[Skip to Content]
[Skip to Content Landing]
Other
July 1940

BILATERAL ACOUSTIC NEUROFIBROMASFURTHER CLINICAL AND PATHOLOGIC DATA ON HEREDITARY DEAFNESS AND RECKLINGHAUSEN'S DISEASE

Author Affiliations

CLEVELAND; NEW HAVEN, CONN.

From the Department of Neurological Surgery, the Cleveland Clinic.

Arch NeurPsych. 1940;44(1):76-99. doi:10.1001/archneurpsyc.1940.02280070084004
Abstract

In 1930, Gardner and Frazier1 reported a family in which von Recklinghausen's disease, in the form of bilateral tumors of the acoustic nerve, had been transmitted as a dominant mendelian trait through five generations. At the time of the original report, 38 of the 217 members of the family were affected. Necropsy was performed on 2 members of the family and revealed bilateral acoustic neurofibromas. After the publication of the first report, symptoms of a tumor of the spinal cord developed in 1 of the affected members. This tumor was removed and likewise proved to be a neurofibroma; the case was subsequently reported by Gardner.2

The present report, the third concerning this family, deals chiefly with the pathologic material obtained from 4 members since the last publication.3 One of these members (case 4) represents the only case thus far observed in which the disease has been recognized

×