Imaging plays an important role in the evaluation of congenital sensorineural hearing loss. In children who are candidates for cochlear implantation surgery, it provides vital preoperative information about the inner ear, the vestibulocochlear nerve, and the brain. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging provide excellent delineation of the intricate anatomy of the inner ear: CT depicts the minute details of osseous structures, and MR imaging allows visualization of the fluid-filled spaces and the vestibulocochlear nerve. Together, these complementary modalities can aid decision making about the best management strategy by facilitating the identification and characterization of inner ear malformations and any associated neurologic abnormalities. It is important that the radiologist be familiar with the key imaging features when interpreting CT and MR images obtained in this patient group. A broad spectrum of inner ear malformations have been described and linked to developmental insults at different stages of embryogenesis, and various systems have been proposed for classifying them. In this article, these malformations are described by using classification systems used by otolaryngologists for ease of interpretation. The relevant normal anatomy and development of the inner ear are briefly surveyed, standard imaging protocols for studying the inner ear are reviewed, and the imaging appearances of frequently observed inner ear malformations are described and illustrated. The impact of the identification of these malformations and commonly associated brain abnormalities on clinical management and prognosis also is discussed.
Fibromuscular dysplasia (FMD) is an idiopathic, nonatherosclerotic, noninflammatory disease with segmental involvement of the blood vessels that cause abnormal growth within the wall of an artery in any region of body. Fibromuscular dysplasia has been found in nearly every arterial bed in the body. However, the most common arteries affected are the renal and carotid arteries. It is a heterogeneous group of vascular lesions characterized by an idiopathic, noninflammatory, and nonatherosclerotic angiopathy of small and medium-sized arteries. The prevalence of FMD is estimated between 4 and 6% in the renal arteries and between 0.3 and 3% in the cervicoencephalic arteries.Imaging and radiologists play an important role in diagnosing the abnormality with knowledge of patient complaints with respect to fibromuscular disease. The most common imaging finding is dilatations, beaded appearance of vessels, and aneurysms. The less common findings are tortuous vessels, ectasia, kinking, loops, and dissection. The radiologist should be aware of these so that FMD can be diagnosed in young females with hypertension not responding well to treatment or familial hypertension.Its signs and symptoms help the radiologist to diagnose early. The objective of this review is therefore to increase radiologists' and clinicians' awareness of FMD's epidemiology, pathophysiology, clinical presentation, classical and minor/ rare radiological findings, and possible complications in other arteries in the abdomen. Epidemiology:The prevalence is unknown. It is most common in young women with a female to male ratio of 3:1, and is typically diagnosed between the ages of 30 and 50 years. It is less than 2% of all hypertensions.
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