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"Perez-Girbes" "email" => array:1 [ 0 => "aperezgirbes@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Miguel" "apellidos" => "Mazón" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Elena" "apellidos" => "Pont" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Radiología, Hospital Universitari i Politècnic La Fe, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Otorrinolaringología, Hospital de Manises, Manises, Valencia, Spain" "etiqueta" => "b" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Alagille: hipoplasia de canales semicirculares posteriores" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2480 "Ancho" => 2500 "Tamanyo" => 518589 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 22-year-old woman diagnosed with Alagille's syndrome in infancy presenting with hearing loss and tinnitus. No disorders were found on physical examination or audiometry.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Temporal bone CT scan of both ears showed a defect in the development of the posterior semicircular canal (pSCC) (arrows in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), stenosis of the superior semicircular canal (sSCC) (tips of the arrow in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B) and normality of the lateral semicircular canal (lSCC) (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C). MRI confirmed the findings; volumetric reconstruction of the T2- weighted high-resolution sequences (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D) showed the pSCC defect (arrow) and stenosis of the sSCC (arrow tip), the remainder of the labyrinth was normal.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Isolated malformations of the semicircular canals can present as a single disorder or associated with a systemic syndrome. In both cases, lSCC involvement is practically constant since it is the last to form.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Alagille's syndrome is a multi-organ disorder secondary to a mutation of the <span class="elsevierStyleItalic">JAG1</span> gene. The otologic manifestations include chronic otitis media, malformations of the temporal bone and deafness. The patient we describe had bilateral hypoplasia of the pSCC (incomplete development with blind ending), stenosis of the sSCC and the remainder of the labyrinth was normal.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Isolated anomalies of the pSCC and sSCC with no involvement of the lSCC are exceptional findings and have only been described in Waardenburg syndrome type II and Alagille's syndrome. The development of radiological techniques has resulted in images with high spatial resolution that enable the detection of small congenital anomalies of the ear.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Perez-Girbes A, Mazón M, Pont E. Síndrome de Alagille: hipoplasia de canales semicirculares posteriores. Acta Otorrinolaringol Esp. 2018;69:58–59.</p>" ] ] "multimedia" => array:1 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2480 "Ancho" => 2500 "Tamanyo" => 518589 ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/21735735/0000006900000001/v1_201802131610/S2173573517301230/v1_201802131610/en/main.assets" "Apartado" => array:4 [ "identificador" => "5869" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images in otorhinolaryngology" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/21735735/0000006900000001/v1_201802131610/S2173573517301230/v1_201802131610/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173573517301230?idApp=UINPBA00004N" ]
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Images in Otorhinolaryngology
Alagille's Syndrome: Hypoplasia of Posterior Semicircular Canals
Síndrome de Alagille: hipoplasia de canales semicirculares posteriores