Neuroanatomical anomalies due to a defect in the FGF3 gene, associated with the Labyrinthine Aplasia, Microtia and Microdontia syndrome: insights from the placement of auditory brainstem implants in two siblings.

Here, we describe two congenitally deaf male siblings with the same compound heterozygotic, likely pathogenic mutations in the FGF3 gene, associated with the labyrinthine aplasia, microtia and microdontia (LAMM) syndrome. Both children had bilateral cochleovestibular aplasia, precluding cochlear implantation. The elder brother received an auditory brainstem implant (ABI) with very limited auditory responses.

Automated segmentation of clinical CT scans of the cochlea and analysis of the cochlea's vertical profile.

Purpose: Knowledge of the cochlear anatomy in individual patients is helpful for improving electrode selection and placement during cochlear implantation, as well as in surgical planning. The aim of this study was to develop a model-free automated segmentation algorithm to obtain 3D surfaces from clinical computed tomography (CT) scans that describe an individual's cochlear anatomy and can be used to quantitatively analyze the cochlea's vertical trajectory.

Methods: Clinical CT scans were re-oriented and re-sliced to obtain mid-modiolar slices.

Robust IMPT and follow-up toxicity in skull base chordoma and chondrosarcoma-a single-institution clinical experience.

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.

Geometrical accuracy of magnetic resonance imaging for ocular proton therapy planning.

Background & Purpose: Magnetic resonance imaging (MRI) is increasingly used in treatment preparation of ocular proton therapy, but its spatial accuracy might be limited by geometric distortions due to susceptibility artefacts. A correct geometry of the MR images is paramount since it defines where the dose will be delivered. In this study, we assessed the geometrical accuracy of ocular MRI.

Combining morphological and functional imaging parameters to diagnose primary bone neoplasms in the skull base, spine and sacrum.

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.

F-fluoro-ethyl-tyrosine PET co-registered with MRI in patients with persisting acromegaly.

Objective: To report our experience with F-fluoro-ethyl-tyrosine (FET) positron emission tomography-computed tomography (PET-CT) co-registered with magnetic resonance imaging (MRI) (FET-PET/MRI) in the care trajectory for persistent acromegaly.

Design: Prospective case series.

Patients: Ten patients with insufficiently controlled acromegaly referred to our team to evaluate surgical options.

Visualization and clinical relevance of the endolymphatic duct and sac in Ménière's disease.

Background: Ménière's disease (MD) is a chronic inner ear disorder with a multifactorial etiology. Decreased visualization of the endolymphatic duct (ED) and sac (ES) is thought to be associated with MD, although controversy exists about whether this finding is specific to MD. Recent literature has revealed that two distinct ES pathologies, developmental hypoplasia and epithelial degeneration, can be distinguished in MD using the angular trajectory of the vestibular aqueduct (ATVA) or ED-ES system as a radiographic surrogate marker.

Automated 2-Dimensional Measurement of Vestibular Schwannoma: Validity and Accuracy of an Artificial Intelligence Algorithm.

Objective: Validation of automated 2-dimensional (2D) diameter measurements of vestibular schwannomas on magnetic resonance imaging (MRI).

Study Design: Retrospective validation study using 2 data sets containing MRIs of vestibular schwannoma patients.

Setting: University Hospital in The Netherlands.

Inter-Observer Variability in MR-Based Target Volume Delineation of Uveal Melanoma.

Purpose: Several efforts are being undertaken toward MRI-based treatment planning for ocular proton therapy for uveal melanoma (UM). The interobserver variability of the gross target volume (GTV) on magnetic resonance imaging (MRI) is one of the important parameters to design safety margins for a reliable treatment. Therefore, this study assessed the interobserver variation in GTV delineation of UM on MRI.

Automatic Three-Dimensional Magnetic Resonance-based measurements of tumour prominence and basal diameter for treatment planning of uveal melanoma.

Background And Purpose: Three-dimensional (3D) Magnetic Resonance Imaging (MRI) is increasingly used to complement conventional two-dimensional ultrasound in the assessment of tumour dimension measurement of uveal melanoma. However, the lack of definitions of the 3D measurements of these tumour dimensions hinders further adaptation of MRI in ocular radiotherapy planning. In this study, we composed 3D MR-based definitions of tumour prominence and basal diameter and compared them to conventional ultrasound.

Clinical and imaging clues to the diagnosis and follow-up of ptosis and ophthalmoparesis.

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.

Fully Automated 3D Vestibular Schwannoma Segmentation with and without Gadolinium-based Contrast Material: A Multicenter, Multivendor Study.

Purpose: To develop automated vestibular schwannoma measurements on contrast-enhanced T1- and T2-weighted MRI scans.

Materials And Methods: MRI data from 214 patients in 37 different centers were retrospectively analyzed between 2020 and 2021. Patients with hearing loss (134 positive for vestibular schwannoma [mean age ± SD, 54 years ± 12;64 men] and 80 negative for vestibular schwannoma) were randomly assigned to a training and validation set and to an independent test set.

Comparison of Magnetic Resonance Imaging-Based and Conventional Measurements for Proton Beam Therapy of Uveal Melanoma.

Objective: Conventionally, ocular proton therapy (PT) is planned using measurements obtained by an ophthalmologist using ultrasound, fundoscopy, biometry, and intraoperative assessments. Owing to the recent advances in magnetic resonance imaging (MRI) of uveal melanoma (UM), it is possible to acquire high-resolution 3-dimensional images of the eye, providing the opportunity to incorporate MRI in ocular PT planning. In this study, we described how these measurements can be obtained using MRI, compared the MRI-based measurements with conventional ophthalmic measurements, and identified potential pitfalls for both modalities.

Implementation of functional imaging using C-methionine PET-CT co-registered with MRI for advanced surgical planning and decision making in prolactinoma surgery.

Purpose: To report the first experience of our multidisciplinary team with functional imaging using C-methionine positron emission tomography-computed tomography (C-methionine PET-CT) co-registered with MRI (Met-PET/MRI) in clinical decision making and surgical planning of patients with difficult to treat prolactinoma.

Methods: In eighteen patients with prolactinoma, referred to our tertiary referral centre because of intolerance or resistance for dopamine agonists (DA), Met-PET/MRI was used to aid decision-making regarding therapy.

Results: Met-PET/MRI was positive in 94% of the patients.

Prolonged Insertion Time Reduces Translocation Rate of a Precurved Electrode Array in Cochlear Implantation.

Hypothesis: Insertion speed during cochlear implantation determines the risk of cochlear trauma. By slowing down insertion speed tactile feedback is improved. This is highly conducive to control the course of the electrode array along the cochlear contour and prevent translocation from the scala tympani to the scala vestibuli.

Intracochlear electrode array position and cochlear implant outcomes using the nucleus slim modiolar electrode and the extended round window approach: a follow-up study.

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.

Fewer head and neck cancer diagnoses and faster treatment initiation during COVID-19 in 2020: A nationwide population-based analysis.

Background: Inevitably, the emergence of COVID-19 has impacted non-COVID care. Because timely diagnosis and treatment are essential, especially for patients with head and neck cancer (HNC) with fast-growing tumours in a functionally and aesthetically important area, we wished to quantify the impact of the COVID-19 pandemic on HNC care in the Netherlands.

Material And Methods: This population-based study covered all, in total 8468, newly diagnosed primary HNC cases in the Netherlands in 2018, 2019 and 2020.

Ultra-High-Resolution CT to Detect Intracochlear New Bone Formation after Cochlear Implantation.

Background Histopathologic studies reported that cochlear implantation, a well-established means to treat severe-to-profound sensorineural hearing loss, may induce inflammation, fibrosis, and new bone formation (NBF) with possible impact on loss of residual hearing and hearing outcome. Purpose To assess NBF in vivo after cochlear implantation with ultra-high-spatial-resolution (UHSR) CT and its implication on long-term residual hearing outcome. Materials and Methods In a secondary analysis of a prospective single-center cross-sectional study, conducted between December 2016 and January 2018, patients with at least 1 year of cochlear implantation experience underwent temporal bone UHSR CT and residual hearing assessment.

Hydropic Ear Disease: Correlation Between Audiovestibular Symptoms, Endolymphatic Hydrops and Blood-Labyrinth Barrier Impairment.

To investigate the correlation between clinical features and MRI-confirmed endolymphatic hydrops (EH) and blood-labyrinth barrier (BLB) impairment. Retrospective cross-sectional study. Vertigo referral center (Haga Teaching Hospital, The Hague, the Netherlands).

MR imaging characteristics of uveal melanoma with histopathological validation.

Purpose: To evaluate the magnetic resonance imaging (MRI) characteristics of uveal melanoma (UM), to compare them with fundoscopy and ultrasound (US), and to validate them with histopathology.

Methods: MR images from 42 UM were compared with US and fundoscopy, and on 14 enucleated cases with histopathology.

Results: A significant relationship between the signal intensity on T1 and pigmentation on histopathology was found (p=0.

Factors Influencing Speech Perception in Adults With a Cochlear Implant.

Objectives: The primary objective of this study is to identify the biographic, audiologic, and electrode position factors that influence speech perception performance in adult cochlear implant (CI) recipients implanted with a device from a single manufacturer. The secondary objective is to investigate the independent association of the type of electrode (precurved or straight) with speech perception.

Design: In a cross-sectional study design, speech perception measures and ultrahigh-resolution computed tomography scans were performed in 129 experienced CI recipients with a postlingual onset of hearing loss.

Transimpedance Matrix (TIM) Measurement for the Detection of Intraoperative Electrode Tip Foldover Using the Slim Modiolar Electrode: A Proof of Concept Study.

Objectives: The aim of this study is to report on our preliminary experience with Transimpedance Matrix (TIM)-measurement for the detection of cochlear implant electrode tip foldovers compared with intraoperative imaging in patients implanted with the slim modiolar electrode (SME).

Study Design: Proof of concept study.

Setting: Tertiary university referral center.