title: Mondini deformity in a case of Turner syndrome. A radiological finding.
abstract: Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old woman with TS who presented progressive sensorineural hearing loss. The computed tomography of the ears showed bilateral Mondini deformity.
authors.affiliation: Servicio de Otorrinolaringología, Hospital de Santa María de Lleida, Lleida, España. ebodet@hotmail.com
authors.forename: Eduard
authors.initials: E
authors.lastname: Bodet Agustí
authors.forename: Xavier
authors.initials: X
authors.lastname: Galido Ortego
authors.forename: Fares
authors.initials: F
authors.lastname: Ghani Martínez
authors.forename: Begoña
authors.initials: B
authors.lastname: García González
authors.forename: Montserrat
authors.initials: M
authors.lastname: Borràs Perera
authors.forename: Angel
authors.initials: A
authors.lastname: Seara Gil
mesh_terms: D000328:Adult; D001301:Audiometry, Pure-Tone; D003051:Cochlea / Q000002:abnormalities* / Q000000981:diagnostic imaging; D018450:Disease Progression; D005260:Female; D006310:Hearing Aids; D006312:Hearing Loss, Bilateral / Q000175:diagnosis / Q000209:etiology*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008876:Middle Ear Ventilation; D010033:Otitis Media / Q000150:complications / Q000601:surgery; D012008:Recurrence; D012665:Semicircular Canals / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014424:Turner Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D014722:Vestibule, Labyrinth / Q000002:abnormalities* / Q000000981:diagnostic imaging