Tumor of the Spinal Cord Associated with Bilateral Acoustic Tumors


The patient, a report of whose case follows, is a member of a family in which bilateral deafness has been transmitted as a true mendelian dominant character. The condition has been traced through five generations of the family, which includes 217 members.1 Thirty-eight members have been affected. Of these thirty-eight, fifteen subsequently became blind, the blindness being preceded by headache and vomiting in each case in which information was available. Of the deaf and blind persons, four were examined prior to death, and were found to have choking of the optic disks with secondary atrophy. Of the deaf persons, seven were personally examined. Five of these had entire absence of vestibular responses in the Barany test. In the other two, a sluggish response was obtained from the left horizontal canal, but the remaining semicircular canals were nonfunctioning. In addition, four subjects were found who had little or no impairment of hearing, but whose vestibular responses were absent in the Barany test. These Barany observations, together with the neurologic signs which these persons presented, made the diagnosis of bilateral acoustic tumors practically indisputable. The two affected members of this family who came to necropsy had bilateral acoustic neurofibromas. There was practically no associated evidence of von Recklinghausen’s disease in this family, and at the time of the investigation, there was nothing to indicate the presence of tumors elsewhere than on the acoustic nerves.

Following publication of the first report on this family, however, one of the affected members,: the subject of. . .



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