The diaphragm pacing system (DPS) is a minimally invasive alternative to mechanical ventilation in patients with quadriplegia due to cervical myelopathy primarily caused by high cervical spinal cord injury. We evaluated 36 patients, 29 of whom had traumatic spinal cord injury, two who had a history of remote meningitis and demyelinating disease, and five who had cervical myelopathies of unknown etiology. Phrenic nerve conduction studies were performed with simultaneous fluoroscopic observation of diaphragm excursion to assess diaphragm viability. In the preoperative evaluation, diaphragm compound muscle action potentials (CMAPs) were recorded only when the diaphragm moved on fluoroscopy with ipsilateral stimulation. Twenty-six patients who were determined to have a viable diaphragm underwent DPS. Following DPS the primary outcome was the time (hours per day) that patients were able to pace and stay off the ventilator. Of 26 implanted patients, 96% (25 patients) were able to pace and tolerate being off the ventilator for more than 4 h per day. This study demonstrates that the presence of a diaphragm CMAP is associated with diaphragm movement observed by fluoroscopy in cervical myelopathy. In addition, DPS can help patients with cervical spinal cord injury to breathe unassisted by a ventilator.