Medical Excellence - Issue 2

Otoscopy, a neuro-otological examination, pure-tone audi- ometry, tympanometry, stapedial reflexes, otoacoustic emissions (OAEs), auditory brainstem response and speech audiometry results were normal. She had particularly strong transient OAE responses and spontaneous OAE activity bilaterally. This was consistent with increased cochlear gain, suggestive of reduced ef- ficacy of inhibitory feedback in the auditory system. Several ses- sions of auditory rehabilitation were carried out with a hearing therapist, involving counseling, communication tactics, tinnitus and hyperacusis retraining, advice regarding ear-level noise gen- erators to enable desensitization, relaxation techniques and stress management. Her symptoms had greatly improved at review nine months later, and she was discharged. She was referred again to our clinic by her general practi- tioner five years later. Her primary complaint was recurrent one- hour episodes of painful cutaneous erythema of the right exter- nal ear (Figures 1 and 2) that was associated with severe right temporal pain radiating down to the mastoid area with transient subjectively reduced hearing, right conjunctival injection, intol- erance to noise and light, which was exacerbated during these episodes; the latter symptom was suggestive of involvement of pathways outside the auditory pathway. These symptoms caused our patient considerable distress, resulting in weekly attendance for three months at her general practitioner’s clinic, in addition to presentation at the local emergency departments and to ear, nose and throat clinics, prior to referral to our department. During the previous three months, she had also experienced continuous headaches and fatigue with occasional light-headedness during episodes of erythematous ear. She reported no nausea, visual field symptoms, tinnitus or vertigo. Differential diagnoses of dermatological, temporoman- dibular joint, dental, pharyngeal and cervical problems were excluded on the basis of a head and neck examination. On in- spection, there was no evidence of erythema or of infection in the ear or mastoid area. The otoscopy findings were normal. A neuro-otological examination was unremarkable, including extra-ocular eye movements, cranial nerves, cerebellar function and clinic room balance tests. Pure-tone audiometry and tym- panometry showed normal hearing and middle-ear function. Magnetic resonance imaging of the brain was normal. Routine blood tests were negative. She was diagnosed with RES associ- ated with hyperacusis. She was reassured that she had no major structural pathol- ogy. There were some migrainous features in her medical history. Management of her migraine included starting behavioral modi- fications, such as reducing caffeine intake, stress reduction, op- timizing fluid intake, improving sleep pattern, relaxation tech- niques and starting exercise. She was advised to keep a symptom diary to identify further triggers. She was offered migraine prophylaxis but declined it. On review after four months of supportive measures, she was feeling much better, with complete resolution of her headaches and much reduced frequency of episodes of erythema- tous ear. She reported absence of pain in the ear and less sensation of swelling. Triggers for red ear episodes identified from her symptom diary included stress and, surprisingly, orange juice. Her symptoms were managed successfully without medication for four years. Fig. 1: Normal appearance of the right ear of the patient (photograph taken by patient). Fig. 2: Right ear during the patient’s “red ear” episode (photograph taken by patient). 2  |  Issue-2 MEDICAL EXCELLENCE