Inner ear malformations (classification)
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Cochlear anomalies are a variety of congenital anomalies which, depending on the exact time at which an insult occurs during embryogenesis, may have different manifestations. Terminology is often used imprecisely leading to confusion not only among clinicians, but also in the literature.
Classification
A classification first proposed in 1987 by Jackler et al. 2 has become widely accepted (with various modification), which divides congenital cochlear anomalies according to the timing of the developmental arrest.
Conveniently starting at 3rd week till the end of 7thweek of gestation, an insult during each subsequent week results in a distinct inner ear abnormality:
- complete labyrinthine aplasia or Michel deformity: 3rd week
- cochlear aplasia: 4th week
- common cavity malformation to the cochlea and vestibule: early 5th week
- cochlear incomplete partition type I including cystic cochleovestibular anomaly: late 5th week
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cochlear hypoplasia:
6th6th week - cochlear incomplete partition type II including Mondini dysplasia: 7th week
See also
-<a href="/articles/cochlear-hypoplasia">cochlear hypoplasia</a>: 6th week</li>- +<a href="/articles/cochlear-hypoplasia">cochlear hypoplasia</a>: 6<sup>th</sup> week</li>
-<a title="Cochlear incomplete partition type II" href="/articles/cochlear-incomplete-partition-type-ii">cochlear incomplete partition type II</a> including <a href="/articles/mondini-malformation">Mondini dysplasia</a>: 7<sup>th </sup>week</li>- +<a href="/articles/cochlear-incomplete-partition-type-ii">cochlear incomplete partition type II</a> including <a href="/articles/mondini-malformation">Mondini dysplasia</a>: 7<sup>th </sup>week</li>
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