Hereditary Deafness Due to Bilateral Acoustic Tumors

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That hereditary deafness may be the result of bilateral acoustic tumors has not hitherto been recorded. The investigation described in this report was suggested by a statement in the history of such a case which intimated deafness in a number of the family connections for five generations. The survey of this family disclosed a history of bilateral deafness in thirty-eight members, with subsequent blindness in fifteen instances. Of the deaf and blind persons, four were examined prior to death and were found to have choking of the optic discs with secondary atrophy. The seven affected members living were personally examined and presented the clinical findings of bilateral acoustic tumors. The presence of these lesions was proved by necropsy in two cases. Following is the case record of the patient whose history stimulated the investigation:

VA 6, a man, aged 28 (see Fig. 7), was admitted to the neurosurgical service of the University Hospital on October 9, 1928. He was referred to the clinic by Dr. T. K. Wood, of Muncy, Pa., who apparently was the first physician to recognize the unique hereditary tendency of the disease in this family. The chief complaint was deafness and blindness.

The history of this patient's illness was essentially a repetition of a common complaint that had affected many members of his family for five generations. The patient stated that partial deafness had begun at the age of 17. Whether or not the onset and progress of the condition in both ears was concurrent is. . .