Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience

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. doi: https://doi.org/10.12669/pjms.36.7.3134 How to cite this:Ahmed J, Saqulain G, Khan MIJ, Kausar M. Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience . Pak J Med Sci. 2020;36(7):1511-1516.  doi: https://doi.org/10.12669/pjms.36.7.3134 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.