Temporal Bone Computed Tomography Research Articles

Structure-constrained deep feature fusion for chronic otitis media and cholesteatoma identification.

Chronic suppurative otitis media (CSOM) and middle ear cholesteatoma (MEC) were two most common chronic middle ear disease(MED) clinically. Accurate differential diagnosis between these two diseases is of high clinical importance given the difference in etiologies, lesion manifestations and treatments. The high-resolution computed tomography (CT) scanning of the temporal bone presents a better view of auditory structures, which is currently regarded as the first-line diagnostic imaging modality in the case of MED. In this paper, we first used a region-of-interest (ROI) network to find the area of the middle ear in the entire temporal bone CT image and segment it to a size of 100*100 pixels. Then, we used a structure-constrained deep feature fusion algorithm to convert different characteristic features of the middle ear in three groups as suppurative otitis media (CSOM), middle ear cholesteatoma (MEC) and normal patches. To fuse structure information, we introduced a graph isomorphism network that implements a feature vector from neighbourhoods and the coordinate distance between vertices. Finally, we construct a classifier named the "otitis media, cholesteatoma and normal identification classifier" (OMCNIC). The experimental results achieved by the graph isomorphism network revealed a 96.36% accuracy in all CSOM and MEC classifications. The experimental results indicate that our structure-constrained deep feature fusion algorithm can quickly and effectively classify CSOM and MEC. It will help otologist in the selection of the most appropriate treatment, and the complications can also be reduced.

Feasibility of size-specific organ-dose estimates based on water equivalent diameter for common head CT examinations: a Monte Carlo study

Computed tomography dose index (CTDI) is an unreliable dose estimate outside of the standard CTDI phantom diameters (16 and 32 cm). Size-specific dose estimate (SSDE) for head computed tomography (CT) examination was studied in the American Association of Physicists in Medicine Report 293 to provide SSDE coefficient factors based on water equivalent diameter as size metrics. However, it is limited to one protocol and for a fully irradiated organ. This study aimed to evaluate the dependency of normalized organ dose (ND) on water equivalent diameter as a size metric in three common protocols: routine head, paranasal sinus, and temporal bone. CTDIw measurements were performed for outlined protocols in the Siemens Emotion 16-slice-configuration scanner. Geant4 Application for Tomographic Emission Monte Carlo simulation platform, coupled with ten GSF patient models, was used to estimate organ doses. CT scanner system was modeled. Helical CT scans were simulated using constructor scan parameters and calculated scan lengths of each patient model. Organ doses provided by simulations were normalized to CTDIvol. The water equivalent diameters (D w) of patient models were obtained via relationships between D w and both effective diameter for a sample of patients’ data.NDs received by fully, partially, and non-directly irradiated organs were then reported as a function of D w. For fully irradiated organs, brain (R 2 > 0.92), eyes (R 2 > 0.88), and eye lens (R 2 > 0.89) correlate well with D w. For the rest of the results, a poor correlation was observed. For partially irradiated organs, the exception was scalp (R 2 = 0.93) in temporal bone CT. For non-directly irradiated organs, the exception was thyroid (R 2 > 0.90) and lungs (R 2 > 0.91) in routine head CT. ND correlates well in routine head CT than other protocols. For the most part, no relationship seems to exist between R 2 and scan percentage coverage. The results have revealed additional factors that may influence the ND and D w relationship, which explains the need for more studies in the future to investigate the effect of scan conditions and organ anatomy variation.

Deep learning-based approach for the automatic segmentation of adult and pediatric temporal bone computed tomography images.

Automatic segmentation of temporal bone computed tomography (CT) images is fundamental to image-guided otologic surgery and the intelligent analysis of CT images in the field of otology. This study was conducted to test a convolutional neural network (CNN) model that can automatically segment almost all temporal bone anatomy structures in adult and pediatric CT images. A dataset comprising 80 annotated CT volumes was collected, of which 40 samples were obtained from adults and 40 from children. A further 60 annotated CT volumes (30 from adults and 30 from children) were used to train the model. The remaining 20 annotated CT volumes were employed to determine the model's generalizability for automatic segmentation. Finally, the Dice coefficient (DC) and average symmetric surface distance (ASSD) were utilized as metrics to evaluate the performance of the CNN model. Two independent-sample t-tests were used to compare the test set results of adults and children. In the adult test set, the mean DC values of all the structures ranged from 0.714 to 0.912, and the ASSD values were less than 0.24 mm for 11 structures. In the pediatric test set, the mean DC values of all the structures ranged from 0.658 to 0.915, and the ASSD values were less than 0.18 mm for 11 structures. There was no statistically significant difference between the adult and child test sets in most temporal bone structures. Our CNN model shows excellent automatic segmentation performance and good generalizability for both adult and pediatric temporal bone CT images, which can help to advance otologist education, intelligent imaging diagnosis, surgery simulation, application of augmented reality, and preoperative planning for image-guided otology surgery.

Uneventful Cochlear Implantation through Round Window Approach in a Child with Persistent Stapedial Artery: A Case Report

Persistent stapedial artery (PSA) is a rare congenital vascular anomaly, with both clinical and surgical implications. When encountered during middle ear surgery, it represents a challenge to the otologic surgeon, since damage to this artery could lead to massive intraoperative bleeding and poor surgical exposure. Here we report the case of a 3-year-old boy with prelingual and profound bilateral sensorineural hearing loss who was referred to our Otolaryngology-Head and Neck Surgery Department for cochlear implant evaluation. Pre-operative computed tomography (CT) examination of the temporal bone confirmed the diagnosis of PSA. Further evaluation showed no contraindication for surgery and the patient underwent cochlear implantation using the classic transmastoid, facial recess approach. During surgery the vessel was coursing far enough from the round window niche, making it possible to achieve safe and complete electrode insertion into the scala tympani of the cochlear basal turn. This case demonstrates that cochlear implantation is safe and feasible in patients with PSA. Thorough analysis of the preoperative temporal bone CT scan might help with surgical planning by identifying this anomaly prior to surgery.

Absent Stapedial Tendon: Imaging Features of an Underrecognized Entity : Clinical Neuroradiology.

Congenital absence of the stapedial tendon is arare entity with characteristic imaging findings, which can go unrecognized due the scarcity of the diagnosis and limited previous description in the imaging literature. We aim to characterize the imaging features of this entity. Aseries of 9cases with surgical confirmation of stapedial tendon absence were retrospectively reviewed and the most common abnormalities on high resolution computed tomography (CT) of the temporal bone described. Congenital fixation of the stapes footplate was present in nearly all cases of stapedial tendon absence (n = 8, 89%), aclinically important association because the stapes footplate abnormality was not detectable on preoperative CT. Absence or hypoplasia of the pyramidal eminence and aperture was identified in almost all cases (n = 8, 89%), which may be the sole imaging finding to suggest stapedial tendon absence and associated stapes footplate fixation prior to surgery. The most reliable indicator of stapedial muscle absence on temporal bone CT is the absence or hypoplasia of the pyramidal eminence and aperture. Importantly, most patients had congenital stapes footplate fixation confirmed intraoperatively with anormal stapes footplate on CT, meaning the pyramidal eminence/aperture abnormality was the only preoperative imaging finding that could have suggested the footplate fixation.

Association of Mastoid Pneumatization with Deviated Nasal Septum: A Clinicoradiological Study

Background: Deviated nasal septum (DNS) has been reported to jeopardizing the nasal aerodynamics. This in turn may affect the pressure dynamics in the nasopharynx and eventually affect the development and pneumatization of mastoid air cells. Few studies done previously have shown a positive correlation between nasal pathologies and mastoid pneumatization. Objectives: This study was undertaken to see for the possible association of mastoid cell pneumatization, with DNS. Materials and Methods: Limited high-resolution computed tomography temporal bone and computed tomography nose and paranasal sinuses were used to calculate the mastoid air cell volume and angle of septal deviation. The degree of septal deviation was graded into mild, moderate, and severe. The mastoid air cell system volume was calculated on the side of the convexity of the septal deviation and the other side. Results: It was found that there is a statistically significant difference in the mastoid volume on the affected side of the DNS vis a vis the unaffected side. Further, the degree of reduction of the volume was not a linear function of the degree of septal deviation. Conclusions: Septal deviation significantly reduces the mastoid air cell volume on the affected side Thus, early correction of pathological DNS in adults, irrespective of the etiology, is important, so as to avoid the chances of developing chronic ear pathologies in later life.

Comparative analysis of preoperative high-resolution computed tomography temporal bone and intraoperative surgical findings in cases of cholesteatoma

Comparative analysis of preoperative high-resolution computed tomography temporal bone and intraoperative surgical findings in cases of cholesteatoma

Symptoms: Purple Ear Drum and Hearing Loss

Symptoms: Purple Ear Drum and Hearing Loss

Development of the sphenoid sinus from newborn to age 18: A computed tomography imaging analysis

The sphenoid sinus (SS) is an important anatomical formation in terms of its location and relationship with important neurovascular structures. It is essential to evaluate the pneumatization and dimensions of the SS with Computed Tomography (CT) in the preoperative period of transsphenoidal interventions to be performed on tumors located in this region. The aim of this study was to evaluate the correlation of the volume and dimensions of the SS measured with CT with age in the period from birth to 18 years of age. In this retrospective study, a total of 360 brain, paranasal sinus, temporal bone and maxillofacial CT scans of individuals from birth to 18 years of age were evaluated. The cohort was divided into 18 groups with 20 (10 female, 10 male) patients in each age group. The height, width, length and volume values of the SS were measured on CT and their correlations with age and the differences between genders were evaluated statistically. It has been determined that the increases in the size and volume of the SS accelerate especially between 1 and 2 years of age and begins to reach adult dimensions after 12 years of age. Height and length of the sphenoid sinus showed a high correlation coefficient (r=0.717 and r=0.731 respectively) with age, while its width and volume showed a moderate correlation coefficient (r=0.662 and 0.543 respectively). There was no statistically significant difference between the genders in terms of SS height, width, length and volume. SS dimensions and volume showed a logaritmic growth pattern from birth to 18 years. The results of this study may help to better understand the normal development of SS in children and may serve as a reference for more comprehensive CT studies. In addition, it can help surgeons to evaluate the developmental characteristics of SS in surgical interventions for SS in children.

Study of correlation between preoperative high resolution computed tomography temporal bone and intraoperative surgical findings in 60 cases of unsafe chronic otitis media

<p><strong>Background:</strong> Cholesteatoma is a serious health hazard which can give rise to alarming intracranial and extracranial complications as it can progressively enlarge and invade into neighbouring structures. Thus, early diagnosis and prompt management of a suspected cholesteatoma is necessary. High resolution computed tomography (HRCT) temporal bone scan clearly defines the extent and location of soft tissue density in middle ear with adjoining bony erosions. The aim of the study was to determine the role of HRCT temporal bone in early detection of cholesteatoma and finding the correlation between preoperative HRCT temporal bone and intraoperative surgical findings.</p><p><strong>Methods: </strong>Total 60 cases with unsafe chronic otitis media (COM) attending ENT OPD of Rajindra hospital, Patiala were selected for this prospective study. All patients were scanned for HRCT temporal bone and preoperative radiological data was correlated with intraoperative surgical findings. The results were calculated and analysed statistically.</p><p><strong>Results:</strong> This study demonstrated good correlation between preoperative temporal bone HRCT scans with intraoperative surgical findings, particularly in ossicular erosion (except stapes), LSC erosion, facial canal erosion, lateral cortical wall erosion and dural and sinus plate erosion.</p><p><strong>Conclusions: </strong>HRCT temporal bone scan evidently demarcates the soft tissue density in middle ear and mastoid with adjacent bone erosions and it also provide details of anatomical variations. Hence HRCT temporal bone is valuable in diagnosing and guiding surgical management of unsafe COM making it prerequisite before any surgical manipulation.</p>

Diameter of the Cochlear Nerve Canal predicts Cochlear Nerve Deficiency in Children with Sensorineural Hearing Loss.

Detection of cochlear nerve deficiency (CND) is usually straightforward using magnetic resonance imaging (MRI). In patients in whom MRI cannot be performed or imaging provides equivocal findings, computed tomography (CT) of the temporal bone might offer indirect evidence of CND. Our study aimed to derive a cut-off value for the diameter of the cochlear nerve canal (CNC) and internal auditory canal (IAC) in temporal bone CT to predict CND. This retrospective study included 70children with sensorineural hearing loss (32 with CND and 38 control patients). The height, width, and cross-sectional area of the IAC and diameter of the CNCs were determined using temporal bone CT. Receiver operating characteristic (ROC) and Student's t-tests were performed for each parameter. The mean diameter of the CNCs was significantly smaller in children with CND than in the control group (1.2 mm versus 2.4 mm, p < .001). The optimal threshold for CNC for separation of the two groups was 1.9 mm, resulting in a sensitivity of 98.7 % and specificity of 89.2 %. The IAC dimensions could not distinguish between children with CND and controls. A CNC diameter of less than 1.9 mm is a reliable predictor of CND in children with sensorineural hearing loss. · A small cochlear nerve canal predicts cochlear nerve deficiency (CND). · The size of the internal auditory canal cannot predict CND. · Whenever MRI is impossible or ambigous, CT can rule out CND. · Sorge M, Sorge I, Pirlich M et al. Diameter of the Cochlear Nerve Canal predicts Cochlear Nerve Deficiency in Children with Sensorineural Hearing Loss. Fortschr Röntgenstr 2022; 194: 1132 - 1139.

A novel mutation of X-linked recessive deafness gene POU3F4 in a boy with congenital deafness.

PurposeTo report an interstitial deletion of Xq21.1 in chromosome X in a boy with congenital deafness.MethodsThe proband underwent a thorough physical examination and a detailed audiological and temporal bone computed tomography (CT) scan. Cochlear implantation was performed on the proband, and follow‐up was conducted. High throughput sequencing and copy number analysis was made of peripheral blood samples from the proband, family members, and control subjects.ResultsSensorineural hearing loss was present in the boy and temporal bone CT scan showed a bilateral incomplete partition type III anomaly (IP‐III). Q21.1 (79.40–83.32 Mb) of chromosome X in the proband had a copy number deletion with a fragment size of about 3.92 Mb. Categories of auditory performance scores and SIR scores of the cochlea in this child improved after surgery.ConclusionThrough the analysis of POU3F4, a novel mutation site with potentially pathogenic significance was found.Level of Evidence: 5.

The New Staging System for Computed Tomography Evaluation of Chronic Otitis Media And Mastoiditis

Objective: To predict risks for complications and determine the type of operation, several studies developed the grading system based on clinical assessment. However, there is no evidence regarding the score for tomographic opacification and bone erosion of temporal bone on computed tomography (CT) scan. Therefore, the aim of this study was primarily to design a new computerized tomography staging system for the evaluation of chronic otitis media (COM) and mastoiditis. Materials and Methods: Between August 1, 2020, and December 30, 2021, 40 patients with the clinical diagnosis of COM (males and females) have received temporal bone CT scans at medical institutions. The patients were retrospectively evaluated and verified the correlation of the score of CT imaging in preoperative evaluation with the clinical picture and surgical planning. Descriptive statistics, one-way ANOVA analysis, and Spearman's correlation test were employed to evaluate the accumulated data. The P values were calculated for all tests and its outcome &lt;0.05 was considered statistically significant. Results: There are significant correlations between the total score and the size of perforation, the type of surgical intervention, and low-frequency air-bone gap (ABG) (P &lt; 0.05) while no correlation between the total score and high-frequency ABG was revealed. Conclusion: The abnormal landmarks of the temporal bone are presented, and scored systematically and there is an excellent way to analyze and make a decision not only in diagnosis but also in the choice of surgical intervention.

Surgical importance of the tympanic bone: multidetector computed tomography findings.

ObjectivesTo measure tympanic bone thickness (anterior-superior, anterior-inferior, and inferior wall), external ear canal length, and tympanomandibular distance that can be useful in cases that undergo tympanic bone resection.Materials and MethodsThe temporal computed tomography (CT) images of 349 patients were retrospectively evaluated. The anterior-inferior, anterior-superior, and inferior wall thicknesses; tympanomandibular distance; and external auditory canal (EAC) bone canal length were measured from the narrowest part of the canal. The shapes of the EAC in the coronal and sagittal planes were also examined.ResultsThe numbers of female and male patients were similar, and the mean age was 49.45±13.95 years. The anterior-superior, anterior-inferior, and inferior wall thicknesses were 1.92±0.60, 2.54±0.74, and 9.16±2.20 mm, respectively. The anterior-superior and anterior-inferior wall thicknesses and canal lengths were greater on the right side (P<0.001). All measurement values were higher in males, except right tympanomandibular distance (P<0.05). A non-significant negative correlation was found between the age of the participants and the left anterior-inferior wall and tympanomandibular distance on both sides. Intra-observer agreement was high for all measurements. We observed four main shapes in the external ear canal in the coronal plane Type 3, Type 2, Type 1, and Type 4 in order of frequency on the right, and Type 2, Type 3, Type 1, and Type 4 on the left. In the sagittal plane, we detected three shapes oval (74.4%), triangular (16.3%), and round (9.4%).ConclusionThe anterior wall thicknesses and tympanomandibular distance should be measured on preoperative temporal bone CT to safely perform tympanic bone anterior resection, which is required in some otological procedures, and also to prevent temporomandibular joint damage.

Postoperative Pneumolabyrinth Following the Middle Cranial Fossa Approach for Superior Semicircular Canal Dehiscence Repair, 2014-2020.

The present study investigates risk factors and clinical outcomes of postoperative pneumolabyrinth following the middle cranial fossa approach for superior semicircular canal dehiscence repair, a complication that has not been documented previously. Retrospective cohort study. Tertiary/quaternary care referral center. We conducted a retrospective review of 332 middle cranial fossa procedures from 2014 to 2020 at a tertiary/quaternary care institution. Upon identifying pneumolabyrinth cases from postoperative computed tomography temporal bone scans, we conducted multivariable logistic regression analysis to explore demographic and clinical factors that were independently linked to this complication. We also compared the rates of postoperative symptoms among patients with different grades of pneumolabyrinth. We identified 41 (12.3%) pneumolabyrinth cases. Patients with older age, higher preoperative pure tone average, and frank dehiscence were at higher risk for pneumolabyrinth. Furthermore, patients with pneumolabyrinth reported significantly higher rates of postoperative dizziness and exhibited significantly greater pure tone average and air-bone gap postoperatively as compared with patients without pneumolabyrinth. Finally, higher-grade pneumolabyrinth was associated with increased rates of postoperative hearing loss, and grade III pneumolabyrinth was associated with higher rates of postoperative tinnitus vs grade I and II cases. Pneumolabyrinth following the middle cranial fossa approach was associated with poor clinical outcomes, including dizziness and auditory impairment. Old age, high preoperative pure tone average, and frank dehiscence were risk factors for this complication. The highlighted parameters may be important to note when considering this approach as a treatment option and while monitoring postoperative recovery.

Prevalence of otic capsule dehiscence in temporal bone computed tomography scan.

To investigate the radiologic prevalence of otic capsule dehiscence in temporal bone computed tomography (CT) scan. This was a cross-sectional study. The temporal bone CT scans of the patients presenting to a tertiary center were evaluated for possible dehiscence in any part of the otic capsule; then, all of the dehiscence were confirmed by a trained academic radiologist. For each type of otic capsule dehiscence, at least three planes were used with a specific type of reconstruction. When a dehiscence was present in at least two consecutive images in all planes, it was considered as a true dehiscence. Six hundred patients (mean age: 40 ± 18.1years) were enrolled. The prevalence of superior and posterior semicircular canal dehiscence was 6.0% and 2.7%, respectively. The prevalence of cochlear-facial dehiscence, cochlear-internal auditory canal dehiscence, and cochlear-carotid dehiscence was 6.3%, 0.7%, and 0.7%, respectively. The prevalence of vestibular aqueduct-jugular bulb dehiscence was 6.3% and the prevalence of posterior semicircular canal-jugular bulb dehiscence was 0.2%. Considering different types of otic capsule dehiscence described, care should be taken in patients with vestibular and auditory signs and symptoms to diagnose these dehiscences.

Incidence of Concomitant Semicircular Canal Dehiscence With Otosclerosis.

The concurrence of otosclerosis and superior semicircular canal dehiscence (SSCD) presents a diagnostic challenge and failure to differentiate between these 2 diagnoses results in mischaracterization and unsuccessful surgery. The objective of this study is to identify the incidence of SSCD in patients who have computed tomography (CT) evidence of otosclerosis. Retrospective chart review. Tertiary referral hospital. Adults with CT scan of the temporal bone diagnosed with radiological unilateral or bilateral fenestral otosclerosis from January 1995 to April 2018. Retrospective review of patient imaging from a multi-center tertiary-referral health system from January 1995 to April 2018. Imaging was reviewed to quantify the incidence of SSCD among patients with CT-diagnosed bilateral fenestral otosclerosis. Poor quality imaging was excluded from review. One-thousand two-hundred eight patients (1214 CT scans) were identified with otosclerosis, of which 373 were diagnosed with fenestral otosclerosis (663 ears) with imaging of sufficient quality for review. This population was predominantly female (57.2%) with bilateral fenestral otosclerosis (78%). Of these, 23 ears (3.5%) had definitive evidence of SSCD, with an additional 15 ears (2.3%) with possible radiographic evidence of SSCD. There was no significant difference in laterality between the SSCD and otosclerosis. Among 373 patients with fenestral otosclerosis per CT temporal bone imaging at a tertiary referral hospital, as many as 8.3% of patients had radiographic evidence of SSCD. Given this incidence, it continues to be important to consider SSCD when diagnosing and treating otosclerosis.

Unusual Neurological Manifestation of Langerhans Cell Histiocytosis in an Adult.

Langerhans Cell Histiocytosis is a rare disorder characterized by an abnormal proliferation of Langerhans cells in one or multiple organs. It rarely presents with a central vestibular involvement. We report a case of a multisystem Langerhans Cell Histiocytosis with mucosal, hepatic, and neurological involvement, presenting with dizziness and balance disorders. A 39-year-old woman with a histopathologically confirmed mucosal palatal Langerhans Cell Histiocytosis presented with a history of dizziness for a year. Vestibular examination revealed a saccadic eyes pursuit, an up beating spontaneous nystagmus, a bilateral gaze nystagmus and a prolonged positional nystagmus, in the supine roll test. Pure tone audiometry showed a slight left sensorineural hearing loss at the 8000Hz frequency. Computed tomography (CT) scan showed a bilateral maxillary sinus fullness and a peripheral osteosclerosis of the surrounding bony walls. Hepatic magnetic resonance imaging (MRI) showed a typical hepatic involvement with a hepatomegaly with countless cysts. Temporal bone CT scan and MRI were normal. Cerebral MRI showed an hyperintense nodular signal at T2 FLAIR weighted images lateral to the right pons, at the level of the left middle cerebellar peduncle and at the left mesencephalon. Balance disorders can rarely present a sign of a degenerative neurological cerebellar involvement. Such a rare manifestation can present in different neurological disorders such as Langerhans' cell Histiocytosis.

High Heterogeneity of Temporal Bone CT Aspects in Osteogenesis Imperfecta Is Not Linked to Hearing Loss

Objectives: To determine whether temporal bone computed tomography (CT) features are linked to the presence and type of hearing loss in osteogenesis imperfecta (OI) when considering hearing-impaired OI patients and normally hearing (NH) OI ones. A secondary objective was to assess whether other factors influence CT features in a large sample: age, type of mutation, or bone mineral density (BMD). Methods: A total of 41 adults with OI underwent CTs and pure-tone audiometry in 82 ears. Hearing thresholds were normal in 64 out of 82 ears, and most had not been operated on for stapedectomy or stapedotomy. Ossicle density, footplates, oval and round windows, retrofenestral peri- and endolabyrinths, and temporal pneumatization were analyzed twice by an experienced radiologist. CT features were compared to hearing, age, collagen mutations, and bone mineral density. Results: Unexpectedly a high prevalence of footplate, ossicle, and otic capsule anomalies was observed, even in NH ears. Footplate hypodensity or thickening was mostly found in ears without conductive hearing loss. There were significantly more retrofenestral anomalies or window obstruction in ears with a sensorineural hearing loss component than in ears without. Age was significantly higher in ears with middle layer hypodensity than in ears without. Patients with mutations were expected to have reduced collagen quantity and had significantly more footplate or retrofenestral anomalies than those with qualitative mutations. BMD was significantly higher in ears without temporal hyperpneumatization. Conclusion: Temporal bone CT features in OI are present in a large proportion of patients, had they hearing loss or not, and might be determined more by collagen mutation type than by age or BMD.

Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss

Introduction: The rates of cochlear nerve abnormalities and cochlear malformations in pediatric unilateral hearing loss (UHL) are conflicting in the literature, with important implications on management. The aim of this study was to investigate the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL). Methods: A retrospective chart review of pediatric subjects <18 years of age evaluated for UHL or AHL with fine-cut heavily T2-weighted magnetic resonance imaging (MRI) between January 2014 and October 2019 (n = 291) at a tertiary referral center was conducted. MRI brain and computed tomography temporal bone were reviewed for the presence of inner ear malformations and/or CND. Status of the ipsilateral cochlear nerve and inner ear was evaluated. Pure tone average (PTA) at 500, 1,000 and 2,000 Hz was assessed. Results: 204 subjects with UHL and 87 subjects with AHL were included. CND (aplasia or hypoplasia) was demonstrated in 61 pediatric subjects with UHL (29.9%) and 10 with AHL (11.5%). Ipsilateral cochlear malformations were noted in 25 subjects with UHL (12.3%) and 11 with AHL (12.6%), and ipsilateral vestibular malformations in 23 (11.3%) and 12 (13.8%) ears, respectively. Median PTA was statistically significantly higher in ears with CND (98.33) than ears with normal nerves (90.84). Discussion/Conclusion: Imaging demonstrated a high incidence of inner ear malformations, particularly CND, in pediatric subjects with UHL. Auditory findings indicated CND cannot be ruled out by thresholds alone as some CND ears did demonstrate measurable hearing. Radiologic evaluation by MRI should be performed in all patients within this population to guide counseling and management of hearing loss based on etiology, with implications on candidacy for cochlear implantation.