A 22-year-old woman presented to the neuro-otology clinic with the complaint of right-sided tinnitus and bilateral hyperacusis. Additionally, she complained of right ear fullness and difficulty hearing in background noise during stress. These symptoms had occurred twice, five years apart. Both of these times, she had presented to the clinic.
Examination and Investigations
The doctors performed an otoscopy and a complete neuro-otological exam. They further examined the patient with pure-tone audiometry, tympanometry, stapedial reflexes, otoacoustic emissions (OAEs), auditory brainstem response and speech audiometry. The results for all these examinations and investigations came out normal. Although, the doctors noticed strong transient OAE responses and spontaneous OAE activity bilaterally. These findings were consistent with an increase in cochlear gain (which indicated reduced efficacy of inhibitory feedback in the auditory system).
Management: Red Ear Syndrome
The doctors advised auditory rehabilitation by referring her to a hearing therapist, which included counselling, communication tactics, tinnitus and hyperacusis retraining. Moreover, the doctors advised the patient about relaxation techniques, stress management and ear-level noise generators for desensitization. The doctors discharged her once her condition improved. The doctors reviewed her 9 months later and her examination was unremarkable.
Representation
Five years later, she presented to the clinic again with the complaint of recurrent one-hour episodes of painful cutaneous erythema of the right external ear which was associated with severe right temporal pain radiating down to the mastoid area. This caused transiently reduced hearing, right conjunctival injection, and intolerance to noise and light. These symptoms pointed towards the involvement of pathways other than the auditory pathway. Additionally, she reported headaches with fatigue and light-headedness. There was no nausea, visual field symptoms, tinnitus or vertigo.
These symptoms had put the patient in a lot of distress causing her multiple visits to the GP, Emergency Department and ENT clinics.
Examination
Upon examination, the doctors found no erythema, ear or mastoid infection. The otoscopy findings were normal. They performed a neuro-otological examination which was unremarkable, including extra-ocular eye movements, cranial nerves, cerebellar function and clinic room balance tests. Pure-tone audiometry and tympanometry were also normal. Magnetic resonance imaging of the brain was normal. Routine blood tests were also normal. Therefore, the doctors diagnosed her with Red Ear Syndrome with hyperacusis.
Management
The doctors advised her to do a behavioural modification for her migraine. This included reduced caffeine, stress reduction, fluid intake optimization, better sleep patterns, relaxation techniques and exercise. They advised her to monitor her triggers and offered her migraine prophylaxis, which she refused.
Prognosis
The doctors did a follow-up with a patient after four months. She reported feeling better with no headaches and no presence of an erythematous ear. Moreover, there was no pain or swelling in her ear. The diary that she was advised to maintain for the attacks demonstrated stress and orange juice as triggers, which the doctors asked her to avoid in the future. She did not need any medication for her symptoms for the next four years.
Red Ear Syndrome: Discussion
Red Ear Syndrome is a rare disorder (only 100 cases have been reported in the literature so far). It refers to a constellation of symptoms such as erythema of the ear, ear swelling and ear pain that can be triggered by any entity (such as heat, touch, neck movement, sneezing, coughing, hair brushing, exercise, chewing and exposure to cold). The trigger in this case was orange juice, stress and headaches. The duration of these symptoms can range from seconds to minutes to even hours.
The episodes can occur multiple times a day, and remission- periods can be observed multiple times a year. The condition is diagnosed clinically, there is no diagnostic test for the condition.
The pathophysiology of RES has many hypotheses. One hypothesis states that the disorder is found mainly in those with cervical disorders, which causes the release of vasodilator peptides that activate the trigeminal vascular system. Another hypothesis is that RES is a part of auriculoautonomic cephalgia or trigeminal autonomic cephalgia. Another hypothesis suggests that RES is an auricular form of erythromelalgia. All these hypotheses regard sympathetic dysregulation as the main cause of RES.
RES has two types: primary and secondary. Primary is more common in young people and is associated with migraine, whereas the secondary form occurs in older adults and is associated with cervical disorder or trigeminal autonomic cephalgia.
RES has an association with other conditions as well such as upper cervical pathology (arachnoiditis, facet joint spondylosis and cervical root traction), glossopharyngeal and trigeminal neuralgia, temporomandibular joint (TMJ) dysfunction, thalamic syndrome, primary headache disorders, including migraine, chronic paroxysmal hemicranias, hemicrania continua and the short-lasting unilateral neuralgiform headache with conjunctival injection.
The management of RES is varied. These include methysergide, indometacin, propranolol, the application of a cold pack, amitriptyline, or imipramine. Moreover, behavioural modifications play a major role in its management.
Prospect: Red Ear Syndrome
There is very little known about RES and patients may present with a wide range of symptoms. Thus causing them to present to the emergency department, general practice and other specialities (ENT, dermatology, neurology) due to distressful symptoms and anxiety amongst patients. The delay in presentation and visits to various clinics can cause the patient significant distress and is costly. Thus, prompt diagnosis and awareness amongst practitioners regarding this disease can help treat such patients at an early stage.
