TEMPORAL BONE AND BRAIN-STEM HISTOPATHOLOGICAL FINDINGS IN CORNELIA-DE-LANGE-SYNDROME

The temporal bone and the brain stem of an infant with Cornelia de Lan ge syndrome, aged 9 months, were studied histopathologically. Abnormal ities in the inner ear included short cochlea, dilated vestibule, and mesenchyme-filled perilymphatic spaces of the vestibule and semicircul ar canals. The inner and outer hair cells were normal. Many spiral gan glion cells, which normally would be located in the canal of Rosenthal , were seen in the internal auditory meatus. Some of the vestibular ga nglion cells intruded into the facial nerve. The facial nerve formed a loose curve and the geniculum was difficult to recognize. The middle ear was filled with mesenchyme, and there was marked dehiscence of the facial canal. The brain stem, particularly the pens, was smaller than normal, while the fourth ventricle was dilated. The microscopic obser vation of the pens revealed weak staining with Luxol Fast Blue and dec reased number of oligodendroglia. These findings suggested the malform ation of the myelin of transverse fibers.