severe deafness. Nuclear magnetic resonance imaging of the brain was normal. Cholinesterase inhibitors had no effect. We administered 1 mg/kg prednisone to the patient daily for 2 weeks, without any benefit. The patient was discharged in an unchanged state. One month later, acute breathing distress led to respiratory arrest. Endotracheal intubation (EI) was performed and mechanical ventilation was started in an intensive care unit (ICU). Neither upper airway obstruction nor clinical and radiological evidence of upper and lower respiratory airway disease was demonstrated. Tracheostomy was performed 10 days after EI and ventilatory support was continued for 2 months, resulting in gradual recovery of spontaneous respiration. Intermittent nocturnal positive-pressure ventilation by tracheostomy was continued. At home, long-term nocturnal ventilatory support allowed the maintenance of a satisfactory diurnal blood gas equilibrium (pH7.38; pCO244 mmHg; pO 2 86 mmHg; HCO 3 27 mEq/1). To our knowledge, this is the second sporadic case of BVVLS displaying the complete phenotype. Our case also shows subclinical involvement of the optic nerve, which has rarely been reported [1, 8]. The other peculiar features of our case include very late onset of deafness and relatively short latency between hearing loss and onset of bulbo-spinal involvement. We did not observe improvement following steroid treatment, although it has been anecdotally reported . No treatment is currently available. Descriptions of the illness often include an irregular course, usually resulting in death from respiratory muscle failure [3-6]. ICU procedures were reported as unsuccesful in one case . However, following ICU procedures and continued nocturnal home respiratory assistance our patient has mantained satisfactory diurnal respiratory function. Since long-term nighttime ventilation is known to stabilize pulmonary function for years in patients with severe neuromuscular diseases , we suggest that the above-mentioned procedures should also be considered in patients with BVVLS.