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MRI scan of inner ear with axial section showing cochlear anomaly in which cochlea shows 1 and 1/2 turns.
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. Congenital sensorineural hearing loss is one of the most common birth defects with incidence of approximately 1 : 1000 live births. Imaging of cases of congenital sensorineural hearing loss is frequently performed in an attempt to determine the underlying pathology. There is a paucity of literature from India and for this reason we decided to con...
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Enlarged vestibular aqueduct (EVA) is one of the most common congenital inner ear malformations and accounts for 1–12% of sensorineural deafness in children and adolescents. Multiple genetic defects contribute to EVA; therefore, early molecular diagnosis is critical for EVA patients to ensure that the most effective treatment strategies are employe...
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... Congenital sensorineural hearing loss (SNHL) is one of the most common birth defects with an incidence ratio of 1:1000 live births in India [1]. Imaging plays an important role in the evaluation of congenital SNHL [2], and the cochlear implant has emerged as the rehabilitative option of choice for patients with severe to profound SNHL. ...
Background Congenital sensorineural hearing loss (SNHL) is one of the most common birth defects with an incidence ratio of 1:1000 live births in India. Imaging plays an important role in the evaluation of congenital SNHL. As there is a paucity of studies in the Indian setting to determine the prevalence of inner-ear abnormalities, this study attempts to throw light on the various inner-ear anomalies that are prevalent in our setup in the Northern part of Karnataka using high-resolution computed tomography (HRCT) temporal bone scan. Objectives The objectives of this study are estimation of the prevalence of inner-ear anomalies in children with congenital SNHL by employing a radiologic assessment of HRCT temporal bone scans and determination of the factors associated with the identification of these abnormalities like demographic factors and degree of hearing loss. Methods Children with congenital SNHL underwent clinical evaluation with history taking and general and ear examination. Otoacoustic emission (OAE) and brainstem evoked response audiometry (BERA) measurements were obtained. A radiological assessment by HRCT temporal bone scan was done. Using the classification criteria of inner-ear malformations by Jackler and Sennaroglu as a reference, diagnostic standards were established in studying inner-ear malformations. Data were collected and entered in a Performa, which includes patient's demography, audiological findings, and radiological findings, and the results were analyzed. Data were entered into Microsoft Excel, and statistical analysis was carried out using IBM SPSS Statistics for Windows, Version 27 (Released 2020; IBM Corp., Armonk, New York, United States). Categorical variables were presented as frequency and percentage. Then the prevalence of inner-ear anomaly was estimated. Correlation between inner-ear anomaly and other factors was calculated using the Chi-square test. Results The prevalence of inner-ear anomalies identified in congenital SNHL by HRCT scan was as follows: 26.08% (12/46), 26.1% (24/92) of inner ears was anomalous, 23.9% of the cochlea was anomalous, 6.5% of the vestibule was anomalous, 5.4% of the vestibular aqueduct was anomalous, and 3.2% of the semicircular canal was anomalous. Cochlear aplasia, incomplete partition, common cavity, and cochlear hypoplasia were the anomalies found. Few cochleas had an abnormal cochlear height, though they appeared normal structurally. The dilated vestibule was the most common vestibular abnormality. There was a negative association found between the inner-ear anomaly in children with congenital SNHL who had a history of consanguineous marriage in their parents. Conclusion High-resolution temporal CT scanning could provide detailed information on the pathology of the inner ear in congenital SNHL, which can help in better planning the surgery for cochlear implantation and understanding the prognosis.
... 7,15 Conversely, the most common malformations were dilated vestibules for other authors. 9,16 An enlarged vestibular aqueduct has been reported at high rates (40-56%) in several studies, whereas they represent only 2.5% of the malformations in our series. 9,15,17 No documented explanation could justify these significant differences. ...
... 9,16 An enlarged vestibular aqueduct has been reported at high rates (40-56%) in several studies, whereas they represent only 2.5% of the malformations in our series. 9,15,17 No documented explanation could justify these significant differences. ...
Objective The aim of this study was to determine the prevalence and types of inner ear and/or cochlear nerve anomalies in children who are candidates for cochlear implantation.
Methods This was a descriptive cross-sectional study with a retrospective review of medical and imaging records performed in a tertiary care children's hospital. All cochlear implants candidates under 15 years old with imaging assessment between January 2019 and December 2021 were concerned. The studied parameters were reason for consultation, risks factors, inner ear malformations (IEMs) classified and stratified by sensorineural hearing loss (SNHL) degree, gender, and age at diagnosis.
Results In total, 81 children (162 ears) were included in the study. Abnormalities of the inner ear were found in nine children representing a prevalence of 11.1%. These children were aged between 2 and 14 years. The average age at diagnosis of SNHL was 3.5 years. Seven children had bilateral anomalies and two unilateral anomalies, that is, 16 ears presenting 40 malformations. These included 1 complete labyrinthine aplasia (2.5%), 12 cochlear malformations (30%), 1 common cavity (2.5%), 17 vestibular/squamous cell carcinoma (SCC) malformations (42.5%), and 5 internal auditory canal malformations (12.5%). Three ear out of 16 with abnormalities demonstrated a deficient cochlear nerve. There was an association between IEMs and profound deafness, and age at diagnosis of SNHL less than 2 years.
Conclusion This study shows that 11.1% of children with profound deafness have IEMs. SCC malformations and cochlear hypoplasia were the most common. A precise description of these malformations during the imaging assessment is particularly useful for cochlear implantation to better plan this surgery.
... Likewise, 12.5% of cochlea showed type II incomplete partition (Mondini dysplasia), in which the apical and middle turns showed cystic dilatation, with normal basal turn (1 half turns); and in 6.3% out of the inner ear anomalies, the cochlea was absent (aplasia). These findings were contrary to previous studies by Haidar et al. [23] and Agarwal et al. [27] that showed that Mondini dysplasia was the most common cochlea anomalies, 25% and 41% out of bony inner ear anomalies, respectively, among children with congenital SNHL. However, in this study, cochlea hypoplasia was the most common inner ear abnormality. ...
... Furthermore, no VAs were seen in 12.5% of ears with inner ear anomalies. These findings were different from studies performed previously by Haidar et al. [23] and Agarwal et al. [27] that showed more of vestibular duct dilatations, in which VA ectasia was 50.0% and 51.5% of the inner ear anomalies, respectively. However, this study is similar to the same studies by Haidar et al. [23] and Agarwal et al. [27] in terms of multiple anomalies. ...
... These findings were different from studies performed previously by Haidar et al. [23] and Agarwal et al. [27] that showed more of vestibular duct dilatations, in which VA ectasia was 50.0% and 51.5% of the inner ear anomalies, respectively. However, this study is similar to the same studies by Haidar et al. [23] and Agarwal et al. [27] in terms of multiple anomalies. Nevertheless, in 84% of cases in other studies, it is associated with other inner ear anomalies. ...
... Phần lớn các nghiên cứu ở nước ngoài đều cho thấy tỉ lệ nam nhiều hơn nữ. Agarwal, Sangeet Kumar nghiên cứu 280 trẻ điếc tiếp nhận bẩm sinh có tỉ lệ nam:nữ là (1,3:1) tuổi trung bình là 34,3 tháng [2]. ...
... PCKHT: phân chia không hoàn toàn.(2) TĐ-OBK: tiền đình-ống bán khuyên. ...
Mục đích: Mô tả đặc điểm hình ảnh cắt lớp vi tính (CLVT) và cộng hưởng từ (CHT) xương thái dương bệnh nhân (BN) điếc tiếp nhận để lựa chọn BN cho phẫu thuật cấy ốc tai điện tử (OTĐT). Đối tượng và phương pháp nghiên cứu: mô tả đặc điểm hình ảnh tai trong, dây thần kinh (TK) ốc tai kết hợp với thính lực để đưa ra chỉ định cấy ốc tai điện tử ở 132 BN nhi điếc tiếp nhận. Hình ảnh dây TK ốc tai được đánh giá trên chuỗi xung cộng hưởng từ T2 3D gradient-echo phân giải cao. Hình ảnh tai trong được đánh giá trên CHT và CLVT phân giải cao.Kết quả: nghiên cứu gồm 132 BN với 264 tai trong đó 161 tai (61%) không dị dạng tai trong, 34 tai (12,9%) ốc tai bình thường có bất thường TK ốc tai, 65 tai (24,6%) dị dạng tai trong và 4 tai (1,5%) cốt hoá mê đạo. Những BN bất sản TK ốc tai và không có sóng V ABR, BN dị dạng nặng ốc tai, BN cốt hoá ốc tai mức độ nặng không còn chỉ định cấy OTĐT.Kết luận: chỉ định cấy ốc tai điện tử phụ thuộc vào tình trạng tai trong và sự có mặt của dây TK ốc tai trên hình ảnh hoặc đáp ứng thính giác trên thính lực.
... Phần lớn các nghiên cứu ở nước ngoài đều cho thấy tỉ lệ nam nhiều hơn nữ. Agarwal, Sangeet Kumar nghiên cứu 280 trẻ điếc tiếp nhận bẩm sinh có tỉ lệ nam:nữ là (1,3:1) tuổi trung bình là 34,3 tháng [2]. ...
Mục đích: Mô tả đặc điểm hình ảnh cắt lớp vi tính (CLVT) và cộng hưởng từ (CHT) xương thái dương bệnh nhân (BN) điếc tiếp nhận để lựa chọn BN cho phẫu thuật cấy ốc tai điện tử (OTĐT). Đối tượng và phương pháp nghiên cứu: mô tả đặc điểm hình ảnh tai trong, dây thần kinh (TK) ốc tai kết hợp với thính lực để đưa ra chỉ định cấy ốc tai điện tử ở 132 BN nhi điếc tiếp nhận. Hình ảnh dây TK ốc tai được đánh giá trên chuỗi xung cộng hưởng từ T2 3D gradient-echo phân giải cao. Hình ảnh tai trong được đánh giá trên CHT và CLVT phân giải cao. Kết quả: nghiên cứu gồm 132 BN với 264 tai trong đó 161 tai (61%) không dị dạng tai trong, 34 tai (12,9%) ốc tai bình thường có bất thường TK ốc tai, 65 tai (24,6%) dị dạng tai trong và 4 tai (1,5%) cốt hoá mê đạo. Những BN bất sản TK ốc tai và không có sóng V ABR, BN dị dạng nặng ốc tai, BN cốt hoá ốc tai mức độ nặng không còn chỉ định cấy OTĐT. Kết luận: chỉ định cấy ốc tai điện tử phụ thuộc vào tình trạng tai trong và sự có mặt của dây TK ốc tai trên hình ảnh hoặc đáp ứng thính giác trên thính lực.
... In contrast, Agarwal SK et al. reported a frequency of 13.93%, indicating that probably population in this subcontinent was less prone to develop IEM's. 17 Large variations in the results of different studies have been reported. In a Saudi study by Aldhefeeri & Alsanosi Large vestibular aqueduct was commonest pathology (33.3%), followed by dysplasia of SCC (29.1%) and cochlear hypoplasia (4.1%). ...
... 16 While in an Indian study by Agarwal SK et al. the prevalence of cochlear anomaly was 73%, 87.1% had vestibular malformations, 56.4% had vestibular aqueduct malformation, 30.7% were with IAC anomaly and 29.4% had 8 th CN anomalies. 17 In an imaging study by El Sheikh E et al. cochlear hypoplasia the commonest (4.5%), followed by common cavity (3%), IP-1 (3%), IP-2 (16.7%), IP-3 (7.6%), posterior rotated cochlea (6.1%), dilated vestibule (3%), isolated SCC hypoplasia (3.03%), SCC hypoplasia with common cavity (3.03%), dysplastic SCC (6.8%), dilated IAC (4.5%), hypo-plastic IAC (18.2%), ...
Objective:
To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies.
Methods:
This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22.
Results:
The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and left ears was noted with inner ear malformations. The most common anomaly noted were CLA, CH-III and CH-II in decreasing order of frequency in both ears.
Conclusion:
The prevalence of IEM's was found to be 48(10%). Commonest anomalies noted were CLA, CH-III and CH-II. No significant association of gender and age of presentation was noted with type of anomaly in both ears.
... This study demonstrated that CT and MRI were superior to CT alone. 5 A similar experience was observed in our study, where CT and MRI were found superior in decision making 8 In our study, cochlea/ cochlea-vestibular deficiency, endolymphatic duct dilatation, and vestibule/SCC anomaly were found in 12.5, 10.5, and 5.8%, respectively, as compared with 25, 12, and 7% reported by Lin et al. 9 Our finding is also comparable with Agarwal et al. 10 Surgical difficulty was negatively associated with higher size of EAC, distance between tympanic segment of facial canal to EAC, and BTA (p < 0.05), which indicated that patients with narrow size of the above parameters increase the difficulty of the surgery while no association was observed with age and sex (p > 0.05). Similarly, poor mastoid pneumatization, abnormal middle ear cavity, and abnormal vestibule/vestibular aqueduct were more commonly associated with surgical difficulty (p < 0.05). ...
Introduction Preoperative imaging is a standard practice for cochlear implant candidacy. We are discussing association between surgical feasibility and various anatomical details of middle and inner ear by both high-resolution computed tomography (HRCT) and 3T magnetic resonance imaging (MRI) in patients with bilateral sensorineural hearing loss (SNHL) and are used as a guide during surgery as well as foresee complications in cochlear implantation.
Materials and Methods A total of 56 patients of aged 1 to 5 years (prelingual) and > 1 years (postlingual or perilingual) having bilateral SNHL were included in the study. HRCT temporal bone and MRI head was done in all candidates. Based on the imaging findings of CT and MRI, patients were divided into two categories (normal and abnormal). Demographic and clinical values were compared between two groups. Binary logistic regression analysis was used to identify the predictors of the outcome variable (surgical difficulty).
Results Out of 56 patients, 55 underwent cochlear implantation by Veria technique, one patient having Michel’s deformity was denied surgery and was advised brain stem implant. Note that 69.1% patients had no radiological abnormality, while 30.9% patients showed abnormality including acquired disease and malformation. Out of the total study patients, 18% (n = 10) showed various type of congenital inner ear malformation. Large size of the external auditory canal (EAC), high basal turn angle (BTA), and increased distance between tympanic segment of facial canal to EAC (midpoint between level of I-S joint corresponding to EAC and exit of chordae tympanic at level of EAC), all these factors showed inverse relationship with difficulty in surgery, which were found to be statistically significant.
Conclusion The imaging in cochlear implant patients is an essential tool for preoperative assessment of candidacy, surgical planning, and avoid intra- and postoperative complications. BTA and distance between tympanic segment of facial nerve and EAC (midpoint between level of I-S joint corresponding to EAC and exit of chordae tympanic at level of EAC) are important predictors for evaluating intra- and postoperative complications.
... In an additional 10 studies radiology constituted the single investigative modality (table 2) [71,76,79,[97][98][99][100][101][102][103]. The majority of the studies (31) employed computed tomography (CT) with, where indicated, 1 to 2 mm slice thickness. ...
... The majority of the studies (31) employed computed tomography (CT) with, where indicated, 1 to 2 mm slice thickness. Magnetic resonance imaging (MRI) was additionally performed in 16 studies [24,26,32,33,37,45,50,53,54,[58][59][60][101][102][103] (table 3). Kimani et al. reported MRI results only [47]. ...
Background:
The introduction of newborn hearing screening has led to earlier identification of children with congenital sensorineural hearing loss (SNHL). Aetiological clarification offers several benefits. There is currently a lack of agreement on which examinations should be recommended.
Objective:
Descriptive review of the literature reporting investigations performed to establish the aetiology of congenital SNHL and comparison of the management policy in Swiss referral centres.
Methods:
PubMed Search from 1985 to March 2016 with specific search terms; study selection according to inclusion/exclusion criteria; narrative analysis by use of defined criteria and question-naire.
Results:
Ninety-two studies were finally included in this review. Forty studies investigated more than a single aetiology. Overall frequencies of aetiological parameters investigated were: genetic (47 studies), radiological (35), ophthalmic (35), serological (32), cardiac (25), renal (14), endocrine (12), neurological (8). Most of the studies were retrospective and various limitations such as poor population description, incomplete data or deficiencies in methodological quality were frequently detected. The variability detected in the investigative approach chosen by Swiss referral centres reflects the heterogeneous data seen in the literature.
Conclusions:
The evidence in the literature regarding an appro-priate evaluation is mostly of low quality and difficult to assess owing to high heterogeneity. Nevertheless, imaging, genetic testing, neuropaediatric and ophthalmological evaluations, electrocardiograms and cytomegalovirus analysis have been identified as examinations to be included in the assessment of children with congenital SNHL. There is a need for international consensus on the various issues of such an evaluation, such as choice of investigations and diagnostic criteria.
... Stapes footplate fixation is the most common isolated congenital ossicular anomaly, which is usually bilateral. Stapes anomalies include aplasia, hypoplasia or fetal form, absence of the head and crura, fusion of the head to the promontory, and footplate fixation [2,[6][7][8] . ...
Background: We aimed to review the imaging findings of the most common congenital anomalies of the temporal bone and variations of the anatomic landmarks.
Material and methods: We retrospectively reviewed the 1,894 temporal bone computed tomography (CT) and 986 temporal bone magnetic resonance imaging (MRI) with regard to congenital anomaly and accompanying pathologies. Thin-section CT and thin-section T2-weighted gradient echo or fast spin-echo MRI was performed to identify malformations. The cases were admitted to our hospital with hearing loss, tinnitus, vertigo and non-specific infection complaints.
Results: We detected 22 cases with a temporal anomaly among 1,894 temporal CT and 986 temporal MRI images. We discussed the most common congenital anomalies of the temporal bone with imaging findings of our cases.
Conclusion: This clinical/imaging review illustrates and reviews congenital abnormalities of the temporal bone: external, middle, and inner ear anomalies, vascular malformations, and variations in high-resolution CT and MR images.
... Magnetic resonance imaging plays a very important role in assessing the cochlear nerve deficiency. Various MR techniques [3] [4] which include 3D-CISS MR [5] , High resolution T2 MR [6] imaging has been developed to study the cochlear nerves and its size. ...
... Using the definition of the kurtosis tensor (Eq. [2]) in Eq. [3] we find (4) Then, Mean of the kurtosis tensor as the average over all directions has been defined as (5) However, no simple relation to exists in general, because according to Eq. [4], ...
