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IntroductionTreatment with bevacizumab achieves both tumour stabilisation or regression and preservation or improvement of hearing. However, the efficacy of bevacizumab varies between patients and within patients. Side effects due to bevacizumab treatment are also common. It would be of value to predict therapeutic response prior to initiating therapy to prevent unnecessary exposure in patients unlikely to benefit.Methods and analysisWe aim to recruit 25 patients with NF2-related schwannomatosis (NF2) with bilateral vestibular schwannomas. Patients will receive an intravenous injection of 37 MBq [89Zr]bevacizumab followed by positron emission tomography (PET)/CT imaging 4 days later. After clinical evaluation at baseline, patients undergo bevacizumab treatment and are followed up at 3 and 6 months. The primary objective is to examine associations between pretreatment [89Zr]bevacizumab uptake on PET/CT and changes in multiple hearing outcomes and radiological characteristics of the target tumour following treatment. Secondary outcome measures include vestibular functioning, patient reported outcome measures, cranial nerve functionality, peripheral neurology, non-target schwannoma response and renal function. Given the explorative nature of the study, associations between PET-derived metrics and clinical and radiological outcomes will be examined without formal hypothesis testing, using generalised estimating equations to account for within-patient correlation. Pairwise associations will be summarised in an association matrix with multiplicity addressed using an all-resolutions inference approach, and findings will be considered hypothesis generating.Ethics and disseminationThis study was submitted via the Clinical Trials Information System reviewed and approved by the Medical Research Ethics Committee Leiden–The Hague–Delft Delft. The study findings will be disseminated through publication in peer-reviewed scientific journals and by presentation at national and international conferences.Trial registration numberThe trial is registered at ClinicalTrials.gov Protocol Registration and Results System under the registration ID: NCT05685836.

Ophthalmoparesis and ptosis can be caused by a wide range of rare or more prevalent diseases, several of which can be successfully treated. In this review, we provide clues to aid in the diagnosis of these diseases, based on the clinical symptoms, the involvement pattern and imaging features of extra‐ocular muscles (EOM). Dysfunction of EOM including the levator palpebrae can be due to muscle weakness, anatomical restrictions or pathology affecting the innervation. A comprehensive literature review was performed to find clinical and imaging clues for the diagnosis and follow‐up of ptosis and ophthalmoparesis. We used five patterns as a framework for differential diagnostic reasoning and for pattern recognition in symptomatology, EOM involvement and imaging results of individual patients. The five patterns were characterized by the presence of combination of ptosis, ophthalmoparesis, diplopia, pain, proptosis, nystagmus, extra‐orbital symptoms, symmetry or fluctuations in symptoms. Each pattern was linked to anatomical locations and either hereditary or acquired diseases. Hereditary muscle diseases often lead to ophthalmoparesis without diplopia as a predominant feature, while in acquired eye muscle diseases ophthalmoparesis is often asymmetrical and can be accompanied by proptosis and pain. Fluctuation is a hallmark of an acquired synaptic disease like myasthenia gravis. Nystagmus is indicative of a central nervous system lesion. Second, specific EOM involvement patterns can also provide valuable diagnostic clues. In hereditary muscle diseases like chronic progressive external ophthalmoplegia (CPEO) and oculo‐pharyngeal muscular dystrophy (OPMD) the superior rectus is often involved. In neuropathic disease, the pattern of involvement of the EOM can be linked to specific cranial nerves. In myasthenia gravis this pattern is variable within patients over time. Lastly, orbital imaging can aid in the diagnosis. Fat replacement of the EOM is commonly observed in hereditary myopathic diseases, such as CPEO. In contrast, inflammation and volume increases are often observed in acquired muscle diseases such as Graves' orbitopathy. In diseases with ophthalmoparesis and ptosis specific patterns of clinical symptoms, the EOM involvement pattern and orbital imaging provide valuable information for diagnosis and could prove valuable in the follow‐up of disease progression and the understanding of disease pathophysiology.

Purpose of Review Menière’s disease (MD) is a burdensome and not well understood inner ear disorder that has received increasing attention of scientists over the past decade. Until 2007, a certain diagnosis of endolymphatic hydrops (EH) required post-mortem histology. Today, dedicated high-resolution magnetic resonance imaging (MRI) protocols enable detection of disease-related changes in the membranous labyrinth in vivo. In this review, we summarize the current status of MR imaging for MD. Recent Findings The mainstays of hydrops imaging are inversion recovery sequences using delayed acquisition after intravenous or intratympanic contrast administration. Based on these techniques, several methods have been developed to detect and classify EH. In addition, novel imaging features of MD, such as blood-labyrinth barrier impairment, have recently been observed. Summary Delayed contrast enhanced MRI has emerged as a reliable technique to demonstrate EH in vivo, with promising application in the diagnosis and follow-up of MD patients. Therefore, familiarity with current techniques and diagnostic imaging criteria is increasingly important.

Purpose Morphological magnetic resonance (MR) and computed tomography (CT) features are used in combination with histology for diagnosis and treatment selection of primary bone neoplasms. Isolated functional MRI parameters have shown potential in diagnosis. Our goal is to facilitate diagnosis of primary bone neoplasms of the skull base, mobile spine and sacrum, by a comprehensive approach, combining morphological and functional imaging parameters. Materials and methods Pre-treatment MR of 80 patients with histologically proven diagnosis of a primary bone neoplasm of the skull base, mobile spine and sacrum were retrospectively analyzed for morphological and functional MRI parameters. Functional parameters were measured in 4 circular regions of interest per tumor placed on non-adjacent scan slices. Differences in values of functional parameters between different histologies were analyzed with Dunn’s test. Results Chordomas were the predominant histology (60.0%). Most neoplasms (80.0%) originated in the midline and had geographical (78.2%) bone destruction. Amorphous -type calcification (pre-existing bone) was seen only in chordomas. Homogeneous contrast enhancement pattern was seen only in chondrosarcoma and plasmacytoma. Ktrans and Kep were significantly lower in both chordoma, and chondrosarcoma compared to giant cell tumor of the bone (p = 0.006 – 0.011), and plasmacytoma (p = 0.004 – 0.014). Highest diffusion-weighted MRI apparent diffusion coefficient (ADC) values corresponded to chondrosarcoma and were significantly higher to those of chordoma (p = 0.008). Conclusion We identified the most discriminating morphological parameters and added functional MR parameters based on histopathological features that are useful in making a confident diagnosis of primary bone neoplasms in the skull base, mobile spine and sacrum.

PurposeTo report the first experience of our multidisciplinary team with functional imaging using 11C-methionine positron emission tomography-computed tomography (11C-methionine PET-CT) co-registered with MRI (Met-PET/MRICR) in clinical decision making and surgical planning of patients with difficult to treat prolactinoma.MethodsIn eighteen patients with prolactinoma, referred to our tertiary referral centre because of intolerance or resistance for dopamine agonists (DA), Met-PET/MRICR was used to aid decision-making regarding therapy.ResultsMet-PET/MRICR was positive in 94% of the patients. MRI and Met-PET/MRICR findings were completely concordant in five patients, partially concordant in nine patients, and non-concordant in four patients. In five patients Met-PET/MRICR identified lesion(s) that were retrospectively also visible on MRI. Met-PET/MRICR was false negative in one patient, with a cystic adenoma on conventional MRI. Thirteen patients underwent transsphenoidal surgery, with nine achieving full biochemical remission, two clinical improvement and near normalized prolactin levels, and one patient clinical improvement with significant tumour reduction. Hence, nearly all patients (94%) were considered to have a positive outcome. Permanent complication rate was low. Three patients continued DA, two patients have a wait and scan policy.ConclusionMet-PET/MRICR can provide additional information to guide multidisciplinary preoperative and intraoperative decision making in selected cases of prolactinoma. This approach resulted in a high remission rate with a low rate of complications in our expert centre.

Cochlear size and morphology vary greatly and may influence the course of a cochlear implant electrode array during insertion and its final intra-cochlear position. Detailed insight into these variations is valuable for characterizing each cochlea and offers the opportunity to study possible correlations with surgical or speech perception outcomes. This study presents an automatic tracing method to assess individual cochlear duct shapes from clinical CT images. On pre-operative CT scans of 479 inner ears the cochlear walls were discriminated by interpolating voxel intensities along radial and perpendicular lines within multiplanar reconstructions at 1 degree intervals from the round window. In all 479 cochleas, the outer wall could be traced automatically up to 720 degrees. The inner wall and floor of the scala tympani in 192 cochleas. The shape of the cochlear walls were modelled using a logarithmic spiral function including an offset value. The vertical trajectories of the scala tympani exhibited a non-monotonous spiral slope with specific regions at risk for CI-related insertion trauma, and three slope categories could be distinguished. This presented automatic tracing method allows the detailed description of cochlear morphology and can be used for both individual and large cohort evaluation of cochlear implant patients.

Purpose The aim of this study was to evaluate the intracochlear position of the Slim Modiolar Electrode (SME) after insertion via the extended Round Window (eRW) approach, and to correlate this with residual hearing preservation and speech perception outcomes. Methods Twenty-three adult participants, consecutively implanted with the SME via the eRW approach, were included in this prospective, single-center, observational study. Electrode position was evaluated intra-operatively using X-ray fluoroscopy and TIM measurement, and post-operatively using ultra-high resolution CT. Residual hearing [threshold shift in PTA between pre- and post-operative measurement, relative hearing preservation (RHP%)] and speech perception were evaluated at 2 and 12 months after surgery. Results In each of the 23 participants, complete scala tympani positioning of the electrode array could be achieved. In one participant, an initial tip fold-over was corrected during surgery. Average age at implantation was 63.3 years (SD 13.3, range 28–76) and mean preoperative residual hearing was 81.5 dB. The average post-operative PTA threshold shift was 16.2 dB (SD 10.8) at 2 months post-operatively, corresponding with a RHP% score of 44% (SD 34.9). At 12 months, the average RHP% score decreased to 37%. Postoperative phoneme scores improved from 27.1% preoperatively, to 72.1% and 82.1% at 2 and 12 months after surgery, respectively. Conclusion Use of the eRW approach results in an increased likelihood of complete scala tympani insertion when inserting the SME, with subsequent excellent levels of speech perception. However, residual hearing preservation was found to be moderate, possibly as a result of the extended round window approach, emphasizing that it is not an all-purpose approach for inserting this particular electrode array.

Background Chordomas and chondrosarcomas of the skull base are rare, slowly growing malignant bone neoplasms. Despite their radioresistant properties, proton therapy has been successfully used as an adjunct to resection or as a definitive treatment. Herewith, we present our experience with robustly optimized intensity-modulated proton therapy (IMPT) and related toxicities in skull base chordoma and chondrosarcoma patients treated at HollandPTC, Delft, the Netherlands. Methods Clinical data, treatment plans, and acute toxicities of patients treated between July 2019 and August 2021 were reviewed. CT and 3.0T MRI scans for treatment planning were performed in supine position in a thermoplastic mold. In total, 21 dose optimization and 28 dose evaluation scenarios were simulated. Acute toxicity was scored weekly before and during the treatment according to the CTCAE v4.0. Median follow-up was 35 months (range 12–36 months). Results Overall, 9 chordoma and 3 chondrosarcoma patients with 1–3 resections prior to IMPT were included; 4 patients had titanium implants. Brainstem core and surface and spinal cord core and surface were used for nominal plan robust optimization in 11, 10, 8, and 7 patients, respectively. Middle ear inflammation, dry mouth, radiation dermatitis, taste disorder, and/or alopecia of grades 1–3 were noted at the end of treatment among 6 patients without similar complaints at inclusion; symptoms disappeared 3 months following the treatment. Conclusion Robustly optimized IMPT is clinically feasible as a postoperative treatment for skull base chordoma and chondrosarcoma patients. We observed acceptable early toxicities (grade 1–3) that disappeared within the first 3 months after irradiation.

Purpose In cochlear implantation (CI), the two factors that are determined by the surgeon with a potential significant impact on the position of the electrode within the cochlea and the potential outcome, are the surgical technique and electrode type. The objective of this prospective study was to evaluate the position of the slim, perimodiolar electrode (SPE), and to study the influence of the SPE position on CI outcome. Methods Twenty-three consecutively implanted, adult SPE candidates were included in this prospective cohort study conducted between December 2016 and April 2019. Mean age at surgery was 59.5 years. Mean preoperative residual hearing was 92.2 dB. Intra-operative fluoroscopy and high-resolution computed tomography scans were performed to evaluate electrode position after insertion using a cochleostomy (CS) approach. Follow-up was 12 months after implantation; residual hearing (6–8 weeks) and speech perception (6–8 weeks and 12 months) were evaluated in relation to the intracochlear SPE position. Results In most patients in whom the SPE was positioned in the s cala tympani residual hearing was preserved [mean absolute increase in PTA of 4.4 dB and 77.2% relative hearing preservation (RHP%)]. Translocation into the scala vestibuli occurred in 36% of the insertions, resulting in a mean absolute increase in PTA of 17.9 dB, and a RHP% of 19.2%. Participants with a translocation had poorer speech perception scores at 12-month follow-up. Conclusion Given the incidence of CS-associated translocations with the SPE and the negative effect on outcome, it is advised to insert the SPE using the (extended) round window approach.

Objectives: To study the relation between variables related to cochlear implant electrode position and speech perception performance scores in a large patient population. Design: The study sample consisted of 203 patients implanted with a CII or HiRes90K implant with a HiFocus 1 or 1J electrode of Advanced Bionics. Phoneme and word score averages for the 1- and 2-year follow-up were calculated for 41 prelingually deaf and 162 postlingually deaf patients. Analyses to reveal correlations between these performance outcomes and 6 position-related variables (angle of most basal electrode contact, surgical insertion angle, surgical insertion, wrapping factor, angular insertion depth, linear insertion depth) were executed. The scalar location, as an indication for the presence of intracochlear trauma, and modiolus proximity beyond the basal turn were not evaluated in this study. In addition, different patient-specific variables (age at implantation, age at onset of hearing loss, duration of deafness, preoperative phoneme and word scores) were tested for correlation with performance. Results: The performance scores of prelingual patients were correlated with age at onset of hearing loss, duration of deafness and preoperative scores. For the postlingual patients, performance showed correlations with all 5 patient-specific variables. None of the 6 position-related variables influenced speech perception in cochlear implant patients. Conclusions: Although several patient-specific variables showed correlations with speech perception outcomes, not one of the studied angular and linear position-related variables turned out to have a demonstrable influence on performance.