Sarcoidosis is an idiopathic systemic granulomatous disease which occasionally causes fluctuating auditory and vestibular dysfunction. The temporal bones from a 32-year-old man deaf for 5 years from CNS sarcoidosis were examined histologically and compared with other nervous system tissues. It was found that the acoustic, vestibular, and facial nerves were involved in a striking perivascular lymphocytic infiltration resulting in myelin and axonal degeneration. The cochlear and labyrinthine neuroepithelium and stria vascularis had degenerated. It is hypothesized that neurosensory deafness and vestibular dysfunction in sarcoidosis starts as a reversible neuropathy. In some patients, an ischemia secondary to the vasculitis results in irreversible damage to the inner ear neuroepithelium.