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Bilateral Retrofenestral Otospongiosis

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This is a young adult who presented with mixed

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conductive and sensorineural hearing loss.

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Otospongiosis generally occurs in young adults,

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sometimes teenagers.

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And it is a phenomenon that can lead to both

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conductive, as well as sensorineural, as well

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as mixed hearing loss. In this example,

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what we see is this stapes here

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coming to the oval window here.

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And we have this large plaque of lucent bone in

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the fistula ante fenestram. This is the vestibule.

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This is a portion of the stapes here.

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This is the incudostapedial joint, so stapes.

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And we have this large plaque at the fistula

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ante fenestram of demineralized spongiotic bone.

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And not only that,

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but you see that halo that I described

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previously around the cochlea.

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So this patient has both the cochlear form of

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otospongiosis, as well as the fenestral

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form of otospongiosis.

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The latter leading to fixation of the stapes

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and a conductive hearing loss.

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And then the cochlear form more likely to have

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sensorineural hearing loss, secondary to the

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enzymatic degradation of those fine organs of...

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fine neuroepithelium of the organs of Corti.

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Let's just scan,

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pan through this a little bit more.

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So, again, vestibule, big plaque of otospongiosis.

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Here we have the cochlea with peripheral area here

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that's abnormal in cochlear otospongiosis.

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Here's a little halo around the

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apical and middle turn of it.

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See the little halo around the cochlea from

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the cochlear form comes even to here.

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What was sort of interesting in this

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case was as I was scrolling upward,

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you could even see a plaque of otospongiosis

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around the superior semicircular canal.

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So this is the superior semicircular canal and

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this limb has demineralized bone around it,

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going all the way down to the fenestral

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and cochlear form.

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So this is a diffuse process in this patient

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affecting the inner ear structures.

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Let me just quickly show you the contralateral

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side, which was mostly cochlear form.

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So here we have the cochlea basal turn

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and middle turn and apical turn here.

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And you see this plaque of demineralization

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around the cochlea,

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around the cochlea as the cochlear

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form of otospongiosis.

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So, little bit of portion here in the fenestral form as well.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Temporal bone

Non-infectious Inflammatory

Neuroradiology

Infectious

Idiopathic

Head and Neck

CT

Brain

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