Role of Imaging in congenital inner ear anomalies in children with severe -profound sensory neural hearing loss
Main Article Content
Abstract
Introduction
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.
Methodology
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.
Result
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%.
Conclusion
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.
Article Details
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
2. American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement:principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007;120:898–921
3. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, Part 1: Practical aspects for neuroradiologists. Am J Neuroradiol 2012;33:211‑17
4. Chen MM, Oghalai JS. Diagnosis and management of congenital sensorineural hearing loss. Curr Treat Options Pediatr 2016;2:256‑65
5. Bartel‑Friedrich S, Wulke C. Classification and diagnosis of ear malformations. GMS Curr Top Otorhinolaryngol Head Neck Surg 2007;6
6. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002;112:2230-41.
7. Mafong DD, Shin EJ, Lalwani AK. Use of laboratory evaluation and radiologic imaging in the diagnostic evaluation of children with sensorineural hearing loss. Laryngoscope. 2002;112(1):1–7.
8. Sennaroglu, G. Audiological findings and rehabilitation in children with inner ear malformations. Ankara: Hacettepe University, Department of Otolaryngology; 2002
9. Pont E, Mazón M, Montesinos P, Sánchez MÁ, Más-Estellés F. Diagnóstico por imagen: malformaciones congénitas y lesiones adquiridas del oído interno. Acta Otorrinolaringol Esp. 2015;66(4):224-33.
10. Wu W, He X, Tan L,et al. Imaging assessment of profound sensorineural deafness with inner ear anatomical abnormalities. Journal of Otology. 2015;10(1):29-38
11. Dagkiran M, Dagkiran N,et al. Radiological Imaging Findings of Patients with Congenital Totally Hearing Loss. Int Adv Otol. 2016
12. Jackler R,Luxford W,House W et al. The Laryngoscope. 1987;1 (Sup 1):3
13. Casselman JW, Offeciers EF, De Foer B,et al. CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal. Eur J Radiol 2001;40(2): 94–104
14. Joshi V, Navlekar S,et al. CT and MR Imaging of the Inner Ear and Brain in Children with Congenital Sensorineural Hearing Loss. RadioGraphics. 2012;32(3):683-698
15. Wu W, He X, Tan L, Hu P,et al. Imaging assessment of profound sensorineural deafness with inner ear anatomical abnormalities. Journal of Otology. 2015;10(1):29-38
16. Muhammed Dağkıran, Nermin Dağkıran, Özgür Sürmelioğlu, Tuğsan Ballı, Ülkü Tuncer, Erol Akgül, Fikret Çetik Radiological Imaging Findings of Patients with Congenital Totally Hearing Loss J Int Adv Otol 2016; 12(1): 43-8
17. Sennaroğlu L, Saatçi I. Magnetic resonance imaging versus computed tomography in pre-operative evaluation of cochlear implant candidates with congenital hearing loss. J Laryngol Otol 2002; 116: 804-10
18. Congenital Malformations of the Inner Ear. Ramírez L., Cano H., Lubinus F.
19. Bhagat AC, Kumar J, Garg A, Prakash A, Meher R, Arya S. Imaging in congenital inner ear malformations—An algorithmic approach. Indian J Radiol Imaging 2020;30:139-48