AIMS: Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients. METHODS: Ten patients with Pendred syndrome were analyzed for speech perception and production. The control group consisted of 41 non-syndromic implanted patients. All implantees were scored according to speech perception, speech production, and the sum of both. The data were statistically analyzed. RESULTS: No statistical difference was found in language acquisition and production in implantees with Pendred syndrome when compared to non-syndromic patients with cochlear implants. Nor there was any difference in speech production and acquisition between the 2 compared groups regarding surgical age, time elapsed after surgery, or age during the testing. CONCLUSION: In this study evaluating language and speech production and acquisition, patients with Pendred syndrome who underwent cochlear implants show comparable results to their implanted peers with deafness of a different etiology.

Department of Biology and Medical Genetics 2nd Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czechia

Department of Ear Nose and Throat 2nd Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czechia

Department of Neurology and Center of Clinical Neuroscience 1st Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czechia

Department of Otorhinolaryngology and Maxillofacial Surgery 3rd Faculty of Medicine Charles University Prague and Military University Hospital Prague Czechia

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