In the multidisciplinary management of patients with inner ear malformations (IEMs), the correct diagnosis makes the differences in terms of clinical and surgical treatment. The complex anatomical landscape of the inner ear, comprising several small structures, makes imaging of this region particularly challenging for general radiologists. Imaging techniques are important for identifying the presence and defining the type of IEM and the cochlear nerve condition. High-resolution magnetic resonance imaging (MRI) sequences and high-resolution computed tomography (HRCT) are the mainstay imaging techniques in this area. Dedicated MRI and HRCT protocols play an important role in the diagnosis and treatment of patients with inner ear disease. The most suitable technique should be selected depending on the clinical setting. However, in cases of congenital malformation of the inner ear, these techniques should be considered complementary. Since prompt intervention has a positive impact on the treatment outcomes, early diagnosis of IEMs is very important in the management of deaf patients. This article reviews the key concepts of IEMs for clinical radiologists by focusing on recent literature updates, discusses the principal imaging findings and clinical implications for every IEM subgroup, thus providing a practical diagnostic approach.