Background: In this study, we examined whether egocentric representation of space is impaired in chronic unilateral vestibulopathies. The objective was to test current theories attributing a predominant role to vestibular afferents in spatial cognition and to assess whether representational neglect signs are common sign in peripheral vestibular loss. Methods: We investigated the subjective straight ahead (SSA) direction using a horizontal rod allowing to dissociate the translation and rotation components of the body midline representation in 21 patients with unilateral vestibular loss (right = 13; left = 8) and in 12 healthy controls. Results: As compared to the controls, the patients with unilateral vestibulopathy showed a translation bias of their SSA, without rotation bias. The translation bias was not lateralized towards the lesioned side as typically found for biases reported after unilateral vestibular loss. Rather, the SSA bias was rightward whatever the side of the vestibular loss. The translation bias correlated with the vestibular loss, as measured by caloric response and VOR gain, but not with the subjective visual vertical or the residual spontaneous nystagmus. Conclusion: The present data suggest that the dysfunctions of neural networks involved in ego- and allocentred representations of space are differentially compensated for in unilateral vestibular defective patients. Specially, they suggest that asymmetrical vestibular inputs to cortical regions lead to representational spatial disturbances as does defective cortical processing of vestibular inputs in spatial neglect after right hemisphere stroke. They also highlight the predominant role of symmetrical and unaltered vestibular inputs in spatial cognition.