皮質聾を呈した成人出血型もやもや病の1例

Cortical Deafness due to Bilateral Temporal Subcortiocal Hemorrhages Associated with Moyamoya Disease : report of a case

I．はじめに

　皮質聾は側頭葉病変が両側に生じてはじめて発症するため^4,7,8）稀な病態であるが，CTが普及して以来，次々と報告されてきている^{2,3,7,8,10,11,12）}．皮質聾の原因となる頭蓋内疾患は脳血管障害がほとんどであるが，脳出血例に比べて脳梗塞例が多く^7,9,10,12），脳出血に伴った症例は現在までに6例しか報告がない^{2,3,5,8,11,12）}・われわれはもやもや病で両側側頭葉皮質下出血を来たし，皮質聾を呈した症例を経験したので文献的考察を加えて報告する．

A 51-year-old male with moyamoya disease had experienced a left putaminal hemorrhage 14 years previ-ously, in 1983. Left encephalo-duro-arterio-synangiosis (EDAS) was performed in that year. The left puta-minal hemorrhage reoccurred in 1990 and in 1994. Left superficial temporal artery-middle cerebral arteryanastomosis (STA-MCA anastomosis) was performed. He was symptom free after the surgery. However,he suddenly suffered from deafness in 1997, when computerized tomography (CT) revealed the right tem-poral subcortical hemorrhage. Deafness continued for three days. It gradually improved and completelydisappeared in two months. The damage to the bilateral auditory radiation was suggested as the cause ofdeafness in this patient.

Cortical deafness is a rare symptom that is most often associated with cerebral infarction. This may he the first report of moyamoya disease which developed cortical deafness.