Böggering B
Fortschr Med. 1975 Oct 23;93(30):1452-5.
The oto-vertebral syndrome is supposed to be caused by an early embryonic exogenous damage which at the same time affects the development of the ear and vertebral column and possibly also causes cardiac anomalies. Animal studies showed that the malformation syndrome originates in the 6th-7th week of embryonic development. The extent of the malformations of the ear and the vertebral column varies. Frequently seen are dysplasia of the external ear and dystopia and atresia of the external ear canal as well as vertebral malformations, mostly involving the thoracic region. Only 4 case reports have been published in the literature. The differential diagnosis includes Goldenhar-syndrome, oculo-vertebral syndrome. Thalidomide-syndrome and chromosomal aberrations. Therapy depends on the extent of the malformations. In case of atresia of the ear canal hearing is first of all improved with a hearing aid, operative procedures are to be planned later on. Child development is promoted with acustic stimulation.
耳椎综合征被认为是由早期胚胎期的外源性损伤引起的,这种损伤同时影响耳朵和脊柱的发育,还可能导致心脏异常。动物研究表明,这种畸形综合征起源于胚胎发育的第6至7周。耳朵和脊柱的畸形程度各不相同。常见的有外耳发育异常、外耳道异位和闭锁,以及脊柱畸形,主要累及胸部区域。文献中仅发表了4例病例报告。鉴别诊断包括Goldenhar综合征、眼椎综合征、沙利度胺综合征和染色体畸变。治疗取决于畸形的程度。对于耳道闭锁的情况,首先使用助听器改善听力,后续再计划手术程序。通过听觉刺激促进儿童发育。