어지럼증을 주소로 내원한 아놀드 키아리 기형 1예

A type 1 Arnold-Chiari malformation is defined by herniation of cerebellar tonsil through the foramen magnum. It is usually asymptomatic until three decades to five decades. Symptoms of type 1 Arnold-Chiari malformation are headache, lower cranial palsy, motor weakness and ataxia. However, isolated vertigo has rarely been described as a first presenting symptom of the disease. A seven-year-old boy presented with acute vertigo. A left beating horizontal nystagmus was identified, while the caloric response was normal. And other neurologic deficits were not evident. Magnetic resonance image confirmed a type 1 Arnold-Chiari malformation and decompressive surgery has been performed. After surgery, nystagmus was disappeared and subjective dizziness was improved. An isolated vertigo might be a first symptom of type 1 Arnold-Chiari malformation. We must consider the possibility of congenital malformation in the differential diagnosis of vertigo. (J Clinical Otolaryngol 2017;28:81-85)