Indian Pediatrics 2000;37: 1274-1276.
Onset Profound Deafness in Association with Antiphospholipid
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.
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.