Audiological evaluation of patients with cleidocranial dysplasia (CCD).

OBJECTIVE: The description of clinical manifestation of hearing problems in cleidocranial dysplasia (CCD) remains limited and incomplete, since CCD constitutes a rare congenital disorder. The study aims to provide a complex panel analysis of the auditory system in patients suffering from the disease. PATIENTS AND METHODS: The study group consisted of 4 children with CCD (aged: 12-15), who underwent orthodontic treatment. A full panel analysis of their auditory systems was performed, including high-frequency audiometry and a new method of middle ear assessment - WBT (Wideband Tympanometry). RESULTS: A slight conductive hearing loss was diagnosed in 3 out of 4 patients. While high frequency audiometry has shown a deterioration of hearing in 3 patients, in one case, the obtained thresholds were within the normal range. A decrease of absorbance in low frequencies has been observed in one or both ears. Only one patient has had a shift of maximum absorbance towards high frequencies in the left ear. CONCLUSIONS: The presented manuscript is the first with a complete evaluation of the auditory system comprising 4 cases of children in a similar age group. All of the examined patients presented an air-bone gap indicating conductive disorders.

Executive function in pediatric patients with cystic fibrosis, inflammatory bowel disease and in healthy controls.

OBJECTIVE: The main aim of this study was to analyze and compare executive function performance in pediatric patients with cystic fibrosis (CF), inflammatory bowel disease (IBD) and in healthy controls. PATIENTS AND METHODS: 28 patients with CF, 30 patients with IBD and 30 healthy participants took part in the study (all in the age range of 7-17). All participants were in the intellectual norm. The Wisconsin Card Sorting Test (WCST) was applied to assess executive functions. RESULTS: The CF group received significantly (p < 0.05) poorer scores than the control and IBD groups in the following WCST indicators: Number of trials administered, Total number of errors, Perseverative errors, Non-perseverative errors, Percent of conceptual level responses, Trials to complete the first category. The IBD group was not significantly different from the control group in any of the WCST indicators. CONCLUSIONS: To our best knowledge, results of this study are the first report of the presence of executive function deficits among pediatric patients with CF. It is also the first study that describes the performance of the executive function in IBD pediatric patients, and the first that compares cognitive functioning between CF and IBD patients.