Auditory Nerve Disease, New Classification: Auditory and Vestibular Neuropathy

Five of eight patients who were evaluated had auditory nerve disease (AN), with simultaneous hearing and balance problems, and their complaints had not changed over the past few years. Clinical tests of the balance system in these five patients indicated abnormality on the Mann test with eyes closed. Ice water caloric stimulation failed to elicit nystagmus in these patients. Strong rotational testing yielded results consistent with bilaterally impaired function of the horizontal semicircular canals and/or vestibular nerves. In the same five patients, the vestibular-evoked myogenic potential (VEMP) was abolished. It is suggested that the terms "auditory neuropathy only," "auditory-vestibular neuropathy," and "vestibular neuropathy only" in AN could be used to characterize these patients with involvement of only the auditory branch of the VIII cranial nerve in both the auditory and vestibular branches. This usage may help to categorize this disorder more pathophysiologically.