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Objectives Describe the histopathology of the temporal bones in MELAS (myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome. The syndrome results from a known point mutation in mitochondrial DNA. this website Methods Histopathology analysis of a pair of temporal bones from the oldest surviving MELAS syndrome temporal bone donor. Histopathologic findings were correlated with known premortem clinical data. Results The inner ears showed severe but incomplete atrophy of the stria vascularis for the length of the cochleae. In contrast, the organ of Corti and inner hair cells appeared intact with some loss of outer hair cells. Other than moderate loss at the basal turn, spiral ganglion cells numbers were normal. The vestibular neuroepithelium was mostly normal with the exception of moderate degeneration of the macula sacculi and partial collapse of the saccular wall on the right. The cerebral cortex had infarct-like lesions with adjacent gliosis. Conclusion This is an analysis of the oldest patient with MELAS syndrome to date, an addition to only two previously published patients. It supports the notion that hearing loss is a result of dysfunction of the stria vascularis and not loss of hair cells or neurons. Patterns of vestibular pathology are in agreement to in-vivo measurements. These findings support auditory rehabilitation with cochlear implants and may be relevant to hearing loss due to other mitochondrial mutations. Level of evidence 4. © 2020 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.Contemporary histopathology of the ear is based on an evolution of equipment and histological techniques over the last 500 years, including the invention of the light microscope and protocols for fixation, embedment, sectioning, and staining of tissue samples, and visual documentation of findings. Several recent techniques which can be utilized in otopathology hold promise for significant improvement in methods and a better understanding of pathologic processes in diseases of the ear. © 2019 The Author. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.Objectives To investigate the landscape of cognitive impairment (CI) screening for adults with age-related hearing loss (ARHL) among otolaryngologists and audiologists. To identify provider factors and patient characteristics that impact rates of CI screening and referral. Methods A 15 question online survey was sent to members of the Georgia Society of Otolaryngology (GSO), Georgia Academy of Audiology (GAA), American Otological Society and American Neurotology Society (AOS/ANS), and posted on the web forum for two hearing disorders special interest groups within the American-Speech-Language-Hearing Association (ASHA). Responses were collected anonymously. Chi-square tests were used to compare responses. Results Of the 66 included respondents, 61% (n = 40) were otolaryngologists and 35% (n = 23) were audiologists. Respondents were significantly more likely to refer patients for CI assessment than to screen (64% vs 21%, respectively, P less then .001). The complaint of a neurological symptom, such as memory loss, would prompt screening or referral for only 27.3% (n = 18) and 51.52% (n = 34) of respondents, respectively. Forty-two percent (n = 28) of respondents suggested CI screening with the MMSE vs 20% (n = 13) with the Montreal Cognitive Assessment. Conclusions Despite recommendations for cognitive assessment in high-risk populations, such as older adults with ARHL, the practice of CI screening and referral is not yet commonplace among otolaryngologists and audiologists. These providers have a unique opportunity to assess adults with ARHL for CI and ensure appropriate referral. Level of Evidence 5. © 2019 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.Background We reported that tinnitus patients showed reduced levels of auditory functional connectivity (FC) in comparison with normal hearing control subjects, and that we succeeded in objective diagnosis of tinnitus with 86% sensitivity and 74% specificity by focusing only on auditory-related FC. However, the age-related change of auditory FC is not clarified. In this study, we examine age-related change of the auditory FC using the database of Human Connectome Project (HCP) and compared with our database of tinnitus patients. Method From the HCP database HCP Lifespan Pilot project, we studied five age groups, 8 to 9 years old, 14 to 15, 25 to 35, 45 to 55, and 65 to 75. We also applied our tinnitus patients' resting-state functional magnetic resonance imaging (fMRI) database, which is divided into three generations; 20 to 40 years old, 40 to 60, and 60 to 80 to compare with the HCP database. The resting state fMRI analyses were performed using the CONN toolbox version 18. As auditory-related regions, Heschl's gyrus, planum temporale, planum polare, operculum, insular cortex, and superior temporal gyrus were set as the regions of interest from our previous reports. Result Auditory FC is strongest among adolescents and reduces with age. But the auditory FC of tinnitus patients were significantly less than those of HCP data in each generation. Conclusion Although auditory FC decreases with age, tinnitus patients have less auditory FC compared with age-matched controls. The age-matched cutoff values are necessary for an objective diagnosis of tinnitus with resting state fMRI. © 2019 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.Objectives The treatment of inner ear disorders remains challenging due to anatomic barriers intrinsic to the bony labyrinth. The purpose of this review is to highlight recent advances and strategies for overcoming these barriers and to discuss promising future avenues for investigation. Data Sources The databases used were PubMed, EMBASE, and Web of Science. Results Although some studies aimed to improve systemic delivery using nanoparticle systems, the majority enhanced local delivery using hydrogels, nanoparticles, and microneedles. Developments in direct intracochlear delivery include intracochlear injection and intracochlear implants. Conclusions In the absence of a systemic drug that targets only the inner ear, the best alternative is local delivery that harnesses a combination of new strategies to overcome anatomic barriers. The combination of microneedle technology with hydrogel and nanoparticle delivery is a promising area for future investigation. Level of Evidence NA. © 2019 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc.
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