Cognitive and language outcomes for pediatric hearing loss with otologic surgery.

Objectives: To test the hypothesis that surgical otologic intervention for any type of pediatric hearing loss decreases the odds for incident adverse cognitive and linguistic developmental outcomes.

Study Design: Retrospective cohort database study.

Methods: Electronic medical record data from the TriNetX Research Network were queried for children with congenital, sensorineural, conductive, and mixed hearing loss (HL) between ages 0 and 5 years.

Cortical Auditory Evoked Potential Testing in Children With Auditory Neuropathy Spectrum Disorder.

Purpose: In the present report, we reviewed the role of cortical auditory evoked potentials (CAEPs) as an objective measure during the evaluation and management process in children with auditory neuropathy spectrum disorder (ANSD).

Method: We reviewed the results of CAEP recordings in 66 patients with ANSD aged between 2 months and 12 years and assessed the relationship between their characteristics (prevalence, morphology, latencies, and amplitudes) and various clinical features, including the mode of medical management.

Results: Overall, the CAEPs were present in 85.

Cumulus expansion is impaired with advanced reproductive age due to loss of matrix integrity and reduced hyaluronan.

Reproductive aging is associated with ovulatory defects. Age-related ovarian fibrosis partially contributes to this phenotype as short-term treatment with anti-fibrotic compounds improves ovulation in reproductively old mice. However, age-dependent changes that are intrinsic to the follicle may also be relevant.

A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics.

Objectives: The aim of the study was to review the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020 and examine whether or not our diagnostic capabilities in an ANSD population have evolved as our institutional experience has grown and knowledge in the field has expanded.

Design: This was a retrospective study reviewing the demographic data, medical history, imaging studies, audiological and speech-language data, type of audiological intervention and mode of communication in 260 pediatric patients diagnosed with ANSD over a 15-year period.

Results: The study revealed that male and female children were equally affected with all levels of hearing detection being represented and that about 40% of affected children were premature and most were admitted to the neonatal intensive care unit.

A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes.

Objectives: Following a review of the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020, the present report highlights the type and timing of intervention and outcomes in the same 260 patients with ANSD.

Design: This was a retrospective study reviewing the demographic data, medical history, imaging studies, audiological and speech language data, type of audiological intervention (hearing aids or cochlear implants), and mode of communication in 260 pediatric patients diagnosed with ANSD over a 15-year period.

Results: A significant decrease over time in the age at hearing aid fitting was observed.

Role of HNF4alpha-cMyc interaction in liver regeneration and recovery after acetaminophen-induced acute liver injury.

Background And Aims: Overdose of acetaminophen (APAP) is the major cause of acute liver failure in the western world. We report a novel signaling interaction between hepatocyte nuclear factor 4 alpha (HNF4α) cMyc and nuclear factor erythroid 2-related factor 2 (Nrf2) during liver injury and regeneration after APAP overdose.

Approach And Results: APAP-induced liver injury and regeneration were studied in male C57BL/6J (WT) mice, hepatocyte-specific HNF4α knockout mice (HNF4α-KO), and HNF4α-cMyc double knockout mice (DKO).

Hearing outcomes in children with Congenital Cytomegalovirus: A multi-center, single-enterprise experience.

Background: Cytomegalovirus (CMV) is the most common cause of non-genetic sensorineural hearing loss (SNHL) in the United States; yet screening for congenital CMV (cCMV) remains controversial. CMV related SNHL can be present at birth, or develop in a delayed manner, and it is a consistent feature in children with either symptomatic or asymptomatic disease. A retrospective chart review was performed to determine the characteristics of patients diagnosed with cCMV and SNHL.

Minimally invasive endoscopic approaches to pediatric skull base pathologies.

Objective: Endoscopic endonasal approaches to pediatric skull base lesions are rare, challenging, and present distinct considerations from adult pathology. In this study, we describe our endoscopic technical approach to various skull base lesions demonstrating the efficacy and nuances of these approaches in pediatric patients.

Methods: Pediatric patients underwent endoscopic approach for skull base lesions from January of 2015 to April of 2021 were included in the study.

Regulation of Liver Regeneration by Hepatocyte O-GlcNAcylation in Mice.

Background & Aims: The liver has a unique capacity to regenerate after injury in a highly orchestrated and regulated manner. Here, we report that O-GlcNAcylation, an intracellular post-translational modification regulated by 2 enzymes, O-GlcNAc transferase (OGT) and O-GlcNAcase (OGA), is a critical termination signal for liver regeneration following partial hepatectomy (PHX).

Methods: We studied liver regeneration after PHX on hepatocyte specific OGT and OGA knockout mice (OGT-KO and OGA-KO), which caused a significant decrease (OGT-KO) and increase (OGA-KO) in hepatic O-GlcNAcylation, respectively.

Hyaluronan and Collagen Are Prominent Extracellular Matrix Components in Bovine and Porcine Ovaries.

The extracellular matrix (ECM) is a major component of the ovarian stroma. Collagen and hyaluronan (HA) are critical ovarian stromal ECM molecules that undergo age-dependent changes in the mouse and human. How these matrix components are regulated and organized in other mammalian species with reproductive characteristics similar to women such as cows and pigs, has not been systematically investigated.

Ovarian stiffness increases with age in the mammalian ovary and depends on collagen and hyaluronan matrices.

Fibrosis is a hallmark of aging tissues which often leads to altered architecture and function. The ovary is the first organ to show overt signs of aging, including increased fibrosis in the ovarian stroma. How this fibrosis affects ovarian biomechanics and the underlying mechanisms are unknown.

Cochlear implantation in a patient with mucopolysaccharidosis IVA.

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation.

Transmastoid access in branchio-oto-renal syndrome: A reappraisal of computed tomography imaging.

Objective: To evaluate for temporal bone abnormalities that might affect transmastoid surgery such as cochlear implantation in cases of branchio-oto-renal syndrome (BOR).

Study Design: Retrospective review.

Methods: Qualitative assessment of temporal bone computed tomography imaging was performed by a neuroradiologist for 30 individuals with BOR (60 ears) and 20 controls with normal hearing (20 ears).

Natural History of Tympanic Membrane Retraction in Children with Cleft Palate.

Objectives: The natural history of tympanic membrane retraction is unpredictable. To obtain prognostic information for guiding surveillance and treatment, a cohort of children with retraction from cleft palate were prospectively followed for over 5 years.

Materials And Methods: This was a prospective observational study at a tertiary academic institution.

Monopoly Airplane Lands in Esophagus Leading to Difficult Extraction: A Case Report on Anesthesia and Surgical Considerations.

A 2-year-old child presented with an airplane game piece from the board game Monopoly lodged in her esophagus. The airplane's wings, engines, and winglets acted like fish hooks that entered the esophageal mucosa easily but were difficult to extract. Chest radiographs were used to estimate the airplane wingspan dimensions, and a Foley catheter was used to dilate the esophagus to allow foreign body extraction via rigid esophagoscopy with optical forceps.

Preliminary experience using a cochlear implant with a novel linear pedestal design.

Objective: To assess the safety and efficiency of cochlear implantation using a novel device with a linear silastic pedestal (2 mm wide, 2 mm deep, 10 mm long) on the flat undersurface.

Methods: Operative times required to drill a linear groove (LG) for the new linear pedestal design were prospectively accrued for 46 implantations in 30 children (median age 3). Intra-operative safety was assessed during each case.

Assessment of Mastoid Function with Magnetic Resonance Imaging after Canal Wall Up Cholesteatoma Surgery.

Objective: To use magnetic resonance imaging (MRI) to assess the extent of mastoid opacification after canal wall up (CWU) cholesteatoma surgery.

Materials And Methods: Thirty-five children in whom post-operative MRI had been obtained after CWU surgery. Cholesteatoma confined to the meso- and/or epi-tympanum was removed using a transcanal approach (n=18).

Stimulation from Cochlear Implant Electrodes Assists with Recovery from Asymmetric Perceptual Tilt: Evidence from the Subjective Visual Vertical Test.

Vestibular end organ impairment is highly prevalent in children who have sensorineural hearing loss (SNHL) rehabilitated with cochlear implants (CIs). As a result, spatial perception is likely to be impacted in this population. Of particular interest is the perception of visual vertical because it reflects a perceptual tilt in the roll axis and is sensitive to an imbalance in otolith function.

Vestibular evoked myogenic potential testing as an objective measure of vestibular stimulation with cochlear implants.

Objectives/hypothesis: To determine if vestibular potentials could be elicited with electrical stimulation from cochlear implants.

Study Design: Prospective cohort study.

Methods: Vestibular responsiveness to electrical stimulation from cochlear implants was assessed via vestibular evoked myogenic potential (VEMP) testing in 53 pediatric and young adult patients.

Advances in the diagnosis, management, and treatment of neonates with laryngeal disorders.

Neonatal airway obstruction due to laryngeal pathology may cause significant morbidity and even mortality. The most frequently occurring etiologies anatomically from superiorly to inferiorly include: laryngomalacia, saccular cyst, vocal fold paralysis, anterior glottic web, laryngeal atresia, laryngeal cleft, subglottic stenosis, and subglottic hemangioma. The pathophysiology, presentation, and treatment options for each of these entities are discussed with a focus on a multidisciplinary, evidence-based approach.

A preliminary report of percutaneous craniofacial osteoplasty in a rat calvarium.

Objectives/hypothesis: To evaluate the potential for injectable, permanent bone augmentation by assessing the biocompatability and bioactivity of subperiosteal hydroxylapatite (Radiesse) deposition in a rat model.

Study Design: Randomized controlled animal model.

Methods: Fourteen adult Sprague Dawley rats were injected in the parietal skull with 0.

Incidence and management of rhinosinusitis after complex orbitofacial reconstruction.

Objectives/hypothesis: To examine the sinus-related sequelae of free flap reconstruction for complex orbitofacial defects.

Study Design: Retrospective chart review.

Methods: Demographic, clinical, and radiographic data on a series of 55 patients who had undergone free tissue transfer for orbitofacial reconstruction was retrospectively reviewed.

Resource analysis of tonsillectomy in children.

Objectives/hypothesis: To analyze variables that affect time and cost parameters of pediatric adenotonsillectomy.

Study Design: Longitudinal 7-month retrospective review of sequential tonsil and adenoid surgery at a university pediatric tertiary care hospital.

Methods: All children aged 2 to 12 years who underwent adenotonsillectomy from May 2008 to October 2008 had charts and billing records analyzed for variations in charges and times of adenotonsillectomy according to patient age, body mass index for age (BMIFA), American Society of Anesthesiologists (ASA) status, surgical indication, technology used, and teaching status of case.

Microvascular free flap reconstruction of orbitocraniofacial defects.

Purpose: To examine the etiology, surgical extent and techniques, complications, and outcomes of microvascular free flaps (MFF) in the reconstruction of orbitocraniofacial defects.

Design: A retrospective, institutional review board approved study was performed of all patients undergoing MFF to repair orbitocraniofacial defects over 51 months.

Participants: Fifty-eight patients undergoing MFF to repair orbitocraniofacial defects were included.