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Case Reports

Indian Pediatrics 2000;37: 1274-1276.

Sudden Onset Profound Deafness in Association with Antiphospholipid 
Antibodies in a Child with SLE

Kishore S. Agarwal
Jacob M. Puliyel
Uma Khanduri

From the Department of Pediatrics, St. Stephens Hospital, Tis Hazari, Delhi 110 054, India.

Reprint requests: Dr. Jacob M. Puliyel, Department of Pediatrics, St. Stephens Hospital, Tis Hazari, Delhi 110 054, India.

E-Mail: puliyel@vsnl.com

Manuscript received: November 26, 1999;
Initial review completed: January 21, 2000;
Revision accepted: May 19, 2000

Systemic lupus erythematosus (SLE) is associated with a variety of antibodies in the serum. Of late antiphospholipid antibodies have been found to be raised in some cases of SLE and this may be associated with thrombotic phenomena. We report a case of a 12-year-old girl with SLE who developed profound deafness in association with antiphospholipid antibodies.

 Case study

A 12-year-old girl was admitted with a 3 month history of sudden onset profound hearing loss, intermittent fever, oral ulcers, malar rash with photosensitivity and a discoid rash on the back and scalp leading to scaly areas of alopecia. There was no history of ear discharge, recent trauma or preceding intake of drugs that could cause ototoxicity or precipitate SLE. There was no family history of deafness or nephritis. The liver was 3 cm in the right midclavicular line. There was no splenomegaly or lymphadenopathy. Examina-tion of fundus, other cranial nerves and CNS was normal.

Her pure tone audiometery showed bilateral severe sensorineural deafness with no response upto 120 decibels. Her ESR was 124 mm in the first hour. She had ultrasound evidence of ascites, left pleural effusion and pericardial effusion. Blood counts, renal and liver function tests were normal. Test for prothrombin time and partial thromboplastin time (PTTK) were done twice and were normal. CT scan head did not show any abnormality. Urine examination was unremarkable. Antinuclear antibody (ANA) index was 1.8 (normal upto 1). VDRL was negative. Anticardiolipin antibody IgM was 8.9 MPL - U/ml (normal 0.000 -6.00) and IgG was 13.10 GPL - U/ml (normal 0.00 -12). SLE was diagnosed based on the 1982 revised criteria for classification of SLE(1). She was put to oral prednisone 2 mg/kg/day in three divided doses. Her fever and malar rash responded dramatically but there was no improvement in hearing.


Milder degrees of deafness, detected on audiogram, have been demonstrated in 8% of cases of SLE(2). However, profound deafness with SLE is extremely uncommon. There are sporadic case reports of profound deafness with SLE among adults but none in the pediatric age group(3-6).

The antiphospholipid syndrome (APS) was first described by Hughes et al.(7). It is of two types, primary and secondary. Secondary APS is associated with autoimmune disease. APS is characterized by the presence of various autoantibodies whose specificity is directed not only against phospholipid but their complex with plasma proteins(8). Anti-cardiolipin antibodies (aCL) and lupus anticoagulant tests are widely performed to diagnose APS(8). Our case, seen three months after the onset of deafness, had raised IgM and IgG anticardiolipin antibodies level. Lupus anticoagulants were not tested for as PTTK was repeatedly normal. PTTK is, however, a relatively insensitive test and lupus anticoagulant may be positive sometimes even when PTTK is normal. It is said that the anticardiolipin antibodies affect platelet membranes or the endothelium and lead to reduced levels of prostacyclin release and this in turn leads to thrombosis. It is postulated that thrombosis may cause dysfunction of the cochlea and this may result in deafness(2,3). The first case of SLE with anticardiolipin antibody and deafness was described by Hisashi et al.(3). Ours is arguably the first case of the anticardiolipin antibody syndrome with profound deafness to be reported in pediatric literature.

Contributors: KSA and JMP worked up the case, performed the literature search and drafted the paper. UK helped with the hematological work up and interpretation of investigation. JMP will act as a guarantor for this paper.

Funding: None.
Competing interests:
None stated.

Key Messages

  • Secondary antiphospholipid antibody syndrome (APS) may be associated with SLE in children.

  • We report a child with SLE who had sudden onset deafness in association with antiphopholipid antibodies.

  1. Tan EM, Cohen AS, Fries JF, Mari AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of sys-temic lupus erythematosus. Arthritis Rheum 1982; 25: 1271-1277.

  2. Bowman CA, Linthicum FH Jr., Nelson RA, Mikami K, Quismorio F. Sensorineural hear-ing loss associated with systemic lupus erythematosus. Otolaryngol Head Neck Surg 1986; 94: 194-204.

  3. Hisashi K, Komune S, Taira T, Umemura T, Sadoshima S, Tsuda H. Anticardiolipin anti-body induced sudden profound sensorineural hearing loss. Am J Otolarngol 1993; 14: 275-277.

  4. Tumiati B, Casoli P. Sudden sensorineural hearing loss and anticardiolipin antibody. Am J Octolaryngol 1995; 16: 220.

  5. Naarendorp M, Spiera H. Sudden sensorineural hearing loss in patient with systemic lupus erythematosus or lupus like syndromes and antiphospholipid antibodies. J Rheumatol, 1998; 25: 589-592.

  6. Dayal VS, Ellman MH. Sensorineural hearing loss and lupus. J Rheumatol 1999; 26: 2065.

  7. Hughes GRV. Thrombosis, abortion, cerebral disease and the lupus anticoagulant. BMJ 1983; 287: 1088-1089.

  8. Bertolaccini ML, Roch B, Amengual O, Atsumi T, Khamashta MA, Hughes GR. Multiple antiphospholipid tests do not increase the diagnostic yield in phospholipid syndrome. Br J Rheumatol 1998; 37: 1229-1232.


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