抄録
A 36- year-old man with Recklinghausen's disease had impaired hearing of the right ear for over 20 years but otherwise had been free of any other symptoms. Four months prior to his initial visit to our hospital, he noted gradual progressive hearing loss of the left ear. A diagnosis of tumors of the auditory nerves on both sides was made on neuro-otologic examination and the final diagnosis of bilateral acoustic neuromas associated with Recklinghausen's disease was confirmed by skin biopsies, and at operation and autopsy.
The results of auditory and x-ray examinations were as follows:
Complete deafness of the right ear. Pure hearing loss of the left ear with poor speech discrimination, no recruitment phenomenon and transient elevation of hearing threshhold were indicative of typical retrolabyrinthine deafness.
No fixation, positional or positioning nystagmus.
No reaction to caloric test of the right ear but the left side was normal. CP on the right.
Decreased response to the rotatory test on both sides. No difference between the right and left sides.
X-ray examinations with Stenver's and Town's methods and tomograms of the inner ear revealed destruction of the right petrous portion of the temporal bone and enlargement of the internal auditory canal.
