Objectives: Congenital hearing loss is a significant health concern, with diverse etiologies encompassing cochlear and cochleovestibular pathologies. Preoperative radiological evaluation in cochlear implant candidates is pivotal for treatment planning. We aim to elucidate the spectrum of radiological findings in patients with congenital hearing loss undergoing cochlear implant assessment. Methods: An analysis included 389 sensorineural hearing loss (SNHL) patients who underwent cochlear implantation at a tertiary university hospital, of whose 177 were congenital SNHL. Computed tomography (CT) and magnetic resonance imaging (MRI) data were meticulously assessed for diverse congenital pathologies, focusing on congenital malformations. Results: In the congenital SNHL group, comprising 177 patients (80 females and 97 males), congenital malformations were evident in 56 ears of 29 cases. Among the various anomalies, incomplete partitions and cochlear hypoplasia emerged as significant patterns. Different congenital cochleovestibular malformations, ranging from labyrinthine aplasia to isolated large vestibular aqueducts, were detected. Conclusion: This study offers a comprehensive radiological analysis of congenital SNHL patients undergoing cochlear implantation, revealing a spectrum of anomalies. It demonstrates the diverse nature of anomalies affecting the external auditory canal, middle ear structures, and cochleovestibular system. These insights provide a deeper understanding of congenital SNHL and contribute to developing informed treatment strategies.