British Journal of Rheumatology 1992;31:711-713

CASE REPORT

SENSORINEURAL HEARING LOSS IN JUVENILE CHRONIC ARTHRITIS BY P. J. DEKKER AND A. H. ISDALE Departments of ENT Surgery and Rheumatology, York District Hospital, Wigginton Road, York YO3 7HE

SUMMARY An 18-year-old female with pauciarticular juvenile chronic arthritis is described who has developed a profound bilateral sensorineural hearing loss. The association of sensorineural hearing loss with juvenile chronic arthritis has not been reported before. KEY WORDS:

Juvenile chronic arthritis, Sensorineural hearing loss.

CASE REPORT NM, an 18-year-old female, first presented in 1972 at the age of 8 months with swinging fever, a swollen left knee, raised erythrocyte sedimentation rate (ESR) and a neutrophil leucocytosis. Septic arthritis was suspected despite negative cultures and she settled on cloxacillin therapy. However, 2 months later she re-presented with a swollen left knee, sore eyes and a rash. A diagnosis of Still's disease was made. Prednisolone was started with a good clinical response. Over the following year she had recurrent swelling in the knees and right foot which responded rapidly to 20 mg prednisolone daily. She remained on maintenance prednisolone but in December 1974 at the age of 2i she developed acute right iridiocyclitis with an hypopyon and this marked the beginning of frequent and severe episodes of iridocyclitis in the right eye. She required frequent subconjunctival, and at times retrobulbar, Depo-Medrone to control her eye disease. Additionally, episcleritis, keratitis and band keratopathy developed in the right eye. In January 1977 her right cornea perforated and although it was sutured, any remaining vision was lost. During this time she continued with intermittent pauciarticular arthritis which responded rapidly to boosting prednisolone dosage but as a result she developed a cushingoid appearance and was growth retarded. Penicillamine

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Submitted 3 May; revised version accepted 6 September 1991. Correspondence to Mr P. Dekker, FRCS, Department of ENT, Leeds General Infirmary, Great George Street, Leeds LSI 3EX.

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FIG. 1.—Pure tone audiogram: O, right ear; X, left ear.

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was started in March 1976 in an attempt to reduce steroid requirements but was stopped 2 years later as no beneficial response was obtained. She continued having recurrent iritis in the blind right eye but it was not until October 1978 that shefirstdeveloped iritis in the left eye which recurred frequently thereafter. A year later the right eye was enucleated in the hope that this would reduce the attacks in her left eye by removing a source of immune complexes. Histological examination of the eye at the Institute of Ophthalmology showed sclero-kerato-uveitis with abscess and a cataract. Despite enucleation she continued to have frequent severe iritis in the left eye and in February 1981 developed a left-sided cataract. Unfortunately she then sustained trauma to the left eye which resulted in a limbal staphyloma. She received a corneal graft and although this was complicated by infection, the graft took, but vision was by this time severely limited. From 1981 to 1986 she remained reasonably stable on 12.5 mg prednisolone on alternate days, with only occasional episodes of pauciarticular arthritis but with gradual loss of remaining vision from recurrent iritis. It was not until 1986 at the age of 14 years that shefirstcomplained of a hearing problem. Up until this time, she had progressed normally as regards her speech development and a hearing deficit had never been suspected. There was no

PAUCIARTICULAR juvenile chronic arthritis (JCA) is the most frequent presentation of chronic arthritis in children. Although a number of subgroups are described, those children with antinuclear factor (ANF) are at high risk of developing chronic iridocyclitis. In this particular subgroup the eye disease has a much greater morbidity than the joint disease which, although often recurrent during childhood, has a good functional outcome. Although sensorineural hearing loss has been described in association with a number of rheumatological conditions, it has not previously been reported in JCA. In the absence of auditory toxins an autoimmune aetiology is presumed.

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family history of hearing impairment. Pure tone audiometry in 1986 revealed a bilateral predominantly high tone sensorineural hearing loss with pure tone averages in the speech frequencies (125-500 Hz) of 25 dB in the right ear and 35 dB in the left ear (Fig. 1). Impedance audiometry did not show any evidence of middle ear pathology at this time. There was no history of head trauma and no history of intercurrent illness. Her hearing remained stable until the following year when she developed a sudden deterioration in the right ear. Evoked response audiometry demonstrated a profound sensorineural hearing loss with air conduction thresholds of 60-70 dB in the right ear and 40-60 dB in the left ear. Again there was no evidence of middle ear pathology. She was fitted with a hearing aid and given 40 mg prednisolone daily without benefit after a week, so this was increased to 60 mg a day. Although there was a slight improvement in the right ear, hearing in the left ear slowly diminished, despite the addition of azathioprine, until no response could be obtained with pure tone audiometry. During this time she developed rightsided serous otitis media, which necessitated insertion of a Goode's T-tube but this was able to be removed without sequelae in September 1989. By this stage hearing in her right ear had slowly diminished to average pure tone thresholds of 75 dB (Fig. 2). She remained stable until June 1990 when she presented with a 10-day history of right-sided otalgia and total deafness. No responses could be obtained on pure tone audiometry (Fig. 3). On brain stem evoked audiometry, no obvious audiologically evoked potentials could be obtained even in the region of 100 dB using clicks in the 500-1700 Hz range. She was treated with bedrest, carbogen inhalations and prednisolone 75 mg/day but failed to show any improvement in her hearing. CT scan of her temporal bones showed normal patent cochleas with no bony abnormalities and she showed a good response to promontory stimulation suggesting that her hearing problem was cochlear in origin. There has never been any evidence of a vestibular disorder. In view of her blindness, investigation of vestibular function would be complicated and has not been attempted. At presentation she was both rheumatoid factor and ANF negative and has remained so. She is also HL A-B27 negative. There has been no evidence of a vasculitic process either clinically or on laboratory testing. She is antineutrophil cytoplasmic antibody negative. Her renal function has always

DISCUSSION Sensorineural hearing loss is reported in up to 29.4% of patients with rheumatoid arthritis (RA) [1] and is reported in other connective tissue diseases including systemic lupus erythematosus [2, 3], polyarteritis nodosa [4], relapsing polychondritis [5] and Sjogren's syndrome [6]. A recent case report described the occurrence of sensorineural deafness in a patient with adult-onset Still's disease which responded completely to high dose prednisolone therapy [7], but it has not been described in JCA. The mechanism is not clear. Lenhardt put forward the concept of inner ear autoimmunity in 1958 [8], but it was not until 1979 that McCabe [9] proposed autoimmune sensorineural hearing loss as a distinct entity. He collected 18 patients with sensorineural hearing loss over a 10-year period at the University of Iowa who did not fit into any previously delineated diagnostic categories and who had certain features in common. These included an end organ-type sensorineural hearing loss which was typically bilateral but usually asymmetrical, often with bilateral symmetrical balance organ involvement but without vertigo. Five patients had a unilateral facial palsy and in a few tissue destruction of the middle ear and surrounding structures occurred. All responded to a combination of dexamethasone and cyclophosphamide and of thefivepatients

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been normal and she has never had proteinuna. Muscle biopsies and angiography have never been performed. She has received no ototoxic drugs apart from salicylates and these were administered in modest doses earlier in the course of the disease before she developed deterioration in her hearing. Currently she is maintained on prednisolone 12.5 mg on alternate days. Although she has recurrent episodic arthritis it is virtually confined to the left knee and right elbow and settles rapidly with intra-articular injections and rest or higher doses of prednisolone. She has no functional joint problems but is of short stature and remains cushingoid. She is completely deaf and blind and she is presently under consideration for cochlear implantation.

DEKKER AMD ISDALE: SENSORINEURAL HEARING LOSS IN JCA

Additionally, the cases of sensorineural hearing loss that have been described in rheumatoid arthritis have all been mild and usually asymptomatic. It remains to be seen whether cochlear implantation will give our patient any useful hearing. REFERENCES

1. Elwany S, Elgarf A, Kamel T. Hearing and middle ear function in rheumatoid arthritis. J Rheumatol 1986;13:878-81. 2. Bowan CA, Linthicum FH, Nelson RA, Mikari K, Quismorio F. Sensorineural hearing loss associated with systemic lupus erythematosis. Otolaryngol Head Neck Surg 1986;94:197-204. 3. Cardarelli DD, Regowski JE, Corey JP. Sensorinerual hearing loss in lupus erythematosis. Am J Otolaryngol 1986;7:210-13. 4. Jakus HA, Pollack AM, Fisch U. Polyarteritis as a cause of sudden deafness. Am J Otalaryngol 1981;2:99-107. 5. Damiani JM, Levin HL. Relapsing polychondritis— report of 10 cases. Laryngoscope 1979;89:929-46. 6. Doig JA, Whaley K, Dick WC, Nuki G, Williamson J, Buchanan WW. Otolaryngological aspects of Sjogren's syndrome. Br Med J 1971;4:460-3. 7. Markusse HM, Stolk B, vol May AGL, de Jong Bok JM, Heering KJ. Sensorineural hearing loss in adult onset Still's disease. Ann Rheum Dis 1988;47:600-2. 8. Lenhaldt E. Plotzliche Horstorungen aud beiden sieten gleichseitig oder nacheinander aufgetreten. Z Laryng Rhino 1958;37:1-16. 9. McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88:585-9.

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with facial palsy, four had complete recovery and one virtual complete recovery. Only one patient (who had tissue destruction) had a biopsy and this showed a vasculitis. No specific details are given other than on his initial case. During the time of collection a lymphocyte inhibition assay using inner ear antigen was developed which proved positive in all six cases tested and negative in some control patients. Further work has not been forthcoming and it remains a rather nebulous, ill-defined and rare condition. The inaccessibility of the inner ear for biopsy material limits delineation of the pathology but a vasculitis with an immune complex deposition seems plausible. Genetic hearing loss is unlikely in view of the late age of onset with normal speech development and a negative family history. Unfortunately prior to 1986 she had not had routine audiometry performed. The patient we have described has had no evidence of vasculitis but she has had an unusual course with an ANF negative severe recurrent anterior uveitis which remained unilateral for 6 years. Despite ophthalmic vigilance this has left her blind, a not uncommon sequel. As one would expect in pauciarticular JCA she has not developed deforming arthropathy and her joints have not posed functional problems. Unlike the patient with adult-onset Still's disease described by Markusse et al, [7] our patient failed to respond to immunosuppressive therapy on either occasion. Although it could be argued that she received this too late to rescue hearing, one might have expected that further deterioration would have been prevented.

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Sensorineural hearing loss in juvenile chronic arthritis.

An 18-year-old female with pauciarticular juvenile chronic arthritis is described who has developed a profound bilateral sensorineural hearing loss. T...
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