Thirteen previous cases have been reported as neuronal intranuclear hyaline inclusion disease. The majority of patients have presented with movement disorders at less than 12 years of age followed by a progressive worsening of symptoms and, frequently, loss of cognitive function. Death has usually occurred by the second or third decade. Three have presented in the fifth through seventh decade with either movement disorders or dementia. These cases have been linked by the presence of eosinophilic neuronal intranuclear inclusions diffusely within the CNS and in peripheral ganglion cells. The patient in this case report also presented with a rapidly progressive movement disorder and at autopsy showed the characteristic intranuclear inclusions. Investigation of these inclusions did not reveal shared epitopes with neurofilaments or other intermediate filaments.

Objective To perform a systematic review of proposed sinus computed tomography (CT) scoring systems and determine their association with patient-reported outcome measures (PROMs). Data Sources PubMed, CINAHL, Scopus, and Cochrane Library. Review Methods A systematic search was conducted following the PRISMA guidelines (Preferred Reporting Items for Systematic Reviews and Meta-analyses) for studies describing CT scores and PROMs in patients with chronic rhinosinusitis. Results A total of 144 studies were included. Out of 20,741 patients, 53.6% were male and 55.5% had nasal polyposis. A meta-analysis of correlations revealed a moderate correlation between Lund-McKay (LM) and the 22-item Sinonasal Outcome Test (SNOT-22; r = 0.434, P < .001) and a weaker correlation between LM and the 20-item Sinonasal Outcome Test (SNOT-20; r = 0.257, P = .039). Meta-regression also revealed a weak association between LM and SNOT-20 (n = 25 studies) but no significant associations between Zinreich score and SNOT-22 or LM scores and PROMs, including SNOT-22 (n = 94 studies), Rhinosinusitis Disability Index (n = 25), nasal obstruction visual analog scale (n = 15), Chronic Sinusitis Survey (n = 12), Total Nasal Symptom Score (n = 4), Total Symptom Score (n = 3), and 12-Item Short Form Health Survey (n = 3). Conclusion There is essentially little association between radiologic grade and PROMs. CT grading systems with improved clinical utility are needed.

Background Left‐to‐right (L‐R) shunts are characterized by a pathological connection between high‐ and low‐pressure systems, leading to a mixing of oxygen‐rich blood with low oxygenated blood. They are typically diagnosed by phase‐contrast cardiac magnetic resonance imaging (MRI) which requires extensive planning. T2 is sensitive to blood oxygenation and may be able to detect oxygenation differences between the left (LV) and right ventricles (RV) caused by L‐R shunts. Purpose To test the feasibility of routine T2 mapping to detect L‐R shunts. Study Type Retrospective. Population Patients with known L‐R shunts (N = 27), patients with RV disease without L‐R shunts (N = 21), and healthy volunteers (HV; N = 52). Field Strength/Sequence 1.5 and 3 T/balanced steady‐state free‐precession (bSSFP) sequence (cine imaging), T2‐prepared bSSFP sequence (T2 mapping), and velocity sensitized gradient echo sequence (phase‐contrast MRI). Assessment Aortic (Qs) and pulmonary (Qp) flow was measured by phase‐contrast imaging, and the Qp/Qs ratio was calculated as a measure of shunt severity. T2 maps were used to measure T2 in the RV and LV and the RV/LV T2 ratio was calculated. Cine imaging was used to calculate RV end‐diastolic volume index (RV‐EDVi). Statistical Tests Wilcoxon test, paired t‐tests, Spearmen correlation coefficient, receiver operating curve (ROC) analysis. Significance level P < 0.05. Results The Qp/Qs and T2 ratios in L‐R shunt patients (1.84 ± 0.84 and 0.89 ± 0.07) were significantly higher compared to those in patients with RV disease (1.01 ± 0.03 and 0.72 ± 0.10) and in HV (1.04 ± 0.04 and 0.71 ± 0.09). A T2 ratio of >0.78 showed a sensitivity, specificity, and negative predictive value of 100%, 73.9%, and 100%, respectively, for the detection of L‐R shunts. The T2 ratio was strongly correlated with the severity of the shunt (r = 0.83). Data Conclusion RV/LV T2 ratio is an imaging biomarker that may be able to detect or rule‐out L‐R shunts. Such a diagnostic tool may prevent unnecessary phase‐contrast acquisitions in cases with RV dilatation of unknown etiology. Level of Evidence 3 Technical Efficacy Stage 2

Background: While various sinus computed tomography (CT) scoring systems have been proposed and used in the literature, no single system has been identified as superior. The strength of associations between CT scoring systems and measures of olfaction also remains unclear.Methods: A systematic review of PubMed, CINAHL, Scopus, and the Cochrane Library was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies that reported both CT scores and measures of olfaction in a cross-sectional manner were included. Results: A total of 37 studies were eligible for meta-analysis. Of 8035 patients with chronic rhinosinusitis, 55.6% were male patients and 53.2% had chronic rhinosinusitis with nasal polyps. Analysis by meta-regression was performed of Lund-Mackay (LM) versus Smell Identification Test-40 (SIT-40; 12 studies), Brief Smell Identification Test (BSIT; 10 studies), Sniffin' Sticks (SS; 10 studies), and Toyota & Takagi (T&T) olfactometry (four studies). A significant moderate association was found between LM and SIT-40 (R 2 = 0.612, p < 0.001) and LM and SS (R 2 = 0.612, p < 0.001). An association between LM and BSIT approached significance (R 2 = 0.461, p = 0.054). No significant associations were noted between LM and T&T olfactometry and between LM and SS when stratified by nasal polyp status. Conclusion: There is a significant moderate association of current CT scoring systems to SIT-40 and SS. Further research should focus on associations of objective measures of olfaction to CT scores of the nasal cavity, sinuses, and olfactory cleft, as well as other disease markers.

Ear and hearing-related conditions pose a significant global health burden, yet public health policy surrounding ear and hearing care (EHC) in low- and middle-income countries is poorly understood. The present study aims to characterize the inclusion of EHC in national health policy by analyzing National Health Policies, Strategies, and Plans in English, French, Spanish, Portuguese, and Arabic. Three ear and hearing care keywords were searched, including ear*, hear*, and deaf*. The terms “HIV/AIDS,” “tuberculosis,” and “malaria” were included as comparison keywords as these conditions have historically garnered political priority in global health. Of the 194 World Health Organization member states, there were 100 national policies that met inclusion criteria of document availability, searchable format, language, and absence of an associated national EHC strategy. These documents mentioned EHC keywords significantly less than comparison terms, with mention of hearing in 15 documents, ears in 11 documents, and deafness in 3 documents. There was mention of HIV/AIDS in 92 documents, tuberculosis in 88 documents, and malaria in 70 documents. Documents in low- and middle-income countries included significantly fewer mentions of EHC terms than those of high-income countries. We conclude that ear and hearing conditions pose a significant burden of disease but are severely underrepresented in national health policy, especially in low- and middle-income countries.