INTRODUCTION Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous.

AIM To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1. DEVELOPMENT. We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: ‘Chiari malformation’, ‘Arnold-Chiari malformation’, ‘Chiari type 1 malformation’, ‘Arnold-Chiari type 1 malformation’, ‘evoked potentials’, ‘brainstem auditory evoked potentials’ and ‘somatosensory evoked potentials’ as well as records of patients with CM-1 from the neurosurgery and neurophysiology departments at the Hospital Universitari Vall d’Hebron.

CONCLUSIONS Common findings of SSEP are a reduction in cortical amplitude from the posterior tibial nerve, a reduction or absence of cervical median nerve potential, and an increased N13-N20 interval. In BAEP, the most frequent findings are an increased I-V interval and a peripheral or cochlear auditory disturbance.