A 63-year-old woman with multiple disturbances of the cranial nerves is reported. She initially complained of bilateral hearing loss and otorrhea, followed by right facial palsy and vertigo and was hospitalized on August 25, 1983. Despite steroid therapy, her condition deteriorated and finally complete bilateral deafness and complete bilateral facial palsy developed.
Histologial examination of the mastoid mucosa, obtained by exploratory tympanotomies in both ears, showed non-specific inflammation. Noteworthy findings or laboratory examinations were extremely high levels of ESR and CRP, positive RA test, and negative tuberculin test.
Tracheotomy was performed because of recurrent bilateral nerve palsy. Scleritis occurred in March, 1984, and the disturbances of eye movement, ptosis and atrophy of tongue developed in September, 1984. Long-term steroid therapy did not improve cranial palsy. There were no neurological signs such as pathological reflex in the limbs, involuntary movement or meningeal irritation symptom. There were no remarkable findings on CT or MRI.
Although treatment by a local hospital continued, the III, IV, VI, VII, VIII, X and XII cranial nerves remained paralyzed. She finally died of systemic weakness in March, 1989.
This was a rare case that began with bilateral otitis media and proceeding to paralysis of initially occurred and cranial nerves in order of VII, VIII, X, XII, III, IV and VI nerves. The background of this case included systemic vascular disease, especially Cogan's syndrome, because of otologic and ocular symptoms and multiple disturbances of the cranial nerves.
