Exploring Cochlear and Vestibular Abnormalities in Children with Hearing Impairment; a Tertiary Care Experience
Exploring Cochlear and Vestibular Abnormalities in Children with Hearing Impairment
DOI:
https://doi.org/10.54393/pjhs.v7i1.3585Keywords:
Anatomical Defects, Cochlear Implant, Hearing Impairment, Inner Ear MalformationsAbstract
Inner ear malformations have a great impact on hearing. Diagnosing them accurately through advanced imaging modalities can help plan management strategies. Objectives: To determine anatomical defects of inner ear malformations in children requiring hearing aids and cochlear implants coming to the tertiary care hospital. Methods: A cross-sectional research design was used with a sample of 104 hearing-impaired children, aged 1 to 14 years, who had moderate to profound hearing loss, collected through a purposive sampling technique. Demographic information was collected through a specially designed questionnaire, which included age, gender, developmental delay, etc. were also assessed. Results: 7 (6.7%; 95% CI (0.019, 0.115)) cases had anatomical defects of the inner ear, including the temporal bone. Among 7 cases, all had bilateral cochlear hypoplasia CH-III, semicircular canal malformations, 3 (42.9%) bilateral hypoplasia, 3 (42.9%) bilateral dysplasia, 1 (14.4%) had bilateral aplasia of anterior and lateral semicircular canals, 3 (42.9%) bilateral dilated vestibule, and 1 (14.3%) narrowing of the internal auditory canal left side. In these 7 cases, 5 (71.4%) with profound degree hearing loss were candidates for cochlear implant and 2 (28.6%) with severe degree hearing loss were using hearing aids. Results of chi-square showed that age at the time of diagnosis (p<0.05), family history of hearing impairment (p<0.001), and severity of hearing loss (p<0.001) had a significant association with the type of hearing impairment. Conclusions: The frequency of inner ear malformations in hearing-impaired children using hearing aids or candidates for cochlear implant was 6.7%. The most common malformations were CH-III and semicircular canal malformations.
References
World Health Organization. Addressing the Rising Prevalence of Hearing Loss. 2018.
World Health Organization. Deafness and Hearing Loss [Internet]. Geneva: WHO; 2025. Available from: https://www.who.int/health-topics/hearing-loss#tab=tab_2.
Bhatti MA, Shaikh SA, Memon SF, Aamir K, Ramzan A, Shah H. Frequency of Hearing Impairment in School-Going Children of District Hyderabad, Sindh, Pakistan: Frequency of Hearing Impairment. Pakistan Journal of Health Sciences. 2024 Jul: 06-10. doi: 10.54393/pjhs.v5i07.1619. DOI: https://doi.org/10.54393/pjhs.v5i07.1619
Tanon-Anoh MJ, Badou-Nguessan EK, Bi OD, Assebian-Houenou CM, Kabas RM, Langouo YJ. Inner Ear Malformations in Cochlear Implantation Candidates at Mother and Child Hospital of Bingerville (Côte d’Ivoire). Annals of Otology and Neurotology. 2022 Aug; 5(02): 073-7. doi: 10.1055/s-0043-1764179. DOI: https://doi.org/10.1055/s-0043-1764179
Young A and Ng M. Genetic Hearing Loss. In Stat Pearls [Internet]. 2023 Apr.
Feraco P, Piccinini S, Gagliardo C. Imaging of Inner Ear Malformations: A Primer for Radiologists. La radiologia medica. 2021 Oct; 126(10): 1282-95. doi: 10.1007/s11547-021-01387-z. DOI: https://doi.org/10.1007/s11547-021-01387-z
Brotto D, Ariano M, Sozzi M, Cenedese R, Muraro E, Sorrentino F et al. Vestibular Anomalies and Dysfunctions in Children with Inner Ear Malformations: A Narrative Review. Frontiers in Pediatrics. 2023 Feb; 11: 1027045. doi: 10.3389/fped.2023.1027045. DOI: https://doi.org/10.3389/fped.2023.1027045
Sennaroğlu L and Bajin MD. Classification and Current Management of Inner Ear Malformations. Balkan Medical Journal. 2017 Sep 29; 34(5): 397-411. doi: 10.4274/balkanmedj.2017.0367. DOI: https://doi.org/10.4274/balkanmedj.2017.0367
Shaheen F, Humayoon QS, Malik S, Mumtaz S. Clinical and Genetic Attributes of Congenital Anomalies Ascertained at a Tertiary Care Hospital in Rawalpindi, Pakistan. Pakistan Journal of Medical Sciences. 2023 Nov; 39(6): 1673. doi: 10.12669/pjms.39.6.7408. DOI: https://doi.org/10.12669/pjms.39.6.7408
Hussain S, Asghar I, Sabir MU, Chattha MN, Tarar SH, Mushtaq R. Prevalence and Pattern of Congenital Malformations Among Neonates in the Neonatal Unit of a Teaching Hospital. The Journal of the Pakistan Medical Association. 2014 Jun; 64(6): 629-34.
Sennaroglu L and Saatci I. A New Classification for Cochleovestibular Malformations. The Laryngoscope. 2002 Dec;112(12): 2230-41. doi: 10.1097/00005537-200212000-00019. DOI: https://doi.org/10.1097/00005537-200212000-00019
Javad-Rashid R, Pirzadeh A, Naderpour M, Mirhaji R. Frequency of Inner Ear Anomalies Among Cochlear Implant Candidates: A Case Study in the Northwest of Iran. Malaysian Journal of Medicine and Health Sciences. 2021 Jul; 1(3).
Maqbool S, Brown N, Farid A, Ali A, Iftikhar K, Bari A et al. Construction and Validation of the Shamaq Developmental Screening Tool. Child: Care, Health and Development. 2024 Jan; 50(1): e13123. doi: 10.1111/cch.13123. DOI: https://doi.org/10.1111/cch.13123
Ahmed J, Saqulain G, Khan MI, Kausar M. Prevalence and Features of Inner Ear Malformations Among Children with Congenital Sensorineural Hearing Loss: A Public Cochlear Implant Centre Experience. Pakistan Journal of Medical Sciences. 2020 Nov; 36(7): 1511. doi: 10.12669/pjms.36.7.3134. DOI: https://doi.org/10.12669/pjms.36.7.3134
Quirk B, Youssef A, Ganau M, D'Arco F. Radiological Diagnosis of the Inner Ear Malformations in Children with Sensorineural Hearing Loss. British Institute of Radiology's Open. 2019 Jun; 1(1): 20180050. doi: 10.1259/bjro.20180050. DOI: https://doi.org/10.1259/bjro.20180050
Khan J, Slehria AU, Bilal R, Aquil H, Jadoon HK, Shehzadi Z. A Comparative Analysis of Role of Magnetic Resonance Imaging and High-Resolution Computed Tomography-Temporal Bone in Preoperative Assessment of Patients for Cochlear Implantation. Pakistan Armed Forces Medical Journal. 2025 Apr; 75(2): 328. doi: 10.51253/pafmj.v75i2.10861. DOI: https://doi.org/10.51253/pafmj.v75i2.10861
Young YM, Leung HS, Chang WT, Yu SM, Wang K, Wong KT et al. Imaging of Inner Ear Malformation in Paediatric Patients—A 10-Year Tertiary Centre Review. European Radiology. 2025 May: 1-0. doi: 10.1007/s00330-025-11663-5. DOI: https://doi.org/10.1007/s00330-025-11663-5
Karamert R, Tutar H, Altinyay Ş, Düzlü M, Yildiz M, Akdulum İ et al. Cochlear Implantation in Inner Ear Malformations: Considerations Related to Surgical Complications and Communication Skills. Journal for Oto-Rhino-Laryngology. 2022 Sep; 84(3): 211-8. doi: 10.1159/000517562. DOI: https://doi.org/10.1159/000517562
Abbas AM, Hamed MS, Ahmed AS. Cochlear Implantation in Children with Congenital Inner Ear Malformations. Polski Przegląd Otorynolaryngologiczny. 2022 Dec; 11(4): 7-12. doi: 10.5604/01.3001.0016.1567. DOI: https://doi.org/10.5604/01.3001.0016.1567
Berrettini S, Forli F, Genovese E, Santarelli R, Arslan E, Maria Chilosi A et al. Cochlear Implantation in Deaf Children with Associated Disabilities: Challenges and Outcomes. International Journal of Audiology. 2008 Jan; 47(4): 199-208. doi: 10.1080/14992020701870197. DOI: https://doi.org/10.1080/14992020701870197
Bee-See G, Zulkefli NA, Abdullah A, Umat C, Nor NK, Ismail J, O’Leary SJ. Cochlear Implantation Outcomes in Children with Multiple Disabilities: A Topic That’s Worth Revisiting. Brazilian Journal of Otorhinolaryngology. 2024 Aug; 90(4): 101423. doi: 10.1016/j.bjorl.2024.101423/ DOI: https://doi.org/10.1016/j.bjorl.2024.101423
Asha BA, Jagini JR, Sudha CR, Sandeep N. Prevalence of Temporal Bone Malformations in Prospective Cochlear Implant Candidates. Indian Journal of Otolaryngology and Head and Neck Surgery. 2023 Sep; 75(3): 2118-23. doi: 10.1007/s12070-023-03746-w. DOI: https://doi.org/10.1007/s12070-023-03746-w
Yoshimura H, Okubo T, Shinagawa J, Nishio SY, Takumi Y, Usami SI. Epidemiology, Aetiology and Diagnosis of Congenital Hearing Loss Via Hearing Screening of 153 913 Newborns. International Journal of Epidemiology. 2024 Jun; 53(3): dyae052. doi: 10.1093/ije/dyae052. DOI: https://doi.org/10.1093/ije/dyae052
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