Early-stage use of hearing aids preserves auditory cortical structure in children with sensorineural hearing loss.

Hearing is critical to spoken language, cognitive, and social development. Little is known about how early auditory experiences impact the brain structure of children with bilateral sensorineural hearing loss. This study examined the influence of hearing aid use and residual hearing on the auditory cortex of children with severe to profound congenital sensorineural hearing loss. We evaluated cortical preservation in 103 young pediatric cochlear implant candidates (55 females and 48 males) by comparing their multivoxel pattern similarity of auditory cortical structure with that of 78 age-matched children with typical hearing. The results demonstrated that early-stage hearing aid use preserved the auditory cortex of children with bilateral congenital sensorineural hearing loss. Children with less residual hearing experienced a more pronounced advantage from hearing aid use. However, this beneficial effect gradually diminished after 17 months of hearing aid use. These findings support timely fitting of hearing aids in conjunction with early implantation to take advantage of neural preservation to maximize auditory and spoken language development.

COMPARING THE EFFECTS OF DIPOTASSIUM ETHYLENEDIAMINETETRAACETIC ACID AND LITHIUM HEPARIN ON HEMATOLOGIC VALUES IN BLANDING'S TURTLES (EMYDOIDEA BLANDINGII), PAINTED TURTLES (CHRYSEMYS PICTA), AND COMMON SNAPPING TURTLES (CHELYDRA SERPENTINA).

Hematology is a routine component of clinical management in veterinary patients. Anticoagulant choice can profoundly influence morphologic assessment of erythrocytes, leukocytes, thrombocytes, and their subsequent quantification. Previous chelonian studies suggest that lithium heparin (LH) is a superior anticoagulant due to hemolysis resulting from dipotassium ethylenediaminetetraacetic acid (dEDTA) in some species. The aim of this study was to compare the effects of dEDTA and LH on hematologic values in Blanding's turtles (Emydoidea blandingii, n = 35), painted turtles (Chrysemys picta, n = 34), and common snapping turtles (Chelydra serpentina, n = 36). We collected samples from free-ranging turtles and immediately divided whole blood into LH and dEDTA tubes. Packed cell volume, total solids, erythrocyte sedimentation rate, white blood cell counts, and differential leukocyte counts were determined. Hemolysis was observed macro- and microscopically in dEDTA samples from painted turtles and common snapping turtles. Packed cell volume and heterophil:lymphocyte was lower and erythrocyte sedimentation rate was higher in LH samples from painted turtles (p, 0.05). In snapping turtles, the PCV, number of monocytes, and number of eosinophils was lower in LH samples (p, 0.05). In Blanding's turtles, the number of eosinophils and basophils was higher in LH samples, while heterophil counts were lower (p, 0.05). Anticoagulant choice created constant and proportional bias for multiple analytes in a species-dependent fashion. LH is the recommended anticoagulant for hematology in painted turtles and common snapping turtles. Either LH or dEDTA may be used in Blanding's turtles, though anticoagulant-specific reference intervals may be necessary.

Revolutionizing pediatric neuroimaging: the era of CT, MRI, and beyond.

PURPOSE: To review the evolution of cross-sectional imaging in pediatric neuroradiology from early developments to current advancements and future directions. METHODS: Information was obtained through a PubMed literature search as well as referenced online resources and personal experience from radiologists currently practicing pediatric neuroimaging and those who experienced the era of nascent cross-sectional imaging. RESULTS: The advent of computed tomography (CT) and magnetic resonance imaging (MRI) in the 1970s and 1980s brought about a revolutionary shift in the field of medical imaging, neurosurgical and neurological diagnosis. These cross-sectional imaging techniques ushered in a new era by enabling the visualization of soft tissue structures within the brain and spine. Advancements in these imaging modalities have continued at a remarkable pace, now providing not only high high-resolution and 3-dimensional anatomical imaging, but also functional assessment. With each stride forward, CT and MRI have provided clinicians with invaluable insights, improving the accuracy and precision of diagnoses, facilitating the identification of optimal surgical targets, and guiding the selection of appropriate treatment strategies. CONCLUSION: This article traces the origins and early developments of CT and MRI, chronicling their journey from pioneering technologies to their current indispensable status in clinical applications and exciting possibilities that lie ahead in the realm of medical imaging and neurologic diagnosis.

Feasibility of rapid spine magnetic resonance evaluation for spinal cord syrinx in the pediatric population.

PURPOSE: This study investigates the feasibility of ultrafast fluid sensitive techniques for evaluation of pediatric spinal cord syrinx. Rapid imaging could obviate the need for sedation, which is often required for children undergoing lengthier standard spine imaging. METHODS: Children undergoing standard spine imaging for Chiari malformation, suspected Chiari malformation, or syrinx were included. Patients who provided informed consent were imaged with rapid acquisition sagittal and axial T2 HASTE spine sequences in addition to standard spine imaging. Standard and rapid spine imaging were then reviewed separately by a pediatric neuroradiologist. The presence or absence of syrinx, syrinx diameter, and length were assessed. The degree of cerebellar tonsillar ectopia, conus position, and evaluation of the filum were also recorded. RESULTS: Seventy-six patients aged 1 month to 18 years (mean 7 years) met the inclusion criteria. The sensitivity and specificity of rapid spine imaging for syrinx was 87.8% and 94.7% respectively. All syrinxes > 2.3 mm in diameter were identified with the rapid spine sequences. There was no statistically significant difference between rapid and standard spine imaging in assessment of syrinx diameter or length. Compared with standard spine imaging, rapid spine sequences demonstrated a 100% sensitivity for low-lying conus and a 98.2% sensitivity for cerebellar tonsillar ectopia. The filum was identified on only 31.6% of the rapid spine studies. CONCLUSION: Rapid T2 imaging demonstrated a high sensitivity for the presence and extent of spinal cord syrinx and may provide an alternative to traditional, lengthier standard spine imaging in selected patients.

Gorham Stout disease of the temporal bone with cerebrospinal fluid leak.

Gorham Stout disease (GSD) is a rare disease characterized by the proliferation of endothelial lined vessels and replacement of bone by fibrous tissue. The main imaging features are progressive osteolysis and cortical resorption. Temporal bone involvement is rare but presents as a destructive bone lesion that may be misinterpreted as more common lytic processes in the pediatric population, such as infection or Langerhans cell histiocytosis. GSD of the temporal bone is associated with cerebrospinal fluid (CSF) leaks, may present with otorrhea, and can mimic other causes of ear drainage. Here, we report the clinical course, imaging features, and outcomes of a 3-year-old girl with GSD of the temporal bone presenting with CSF leak initially attributed to infection.

Tragic Choices, Revisited: COVID-19 and the Hidden Ethics of Rationing.

Early in the COVID-19 pandemic in the United States, concern that there could be a shortage of ventilators raised the possibility of rationing care. Denying patients life-saving care captures our moral imagination, prompting the demand for a defensible framework of ethical principles for determining who will live and who will die. Behind the moral dilemma posed by the shortage of a particular medical good lies a broad moral geography encompassing important and often unarticulated societal values, as well as assumptions about the nature and purpose of health care and the consequences of long-standing choices about health care as a social good. This article explores what COVID-19 has exposed concerning values and choices around health care in the United States. Employing the lens of Catholic Social Thought, it argues for an approach to rationing that is grounded in respect for human dignity, committed to distributing social goods in light of the common good, and self-conscious about the construction of vulnerability to illness and death.

Neural preservation underlies speech improvement from auditory deprivation in young cochlear implant recipients.

Although cochlear implantation enables some children to attain age-appropriate speech and language development, communicative delays persist in others, and outcomes are quite variable and difficult to predict, even for children implanted early in life. To understand the neurobiological basis of this variability, we used presurgical neural morphological data obtained from MRI of individual pediatric cochlear implant (CI) candidates implanted younger than 3.5 years to predict variability of their speech-perception improvement after surgery. We first compared neuroanatomical density and spatial pattern similarity of CI candidates to that of age-matched children with normal hearing, which allowed us to detail neuroanatomical networks that were either affected or unaffected by auditory deprivation. This information enables us to build machine-learning models to predict the individual children's speech development following CI. We found that regions of the brain that were unaffected by auditory deprivation, in particular the auditory association and cognitive brain regions, produced the highest accuracy, specificity, and sensitivity in patient classification and the most precise prediction results. These findings suggest that brain areas unaffected by auditory deprivation are critical to developing closer to typical speech outcomes. Moreover, the findings suggest that determination of the type of neural reorganization caused by auditory deprivation before implantation is valuable for predicting post-CI language outcomes for young children.

Aplasia of the Optic Nerve: A Report of Seven Cases.

Optic nerve aplasia (ONA) is a rare congenital anomaly with a limited number of published reports. A retrospective review was performed on seven patients with ONA seen during 2004-2017. Patient's ocular and extraocular manifestations, imaging findings, and clinical course were described. Magnetic resonance imaging (MRI) showed anomalies of the optic chiasm and tracts and other central nervous system involvement. In conclusion, in addition to thorough ophthalmic examinations, MRI is important in evaluating and diagnosing ONA. The patients need to be monitored for both ocular and extraocular concerns.

Rapid MRI evaluation of acute intracranial hemorrhage in pediatric head trauma.

INTRODUCTION: Rapid MRI with ultrafast T2 sequences can be performed without sedation and is often used in place of computed tomography (CT) to evaluate pediatric patients for indications such as hydrocephalus. This study investigated the sensitivity of rapid magnetic resonance imaging (MRI) for detection and follow-up of acute intracranial hemorrhage in comparison to CT, which is commonly the first-line imaging. METHODS: Patients presenting to a pediatric hospital with acute intracranial hemorrhage on CT and follow-up rapid MRI within 48 h were included. Rapid MRI studies consisted of three plane ultrafast T2 sequences either with or without axial gradient echo (GRE) sequences. Identification of hemorrhage on rapid MRI was assessed by readers both blinded and unblinded to prior CT results. RESULTS: One hundred two acute hemorrhages in 61 patients were identified by CT. Rapid MRI detection of subdural and epidural hemorrhages was modest in the absence of prior CT for comparison (sensitivity 61-74 %), but increased with review of the prior CT (sensitivity 80-86 %). Hemorrhage size was a significant predictor of detection (p < 0.0001). Three plane fast T2 images alone without GRE sequences were poor at detecting subarachnoid hemorrhage (sensitivity 10-25 %); rapid MRI with GRE sequences identified the majority of subarachnoid hemorrhage (sensitivity 71-93 %). GRE modestly increased detection of other extra-axial hemorrhages. CONCLUSIONS: Rapid MRI with GRE sequences is sensitive for most acute intracranial hemorrhages only when a prior CT is available for review. Rapid MRI is not adequate to replace CT in initial evaluation of intracranial hemorrhages but may be helpful in follow-up of known hemorrhages.

Good Deaths, "Stupid Deaths": Humane Medicine and the Call of Invisible Bodies.

Jeffrey Bishop's The Anticipatory Corpse exposes a functional metaphysics at the root of contemporary medical practice that gives rise to inhumane medicine, especially at the end of life. His critique of medicine argues for alternative spaces and practices in which the communal significance of the body, its telos, can be restored and the meaning of a "good death" enriched. This essay develops an alternative epistemology of the body, drawing from Christian theological accounts of the communal or Eucharistic body and linking liturgical practices to the cultivation of solidarity. It argues for an epistemology of the body that makes visible the invisible bodies at medicine's margins and illumines the disparate worlds of health care globally.

Imaging findings of anaplastic astrocytoma in a child with maple syrup urine disease: a case report.

Maple syrup urine disease (MSUD) is an inborn error of branched-chain amino acid metabolism, which usually presents in childhood with encephalopathy due to cerebral edema and dysmyelination. Even with treatment, metabolic stressors may precipitate later episodes of acute decompensation. Changes related to cerebral and white matter edema have been described by magnetic resonance imaging (MRI), and imaging can aid in both initial diagnosis and evaluation of decompensation. To date, there are no published known reports of cancer in patients with MSUD. Here, we present the first case report of an anaplastic astrocytoma in a teenager with MSUD, with a discussion of imaging findings and the use of magnetic resonance spectroscopy (MRS) to help distinguish between tumor and metabolic changes.

Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation.

Cochlear implantation is the only U.S. Food and Drug Administration-approved treatment for children with marked bilateral sensorineural hearing loss. It provides auditory benefits that range from simple sound detection to substantial word understanding. Improved hearing through cochlear implantation has been demonstrated to enhance the rate of language acquisition, enable development of spoken language, and advance literacy in deaf children. Magnetic resonance imaging and computed tomography both have roles in the preoperative assessment of inner-ear abnormalities, cochlear nerve deficiency, and variant anatomy that may affect the decision to implant and the prognosis for auditory improvement and increase the risk for complications. Most cochlear abnormalities may be successfully treated with cochlear implantation, but the presence of a cochlear malformation may increase the risk for intraoperative cerebrospinal fluid leakage and postoperative bacterial meningitis. Eighth-nerve deficiency correlates with poor auditory outcomes and may affect eligibility for cochlear implantation. Another important consideration for implantation is the presence of labyrinthitis ossificans in some children with deafness resulting from bacterial meningitis, which may cause obstruction that limits electrode insertion. Anatomic variations of the facial nerve or middle-ear cavity, which are more common in syndromic patients, may also affect the surgical approach and make implantation difficult.

Maternal to fetal transmission of cervical carcinoma.

Reported cases of metastases to the placenta are uncommon, and maternal transmission of tumor to the fetus is even more infrequent. However, vertical transmission of tumor can occur and should be considered as a potential etiology of malignancy in newborns and infants born to mothers with a history of cancer during gestation. Here, we present the imaging findings and clinical course of an unusual case of maternal cervical neuroendocrine carcinoma presenting as bilateral petrous temporal bone lesions in an infant.

Emydomyces testavorans Surveillance in Multiple Free-Ranging Terrestrial and Aquatic Chelonian Species in Illinois, USA.

The order Onygenales contains multiple fungal pathogens that affect free-ranging and zoo-housed reptilian species. Emydomyces testavorans, an onygenalean fungus associated with skin and shell disease, has been sporadically detected in aquatic chelonians. Because of the recent discovery of this organism, little is known about its prevalence in free-ranging chelonians. The objective of this study was to perform surveillance for E. testavorans in six free-ranging aquatic and terrestrial chelonian species in Illinois, USA: Blanding's turtles (n=437; Emydoidea blandingii), painted turtles (n=199; Chrysemys picta), common snapping turtles (n=35; Chelydra serpentina), red-eared sliders (n=62; RES; Trachemys scripta elegans), eastern box turtles (n=73; Terrapene carolina carolina) and ornate box turtles (n=29; Terrapene carolina ornata). Combined cloacal-oral swabs (COSs) or shell (carapace and plastron surfaces) swabs were collected from 2019 to 2021 and tested for E. testavorans using quantitative PCR. The PCR detected E. testavorans in COSs of an adult male, subadult female, and juvenile male Blanding's turtle (0.6%; 95% confidence interval [CI], 0.2-1.9%) and a shell swab from an adult female RES (1.6%; 95% CI, 0-8.7%). Shell lesions consistent with E. testavorans infection were present in two of the positive Blanding's turtles. These results document the rarity of this pathogen on the landscape in Illinois. Additional studies should determine this pathogen's impact on individuals and clarify its significance for conservation efforts of Blanding's turtle, in which E. testavorans has not been reported previously.

The gender of pregnancy: masculine lesbians talk about reproduction.

Heterosexism and patriarchy collude to create an expectation of pregnancy for all women. In addition, the bodily production of pregnancy has been socially gendered as feminine because of its association with female-bodied people. These two ideological codes--that all women should become mothers through pregnancy and that pregnancy is a femininely gendered endeavor--suggest conundrums for masculine lesbians. This study relies on interview data with 14 childfree masculine-identified lesbians about the ways in which they are able (or unable) to imagine themselves as pregnant people in their future lives. Participants' navigation of the concept of pregnancy reveal the complexity of gendered bodies and gender practice.

A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI.

Cervical cord compression is a sequela of mucopolysaccharidosis VI, a rare lysosomal storage disorder, and has devastating consequences. An international panel of orthopedic surgeons, neurosurgeons, anesthesiologists, neuroradiologists, metabolic pediatricians, and geneticists pooled their clinical expertise to codify recommendations for diagnosing, monitoring, and managing cervical cord compression; for surgical intervention criteria; and for best airway management practices during imaging or anesthesia. The recommendations offer ideal best practices but also attempt to recognize the worldwide spectrum of resource availability. Functional assessments and clinical neurological examinations remain the cornerstone for identification of early signs of myelopathy, but magnetic resonance imaging is the gold standard for identification of cervical cord compression. Difficult airways of MPS VI patients complicate the anesthetic and, thus, the surgical management of cervical cord compression. All patients with MPS VI require expert airway management during any surgical procedure. Neurophysiological monitoring of the MPS VI patient during complex spine or head and neck surgery is considered standard practice but should also be considered for other procedures performed with the patient under general anesthesia, depending on the length and type of the procedure. Surgical interventions may include cervical decompression, stabilization, or both. Specific techniques vary widely among surgeons. The onset, presentation, and rate of progression of cervical cord compression vary among patients with MPS VI. The availability of medical resources, the expertise and experience of members of the treatment team, and the standard treatment practices vary among centers of expertise. Referral to specialized, experienced MPS treatment centers should be considered for high-risk patients and those requiring complex procedures. Therefore, the key to optimal patient care is to implement best practices through meaningful communication among treatment team members at each center and among MPS VI specialists worldwide.