• Jawwad Ahmed, Ghulam Saqulain, Muhammad Iqbal Javed Khan, and Mobeen Kausar.
• Dr. Jawwad Ahmed, FCPS (Otolaryngology), Associate Surgeon, Department of Otolaryngology & Cochlear Implantation, Capital Hospital PGMI, Islamabad, Pakistan.
• Pak J Med Sci. 2020 Nov 1; 36 (7): 1511-1516.

ObjectiveTo 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.MethodsThis 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.ResultsThe 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.ConclusionThe 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.Copyright: © Pakistan Journal of Medical Sciences.

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