The red ear syndrome in upper cervical degenerative spine pathology; Case report and literature review

- Red ear syndrome (RES) is a very rare disorder, having been described so far about 100 cases in literature.
- It is a disorder that causes unilateral or bilateral pain and redness of the ear externally, with very variable characteristics regarding the frequency, intensity, type of pain, presenting sometimes moderate, sometimes severe, lasting a few seconds, few hours and with short or long periods of remission.
- RES was subdivided in primary and secondary form, the first associated with migraine headache or autonomic trigeminal cephalalgia, the second consequent to pathology of the cervical spine, mainly degenerative, and to disorders of the temporo-mandibular joint.
- Some cases have been associated with lupus erythematosus and erythromelalgia.
- The pathophysiological hypothesis is based on the involvement of the trigeminal-vascular system or dysfunction of the cervical spine nerves, with activation of the cutaneous sensory fibres, causing, through the release of algogenic substances, pain and redness of the ear.