[Deep transfer learning radiomics model based on temporal bone CT for assisting in the diagnosis of inner ear malformations].

Objective:To evaluate the diagnostic efficacy of traditional radiomics, deep learning, and deep learning radiomics in differentiating normal and inner ear malformations on temporal bone computed tomography（CT）. Methods:A total of 572 temporal bone CT data were retrospectively collected, including 201 cases of inner ear malformation and 371 cases of normal inner ear, and randomly divided into a training cohort（n=458） and a test cohort（n=114） in a ratio of 4∶1. Deep transfer learning features and radiomics features were extracted from the CT images and feature fusion was performed to establish the least absolute shrinkage and selection operator. The CT results interpretated by two chief otologists from the National Clinical Research Center for Otorhinolaryngological Diseases served as the gold standard for diagnosis. The model performance was evaluated using receiver operating characteristic（ROC）, and the accuracy, sensitivity, specificity, and other indicators of the models were calculated. The predictive power of each model was compared using the Delong test. Results:1 179 radiomics features were obtained from traditional radiomics, 2 048 deep learning features were obtained from deep learning, and 137 features fusion were obtained after feature screening and fusion of the two. The area under the curve（AUC） of the deep learning radiomics model on the test cohort was 0.964 0（95%CI 0.931 4-0.996 8）, with an accuracy of 0.922, sensitivity of 0.881, and specificity of 0.945. The AUC of the radiomics features alone on the test cohort was 0.929 0（95%CI 0.882 2-0.974 9）, with an accuracy of 0.878, sensitivity of 0.881, and specificity of 0.877. The AUC of the deep learning features alone on the test cohort was 0.947 0（95%CI 0.898 2-0.994 8）, with an accuracy of 0.913, sensitivity of 0.810, and specificity of 0.973. The results indicated that the prediction accuracy and AUC of the deep learning radiomics model are the highest. The Delong test showed that the differences between any two models did not reach statistical significance. Conclusion:The feature fusion model can be used for the differential diagnosis of normal and inner ear malformations, and its diagnostic performance is superior to radiomics or deep learning models alone.

Cochlear Implantation by Veria Technique in Abnormal Cochlea: Our Experience.

This study describes the clinical profile, operative findings, surgical technique, type of implant and complications encountered in implantation of abnormal cochlea by the transcanal (veria) technique in a tertiary care centre. This is a retrospective study done amongst 337 patients who underwent cochlear implantation by veria technique between January 2013 to August 2023. Based on pre-operative imaging with high-resolution computed tomography and magnetic resonance imaging the cochleovestibular malformations in this study were classified according to Sennaroglu classification and the cochlear ossification was graded from I to IV. Amongst the 337 patients who underwent cochlear implantation during the study period there were 79 cases (23.4%) with abnormalities of cochlea. This included 45 patients (13.4%) with congenital malformations, 20 patients (5.9%) with cochlear fibrosis and 14 patients (4.2%) with cochlear ossification. The commonest cochlear malformation was Incomplete Partition 2 which was present in 21 patients (46.7%). The other common malformations were cochlear hypoplasia (31.1%), Incomplete Partition 1(13.3%) and common cavity (8.9%). Out of the 20 patients with cochlear fibrosis, 17 patients had fibrosis only in the basal turn. Out of the 14 patients with cochlear ossification 8 patients (57.1%) had a grade 1 ossification, 2 patients (14.3%) had a grade 2 ossification and 4 patients (28.6%) had a grade 3 ossification. One of the patients required re-exploration for correcting the placement of electrode. The type of implant were chosen depending on individual malformation or length of ossification and fibrosis. In the past, inner ear anomalies were considered as a contraindication for cochlear implantation however it is now possible to implant most of these abnormal cochlea by careful planning, a modification of the surgical technique and the type of electrode used.

Study on characteristics of wideband acoustic immittance in patients with Inner Ear Malformations.

OBJECTIVE: To compare the differences in wideband absorbance and the resonance frequency (RF) between patients with inner ear malformations and normal control, and to explore the auditory diagnostic value of wideband acoustic immittance (WAI). METHODS: A total of 38 patients (59 ears) with enlarged vestibular aqueduct (EVA), 13 patients (14 ears) with incomplete partition type I (IP-I) and 13 patients (26 ears) with incomplete partition type II (IP-II) were included. 50 normal control (100 ears). All subjects underwent WAI tests to compare the absorbance configuration and resonance frequency. RESULTS: All the group showed lower absorbance at ambient pressure than at peak pressure in certain frequencies under 2000Hz. Under 1000Hz, the absorbance of EVA was higher than that of other groups. The average absorbance and highest absorbance of IP-I were the lowest(P＜0.05). However, IP-II and normal group had similarity on some characteristics. The three IEM groups mainly different at low and high frequencies, but not at medium frequencies. The highest absorbance of all the groups were appeared around 3000Hz. The RF of all the groups from low to high were EVA＜IP-II＜normal control＜IP-I, and the lowest was EVA(P＜0.05). CONCLUSION: Inner ear malformations can affect energy absorbance and RF. WAI is sensitive and non-invasive to provide useful information about inner ear status and facilitate detection of ear pathology.

Role of Imaging in Evaluating Patients for Cochlear Implantation.

Role of Imaging in evaluating patients for Cochlear Implantation. To assess the role of imaging using High resolution computed tomography (HRCT) and Magnetic resonance imaging (MRI) temporal bone for evaluating candidates for cochlear implantation (CI). It was a prospective study conducted in the department of Otorhinolaryngology at a tertiary care centre, 30 children up to 5 years of age with severe to profound sensorineural hearing loss(SNHL) were included in the study, radiological evaluation was done in all children. 20(66.67%) children were in 2-4 years age group with female preponderance. Radiological abnormalities were reported in 13(43.33%) children. Abnormalities of inner ear were seen in 8(26.67%) cases, which included bilateral cochlear nerve aplasia, unilateral cochlear aplasia with bilateral cochlear nerve aplasia, bilateral severe cochlear hypoplasia, mondini's dysplasia. Cochlear nerve deficiency was found in 3(10%) children and narrow Internal auditory canal in 4(13.33%) children. 2(6.67%) patients out of 30 were not the candidates for CI, they had bilateral absent cochlear nerve which is an absolute contraindication for CI. 28(93.33%) patients were evaluated as candidates for CI. Imaging is a fundamental part of the preoperative workup for cochlear implantation, HRCT and MRI temporal bone are complementary to each other in evaluating children for cochlear implantation as HRCT is excellent for demonstrating bony details but, lack in providing details of inner ear neural structures and MRI is better than CT in demonstrating vestibulocochlear nerves.

Unimodal versus bimodal auditory stimulation in inner ear malformations: Cognitive, language, and motor performance.

Purpose: New perspectives on rehabilitation options for inner ear malformations have still been studied in the literature. This study investigated the cognitive, language, and motor skills of auditory brainstem implant (ABI) users in unimodal and bimodal groups. Methods: The motor competency of the participants was assessed with Bruininks-Oseretsky Motor Proficiency Test-2 Short Form (BOT2 SF). Language performance was evaluated by the test of Early Language Development-3 and Speech Intelligibility Rating. Word identification, sentence recognition tests, and Categories of Auditory Performance were used to assess auditory perception skills. To examine the cognitive performance, Cancellation Test and Gesell Copy Form were administered. All the tests were conducted in a quiet environment without any distractions. Results: The participants were divided into two groups: (1) 17 children in the unimodal group and (2) 11 children in the bimodal (who used a cochlear implant on one side and ABI on the other side) group. There were significant correlations between the chronological age of participants and BOT2 SF total score, cancellation tests, auditory perception tests, and language performance. Similarly, there were significant correlations between the duration of ABI use and auditory perception tests, language performance, cancellation test, and some BOT2 SF subtests (r = -0.47 to -0.60, p < .001). There was no significant difference between the unimodal and bimodal groups in any task (p > .05). However, there were moderate-to-strong correlations among the auditory perception tests, cancellation test, language test, and BOT2 SF total score and subtests (r = 0.40 to 0.55, p < .05). Conclusion: Although there were no significant differences between bimodal and unimodal groups, a holistic approach, which indicates that hearing and balance issues can have broader impacts on a person's physical, emotional, social, and psychological aspects, should be used in the assessment process. Level of Evidence: Level 4.

Effect of congenital inner ear malformations (IEMs) on electrically evoked compound action potential (ECAP) responses in cochlear implant children.

PURPOSE: The study was designed to assess the electrically evoked compound action potential (ECAP) responses in children with inner ear malformations compared to children with normal inner ear anatomy. METHODS: The study included 235 prelingual deaf children who were implanted in cochlear implant unit in King Fahad University hospital-Imam Abdulrahman Bin Faisel University. Subjects were using either Cochlear Nucleus or Medel cochlear implant devices. We had 171 (64.5%) subjects with normal inner ear anatomy and 94 (35.5%) subjects with inner ear malformations (IEMs) and they were classified into 6 groups according to inner ear anatomy. Fourteen subjects (14.9%) subjects had enlarged vestibular aqueduct (EVA), 30 (32%) subjects had Mondini deformity, 25 (26.6%) subjects had incomplete partition type two (IPII), 9 (9.6%) subjects had incomplete partition type one (IPI) and 16 (17%) subjects had hypoplastic cochlea type III or IV. Intraoperative electrically evoked compound action potential (ECAP) responses were analyzed and compared in all subjects. RESULTS AND CONCLUSIONS: Measurable ECAP responses can be elicited in patients with IEMs in most of the channels. Severe malformations can affect the prevalence of measuring ECAP and getting identifiable waveform morphology. Additionally, increased thresholds and lower slope of AGF was observed in IEMs specially in more severe malformations (e.g. IPI). IPI patients with better word recognition scores tended to show more identifiable ECAP measurements. This could suggest the presence of some correlation between ECAP responses and patients' performance after cochlear implantation.

Audiological and Speech Outcomes of Cochlear Implantation in Inner Ear Malformation Compared to the Normal Inner Ear.

To compare the overall clinical outcomes of cochlear implantation in children with structural inner ear abnormalities, with results of implantation in children with radiologically 'normal' inner ears. To study the incidence and performance outcomes of cochlear implantation in children with inner ear malformations (IEMs). It is a retrospective case control study of 57 normal and 57 abnormal inner ear patients operated for cochlear implant between Jan 1, 2014 and Aug 1, 2017, by Veria technique. The age range was between 12 months and 15 years. The prevalence of IEMs was 13.8%. Of the 57 cases with IEMs, 33.3% were of enlarged vestibular acqueduct, 7% were of isolated Incomplete Partition type-2, 21.1% cases were of Mondini's dysplasia, 3.5% of Incomplete partition type-1 and 8.8% of cochlear nerve hypoplasia. Most commonly encountered malformation was EVA while IP-1 was the least common malformation. A CSF gusher was experienced in 11 cases. There was a statistically significant difference between the CAP and SIR scores of both IEM and control groups, both at 1 year and at 2 years (1 year CAP score p value < 0.001, 2 year CAP score p value 0.002 and 1 year SIR score, p value < 0.001, 2 year SIR score, p value 0.013). There was progressive improvement in the scores (of both groups) at the 2 year mark, compared to 1 year scores. Cochlear implantation is safe and surgically feasible in children with IEMs. However, the hearing outcome and speech perception outcomes are poor than those of patients with normal anatomy, with an exception seen in enlarged vestibular aqueduct.

Recurrent Meningitis in a Non-user Cochlear Implantee.

Meningitis is a known complication in patients with inner ear malformations. Here we present a case of recurrent meningitis following cochlear implantation, in a patient with cochleovestibular anomaly. Good knowledge in radiology to identify the inner ear malformations, presence of cochlea and cochlear nerve is essential before planning cochlear implantation and meningitis can occur several decades after cochlear implantation.

Speech Perception and Sound Localization Skills in Inner Ear Malformations: Children With Incomplete Partition Type-II.

OBJECTIVE: The present study aimed to evaluate binaural auditory skills in bimodal and bilateral pediatric cochlear implant (CI) users with incomplete partition type-II (IP-II) and to reveal the effect of IP-II on performance by comparing the results to pediatric CI users with normal cochlear morphology. STUDY DESIGN: Cross-sectional study. SETTING: Tertiary referral center. METHODS: Forty-one CI users (mean age 8.8 ± 1.9) were grouped as bimodal (BIM-IP) and bilateral (BIL-IP) users with IP-II; bimodal (BIM-N) and bilateral (BIL-N) users with normal cochlear anatomy. Speech perception in noise and sound localization skills were compared under 2 conditions; binaural (bilateral or bimodal) and monaural (first CI alone). RESULTS: BIM-IP and BIL-IP showed no performance difference in binaural tasks. The BIM-N group showed remarkably poor performance in comparison to the groups of BIL-IP (p = .007), BIM-IP (p < .001), and BIL-N (p = .004) in terms of speech-in-noise skills. In sound localization abilities, similar significant differences were found between the group of BIM-N and the groups of BIL-IP (p = .001), BIM-IP (p < .001), and BIL-N (p = .004). All groups showed statistically significant improvements in binaural condition on both tasks (p < .05). CONCLUSION: We revealed that bilateral and bimodal pediatric CI users with IP-II benefitted from implantation as much as bilateral users with normal anatomy. Differences in residual hearing between groups may explain the poor performance of bimodal users with normal cochlear morphology. To the best of our knowledge, it is the first study to unveil binaural performance characteristics in children diagnosed with a specific inner ear malformation subgroup.

Vestibular anomalies and dysfunctions in children with inner ear malformations: A narrative review.

About 20% of children with congenital hearing loss present malformations of the inner ear. In the past few years much has been understood about the morphology and function of the anterior part of the labyrinth, since hearing loss may have a dramatic effect on the overall development of a child. Nowadays, for most of them, a chance for hearing rehabilitation is available, making hearing loss a treatable condition. The anomalies range from the lack of development of the whole inner ear to specific anomalies of isolated structures. Despite the frequent concomitant involvement of the posterior part of the labyrinth, this part of the inner ear is frequently neglected while discussing its morphology and dysfunction. Even though vestibular and balance function/dysfunction may have a significant impact on the global development of children, very little is known about these specific disorders in patients with inner ear malformations. The aim of this review is to summarize the available literature about vestibular anomalies and dysfunctions in children with inner ear malformations, discussing what is currently known about the topic.

Hearing-related quality of life assessment of pediatric cochlear implant users with inner ear malformations.

OBJECTIVES: To assess the quality of life (QoL) in child and adolescent cochlear implant users with inner ear malformations (IEM) and to compare their outcomes to their cochlear implant using peers with normal inner ear structures. METHODS: The present sample consisted of 100 children (45 with IEM, 55 without IEM) and 100 adolescents (46 with IEM, 54 without IEM). The following QoL questionnaires were used to assess the hearing-related QoL: The Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26 for children between 7 and 12 years of age) and HEAR-QL-28 (for adolescents between 13 and 18 years of age). Both questionnaires were based on a 5-points Likert scale from 0 to 4, with higher scores indicating a better perception of QoL. The scores were converted to percentage values (never = 100, almost never = 75, sometimes = 50, often = 25, almost always = 0). RESULTS: For the patients with IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 50.4 (SD = 18.9) and 54.5 (SD = 19.6), respectively. For the patients without IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 72.7 (SD = 18.0) and 65.0 (SD = 19.1), respectively. For both child and adolescent subgroups, statistically significant differences were observed between QoL scores from patients with and without IEM (p < 0.001). There were no statistically significant effects of the malformation type on the QoL findings (p ≥ 0.05). CONCLUSION: Child and adolescent cochlear implant users with IEM had significantly lower scores on validated HEAR-QL versions in comparison to their implanted peers without IEM.

Feasibility of day-case pediatric cochlear implantation.

PURPOSE: Pediatric cochlear implantation is performed on an outpatient basis in an inconstant way. The aim of this study is to assess the feasibility of day-case pediatric cochlear implantation by determining the acceptability of outpatient-to-inpatient conversion rate (acceptability threshold of 5%) and to assess the safety by comparing outpatient and inpatient postoperative events. We also want to identify conversion predictive factors. METHODS: We conducted a monocentric retrospective study including 267 cases aged 6 months to 18 years who underwent unilateral cochlear implantation between 2016 and 2020. This population was divided into two groups: outpatient group (190 cases) and inpatient group (77 cases). RESULTS: Among the 190 cases scheduled as day surgery, 9 cases required conversion to conventional hospitalization which leads to an outpatient-to-inpatient conversion rate of 4.7%. Postoperative nausea and vomiting (PONV) were involved in all cases of conversion. Conversion predictive factors were the presence of an inner ear malformation at risk of gusher (OR 32.51, 95% CI [4.98-370.27], p 0.001) and the intraoperative administration of morphine (OR 8.52, 95% CI [1.38-86.84], p 0.035). There was no statistically significant difference in immediate postoperative complications (outpatient 14.2% vs inpatient 16.9% p 0.715), early-stage complications (outpatient 12.6% vs inpatient 10.4% p 0.812) and early-stage unplanned consultations (outpatient 4.7% vs inpatient 3.9% p 0.748) between outpatient and inpatient groups. Unplanned readmissions were found only in the outpatient group. CONCLUSION: Day-case pediatric cochlear implantation is a feasible and safe procedure even in infants. The outpatient-to-inpatient conversion rate of 4.7% is considered acceptable but requires anticipation of human and accommodation needs. No causes of conversion were life-threatening. The risk of postoperative complications and unplanned consultations are not influenced by the mode of hospitalization. Special attention should be paid to the prevention of PONV and the presence of inner ear malformations.

Subtotal Petrosectomy (SP) in Cochlear Implantation (CI): A Report of 92 Cases.

In most cases, cochlear implantation is a straightforward procedure. Nevertheless, there are clinical situations in which the presence of the middle ear may compromise access and/or the outcome in terms of complications. This article includes a series of patients for whom we eliminated the middle ear to facilitate placement of the electrode array of the implant and/or reduce potential complications. A total of 92 cases in 83 patients, managed by the senior author, are included in this series. Different indications are outlined that justify associating a subtotal petrosectomy technique with cochlear implantation. The steps of the technique are described. We include complications from this series that compare favorably with standard techniques.

Pediatric cochlear implant fitting parameters in inner ear malformation: Is it same with normal cochlea?

OBJECTIVES: The aim was to evaluate the cochlear implant (CI) mapping parameters of CI users with inner ear malformation (IEM) and to reveal the changes in parameters over time. METHODS: In total, 118 CI users were included with 127 ears (68-IEM; 59-normal cochlear anatomy) in present retrospective study. The impedance measurements, thresholds levels-THR, most comfortable levels- MCL, pulse width-PW and rate values were analyzed in both IEM and control group at the initial activation, 6th,12th and 24th months postoperatively. RESULTS: There were statistically significant differences in impedance measurements in several time points. And also, there was a remarkable difference in THR & MCL and PW values between IEM and control groups in all time points (p < 0.05). THR & MCL levels and PW values increased significantly between all time periods in both groups (p < 0,008) and values of parameters in IEM-group were higher than those of control group. When comparing rates, statistically significant difference was observed only at the initial activation in both within (p < 0.001) and between groups (p = 0.03). CONCLUSION: Pediatric CI users with IEM need individual changes in fitting parameters. More frequent map sessions should be planned as they require more PW, THR and MCL increase over time. The increase rate differs between IEM subgroups depending on the deviation of malformation from the normal cochlear anatomy. This study is the first to in its attempt to reveal the mapping characteristics and long-term changes in pediatric CI users with different IEM subgroups.

Cochlear Implant in Klippel-Feil Syndrome: Challenges and Concerns.

Cochlear implantation (CI) can be safely performed in patients with syndromic hearing loss. Here we present a case of CI in a child with Klippel-Feil syndrome with various skeletal, extraskeletal, cochleovestibular and Arnold-Chiari malformations. Multidisciplinary approach and good preoperative imaging play a key role in planning for surgery.

Genetics of Inner Ear Malformations: A Review.

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

Imaging in a Rare Case of Unilateral External and Middle Ear Anomalies Associated with Inner Ear Malformation.

External ear malformations are commonly associated with middle ear malformations due to their closely related embryogenesis. Inner ear anomalies are less consistently seen due to their independent development. Cross-sectional imaging has come to play a critical role in the workup of congenital deafness in the pediatric age group. To optimize speech and language development, early diagnosis and treatment are very crucial. Identification of these anomalies is additionally important when operative intervention is being planned because they may complicate or perhaps preclude the surgery.

Imaging of inner ear malformations: a primer for radiologists.

In the multidisciplinary management of patients with inner ear malformations (IEMs), the correct diagnosis makes the differences in terms of clinical and surgical treatment. The complex anatomical landscape of the inner ear, comprising several small structures, makes imaging of this region particularly challenging for general radiologists. Imaging techniques are important for identifying the presence and defining the type of IEM and the cochlear nerve condition. High-resolution magnetic resonance imaging (MRI) sequences and high-resolution computed tomography (HRCT) are the mainstay imaging techniques in this area. Dedicated MRI and HRCT protocols play an important role in the diagnosis and treatment of patients with inner ear disease. The most suitable technique should be selected depending on the clinical setting. However, in cases of congenital malformation of the inner ear, these techniques should be considered complementary. Since prompt intervention has a positive impact on the treatment outcomes, early diagnosis of IEMs is very important in the management of deaf patients. This article reviews the key concepts of IEMs for clinical radiologists by focusing on recent literature updates, discusses the principal imaging findings and clinical implications for every IEM subgroup, thus providing a practical diagnostic approach.

Nonsyndromic Congenital Causes of Sensorineural Hearing Loss in Children: An Illustrative Review.

OBJECTIVE. The purpose of this article is to provide an illustrative review of nonsyndromic congenital causes of sensorineural hearing loss (SNHL) in children. CONCLUSION. Early recognition and treatment are essential in maximizing developmental outcomes in children with congenital SNHL. Because imaging plays an integral role in identifying underlying causes of SNHL, it is imperative that radiologists be able to recognize, describe, and appropriately categorize the spectrum of congenital inner ear malformations in children.

Management of stapes footplate fistula in inner ear malformations.

BACKGROUND: Certain inner ear malformations have stapes footplate fistula which may cause meningitis during otitis media. This may result in fatality. It is the responsibility of the otolaryngologist to diagnose and treat the condition to prevent further attacks of meningitis. MATERIALS AND METHODS: Surgical findings of the 17 patients who have inner ear malformations with oval window fistula were retrospectively analyzed. Inner ear malformations were classified according to Sennaroglu classification. Different stages of stapes footplate fistula are classified. FINDINGS: Seventeen patients had spontaneous stapes footplate fistula at the oval window. No patient had spontaneous leakage at the round window site. Proper sealing of the leakage area with fascia in a dumbbell fashion is mandatory. Keeping the stapes in place and lumbar drainage are the two most important factors in the successful management of fistula. Particularly important is the simultaneous fistula repair and cochlear implantation where combined postauricular-transcanal approach provides the best method. Vaccination is important but not sufficient to prevent meningitis in inner ear malformations unless repair of the fistula is performed. CONCLUSION: If the patient has a history of meningitis in the presence of inner ear malformation, particular attention should be given to oval window area to look for an opacity, cyst or a leaking lesion at the stapes footplate. Immediate surgical exploration and repair of the leak is mandatory to prevent further attacks of meningitis. Surgeon should not leave the operation without fully controlling the leak.