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Two children with a congenital Cornelia de Lange syndrome and a verified sensorineural hearing loss are presented. One could be tested by conventional audiometry, the other only with the help of the newer computerized electronic methods. A short review of some earlier reports on congenital hearing loss in this syndrome is made.
Two patients with labyrinthine fistulae following chronic otitis media are presented. Anaerobic bacteria, especially Bacteroides spp. and Bacteroides fragilis were recognized as important pathogens in the disease.