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Goh -CONDUCTIVE HEARING LOSS FINAL Version.Pptx ASHNR 2016 ASHNR 2016 CONDUCTIVE HEARING LOSS DR JULIAN GOH DIAGNOSTIC RADIOLOGY TAN TOCK SENG HOSPITAL SINGAPORE ASHNR 2016 ASHNR 2016 Ossicles: fused / disrupted / absent / malformaon rd Dehiscence – 3 window Fluid or so ssue Vascular structures Abnormal interface with inner ear (oval / round windows) DECLARATIONS - NIL IP-III Bone, so ssue, cerumen ASHNR 2016 ASHNR 2016 IMAGING TECHNIQUES CT MSCT / CBCT • Double oblique • Wide window sengs WIDE WINDOWS MRI Pre & post contrast + DWI • Congenital cholesteatoma NARROW (BONE) • Cholesterol granulomas WINDOWS www.teachmesurgery.co • So; <ssue masses (extent, perineural spread) m ASHNR 2016 ASHNR 2016 EPITYMPANUM CHL – MIDDLE EAR PERFORATED INTACT TM TM EAC MESOTYMPANUM EUSTACHIAN NORMAL TUBE INFLAMMATION RED HYPOTYMPANUM TRAUMA WHITE BLUE ASHNR 2016 ASHNR 2016 INTACT TM - NORMAL CONGENITAL OSSICULAR CHAIN ANOMALIES • Usually in associaon with EAC anomalies • Isolated anomalies (with normal external ear) much less common1 • • Chronic effusions (chronic OME, Lack of progression, early age of onset dis<nguish from fenestral otosclerosis NPC) • Unilateral (sporadic) / bilateral (gene<c, AD) • Congenital ossicular anomalies Ossicular deformity: abnormal size, shape, • Oval window atresia orientaon • Otosclerosis Ossicular fixaon: bony bar from ossicles to middle ear wall Courtesy Berit Verbist, Leiden Courtesy Berit Verbist, Leiden Swartz JD, Harnsberger HR. Imaging of the Temporal Bone, 3rd edi<on. New York: Thieme 1998 ASHNR 2016 ASHNR 2016 OSSICULAR DEFORMITIES UNICRURAL STAPES CONGENITAL THICKENING • 2nd branchial arch dysplasia • Distal incus (long, len<cular processes) 2nd branchial • Incudostapedial joint (ISJ) arch (ReichErt’s discon<nuity carlage) • Bony deformies (hypoplas<c, fibrous band) • Stapes superstructure ASHNR 2016 ASHNR 2016 MONOPODAL/COLUMELLAR STAPES ABSENT DISTAL INCUS AbsEnt long & lEnIcular procEsses (right) Courtesy Jan Casselman Normal le side ASHNR 2016 ASHNR 2016 ABSENT LENTICULAR PROCESS LEFT INCUS ABSENT LENTICULAR PROCESS LEFT INCUS NORMAL ABSENT LENTICULAR PROCESS NORMAL ABSENT LENTICULAR PROCESS ASHNR 2016 ASHNR 2016 INCUDOSTAPEDIAL JOINT (ISJ) DISCONTINUITY Middle aged male • Acquired > Congenital Chronic right o<<s, CHL • Congenital – hypoplasia / aplasia long process with disrupted ISJ Non-cholesteatomatous • Acquired – previous cholesteatoma; trauma#; resorp<on erosion post-stapedectomy; non-cholesteatomatous erosion* • Can be seen in 15.7 % cases* Right len<cular process • Severely retracted TM; osteoclas<c resorp<on; impaired vascular erosion supply Le; len<cular process intact *Connor et al. Discon<nuity of the incudo-stapedial joint within a fully aerated middle ear and mastoid on computed tomography: A clinico-radiological study of its ae<ology and clinical consequence. Clin Radiol 2012 #Joel D Swartz, Seth Zwillenberg, Alan S Berger. Acquied Disrup<ons of the Incudostapedial Ar<culaon: Diagnosis with CT. Radiology 1989 ASHNR 2016 ASHNR 2016 OSSICULAR FIXATION • Bony bars • Calcified / ossificaon of suppor<ng ligaments, tendons • Other structures e.g. persistent stapedial artery, dehiscent facial nerve Congenital footplate/OW thickening Normal footplate – double oblique • Stapes foot plate fixaon most common FOOTPLATE – STAPES • Failure of annular ligament formaon • DDx – otosclerosis but hearing loss not progressive PATHOLOGY Congenital closed footplate/OW Courtesy Jan Casselman ASHNR 2016 ASHNR 2016 OSSIFIED STAPEDIUS TENDON INCUDOMALLEAL FUSION (NORMAL EXTERNAL EAR) ASHNR 2016 ASHNR 2016 OSSIFIED MEDIAL INCUDOMALLEAL MALLEAL BAR LIGAMENT ASHNR 2016 ASHNR 2016 OTOSCLEROSIS • AD, variable penetrance; 3rd – 4th decades; F:M = 2:1 ANTERIOR MALLEAL BAR • CHL + SNHL; bilateral (70%) > unilateral • Fenestral (85-90%) & retrofenestral / cochlear (10-15%) • Early spongioc (ly<c) phase followed by late sclero<c phase • Fissula ante fenestrum, oval window; pericochlear • CT. MRI – increased T2, enhancement. • Differen<als: • Paget’s; osteogenesis imperfecta; syphilis; fibrous dysplasia PAGET’S FIBROUS DYSPLASIA MRI of cochlear otosclerosis. J Goh, Chan LL, Tan TY. BJR 2002 Courtesy Berit Verbist, Valvassori. Imaging of otosclerosis. Otolaryngologic Clinics of North America 1993 Leiden ASHNR 2016 ASHNR 2016 COCHLEAR CLEFT WHY IMAGE? • Other causes of unilateral CHL • Surgical issues: • Extent (cochlear implants) • Round window involvement • Oval window obliteraon • Facial nerve posi<on • Ossicular status • Malleus fixaon • Incus defects (inflammatory erosions; discon<nuity) • Chronic o<<s media • PIOF (tympanosclerosis) • Gusher anomaly (e.g. IP – III) ASHNR 2016 ASHNR 2016 OVAL WINDOW ATRESIA • Stapes blastema fails to contact oc capsule ! failed oval window development • Displaced VII nerve RETROFENESTRAL; ROUND FENESTRAL WINDOW INVOLVEMENT ASHNR 2016 ASHNR 2016 Axial image showing incudostapedial joint and OVAL WINDOW ATRESIA posteriorly angulated incus and stapes. Stapes fused to • CT pyramidal eminence and • Atre<c window (coronal plane) facial nerve canal • Displaced facial nerve • Stapes abnormali<es • Surgical issues: AtrEIc oval windoW; displacEd VII Coronal image showing • Facial nerve posi<on nerVE absent oval window and • Presence / absence of stapes superstructure inferiorly displaced facial nerve Zeifer B. Sabini P, Sonne J. Congenital absence of the oval window: Radiologic diagnosis and associayed anomalies. AJNR 2000; 21 : 322-327 Booth TN, Vezina LG, Gerald Karcher, Dubovsky EC. Imaging and clinical evaluaon of isolated atresia of the oval window. AJNR 2000; 21 : 171-174 Lambert PR. Congenital absence of the oval window. Laryngoscope 1990; 100: 37 – 40 Jahrsdoerfer RA. Congenital absence of the oval window. ORL J Otorhinolaryngol Relat Spec 1977; 84: 904 – 914 ASHNR 2016 ASHNR 2016 MIDDLE EAR – WHITE TM CONGENITAL CHOLESTEATOMA • Young paent; normal middle ear & mastoid pneumasaon; white avascular mass. No h/o inflammaon or dischargE • Tympanosclerosis • Posi<on: meso/hypotympanum; medial to ossicles • Theories: • Congenital • Congenital ectodermal rest • Failure of regression of epidermoid cholesteatoma inclusion • 30% have CHL • Associaon with EAC dysplasia ASHNR 2016 ASHNR 2016 CONGENITAL CHOLESTEATOMA CONGENITAL CHOLESTEATOMA ASHNR 2016 ASHNR 2016 FACIAL NERVE SCHWANNOMA Locaons: CPA – IAC; Temporal bone (geniculate, tympanic, mastoid) AtyPical VII n WEaknEss/ twitching + CHL • Tubular enlargement facial nerve canal • Smooth, scalloped borders • MRI: intense enhancement post- Gd De Foer et al. Neuroradiology 2010 ASHNR 2016 ASHNR 2016 LEFT JACOBSON’S SCHWANNOMA MIDDLE EAR – BLUE TM • Cholesterol granuloma • Dehiscent jugular bulb • “Glue ear” Courtesy Drs Raymond Ngo, Amit Karandikar ASHNR 2016 ASHNR 2016 CHOLESTEROL GRANULOMA CHOLESTEROL GRANULOMA • Chronic inflammaon • Obstruc<on – vacuum hypothesis • Anywhere in pneumased temporal • bone (middle ear/mastoid; petrous Obstruc<on primary cause. apex) • Middle ear: Eustachian tube dysfunc<on ! repeated obstruc<on & reduced pressure ! repeated haemorrhage & granulaon <ssue • Expansile middle ear lesion, • Petrous apex: obstruc<on of channels from petrous apex cells blue drum • • Exposed marrow theory M = F. Younger – middle-aged • Hyperplas<c mucosa invades bone, exposes marrow ! repeated adults; late symptom onset haemorrhage • Tinnitus, + CHL • • DDx: glomus; dehiscent jugular Trapped blood, chronic inflammaon. Cholesterol crystals in fluid bulb; “glue ear” • Foreign body reac<on with fibrosis, vessel proliferaon. ASHNR 2016 ASHNR 2016 CHOLESTEROL GRANULOMA CHOLESTEROL GRANULOMA • CT • Mimics granulaon <ssue • Expansile, smooth scalloped borders + ossicular erosion T1 T2 • MR • high T1 prE-contrast (methaemaglobin), high T2. • No real enhancement T1 + C DWI ASHNR 2016 ASHNR 2016 CHOLESTEROL GRANULOMA ASHNR 2016 ASHNR 2016 DEHISCENT JUGULAR BULB RED TM • Trauma • Vascular anomalies • Aberrant ICA • Persistent stapedial artery • Paragangliomas PULSATILE TINNITUS ASHNR 2016 ASHNR 2016 PARAGANGLIOMAS PARAGANGLIOMAS CT • Benign, highly vascular. From extra-adrenal neuroendocrine system. F > • Isoaenuang so; <ssue masses (NECT), intense enhancement (CECT). Permeave bone changes M; 2/3 in 4th to 6th decades • Sporadic / familial (AD, variable penetrance). MRI • Pulsale nnitus, CHL, ver<go + cranial nerve palsies. Red TM • Low T1, high T2, intense enhancement. “Salt & pepper” appearance (haemorrhage). Flow voids (lesions > 2cm) • CHL – Engulf (not ErodE) ossicular chain • Types: AngiograPhy • Jugular – Arnold’s nerve, jugular foramen (permeave erosion of bony margins) • Intense tumour blush, early draining veins • Tympanic – Jacobson’s nerve, cochlear promontory • Jugulotympanic Scingraphy • Indium-111 octreo<de accumulaon ASHNR 2016 ASHNR 2016 Large tympanic paragaglioma growing along Eustachian tube and EAC; “salt & pepper” Courtesy Dr Wendy Smoker ASHNR 2016 ASHNR 2016 CT images in the (A) coronal and (B) axial planes showing a jugulo- tympanicum lesion (red arrows) engulfing, but not eroding, the ossicles ASHNR 2016 ASHNR 2016 ABERRANT ICA Aberrant ICA on promontory. ‘Steno<c’ segment at entry into horizontal canal ASHNR 2016 ASHNR 2016 INNER EAR –SEMICIRCULAR CANAL DEHISCENCE PERSISTENT STAPEDIAL ARTERY (SCD) • “3rd window”. Superior >> posterior • Pseudo-CHL (bone thresholds high) • Autophony; <nnitus; hyperacusis; ves<bular symptoms (Tullio’s phenomenon – sound-induced ver<go + nystagmus) • Focal dehiscence on CT + ves<bular symptoms => semicircular canal dehiscence ASHNR 2016 ASHNR 2016 SEMICIRCULAR CANAL DEHISCENCE (SCD) • Usually unilateral. • Incidence ~ 2%. 2.1% local study (10/481),
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