Evidence of central involvement in essential tremor: a detailed study of auditory pathway physiology

Essential tremor (ET) is a common tremor disorder that is likely neurodegenerative. The pathophysiology of ET involves the cerebellum and its connections in the brainstem and thalamus. Hearing dysfunction has been shown to be a non-motor finding in ET patients. A limited number of studies have suggested that cochlear pathology is the cause, but studies have not evaluated the integrity of the primary auditory pathway in ET. The main aim of this study is to investigate the integrity of the auditory pathway via auditory brainstem response (ABR) and auditory middle latency response (AMLR), thereby allowing us to evaluate the auditory pathway from the 8th cranial nerve to the cerebral cortex. Sixteen ET patients and sixteen age- and gender-matched controls (64 ears) were evaluated. In the ABR study, we detected prolongation of wave V peak latencies (ms) in ET (p = 0.02). In the AMLR study, P0 (p = 0.03), Pa (p = 0.008), Na (p = 0.03), and Nb (p = 0.01) waves differed between the two groups. Eleven ET patients and four control subjects had abnormal electrophysiological findings (ABR or AMLR or both) (68.8% vs. 25%, p = 0.01). Tremor duration was greater in ET patients with abnormal electrophysiological findings (p = 0.01). Finally, we observed prolongation of latencies after the ABR III wave, indicating that abnormalities exist within the superior olivary complex. For the first time, our research provides evidence that ET-related pathology is present at the subcortical and cortical levels of the auditory pathway.