Skarzyński Henryk, Porowski Marek, Podskarbi-Fayette Robert
International Center of Hearing and Speech of the Institute of Physiology and Pathology of Hearing, Warsaw/Kajetany, Poland.
Int J Pediatr Otorhinolaryngol. 2009 Jul;73(7):915-21. doi: 10.1016/j.ijporl.2009.01.015. Epub 2009 Feb 8.
The oculoauriculovertebral dysplasia is a rare congenital malformation that is characterized by a hemifacial microsomia with ocular abnormalities and coexisting disorders in the spinal column and other organs. Characteristic features of the disease were first described by Maurice Goldenhar in 1952. A broad spectrum of hearing impairment is a result of complex malformations of the external ear, the middle ear and in some cases-the inner ear. The degree of hearing loss can range from mild to moderate conductive type in cases of chronic otitis media with effusion and severe to profound sensorineural hearing impairment with malformations of the inner ear in various forms of cochlear hypoplasia. This study focuses on surgical methods of hearing improvement in patients with the oculoauriculovertebral dysplasia (OAVD). We present 11 patients diagnosed with OAVD who are under supervision of the Institute of Physiology and Pathology of Hearing in Warsaw. It is, to our knowledge, one of the largest groups of patients reported by a single ORL medical center. The degree of hearing impairment was thoroughly described in all patients and individual treatment was introduced. The aim of this report was to present algorithm for diagnostics and treatment of symptoms of Goldenhar syndrome (GS) based upon methodology used in modern otosurgery.
眼耳脊椎发育不良是一种罕见的先天性畸形，其特征为半侧颜面短小并伴有眼部异常，以及脊柱和其他器官的并存病症。该病的特征性表现最早由莫里斯·戈尔登哈于1952年描述。广泛的听力障碍是外耳、中耳以及某些情况下内耳复杂畸形的结果。听力损失程度在伴有积液的慢性中耳炎病例中可从轻度至中度传导性听力损失，在各种形式的耳蜗发育不全导致内耳畸形的情况下则可从重度至极重度感音神经性听力损失。本研究聚焦于眼耳脊椎发育不良（OAVD）患者听力改善的手术方法。我们介绍了11例被诊断为OAVD且在华沙听力生理与病理研究所接受监测的患者。据我们所知，这是单个耳鼻喉科医疗中心报告的最大患者群体之一。对所有患者的听力障碍程度进行了全面描述，并采用了个体化治疗。本报告的目的是基于现代耳外科所使用的方法，呈现戈尔登哈综合征（GS）症状的诊断和治疗方案。
