Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of\nan acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner\near malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed\ntomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for\ncochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation.\nWe also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the\nmanagement of language developmental disorders. Methods.The present study is a retrospective observational review of cochlear\nimplant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations\nwith MRI and HRCT. Furthermore, genetic results frommolecular genetic investigations (GJB2/GJB6 and, additionally, in selected\ncases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional\nanalysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department\nof the University Hospital of Ferrara, 620 cochlear implantations were performed.There were 426 implanted children at the time\nof the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain\nanomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143\nimplanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear\nimplants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies\nrepresent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity\nand stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent.
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