Labyrinthitis ossificans
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Labyrinthitis ossificans, also known as labyrinthine ossification, represents pathological ossification of the membranous labyrinth as a response to an insult to the inner ear.
Clinical presentation
It is usually associated with profound sensorineural hearing loss, and may sometimes be associated with dizziness and/or vertigo. It is most common cause of acquired sensorineural hearing loss in children.
Pathology
The disorder is most commonly the end result of prior suppurative labyrinthitis, either related to otomastoiditis (tympanogenic etiology) or meningitis (meningogenic etiology) 1,5,6,7-7. Other causes include temporal bone surgery or trauma 1,5, autoimmune inner ear disease 9,10, and sickle cell disease 5.
Radiographic features
CT
High-density bone deposition within the membranous labyrinth:
- mild disease: hazy increase in density within fluid spaces of the membranous labyrinth
- moderate disease: focal areas of bony encroachment on fluid spaces of the membranous labyrinth
- severe disease: membranous labyrinth completely obliterated by bone replacing fluid spaces
MRI
- It is more sensitive in diagnosing early stage of disease in which fibrous tissue is present
. Loss -
loss of normal high signal of fluid within the membranous labyrinth is seen on heavily T2 weighted images (as low signal intensity foci in the labyrinth)
. -
Laterallateral semicircular canal and basal turn of cochlea are earliest to be involved.
Treatment and prognosis
It may complicate or preclude cochlear implantation. It is considered to be a relative contraindication for Cochlear implant.
Differential diagnosis
Possible imaging differential considerations include:
- labyrinthine aplasia or cochlear aplasia
- retrofenestral/cochlear otosclerosis
-<p><strong>Labyrinthitis ossificans</strong>, also known as <strong>labyrinthine ossification</strong>, represents pathological ossification of the <a href="/articles/membranous-labyrinth">membranous labyrinth </a>as a response to an insult to the <a href="/articles/inner-ear">inner ear</a>.</p><h4>Clinical presentation</h4><p>It is usually associated with profound <a href="/articles/sensorineural-hearing-loss">sensorineural hearing loss</a>, and may sometimes be associated with dizziness and/or vertigo. It is most common cause of acquired sensorineural hearing loss in children.</p><h4>Pathology</h4><p>The disorder is most commonly the end result of prior suppurative labyrinthitis, either related to <a href="/articles/chronic-otomastoiditis">otomastoiditis</a> (tympanogenic etiology) or <a href="/articles/leptomeningitis">meningitis</a> (meningogenic etiology) <sup>1,</sup><sup>5,6,7</sup>. Other causes include <a href="/articles/temporal-bone-1">temporal bone</a> surgery or <a href="/articles/temporal-bone-fracture-1">trauma</a> <sup>1,5</sup>, <a href="/articles/autoimmune-inner-ear-disease">autoimmune inner ear disease</a> <sup>9,10</sup>, and <a href="/articles/sickle-cell-disease">sickle cell disease</a> <sup>5</sup>.</p><h4>Radiographic features</h4><h5>CT</h5><p>High-density bone deposition within the membranous labyrinth:</p><ul>- +<p><strong>Labyrinthitis ossificans</strong>, also known as <strong>labyrinthine ossification</strong>, represents pathological ossification of the <a href="/articles/membranous-labyrinth">membranous labyrinth</a> as a response to an insult to the <a href="/articles/inner-ear">inner ear</a>.</p><h4>Clinical presentation</h4><p>It is usually associated with profound <a href="/articles/sensorineural-hearing-loss">sensorineural hearing loss</a>, and may sometimes be associated with dizziness and/or vertigo. It is most common cause of acquired <a href="/articles/sensorineural-hearing-loss">sensorineural hearing loss</a> in children.</p><h4>Pathology</h4><p>The disorder is most commonly the end result of prior suppurative labyrinthitis, either related to <a href="/articles/chronic-otomastoiditis">otomastoiditis</a> (tympanogenic etiology) or <a href="/articles/leptomeningitis">meningitis</a> (meningogenic etiology) <sup>1,</sup><sup>5-7</sup>. Other causes include <a href="/articles/temporal-bone-1">temporal bone</a> surgery or <a href="/articles/temporal-bone-fracture-1">trauma</a> <sup>1,5</sup>, <a href="/articles/autoimmune-inner-ear-disease">autoimmune inner ear disease</a> <sup>9,10</sup>, and <a href="/articles/sickle-cell-disease">sickle cell disease</a> <sup>5</sup>.</p><h4>Radiographic features</h4><h5>CT</h5><p>High-density bone deposition within the membranous labyrinth:</p><ul>
-<li>It is more sensitive in diagnosing early stage of disease in which fibrous tissue is present. Loss of normal high signal of fluid within the membranous labyrinth is seen on heavily T2 weighted images (as low signal intensity foci in the labyrinth).</li>-<li>Lateral semicircular canal and basal turn of cochlea are earliest to be involved.</li>-</ul><h4>Treatment and prognosis</h4><p>It may complicate or preclude <a href="/articles/cochlear-implant">cochlear implantation</a>. It is considered to be relative contraindication for Cochlear implant.</p><h4>Differential diagnosis</h4><p>Possible imaging differential considerations include:</p><ul>- +<li>It is more sensitive in diagnosing early stage of disease in which fibrous tissue is present</li>
- +<li>loss of normal high signal of fluid within the membranous labyrinth is seen on heavily T2 weighted images (as low signal intensity foci in the labyrinth)</li>
- +<li>lateral semicircular canal and basal turn of cochlea are earliest to be involved</li>
- +</ul><h4>Treatment and prognosis</h4><p>It may complicate or preclude <a href="/articles/cochlear-implant">cochlear implantation</a>. It is considered to be a relative contraindication for <a title="Cochlear implant" href="/articles/cochlear-implant">Cochlear implant</a>.</p><h4>Differential diagnosis</h4><p>Possible imaging differential considerations include:</p><ul>
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