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<meta name="DC.Title" content="Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency" />
<meta name="DC.Identifier" content="10.3174/ajnr.A2178" />
<meta name="DC.Date" content="2010-11-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
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<meta name="DC.Description" content="BACKGROUND AND PURPOSE: Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD.

MATERIALS AND METHODS: Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests.

RESULTS: CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves ( P = .01) or unilateral CND ( P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND ( P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND ( P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P &lt; .001).

CONCLUSIONS: Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

A
:   anterior
ABI
:   auditory brain stem implantation
ANSD
:   auditory neuropathy spectrum disorder
BCNC
:   bony cochlear nerve canal (cochlear aperture)
CISS
:   constructive interference in steady state
CNVIII
:   vestibulocochlear nerve (eighth cranial nerve)
CND
:   cochlear nerve deficiency
FA
:   flip angle
IAC
:   internal auditory canal
MCP
:   middle cerebellar peduncle
P
:   posterior
PVL
:   periventricular leukomalacia
SCC
:   semicircular canal
SNHL
:   sensorineural hearing loss
WM
:   white matter" />
<meta name="DC.Contributor" content="B.Y. Huang" />
<meta name="DC.Contributor" content="J.P. Roche" />
<meta name="DC.Contributor" content="C.A. Buchman" />
<meta name="DC.Contributor" content="M. Castillo" />
<meta name="article:published_time" content="2010-11-01" />
<meta name="article:section" content="Pediatrics" />
<meta name="citation_title" content="Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;BACKGROUND AND PURPOSE:&lt;/h3&gt;
&lt;p&gt;Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD.&lt;/p&gt;&lt;h3&gt;MATERIALS AND METHODS:&lt;/h3&gt;
&lt;p&gt;Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests.&lt;/p&gt;&lt;h3&gt;RESULTS:&lt;/h3&gt;
&lt;p&gt;CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; &lt;i&gt;P&lt;/i&gt; = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves (&lt;i&gt;P&lt;/i&gt; = .01) or unilateral CND (&lt;i&gt;P&lt;/i&gt; = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND (&lt;i&gt;P&lt;/i&gt; ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND (&lt;i&gt;P&lt;/i&gt; = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both &lt;i&gt;P&lt;/i&gt; &amp;lt; .001).&lt;/p&gt;&lt;h3&gt;CONCLUSIONS:&lt;/h3&gt;
&lt;p&gt;Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.&lt;/p&gt;" />
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<meta name="twitter:title" content="Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency" />
<meta name="twitter:card" content="summary_large_image" />
<meta name="twitter:image" content="http://ajnr-stage2.stage.highwire.org/sites/default/files/highwire/ajnr/31/10.cover.gif" />
<meta name="twitter:description" content="BACKGROUND AND PURPOSE: Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD.

MATERIALS AND METHODS: Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests.

RESULTS: CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves ( P = .01) or unilateral CND ( P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND ( P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND ( P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P &lt; .001).

CONCLUSIONS: Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

A
:   anterior
ABI
:   auditory brain stem implantation
ANSD
:   auditory neuropathy spectrum disorder
BCNC
:   bony cochlear nerve canal (cochlear aperture)
CISS
:   constructive interference in steady state
CNVIII
:   vestibulocochlear nerve (eighth cranial nerve)
CND
:   cochlear nerve deficiency
FA
:   flip angle
IAC
:   internal auditory canal
MCP
:   middle cerebellar peduncle
P
:   posterior
PVL
:   periventricular leukomalacia
SCC
:   semicircular canal
SNHL
:   sensorineural hearing loss
WM
:   white matter" />
<meta name="og-title" property="og:title" content="Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency" />
<meta name="og-url" property="og:url" content="http://ajnr-stage2.stage.highwire.org/content/31/10/1972" />
<meta name="og-site-name" property="og:site_name" content="American Journal of Neuroradiology" />
<meta name="og-description" property="og:description" content="BACKGROUND AND PURPOSE: Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD.

MATERIALS AND METHODS: Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests.

RESULTS: CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves ( P = .01) or unilateral CND ( P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND ( P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND ( P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P &lt; .001).

CONCLUSIONS: Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

A
:   anterior
ABI
:   auditory brain stem implantation
ANSD
:   auditory neuropathy spectrum disorder
BCNC
:   bony cochlear nerve canal (cochlear aperture)
CISS
:   constructive interference in steady state
CNVIII
:   vestibulocochlear nerve (eighth cranial nerve)
CND
:   cochlear nerve deficiency
FA
:   flip angle
IAC
:   internal auditory canal
MCP
:   middle cerebellar peduncle
P
:   posterior
PVL
:   periventricular leukomalacia
SCC
:   semicircular canal
SNHL
:   sensorineural hearing loss
WM
:   white matter" />
<meta name="og-type" property="og:type" content="article" />
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      <h1 class="highwire-cite-title" id="page-title">Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">B.Y. Huang</span>, <span class="highwire-citation-author" data-delta="1">J.P. Roche</span>, <span class="highwire-citation-author" data-delta="2">C.A. Buchman</span> and <span class="highwire-citation-author" data-delta="3">M. Castillo</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">November 2010,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">31 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1972-1979; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A2178 </span></div>
  
  
  
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<p id="p-1"><strong>BACKGROUND AND PURPOSE:</strong> Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD.</p>
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<p id="p-2"><strong>MATERIALS AND METHODS:</strong> Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests.</p>
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<p id="p-3"><strong>RESULTS:</strong> CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; <em>P</em> = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves (<em>P</em> = .01) or unilateral CND (<em>P</em> = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND (<em>P</em> ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND (<em>P</em> = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both <em>P</em> &lt; .001).</p>
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<p id="p-4"><strong>CONCLUSIONS:</strong> Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.</p>
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</div><div class="section glossary" id="glossary-1"><h2>Abbreviations</h2><dl id="def-list-1"><dt id="def-item-1">A<span class="def-item-dt-sep"></span></dt><dd>anterior<span class="def-item-dd-sep"></span></dd><dt id="def-item-2">ABI<span class="def-item-dt-sep"></span></dt><dd>auditory brain stem implantation<span class="def-item-dd-sep"></span></dd><dt id="def-item-3">ANSD<span class="def-item-dt-sep"></span></dt><dd>auditory neuropathy spectrum disorder<span class="def-item-dd-sep"></span></dd><dt id="def-item-4">BCNC<span class="def-item-dt-sep"></span></dt><dd>bony cochlear nerve canal (cochlear aperture)<span class="def-item-dd-sep"></span></dd><dt id="def-item-5">CISS<span class="def-item-dt-sep"></span></dt><dd>constructive interference in steady state<span class="def-item-dd-sep"></span></dd><dt id="def-item-6">CNVIII<span class="def-item-dt-sep"></span></dt><dd>vestibulocochlear nerve (eighth cranial nerve)<span class="def-item-dd-sep"></span></dd><dt id="def-item-7">CND<span class="def-item-dt-sep"></span></dt><dd>cochlear nerve deficiency<span class="def-item-dd-sep"></span></dd><dt id="def-item-8">FA<span class="def-item-dt-sep"></span></dt><dd>flip angle<span class="def-item-dd-sep"></span></dd><dt id="def-item-9">IAC<span class="def-item-dt-sep"></span></dt><dd>internal auditory canal<span class="def-item-dd-sep"></span></dd><dt id="def-item-10">MCP<span class="def-item-dt-sep"></span></dt><dd>middle cerebellar peduncle<span class="def-item-dd-sep"></span></dd><dt id="def-item-11">P<span class="def-item-dt-sep"></span></dt><dd>posterior<span class="def-item-dd-sep"></span></dd><dt id="def-item-12">PVL<span class="def-item-dt-sep"></span></dt><dd>periventricular leukomalacia<span class="def-item-dd-sep"></span></dd><dt id="def-item-13">SCC<span class="def-item-dt-sep"></span></dt><dd>semicircular canal<span class="def-item-dd-sep"></span></dd><dt id="def-item-14">SNHL<span class="def-item-dt-sep"></span></dt><dd>sensorineural hearing loss<span class="def-item-dd-sep"></span></dd><dt id="def-item-15">WM<span class="def-item-dt-sep"></span></dt><dd>white matter</dd></dl></div><ul class="copyright-statement"><li class="fn" id="copyright-statement-1">Copyright © American Society of Neuroradiology</li></ul><span class="highwire-journal-article-marker-end"></span></div><span class="related-urls"></span></div><a href="http://ajnr-stage2.stage.highwire.org/content/31/10/1972.full" class="hw-link hw-link-article-full-text" data-icon-position="" data-hide-link-title="0">View Full Text</a></div>  </div>

  
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