Rationale: Congenital central hypoventilation syndrome (CCHS) is a genetic disorder mainly characterized by failure of automatic control of breathing causing alveolar hypoventilation. Little is known regarding cough in CCHS. Parental reports indicate that patients cough normally during airway infections; however, previous studies have demonstrated no cough response to fog inhalation. Objectives: To evaluate the sensory and motor components of cough, respiratory sensations and changes in ventilation evoked by fog inhalation in CCHS children and in sex- and age-matched control subjects. Methods: Cough threshold was measured and cough intensity was indexed in terms of cough peak expiratory flow (PFC) and integrated abdominal electromyographic activity (IEMG). The pattern of breathing was recorded by inductive plethysmography. Respiratory sensations were also investigated. Measurements and Main Results: All control subjects and 6 out of 7 patients coughed in response to fog inhalation. The seventh coughed with citric acid aerosol. Cough threshold values were similar in controls (range 0.40 to 2.22 ml/min), and patients (range 0.40 to 3.26 ml/min). Mean values of PFC and of IEMG-related variables during coughing were also similar and corresponded to 80% of those recorded during maximum voluntary cough. Cough appearance was preceded by respiratory sensations and increases (P<0.01) in ventilation in the controls but not in the patients. Conclusions: CCHS children have normal cough threshold and motor responses to fog inhalation. However, the lack of respiratory sensations and the likely related ventilatory changes typically elicited by tussigenic fog concentrations suggest a neural sensory deficit which may increase the risk of respiratory disease in these patients.