Reproducibility and accuracy of vessel wall MRI in diagnosing giant cell arteritis: a study with readers of varying expertise.

OBJECTIVE: To evaluate the reproducibility of vessel wall magnetic resonance imaging (VW-MRI) in diagnosing giant cell arteritis (GCA) among groups of radiologists with varying levels of expertise. METHODS: This institutional review board-approved retrospective single-center study recruited patients with suspected GCA between December 2014 and September 2021. Patients underwent 3 -T VW-MRI before temporal artery biopsy. Ten radiologists with varying levels of expertise, blinded to all data, evaluated several intracranial and extracranial arteries to assess GCA diagnosis. Interobserver reproducibility and diagnostic performance were evaluated. RESULTS: Fifty patients (27 women and 23 men) with a mean age of 75.9 ± 9 years were included. Thirty-one of 50 (62%) had a final diagnosis of GCA.VW-MRI had an almost perfect reproducibility among expert readers (kappa = 0.93; 95% CI 0.77-1) and substantial reproducibility among all readers, junior and non-expert senior readers (kappa = 0.7; 95% CI 0.66-0.73; kappa = 0.67 95% CI 0.59-0.74; kappa = 0.65; 95% CI 0.43-0.88 respectively) when diagnosing GCA. Substantial interobserver agreement was observed for the frontal branch of superficial temporal artery. Moderate interobserver agreement was observed for the superficial temporal artery and its parietal branch, as well as ophthalmic arteries in all groups of readers. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy varied depending on the group of readers. CONCLUSION: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers. This study advocates for the use of VW-MRI when diagnosing GCA even in less-experienced centers. CLINICAL RELEVANCE STATEMENT: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers, and it could be used as a first-line diagnostic tool for GCA in centers with limited expertise in GCA diagnosis. KEY POINTS: • Vessel wall magnetic resonance imaging (VW-MRI) is a reproducible and accurate imaging modality for detecting giant cell arteritis (GCA) in both extracranial and intracranial arteries. • The reproducibility of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis was high among expert readers and moderate among less-experienced readers. • The use of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis can be recommended even in centers with less-experienced readers.

Local budesonide therapy in the management of persistent hyposmia in suspected non-severe COVID-19 patients: Results of a randomized controlled trial.

OBJECTIVES: Consequences of COVID-19 on olfactory functions remained unclear during the pandemic. We assessed the efficacy of local budesonide in addition to olfactory rehabilitation when managing non-severe COVID-19 patients with persistent hyposmia. METHODS: A multicentric, randomized, superiority trial was conducted (ClinicalTrials.gov NCT04361474). The experimental group (EG) received budesonide and physiological saline nasal irrigations administered via three syringes of 20 ml in each nasal cavity in the morning and evening for 30 days. The control group (CG) received a similar protocol without budesonide. Patients were included if they were >18 years old, with a SARS-CoV-2 infection and presenting an isolated hyposmia persisting 30 days after symptom onset. The primary endpoint was the percentage of patients with improvement of more than two points on the ODORATEST score after 30 days of treatment. RESULTS: In total, 123 patients were included and randomized (EG: 62 vs CG: 61). Two patients from the EG met the primary endpoint with no statistical difference between the two groups (P = 0.5). CONCLUSION: To our knowledge, this is the first study evaluating local budesonide for COVID-19 related hyposmia treatment even though previous trials were performed with other local corticosteroids. Local budesonide efficacy was not demonstrated for persistent hyposmia related to COVID-19.

Complication of Ear Impression: Foreign Body in the Eustachian Tube Eroding the Carotid Canal.

The realization of customized earing plugs and earmolds for hearing aids requires an impression of the external auditory canal to obtain a siliconized mold. Silicone used for ear impressions is known to be safe and inert but deposition of silicone in the middle ear can middle and inner ear damages. We present a case of accidental injection of silicone in the middle ear and the Eustachian tube resulting in an erosion of the carotid canal. Laryngoscope, 133:3358-3360, 2023.

Early diffusion-weighted MRI at 3 Tesla detects ischemic changes of the optic nerve in anterior ischemic optic neuropathy.

OBJECTIVES: To assess the impact of timing from visual symptoms' onset to diffusion-weighted (DW) 3 T MRI completion to detect ischemic changes of the optic disc and optic nerve in AION patients. METHODS: This IRB-approved retrospective single-center study included 3 T MRI data from 126 patients with AION and 111 controls with optic neuritis treated between January 2015 and May 2020. Two radiologists blinded to all data individually analyzed imaging. A senior neuroradiologist resolved any discrepancies by consensus. The primary judgment criterion was the restricted diffusion of the optic disc and/or the optic nerve assessed subjectively on the ADC maps. ADC values were also measured. Spearman rank correlations were used to examine the relationships between timing from visual symptoms' onset to MRI completion and both the restricted diffusion and the ADC values. RESULTS: One hundred twenty-six patients (47/126 [37.3%] women and 79/126 [62.7%] men, mean age 69.1 ± 13.7 years) with AION were included. Restricted diffusion of the optic disc in AION eyes was more frequent in the early MRI group than in the late MRI group: 35/49 (71.4%) eyes versus 3/83 (3.6%) eyes, p < 0.001. ADC values of the pathological optic discs and optic nerves were lower in the early MRI group than in the late MRI group: 0.61 [0.52-0.94] × 10-3 mm2/s versus 1.28 [1.01-1.44] × 10-3 mm2/s, p < 0.001, and 0.74 [0.61-0.88] × 10-3 mm2/s versus 0.89 [0.72-1.10] × 10-3 mm2/s, p < 0.001, respectively. CONCLUSIONS: DWI MRI showed good diagnostic performance to detect AION when performed early after the onset of visual symptoms. KEY POINTS: • Restricted diffusion of the optic disc in eyes affected by AION was significantly more likely to be observed in patients who had undergone MRI within 5 days after onset of visual symptoms. • ADC values of the pathological optic discs and optic nerves were significantly lower in patients who had undergone MRI within 5 days after onset of visual symptoms of AION: 0.61 × 10-3 mm2/s versus 1.28 × 10-3 mm2/s, p < 0.001, and 0.74 × 10-3 mm2/s versus 0.89 × 10-3 mm2/s, p < 0.001, respectively. • The optimal threshold for timing from visual symptoms' onset to MRI completion to detect restricted diffusion of the optic disc and/or optic nerve was 5 days, with an AUC of 0.88 (CI95%: 0.82-0.94).

MRI characteristics of intralabyrinthine schwannoma on post-contrast 4 h-delayed 3D-FLAIR imaging.

PURPOSE: The purpose of this study was to describe the MRI characteristics of intralabyrinthine schwannoma (ILS) on post contrast three-dimensional (3D) fluid-attenuation-inversion-recovery (FLAIR) images obtained four hours after intravenous administration of a gadolinium-based contrast agent (4h-3D-FLAIR). MATERIALS AND METHODS: This IRB-approved retrospective multi-center study included patients presenting with typical ILS from January 2016 to October 2020. All medical charts were systematically collected. All MRI examinations, including 4h-3D-FLAIR images, were reviewed by two board-certified neuroradiologists. Main outcome measures were location, signal intensity and associated anomalies of ILS. RESULTS: Twenty-seven out of 8730 patients (0.31%) referred for the investigation of a cochleovestibular disorder had a final diagnosis of ILS. There were 13 men and 14 women with a mean age of 52 ± 17 (SD) years (age range: 20-86 years). The most common clinical presentation was unilateral progressive sensorineural hearing loss (16/27; 59%). All ILS were unilateral and 15 (15/27; 55%) were intracochlear. All ILS presented as a hypointense filling defect within the labyrinth on T2-weighted images that enhanced on post-contrast T1-weighted images. On 4h-3D-FLAIR images, all ILS presented as a hypointense filling defect, associated with diffuse perilymphatic hyperintensity. Two patients (2/27; 7%) presented with ipsilateral endolymphatic hydrops. CONCLUSION: ILS displays consistent features on post-contrast 4h-3D-FLAIR images. ILS should not be confused with endolymphatic hydrops and requires a systematic analysis of the corresponding T2-weighted images.

IgG4-related pachymeningitis and mastoiditis, associated with cerebral venous thrombosis: A case report.

IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition; this can be a challenging diagnosis that requires clinico-pathologic correlation. We report a young woman, presenting with cranial nerve palsy. The work-up revealed pachymeningitis, cerebral venous thrombosis (CVT), and a destructive lesion in the mastoid. We diagnosed IgG4-RD through mastoidectomy. Thus, a biopsy of asymptomatic, infrequently affected organs, like the mastoid, can meet all histopathological criteria. In neuro-meningeal presentations, CVT may be secondary to the local inflammatory environment of pachymeningitis. Since our patient had a deep vein thrombosis one year prior, we discuss a possible higher risk of thrombosis in IgG4-RD patients.

Study of the Comorbidity Between Cases of Acute Peripheral Vestibulopathies and COVID-19.

IMPORTANCE: An infective etiology of acute peripheral vestibulopathy (APV) has long been hypothesized. In the context of coronavirus disease 2019 (COVID-19), we examined the possible comorbidity between these two entities. OBJECTIVES: APV is the second most common cause of vestibular disorders and results from a sudden and unilateral loss of vestibular inputs. The characteristic signs and symptoms include sudden and prolonged vertigo, absence of auditory symptoms, and absence of other neurological symptoms. An infective etiology of APV has long been hypothesized on the basis of its association with respiratory tract infections and its frequent occurrence in epidemics. Possible comorbidity with herpes simplex virus type 1 reactivation or influenza virus infection has also been proposed. This study was designed to assess the possible comorbidity between APV and COVID-19. DESIGN/SETTING/PARTICIPANTS: Quantification of the number of hospital admissions for APV over the period from February to May 2020 was carried out in 5 French hospitals. A comparison with 2018 and 2019 entries over the same period was made. Comorbidity between APV and COVID-19 infection was investigated. RESULTS: No significant increase in admission for APV was noticed over the examination period. No significant difference was noticed among hospitals located in COVID-19 high- and low-risk zones for SARS-CoV-2. No significant increase in the severity of the APV cases was noticed. No case of comorbidity between APV and SARS-CoV-2 infection was reported. Based on our observations, no correlation was made between APV and COVID-19. CONCLUSION: Based on our observations, COVID-19 is not statistically correlated with APV.

High-resolution MRI demonstrates signal abnormalities of the 3rd cranial nerve in giant cell arteritis patients with 3rd cranial nerve impairment.

OBJECTIVE: To determine the sensitivity and specificity of high-resolution (HR) MRI for detecting signal abnormalities of cranial nerves (CN) in giant cell arteritis (GCA) patients presenting with diplopia. METHODS: This IRB-approved retrospective single-center study included GCA patients who underwent 3-T HR MRI from December 2014 to January 2020. Two radiologists, blinded to all data, individually assessed for the presence of enhancement of the 3rd, 4th, and/or 6th CN on post-contrast HR imaging and high signal intensity on HR T2-WI, for signal abnormalities of extraocular muscles and the brainstem, and for inflammatory changes of the ophthalmic and extracranial arteries. A Fisher's exact test was used to compare patients with or without diplopia. RESULTS: In total, 64 patients (42/64 (66%) women and 22/64 (34%) men, mean age 76.3 ± 8 years) were included. Of the 64 patients, 14 (21.9%) presented with diplopia. Third CN enhancement was detected in 7/8 (87.5%) patients with 3rd CN impairment, as compared to no patients with 4th or 6th CN impairment or to patients without diplopia (p < 0.001). Third CN abnormal high signal intensity on HR T2-WI was detected in 4/5 patients (80%) with 3rd CN impairment versus none of other patients (p < 0.001). Sensitivity, specificity, positive predictive value, and negative predictive value for detecting 3rd CN signal abnormalities were of 0.88, 1, 1, and 0.99 and 0.8, 1, 1, and 0.98 for post-contrast HR imaging and HR T2-WI, respectively. CONCLUSIONS: HR MRI had excellent diagnostic sensitivity and specificity when detecting signal abnormalities of the 3rd CN in GCA patients presenting with 3rd CN impairment. KEY POINTS: • Third cranial nerve enhancement was detected in all patients with 3rd cranial nerve impairment except for one with transient diplopia. • The "check mark sign" might be useful to identify 3rd cranial nerve signal abnormalities in the orbital apex. • No signal abnormalities of the 4th or 6th cranial nerves could be detected on high-resolution MRI.

MRI diagnosis of saccular hydrops: Comparison of heavily-T2 FIESTA-C and 3D-FLAIR sequences with delayed acquisition.

PURPOSE: Currently, 3D-FLAIR sequence performed 4hours after the intravenous administration of a single dose of contrast media is the imaging technique of choice for the diagnosis of saccular hydrops (SH). Recently, the diagnosis of SH has also been reported with heavily-T2 weighted sequences. MATERIALS AND METHODS: In this retrospective imaging study, we performed 3D-FLAIR sequences 4hours after contrast media administration and 3D FIESTA-C sequences before and 4hours after contrast media administration in 30 patients with unilateral definite, probable or possible clinical diagnosis of Menière's disease (MD). Two radiologists, blinded to the clinical data, independently assessed the presence of saccular hydrops. Inter-reader agreement tests were performed. RESULTS: On delayed post-contrast 3D-FLAIR sequence, 15 patients out of 30 referred with a SH that was never seen on the controlateral asymptomatic side. The specificity and the sensitivity to detect MD side were 100% and 50% respectively. On non-enhanced 3D FIESTA-C sequence, 16 patients out of 30 (53%) referred with a saccular hydrops that was observed in 6 patients on the clinical asymptomatic ear. The specificity and the sensitivity to detect MD side were 80% and 33% respectively. On delayed 3D FIESTA-C sequence, 13 patients out of 30 (43%) referred with a saccular hydrops that was seen in 4 patients on the controlateral asymptomatic side. The specificity and the sensitivity to detect MD side were 83% and 27% respectively. CONCLUSION: Delayed post-contrast 3D-FLAIR is highly specific of MD symptoms while 3D FIESTA-C sequences are less sensitive and specific for the diagnosis of SH.

Efficacy of local budesonide therapy in the management of persistent hyposmia in COVID-19 patients without signs of severity: A structured summary of a study protocol for a randomised controlled trial.

OBJECTIVES: To assess the efficacy of local intranasal treatment with budesonide (nasal irrigation), in addition to olfactory rehabilitation, in the management of loss of smell in COVID-19 patients without signs of severity and with persistent hyposmia 30 days after the onset of symptoms. To search for an association between the presence of an obstruction on MRI and the severity of olfactory loss, at inclusion and after 30 days of treatment. TRIAL DESIGN: Two center, open-label, 2-arm (1:1 ratio) parallel group randomized controlled superiority trial. PARTICIPANTS: Inclusion criteria - Patient over 18 years of age; - Patient with a suspected SARS-CoV-2 infection, whether or not confirmed by PCR, or close contact with a PCR-confirmed case, typical chest CT scan (unsystematic frosted glass patches with predominantly sub-pleural appearance, and at a later stage, alveolar condensation without excavation or nodules or masses) or positive serology ; - Patient with isolated sudden onset hyposmia persisting 30 days after the onset of symptoms of CoV-2 SARS infection; - Affiliate or beneficiary of a social security scheme; - Written consent to participate in the study. Non-inclusion criteria - Known hypersensitivity to budesonide or any of the excipients; - Hemostasis disorder or epistaxis; - Oral-nasal and ophthalmic herpes virus infection; - Long-term corticosteroid treatment; - Treatment with potent CYP3A4 inhibitors (e.g., ketoconazole, itraconazole, voriconazole, posaconazole, clarithromycin, telithromycin, nefazodone and HIV protease inhibitors); - Severe forms of SARS-CoV-2 with respiratory or other signs; - Hyposmia persisting for more than 90 days after the onset of symptoms - Other causes of hyposmia found on interrogation or MRI; - Patient benefiting from a legal protection measure; - Pregnant or breastfeeding women. The participants will be recruited from: Hôpital Fondation Adolphe de Rothschild and Hôpital Lariboisière in Paris, France INTERVENTION AND COMPARATOR: Intervention: Experimental group: Nasal irrigation with budesonide and physiological saline (Budesonide 1mg/2mL diluted in 250mL of physiological saline 9°/00): 3 syringes of 20mL in each nasal cavity, morning and evening, for 30 days, in addition to olfactory rehabilitation twice a day. CONTROL GROUP: Nasal irrigation with physiological saline 9°/00 only: 3 syringes of 20cc in each nasal cavity, morning and evening, for 30 days, in addition to olfactory rehabilitation twice a day. MAIN OUTCOMES: Percentage of patients with an improvement of more than 2 points on the ODORATEST score after 30 days of treatment. RANDOMISATION: Patients will be randomized (1:1) between the experimental and control groups, using the e-CRF. The randomization list will be stratified by centre. BLINDING (MASKING): Participants and caregivers are aware of the group assignment. People assessing the outcomes are blinded to the group assignment Numbers to be randomised (sample size) 120 patients are planned to be randomized into two groups of 60 patients. TRIAL STATUS: MDL_2020_10. Version number 2, May 22, 2020. Recruitment started on May 22, 2020. The trial will finish recruiting by August 2020. TRIAL REGISTRATION: EUDRACT number: 2020-001667-85; date of trial registration: 15 May 2020 Protocol registered on ClinicalTrial.gov, registration number: NCT04361474 ; date of trial registration: 24 April 2020. FULL PROTOCOL: The full protocol is attached as an additional file, accessible from the Trials website (Additional file 1). In the interest in expediting dissemination of this material, the familiar formatting has been eliminated; this Letter serves as a summary of the key elements of the full protocol.

Endolymphatic Hydrops Secondary to Spontaneous Intracranial Hypotension.

BACKGROUND: Spontaneous intracranial hypotension may be associated with neuro-otological symptoms that might mimic Menière's disease. CASE PRESENTATION: We report the case of a 53-year-old male presenting bi-frontal headache with recurrent spells of vertigo, left fluctuating hearing loss, and tinnitus. Dedicated brain and inner ear Magnetic Resonance Imaging, including a post-contrast 4 hours delayed FLAIR sequence, revealed typical signs of spontaneous intracranial hypotension associated with endolymphatic hydrops involving the left saccule and cochlea. CONCLUSIONS: Audio vestibular manifestations mimicking Menière's disease in spontaneous intracranial hypotension could be explained by endolymphatic hydrops, which can be detected using dedicated magnetic resonance imaging sequences.

Myelin oligodendrocyte glycoprotein antibody-associated diffuse orbital inflammation.

An 87-year-old man presented with a 2-day history of painful bilateral visual loss. On examination, exophthalmos, lid edema, chemosis, and optic disc edema, on the left side only, were found. Visual acuity was 4/10 OD and no light perception OS. Magnetic resonance imaging (MRI) revealed bilateral optic neuritis and a diffuse and severe infiltration of the intra- and extraconal fat on the left. Laboratory testing was negative except for serum myelin oligodendrocyte glycoprotein (MOG) antibodies. This presentation adds a new variant to the MOG-associated disease spectrum. Testing for MOG antibodies should be considered in patients presenting with diffuse orbital inflammation and optic neuritis.

Clinical and radiological characteristics of patients with collapse or fistula of the saccule as evaluated by inner ear MRI.

Background: Delayed 3D-FLAIR sequences enable the distinction between the utricle and the saccule.Aims/objectives: We sought to evaluate the clinical and radiological findings in patients with no visible saccule (NVS) on 4-hour post-contrast MRI.Material and Methods: We retrospectively assessed the presence of NVS signs in 400 patients who underwent delayed inner ear MRI.Results: We reported on 28 patients with NVS. Among this group, on the NVS affected side: 14 had isolated sensorineural hearing loss (SNHL); 4 had fluctuating cochleo-vestibular disease; 3 had definite Menière's disease; 3 had Minor syndrome; 2 had delayed endolymphatic hydrops (EH); 2 had inner ear malformations; 1 had sudden cochleo-vestibular deficit following stapes surgery; 1 had a perilymphatic fistula and 1 had a contralateral fluctuating SNHL. Sixteen out of these 28 patients (57.1%) had cochlear hydrops on the same side as the NVS, while 10 patients (35.7%) had saccular hydrops on the contralateral side. Moreover, isolated blood labyrinth barrier (BLB) impairment on the NVS side was observed in 7 patients. Two patients (7.1%) had large vestibular aqueduct and NVS on the same side and one patient had perilymphatic fistula.Conclusions and significance: NVS seems to be multifactorial and could be linked to hydropic ear disease, third-mobile window pathologies and congenital malformation.

Increased diagnostic accuracy of giant cell arteritis using three-dimensional fat-saturated contrast-enhanced vessel-wall magnetic resonance imaging at 3 T.

OBJECTIVES: To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-VW) MRI of extracranial and intracranial arteries in the diagnosis of GCA. METHODS: This prospective two-center study was approved by a national research ethics board and enrolled participants from December 2014 to October 2017. A protocol including both a 2D and a 3D CE-VW MRI at 3 T was performed in all patients. Two neuroradiologists, blinded to clinical data, individually analyzed separately and in random order 2D and 3D sequences in the axial plane only or with reformatting. The primary judgment criterion was the presence of GCA-related inflammatory changes of extracranial arteries. Secondary judgment criteria included inflammatory changes of intracranial arteries and the presence of artifacts. A McNemar's test was used to compare 2D to 3D CE-VW MRIs. RESULTS: Seventy-nine participants were included in the study (42 men and 37 women, mean age 75 (± 9.5 years)). Fifty-one had a final diagnosis of GCA. Reformatted 3D CE-VW was significantly more sensitive than axial-only 3D CE-VW or 2D CE-VW when showing inflammatory change of extracranial arteries: 41/51(80%) versus 37/51 (73%) (p = 0.046) and 35/50 (70%) (p = 0.03). Reformatted 3D CE-VW was significantly more specific than 2D CE-VW: 27/27 (100%) versus 22/26 (85%) (p = 0.04). 3D CE-VW showed higher sensitivity than 2D CE-VW when detecting inflammatory changes of intracranial arteries: 10/51(20%) versus 4/50(8%), p = 0.01. Interobserver agreement was excellent for both 2D and 3D CE-VW MRI: κ = 0.84 and 0.82 respectively. CONCLUSIONS: 3D CE-VW MRI supported more accurate diagnoses of GCA than 2D CE-VW. KEY POINTS: • 3D contrast-enhanced vessel-wall magnetic resonance imaging is a high accuracy, non-invasive diagnostic tool used to diagnose giant cell arteritis. • 3D contrast-enhanced vessel-wall imaging is feasible for clinicians to complete within a relatively short time, allowing immediate assessment of extra and intracranial arteries. • 3D contrast-enhanced vessel-wall magnetic resonance imaging might be considered a diagnostic tool when intracranial manifestation of GCA is suspected.