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Autoimmune ear disease, an audit in a Tertiary Centre

 Department of Rheumatology, PD Hinduja Hospital, Mumbai, Maharashtra, India

Date of Submission31-Jul-2021
Date of Acceptance22-Oct-2021
Date of Web Publication01-Jul-2022

Correspondence Address:
Asna shaikh,
PD Hinduja Hospital, Mahim, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_167_21

How to cite this URL:
shaikh A, Balakrishnan C. Autoimmune ear disease, an audit in a Tertiary Centre. Indian J Rheumatol [Epub ahead of print] [cited 2022 Dec 5]. Available from:

Dear Editor,

Autoimmune ear disease (AIED) is an enigmatic ear disease referred to rheumatologists for 2 reasons 1) To see if there is a systemic auto-immune disease underlying it (secondary AIED– 15%–30%) and 2) to help manage the immunosuppressive therapy. The classic clinical features of AIED are rapidly progressive onset over weeks or months (3 and 90 days) with bilateral, asymmetrical SNHL (sensorineural hearing loss) with or without tinnitus.[1] Most patients are steroid responsive and reversible. Thorough clinical examination and work up like Rheumatoid factor, Anti-Cyclic Citrullinated peptide antibody, Anti-Neutrophil Cytoplasmic Antibodies, Anti-Nuclear Antibody, Erythrocyte sedimentation rate, C-reactive protein, serology for syphilis and MRI (Magnetic resonance imaging) brain to rule out retro-cochlear lesions are advised.

Over the past two decades, we have encountered 6 patients with primary AIED [Table 1]. All six patients were diagnosed at baseline with Audiometry documented, gradually progressive asymmetrical SNHL, secondary causes were ruled out. All of them had partial response to steroids. 50% of patients had vestibular involvement in form of tinnitus and giddiness as well. Inflammatory markers were within the normal limits in all of our patients. All 6 patients were treated with steroids and out of them 3 additionally received methotrexate[2] as a steroid sparer with good response. One patient was treated with intra-tympanic steroids.
Table 1: Clinical profile of patients presenting with AIED

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Complete recovery of the hearing loss was not seen in any of our patients. Two patients had relapsed on stopping the treatment. Audiometry at baseline and regular monitoring on treatment with prednisolone 1 mg/kg for 4 weeks is advised. Literature supports the use of methotrexate, cyclophosphamide and tumor necrotic factor inhibitors have also been tried in selected situations. The cochlear implant is advised for those with irreversible hearing loss.

The reason for this communication is to spread awareness among treating otorhinolaryngologist and rheumatologist about this reversible cause of hearing loss so that they may keep high index of suspicion while coming across such patient. This may improve outcomes in an already guarded prognosis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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. 1
Matteson EL, Tirzaman O, Facer GW, Fabry DA, Kasperbauer J, Beatty CW, et al. Use of methotrexate for autoimmune hearing loss. Ann Otol Rhinol Laryngol 2000;109:710-4.  Back to cited text no. 2


  [Table 1]


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