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Year : 2020  |  Volume : 8  |  Issue : 1  |  Page : 27-28

Management of autoimmune ear disease and idiopathic polyarthritis with adalimumab

1 Consultant Rheumatologist, Apollo Hospital, Mysore, Karnataka, India
2 Consultant ENT Surgeon, Neha ENT Centre, Ramanuja Road, Mysore, Karnataka, India
3 Medical Advisor, Zydus Cadila Health Care Ltd., Ahmedabad, Gujarat, India

Date of Submission13-Jul-2019
Date of Decision23-Jul-2019
Date of Acceptance08-Aug-2019
Date of Web Publication14-Jan-2020

Correspondence Address:
Dr. Shiva Prasad
Apollo Hospital, Mysore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AJIM.AJIM_29_19

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Autoimmune inner ear disease (AIED) is a rare form of sensorineural hearing loss of autoimmune origin and responding to immunosuppressive therapy with corticosteroids or other immunosuppressants. Novel treatment approaches include intratympanic steroids and biological response modulators against tumor necrosis factor-α. We present a case of AIED, who concomitantly developed seronegative inflammatory polyarthritis (IPA). The treatment for IPA was initiated with methotrexate, hydroxychloroquine, and intra-articular methyl prednisolone. When there was no adequate response, the patient was started on adalimumab, which in addition to IPA also showed beneficial effects on AIED.

Keywords: Adalimumab, arthritis, autoimmune ear disease

How to cite this article:
Prasad S, Malloli JR, Sudhakar A. Management of autoimmune ear disease and idiopathic polyarthritis with adalimumab. APIK J Int Med 2020;8:27-8

How to cite this URL:
Prasad S, Malloli JR, Sudhakar A. Management of autoimmune ear disease and idiopathic polyarthritis with adalimumab. APIK J Int Med [serial online] 2020 [cited 2021 Jul 29];8:27-8. Available from: https://www.ajim.in/text.asp?2020/8/1/27/275978

  Introduction Top

Autoimmune inner-ear disease (AIED) is a rare autoimmune disorder first described in 1979 wherein the affected patient has progressive bilateral asymmetric fluctuating sensorineural hearing loss (SNHL) that manifests over several weeks to months.[1] Around half of the all AIED patients also have vestibular symptoms, tinnitus, and aural fullness.[2] AIED is one of the few variants of SNHL that actually responds to treatment.[3] Thus, proper diagnosis and institution of appropriate treatment can improve the quality of life of the AIED patient. The diagnosis of AIED depends on demonstration of progressive SNHL, ruling out of other etiologies of SNHL, demonstration of nonspecific markers of autoimmune disease by blood tests, erythrocyte sedimentation rate (ESR), and autoantibodies.[4] Probably, the best proof of a diagnosis of AIED is an improved hearing response to immunosuppressive therapy with corticosteroids. In addition, biologics modulating tumor necrosis factors (TNF)-α such as etanercept and adalimumab have also shown to benefit AIED.[2]

We present a case of an AIED patient who developed inflammatory polyarthritis (IPA), and adalimumab initiation improved both the concomitant conditions.

  Case Report Top

A 44-year-old female presented with complaints of joints pain, involving both small and large limb joints, associated with swelling and early morning stiffness lasting for 3–4 h, for 6 months. Spine, shoulder, and pelvic girdle involvement or extra-articular features were absent. She was a known case of AIED of the left ear for 6 years and showed good response with intratympanic steroid whenever she had the attacks. With the onset of arthritis, the frequency and severity of hearing loss/tinnitus/giddiness had increased. The proximal interphalangeal, metacarpophalangeal, metatarsophalangeal joints and joints of wrist, ankle, and knee were diffusely swollen and tender. There were no deformities, vasculitic lesions or nodules. The otological examination was normal. Audiometry revealed 90% SNHL on the left side. There was no other neurological abnormality. Rest of the systemic examination and vitals were within normal limits. Laboratory investigations revealed elevated ESR (58 mm) and C-reactive protein (24 mg/L). Hemogram, liver function test, and renal function tests were normal. Inner ear magnetic resonance imaging was normal. Rheumatoid factor, antinuclear antibody (IF), antineutrophil cytoplasmic antibody (IF), and anti-cyclic citrullinated peptide antibodies were negative. C3 and C4 were within normal limits. A diagnosis of seronegative idiopathic polyarthritis (IPA) with active disease (DAS-28: 7.55) was made; the patient was started with methotrexate (10 mg weekly, later increased to 25 mg weekly over 3 months), hydroxychloroquine (200 mg daily), and intermittent intra-articular/intramuscular steroids as per disease activity. Over the next 6 months, the patient showed partial response to the treatment: severity of the disease gradually decreased, but the disease persisted (DAS-28: 4.82). The patient was initiated on adalimumab after obtaining negative serology for hepatitis B virus, hepatitis C virus, and HIV. Mantoux and interferon gold tuberculosis tests were negative. Subcutaneous adalimumab (Exemptia, Zydus Cadila) was initiated at 40 mg once fortnightly. Follow-up after 4 weeks the hearing/tinnitus/giddiness had also significantly improved. There was a significant improvement in the IPA with the disease being in remission (DAS-28: 1.62). After 1 year of follow-up, the patient is in remission for IPA and also AIED. Repeat audiometry did not show any change from the baseline.

  Discussion Top

Immune-mediated inner ear disease includes clinical conditions associated with rapidly progressive unilateral or bilateral SNHL. A systemic autoimmune disorder may be present in less than one-third of cases. AIED has been reported to occur in association with autoimmune diseases [Table 1].[5] A case of AIED with rheumatoid arthritis (RA) responding to adalimumab was reported in 2010 had arthritis as the initial symptom, and the patient later developed SNHL, diagnosed as AIED.[6] By contrast, our case had AIED first and then developed arthritis.
Table 1: Systemic autoimmune diseases associated with autoimmune inner ear disease

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The role of anti-TNF agents in inflammatory polyarthritis is known. However, its role in AIED is controversial. A retrospective case series reported improvement or stabilization of hearing and tinnitus in 92% of patients with AIED when treated with subcutaneous etanercept 25 mg twice weekly for 6 months.[7] By contrast, in an open-label prospective study, only 7/23 patients (30%) with bilateral immune-mediated cochleovestibular disorders treated with etanercept for 24 weeks showed improvement in audiometric criteria.[8] Infliximab and rituximab have also failed to show any positive therapeutic response. The role of plasmapheresis is also controversial. At present, only anti-TNF agents (etanercept and adalimumab) have proven efficacy in refractory cases of AIED with RA.

Our case is interesting as the patient had unilateral SNHL, and developed arthritis later in the course. She responded to disease-modifying antirheumatic drugs partially and is in remission after initiating therapy with adalimumab. Our case demonstrates the efficacy of adalimumab in the management of IPA with AIED that did not respond to steroids/disease-modifying drugs. Further studies into the use of adalimumab in patients with IPA and AIED are warranted.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979;88:585-9.  Back to cited text no. 1
Mijovic T, Zeitouni A, Colmegna I. Autoimmune sensorineural hearing loss: The otology-rheumatology interface. Rheumatology (Oxford) 2013;52:780-9.  Back to cited text no. 2
Buniel MC, Geelan-Hansen K, Weber PC, Tuohy VK. Immunosuppressive therapy for autoimmune inner ear disease. Immunotherapy 2009;1:425-34.  Back to cited text no. 3
Lobo DR, García-Berrocal JR, Ramírez-Camacho R. New prospects in the diagnosis and treatment of immune-mediated inner ear disease. World J Methodol 2014;4:91-8.  Back to cited text no. 4
Bovo R, Aimoni C, Martini A. Immune-mediated inner ear disease. Acta Otolaryngol 2006;126:1012-21.  Back to cited text no. 5
Morovic Vergles J, Radic M, Kovacic J, Salamon L. Successful use of adalimumab for treating rheumatoid arthritis with autoimmune sensorineural hearing loss: Two birds with one stone. J Rheumatol 2010;37:1080-1.  Back to cited text no. 6
Rahman MU, Poe DS, Choi HK. Etanercept therapy for immune-mediated cochleovestibular disorders: Preliminary results in a pilot study. Otol Neurotol 2001;22:619-24.  Back to cited text no. 7
Matteson EL, Choi HK, Poe DS, Wise C, Lowe VJ, McDonald TJ, et al. Etanercept therapy for immune-mediated cochleovestibular disorders: A multi-center, open-label, pilot study. Arthritis Rheum 2005;53:337-42.  Back to cited text no. 8


  [Table 1]


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