Persistent geotropic direction-changing positional nystagmus with a null plane: the light cupula

The aim of this study was to characterize the clinical features and typical positional nystagmus in patients with persistent geotropic direction-changing positional nystagmus (DCPN) and address the possible pathophysiology of the disease. Furthermore, the proportion of light cupula among the patients showing geotropic DCPN was investigated to assume the incidence of light cupula in those patients.

Prospective case series.

We conducted a prospective case series study in 19 patients with persistent geotropic DCPN. Positional nystagmus during the bow and lean test and the supine head roll test was analyzed using videonystagmography.

All of the 19 patients showed persistent geotropic DCPN without latency. A null plane in which the nystagmus ceases was identified in all of 19 patients, and the intensity of nystagmus was stronger on one side in13 patients (68%) on supine head roll test. Overall, the affected side could be identified in 18 patients (95%). About 14.2% (19 of 134) of patients with geotropic DCPN could be diagnosed as having light cupula in the horizontal semicircular canal.

The patients with light cupula show persistent geotropic DCPN without latency. Affected side(s) can be determined by the direction and intensity of the characteristic positional nystagmus and the side of the null plane. The pathophysiology and treatment of light cupula still remain to be elucidated.