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Goldenhar syndrome or oculoauriculovertebral spectrum (OAVS) is characterized by preauricular skin tags, microtia, facial asymmetry, ocular abnormalities and vertebral anomalies of different size and shape. The presence of anomalies of the ear is necessary for diagnosis. In addition to craniofacial anomalies, there may be cardiac, genitourinary and central nervous system defects. This syndrome is usually thought to be caused by abnormalities of the first and second branchial arches. The phenotypical findings of this syndrome are variable due to heterogenous etiology. We report here a newborn with OAVS presented with left hemifacial microsomia, hypoplasia of depressor anguli oris muscle, microtia, deafness and ventricular septal defect and diognosed OAVS. [ABSTRACT FROM AUTHOR]

Autoimmune inner ear disease is characterized as a disease with progressive sensorineural hearing loss. The disease can occur primarily or it can accompany other systemic autoimmune diseases. The diagnosis is based on clinical presentation and response to the administration of corticosteroids. The first choice of therapy is the administration of steroids. If the disease is refractory to steroids, immunosuppressants are the second choice. Autoimmune inner ear disease should be kept in mind, when differential diagnosis of any patient with progressive sensorineural hearing loss is made. Here, we present distinguishing characteristics of autoimmune inner ear disease in the lights of literature. [ABSTRACT FROM AUTHOR]