Journal of Clinical Otolaryngology Head and Neck Surgery

The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery

J Clin Otolaryngol Head Neck Surg 2015; 26(2):257-262

pISSN: 1225-0244, eISSN: 2713-833X

DOI: https://doi.org/10.35420/jcohns.2015.26.2.257

증례

Refractory Bilateral Sudden Sensorineural Hearing Loss Associated with Systemic Lupus Erythematosus : Clinical Implication of Plasmapheresis

Ki Hwan Kuak¹, SungHee Kim¹, Seung Woo Han², Eun Jung Lim¹^,^*

¹Department of Otolaryngology-Head and Neck Surgery, Daegu, Korea

²Rheumatology, Daegu Fatima Hospital, Daegu, Korea

^*교신저자: 임정은, 41199 대구광역시 동구 아양로 99 대구파티마병원 이비인후과 전화 :(053) 940-7350·전송:(053) 940-7352 E-mail:ejlim@fatima.or.kr

© Copyright 2015 The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery. This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: Jul 28, 2015; Revised: Sep 07, 2015; Accepted: Oct 08, 2015

Published Online: May 31, 2020

ABSTRACT

Sudden sensorineural hearing loss (SNHL) is a rare manifestation of systemic lupus erythematosus (SLE). A 38-year-old female presented headache followed by bilateral SNHL. The hearing threshold level was 75dB in the right and 45dB in the left ear. In spite of high dose steroid treatment, the hearing of both ears became profound hearing loss in several days. Serologic tests showed positive autoimmune markers corresponding to SLE with suspicious antiphospholipid (antibody) syndrome (APS). Follow up hearing tests showed no improvement in spite of anticoagulant, intravenous immunoglobulin and high dose steroid treatment. The patient underwent two cycles of plasmapheresis followed by immunosuppressant, and the hearing level in left side improved to 60dB after plasmapheresis. We presume that elevated blood viscosity and vasculitis due to immune deposits can cause cochlear vessel occlusion, which is responsible for the hearing loss. Prompt immunosuppressive treatment and, in refractory case, plasmapheresis can possibly reverse the hearing loss. (J Clinical Otolaryngol 2015;26:257-262)

Keywords: Sudden deafness; Antiphospholipid syndrome; Lupus anticoagulant