Antiphospholipid syndrome associated with autoimmune hearing loss

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Taqdees Suhail Abbas Ismail

Rheumatology, Volume 57, Issue suppl_3, April 2018, key075.246, doi.org/10.1093/rheumatolog...

Published: 25 April 2018

Background: Autoimmune hearing loss is a very rare initial presentation of antiphospholipid syndrome. It has been suggested that antiphospholipid antibodies disrupt the microcirculation in labyrinthine structure and causes micro thrombi leading to internal ear dysfunction. There is limited literature regarding long term treatment of the associated hearing loss. We present a case of antiphopholipid syndrome presenting as initial autoimmune hearing loss.

Methods: A 62 year old male presented to ENT with bilateral sudden hearing loss. Extensive investigations were undertaken including CT, MRI scan, internal ear exam and autoimmune screen, all of which were negative. He was given a trial short course of high dose of steroids and he responded very well to it with hearing improving. ENT follow up continued with no definitive diagnosis being made for the continued hearing loss as all investigations and tests were negative. The patient started self-medicating with steroids as this would improve the hearing loss. Later, a diagnosis of autoimmune hearing loss was made. He was referred to rheumatology for consideration of immunosuppression therapy but he was discharged as there was limited evidence to support the use of disease modifying anti-rheumatic drugs or biologics for treating autoimmune hearing loss. Three years later the patient suffered a basilar artery and pontine stroke and was found to have very high levels of anticardiolipin antibodies. The patient fulfilled the clinical and laboratory criteria for the diagnosis of antiphospholipid syndrome.

Results: A definitive diagnosis of immune mediated vasculopathy and autoimmune hearing loss secondary to antiphospholipid syndrome was made. He was started on treatment with warfarin. Due to the long-term complications of steroid treatment the decision was made to commence treatment with immunosuppressants. The patient was weaned off steroids. He has responded well to immunosuppressant treatment with azathioprine. The severity of his symptoms have decreased and his hearing loss has improved.

Conclusion: Autoimmune hearing loss is a very rare initial presentation of antiphospholipid syndrome. There is limited evidence to support a definitive treatment regime for this presentation. This case report indicates that immunosuppressive therapy with azathioprine as oppose to long term steroids could be an effective treatment choice to treat sensory hearing loss in patients with antiphospholipid syndrome.

Disclosures: The authors have declared no conflicts of interest.

Source: academic.oup.com/rheumatolo...

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