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The syndrome of absent ear and congenital facial palsy
Abstract
The syndrome of complete absence of one ear and ear canal, and ipsilateral facial palsy is one of the extremely rare manifestations of genetic abnormalities.
The occurrence of microtia/anotia syndrome in association with congenital facial palsy has been reported in association with:
1-Teratogenic effect of thalidomide and retinoic acid.
2-Well-Known syndromes including Berry Treacher Collins and Goldenhar.
3-A poorly characterized cardiofacial syndrome or syndromes including the cases of:
Matthies (1966).
Pearl (1984).
Hall (1985).
Bretton Chappuis and Engel (1989).
Girisha and Phadke (2005).
There have been very few reports of the occurrence of microtia/anotia syndrome in association with congenital facial palsy without other congenital cardiac defects or other congenital abnormalities. These reports include the reports of:
Parkash and colleagues (1982).
Gathwala and colleagues (2013).
Mahale et al (2016).
The aim of this book is to describe the extremely rare occurrence of the syndrome of complete absence of one ear and ipsilateral facial palsy in an Iraqi infant.
This Iraqi patient most probably represents the fourth patient with the syndrome of microtia/anotia syndrome and congenital facial palsy without other abnormalities.
... Isolated anotia and microtia were thought to have a genetic basis. Anotia/microtia occurs when tissues associated with the formation of the auricle do not develop [1,2]. ...
... The association of ear malformation and cranial nerve palsies was originally reported to occur in thalidomide children. Matthies reported the occurrence of anotia and facial paralysis facial paralysis in association with cardiac defect in non-thalidomide children [2][3][4]. ...
... Well-Known syndromes associated with microtia/anotia and congenital facial palsy include Berry Treacher Collins and Goldenhar [1,2]. ...
The syndrome of unilateral anotia, and ipsilateral facial palsy is one of the extremely rare manifestations of genetic abnormalities. The occurrence of microtia/anotia syndrome in association with congenital facial palsy has been reported in association with teratogenic effect of thalidomide and retinoic acid, well-Known syndromes such as Berry Treacher Collins and Goldenhar, and poorly characterized cardiofacial syndrome or syndromes including the cases of Matthies, Pearl, Hall, Bretton Chappuis and Engel, and Girisha and Phadke. There have been very few reports of the occurrence of microtia/anotia syndrome in association with congenital facial palsy without other congenital cardiac defects or other congenital abnormalities. These reports include the reports of Parkash and colleagues, Gathwala and colleagues and Mahale, et al. The aim of this paper is to describe the extremely rare occurrence of the syndrome of congenital ipsilateral facial palsy and unilateral anotia in an Iraqi infant. This Iraqi patient most probably represents the fourth patient with the syndrome of microtia/anotia syndrome and congenital facial palsy without other abnormalities.
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