The Temporal Bone Anatomy & Pathology

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Department of Radiology University of California San Diego The Temporal Bone Anatomy & Pathology John R. Hesselink, M.D.

Temporal Bone Axial View

Temporal Bone Coronal View

Longitudinal Fracture

The Temporal Bone Longitudinal fractures Most common fracture (80%) Pass through EAC, mastoid & middle ear High incidence of ossicular derangement Inner ear usually spared Facial paralysis in about 15% CSF otorrhea or rhinorrhea

History: 40 y/o man with hearing loss following a 40 foot fall Dx: Transverse fracture 525

The Temporal Bone Transverse Fractures Commonly involve the inner ear Cochlear fracture - sensorineural hearing loss Labyrinthine fracture - severe vertigo Facial palsy in 50% Perilymph fistula Injury to carotid artery

Diseases of the Mastoid and Middle Ear Acute otomastoiditis Chronic otomastoiditis Acquired cholesteatoma Paraganglioma Glomus jugulare Glomus tympanicum

Temporal Bone Inflammation Otitis Media / Mastoiditis

History: 5 month old girl with left ear pain Dx: Otomastoiditis and external otitis 384

History: 24 y/o man with a left conductive hearing loss 665 Dx: Cholesteatoma

Acquired Cholesteatoma Critical Imaging Findings Erosion of scutum and ossicles Integrity of lateral semicircular canal Facial nerve involvement Erosion through tegmen or sigmoid sinus plate

History: 33 y/o male with left ear deafness 177

History: 48 y/o woman with sensorineural hearing loss 772-07406796

{Page 2}

{Page 3} Dx: Glomus jugulare (paraganglioma)

Paragangliomas Origin: paraganglia along CN 9 and 10 Glomus jugulare - Nerve of Arnold (X) Glomus tympanicum - Jacobson's nerve (IX) Presentation: pulsatile tinnitus, conductive hearing loss, retrotympanic mass, and cranial nerve deficits Biological behavior - slow growing but locally invasive

Location of Glomus Tissue

History: 60 y/o man with polycystic kidney disease, tinnitus, hoarseness & paretic right vocal cord 409

{Page 2} Dx: Glomus jugulare

Aberrant Carotid Artery Q 49-53

Aberrant Carotid Artery B 5,6

History: 13 y/o girl with pulsatile tinnitus 411

{Page 2} Dx: High dehiscent jugular bulb

Middle Ear Masses Differential Diagnosis Paraganglioma High & exposed jugular bulb Aberrant carotid artery Persistent stapedial artery Schwannoma Primary & metastatic bone tumors Other middle ear tumors - cholesteatoma, squamous cell carcinoma

Diseases of the Inner Ear Labyrinthitis Otosclerosis Acoustic schwannoma Endolymphatic sac tumors

Dr. Harris "The ringing in your ears - - I think I can help."

History: 55 y/o woman with acute hearing loss in the right ear 10 days ago Dx: Viral labyrinthitis on steroids for 8 days 619

Labyrinthitis Presentation: sensorineural hearing loss and vertigo Viral most common, but other agents possible Source: tympanic, meningeal, hematogenous, posttraumatic or postsurgical Imaging features: Acute & subacute: enhancement with gadolinium Chronic: fibrosis and ossification (labyrinthitis ossificans)

Otosclerosis Acute phase: otospongiosis - resorption of bone Chronic phase: otosclerosis - sclerosis of bone Resorption of enchondral bone around otic capsule & replacement with spongy vascular new bone 65% females & 80% of cases are bilateral Onset during the 2nd and 3rd decades Two types: Fenestral & Cochlear

History: 58 y/o woman with progressive bilateral hearing loss for 8 years 699

{Page 2} Dx: Cochlear otosclerosis

Retrofenestral Otosclerosis (Cochlear Otospongiosis) Sensorineural hearing loss CT findings: Acute phase - patchy band-like lucency around the cochlear Chronic phase - no visible abnormalities Cochlear implantation does not work

History: 58 y/o woman with vertigo & hearing loss 700

{Page 2} Dx: Fenestral otosclerosis

Fenestral Otosclerosis Most common form Conductive hearing loss Fixation of the foot plate of the stapes CT findings: lucent focus just anterior to oval window dense bone encroaches on oval window Surgical correction possible

History: Right sensorineural hearing loss & facial palsy Courtesy Mahmood Mafee,

Causes of Sensorineural Hearing Loss Vestibular Schwannoma Other CP angle & petrous tumors Brain stem lesions Labyrinthitis Cochlear otosclerosis Bone dysplasias Leptomeningeal processes Congenital Trauma (transverse fx's)

Diseases of the Petrous Apex Cholesterol granuloma (cyst) Primary cholesteatoma Petrous apicitis (Gradenigo's syndrome) Intrapetrous carotid artery aneurysm Chondrosarcoma Metastatic tumors

Diseases of the Petrous Apex Cholesterol Granuloma Chronic inflammatory cysts Contain blood products & cholesterol crystals Cyst capsule lined by fibrous tissue Hyperintense on T1 & T2-weighted images

Diseases of the Petrous Apex Primary Cholesteatoma Also called epidermoid tumor or cyst Arise from epithelial rests Capsule lined by stratified squamous epithelial Cyst contents are primarily desquamated keratin Usually high T2 signal & low T1 signal

Cholesterol Granuloma 568

Temporal Bone Inflammation Petrous Apicitis (Gradinego's Syndrome) Schwartz & Harnsberger, Imaging of the Temporal Bone, Thieme, 1992, p. 334

History: 62 y/o female with right ear deafness & pain 596

Dx: Multiple myeloma MGUS for several years {Page 2}

History: 60 y/o woman with vertigo, left sensorineural hearing loss & left facial palsy 787-26551986

Dx: AML - Chloroma {Page 2}

The Facial Nerve Normal Anatomy Schuknecht HF, Pathology of the Ear, Lea & Febiger, 1993, pp45-66

The Facial Nerve Inflammatory Disease Bell's palsy Ramsey Hunt syndrome Herpes zoster oticus Lyme disease Syphilis Imaging - gadolinium enhancement of the 7th nerve

History: 37 HIV + male with 7th nerve deficits Dx: Bell's palsy 414

History: 81 y/o woman with chronic lymphocytic leukemia and a right 7 th nerve palsy Dx: Bell s palsy Herpes zoster 711

Facial Nerve Tumors Differential Diagnosis Schwannoma Hemangioma Epidermoid tumor Acquired cholesteatoma Tympanic segment - glomus tympanicum, cholesteatoma, persistent stapedial artery Stylomastoid segment - glomus jugulare

PD 15-44 Facial Nerve Schwannoma

History: 21 y/o woman with progressive left facial weakness & hearing loss for 11 months 670

Dx: Plexiform neurofibroma {Page 2}

Congenital Anomalies External & Middle Ear Associated with facial, cervical & skeletal dysplasias External ear deformities Conductive hearing loss More common anomalies Atresia of external ear First branchial arch dysplasia Second branchial arch dysplasia

Atresia of External Auditory Canal Critical Imaging Information Thickness of atresia plate Size of tympanic cavity Status of the ossicles and oval window Presence of any congenital cholesteatoma

Atresia of External Auditory Canal PB 8-10

Membranous Labyrinth Superior semicircular duct Superior ampulla Saccule Utricle Lateral ampulla Lateral semicircular duct Cochlear duct Endolymphatic duct Endolymphatic sac Ductus reuniens Utriculosaccular duct Posterior semicircular duct Posterior ampulla Swartz et al, AJNR 17:17-21, 1996

Inner Ear Anatomy Som & Curtin, Head & Neck Imaging, Mosby, 1996, p1412

Inner Ear Anomalies Semicircular canals & vestibule Cochlear anomalies Labyrinthine aplasia (Michel's deformity) Incomplete partition (Mondini malformation) Atresia/stenosis of the internal auditory canal Perilymphatic hydrops

Cochlear Aplasia with Labyrinthine Dysgenesis Hasso et al., in Som & Curtin, Head & Neck Imaging, Mosby, 1996, p1367

History: 17 y/o male was partially deaf in the left ear Dx: 192

Perilymphatic Hydrops Increased pressure in the inner ear due to CSF fistula Can lead to perforation of the stapes foot plate Etiology: Defective lamina cribrosa Congenitally wide cochlear aqueduct Trauma most common

History: 27 y/o man with deaf right ear & progressive sensorineural hearing loss in left ear 625-1183581

{Page 2} Dx: Endolymphatic hydrops Vestibular aqueduct syndrome

Endolymphatic Hydrops Vestibular Aqueduct Syndrome Sensorineural hearing loss & vertigo Deficient absorption of endolymph Can be congenital, acquired, or idiopathic Meniere's disease: Idiopathic form Imaging: Dilated endolymphatic sac and vestibular aqueduct < 1.5 mm No larger than semicircular canal

History: 77 y/o man developed vertigo with loud noises Dx: Dehiscence SSC 651

Superior Canal Dehiscence Syndrome Caused by dehiscence of bone overlying the superior semicircular canal Causes vertigo & oscillopsia with sound or pressure stimuli Diagnosed with thin-section coronal CT Rx: Surgical plugging of the SSC