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.