Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome)

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.