Role of Imaging in congenital inner ear anomalies in children with severe -profound sensory neural hearing loss

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Nitika Yadav, MBBS MS ENT-HNS Ankit Mishra, MBBS MS ENT-HNS Dinesh K Patel, MBBS MS ENT-HNS Shilpi Parihar, MBBS DNB ENT


Sensory neural hearing loss (SNHL) is quite a significantly burdened cause of childhood disability; the estimated prevalence is 1 in 2000 neonates and 6 in 1000 children present with SNHL by 18 years of age. High-resolution computed tomography (HRCT) of the temporal bone and magnetic resonance imaging (MRI) of the temporal region and brain provide invaluable evaluation and characterization of inner ear structures and their anomalies. Radiological imaging also plays a major role in cochlear implantation with regard to intraoperative monitoring, postoperative evaluation and also in research and experimental techniques. Imaging the complete intracranial as well as extracranial auditory pathway of the implant candidate is necessary to screen for morphological conditions that will preclude or complicate the implantation process including final outcome in terms of development of speech. In this study we have analysed the inner ear congenital anomalies and their prevalence which we encountered while imaging congenitally deaf and mute children to decide their candidature for cochlear implantation and also there is a paucity of literature in this regard in Indian context for this reason we conducted this study.

This prospective study was conducted at a tertiary care center in central India, which is also a designated center for cochlear implant surgery. All children attending the outpatient department with hearing loss in the age group of 1–7 years were screened, and those who had severe to profound sensorineural hearing loss [>90 dB] were selected as subjects. All patients were subjected to an audiological assessment after ear examination, HRCT, and MRI of the temporal bone.

The total number of children analyzed was 210 with congenital SNHL. The number of radiologically abnormal cases with one or more anomalies as per HRCT and MRI temporal bone along with congenital SNHL was 35 Out of all inner ear anomalies observed, it was noticed that cochlear anomalies bear most of the burden. We observed that nearly 60% of children have one or the other cochlear anomaly present, whereas facial nerve anomalies were the least observed, i.e., only 8.57%. Among various abnormalities of vestibule, nearly 53% had dilated vestibule whereas it was hypoplastic in 13%.

With the appropriate and timely use of imaging studies and understanding the diagnostic yield of HRCT and MRI of temporal bone, it is possible to understand and clearly find out the exact cause of hearing loss in children, which can be further utilized to plan and manage the children for various options available for management of their hearing.

Keywords: Imaging, hearing loss, neural hearing loss, ear anomalies in children, sensory neural hearing loss

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YADAV, Nitika et al. Role of Imaging in congenital inner ear anomalies in children with severe -profound sensory neural hearing loss. Medical Research Archives, [S.l.], v. 12, n. 4, may 2024. ISSN 2375-1924. Available at: <>. Date accessed: 27 may 2024. doi:
Research Articles


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