The Red Ear Syndrome: Five New Cases

Dear Sir   We read with great attention the article of Kumar and Swanson (1), which report two new cases of red ear syndrome (RES), since we are very interested in this disorder (2).

In the last years we have diagnosed other five patients with this syndrome whose reports we believe can complement the cases published before.

We have summarized the clinical and laboratory findings of our cases in Table 1.

Table 1

Clinical characteristics of the five cases

Case	Age/sex	Clinical presentation	Attacks: duration frequency, precipitating	Duration of syndrome (follow-up)	Neurological examination	Laboratory studies	Presumed cause	Treatment
1	70, F	Facial palsy, pain and rash (left C3–C6 dermatomes)	Four weeks One attack Spontaneous	Four weeks (2 years)	Reddening of the left ear	–	Herpes zoster∗	Famciclovir, prednisone, amitryptyline
2	23, F	Right occipital pain	Seconds–minutes	One month	Normal	Cervical	Idiopathic	Amitryptyline
			Twenty (per day)Spontaneous	(3 months)		SpineMRI: normal
3	41, M	Severe left headache	HoursTwo attacksPhysical activity†	Unknown	Normal	Cranial MRI: tonsillar herniationof 5 mm	Chiari I malformation	Amitryptyline andindomethacin
4	26, F	Right occipital pain	MinutesCountlessSpontaneous	Unknown	Normal	–	Idiopathic‡	Amitryptyline andpiroxicam
5	48, F	Left facial pain	HoursOne (per day)Spontaneous	Four months(9 months)	Normal	Cranial and cervical spine MRI: normal	Temporomandibularjoint dysfunction	Amitryptyline

∗

Red ear syndrome 3 days later.

†

Sexual intercourse in one episode.

‡

Association with history of migraine.

We would like to emphasize some aspects of our series. First, the motive of consultation was not in any case a reddening of the ear, but facial paralysis (case 1) and headache or facial pain in the remainder. Second, we have observed in addition to the two cases reported by Lance (3) another patient who presented the syndrome associated with a temporomandibular joint dysfunction (case 5).

As for the aetiology, besides the case associated with dysfunction of the temporomandibular joint, two others were the result of injury of the upper cervical roots of infectious (virus herpes zoster, case 1) and compressive cause (Chiari I malformation, case 3). In this case, the RES was presumably caused by temporary compression of the cerebellar tonsils during physical activity (sexual); this sensitivity to exercise is consistent with reduced compliance of the subarachnoid space (4).

Finally, there was a complete resolution of symptoms in three of four cases, including the case previously reported (2), in which there was follow-up; his relationship with the treatment is unknown.

In conclusion, we think that RES is an underdiagnosed disorder, since in our patients the motive for consultation was different from the discomfort and reddening of the ear and could have identified several cases in a short period, probably because we have developed a specific search for similar cases after diagnosing the first (2).