Giant Cell (Temporal) Arteritis with Persistent Bilateral Sensorineural Hearing Loss – A Likely Consequence of Delayed Institution of Glucocorticoid Therapy

Patient: Male, 66 Final Diagnosis: Giant cell arteritis Symptoms: Hearing loss Medication: — Clinical Procedure: — Specialty: Rheumatology OBJECTIVE: Unusual clinical course BACKGROUND: The classical picture of giant cell (temporal) arteritis (GCA) is not always evident. A wide variety of unusual presentations have been increasingly reported. Sensorineural hearing loss (SNHL) as an initial manifestation of GCA is a rare, yet important entity. Similar to blindness, SNHL also deserves serious attention as timely intervention may play a key role in overall prognosis. Only a few cases of SNHL associated with GCA have been reported in the literature, the majority of them were diagnosed early and responded well to glucocorticoid therapy. Our report focuses on a case of persistent audiometry-proven SNHL despite optimal doses and duration of glucocorticoids required in GCA patients with ischemic complications. CASE REPORT: We present the case of a 66-year-old male with severe bi-temporal headache, left jaw claudication, and progressively worsening hearing loss, the latter symptom reported over the preceding year. Examination of his temporal artery remained insignificant, but the laboratory data showed raised erythrocyte sedimentation rate (ESR) of 52 mm/hour. Audiometry performed and the interpretations were coincided with the bilateral high frequency mild to moderately-severe SNHL. Prompt administration of IV methylprednisolone started at high-optimum doses that were gradually tapered over the subsequent six months. On biopsy of the left temporal artery, the findings were consistent with the GCA. The patient responded well in terms of headache and general condition improvement, but results of repeat audiometry at follow-up visits proved disappointing and the interpretations were that there was no change in the audiometry results based on first presentation. CONCLUSIONS: This case is purposefully reported to draw the attention of practicing physicians, and encourage them not only to better understand atypical presentations of GCA but also to intervene in a timely fashion. This case should encourage literature to reset recommendations and encourage have high-indexed suspicion when elderly patients present with deafness since early diagnosis and treatment may have profound effects on overall long-term prognosis of other cranial ischemic complications as well.