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Item A single-cell level comparison of human inner ear organoids with the human cochlea and vestibular organs(Cell Press, 2023) van der Valk, Wouter H.; van Beelen, Edward S. A.; Steinhart, Matthew R.; Nist-Lund, Carl; Osorio, Daniel; de Groot, John C. M. J.; Sun, Liang; van Benthem, Peter Paul G.; Koehler, Karl R.; Locher, Heiko; Otolaryngology -- Head and Neck Surgery, School of MedicineInner ear disorders are among the most common congenital abnormalities; however, current tissue culture models lack the cell type diversity to study these disorders and normal otic development. Here, we demonstrate the robustness of human pluripotent stem cell-derived inner ear organoids (IEOs) and evaluate cell type heterogeneity by single-cell transcriptomics. To validate our findings, we construct a single-cell atlas of human fetal and adult inner ear tissue. Our study identifies various cell types in the IEOs including periotic mesenchyme, type I and type II vestibular hair cells, and developing vestibular and cochlear epithelium. Many genes linked to congenital inner ear dysfunction are confirmed to be expressed in these cell types. Additional cell-cell communication analysis within IEOs and fetal tissue highlights the role of endothelial cells on the developing sensory epithelium. These findings provide insights into this organoid model and its potential applications in studying inner ear development and disorders.Item A Study of the Correlation of Incisive Biting Forces with Age, Sex and Anterior Occlusion(1971) Kotwal, Navroze Shavak; Standish, S. Miles; Chalian, Varoujan A.; Shanks, James C.This study was designed to prove or disprove the fact that incisive biting force can be correlated with the variables chosen which were age, sex and anterior occlusion (overjet, overbite and cuspid relation). The amount of linear contact made by the incisal edges of the upper and lower incisors was also included as one of the variables. One hundred and fifty individuals, 80 males and 70 females, between the ages of 10 and 25 were selected for this study. This was a cross sectional study in which four readings of the maximum biting ability were recorded for every individual just once during the study and an average of the four recordings was taken. An intra-oral force transducer using strain gages was employed to record incisive biting forces. The results indicated that age, sex and amount of linear contact were correlated with biting force when considered singly. When the variables were considered jointly through a stepwise regression analysis overbite also entered as a significant predictor of biting force in addition to the three mentioned variables. Multiple correlation coefficient R was 0.54 for the four entered variables (age squared, sex, overbite and linear contact squared) with biting force. The R squared value was 0.29 so that 29 percent of the variation in incisive biting force of this sample was due to these four variables. The correlated R-squared value indicates a large error of prediction and a low degree of precision in estimating biting force from these four variables. Therefore, a regression equation is not demonstrated.Item Adult Diffuse Hepatic Hemangiomatosis: A Rare Cause of Dilated Cardiomyopathy and Sudden Cardiac Arrest(Springer, Part of Springer Science+Business Media, 2014-01) Supakul, Rodjawan; Vakili, Saeed T.; Liangpunsakul, Suthat; Department of Medicine, IU School of MedicineItem An analysis of hearing aid fittings in adults using cochlear implants and contralateral hearing aids(Wiley, 2010-12) Harris, Michael S.; Hay-McCutcheon, Marcia; Otolaryngology -- Head and Neck Surgery, School of MedicineOBJECTIVES/HYPOTHESIS: The objective of this study was to assess the appropriateness of hearing aid fittings within a sample of adult cochlear implant recipients who use a hearing aid in the contralateral ear (i.e., bimodal stimulation). METHODS: The hearing aid gain was measured using real ear testing for 14 postlingually deaf English-speaking adults who use a cochlear implant in the contralateral ear. Unaided and aided audiometric testing assessed the degree of functional gain derived from hearing aid use. RESULTS: On average, the target to actual output level difference was within 10 dB only at frequencies of 750 Hz and 1,000 Hz. Only 1 of the 14 study participants had a hearing aid for which the majority of the tested frequencies were within 10 dB of the target gain. In addition, a greater amount of functional gain (i.e., the increase in unaided behavioral thresholds after amplification) was provided for lower frequencies than higher frequencies. CONCLUSIONS: Hearing aid settings in our sample were suboptimal and may be regarded as a contributing factor to the variability in bimodal benefit. Refining hearing aid fitting strategies tailored to the needs of the concurrent cochlear implant and hearing aid user is recommended.Item Anterior mediastinal neuroblastoma in an adult: an additional case of a rare tumor in an unusual location with review of the literature(BMC, 2023-11-29) Collins, Katrina; Ulbright, Thomas M.; Davis, Jessica L.; Pathology and Laboratory Medicine, School of MedicineNeuroblastoma is rare in the adult population, especially in thoracic or mediastinal locations, with only 25 previously reported cases. We report an additional example of primary thymic neuroblastoma in a previously asymptomatic 71-year-old man with an anterior mediastinal mass who underwent robotic excision with pericardium and adjacent lung. The tumor was a 5.2 cm partially encapsulated, white-tan and rubbery mass with grossly identifiable areas of necrosis (25%) and hemorrhage. Histologically, the specimen showed a rim of adipose tissue and residual thymic tissue with areas of cystic thymic epithelium and prominent lymphoid tissue containing Hassall’s corpuscles. The tumor was composed of uniform, round cells with scant cytoplasm and small nuclei with inconspicuous nucleoli set within a background of conspicuous neuropil. Mitotic figures were easily found. By immunohistochemistry, the tumor cells expressed synaptophysin, chromogranin, NKX2.2 (diffuse, nuclear), GFAP (patchy), SMI31 (neurofilament) (focal, cytoplasmic), and TdT (diffuse, nuclear), while lacking expression of CD99, TTF-1, CK 20, MCPyV, PHOX2B, Olig2, OCT3/4, CD45, CD3 and PAX5. S100 protein was negative in the neuroblastic cells, with scattered positive cells in a vague sustentacular-like pattern. Fluorescence in situ hybridization for isochromosome 12p and EWSR1 gene rearrangement were negative. As thymic neuroblastoma is extremely rare in adults, a neuroblastic tumor of germ cell origin (either primary or metastatic) or spread from a sinonasal tract tumor should be excluded because of differing treatments and prognoses. The properties of these rare neoplasms appear similar to olfactory neuroblastoma rather than pediatric-type neuroblastoma.Item ARID1B-related disorder in 87 adults: Natural history and self-sustainability(Elsevier, 2024-07-23) van der Sluijs, P. J.; Gösgens, M.; Dingemans, A. J. M.; Striano, P.; Riva, A.; Mignot, C.; Faudet, A.; Vasileiou, G.; Walther, M.; Schrier Vergano, S. A.; Alders, M.; Alkuraya, F. S.; Alorainy, I.; Alsaif, H. S.; Anderlid, B.; Bache, I.; van Beek, I.; Blanluet, M.; van Bon, B. W.; Brunet, T.; Brunner, H.; Carriero, M. L.; Charles, P.; Chatron, N.; Coccia, E.; Dubourg, C.; Earl, R. K.; Eichler, E. E.; Faivre, L.; Foulds, N.; Graziano, C.; Guerrot, A. M.; Hashem, M. O.; Heide, S.; Heron, D.; Hickey, S. E.; Hopman, S. M. J.; Kattentidt-Mouravieva, A.; Kerkhof, J.; Klein Wassink-Ruiter, J. S.; Kurtz-Nelson, E. C.; Kušíková, K.; Kvarnung, M.; Lecoquierre, F.; Leszinski, G. S.; Loberti, L.; Magoulas, P. L.; Mari, F.; Maystadt, I.; Merla, G.; Milunsky, J. M.; Moortgat, S.; Nicolas, G.; O'Leary, M.; Odent, S.; Ozmore, J. R.; Parbhoo, K.; Pfundt, R.; Piccione, M.; Pinto, A. M.; Popp, B.; Putoux, A.; Rehm, H. L.; Reis, A.; Renieri, A.; Rosenfeld, J. A.; Rossi, M.; Salzano, E.; Saugier-Veber, P.; Seri, M.; Severi, G.; Sonmez, F. M.; Strobl-Wildemann, G.; Stuurman, K. E.; Uctepe, E.; Van Esch, H.; Vitetta, G.; de Vries, B. B. A.; Wahl, D.; Wang, T.; Zacher, P.; Heitink, K. R.; Ropers, F. G.; Steenbeek, D.; Rybak, T.; Santen, G. W. E.; Pediatrics, School of MedicinePurpose: ARID1B is one of the most frequently mutated genes in intellectual disability cohorts. Thus, far few adult-aged patients with ARID1B-related disorder have been described, which limits our understanding of the disease's natural history and our ability to counsel patients and their families. Methods: Data on patients aged 18+ years with ARID1B-related disorder were collected through an online questionnaire completed by clinicians and parents. Results: Eighty-seven adult patients with ARID1B were included. Cognitive functioning ranged from borderline to severe intellectual disability. Patients identified through the genetic workup of their child were either mosaic or had a variant in exon 1. New clinical features identified in this population are loss of skill (16/64, 25%) and recurrent patella luxation (12/45, 32%). Self-sustainability data showed that 88% (45/51) could eat independently, and 16% (7/45) could travel alone by public transport. Facial photo analysis showed that patients' photographs taken at different ages clustered consistently, separate from matched controls. Conclusion: The ARID1B spectrum is broad, and as patients age, there is a significant shift in the medical aspects requiring attention. To address the changing medical needs with increasing age, we have formulated recommendations to promote timely intervention in an attempt to mitigate disease progression.Item Attention-deficit/hyperactivity disorder medication and seizures(American Academy of Neurology, 2018-03-27) Wiggs, Kelsey K.; Chang, Zheng; Quinn, Patrick D.; Hur, Kwan; Gibbons, Robert; Dunn, David; Brikell, Isabell; Larsson, Henrik; D'Onofrio, Brian M.; Psychiatry, School of MedicineOBJECTIVE: Individuals with attention-deficit/hyperactivity disorder (ADHD) are at increased risk of seizures, but there is uncertainty about whether ADHD medication treatment increases risk among patients with and without preexisting seizures. METHODS: We followed a sample of 801,838 patients with ADHD who had prescribed drug claims from the Truven Health MarketScan Commercial Claims and Encounters databases to examine whether ADHD medication increases the likelihood of seizures among ADHD patients with and without a history of seizures. First, we assessed overall risk of seizures among patients with ADHD. Second, within-individual concurrent analyses assessed odds of seizure events during months when a patient with ADHD received ADHD medication compared with when the same individual did not, while adjusting for antiepileptic medications. Third, within-individual long-term analyses examined odds of seizure events in relation to the duration of months over the previous 2 years patients received medication. RESULTS: Patients with ADHD were at higher odds for any seizure compared with non-ADHD controls (odds ratio [OR] = 2.33, 95% confidence interval [CI] = 2.24-2.42 males; OR = 2.31, 95% CI = 2.22-2.42 females). In adjusted within-individual comparisons, ADHD medication was associated with lower odds of seizures among patients with (OR = 0.71, 95% CI = 0.60-0.85) and without (OR = 0.71, 95% CI = 0.62-0.82) prior seizures. Long-term within-individual comparisons suggested no evidence of an association between medication use and seizures among individuals with (OR = 0.87, 95% CI = 0.59-1.30) and without (OR = 1.01, 95% CI = 0.80-1.28) a seizure history. CONCLUSIONS: Results reaffirm that patients with ADHD are at higher risk of seizures. However, ADHD medication was associated with lower risk of seizures within individuals while they were dispensed medication, which is not consistent with the hypothesis that ADHD medication increases risk of seizures.Item Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review(Taylor & Francis, 2024) Griffith-Linsley, Jackson; Bell, William Robert; Cohen-Gadol, Aaron; Donegan, Diane; Richardson, Angela; Robertson, Michael; Shiue, Kevin; Nevel, Kathryn; Pathology and Laboratory Medicine, School of MedicineAim: Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive primary CNS neoplasm, predominantly observed in children. The use of autologous stem cell transplantation (ASCT) in pediatric ATRT has shown promise; however, its utility in adult ATRT remains unclear. Patients & methods: This study presents the case of an adult patient with ATRT who is in remission after ASCT and reviews the literature on ASCT in adults with ATRT. Four cases of ATRT in adults who underwent ASCT were identified, with pertinent data summarized. Results: All five patients survived longer than the historical average survival rate, four of whom had no clinical or radiographic evidence of disease at the final follow-up. Conclusion: Based on limited data, there may be a role for ASCT in the treatment of adults with ATRT.Item Beta cell function in type 1 diabetes determined from clinical and fasting biochemical variables(Springer Nature, 2019-01) Wentworth, John M.; Bediaga, Naiara G.; Giles, Lynne C.; Ehlers, Mario; Gitelman, Stephen E.; Geyer, Susan; Evans-Molina, Carmella; Harrison, Leonard C.; Medicine, School of MedicineAIMS/HYPOTHESIS: Beta cell function in type 1 diabetes is commonly assessed as the average plasma C-peptide concentration over 2 h following a mixed-meal test (CPAVE). Monitoring of disease progression and response to disease-modifying therapy would benefit from a simpler, more convenient and less costly measure. Therefore, we determined whether CPAVE could be reliably estimated from routine clinical variables. METHODS: Clinical and fasting biochemical data from eight randomised therapy trials involving participants with recently diagnosed type 1 diabetes were used to develop and validate linear models to estimate CPAVE and to test their accuracy in estimating loss of beta cell function and response to immune therapy. RESULTS: A model based on disease duration, BMI, insulin dose, HbA1c, fasting plasma C-peptide and fasting plasma glucose most accurately estimated loss of beta cell function (area under the receiver operating characteristic curve [AUROC] 0.89 [95% CI 0.87, 0.92]) and was superior to the commonly used insulin-dose-adjusted HbA1c (IDAA1c) measure (AUROC 0.72 [95% CI 0.68, 0.76]). Model-estimated CPAVE (CPEST) reliably identified treatment effects in randomised trials. CPEST, compared with CPAVE, required only a modest (up to 17%) increase in sample size for equivalent statistical power. CONCLUSIONS/INTERPRETATION: CPEST, approximated from six variables at a single time point, accurately identifies loss of beta cell function in type 1 diabetes and is comparable to CPAVE for identifying treatment effects. CPEST could serve as a convenient and economical measure of beta cell function in the clinic and as a primary outcome measure in trials of disease-modifying therapy in type 1 diabetes.Item Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review(Cureus, 2021-02-10) Witten, Andrew J.; Mendenhall, Stephen K.; DeWitt, Logan S.; Vortmeyer, Alexander; Cohen-Gadol, Aaron; Neurological Surgery, School of MedicineChoroid plexus tumors (CPTs) are rare intraventricular neoplasms that primarily occur in children and are rare in adults. Of the CPT subtypes, choroid plexus carcinomas (CPC) are highly aggressive and malignant and of World Health Organization (WHO) Grade III. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. In this case report, we present a 33-year-old female with a past medical history notable for schizophrenia and bipolar disease who suffered from left-sided acute vision loss and hearing loss. Magnetic resonance imaging (MRI) demonstrated multiple enhancing masses found in the left cerebellopontine angle (CPA), right internal auditory canal, the atrium of the left ventricle, and the left foramen of Monroe. After surgical decompression of the CPA tumor, the permanent final pathology was consistent with CPC. To our knowledge, this is the first reported case of a primary CPC occurring within the CPA in an adult. The unique presentation and progression of this rare adult-onset CPC provide insight for the diagnosis and treatment of other rare instances of CPTs.