Miopatías RYR1 en la infancia: correlación fenotipo-genotipo e incidencia / RYR1 myopathies in childhood: phenotype-genotype correlation and incidence

Introducción: Las miopatías relacionadas con el receptor de rianodina de tipo 1 (RYR1-RM) constituyen la categoría más frecuente de miopatías congénitas. La introducción de técnicas genéticas ha cambiado el paradigma diagnóstico y sugiere la prioridad de estudios moleculares sobre biopsias. Este estudio busca explorar las características clinicoepidemiológicas de pacientes con variantes del gen RYR1 en un hospital pediátrico de tercer nivel con el objetivo de ampliar la comprensión de la correlación genotipo-fenotipo en las RYR1-RM. Pacientes y métodos: Estudio observacional, descriptivo y transversal, de pacientes menores de 14 años con síntomas miopáticos y variantes potencialmente patógenas del gen RYR1 entre enero de 2013 y diciembre de 2023, considerando variables como sexo, edad, desarrollo motor, variantes genéticas, patrón de herencia y otras manifestaciones. Todas las variables fueron tabuladas frente a la variante genética. Resultados: De los nueve pacientes incluidos, la incidencia estimada fue de aproximadamente 1/10.000 nacidos vivos. La mediana en el momento del diagnóstico fue de 6 años, con una variabilidad fenotípica significativa. Se observaron síntomas comunes, como debilidad y retraso del desarrollo motor. Las variantes genéticas afectaron al gen RYR1 de manera diversa, y hubo cinco variantes previamente no descritas. La biopsia muscular se realizó en cinco pacientes, en dos de ellos de tipo miopatía central core; en uno, multiminicore; en uno, desproporción congénita de fibras; y en otro, de patrón inespecífico. Conclusiones: Las RYR1-MR de nuestra serie ofrecieron variabilidad fenotípica y de afectación, con una incidencia en nuestra área de en torno a 1/10.000 recién nacidos. La mayoría de los casos fueron varones, de variantes missense dominantes. Aportamos cinco variantes genéticas no descritas con anterioridad.(AU)

Introduction: Ryanodine receptor type 1-related myopathies (RYR1-RM) represent the most prevalent category of congenital myopathies. The introduction of genetic techniques has shifted the diagnostic paradigm, suggesting the prioritization of molecular studies over biopsies. This study aims to explore the clinical and epidemiological characteristics of patients with RYR1 gene variants in a tertiary pediatric hospital, intending to enhance the understanding of the genotype-phenotype correlation in RYR1-RM. Patients and methods: An observational, descriptive, and cross-sectional study was conducted on patients under 14 years old with myopathic symptoms and potentially pathogenic RYR1 gene variants from January 2013 to December 2023. Variables such as gender, age, motor development, genetic variants, inheritance pattern, and other manifestations were considered. All variables were tabulated against the genetic variant. Results: Of the nine included patients, the estimated incidence was approximately 1 in 10,000 live births. The median age at diagnosis was six years, with significant phenotypic variability. Common symptoms such as weakness and delayed motor development were observed. Genetic variants affected the RYR1 gene diversely, including five previously undescribed variants. Muscle biopsy was performed in five patients, revealing central core myopathy in two, multiminicore in one, congenital fiber-type disproportion in one, and a nonspecific pattern in another.(AU)

Developmental cervical spinal canal size: population reference range.

Background: The developmental size of the cervical spinal canal varies considerably. Neural compression and injury are more likely with a developmentally small spinal canal. This study was designed to develop a population reference range for developmental cervical spinal canal size for the Hong Kong population. Methods: Prospective study of 522 ambulatory patients (256 males, 266 females, mean age 55±18 years; range, 20-89 years) who underwent computed tomography (CT) neck examinations. Using a manually operated segmentation program, spinal canal, and vertebral body cross-sectional area (CSA), anteroposterior (AP) sagittal diameter, and width were measured at each level from C3-C7. Patient height and weight were measured. Results: Considerable variation in spinal canal size existed with, for example, a 164-168% variation exists for males and females between the largest and smallest spinal canal CSA at C5. All spinal canal measurements increased slightly with height (r=0.25-0.36, P<0.001), while vertebral body AP sagittal diameter increased with age (r=0.48-0.51, P<0.001). All spinal canal measurements were larger (<0.0001) in males. Although spinal canal CSA was larger in males (at C5, males 276.0±41.5 mm2; females 252.6±38.4 mm2), relative to vertebral body CSA, spinal canal CSA was larger in females. Arbitrary population thresholds indicating the smallest 25% spinal canal CSA and AP sagittal diameter as well as other parameters were defined. Conclusions: There is a large variation in developmental cervical spinal canal size within the Hong Kong population. A reference range of developmental spinal canal size was developed which will enable an objective assessment of an individual's cervical spinal canal size relative to the wider population.

Phase transformation behavior and mechanical properties of HyFlex EDM nickel-titanium endodontic rotary instrument: Evaluation at body temperature.

Background/purpose: Temperature-dependent phase compositional changes influence the mechanical properties of heat-treated nickel-titanium (NiTi) rotary instruments. This study evaluated the phase composition, bending properties, and cyclic fatigue resistance of HyFlex EDM NiTi rotary instruments against differently heat-treated and non-heat-treated NiTi instruments at body temperature (BT). Materials and methods: HyFlex EDM OneFile (EDM) instruments, heat-treated HyFlex CM (CM) and Twisted File (TF) instruments, and non-heat-treated K3 instruments (size #25/.08) were subjected to differential scanning calorimetry, and the martensitic, R-phase, and reverse transformation starting and finishing temperatures were determined. A cantilever bending test and a cyclic fatigue test were conducted at BT (37 °C ± 1.0 °C), and the bending load and number of cycles to failure (NCF) were recorded. Statistical analysis was performed using Kruskal-Wallis and Mann-Whitney U tests (α = 0.05). Results: TF and K3 had reverse transformation finishing temperatures lower than BT, while those for EDM and CM were higher than BT. The bending loads at a 0.5 mm deflection were in the order of EDM < TF < CM < K3 (P < 0.05), and those at a 2.0 mm deflection were EDM < CM and TF < K3 (P < 0.05). EDM had the highest NCF among the four instruments (P < 0.05). Conclusion: The EDM instrument had a reverse transformation finishing temperature higher than BT indicating its martensite/R-phase composition at BT. The EDM instrument had superior flexibility and greater resistance to cyclic fatigue than the CM, TF, and K3 instruments at BT.

Effect of canal medicaments Triple Antibiotic Paste, Bio-C Temp, and Nano-Silver Gel activated by visible blue light on canal dentin microhardness and extrusion bond strength of AH plus sealer: A SEM and EDX analysis.

AIM: Assessment of contemporary canal medicaments (Triple antibiotic paste (TAP), Bio-C Temp, and Nano silver gel activated by visible blue light on the dentin microhardness (MH) and push-out bond strength (PBS) of AH plus endodontic sealer METHOD: : Sixty extracted premolars were obtained and decontaminated. Canal cleaning and shaping were performed. The samples were randomly allocated into four groups based on the intracanal medicaments. Group 1= CH paste, Group 2= TAP, Group 3= Bio-C Temp, and Group 4= Nano-silver gel activated by visible blue light. MH assessment was performed using a Vickers Microhardness tester. Forty specimens, ten from each group underwent root canal obturation. PBS and failure mode evaluation were performed. ANOVA and Post Hoc Tukey test were utilized to conduct intra and inter-group comparisons RESULTS: : The maximum outcome of surface hardness was presented by Group-3 (Bio-C Temp®) specimens. However, minimum scores of MH were displayed by Group 1 (CH) treated teeth. The highest outcomes of EBS were exhibited by the cervical third of Group 3 (Bio-C Temp®) samples. The apical section of Group 4 Teeth with Nano Silver gel activated by visible blue light revealed the lowest scores of bond integrity CONCLUSION: : Bio-C Temp and TAP proved to be better intracanal medicament than other tested groups in terms of the push-out bond strength of the sealer. TAP displayed lower microhardness as compared to the Bio-C Temp.

The effect of apical patency on postoperative pain following endodontic therapy: A systematic review and meta-analysis.

This systematic review and meta-analysis aimed to determine whether apical patency increases postoperative pain after endodontic therapy. This study explored the degree and incidence of postoperative pain during root canal therapy, as well as the number of required analgesic doses. We searched PubMed, Scopus, Embase, Web of Science, Cochrane Library, and gray literature from the date of database inception until May 2023. RevMan 5.4 software was used for data analysis. Twelve studies were considered eligible for meta-analysis. The mean pain scores on days 1 (mean difference [MD] = -1.69) and 2 (MD = -0.85) differed significantly between the apical patency and non-patency groups. The odds for pain after 24 h were significantly lower (OR 0.59) in the apical patency group than in the non-patency group. Furthermore, the mean number of required analgesic doses was not significantly different between the two groups. In conclusion, apical patency significantly alleviated postoperative pain (low-quality evidence) and reduced the incidence of pain (moderate evidence). However, high-quality randomized controlled trials are required to validate these findings.

Comparative evaluation of ethanol versus citric acid in removal of oil-based calcium hydroxide from the apical third of root canal system.

Objectives: To evaluate the effectiveness of ethanol compared to citric acid in the removal of oil-based calcium hydroxide from the apical third of the root canal system using passive ultrasonic irrigation. METHODS: The in vitro study was conducted from September to October 2021 at the dental clinics of the Aga Khan University Hospital, Karachi, and comprised single-rooted teeth that were selected from institutional bank of extracted teeth. They were randomly divided into group A having 70% ethanol + passive ultrasonic irrigation, group B 10% citric acid + passive ultrasonic irrigation, group C positive controls and group D negative controls. The specimens were sectioned at 1mm and 3mm from the apex and examined under a dental operating microscope. A single examiner scored the specimens on two different occasions. Data was analysed using SPSS 25. RESULTS: Of the 90 teeth, there were 40(44.4%) in each of the 2 experimental groups and 5(5.5%) in each of the 2 control groups. At 3mm apical sections, ethanol was significantly more effective in the removal of oil-based calcium hydroxide (p=0.01). However, at 1mm from the apex, there was no significant difference between the experimental groups (p=0.064). Intragroup comparison showed that for groups A and B, residual medicament at 1mm sections was significantly greater than at 3mm sections (p<0.001, p=0.003). CONCLUSIONS: Neither irrigant showed complete removal at 1mm and 3mm from the apex. However, at 3mm apical sections, 70% ethanol was significantly more effective compared to 10% citric acid.

Fetal left and right ventricular strain parameters using speckle tracking in congenital heart diseases.

Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark. The study was completed via a single center retrospective analysis on 72 fetal echocardiograms (26 normal, 15 dTGA, and 31 AVC). Tomtec Arena™ echocardiography analysis software was used for analysis. In normal fetuses, mean left ventricular (LV) global longitudinal strain (GLS) was - 22.6% (95% CI -24, -21.1) and mean right ventricular (RV) GLS was - 22.1% (95% CI -23.6, -20.6). In AVC patients LV GLS was-26.6% (95% CI -28,-25.3) and mean RV GLS was - 26.5% (95% CI -27.9,-25.2). In dTGA patients LV GLS was - 22.9% (95% CI of -24.8, -21) and RV GLS was - 21.3% (95% CI was - 23.4, -20.8). There was good intra-rater reliability though poor to fair inter-rater reliability. Notwithstanding its current limitations, strain imaging can provide useful information that can increase confidence of cardiac functional assessment in fetal patients. However, to be reliable across the board, further automation and standardization is required.

The Impact of Frailty on Surgical Outcome of Patients with Lumbar Spinal Canal Stenosis.

Introduction: Frailty is an important factor in surgical outcomes. The current study aimed to evaluate the effect of preoperative frailty on postoperative outcomes in older patients with lumbar spinal canal stenosis (LSCS). Methods: We retrospectively examined 209 patients aged ≥65 years who underwent surgery for LSCS. Health-related quality-of-life (HRQOL) tools, including the Roland-Morris Disability Questionnaire (RDQ), Zurich Claudication Questionnaire (ZCQ), and Japanese Orthopaedic Association Back Pain Evaluation Questionnaire (JOABPEQ), were used in the assessment conducted before surgery and at 6 months and 1 year after surgery. Frailty was categorized based on the 11-item modified frailty index (mFI-11). Patients with mFI-11 of 0, <0.21, and >0.21 were classified under the robust (R), pre-frailty (P), and frailty (F) groups, respectively. Results: According to the mFI-11, 24, 138, and 47 patients were included in the R, P, and F groups, respectively. Regarding preoperative radiographic parameters, there was a remarkable increase in the sagittal vertical axis and a significant decrease in the development of lumbar lordosis with frailty progression. The preoperative scores of RDQ and ZCQ, and lumbar function, walking ability, social life, and psychological disorder domain scores of JOABPEQ differed significantly among these groups. The frequency of revision surgery was not higher in the F group than in the other groups. After adjustment for factors have shown different distributions among the three groups, the frequency of effective surgical cases did not show a clear trend among the three groups in all domains of the JOABPEQ. Conclusions: The preoperative HRQOL scores and the radiographic parameters of patients with LSCS worsened with frailty severity. However, frailty did not affect the rate of revision surgery and surgical efficacy in patients with LSCS. Although this study has limitations, our findings indicated that even LSCS patients with frailty can be considered for surgery if they have an indication for LSCS surgery.

Deep learning-based automatic segmentation of the mandibular canal on panoramic radiographs: A multi-device study.

Purpose: The objective of this study was to propose a deep-learning model for the detection of the mandibular canal on dental panoramic radiographs. Materials and Methods: A total of 2,100 panoramic radiographs (PANs) were collected from 3 different machines: RAYSCAN Alpha (n=700, PAN A), OP-100 (n=700, PAN B), and CS8100 (n=700, PAN C). Initially, an oral and maxillofacial radiologist coarsely annotated the mandibular canals. For deep learning analysis, convolutional neural networks (CNNs) utilizing U-Net architecture were employed for automated canal segmentation. Seven independent networks were trained using training sets representing all possible combinations of the 3 groups. These networks were then assessed using a hold-out test dataset. Results: Among the 7 networks evaluated, the network trained with all 3 available groups achieved an average precision of 90.6%, a recall of 87.4%, and a Dice similarity coefficient (DSC) of 88.9%. The 3 networks trained using each of the 3 possible 2-group combinations also demonstrated reliable performance for mandibular canal segmentation, as follows: 1) PAN A and B exhibited a mean DSC of 87.9%, 2) PAN A and C displayed a mean DSC of 87.8%, and 3) PAN B and C demonstrated a mean DSC of 88.4%. Conclusion: This multi-device study indicated that the examined CNN-based deep learning approach can achieve excellent canal segmentation performance, with a DSC exceeding 88%. Furthermore, the study highlighted the importance of considering the characteristics of panoramic radiographs when developing a robust deep-learning network, rather than depending solely on the size of the dataset.

Comparison of the Antibacterial Effect of AH26, Adseal and Beta RCS Root Canal Sealers against Enterococcus Faecalis, an in Vitro Study.

Objectives: Antibacterial activity against endodontic pathogens is a desirable feature for root canal sealers. The objective of this study was to compare the antibacterial effect of three resin-based endodontic sealers (AH26, Adseal, and Beta RCS) against Enterococcus faecalis in vitro. Materials and Methods: The antibacterial properties of the sealers were assessed against E. faecalis using agar diffusion test (ADT) for fresh state (N=10) and direct contact test (DCT) for freshly-mixed and set states of the materials (N=10). In ADT, the diameter of the zones of inhibition was measured after 24h of contact. In DCT, the colony-forming units of the bacteria were counted after 30 minutes and 180 minutes of exposure. The results were analyzed with two-way ANOVA and independent sample t-test. P<0.05 was considered significant. Results: Regarding DCT results, all test materials indicated an antibacterial effect, both in freshly-mixed and set states. The highest antibacterial effect was related to Adseal, whereas the lowest was observed in Beta RCS. There was a significant difference between all study groups (different sealers, setting states, and contact times; P<0.001), except for freshly-mixed AH26 and Adseal at 180 minutes (P>0.05). According to ADT, AH26 and Adseal represented the widest and the smallest inhibition zones, respectively (P<0.001). Conclusion: Within the limitations of this in vitro study, AH26, Adseal, and Beta RCS showed antibacterial effects against E. faecalis in both freshly-mixed and set states. The antibacterial effect increased over time in all of the studied sealers.

Genomic analysis of Salmonella isolated from canal water in Bangkok, Thailand.

Antimicrobial resistance (AMR) poses an escalating global public health threat. Canals are essential in Thailand, including the capital city, Bangkok, as agricultural and daily water sources. However, the characteristic and antimicrobial-resistance properties of the bacteria in the urban canals have never been elucidated. This study employed whole genome sequencing to characterize 30 genomes of a causal pathogenic bacteria, Salmonella enterica, isolated from Bangkok canal water between 2016 and 2020. The dominant serotype was Salmonella Agona. In total, 35 AMR genes and 30 chromosomal-mediated gene mutations were identified, in which 21 strains carried both acquired genes and mutations associated with fluoroquinolone resistance. Virulence factors associated with invasion, adhesion, and survival during infection were detected in all study strains. 75.9% of the study stains were multidrug-resistant and all the strains harbored the necessary virulence factors associated with salmonellosis. One strain carried 20 resistance genes, including mcr-3.1, mutations in GyrA, ParC, and ParE, and typhoid toxin-associated genes. Fifteen plasmid replicon types were detected, with Col(pHAD28) being the most common type. Comparative analysis of nine S. Agona from Bangkok and 167 from public databases revealed that specific clonal lineages of S. Agona might have been circulating between canal water and food sources in Thailand and globally. These findings provide insight into potential pathogens in the aquatic ecosystem and support the inclusion of environmental samples into comprehensive AMR surveillance initiatives as part of a One Health approach. This approach aids in comprehending the rise and dissemination of AMR and devising sustainable intervention strategies.IMPORTANCEBangkok is the capital city of Thailand and home to a large canal network that serves the city in various ways. The presence of pathogenic and antimicrobial-resistant Salmonella is alarming and poses a significant public health risk. The present study is the first characterization of the genomic of Salmonella strains from Bangkok canal water. Twenty-two of 29 strains (75.9%) were multidrug-resistant Salmonella and all the strains carried essential virulence factors for pathogenesis. Various plasmid types were identified in these strains, potentially facilitating the horizontal transfer of AMR genes. Additional investigations indicated a potential circulation of S. Agona between canal water and food sources in Thailand. The current study underscores the role of environmental water in an urban city as a reservoir of pathogens and these data obtained can serve as a basis for public health risk assessment and help shape intervention strategies to combat AMR challenges in Thailand.

The alveolar canals and foramina in the first edition of Terminologia Oroanatomica: a preview.

Anatomy was initially developed out of necessity to decrease surgery complications. Over time, anatomists and surgeons have sometimes used different terms for the same anatomical structures, thus resulting in numerous discrepancies in terminology between anatomy and surgery. To avoid any confusion or misunderstanding and to better elucidate the oral anatomy terms, the Federative International Programme for Anatomical Terminology (FIPAT) organized a group of specialists on oral anatomy, Terminologia Oroanatomica (ToA) working group, composed of dentists, anatomy researchers, anatomy educators, oral and maxillofacial surgeons, and oral and maxillofacial radiologists. Within the ToA working group, major anatomical structures in the mandible, such as the mandibular canal, were focused and discussed to determine the most appropriate term, i.e., inferior alveolar canal. Although yet to be approved by the International Federation of Associations of Anatomists (IFAA), this article will preview some changes suggested by the ToA.

A Peculiar Case of Isolated Bilateral Tympanic Plate Fractures.

Tympanic plate fractures are uncommon injuries and carry the risk of external auditory canal stenosis. These injuries are often associated with fractures of adjacent bones like the mandible, maxilla, and temporal bone. Isolated bilateral tympanic bone fractures have rarely been reported. The most frequently advocated treatment for these injuries is surgical to prevent canal stenosis in the future. The effectiveness of non-operative management has been seldom reported. In the current case report, we present an uncommon injury with isolated bilateral tympanic plate fractures secondary to trauma to the mandible with no associated mandible or condylar fractures that were treated non-operatively. The functional outcomes were favorable at one year of follow-up.

Pneumorrhachis as a complication of a severe pressure ulcer: An unique case report.

Pneumorrhachis is a medical condition that refers to the presence of air within the spinal canal. Many circumstances, including trauma, infection, or medical procedures, might lead to this syndrome.In some cases, pneumorrhachis may not cause any symptoms and can resolve on its own. However, it can also be associated with more severe underlying conditions, such as spinal fractures, spinal infections, or underlying lung pathologies that lead to air escaping into the spinal canal. In this case we report an incidental finding of pneumorrhachis in a patient who came to our attention for suspected sepsis.

Novel bioactive nanospheres show effective antibacterial effect against multiple endodontic pathogens.

Aim: The current study evaluated the antibacterial activity of a newly developed quaternary ammonium polymethacrylate (QAPM)-containing bioactive glasses (BGs) via a two-step method by our group, namely BGs-HAEMB, and explored its cytotoxicity and biocompatibility. Methods: The antibacterial effects of the BGs-HAEMB against planktonic bacteria, bacterial biofilm formation, and experimental root canal biofilms of persistent pathogens (Enterococcus faecalis, Streptococcus sanguis and Porphyromonas endodontalis) associated with endodontic infection were evaluated in vitro by agar diffusion tests, direct contact tests and live/dead staining. The cytotoxicity and biocompatibility of BGs-HAEMB were evaluated by CCK-8 assays in vitro and a skin implantation model in vivo. Results: Compared to three clinically used endodontic sealers (Endofill, AH Plus, and iRoot SP), BGs-HAEMB exhibited the relatively strongest antibacterial effect against E. faecalis, S. sanguis and P. endodontalis after sitting for 14 and 28 days (P < 0.01). SEM images and CLSM images also showed that for each tested bacteria, BGs-HAEMB killed the most microorganism among all the experimental groups, regardless of treatment for 7 days or 28 days (P < 0.05). Besides, the BGs-HAEMB-treated groups showed a relatively low cytotoxicity (RGRs ranging from 88.6% to 102.9%) after 1, 3, and 7 days of exposure. Meanwhile, after 28 days of implantation, the inflammatory grade in BGs-HAEMB treated group was assessed as Grade I, in which the average numbers of inflammatory cells (6.7 ± 2.1) were less than 25. Conclusions: BGs-HAEMB exerted a long-term and stable antibacterial effect. The remarkable biocompatibility of BGs-HAEMB in vitro and in vivo confirmed its possible clinical application as a potential alternative in the development of the next generation of endodontic sealers.

Improved optic nerve visualization and treatment planning through a dedicated optic nerve MRI protocol.

OBJECTIVE: This study describes an innovative optic nerve MRI protocol for better delineating optic nerve anatomy from neighboring pathology. METHODS: Twenty-two patients undergoing MRI examination of the optic nerve with the dedicated protocol were identified and included for analysis of imaging, surgical strategy, and outcomes. T2-weighted and fat-suppressed T1-weighted gadolinium-enhanced images were acquired perpendicular and parallel to the long axis of the optic nerve to achieve en face and in-line views along the course of the nerve. RESULTS: Dedicated optic nerve MRI sequences provided enhanced visualization of the nerve, CSF within the nerve sheath, and local pathology. Optic nerve sequences leveraged the "CSF ring" within the optic nerve sheath to create contrast between pathology and normal tissue, highlighting areas of compression. Tumor was readily tracked along the longitudinal axis of the nerve by images obtained parallel to the nerve. The findings augmented treatment planning. CONCLUSIONS: The authors present a dedicated optic nerve MRI protocol that is simple to use and affords improved cross-sectional and longitudinal visualization of the nerve, surrounding CSF, and pathology. This improved visualization enhances radiological evaluation and treatment planning for optic nerve lesions.

High Prevalence of Cochlear Nerve Deficiency in Pediatric Patients With Cochlear Aperture Stenosis.

OBJECTIVE: Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS. STUDY DESIGN: Retrospective study. SETTING: Tertiary care center. METHODS: We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI. RESULTS: Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartile range [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears. CONCLUSION: CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis.