICC)., Results: The 100 patients had a median average tumor volume of 903 mm 3 (IQR: 193-3101). Patients were divided into four volumetric size categories based on tumor volume quartile. The smallest tumor volume quartile showed a LOAM relative to the mean of 26.8% (95% CI: 23.7-33.6), whereas for the largest tumor volume quartile this figure was found to be 7.3% (95% CI: 6.5-9.7) and when excluding peritumoral cysts: 4.8% (95% CI: 4.2-6.2)., Conclusion: Agreement limits within volumetric annotation of VS are affected by tumor volume, since the LoA improves with increasing tumor volume. As a result, for tumors larger than 200 mm 3 , growth can reliably be detected at an earlier stage, compared to the currently widely used cutoff of 20%. However, for very small tumors, growth should be assessed with higher agreement limits than previously thought., (© 2024. The Author(s).)

Objective: Head-neck paragangliomas (HNPGLs) are rare tumors with approximately half arising due to germline pathogenic variants (PVs) in succinate dehydrogenase genes (SDHx). Patients with HNPGL have heterogeneous propensity to recur and metastasize. Thus, we aim to assess prevalence and predictors of recurrent (RD) and/or metastatic disease in patients with and without SDHx-related HNPGLs., Design and Methods: This cross-sectional study used retrospective data of 214 patients enrolled in six referral centers. Data included sex, age, primary tumor treatment, location, and size, biochemical phenotype, germline PVs, presence of RD (locoregional or new tumor), and/or metastasis., Results: Patients with and without SDHx-related HNPGLs showed 74% and 40% prevalence of RD, respectively. Patients without SDHx-related HNPGLs presented with recurrent tumors only in head-neck regions. The only independent predictor for RD in the entire cohort was presence of SDHx PVs. Metastatic prevalence reached 9%-13%. For patients with SDHx-related HNPGLs, large tumor size (>2.3 cm, OR:50.0, CI:2.6-977.6), young age at initial diagnosis (<42years, OR:27.3, CI:1.8-407.2), and presence of SDHB PV (OR:15.6; CI:1.5-164.8) were independent predictors of metastasis. For patients without SDHx-related HNPGLs, only carotid-body location was an independent predictor of metastasis (OR:18.9, CI:2.0-182.5)., Conclusions: Patients without SDHx-related HNPGLs require long-term follow-up due to high prevalence of RD with imaging largely restricted to head-neck regions. As carotid-body HNPGLs have the highest metastatic risk among sporadic tumors, radical treatment with frequent follow-up is suggested until population-based data are available. Importantly, patients with SDHx-related HNPGLs might benefit from early radical treatment when tumors are still small to reduce metastatic risk., Competing Interests: Conflict of interest: The authors have no conflicts of interest to declare., (© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Objective: To assess the effect of wait and scan (W&S) and stereotactic radiosurgery (SRS) on health-related quality of life (HRQoL) over time in patients with a stable vestibular schwannoma (VS) and growing VS., Study Design: Longitudinal, multicenter, observational study., Setting: Tertiary expert center for VS (Radboudumc Nijmegen) and Gamma-Knife center ETZ Hospital Tilburg., Methods: Changes in HRQoL, measured with the Penn Acoustic Neuroma Quality-of-Life (PANQOL), and the physical and mental component summary scores (PCS and MCS, respectively) derived from the 36-Item Short Form Health Survey (SF-36), were compared among patients managed by W&S and SRS between 2017 and 2022. Second, HRQoL over time in patients with a growing VS was compared between W&S and SRS., Results: Differences in PANQOL total and subdomain scores, PCS and MCS scores over time in the W&S (n = 73) and SRS (n = 170) groups were nonsignificant and on average did not exceed the minimal clinically important differences (mean difference of -2.56 [PANQOL total], 1.22 [PCS], and -1.76 [MCS]; all P > .05). In growing VS, comparison of W&S (n = 29) and SRS (n = 154) also revealed no significant difference (mean difference of 1.19 (PANQOL total), 1.83 (PCS) and -0.12 (MCS); all P > .05)., Conclusion: Differences in HRQoL in patients with VS are minor and not significantly different or clinically relevant between patients managed with W&S or SRS. Similarly, patients with a growing VS managed with W&S or SRS exhibit no significant or clinical relevant difference in HRQoL during follow-up. This can aid clinicians in the counseling of patients regarding HRQoL when deciding on a management strategy after diagnosis or documented growth., (© 2024 The Authors. Otolaryngology–Head and Neck Surgery published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Background: Volumetric natural history studies specifically on large vestibular schwannomas (VSs), commonly classified as Koos grade 4, are lacking. The aim of the current study is to present the volumetric tumor evolution in sporadic Koos grade 4 VSs and possible predictors for tumor growth., Methods: Volumetric tumor measurements and tumor evolution patterns from serial MRI studies were analyzed from selected consecutive patients with Koos grade 4 VS undergoing initial wait-and-scan management between January 2001 and July 2020. The significant volumetric threshold was defined as a change in volume of ≥10%., Results: Among 215 tumors with a median size (IQR) of 2.7 cm 3 (1.8-4.2), 147 tumors (68%) demonstrated growth and 75 tumors (35%) demonstrated shrinkage during follow-up. Growth-free survival rates (95% CI) at 1, 2, 5, and 10 years were 55% (48-61), 36% (29-42), 29% (23-36), and 28% (21-34), respectively and did not significantly differ in tumors> 20 mm (Chi-square = .40; P -value = .53). Four tumor evolution patterns (% of total) were observed: continued growth (60); initial growth then shrinkage (7); continued shrinkage (27); and stability (5). Good hearing (adjusted HR 2.21, 95% CI 1.48-3.30; P  < .001) and peritumoral edema (adjusted HR 2.22, 95% CI 1.18-4.13; P = .01) at diagnosis were significantly associated with an increased likelihood of growth., Conclusions: Koos grade 4 VSs show a wide variety in size and growth. Due to variable growth patterns, an initial wait-and-scan strategy with short scan intervals may be an acceptable option in selected tumors, if no significant clinical symptoms of mass effect that warrant treatment are present., Competing Interests: None declared., (© The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Purpose: During counseling and management of patients with vestibular schwannoma (VS), the emphasis is shifting from tumour control and nerve preservation towards maintaining or improving health-related quality of life (HRQoL). Understanding the patients' perspective and impact of VS is, therefore, of utmost importance. The current study aimed to identify treatment outcomes preferred by patients and to explore the patient-reported VS symptoms and management-related side effects and their impact on HRQoL., Methods: Patients with VS were contacted through the Dutch VS association Stichting Hoormij and questioned using a semi-structured, cross-sectional online survey. Patients were asked to report and rank symptoms and side effects, with their impact on HRQoL and frequency of occurrence. Results were structured through qualitative content analysis. Coded symptoms, side effects, impacts, frequencies, and patient-preferred outcomes were analysed and summarized with descriptive statistics., Results: Of the 231 respondents, 71% were actively treated. Hearing (symptoms vs. side effects: 78.8% vs. 63.6%), balance (62.3%; 48.8%), and energy issues (33.8%; 32.6%) were the most frequently mentioned symptoms and management-related side effects. Fatigue, deafness, headaches, and hearing loss had the highest impact on HRQoL. The majority of patients identified hearing preservation (61%), balance preservation (38.5%), and reduced tinnitus (34.6%) to be the patient-preferred outcomes., Conclusion: This qualitative study demonstrates that in this population many patients with VS encounter participation difficulties in their daily physical and social activities and value hearing and balance preservation, reduced tinnitus, and restored energy as preferred outcomes as they are hampered by symptoms and side effects related to hearing, balance, and energy. Healthcare professionals should consider these key points and use these and the patient-preferred outcomes in consultation, shared decision making, treatment, and follow-up to optimize patient-centred care., (© 2023. The Author(s).)

Objectives: Lateral skull base procedures, such as translabyrinthine approach (TLA), are challenging. An autonomous surgical robot might be a solution to these challenges. Our aim is to explore in an early phase the economic consequences of an autonomous surgical robot compared with conventional TLA., Methods: An early decision analytic model was constructed in order to perform a step-wise threshold analyses and a sensitivity analysis to analyze the impact of the several factors on the incremental costs., Results: Using surgical robot results in incremental costs - EUR 5,562 per procedure - compared to conventional TLA. These costs are most reduced by higher number of procedures, followed by lower price of the robot, saved operation time, and reduced risk of complication, respectively., Conclusions: The incremental costs of using an autonomous surgical robot can be decreased by choosing applications with a high turnover rate, a long operation time, and a high complication rate.

Objective: Vestibular schwannoma (VS) growth of ≥2 mm during serial MRI observation, irrespective of size, is the benchmark for treatment initiation in almost all centers. Although the probability of less optimal outcomes significantly increases in VS closer to the brainstem, early intervention does not improve long-term quality of life. Moving beyond the recommendation of definitive treatment for all VS after detected growth, we subclassified Koos 2 tumors based on extrameatal extension and relation to the brainstem. The aim of the current study was to evaluate the Koos 2 subclassification's validity and the inter-and intra-rater reliability of the entire Koos classification., Methods: Six experts, including neurosurgeons, otorhinolaryngologists and radiologists from two tertiary referral centers, classified 43 VS MRI scans. Validity of the Koos 2 subclassification was evaluated by the percentage agreement against the multidisciplinary skull base tumor board management advice. Inter- and intra-rater reliability were calculated using the intraclass correlation coefficient (ICC)., Results: Validity was almost perfect in Koos 2a VSs with a 100% agreement and 87.5% agreement for Koos 2b. Inter-rater reliability for all Koos grades was significantly excellent (ICC 0.91; 95%CI 0.866 to 0.944, p= <0.001). Five raters had an excellent intra-rater reliability (ICC > 0.90; p= <0.01) and one rater had a good intra-rater reliability (ICC 0.88; 95% CI 0.742 to 0.949)., Conclusions: Although multiple factors influence decision-making, the classification of Koos 2a and 2b with excellent inter- and intra-rater reliability, can aid in recommending treatment initiation, moving beyond detected tumor growth, aiming to optimize patient centered care., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a young age or late in the disease course, which presents challenges in balancing surgical intervention with various medical and radiotherapeutic approaches. The axiom-first, do no harm-should always be considered and an initial period of observation (ie, watchful waiting) is often appropriate to characterise tumour behaviour in patients with these pathogenic variants. These patients should be referred to specialised high-volume medical centres. This consensus guideline aims to help physicians with the clinical decision-making process when caring for patients with SDHD PPGLs., Competing Interests: Declaration of interests DT has received personal honoraria for lectures and consulting and support for meeting attendance from Advanced Accelerator Applications and Novartis. CL-L has received personal honoraria for lectures and support for meeting attendance from Ipsen. SN has received research grant to their institution from German Research Foundation. LA has received personal honoraria for lectures from Servier and Ipsen. ALE has received fees for consulting from WL Gore and fees for participation on an advisory board from Artivion. ML has received research grants to their institution from Arbor, Bristol Myers Squibb, Accuray, Biohaven, and Urogen; honoraria for research consulting from VBI Vaccines, InCephalo Therapeutics, Merck, Pyramid Bio, Insightec, Biohaven, Sanianoia, Hemispherian, Novocure, Noxxon, InCando, Century Therapeutics, and CraniUs; honoraria for non-research consulting from Stryker; is a shareholder of Egret Therapeutics; holds patents for the combination of immunotherapy and local chemotherapy to treat malignancies (10864180) and focused radiation to augment immune-based strategies against cancer (9132281); and is a member of the data and safety monitoring board of Cellularity. ELP has received fees for participation on an advisory board from Vysioneer. RTC has received personal honoraria for lectures from Novartis, support for meeting attendance from Ipsen, and serves as a board member for the Society for Endocrinology clinical committee and the UK and Ireland Neuroendocrine Tumour Society clinical committee. JKL has received honoraria for lectures from and is a consultant for Stryker. ERM has received fees for consulting and personal honoraria for lectures from MSD. NN has received an intramural research grant from the National Institutes of Health. NP-T has received research grants to their institution from Innervate, Clarity pharma; fees for consulting from Progenics, Lantheus, and Innervate Lifesciences. NP-T is a member of the data and safety monitoring board of Progenics and Lantheus, and serves as a board member for Society of Nuclear Medicine and Molecular Imaging. All other authors declare no competing interests., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Anecdotally, cystic vestibular schwannomas (cVSs) are regarded to have unpredictable biologic activity with poorer clinical results, and most studies showed a less favorable prognosis following surgery. While stereotactic radiosurgery (SRS) is a well-established therapeutic option for small- to medium-sized VSs, cVSs are often larger, thus making upfront SRS more complicated. The purpose of this retrospective study was to assess the efficacy and safety of upfront SRS for large cVSs. The authors reviewed the data of 54 patients who received upfront, single-session Gamma Knife radiosurgery (GKRS) with a diagnosis of large cVS (> 4 cm3). Patients with neurofibromatosis type 2, multiple VSs, or recurrent VSs and < 24 months of clinical and neuroimaging follow-up were excluded. Hearing loss (48.1%) was the primary presenting symptom. The majority of cVSs were Koos grade IV (66.7%), and the most prevalent cyst pattern was "mixed pattern of small and big cysts" (46.3%). The median time between diagnosis and GKRS was 12 months (range, 1-147 months). At GKRS, the median cVS volume was 6.95 cm 3 (range, 4.1-22 cm 3 ). The median marginal dose was 12 Gy (range, 10-12 Gy). The mean radiological and clinical follow-up periods were 62.2 ± 34.04 months (range, 24-169 months) and 94.9 ± 45.41 months (range, 24-175 months), respectively. At 2, 6, and 12 years, the tumor control rates were 100%, 95.7%, and 85.0%, respectively. Tumor shrinkage occurred in 92.6% of patients (n = 50), tumor volume remained stable in 5.6% of patients (n = 3), and tumor growth occurred in 1.9% of patients (n = 1). At a median follow-up of 53.5 months, the pre-GKRS tumor volume significantly decreased to 2.35 cm 3 (p < 0.001). While Koos grade 3 patients had a greater possibility of attaining higher volume reduction, "multiple small thick-walled cyst pattern" and smaller tumor volumes decreased the likelihood of achieving higher volume reduction. Serviceable hearing (Gardner-Robertson Scale I-II) was present in 16.7% of patients prior to GKRS and it was preserved in all of these patients following GKRS. After GKRS, 1.9% of patients (n = 1) had new-onset trigeminal neuralgia. There was no new-onset facial palsy, hemifacial spasm, or hydrocephalus. Contrary to what was believed, our findings suggest that upfront GKRS seems to be a safe and effective treatment option for large cVSs., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Certain cases of superior semicircular canal dehiscence or benign paroxysmal positional vertigo can be treated by plugging of the affected semicircular canal. However, the extent of the impact on vestibular function and hearing during postoperative follow-up is not known., Objective: To evaluate the evolution of vestibular function and hearing after plugging of a semicircular canal., Methods: Six patients underwent testing before and 1 week, 2 months, and 6 months after plugging of the superior or posterior semicircular canal. Testing included caloric irrigation test, video Head Impulse Test (vHIT), cervical and ocular Vestibular Evoked Myogenic Potentials (VEMPs) and audiometry., Results: Initially, ipsilateral caloric response decreased in all patients and vHIT vestibulo-ocular reflex (VOR) gain of each ipsilateral semicircular canal decreased in 4/6 patients. In 4/6 patients, postoperative caloric response recovered to > 60% of the preoperative value. In 5/6 patients, vHIT VOR gain was restored to > 85% of the preoperative value for both ipsilateral non-plugged semicircular canals. In the plugged semicircular canal, this gain decreased in 4/5 patients and recovered to > 50% of the preoperative value. Four patients preserved cervical and ocular VEMP responses. Bone conduction hearing deteriorated in 3/6 patients, but recovered within 6 months postoperatively, although one patient had a persistent loss of 15 dB at 8 kHz., Conclusion: Plugging of a semicircular canal can affect both vestibular function and hearing. After initial deterioration, most patients show recovery during follow-up. However, a vestibular function loss or high-frequency hearing loss can persist. This stresses the importance of adequate counseling of patients considering plugging of a semicircular canal., (© 2021. The Author(s).)

Cholesterol granulomas are the most common primary lesions of the petrous apex. Although their benign character, impingement of critical neurovascular structures can cause significant symptomatology such as hearing impairment. We present unique results after treatment of a cholesterol granuloma located in the petrous apex causing sensorineural hearing impairment. A transmastoidal approach was performed using an intraoperative computed tomography-guided navigation system. The video, which is included for reference, illustrates clear drainage of the cyst and drain positioning. The hearing improved completely in the lower frequencies at 500 and 1000 Hz and with a 19 dB improvement in overall bone conduction in the affected ear.

Objectives: Otomastoiditis caused by the anaerobic Fusobacterium necrophorum (F. necrophorum) often induces severe complications, such as meningitis and sinus thrombosis. Early diagnosis is difficult, partly because little is known about specific early signs. Comprehensive research about clinically chosen antimicrobial therapy has not been done yet and prognostic information about otomastoiditis caused by F. necrophorum is scarce. More knowledge about this subject is required., Methods: In this retrospective cohort study, we included all cases of otomastoiditis caused by F. necrophorum treated in two university medical centres in the Netherlands during the past 10 years. Data was gathered from patient records and analysed using independent sample T-tests and Chi 2 -tests., Results: This study reveals that otomastoiditis caused by F. necrophorum potentially induces neurological sequelae. Thereby, 80% of all included patients (n = 16) needed readmission within six months due to recurrence or complications of otomastoiditis caused by F. necrophorum. Mean (range) of age, CRP and temperature were 4.5 years (0.9-29.3), 243 mg/L (113-423) and 40 °C (37-41). All patients were hospitalized and treated with antibiotics, mostly metronidazole (n = 13/16) and a β -lactam (n = 15/16). Additional treatment contained low molecular weight heparin (83%, n = 10/12), dexamethasone (78%, n = 7/9) and/or surgery (80%, n = 12/16, whereof 9/12 mastoidectomy)., Conclusions: Patients and/or their parents need to be informed about this potential unfortunate prognosis when otomastoiditis caused by F. necrophorum is diagnosed. To improve early diagnosis, otomastoiditis caused by F. necrophorum should be suspected and therefore immediately cultured when a) young children present with otomastoiditis, with b) high CRP values, and/or c) vomiting and decreased consciousness., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: T4-classified squamous cell carcinoma (SCC) of external auditory canal (EAC) can potentially involve different anatomical structures, which could translate into different treatment strategies and survival outcomes within one classification. Our aim is to evaluate the clinical added value of T4-subclasses proposed by Lavieille and by Zanoletti., Methods: Retrospective data, including patients with primary operated cT4-classified EAC SCC, was obtained from 12 international hospitals. We subclassified according to the T4-subclasses. The treatment strategies, disease-free survival (DFS) and overall survival per subclass were calculated., Results: A total of 130 T4-classified EAC SCC were included. We found commonly used treatment strategies per subclass according to Lavieille and the DFS seems also to differ per subclass. Subclass according to Zanoletti showed comparable treatment strategies and survival outcomes per subclass., Conclusion: Our study suggests that the subclass according Lavieille might have added value in clinical practice to improve care of T4-classified EAC SCC., (© 2022 The Authors. Head & Neck published by Wiley Periodicals LLC.)

Objectives: Necrotizing external otitis (NEO) is a rare infectious disease of the skull base. The purpose of this study was to determine whether clinical outcomes of NEO can be correlated to different infectious spread patterns., Methods: Retrospective chart review from 2010 to 2019 with NEO patients, who were divided into two cohorts: single spreading patterns (group A) or complex spreading patterns (group B) as diagnosed by CT. Clinical symptoms, diagnostic and treatment delay, course of disease, complications, and duration of antibiotic exposure were retrospectively collected from patient records., Results: 41 NEO patients were included, of which 27 patients belonged to group A (66%). The disease-related mortality rate was 12.2% among the entire cohort, no differences were found between group A and B. Higher rates of N.VII (42.9% vs 14.8% P = 0.047) and N. IX palsies were found in group B compared to group A (28.6% vs 3.7%, P = 0.039). The median duration of antibiotic use was significantly different for a complex spreading pattern, clinical recovery and hospitalizations. Complications were associated with higher diagnostic delay and with a complex spread pattern. The median duration of follow-up was 12.0 (IQR 6.0-19.5) months., Conclusion: NEO is a severe disease, with significant mortality and morbidity (cranial nerve palsies). The radiological spread pattern may assist in predicting clinical outcome. Furthermore, complex spread patterns are associated with higher rates of clinical nerve palsies (N. VII and N.IX), complications, surgery rates and longer duration of antibiotic use. Diagnostic delay was associated with mortality, complications and facial palsies., Level of Evidence: Level IV., Competing Interests: The authors declare no conflict of interests., (© 2022 PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd.)

In this study, we investigated the association of ACAN variants with otosclerosis, a frequent cause of hearing loss among young adults. We sequenced the coding, 5'-UTR and 3'-UTR regions of ACAN in 1497 unrelated otosclerosis cases and 1437 matched controls from six different subpopulations. The association between variants in ACAN and the disease risk was tested through single variant and gene-based association tests. After correction for multiple testing, 14 variants were significantly associated with otosclerosis, ten of which represented independent association signals. Eight variants showed a consistent association across all subpopulations. Allelic odds ratios of the variants identified four predisposing and ten protective variants. Gene-based tests showed an association of very rare variants in the 3'-UTR with the phenotype. The associated exonic variants are all located in the CS domain of ACAN and include both protective and predisposing variants with a broad spectrum of effect sizes and population frequencies. This includes variants with strong effect size and low frequency, typical for monogenic diseases, to low effect size variants with high frequency, characteristic for common complex traits. This single-gene allelic spectrum with both protective and predisposing alleles is unique in the field of complex diseases. In conclusion, these findings are a significant advancement to the understanding of the etiology of otosclerosis., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Purpose: Necrotizing external otitis (NEO) is a serious complication of external otitis. NEO can be classified according to-anterior, medial, posterior, intracranial, and contralateral-extension patterns. Currently there is no consensus on the optimal imaging modality for the identification of disease extension. This study compares NEO extension patterns on MR and CT to evaluate diagnostic comparability., Methods: Patients who received a CT and MR within a 3-month interval were retrospectively examined. Involvement of subsites and subsequent spreading patterns were assessed on both modalities by a radiologist in training and by a senior head and neck radiologist. The prevalence of extension patterns on CT and MR were calculated and compared., Results: All 21 included NEO cases showed an anterior extension pattern on CT and MR. Contrary to MR, medial extension was not recognized on CT in two out of six patients, and intracranial extension in five out of eight patients. The posterior extension pattern was not recognized on MR. Overall, single anterior extension pattern (62%) is more prevalent than multiple extension patterns (38%)., Conclusion: All anterior NEO extension pattern were identified on CT as well as MR. However, the medial and intracranial spreading patterns as seen on MR could only be identified on CT in a small number of patients. The posterior spreading pattern can be overlooked on MR. Thus, CT and MR are complimentary for the initial diagnosis and work-up of NEO as to correctly delineate disease extent through the skull base., (© 2021. The Author(s).)

Objectives: To determine the cost-effectiveness of auditory brainstem response prior to MRI (ABR-MRI) compared to standalone MRI to diagnose vestibular schwannoma., Design: A state transition model was developed to simulate costs and effects (quality-adjusted life years [QALY]) for both diagnostic strategies for patients suspected of a vestibular schwannoma. Model input was derived from literature, hospital databases and expert opinions. Scenario and sensitivity analyses addressed model uncertainty., Results: Over a lifetime horizon, ABR-MRI resulted in a limited cost-saving of €68 or €98 per patient (dependent on MRI sequence) and a health loss of 0.005 QALYs over standalone MRI. ABR-MRI, however, did miss patients with other important pathology (2% of the population) that would have been detected when using standalone MRI. In total, €14 203 or €19 550 could be saved per lost QALY if ABR-MRI was used instead of standalone MRI. The results were sensitive to the detection rate of vestibular schwannoma and health-related quality of life of missed patients., Conclusion: The cost-saving with ABR-MRI does not seem to outweigh the number of missed patients with VS and other important pathologies that would have been detected when using standalone MRI., (© 2021 The Authors. Clinical Otolaryngology published by John Wiley & Sons Ltd.)

Objective: To describe a case of complete spontaneous regression of a middle ear melanoma., Patient: We present a case of a 68-year-old man with complaints of unilateral hearing loss and an ipsilateral facial nerve paresis. Radiological and histopathological examination revealed a cT4bN0M0 mucosal melanoma of the middle ear., Interventions: The patient underwent a subtotal petrosectomy and postoperative radiotherapy., Main Outcome Measure: Computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography/computed tomography with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET-CT), and histopathological examination., Results: After subtotal petrosectomy, histopathological examination of the resection specimen showed only fibrosis and a histiocytic and clonal T-cell infiltration, but no residual melanoma at the primary tumor site, consistent with spontaneous tumor regression. Follow-up MRI scanning 6 and 12 months after radiotherapy showed no signs of tumor recurrence., Conclusions: This case describes the concept of spontaneous regression of a mucosal melanoma of the middle ear. Spontaneous tumor regression at this location has not been described before., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Approximately 20% of patients diagnosed with a phaeochromocytoma or paraganglioma carry a germline mutation in one of the succinate dehydrogenase (SDHx) genes (SDHA, SDHB, SDHC and SDHD), which encode the four subunits of the SDH enzyme. When a pathogenic SDHx mutation is identified in an affected patient, genetic counselling is proposed for first-degree relatives. Optimal initial evaluation and follow-up of people who are asymptomatic but might carry SDHx mutations have not yet been agreed. Thus, we established an international consensus algorithm of clinical, biochemical and imaging screening at diagnosis and during surveillance for both adults and children. An international panel of 29 experts from 12 countries was assembled, and the Delphi method was used to reach a consensus on 41 statements. This Consensus Statement covers a range of topics, including age of first genetic testing, appropriate biochemical and imaging tests for initial tumour screening and follow-up, screening for rare SDHx-related tumours and management of elderly people who have an SDHx mutation. This Consensus Statement focuses on the management of asymptomatic SDHx mutation carriers and provides clinicians with much-needed guidance. The standardization of practice will enable prospective studies in the near future.

This study investigates whether there exist differences in facial emotion detection accuracy in patients suffering from Vestibular Schwannoma (VS) due to their facial paresis. Forty-four VS patients, half of them with, and half of them without a facial paresis, had to classify pictures of facial expressions as being emotional or non-emotional. The visual information of images was systematically manipulated by adding different levels of visual noise. The study had a mixed design with emotional expression (happy vs. angry) and visual noise level (10% to 80%) as repeated measures and facial paresis (present vs. absent) and degree of facial dysfunction as between subjects' factors. Emotion detection accuracy declined when visual information declined, an effect that was stronger for anger than for happy expressions. Overall, emotion detection accuracy for happy and angry faces did not differ between VS patients with or without a facial paresis, although exploratory analyses suggest that the ability to recognize emotions in angry facial expressions was slightly more impaired in patients with facial paresis. The findings are discussed in the context of the effects of facial paresis on emotion detection, and the role of facial mimicry, in particular, as an important mechanism for facial emotion processing and understanding.

Objective: To study the genotype and phenotype of a Dutch family with autosomal dominantly inherited hearing loss., Study Design: Genotype-phenotype correlation study. Genetic analysis consisted of linkage analysis, variable number of tandem repeats analysis, and Sanger sequencing. Audiovestibular function was examined. Regression analysis was performed on pure tone audiometry and speech recognition scores and correlated with the age and/or level of hearing loss., Setting: Tertiary referral center., Patients: A large Dutch family presenting with sensorineural hearing loss., Main Outcome Measures: Identification of the underlying genetic defect of the hearing loss in this family. Results of pure tone and speech audiometry, onset age, progression of hearing loss and vestibular (dys)function., Results: A novel mutation in COCH, c.1312C > T p.(Arg438Cys), cosegregates with hearing loss and a variable degree of vestibular (dys)function in this family. The reported mean age of onset of hearing loss is 33 years (range, 18-49 yr). Hearing loss primarily affects higher frequencies and its progression is relatively mild (0.8 dB/yr). Speech perception is remarkably well preserved in affected family members when compared with other DFNA9 families with different COCH mutations., Conclusion: These findings expand the genotypic and phenotypic spectrum of DFNA9. The c.1312C > T mutation, which affects the vWFA2 domain, causes a relatively mild audiovestibular phenotype when compared with other COCH mutations., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objectives: Patients with a vestibular schwannoma (VS) experience a reduced quality of life (QoL). The main objective of this study was to determine the strongest predictors reducing physical and mental QoL from the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire in patients with VS., Design: Observational study., Setting: Radboudumc Skull Base Centre, Nijmegen., Participants: Patients newly diagnosed with VS between 2014 and 2017 managed with either observation, stereotactic radiosurgery or microsurgery., Main Outcome Measures: Quality of life was assessed using the disease-specific PANQOL and general Short-Form (36) Health Survey (SF-36). Multiple linear regression models with PANQOL domains as predictors were used to determine the strongest predictors for SF-36 QoL physical and mental health scores. Standardised beta coefficients (β) were used for ranking., Results: A total of 174 patients (50% females, mean age 58.9 years) returned the questionnaires, providing a 69% response rate. Fifteen patients (9%) were treated with microsurgery, 29 (17%) with stereotactic radiosurgery and 130 patients (75%) were observed in a wait and scan strategy. A lack of energy (β = .28; P ≤ .001), lower general health (β = .22; P ≤ .001), headache (β = .16; P ≤ .001), anxiety (β = .15; P ≤ .001) and balance problems (β = .10; P ≤ .001) are the strongest predictors affecting physical health, while mental health is most affected by anxiety (β = .37; P ≤ .001), a lack of energy (β = .34; P ≤ .001), facial nerve dysfunction (β = .07; P ≤ .001), balance problems (β = .04; P ≤ .001) and headaches (β = .04; P ≤ .001)., Conclusion: A lack of energy, anxiety, headache and balance problems are the strongest predictors of both SF-36 physical and mental QoL in patients with VS. More awareness and supportive care regarding energy, anxiety, headache and balance in informing, evaluating and treating patients with VS could improve QoL., (© 2020 The Authors. Clinical Otolaryngology published by John Wiley & Sons Ltd.)

Objectives: To develop a prediction model to predict vestibular schwannoma (VS) growth for patients in a wait and scan (W&S) strategy., Design: Retrospective cohort study., Setting: Tertiary hospital (Radboud university medical center, Nijmegen, the Netherlands)., Participants: Patients with unilateral VS, entering a W&S strategy and at least one follow-up MRI available. Data on demographics, symptoms, audiometry and MRI characteristics at time of diagnosis were collected from medical records., Main Outcome Measures: Following multiple imputation, a multivariable Cox regression model was used to select variables, using VS growth (≥2 mm) as outcome. Decision curve analyses (DCA) were performed to compare the model to the current strategy., Results: Of 1217 analysed VS patients, 653 (53.7%) showed growth during follow-up. Balance complaints (HR 1.57 (95% CI: 1.31-1.88)) and tinnitus complaints in the affected ear (HR 1.36 (95% CI: 1.15-1.61)), Koos grade (Koos 1 is reference, Koos 2 HR 1.03 (95% CI: 0.80-1.31), Koos 3 HR 1.55 (95% CI: 1.16-2.06), Koos 4 HR 2.18 (95% CI: 1.60-2.96)), time since onset of symptoms (IQR HR 0.83 (95% CI: 0.77-0.88) and intrameatal diameter on MRI (IQR HR 1.67 (95% CI: 1.42-1.96)) were selected as significant predictors. The model's discrimination (Harrell's C) was 0.69 (95% CI: 0.67-0.71), and calibration was good. DCA showed that the model has a higher net benefit than the current strategy for probabilities of VS growth of >12%, 15% and 21% for the first consecutive 3 years, respectively., Conclusions: Patients with balance and tinnitus complaints, a higher Koos grade, short duration of symptoms and a larger intrameatal diameter at time of diagnosis have a higher probability of future VS growth. After external validation, this model may be used to inform patients about their prognosis, individualise the W&S strategy and improve (cost-)effectiveness., (© 2020 The Authors. Clinical Otolaryngology published by John Wiley & Sons Ltd.)

Background: Squamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare disease, which is commonly classified with the modified Pittsburgh classification. Our aim was to evaluate the predictive performance of this classification in relation to disease-free survival (DFS)., Methods: We examined retrospective data from a nationwide Dutch cohort study including patients with primary EAC SCC. These data were combined with individual patient data from the literature. Using the combined data, the predictive performances were calculated using the c-index., Results: A total of 381 patients were included, 294 for clinical and 281 for the pathological classification analyses. The c-indices of the clinical and the pathological modified Pittsburgh classification predicting DFS were 0.725 (0.668-0.782) and 0.729 (0.672-0.786), respectively., Conclusion: The predictive performance of the modified Pittsburgh classification system as such appears to be acceptable to predict the DFS of EAC SCC. Other factors need to be added to a future model to improve the predicted performance., (© 2020 The Authors. Head & Neck published by Wiley Periodicals LLC.)

Objective: Stereotactic radiosurgery (SRS) is one of the treatment modalities for vestibular schwannomas (VSs). However, tumor progression can still occur after treatment. Currently, it remains unknown how to predict long-term SRS treatment outcome. This study investigates possible magnetic resonance imaging (MRI)-based predictors of long-term tumor control following SRS., Study Design: Retrospective cohort study., Setting: Tertiary referral center., Patients: Analysis was performed on a database containing 735 patients with unilateral VS, treated with SRS between June 2002 and December 2014. Using strict volumetric criteria for long-term tumor control and tumor progression, a total of 85 patients were included for tumor texture analysis., Intervention(s): All patients underwent SRS and had at least 2 years of follow-up., Main Outcome Measure(s): Quantitative tumor texture features were extracted from conventional MRI scans. These features were supplied to a machine learning stage to train prediction models. Prediction accuracy, sensitivity, specificity, and area under the receiver operating curve (AUC) are evaluated., Results: Gray-level co-occurrence matrices, which capture statistics from specific MRI tumor texture features, obtained the best prediction scores: 0.77 accuracy, 0.71 sensitivity, 0.83 specificity, and 0.93 AUC. These prediction scores further improved to 0.83, 0.83, 0.82, and 0.99, respectively, for tumors larger than 5 cm., Conclusions: Results of this study show the feasibility of predicting the long-term SRS treatment response of VS tumors on an individual basis, using MRI-based tumor texture features. These results can be exploited for further research into creating a clinical decision support system, facilitating physicians, and patients to select a personalized optimal treatment strategy.

Background: Hearing loss is one of the most prevalent disabilities worldwide, and has a significant impact on quality of life. The adult-onset type of the condition is highly heritable but the genetic causes are largely unknown, which is in contrast to childhood-onset hearing loss., Methods: Family and cohort studies included exome sequencing and characterisation of the hearing phenotype. Ex vivo protein expression addressed the functional effect of a DNA variant., Results: An in-frame deletion of 12 nucleotides in RIPOR2 was identified as a highly penetrant cause of adult-onset progressive hearing loss that segregated as an autosomal dominant trait in 12 families from the Netherlands. Hearing loss associated with the deletion in 63 subjects displayed variable audiometric characteristics and an average (SD) age of onset of 30.6 (14.9) years (range 0-70 years). A functional effect of the RIPOR2 variant was demonstrated by aberrant localisation of the mutant RIPOR2 in the stereocilia of cochlear hair cells and failure to rescue morphological defects in RIPOR2-deficient hair cells, in contrast to the wild-type protein. Strikingly, the RIPOR2 variant is present in 18 of 22 952 individuals not selected for hearing loss in the Southeast Netherlands., Conclusion: Collectively, the presented data demonstrate that an inherited form of adult-onset hearing loss is relatively common, with potentially thousands of individuals at risk in the Netherlands and beyond, which makes it an attractive target for developing a (genetic) therapy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Context: No consensus about classification, treatment, and clinical relevance of occipital condyle fractures (OCFs) exists., Aims: The aim of the study was to determine radiological, clinical, and functional outcome of OCFs and thereby determine its clinical relevance., Settings and Design: This was a retrospective analysis of a prospective follow-up study., Materials and Methods: From May 2005 to May 2008, all OCFs were included from a Level-1 trauma center. Patient files were reviewed for patient and fracture characteristics. Fracture classification was done according to the Anderson criteria. Clinical outcome was assessed by completing two questionnaires, radiological outcome by computed tomography imaging, and functional outcome by measuring active cervical range of motion using a Cybex EDI-320., Statistical Analysis Used: A Fisher's exact Test was used in categorical variables and a one-sample t -test for comparing means of active cervical range of motion in occipital fracture patients with normal values. An independent samples t -test was carried out to compare the means of groups with and without accompanying cervical fractures for each motion., Results: Thirty-nine patients were included (4 type I, 16 type II, and 19 type III). Twenty-seven patients completed follow-up, of whom 26 were treated conservatively. Fracture healing was established in 25 of 28 fractures at a median follow-up of 19 months. Eleven patients had none to minimal pain or disability at follow-up, 12 had mild, and two had moderate pain or disability on questionnaires. No statistically significant difference in active cervical range of motion was identified comparing means stratified for accompanying cervical fractures., Conclusions: Conservatively treated patients with an OCF generally show favorable radiological and clinical outcome., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Craniovertebral Junction and Spine.)

Objective: This study investigates whether there exist differences in lateralization of facial emotion processing in patients suffering from Vestibular Schwannoma (VS) based on the presence of a facial paresis and their degree of facial functioning as measured by the House Brackmann Grading scale (HBG)., Methods: Forty-four VS patients, half of them with a facial paresis and half of them without a facial paresis, rated how emotive they considered images of faces showing emotion in the left versus right visual field. Stimuli consisted of faces with a neutral half and an emotional (happy or angry) half. The study had a mixed design with emotional expression (happy vs. angry) and emotional half (left vs. right visual field) of the faces as repeated measures, and facial paresis (present vs. absent) and HBG as between subjects' factors. The visual field bias was the main dependent variable., Results: In line with typical findings in the normal population, a left visual field bias showed in the current sample: patients judged emotional expressions shown in the left visual field as more emotive than those shown in the right visual field. No differences in visual field bias showed based on the presence of a facial paresis nor based on patients' HBG., Conclusion: VS patients show a left visual field bias when processing facial emotion. No differences in lateralization showed based on the presence of a facial paresis or on patients' HBG. Based on this study, facial paresis thus does not affect the lateralization of facial emotion processing in patients with VS., (© 2020 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Object: Surgery of tumours in the cerebellopontine angle (CPA) can lead to loss of facial nerve function. Different methods of intra-operative nerve monitoring (IOM) (including free-running EMG, direct nerve stimulation and transcranial motor evoked potentials (TcMEP)) have been used to predict facial nerve outcome during surgery. Recent research has shown TcMEP threshold increase and the occurrence of A-trains on the EMG to have great potential in doing so. This study compares these two methods and correlates them to House-Brackmann (HB) scores post-op in patients with tumours in the cerebellopontine angle., Method: Forty-three patients (one was operated twice) with large CPA tumours treated surgically in the Radboud University Medical Center between 2015 and 2019 were included in this study. During surgery, TcMEP threshold increases and A-train activity were measured. Because our treatment paradigm aims at facial nerve preservation (accepting residual tumour), TcMEP threshold increase of over 20 mA or occurrence of A-trains were considered as warning signs and used as a guide for terminating surgery. HB scores were measured post-op, at 6 weeks, 6 months and 1 year after surgery. Spearman's correlation was calculated between the IOM-values and the HB scores for a homogeneous subgroup of 30 patients with vestibular schwannoma (VS) without neurofibromatosis type II (NF-II) and all patients collectively., Results: TcMEP threshold was successfully measured in 39 (90.7%) procedures. In the homogeneous VS non-NFII group, we found a statistically significant moderate-to-strong correlation between TcMEP threshold increase and House Brackmann score immediately post-op, at 6 weeks, 6 months and 1 year after surgery (Spearman's rho of 0.79 (p < 0.001), 0.74 (p < 0.001), 0.64 (p < 0.001) and 0.58 (p = 0.002), respectively). For A-trains, no correlation was found. Similar results were found when including all patients with CPA tumours. A threshold increase of < 20 mA was a predictor of good facial nerve outcome., Conclusion: These results show that TcMEP threshold increases are strongly correlated to post-operative HB scores, while A-trains are not. This suggests TcMEP threshold increases can be a valuable predictor for facial nerve outcome in patients with large tumours when facial nerve preservation is prioritized over total resection. In this study, we found no use for A-trains to prevent facial nerve deficits.

Objectives: The present study aimed to replicate the finding that vestibular schwannoma (VS) patients with facial paresis experience lower health related quality of life (QoL) than those without facial paresis in a Dutch sample, and to extend these findings by measuring VS patients' overall satisfaction with life, social function, and emotion., Methods: Forty-seven VS patients, differing in degree of facial functioning, half of them with and half of them without a facial paresis, answered questionnaires about health related QoL (SF-36 and PANQOL), overall satisfaction with life, fear of being evaluated negatively by others, social avoidance and distress, and characteristics and symptoms of depression., Results: We observed that VS patients with facial paresis experience lower health-related QoL as well negatively impacted social function and emotion compared to VS patients without facial paresis. VS patients with facial paresis experienced lower overall satisfaction with life, more characteristic symptoms of depression, and more fear of being evaluated negatively by others than VS patients without facial paresis., Conclusion: These findings corroborate previous research showing an association between impaired facial functioning and lower QoL, but also extend them by showing differences on the quality of social function and emotion. Being aware of this difference between VS patients with and without facial paresis informs health practitioners regarding the specific support these patients might need. Moreover, it is also relevant to consider the influence of a facial paresis on patients' life when deciding between treatment options and in case of surgery the type of resection., Level of Evidence: 3., (© 2020 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.)

Objectives: To assess the cost-effectiveness of frequently used monitoring strategies for vestibular schwannoma (VS)., Design: A state transition model was developed to compare six monitoring strategies for patients with VS: lifelong annual monitoring; annual monitoring for the first 10 years after diagnosis; scanning at 1-5, 7, 9, 12, 15 years after diagnosis and subsequently every 5 years; a personalised monitoring strategy for small and large tumours; scanning at 1, 2 and 5 years after diagnosis and no monitoring. Input data were derived from literature and expert opinion. Quality-adjusted life years (QALYs) and healthcare costs of each strategy were modelled over lifetime. Net monetary benefits (NMBs) were calculated to determine which strategy provided most value for money. Sensitivity analyses were performed to address uncertainty., Results: Omitting monitoring is least effective with 18.23 (95% CI 16.84-19.37) QALYs per patient, and lifelong annual monitoring is most effective with 18.66 (95% CI 17.42-19.65) QALYs. Corresponding costs were €6526 (95% CI 5923-7058) and €9429 (95% CI 9197-9643) per patient, respectively. Lifelong annual monitoring provided the best value with a NMB of €363 765 (339 040-383 697), but the overall probability of being most cost-effective compared to the other strategies was still only 23%. Sensitivity analysis shows that there is large uncertainty in the effectiveness of all strategies, with largely overlapping 95% confidence intervals for all strategies., Conclusions: Due to the largely overlapping 95% confidence intervals of all monitoring strategies for VS, it is unclear which monitoring strategy provides most value for money at this moment., (© 2019 The Authors. Clinical Otolaryngology Published by John Wiley & Sons Ltd.)

Objective: To evaluate short- and long-term hearing results of surgery for acquired atresia of the external auditory canal (EAC) in a large patient cohort and to define preoperative audiometric conditions useful for patient counseling., Study Design: Retrospective cohort study., Setting: Academic tertiary referral center., Patients: Seventy-eight ears from 72 patients with postinflammatory acquired atresia of the EAC who underwent canal- and meatoplasty were included. Patients with involvement of the ossicular chain, (syndromic) external ear malformations, or congenital aural atresia were excluded., Intervention: Canal- and meatoplasty., Main Outcome Measures: Mean pure-tone averages of thresholds at 0.5, 1, 2, and 3 kHz (PTA0.5,1,2,3) for air conduction (AC), bone conduction, and air-bone gap (ABG) were calculated preoperatively and at short-term (≤0.55 yr) and long-term follow-up (>0.55 yr). Additionally, the numbers of ears with a closed ABG ≤10 dB and ≤20 dB, and with Social hearing (defined as: AC PTA0.5,1,2,3 ≤35 dB) were assessed., Results: At short-term follow-up AC PTA0.5,1,2,3 improved by 18 dB. Social hearing was obtained in 81% of the ears. Postoperatively, 35% of the ears had a closed ABG ≤10 dB, 83% was closed ≤20 dB. During follow-up, significant deterioration of 5 to 7 dB occurred for AC thresholds at 0.25, 0.5, and 1 kHz., Conclusions: Surgery for acquired atresia of the EAC is often beneficial. This study suggests overall advantageous surgery when the preoperative indication criteria ABG PTA0.5,1,2,3 >20 dB and AC PTA0.5,1,2,3 >35 dB are applied.

Purpose: Not long after the introduction of osseointegrated implants outside the oral cavity, auricular prostheses are retrained on osseointegrated implants. New insights have been gained with the next-generation percutaneous osseointegrated titanium implants for bone conduction hearing since its introduction in 2010. As a result, the same technology was introduced in the Vistafix ® system (VXI implant) to retain auricular prostheses. The aim of this study is to evaluate the surgical procedure, clinical outcome, and satisfaction of the patient of osseointegration-retained auricular prosthesis using VXI implants., Materials and Methods: 11 patients who received an auricular prosthesis using VXI implants between December 2012 and November 2017 were evaluated retrospectively. The patient's medical files were reviewed to assess clinical complications and the necessity for revision surgery. The subjective outcome was measured using the Glasgow benefit inventory (GBI)., Results: In total, 31 implants were placed in 11 patients. None of these implants were lost nor revision surgery needed. An adverse skin reaction was observed in 13.0% of the implants and in 27.2% of the patients, adequately treated with an antibiotic ointment. The average follow-up time was 2 years and 7 months. The GBI displayed a positive score in every patient., Conclusions: The VXI implants used are a safe and reliable treatment option for retaining auricular prostheses in patients with an absent auricle. Patients were satisfied with their auricular prosthesis and showed benefit in quality of life. Studies with larger numbers and preferably a prospective character are needed to draw statistically significant conclusions.

Patients with facial palsy present with both cosmetic and functional symptoms. When a facial palsy develops quickly (within 72 hours) with no other symptoms, and no cause can be identified, it is probably an idiopathic facial palsy or 'Bell's palsy'. The diagnosis Bell's palsy is, thus, to a certain extent a diagnosis 'per exclusionem'. We present three cases with an incorrectly diagnosed Bell's palsy or inadequate diagnostics or treatment: a 5-year-old male with recurrent facial palsy caused by acute otitis media; a 46-year-old male with facial palsy caused by a malignant parotid tumour; and a 75-year-old female with facial palsy caused by a facial nerve schwannoma in the mastoid segment of the facial nerve. We, therefore, emphasize the importance of thorough history-taking and adequate diagnostics and imaging when patients present with facial palsy.