Acute Hearing Loss in Giant Cell Arteritis D. A. FRANCIS H. G. BODDIE M.R.C.P

Postgrad Med J: first published as 10.1136/pgmj.58.680.357 on 1 June 1982. Downloaded from

Postgraduate Medical Journal (June 1982) 58, 357-358

CLINICAL REPORTS

Acute hearing loss in giant cell arteritis D. A. FRANCIS H. G. BODDIE M.R.C.P. M.R.C.P. Department of Neurology, North Staffordshire Royal Infirmary, Stoke-on-Trent ST4 7LN

Summary Frequency (cycles per sec) A case of giant cell arteritis presenting with acute hearing loss which was reversed by corticosteroid therapy is described -10 0 Introduction 10 The classical picture ofgiant cell arteritis, an elderly /lk patient with headache and an inflamed temporal In 20 U artery, is well known. It is also well recognized that 41 30 N in some patients sudden blindness may be the first sign of the disease (Healey and Wilske, 1977). 0 40 copyright. However, when these cardinal manifestations are 50 1-0' absent or vague and the patient develops an C 60 uncommon symptom, the diagnosis is less likely to C)0 70 be considered. This case illustrates a rare yet impor- I tant presentation of giant cell arteritis which has not 80 been adequately reported in the literature. 90 4 0

100 http://pmj.bmj.com/ Case history 110 1 1 1 4 I -- A 59-year-old man gave a 4-week history of tem- Right ear poral-occipital headache. The pain was constant but FIG. 1. Pre-treatment right audiogram: 24 hr after admission of varying severity being aggravated by leaning showing bilateral sensorineural deafness. ESR 102 mm. (The forward and coughing. Simple analgesia had proved left audiogram was almost identical.) ineffective. No disturbance of vision had occurred but over the preceding 48 hr he had complained of a alpha2 and gamma globulin bands, serum immuno-

discomfort in both ears associated with increasing globulins were normal. on September 26, 2021 by guest. Protected deafness. He looked unwell and was overtly de- Skull and chest radiology, a computerized axial pressed, complaining of profound lethargy, malaise tomographic brain scan and isotope liver scan all and anorexia. proved normal. General examination was normal and his temporal Despite a negative temporal artery biopsy a and occipital arteries were non-tender with normal diagnosis of giant cell arteritis was made on estab- pulsation. Neurological examination revealed bi- lished clinical criteria (Malmvall et al., 1976) and lateral sensorineural deafness which deteriorated prednisolone therapy started at a dose of 80 mg significantly over the subsequent 24 hr (Fig. 1). daily. Within 24 hr his headache had gone and he felt Investigations: Hb 11 9 g/dl, WBC 11-5x109/1, his hearing had returned to normal. Marked im- erythrocyte sedimentation rate 102 mm in the first provement in the latter was confirmed by repeated hour, serum electrolytes normal, liver function tests audiometry performed during full suppression of mildly deranged. Alkaline phosphatase 199 u./l disease activity (Fig. 2). (normal 30-100), gamma glutamyltransferase >400 u./l (5-55), 5-nucleotidase 25 u./I (3-17). Protein Discussion electrophoresis revealed a marked increase in alpha1, Otolaryngological manifestations of giant cell 0032-5473/82/0600-0357 $02.00 © 1982 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.58.680.357 on 1 June 1982. Downloaded from

358 Clinical reports Frequency (cycles per sec) experienced the typical symptoms of bitemporal headaches, fever and weight loss, and also complained of tinnitus and intermittent vertigo. Audio- -10 grams again demonstrated a bilateral sensorineural hearing loss, and marginal improvement was seen 0 following corticosteroid therapy. 10 The mechanism of deafness in this condition is 3 20 probably explained by arteritic involvement of the S 30.- - posterior circulation or terminal cochleovestibular vasculature (Sofferman, 1980). As in the visual com- = 40 plications of giant cell arteritis, its reversibility may i 50 ..- depend upon the early introduction of treatment. This might also explain some of the conflicting reports in the literature regarding the response to 70 steroids of deafness caused by the arteritic process. This case confirms an original report of acute and progressive hearing loss presenting as an early and prominent symptom in this disease. Its reversibility, following early steroid therapy, is clearly demon- 110o strated and by calling attention to this it is hoped the Right ear index of suspicion of giant cell arteritis as a poten- FIG. 2. Post-treatment right audiogram: During prednisolone tially reversible cause of acute hearing loss will be therapy. ESR 17 mm. (The left audiogram showed a similar raised. improvement compared with that pretreatment.) Acknowledgment arteritis are infrequent but well documented. We would like to thank Mr Stuart, Consultant ENT Patients may present with masticatory claudication, surgeon, for his advice and technical assistance. copyright. tongue pain or frank lingual infarction (Sofferman, Acute and irreversible loss References 1980). potentially hearing CODY, D.T. (1971) Rehabilitation for sensorineural hearing however has received scant attention in the literature. loss. In: Clinical Otology: An International Symposium, Deafness was noted in association with giant cell p. 211 (Ed by Paparella, M.M., Hohmann, A. & Huff, arteritis as early as 1946 when one of seven cases J.S.). The CV Mosby Company, Boston. described unilateral followed by bilateral COOKE, W.T., CLOAKE, P.C.P., GOVAN, A.D.T. & COLBECK, developed J.C. (1946) Temporal arteritis: A generalised vascular deafness which resolved spontaneously (Cooke et al. disease. Journal of Medicine, 14, 47.

Quarterly http://pmj.bmj.com/ 1946). A more recent study found five cases out of a HEALEY, G. A. & WILSKE, K.R. (1977) Manifestations of group of 68 patients suffering from giant cell arteritis giant cell arteritis. Medical Clinics of North America, 61, with loss. In each case audiograms revealed 261. hearing MALMVALL, B.A. & BENGTSSON, B.A. (1978) Giant cell a bilateral sensorineural deafness and when these arteritis. Clinical features and involvement of different were repeated, following steroid therapy, no de- organs. Scandinavian Journal ofRheumatology, 7, 154. monstrable improvement was seen (Malmvall and MALMVALL, B.E., BENGTSSON, B.A., KAYSER, B., NILSSON, Bengtsson, 1978). I.A. & APESTIG, K. (1976) Serum levels of immunoglobulin Rapidly progressive hearing loss as a presenting and complement in giant cell arteritis. Journal of the American Medical Association, 236, 1876. on September 26, 2021 by guest. Protected symptom in giant cell arteritis has been reported in SOFFERMAN, R.A. (1980) Cranial arteritis in otolaryngology. one case previously (Cody, 1971). This patient also Annals of Otology, Rhinology and Laryngology, 89, 215.