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

Abstract

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):---------. 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.

Inner Ear Malformations in Cochlear Implantation Candidates at Mother and Child Hospital of Bingerville (Côte d’Ivoire)

“…MRI essentially makes it possible to look for an anomaly of the cochlear nerve or of the labyrinthine membranous structures and of the brain. 1,4 In most cases, the underlying disorders involve the membranous labyrinth at a microscopic level and therefore radiological examinations are entirely normal. 1 The most used classification is that in eight groups.…”

“…Gender does not seem to be a predisposing factor for ear malformations either in our study or in the literature. 4 , 10 However, studies concerning the prevalence by sex of the different malformations in general are lacking, apart from those on the anomalies of the external ear that note a male predominance. 11 More than the age of the patients included, the present study demonstrated a significant correlation between IEMs and early age at diagnosis.…”

“…3 A variety of congenital anomalies are seen, syndromic and nonsyndromic. 4 These anomalies have been grouped according to various classifications and the most used is proposed by Sennaroglu. 5,6 In cases of severe-to-profound deafness, cochlear implant is proven to be the most beneficial in children.…”

“…3 A variety of congenital anomalies are seen, syndromic and nonsyndromic. 4 These anomalies have been grouped according to various classifications and the most used is proposed by Sennaroglu. 5 , 6…”

Unimodal versus bimodal auditory stimulation in inner ear malformations: Cognitive, language, and motor performance

“…Inner ear malformations (IEMs) account for around 10%–20% of congenital sensorineural hearing loss 1,2 . Sennaroglu 1 classified IEMs as complete labyrinthine aplasia (CLA), rudimentary otocyst (RO), cochlear aplasia (CA), common cavity (CC), incomplete partition type I (IP‐I), type II (IP‐II), type III (IP‐III), cochlear hypoplasia type I (CH‐I), type II (CH‐II), type III (CH‐III), type IV (CH‐IV), enlarged vestibular aqueduct (EVA), and cochlear aperture abnormalities (CAA).…”