The authors report a first case of chronic motor axonal neuropathy involving ENT manifestations, in a 64-year-old male presenting with gait difficulties, effort dyspnoea and dysphonia. Eleven months after the first symptoms, he developed severe hypoventilation, limb weakness and bilateral vocal fold palsy and had to be intubated for respiratory failure. The diagnosis of chronic motor axonal neuropathy was suspected on clinical and electrophysiological grounds. The patient improved dramatically after a five-day course of 0.4 g/kg intravenous immunoglobulin. He is still being treated with methylprednisolone 0.5 mg/kg every other day and remains stable.

We conclude the bilateral vocal fold palsy may be associated with chronic motor axonal neuropathy and that the immunosuppressive treatment may be effective in such cases.