18F-fluoro-ethyl-tyrosine PET co-registered with MRI in patients with persisting acromegaly.

OBJECTIVE: To report our experience with 18F-fluoro-ethyl-tyrosine (FET) positron emission tomography-computed tomography (PET-CT) co-registered with magnetic resonance imaging (MRI) (FET-PET/MRICR) 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. MEASUREMENTS: FET-PET/MRICR was used to support decision-making if MRI alone and multidisciplinary team evaluation did not provide sufficient clarity to proceed to surgery. RESULTS: FET-PET/MRICR showed suspicious (para)sellar tracer uptake in all patients. In five patients FET-PET/MRICR was fully concordant with conventional MRI, and in one patient partially concordant. FET-PET/MRICR identified suggestive new foci in four other patients. Surgical re-exploration was performed in nine patients (aimed at total resection (6), debulking (2), diagnosis (1)), and one patient underwent radiation therapy. In 7 of 9 (78%) operated patients FET-PET/MRICR findings were confirmed intraoperatively, and in six (67%) also histologically. IGF-1 decreased significantly in eight patients (89%). All patients showed clinical improvement. Complete biochemical remission was achieved in three patients (50% of procedures in which total resection was anticipated feasible). Biochemistry improved in five and was unchanged in one patient. No permanent complications occurred. At six months, optimal outcome (preoperative intended goal achieved without permanent complications) was achieved in six (67%) patients and an intermediate outcome (goal not achieved, but no complications) in the other three patients. CONCLUSIONS: In patients with persisting acromegaly without a clear surgical target on MRI, FET-PET/MRICR is a new tracer to provide additional information to aid decision-making by the multidisciplinary pituitary team.

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. 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.

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. Two observers evaluated the presence and location of NBF independently, and tetrachoric correlations were used to assess interobserver reliability. In addition, the scalar location of each electrode was assessed. After consensus agreement, participants were classified into two groups: those with NBF (n = 83) and those without NBF (n = 40). The association between NBF and clinical parameters, including electrode design, surgical approach, and long-term residual hearing loss, was tested using the χ2 and Student t tests. Results A total of 123 participants (mean age ± standard deviation, 63 years ± 13; 63 women) were enrolled. NBF was found in 83 of the 123 participants (68%) at 466 of 2706 electrode contacts (17%). Most NBFs (428 of 466, 92%) were found around the 10 most basal contacts, with an interobserver agreement of 86% (2297 of 2683 contacts). Associations between electrode types and surgical approaches were significant (58 of 79 participants with NBF and a precurved electrode vs 24 of 43 with NBF and a straight electrode, P = .04; 64 of 88 participants with NBF and a cochleostomy approach vs 18 of 34 with NBF and a round window approach, P = .03). NBF was least often seen in full scala tympani insertions, but there was no significant association between scalar position and NBF (P = .15). Long-term residual hearing loss was significantly larger in the group with NBF compared with the group without NBF (mean, 22.9 dB ± 14 vs 8.6 dB ± 18, respectively; P = .04). Conclusion In vivo detection of new bone formation (NBF) after cochlear implantation is possible by using ultra-high-spatial-resolution CT. Most cochlear implant recipients develop NBF, predominately located at the base of the cochlea. NBF adversely affects long-term residual hearing preservation. © RSNA, 2021 An earlier incorrect version appeared online. This article was corrected on December 8, 2021.

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=0.024). T1 hyperintense UM were always moderately or strongly pigmented on histopathology, while T1-hypointense UM were either pigmented or non-pigmented. Mean apparent diffusion coefficient (ADC) of the UM was 1.16 ± 0.26 × 10-3 mm2/s. Two-thirds of the UM had a wash-out and the remaining a plateau perfusion time-intensity curve (TIC). MRI was limited in evaluating the basal diameter of flat tumors. US tends to show larger tumor prominence (0.5mm larger, p=0.008) and largest basal diameter (1.4mm larger, p<0.001). MRI was good in diagnosing ciliary body involvement, extrascleral extension, and optic nerve invasion, but limited on identifying scleral invasion. An increase of tumor prominence was associated with lower ADC values (p=0.030) and favored a wash-out TIC (p=0.028). An increase of tumor ADC correlated with a plateau TIC (p=0.011). CONCLUSIONS: The anatomical and functional MRI characteristics of UM were comprehensively assessed. Knowing the MRI characteristics of UM is important in order to confirm the diagnosis and to differentiate UM from other intra-ocular lesions and because it has implications for treatment planning. MRI is a good technique to evaluate UM, being only limited in case of flat tumors or on identifying scleral invasion.

Implementation of functional imaging using 11C-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 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. METHODS: In 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. RESULTS: Met-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. CONCLUSION: Met-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.

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. 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.

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. Data were collected between December 2016 and January 2018 in the Radboud University Medical Center, Nijmegen, the Netherlands. The participants received either a precurved electrode (N = 85) or a straight electrode (N = 44), all from the same manufacturer. The biographic variables evaluated were age at implantation, level of education, and years of hearing loss. The audiometric factors explored were preoperative and postoperative pure-tone average residual hearing and preoperative speech perception score. The electrode position factors analyzed, as measured from images obtained with the ultrahigh-resolution computed tomography scan, were the scalar location, angular insertion depth of the basal and apical electrode contacts, and the wrapping factor (i.e., electrode-to-modiolus distance), as well as the type of electrode used. These 11 variables were tested for their effect on three speech perception outcomes: consonant-vowel-consonant words in quiet tests at 50 dB SPL (CVC50) and 65 dB SPL (CVC65), and the digits-in-noise test. RESULTS: A lower age at implantation was correlated with a higher CVC50 phoneme score in the straight electrode group. Other biographic variables did not correlate with speech perception. Furthermore, participants implanted with a precurved electrode and who had poor preoperative hearing thresholds performed better in all speech perception outcomes than the participants implanted with a straight electrode and relatively better preoperative hearing thresholds. After correcting for biographic factors, audiometric variables, and scalar location, we showed that the precurved electrode led to an 11.8 percentage points (95% confidence interval: 1.4-20.4%; p = 0.03) higher perception score for the CVC50 phonemes compared with the straight electrode. Furthermore, contrary to our initial expectations, the preservation of residual hearing with the straight electrode was poor, as the median preoperative and the postoperative residual hearing thresholds for the straight electrode were 88 and 122 dB, respectively. CONCLUSIONS: Cochlear implantation with a precurved electrode results in a significantly higher speech perception outcome, independent of biographic factors, audiometric factors, and scalar location.

The evaluation of a slim perimodiolar electrode: surgical technique in relation to intracochlear position and cochlear implant outcomes.

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 scala 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.

Variants affecting diverse domains of MEPE are associated with two distinct bone disorders, a craniofacial bone defect and otosclerosis.

PURPOSE: To characterize new molecular factors implicated in a hereditary congenital facial paresis (HCFP) family and otosclerosis. METHODS: We performed exome sequencing in a four-generation family presenting nonprogressive HCFP and mixed hearing loss (HL). MEPE was analyzed using either Sanger sequencing or molecular inversion probes combined with massive parallel sequencing in 89 otosclerosis families, 1604 unrelated affected subjects, and 1538 unscreened controls. RESULTS: Exome sequencing in the HCFP family led to the identification of a rare segregating heterozygous frameshift variant p.(Gln425Lysfs*38) in MEPE. As the HL phenotype in this family resembled otosclerosis, we performed variant burden and variance components analyses in a large otosclerosis cohort and demonstrated that nonsense and frameshift MEPE variants were significantly enriched in affected subjects (p = 0.0006-0.0060). CONCLUSION: MEPE exerts its function in bone homeostasis by two domains, an RGD and an acidic serine aspartate-rich MEPE-associated (ASARM) motif inhibiting respectively bone resorption and mineralization. All variants associated with otosclerosis are predicted to result in nonsense mediated decay or an ASARM-and-RGD-truncated MEPE. The HCFP variant is predicted to produce an ASARM-truncated MEPE with an intact RGD motif. This difference in effect on the protein corresponds with the presumed pathophysiology of both diseases, and provides a plausible molecular explanation for the distinct phenotypic outcome.

Heterozygous missense variants of LMX1A lead to nonsyndromic hearing impairment and vestibular dysfunction.

Unraveling the causes and pathomechanisms of progressive disorders is essential for the development of therapeutic strategies. Here, we identified heterozygous pathogenic missense variants of LMX1A in two families of Dutch origin with progressive nonsyndromic hearing impairment (HI), using whole exome sequencing. One variant, c.721G > C (p.Val241Leu), occurred de novo and is predicted to affect the homeodomain of LMX1A, which is essential for DNA binding. The second variant, c.290G > C (p.Cys97Ser), predicted to affect a zinc-binding residue of the second LIM domain that is involved in protein-protein interactions. Bi-allelic deleterious variants of Lmx1a are associated with a complex phenotype in mice, including deafness and vestibular defects, due to arrest of inner ear development. Although Lmx1a mouse mutants demonstrate neurological, skeletal, pigmentation and reproductive system abnormalities, no syndromic features were present in the participating subjects of either family. LMX1A has previously been suggested as a candidate gene for intellectual disability, but our data do not support this, as affected subjects displayed normal cognition. Large variability was observed in the age of onset (a)symmetry, severity and progression rate of HI. About half of the affected individuals displayed vestibular dysfunction and experienced symptoms thereof. The late-onset progressive phenotype and the absence of cochleovestibular malformations on computed tomography scans indicate that heterozygous defects of LMX1A do not result in severe developmental abnormalities in humans. We propose that a single LMX1A wild-type copy is sufficient for normal development but insufficient for maintenance of cochleovestibular function. Alternatively, minor cochleovestibular developmental abnormalities could eventually lead to the progressive phenotype seen in the families.

Guidelines in CHARGE syndrome and the missing link: Cranial imaging.

"CHARGE syndrome" is a complex syndrome with high and extremely variable comorbidity. As a result, clinicians may struggle to provide accurate and comprehensive care, and this has led to the publication of several clinical surveillance guidelines and recommendations for CHARGE syndrome, based on both single case observations and cohort studies. Here we perform a structured literature review to examine all the existing advice. Our findings provide additional support for the validity of the recently published Trider checklist. We also identified a gap in literature when reviewing all guidelines and recommendations, and we propose a guideline for neuroradiological evaluation of patients with CHARGE syndrome. This is of importance, as patients with CHARGE are at risk for peri-anesthetic complications, making recurrent imaging procedures under anesthesia a particular risk in clinical practice. However, comprehensive cranial imaging is also of tremendous value for timely diagnosis, proper treatment of symptoms and for further research into CHARGE syndrome. We hope the guideline for neuroradiological evaluation will help clinicians provide efficient and comprehensive care for individuals with CHARGE syndrome.

Comparison of the HiFocus Mid-Scala and HiFocus 1J Electrode Array: Angular Insertion Depths and Speech Perception Outcomes.

The HiFocus Mid-Scala (MS) electrode array has recently been introduced onto the market. This precurved design with a targeted mid-scalar intracochlear position pursues an atraumatic insertion and optimal distance for neural stimulation. In this study we prospectively examined the angular insertion depth achieved and speech perception outcomes resulting from the HiFocus MS electrode array for 6 months after implantation, and retrospectively compared these with the HiFocus 1J lateral wall electrode array. The mean angular insertion depth within the MS population (n = 96) was found at 470°. This was 50° shallower but more consistent than the 1J electrode array (n = 110). Audiological evaluation within a subgroup, including only postlingual, unilaterally implanted, adult cochlear implant recipients who were matched on preoperative speech perception scores and the duration of deafness (MS = 32, 1J = 32), showed no difference in speech perception outcomes between the MS and 1J groups. Furthermore, speech perception outcome was not affected by the angular insertion depth or frequency mismatch.

Intracochlear Position of Cochlear Implants Determined Using CT Scanning versus Fitting Levels: Higher Threshold Levels at Basal Turn.

OBJECTIVES: In this study, the effects of the intracochlear position of cochlear implants on the clinical fitting levels were analyzed. DESIGN: A total of 130 adult subjects who used a CII/HiRes 90K cochlear implant with a HiFocus 1/1J electrode were included in the study. The insertion angle and the distance to the modiolus of each electrode contact were determined using high-resolution CT scanning. The threshold levels (T-levels) and maximum comfort levels (M-levels) at 1 year of follow-up were determined. The degree of speech perception of the subjects was evaluated during routine clinical follow-up. RESULTS: The depths of insertion of all the electrode contacts were determined. The distance to the modiolus was significantly smaller at the basal and apical cochlear parts compared with that at the middle of the cochlea (p < 0.05). The T-levels increased toward the basal end of the cochlea (3.4 dB). Additionally, the M-levels, which were fitted in our clinic using a standard profile, also increased toward the basal end, although with a lower amplitude (1.3 dB). Accordingly, the dynamic range decreased toward the basal end (2.1 dB). No correlation was found between the distance to the modiolus and the T-level or the M-level. Furthermore, the correlation between the insertion depth and stimulation levels was not affected by the duration of deafness, age at implantation or the time since implantation. Additionally, the T-levels showed a significant correlation with the speech perception scores (p < 0.05). CONCLUSIONS: The stimulation levels of the cochlear implants were affected by the intracochlear position of the electrode contacts, which were determined using postoperative CT scanning. Interestingly, these levels depended on the insertion depth, whereas the distance to the modiolus did not affect the stimulation levels. The T-levels increased toward the basal end of the cochlea. The level profiles were independent of the overall stimulation levels and were not affected by the biographical data of the patients, such as the duration of deafness, age at implantation or time since implantation. Further research is required to elucidate how fitting using level profiles with an increase toward the basal end of the cochlea benefits speech perception. Future investigations may elucidate an explanation for the effects of the intracochlear electrode position on the stimulation levels and might facilitate future improvements in electrode design.

Development of Insertion Models Predicting Cochlear Implant Electrode Position.

OBJECTIVES: To assess the possibility to define a preferable range for electrode array insertion depth and surgical insertion distance for which frequency mismatch is minimalized. To develop a surgical insertion guidance tool by which a preferred target angle can be attained using preoperative available anatomical data and surgically controllable insertion distance. DESIGN: Multiplanar reconstructions of pre- and post-operative CT scans were evaluated in a population of 336 patients implanted with the CII HiFocus1 or HiFocus1J implant (26 bilaterally implantees included). Cochlear radial distances were measured on four measurement axes on the preoperative CT scan. Electrode contact positions were obtained in angular depth, distance from the round window and to the modiolus center. Frequency mismatch was calculated based on the yielded frequency as a function of the angular position per contact. Cochlear diameters were clustered into three cochlear size groups with K-sample clustering. Using spiral fitting and general linear regression modeling, the feasibility of different insertion models with cochlear size measures and surgical insertion as input parameters was analyzed. The final developed model was internally validated with bootstrapping to calculate the optimism-corrected R. RESULTS: Frequency mismatch was minimalized for surgical insertion of 6.7 mm and insertion depth of 484°. Cochlear size clusters were derived consisting of a "small" (N = 117), "medium" (N = 171), and "large" (N = 74) cluster with mean insertion depths of 506°, 480°, and 441°, respectively. The relation between surgical insertion (LE16) and insertion depth (θE1) differed significantly between the three clusters (p < 0.01). The insertion models based on spiral fitting showed an R of 62% with mean of the residuals of -0.5 mm (SD = 1.2 mm) between the measured and predicted LE16 and a mean of 15° (SD = 83°) for θE1. Using general linear regression modeling resulted in a residual mean of -0.2 µm (SD = 0.9 mm) for measured and predicted LE16 and 0.01° (SD = 33°) for θE1. The model derived from general linear regression modeling resulted in an R of 78.7% and was validated with bootstrapping. An optimism of 0.6% was calculated using this analysis. The optimism-corrected R of 78.1% defined the estimated performance of the final insertion model in future populations. CONCLUSIONS: A minimal frequency mismatch for an electrode array design can be calculated to define preferable electrode array position within the cochlea. In general, to achieve a minimal frequency mismatch, the surgeon should attempt to insert the HiFocus 1 or 1J array around 6, 7, or 8 mm in case of a "small," "medium," or "large" cochlea, respectively. Development of different insertion models showed the feasibility of obtaining a surgical guidance tool to lead the surgeon during cochlear implantation depending on individual cochlear size and controllable surgical distance. The developed final insertion model predicted 78.1% of the variation in final HiFocus1 or HiFocus1J implant position.

The influence of cochlear implant electrode position on performance.

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.

The Huntington's Disease Dysphagia Scale.

BACKGROUND: Little is known about the swallowing disturbances of patients with Huntington's disease; therefore, we developed the Huntington's Disease Dysphagia Scale. METHODS: The scale was developed in four stages: (1) item generation, (2) comprehension testing, (3) evaluation of reliability, (4) item reduction and validity testing. The questionnaire was presented twice to 50 Huntington's disease patients and their caregivers. The Kruskal-Wallis test was used to evaluate whether the severity of swallowing difficulties increased with advancing disease. Pearson's correlation coefficient was used to examine the construct validity with the Swallowing Disturbance Questionnaire. RESULTS: The final version contained 11 items with five response options and exhibited a Cronbach's alpha coefficient of 0.728. The severity of swallowing difficulties was significantly higher in more advanced Huntington's disease. The correlation with the Swallowing Disturbance Questionnaire was 0.734. CONCLUSION: We developed a valid and reliable 11-item scale to measure the severity of dysphagia in Huntington's disease.

Diversity in cochlear morphology and its influence on cochlear implant electrode position.

OBJECTIVES: To define a minimal set of descriptive parameters for cochlear morphology and study its influence on the cochlear implant electrode position in relation to surgical insertion distance. DESIGN: Cochlear morphology and electrode position were analyzed using multiplanar reconstructions of the pre- and postoperative CT scans in a population of 336 patients (including 26 bilaterally implanted ones) with a CII HiFocus1 or HiRes90K HiFocus1J implant. Variations in cochlear diameter and cochlear canal size were analyzed. The relationship between the outer and inner walls was investigated. Size differences based on sex, age, and ear side were investigated using linear mixed models. Two new methods, spiral fitting and principal component analysis, were proposed to describe cochlear shape, and the goodness of fit was investigated. The relationship between cochlear shape and electrode position, in terms of modiolus proximity and insertion depth, was analyzed using clustering, one-way analysis of variance (ANOVA) and simple linear regression analysis. RESULTS: Large variations in cochlear morphology were found, with cochlear canal sizes ranging from 0.98 to 2.96 mm and average cochlear diameters between 8.85 and 5.92 mm (with standard deviations of around 0.4 mm). The outer and inner walls were significantly correlated (p < 0.01), and a size difference of 4% in favor of males was found. Spiral fitting shows good alignment of the true measurements, with residuals having a mean of 0.01 mm and a standard deviation of 0.29 mm. Principal component analysis (PCA) showed that the use of one component, which describes size, is sufficient to explain 93.6% of the cochlear shape variance. A significant sex difference was also found with spiral fitting and PCA. Cochlear size was found to have a significant influence on modiolus proximity and insertion depth of the electrode (p < 0.01). Cochlear size explained around 13% of the variance in electrode position. When cochlear size was combined with surgical insertion, more than 81% of the variance in insertion depth can be explained. CONCLUSIONS: This study demonstrates a large variety in cochlear morphology, which significantly impacts electrode position in terms of modiolus proximity and insertion depth. The effect size is, however, relatively small compared with surgical insertion distance. PCA is shown to be an accurate reduction method for describing cochlear shape.

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. METHODS: Two data sets were used, 1 containing 1 scan per patient (n = 134) and the other containing at least 3 consecutive MRIs of 51 patients, all with contrast-enhanced T1 or high-resolution T2 sequences. 2D measurements of the maximal extrameatal diameters in the axial plane were automatically derived from a 3D-convolutional neural network compared to manual measurements by 2 human observers. Intra- and interobserver variabilities were calculated using the intraclass correlation coefficient (ICC), agreement on tumor progression using Cohen's kappa. RESULTS: The human intra- and interobserver variability showed a high correlation (ICC: 0.98-0.99) and limits of agreement of 1.7 to 2.1 mm. Comparing the automated to human measurements resulted in ICC of 0.98 (95% confidence interval [CI]: 0.974; 0.987) and 0.97 (95% CI: 0.968; 0.984), with limits of agreement of 2.2 and 2.1 mm for diameters parallel and perpendicular to the posterior side of the temporal bone, respectively. There was satisfactory agreement on tumor progression between automated measurements and human observers (Cohen's κ = 0.77), better than the agreement between the human observers (Cohen's κ = 0.74). CONCLUSION: Automated 2D diameter measurements and growth detection of vestibular schwannomas are at least as accurate as human 2D measurements. In clinical practice, measurements of the maximal extrameatal tumor (2D) diameters of vestibular schwannomas provide important complementary information to total tumor volume (3D) measurements. Combining both in an automated measurement algorithm facilitates clinical adoption.

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. Methods and Materials: Six observers delineated the GTV in 10 different patients using the Big Brother contouring software. Patients were scanned at 3T MRI with a surface coil, and tumors were delineated separately on contrast enhanced 3DT1 (T1gd) and 3DT2-weighted scans with an isotropic acquisition resolution of 0.8 mm. Volume difference and overall local variation (median standard deviation of the distance between the delineated contours and the median contour) were analyzed for each GTV. Additionally, the local variation was analyzed for 4 interfaces: sclera, vitreous, retinal detachment, and tumor-choroid interface. Results: The average GTV was significantly larger on T1gd (0.57cm3) compared with T2 (0.51cm3, P = .01). A not significant higher interobserver variation was found on T1gd (0.41 mm) compared with T2 (0.35 mm). The largest variations were found at the tumor-choroid interface due to peritumoral enhancement (T1gd, 0.62 mm; T2, 0.52 mm). As a result, a larger part of this tumor-choroid interface appeared to be included on T1gd-based GTVs compared with T2, explaining the smaller volumes on T2. Conclusions: The interobserver variation of 0.4 mm on MRI are low with respect to the voxel size of 0.8 mm, enabling small treatment margins. We recommend delineation based on the T1gd-weighted scans, as choroidal tumor extensions might be missed.