Article
Cochlea aplasia in the context of a multiple malformation syndrome in cause of a traumatic damage in early gravidity
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Authors
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Ulrich Vorwerk
- ENT-Department, Univ. of Magdeburg, Germany
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Wilma Vorwerk
- ENT-Department, Univ. of Magdeburg, Germany
| Published: | July 6, 2010 |
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Outline
Text
Introduction: Presented is the cause of a child with aplasia of both cochleae in the context of multiple craniofacial malformations. A genetic reason couldn´t be detected. And now, a traumatic damage in early gravidity, triggered by a traffic accident, will be discussed.
Casuistic: The male baby was born in 17.09.2007 by healthy parents. This young patient was presented in our paedaudiological department in January 2008 to eliminate a hearing loss. Postpartal there was a icterus neonatorum. Conspicuous was a craniofacial dysmorphia with cleft lip and palate on left side and a dysplasia of the outer ear (II°) on both sides. Additional there was mental and statomotorical retardation. The other paediatric diagnostic was without pathological findings.
The paedaudiological diagnostic includes the anamnestic exploration and the deduction of TEOAE and FAEP. For middle ear examination we do the tympanometric analysis. Because we found a deafness in BERA, in the same examination there was done a Notched Noise BERA and a Electrocochleographia. In all of the examinations deafness was confirm. In the case of a velopharyngeal insufficiency we found a concomitant seromucotympanon. To evaluate the anatomical situation a high-resolution CT of the temporal bone and a cerebral MRT was done. In both examinations e cochlea structure was not found.
A genetic evaluation of a CFD-syndrome or a Wolf-syndrome was without findings and shows a typical male Karyotype 46y without aberrations of the chromosomes.
In summary a traumatic damage in early gravidity, triggered by a traffic accident, must be discussed.
