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• W2808555715 abstract "Giant cell arteritis (GCA)—also known as temporal arteritis—is a systemic vasculitis that involves medium and large-caliber arteries such as the cranial arteries, great vessels, and aorta. Although the typical nonocular features of GCA are well known (e.g., new-onset headache, scalp tenderness, jaw claudication), vestibulocochlear presentations are rare and can mimic acute idiopathic sensorineural hearing loss (AISHL).1Amore-Dorado J.C. Llorca J. Garcia-Porrua C. Costa C. Perez-Fernandez N. Gonzalez-Gay M.A. Audiovestibular manifestations in giant cell arteritis: a prospective study.Medicine (Baltimore). 2003; 82: 13-26Crossref PubMed Scopus (71) Google Scholar Although steroids are given for both AISHL and GCA, the lower dose and shorter duration of steroid treatment in AISHL can obscure and delay the diagnosis of GCA.2Narváez J. Bernad B. Roig-Vilaseca D. et al.Influence of previous corticosteroid therapy on temporal artery biopsy yield in giant cell arteritis.Semin Arthritis Rheum. 2007; 37: 13-19Crossref PubMed Scopus (128) Google Scholar We report a case of GCA that presented with acute sensorineural hearing loss associated with eye pain and tenderness. Clinicians should be aware that AISHL is a diagnosis of exclusion, and the presence of ocular manifestations such as eye pain,should prompt consideration for GCA in the elderly. A 74-year-old Caucasian male presented with acute sensorineural hearing loss of the left ear. Past medical history was significant for gross total resection of cutaneous squamous cell carcinoma above the left ear. He had prior mild to moderate, symmetric, age-related sensorineural hearing loss treated with hearing aids. Ocular history was significant for bilateral laser iridotomy for narrow angles. Additionally, he had a prior history of migraines, hypertension, and hyperlipidemia, which were treated medically. The patient initially developed sudden, acute-onset, left-sided hearing loss superimposed on his prior mild to moderate, chronic, bilateral hearing loss. This was followed by left eye pain that was tender to touch. He denied headache, jaw claudication, fever, arthralgia, malaise, loss of appetite, tenderness around the temporal artery, or visual disturbances. A general ophthalmologist found a normal eye examination. Over the next few days, the patient developed worsening vestibular symptoms, which included intermittent dizziness and vertigo. The patient subsequently was seen by an otolaryngologist who found new-onset, asymmetric, low-frequency (250 Hz) sensorineural hearing loss in the left ear. This acute hearing loss was a new finding compared with his prior audiograms, which had shown mild to moderate, age-related, sensorineural hearing loss bilaterally. Magnetic resonance imaging (MRI) of the brain found minimal nonspecific T2 hyperintensity signals in the midbrain and superior pons likely from small vessel ischemic disease, but imaging was otherwise unremarkable for internal auditory canal findings. A preliminary diagnosis of acute idiopathic (presumed autoimmune-related) sensorineural hearing loss was made, and the patient was treated with a 60 mg dose of prednisone tapered over the course of 15 days. The steroid treatment led to the resolution of the patient’s left eye pain, vestibular symptoms, and acute hearing loss. A repeat audiogram after corticosteroid treatment found a return to his chronic baseline. Two weeks after his last dose of steroids, however, the patient returned to his ophthalmologist for recurrent eye pain. The eye examination was unremarkable, but serum erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were mildly elevated at 29.0 mm/hr (normal range, 0–22.0 mm/hr) and 4.20 mg/dL (normal range, 1.0–3.0 mg/L) respectively, despite the 2-week course of steroid therapy the patient received before the laboratory testing. The patient was referred to the Houston Methodist Hospital neuro-ophthalmology service. The neuro-ophthalmic examination was normal, but a left temporal artery biopsy revealed irregular intimal hyperplasia with focal segmental absence of the elastic lamina and CD-68 positive macrophages at the junction of the muscularis and the internal elastic lamina, compatible with healed or treated GCA (Fig. 1A−D). Serial repeat ESR and CRP tests were normal, and there was no recurrence of the hearing loss, vestibular symptoms, or eye pain off steroid therapy. AISHL can be fluctuating or progressive in nature and often presents unilaterally.3Rauch S.D. Clinical practice. Idiopathic sudden sensorineural hearing loss.N Engl J Med. 2008; 359: 833-840Crossref PubMed Scopus (247) Google Scholar, 4Mijovic T. Zeitouni A. Colmegna I. Autoimmune sensorineural hearing loss: the otology-rheumatology interface.Rheumatology. 2013; 52: 780-789Crossref PubMed Scopus (67) Google Scholar The symptoms typically develop acutely (less than 72 hours), which helps distinguish autoimmune hearing loss from the more common cause of hearing loss in the elderly, presbycusis.3Rauch S.D. Clinical practice. Idiopathic sudden sensorineural hearing loss.N Engl J Med. 2008; 359: 833-840Crossref PubMed Scopus (247) Google Scholar, 4Mijovic T. Zeitouni A. Colmegna I. Autoimmune sensorineural hearing loss: the otology-rheumatology interface.Rheumatology. 2013; 52: 780-789Crossref PubMed Scopus (67) Google Scholar AISHL affects 5 to 20 per 100 000 adults between the ages of 40 to 60 years annually.3Rauch S.D. Clinical practice. Idiopathic sudden sensorineural hearing loss.N Engl J Med. 2008; 359: 833-840Crossref PubMed Scopus (247) Google Scholar Although up to 70% of patients recover some function spontaneously, AISHL is considered an otologic emergency requiring prompt referral and steroid treatment. AISHL, however, is a diagnosis of exclusion, and up to 16% of patients have an underlying autoimmune or neurologic etiology.3Rauch S.D. Clinical practice. Idiopathic sudden sensorineural hearing loss.N Engl J Med. 2008; 359: 833-840Crossref PubMed Scopus (247) Google Scholar MRI with gadolinium is typically recommended for all patients with AISHL, but the potential need for other testing (including ESR and CRP) is less well known.3Rauch S.D. Clinical practice. Idiopathic sudden sensorineural hearing loss.N Engl J Med. 2008; 359: 833-840Crossref PubMed Scopus (247) Google Scholar Our report illustrates the case of a patient with GCA who presented with a clinical picture suggestive of a diagnosis of AISHL. Although many cases of GCA presenting with concomitant sensorineural hearing loss and vestibular symptoms have been reported in the literature, our case is unique in that our patient also presented with unilateral eye pain and tenderness. Table 1 summarizes the prior case reports of patients with GCA who presented with acute sensorineural hearing loss.58 Clinicians should be aware that GCA can mimic AISHL in the elderly. Although AIHSL is predominantly unilateral, GCA should be included in the differential for elderly patients who present with acute, unilateral, or bilateral sensorineural hearing loss. The presence of concomitant ocular symptoms should prompt consideration for an ESR, CRP, and a temporal artery biopsy in patients with presumed AISHL. Although both AISHL and GCA are treated with steroids, the markedly shorter duration and lower dose regimen used in the treatment of AISHL may be insufficient to treat GCA, can delay the diagnosis of GCA, and potentially cause irreversible blindness—the most feared complication of GCA.Table 1Summary of patients with GCA presenting with acute unilateral sensorineural hearingReferencePatientSexAge (years)FeverHeadacheJaw ClaudicationTemporal Artery TendernessEye Pain or TendernessVisual DisturbanceSensorineural Hearing LossVertigoTemporal Artery Biopsy ResultsFrancis and Boddie5Francis D.A. Boddie H.G. Acute hearing loss in giant cell arteritis.Postgrad Med J. 1982; 58: 357-358Crossref PubMed Scopus (13) Google Scholar1M59−+−−−−+−−McKennan et al.6McKennan K.X. Nielsen S.L. Watson C. Wiesner K. Meniere’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis).Laryngoscope. 1993; 103: 1103-1107PubMed Google Scholar2F84−−−−−++++McKennan et al.6McKennan K.X. Nielsen S.L. Watson C. Wiesner K. Meniere’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis).Laryngoscope. 1993; 103: 1103-1107PubMed Google Scholar3F85−−−−−−+++McKennan et al.6McKennan K.X. Nielsen S.L. Watson C. Wiesner K. Meniere’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis).Laryngoscope. 1993; 103: 1103-1107PubMed Google Scholar4F79−+−+−−+++Hausch and Harrington7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar5F76−+−−−++−+Hausch and Harrington7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar6F76−−++−−+−+Hausch and Harrington7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar7F62++−−−++−+Hausch and Harrington7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar8F59++−+−−+−+Loffredo et al.8Loffredo L. Parrotto S. Violi F. Giant cell arteritis, oculomotor nerve palsy, and acute hearing loss.Scand J Rheumatol. 2004; 33: 279-280Crossref PubMed Scopus (16) Google Scholar9F80−+−−−−+−+Le et al. 201810M74−−−−+−+++Age, sex, clinical features, and temporal artery biopsy results of 10 patients diagnosed with GCA who presented with sensorineural hearing loss. The data from patients 1–9 were obtained via a review of the literature (from 1982–2017), and patient 10 depicts information about our patient.5Francis D.A. Boddie H.G. Acute hearing loss in giant cell arteritis.Postgrad Med J. 1982; 58: 357-358Crossref PubMed Scopus (13) Google Scholar, 6McKennan K.X. Nielsen S.L. Watson C. Wiesner K. Meniere’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis).Laryngoscope. 1993; 103: 1103-1107PubMed Google Scholar, 7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar, 8Loffredo L. Parrotto S. Violi F. Giant cell arteritis, oculomotor nerve palsy, and acute hearing loss.Scand J Rheumatol. 2004; 33: 279-280Crossref PubMed Scopus (16) Google ScholarThe “+” symbol illustrates the presence of a symptom, and the “−” symbol illustrates the absence of such.The table represents cases of GCA documented between 1982 and 2017 but does not report all cases of GCA presenting with bilateral hearing loss. Open table in a new tab Age, sex, clinical features, and temporal artery biopsy results of 10 patients diagnosed with GCA who presented with sensorineural hearing loss. The data from patients 1–9 were obtained via a review of the literature (from 1982–2017), and patient 10 depicts information about our patient.5Francis D.A. Boddie H.G. Acute hearing loss in giant cell arteritis.Postgrad Med J. 1982; 58: 357-358Crossref PubMed Scopus (13) Google Scholar, 6McKennan K.X. Nielsen S.L. Watson C. Wiesner K. Meniere’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis).Laryngoscope. 1993; 103: 1103-1107PubMed Google Scholar, 7Hausch R.C. Harrington T. Temporal arteritis and sensorineural hearing loss.Semin Arthritis Rheum. 1998; 28: 206-209Crossref PubMed Scopus (32) Google Scholar, 8Loffredo L. Parrotto S. Violi F. Giant cell arteritis, oculomotor nerve palsy, and acute hearing loss.Scand J Rheumatol. 2004; 33: 279-280Crossref PubMed Scopus (16) Google Scholar The “+” symbol illustrates the presence of a symptom, and the “−” symbol illustrates the absence of such. The table represents cases of GCA documented between 1982 and 2017 but does not report all cases of GCA presenting with bilateral hearing loss. The authors have no proprietary or commercial interest in any materials discussed in this article." @default.
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• W2808555715 date "2019-02-01" @default.
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• W2808555715 title "Vestibulocochlear symptoms as the initial presentation of giant cell arteritis" @default.
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