Congenital ossicular anomalies
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Congenital anomalies of the ossicles are most frequently associated with external ear abnormalities also, although they can occur in isolation.
They causeThese anomalies result in conductive hearing loss (CHL).
When bilateral, they are most frequently genetic, with autosomal dominant inheritance, whereas unilateral. Unilateral anomalies are usually sporadic.
Isolated ossicular anomalies can be classified as:
- class I : stapedial ankylosis
- class II : stapedial ankylosis with other associated anomalies
- class III : ossicular anomalies withouth stapedial ankylosis
-
class IV : oval
/ round/round dysplasia
They are associated with numerous syndromes including:
- Klippel-Feil syndrome
-
Wildervanck syndrome (or cervico-ocular-acoustic dysplasia)
- typically diffuse ossicular ankylosis
- Madelung dyschondro-osteosis
- otopalatodigital syndrome
- Pyle disease
- craniometaphyseal dysplasia
-<p>Congenital anomalies of the <a href="/articles/middle-ear-ossicles">ossicles</a> are most frequently associated with external ear abnormalities also, although they can occur in isolation. </p><p>They cause <a href="/articles/conductive-hearing-loss-chl">conductive hearing loss (CHL)</a>.-</p><p>When bilateral they are most frequently genetic, with autosomal dominant inheritance, whereas unilateral anomalies are usually sporadic.-</p><p>Isolated ossicular anomalies can be classified as</p><ul><li><strong>class I</strong> : stapedial ankylosis-</li><li><strong>class II</strong> : stapedial ankylosis with other associated anomalies-</li><li><strong>class III</strong> : ossicular anomalies withouth stapedial ankylosis-</li><li><strong>class IV</strong> : oval / round dysplasia-</li></ul><p>They are associated with numerous syndromes including</p><ul><li><a href="/articles/klippel-feil-syndrome" title="Klippel-Feil syndrome (KFS)">Klippel-Feil syndrome</a><ul><li>incudostapedial deformity (long crus and lentiform process of the <a href="/articles/incus">incus</a>)-</li><li><a href="/articles/stapes">stapes</a> fixation-</li><li>stapes hypoplasia / aplasia-</li></ul></li><li><a href="/articles/wildervanck_syndrome">Wildervanck syndrome</a> (or <a href="/articles/wildervanck_syndrome" title="Wildervanck syndrome">cervico-ocular-acoustic dysplasia</a>)-<ul><li>typically diffuse ossicular ankylosis-</li></ul></li><li><a href="/articles/madelung-dyschondro-osteosis" title="Madelung dyschondro-osteosis">Madelung dyschondro-osteosis</a></li><li><a href="/articles/otopalatodigital-syndrome">otopalatodigital syndrome</a></li><li><a href="/articles/pyle-disease" title="Pyle disease">Pyle disease</a></li><li><a href="/articles/craniometaphyseal-dysplasia" title="craniometaphyseal dysplasia">craniometaphyseal dysplasia</a></li></ul>- +<p>Congenital anomalies of the <a href="/articles/middle-ear-ossicles">ossicles</a> are most frequently associated with external ear abnormalities also, although they can occur in isolation. </p><p>These anomalies result in <a href="/articles/conductive-hearing-loss-chl">conductive hearing loss</a>.</p><p>When bilateral, they are most frequently genetic with autosomal dominant inheritance. Unilateral anomalies are usually sporadic.</p><p>Isolated ossicular anomalies can be classified as:</p><ul>
- +<li>
- +<strong>class I</strong> : stapedial ankylosis</li>
- +<li>
- +<strong>class II</strong> : stapedial ankylosis with other associated anomalies</li>
- +<li>
- +<strong>class III</strong> : ossicular anomalies withouth stapedial ankylosis</li>
- +<li>
- +<strong>class IV</strong> : oval/round dysplasia</li>
- +</ul><p>They are associated with numerous syndromes including:</p><ul>
- +<li>
- +<a href="/articles/klippel-feil-syndrome-3">Klippel-Feil syndrome</a><ul>
- +<li>incudostapedial deformity (long crus and lentiform process of the <a href="/articles/incus">incus</a>)</li>
- +<li>
- +<a href="/articles/stapes">stapes</a> fixation</li>
- +<li>stapes hypoplasia / aplasia</li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/wildervanck-syndrome">Wildervanck syndrome</a> (or <a href="/articles/wildervanck_syndrome">cervico-ocular-acoustic dysplasia</a>)<ul><li>typically diffuse ossicular ankylosis</li></ul>
- +</li>
- +<li><a href="/articles/madelung-dyschondro-osteosis">Madelung dyschondro-osteosis</a></li>
- +<li><a href="/articles/otopalatodigital-syndrome-type-i">otopalatodigital syndrome</a></li>
- +<li><a href="/articles/pyle-disease">Pyle disease</a></li>
- +<li><a href="/articles/craniometaphyseal-dysplasia">craniometaphyseal dysplasia</a></li>
- +</ul>
Tags changed:
- congenital anomaly
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