Dystonia deafness syndrome: A rare deep brain stimulation responsive dystonia

Abstract

Dystonia deafness syndrome (DDS) is a rare syndrome characterized by childhood onset sensorineural deafness followed by adult-onset dystonia. We here report the first case of DDS from India caused by ACTB gene mutation presented with deafness, generalized dystonia and scoliosis who showed improvement after Deep brain stimulation.

Keywords

• deafness
• deep brain stimulation
• dystonia