Association of Ear Anomalies and Hearing Loss Among Children With 22q11.2 Deletion Syndrome.

OBJECTIVE: To identify inner and middle ear anomalies in children with 22q11.2 deletion syndrome (22q11DS) and determine associations with hearing thresholds. STUDY DESIGN: Retrospective study. SETTING: Two tertiary care academic centers. METHODS: Children presenting with 22q11DS between 2010 and 2020 were included. Temporal bone imaging with computed tomography or magnetic resonance imaging was reviewed by 2 neuroradiologists. RESULTS: Twenty-two patients (12 female, 10 male) were identified. Forty-four ears were evaluated on imaging. There were 15 (34%) ears with abnormal semicircular canals, 14 (32%) with abnormal vestibules, 8 (18%) with abnormal ossicles, 6 (14%) with enlarged vestibular aqueducts, 4 (9.1%) with abnormal facial nerve canals, and 4 (9.1%) with cochlear anomalies. There were 25 ears with imaging and audiometric data. The median pure tone average (PTA) for ears with any structural abnormality was 41.0 dB, as compared with 28.5 dB for ears without any structural abnormality (P = .21). Of 23 ears with normal imaging, 6 (26%) had hearing loss in comparison with 13 (62%) of 21 ears with abnormalities (P = .02). Total number of anomalies per ear was positively correlated with PTA (Pearson correlation coefficient, R = 0.479, P = .01). PTA was significantly higher in patients with facial nerve canal anomalies (P = .002), vestibular aqueduct anomalies (P = .05), and vestibule anomalies (P = .02). CONCLUSIONS: Semicircular canal, ossicular, vestibular aqueduct, and vestibular anomalies were detected in children with 22q11DS, especially in the setting of hearing loss. Careful evaluation of anatomic anomalies is needed prior to surgical intervention in these patients.

Successful immunomodulatory treatment for recurrent xanthogranulomatous hypophysitis in an adolescent: illustrative case.

BACKGROUND: Xanthomatous lesions of the pituitary have been linked to ruptured or hemorrhagic Rathke's cleft cysts. Most cases are reported to resolve following radical resection. When recurrence does occur, there is no established treatment regimen. High-dose glucocorticoids have been reported to be beneficial in several published cases; however, their effects are often not sustained once therapy is discontinued. OBSERVATIONS: The authors report the case of an adolescent male who developed recurrent xanthogranulomatous hypophysitis associated with a Rathke's cleft cyst despite two surgical interventions. He was treated with a short course of dexamethasone followed by a maintenance course of celecoxib and mycophenolate mofetil. This regimen proved to be safe and well-tolerated, and it successfully prevented another recurrence of his xanthogranulomatous hypophysitis. LESSONS: This case demonstrates a novel nonsurgical approach to the management of recurrent xanthogranulomatous hypophysitis. It suggests a potential application of a combined corticosteroid-sparing immunosuppressive and anti-inflammatory regimen in other cases of refractory xanthogranulomatous hypophysitis.

Performance of the Neck Imaging Reporting and Data System as applied by general neuroradiologists to predict recurrence of head and neck cancers.

BACKGROUND: The Neck Imaging Reporting and Data System (NI-RADS) is used to assess imaging after head and neck cancer treatment. We evaluated NI-RADS with general neuroradiologists rather than with head and neck subspecialists. METHODS: Computed tomography and magnetic resonance imaging examinations with/without positron emission tomography from May 2018 to September 2020 were retrospectively identified. NI-RADS scores at the primary site and lymph nodes were provided by 21 neuroradiologists. Recurrence status was based on clinical and imaging findings. Area under the curve (AUC) was used to assess accuracy. RESULTS: We assessed 608 scans from 464 patients. For NI-RADS categories 1, 2, and 3, primary site recurrence rates were 5%, 29%, and 65% with AUC of 0.765, while lymph node recurrence rates were 3%, 10%, and 80% with AUC of 0.820. CONCLUSIONS: NI-RADS as used by general neuroradiologists is effective in separating head and neck cancers into discrete categories for predicting recurrent disease.

Pretreatment ADC predicts tumor control after Gamma Knife radiosurgery in solid vestibular schwannomas.

BACKGROUND: Radiosurgery is a well-established treatment for vestibular schwannomas (VSs), but it is often difficult to identify which tumors will respond to treatment. We sought to determine whether pretreatment or posttreatment tumor apparent diffusion coefficient (ADC) values could predict tumor control in patients undergoing Gamma Knife radiosurgery (GKRS) and whether these values could differentiate between cases of pseudoprogression and cases of true progression in the early posttreatment period. METHODS: We retrospectively identified patients who underwent GKRS for solid VSs between June 2008 and November 2016 and who had a minimum follow-up of 36 months. Pretreatment and posttreatment minimum, mean, and maximum ADC values were measured for the whole tumor volume and were compared between patients with tumor control and those with tumor progression. In patients with early posttreatment tumor enlargement, ADC values were compared between patients with pseudoprogression and those with true progression. RESULTS: Of the 44 study patients, 34 (77.3%) demonstrated tumor control at final follow-up. Patients with tumor control had higher pretreatment minimum (1.35 vs 1.09; p = 0.008), mean (1.80 vs 1.45; p = 0.004), and maximum (2.41 vs 1.91; p = 0.011) ADC values than patients with tumor progression. ADC values did not differ between patients with pseudoprogression and those with true progression at early posttreatment follow-up. CONCLUSIONS: ADC values may be helpful in predicting response to GKRS in patients with solid VSs but cannot predict which tumors will undergo pseudoprogression. Patients with higher pretreatment ADC values may be more likely to demonstrate posttreatment tumor control.

Accuracy and Interrater Reliability of CISS Versus Contrast-Enhanced T1-Weighted VIBE for the Presence of Optic Canal Invasion in Tuberculum Sellae Meningiomas.

BACKGROUND: The magnetic resonance imaging sequence used to assess optic canal invasion by tuberculum sella meningiomas (TSMs) has not been standardized. Both constructive interference in steady state (CISS) and contrast-enhanced T1-weighted volume-interpolated breath-hold examination (VIBE) sequences are frequently used. The aim of the present study was to compare the accuracy and interrater reliability of these sequences in predicting optic canal invasion by TSMs. METHODS: In the present retrospective study of 27 patients (54 optic canals) who had undergone endoscopic transtuberculum transplanum resection of TSMs, images from preoperative CISS and contrast-enhanced T1-weighted VIBE sequences were assessed by 5 neuroradiologists who were unaware of the operative findings. The readers evaluated the optic canal in 4 quadrants at 2 locations (the posterior tip of the anterior clinoid process and the optic strut). A quadrant was considered positive for tumor invasion if invasion was present at either of these 2 locations. The reference standard was intraoperative observation of gross optic canal invasion. RESULTS: The interrater agreement was good for the presence or absence of tumor involvement in a particular quadrant (CISS, 0.635; VIBE, 0.643; 95% confidence interval for the difference, -0.086 to 0.010). The mean sensitivity and specificity for optic nerve invasion were 0.643 and 0.438 with CISS and 0.643 and 0.454 with VIBE, respectively. No significant differences were seen between the sequences in terms of reader accuracy when the intraoperative findings were used as the reference standard. CONCLUSION: CISS and VIBE sequences both have good accuracy in predicting for optic canal tumor invasion by TMEs.

Predictors for the extent of pial collateral recruitment in acute ischemic stroke.

BACKGROUND: Pial arterioles can provide a variable degree of collateral flow to ischemic vascular territories during acute ischemic stroke. This study sought to identify predictive factors of the degree of pial collateral recruitment in acute ischemic stroke. METHODS: Clinical information and arteriograms from 62 consecutive patients with stroke due to either middle cerebral artery (MCA) M1 segment or internal carotid artery (ICA) terminus occlusion within 6 h following symptom onset were retrospectively reviewed. Pial collaterals were defined based on the extent of reconstitution of the MCA territory. Patients with slow antegrade flow distal to the occlusion site were excluded and no anesthetics were used prior or during angiography. Results were analyzed using multivariate nominal logistic regression. RESULTS: Better pial collateral recruitment was associated with proximal MCA versus ICA terminus occlusion (p = 0.005; odds ratio (OR) = 9.3; 95% confidence interval (CI), 2.16-53.3), lower presenting National Institutes of Health Stroke Scale Score (NIHSSS) (p = 0.023; OR = 6.51; 95% CI, 1.49-41.7), and lower diastolic blood pressure (p = 0.0411; OR = 5.05; 95% CI, 1.20-29.2). Age, gender, symptom duration, diabetes, laterality, systolic blood pressure, glucose level, hematocrit, platelet level, and white blood cell count at presentation were not found to have a statistically significant association with pial collateral recruitment. CONCLUSIONS: Extent of pial collateral recruitment is strongly associated with the occlusion site (MCA M1 segment versus ICA terminus) and less strongly associated with presenting NIHSSS and diastolic blood pressure.

Size matters: hemorrhage volume as an objective measure to define significant intracranial hemorrhage associated with thrombolysis.

BACKGROUND AND PURPOSE: This study defines significant thrombolysis associated intracranial hemorrhage (ICH) by identifying an objective threshold volume that predicts clinical deterioration attributable to ICH. METHODS: Prospectively collected clinical and radiographic information, from 103 consecutive patients who underwent intraarterial thrombolysis for acute ischemic stroke, was reviewed. Multiple paired comparisons between stratified hematoma volume and change in National Institutes of Health Stroke Scale (NIHSS) score by 24 to 36 hours and by time of hospital discharge was used to identify significant differences. Associations between hemorrhage volume and infarct volume in relation to clinical outcomes were examined. Rates of hemorrhagic transformation (HT), symptomatic hemorrhage, and parenchymal hematoma involving over 30% of the infarct were compared with hemorrhage volume. Multivariate regression analysis was used to determine the relationship between change in discharge NIHSS score and hemorrhage volume adjusting for known predictors of clinical outcomes. RESULTS: Multiple paired comparisons indicate that hemorrhage greater than 25 mL (HV25) had a more distinct impact on NIHSS score by time of hospital discharge than at 24 to 36 hours. Twenty-seven (26.2%) patients had HT and 12 (11.7%) had HV25. Among symptomatic hemorrhage, parenchymal hematoma involving over 30% of the infarct, and HV25, HV25 appeared more reflective of clinical deterioration from ICH. Hemorrhage volume increased with infarct volume but they were independently associated with change in NIHSS score on regression analysis. CONCLUSIONS: Clinical deterioration from ICH and ischemic injury are more effectively distinguished at time of hospital discharge. The authors propose to define significant hemorrhage associated with thrombolysis as hemorrhage volume greater than 25 mL.