Inner ear malformations (classification)
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The cochlea is prone to a variety of congenital anomalies, depending on the exact time at which an insult occurs during embryogenesis. Terminology is often used imprecisely leading to confusion not only among clinicians but also in the literature.
A classification first propsed in 1987 by Jackler et al 2 has become widely accepted (with various modification), which divides congenital cochlear anomalies according to the timing of developmental arrest.
Conveniently starting from the 3rd week of gestation, an insult during each subsequent week results in a distinct inner ear abnormality.
- 3rd week: complete labyrinthine aplasia (or Michel deformity)
- 4th week: common cavity to the cochlea and vestibule
- 5th week: cochlear aplasia
- 6th week: cochlear hypoplasia
- 7th week: incomplete partition and dilatational defects (including Mondini dysplasia)
-<p>The <a href="/articles/cochlea" title="cochlea">cochlea</a> is prone to a variety of congenital anomalies, depending on the exact time at which an insult occurs during embryogenesis. Terminology is often used imprecisely leading to confusion not only among clinicians but also in the literature. </p><p>A classification first propsed in 1987 by <strong>Jackler et al</strong> <sup>2 </sup>has become widely accepted (with various modification), which divides congenital cochlear anomalies according to the timing of developmental arrest. </p><p>Conveniently starting from the 3<sup>rd</sup> week of gestation, an insult during each subsequent week results in a distinct inner ear abnormality. </p><ul>-<li>3<sup>rd</sup> week : complete labyrinthine aplasia (or <a href="/articles/michel-deformity" title="Michel deformity">Michel deformity</a>)</li>-<li>4<sup>th </sup>week : <a href="/articles/common-cavity-of-the-cochlea-and-vestibule" title="common cavity of the cochlea and vestibule">common cavity to the cochlea and vestibule</a>- +<p>The <a href="/articles/cochlea">cochlea</a> is prone to a variety of congenital anomalies, depending on the exact time at which an insult occurs during embryogenesis. Terminology is often used imprecisely leading to confusion not only among clinicians but also in the literature.</p><p>A classification first propsed in 1987 by <strong>Jackler et al</strong> <sup>2 </sup>has become widely accepted (with various modification), which divides congenital cochlear anomalies according to the timing of developmental arrest. </p><p>Conveniently starting from the 3<sup>rd</sup> week of gestation, an insult during each subsequent week results in a distinct inner ear abnormality.</p><ul>
- +<li>3<sup>rd</sup> week: complete labyrinthine aplasia (or <a href="/articles/michel-deformity">Michel deformity</a>)</li>
- +<li>4<sup>th </sup>week: <a href="/articles/common-cavity-of-the-cochlea-and-vestibule">common cavity to the cochlea and vestibule</a>
-<li>5<sup>th </sup>week : <a href="/articles/cochlear-aplasia" title="cochlear aplasia">cochlear aplasia</a>- +<li>5<sup>th </sup>week: <a href="/articles/cochlear-aplasia">cochlear aplasia</a>
-<li>6<sup>th</sup> week : <a href="/articles/cochlear-hypoplasia" title="cochlear hypoplasia">cochlear hypoplasia</a>- +<li>6<sup>th</sup> week: <a href="/articles/cochlear-hypoplasia">cochlear hypoplasia</a>
-<li>7<sup>th </sup>week : incomplete partition and dilatational defects (including <a href="/articles/mondini-malformation" title="Mondini dysplasia">Mondini dysplasia</a>)</li>- +<li>7<sup>th </sup>week: incomplete partition and dilatational defects (including <a href="/articles/mondini-malformation">Mondini dysplasia</a>)</li>
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