We present a case of deep brain stimulation (DBS) of the globus pallidus interna (GPi) and its positive clinical effects in a 30-years old male patient with dystonia-deafness syndrome manifested by generalized dystonia and severe sensorineuronal hearing impairment. The GPi DBS resulted in dramatic improvement of dystonia documented by a 75% decrease in the motor score of the Burke-Fahn-Marsden-Dystonia scale within 10 months after implantation.