title: Inner ear malformations: a practical diagnostic approach.
abstract: Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted. And finally, the radiological findings of each type of malformation are concisely described and depicted. Computed tomography and magnetic resonance imaging play a crucial role in the characterization of inner ear malformations and allow the assessment of the anatomical structures that enable the selection of appropriate treatment and surgical approach.
authors.affiliation: Área Clínica de Imagen Médica, Hospital Universitario y Politécnico La Fe, Valencia, España. Electronic address: mazonmig@gmail.com.
authors.forename: M
authors.initials: M
authors.lastname: Mazón
authors.affiliation: Servicio de Otorrinolaringología, Hospital General de Onteniente, Valencia, España.
authors.forename: E
authors.initials: E
authors.lastname: Pont
authors.affiliation: Área Clínica de Imagen Médica, Hospital Universitario y Politécnico La Fe, Valencia, España.
authors.forename: A
authors.initials: A
authors.lastname: Montoya-Filardi
authors.affiliation: Área Clínica de Imagen Médica, Hospital Universitario y Politécnico La Fe, Valencia, España.
authors.forename: J
authors.initials: J
authors.lastname: Carreres-Polo
authors.affiliation: Área Clínica de Imagen Médica, Hospital Universitario y Politécnico La Fe, Valencia, España.
authors.forename: F
authors.initials: F
authors.lastname: Más-Estellés
mesh_terms: D002648:Child; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans
keywords: Abnormalities; Anormalidades; Aplasia coclear; Cochlear aplasia; Cochlear displasia; Diagnostic imaging; Displasia coclear; Ear, inner; Imagen diagnóstica; Incomplete partition; Oído interno; Partición incompleta