malformations de l`oreille interne

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MALFORMATIONS DE L'OREILLE INTERNE Rappel embryologique (Pearson, 1979) • • •

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Neurectoderme : v•sicule otique, labyrinthe membraneux MÄsoblaste : labyrinthe osseux, platine •trier, espaces p•ri lymphatiques I, IIes poches entoblastiques : trompe eustache, cavit• tympanique, attique, cavit•s post. Ier arc branchial : marteau (t‚te, col), enclume (corps, courte apophyse) IIiÅme arc branchial : •trier, longue apophyse enclume

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MALFORMATIONS DU LABYRINTHE OSSEUX CLASSIFICATION (Jackler. 1987) I - CochlÄe absente ou mal formÄe – Aplasie labyrinthique compl„te (type Michel) – Aplasie cochl•aire – Hypoplasie cochl•aire – Cochl•e mal form•e (Mondini) – V•sicule unique : cochl•e et vestibule fusionn•s canaux normaux ou mal form•s II - Malformations avec cochlÄe normale – Dysplasie vestibule + C.S. externe – Dilatation de l'aqueduc du vestibule, canaux normaux, vestibule normal ou dilat•

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DILATATION AQUEDUC VESTIBULE

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DYSPLASIE CSL - VESTIBULE

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DYSPLASIE CSL - VESTIBULE

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Syndrome de DI GEORGE( d•l•tion 22q 11)

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Syndrome de CHARGE

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Syndrome de charge • • • • •

C:colobome H:heart R: retard mental G:genito-urinaire E:ear; ag•n•sie des canaux semi-circulaires

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Malformations de la cochl•e

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OTOCYSTE

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SURDITE LIEE A L’X DFN3 PARTITION INCOMPLETE TYPE I, ABSENCE DE MODIOLUS

SURDITE DE TRANSMISSION DE OI, OREILLE GEYSER

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PARTITION INCOMPLETE TYPE II

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PARTITION INCOMPLETE TYPE II

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MONDINI DILATATION AQUEDUC VESTIBULE PARTITION INCOMPLETE TYPE II

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Bilan d’imagerie • MALFORMATION OREILLE INTERNE • IMPLANTATION : – OREILLE INTERNE (liquides, aplasie) – OREILLE GEYSER – NERF COCHLEAIRE : • St•nose du CAI 1,5 mm  CAI / Lamina Cribosa : modiolus absent - orifice nerf vestibulaire sup•rieur - canal du facial Dysplasie labyrinthique sÄvÅre (Phelps, 1986) - cochl•e mal form•e sans tour basal - dilatation du vestibule +/- CSC externe - d•fect platine de l'•trier

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DILATATION AQUEDUC DE COCHLEE STENOSE CAI

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M•at acoustique interne • Taille nerf cochl•aire > nerf vestibulaire sup. ou inf. • Nerf cochl•aire > ou = nerf facial (64%) • Sym•trie D/G (70%)

ht ht avant

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CISS 3D

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Ag•n•sie ou hypoplasie du nerf cochl•aire • Aplasie ou hypoplasie du nerf cochl•aire : – avec st•nose CAI (2 cas); anomalies labyrinthe (2 cas) – sans anomalies CAI ou labyrinthe (3cas) Casselman, Radiology, 1997.

• Aplasie ou hypoplasie du nerf cochl•aire : – anomalies labyrinthiques minimes : lamina cribosa ferm•e cochl•e isol•e (=hypoplasie du canal cochl•aire) – modiolus d•ficient (5cas), large aqueduc du vestibule – st•nose CAI (11/12 cas) Glastonbury, AJNR, 2002.

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IMPLANTS COCHLEAIRES SURDITE NEUROSENSORIELLE ET TDM

Normal Common cavity deformity Mondini Mondini and dilated vestibular aqueduct Dilated vestibular aqueduc Narrow IAM Abnormal vestibule/semicircular canals Abnormal cochlea, vestibule, and IAM Cerebral abnormalities Miscellaneous Bamiou, Arch Dis Child, 2000.

71.6 % 0.9 2.6 3.4 5.1 2.6 6.0 4.3 0.9 2.6

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MAR… OD

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MAR…OG

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DHO…OD

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DHO…OG

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AUL..OD

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AUL..OG

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IMPLANTS COCHLEAIRES IMAGERIE 1 - Imaging for cochlear implants

: PHELPS. P, J. Laryngol. Otol. Suppl. 1999 ; 24 : 21-3 CT : malformation OI (20%), labyrinthite ossifiante

2 - Preoperative cochlear implant imaging : is MRI enough ? ELLUL. S, Am. J. Otol. 2000 ; 21 : 528-33 – Perm•abilit• du tour basal : FSE - MRI = CT – Malformations OI IRM > CT – Pr•sence du nerf cochl•aire IRM > CT

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IMPLANTS COCHLEAIRES OSSIFICATION COCHLEAIRE • POST MENINGITIQUE – – – – – –

Si„ge : tour basal, fen‚tre ronde Fibrose : labyrinthite scl•rosante Ossification : labyrinthite ossifiante (73 %) M•ningite virale ou bact•rienne Bilat•rale ou unilat•rale asym•trique D•lai d'ossification (0,5  16 ans) YUNE, 1991

• AUTRES : traumatisme, ostospongiose.....

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LABYRINTHITE OSSIFIANTE

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LABYRINTHITE OSSIFIANTE

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LABYRINTHITE OSSIFIANTE