we report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement , thrombocytopenia , and consistent autoantibodies . favorable outcome was obtained on high - dose corticosteroids , and the hearing fully recovered . sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present . although management remains empirical , the high risk of permanent hearing impairment seems to justify emergency treatment with high - dose corticosteroids . when the clinical and laboratory criteria of antiphospholipid syndrome are met , antiplatelets agents or anticoagulation therapy shall be considered . sudden sensorineural hearing loss is defined as an acquired hearing deficit of up to 30  db loss in 3 different frequencies on an audiogram , building up over a few hours to up to 3 days . five to 20 per 100,000 population are affected , both male and female , typically between 30 and 60 years of age . in the majority of cases , we report the case of a young woman who presented to our hospital for sudden sensorineural hearing loss that was ultimately attributed to systemic lupus erythematosus . we reviewed the medical literature indexed in the medline database ( accessed on december 28 , 2015 ) . our search key words were :  hearing or sensorineural or deafness and lupus ,  in all fields , only english and french language articles . a 19-year - old woman was referred to our hospital in december 2006 for a fever , a sudden profound bilateral hearing loss and a malar rash that had appeared 10 days before hospitalization . besides hearing loss , physical examination revealed a malar rash , ulcerated stomatitis , enlarged cervical lymph nodes , and subungual hemorrhage . an audiogram showed a hearing threshold of 40  db in the right ear and 60  db in the left ear , both in air and bone conduction . laboratory tests revealed neutropenia ( 540  neutrophils/l ) , thrombocytopenia ( 125,000/l ) , elevated liver enzymes ( aspartate aminotransferase 247  iu / l , 8-fold the upper limit of normal range ; alanine transaminase 116 iu / l , 3-fold the upper limit of normal range ; gamma - glutamyl transferase 166 iu / l , 3-fold the upper limit of normal range and alkaline phosphatase 40 iu / l , normal ) , hyperferritinemia ( 10,965  g / l ) and an elevated titer of lactate dehydrogenase ( 989  iu / l , 3-fold the upper limit of normal range ) , and triglycerides ( 4.96 antinuclear antibodies were positive ( titer 1/2560 ) , with antidouble stranded dna ( 31  iu ) and anti - sm antibodies . low levels of anticardiolipin antibodies were detected ( igg : 27  g / l ) . g / l ) ; 0.1  g / l c4 ( lower limit 0.1 renal biopsy revealed world health organisation class ii lupus nephritis with pure mesangial involvement , with igg , c3 , c1q , and igm deposits on immunofluorescence . there was no alteration of blood vessels ( absence of infiltration , necrosis of the vascular wall , or microscopic thromboangiitis ) , nor proliferation on light microscopy . she was treated with high - dose ( 500  mg ) intravenous methylprednisolone for 3 days , and clinically improved within a few days . long - term treatment with prednisone ( 1  mg / kg / day with gradual decrease ) , hydroxychloroquine and aspirin was introduced . one year later , clinical examination was normal with a complete remission of systemic lupus erythematosus ; mycophenolate mofetil was stopped . secondary antiphospholipid syndrome ( antiphospholipid syndrome ) was excluded on the basis of an uneventful follow - up and the quick fading of anticardiolipin antibodies and lupus anticoagulant that were never detected again over 9 years of follow - up . a second flare occurred in 2011 , with myalgia , arthralgia , fatigue , and hemophagocytic syndrome . this flare was treated by a temporary increase of prednisone dose ; no immunosuppressive therapy was necessary . when last seen in february 2016 , the patient was doing well on 5  mg prednisone and 400  mg hydroxychloroquine daily . this patient presented with bilateral sudden sensorineural hearing loss in the context of a hemophagocytic syndrome , revealing systemic lupus erythematosus , and raising questions about pathophysiology and treatment . sudden sensorineural hearing loss should be clearly distinguished from other sensorineural hearing loss in patients with systemic lupus erythematosus . indeed , systematic audiometric tests have shown that 20% to 55% of systemic lupus erythematosus patients have chronic sensorineural hearing impairment , often asymptomatic . thus , the risk of sensorineural hearing loss seems to be much higher in systemic lupus erythematosus patients compared to healthy age - matched controls , with an odds ratio of 20 ( 95% ci : 2.93139.6 ) . in contrast , sudden sensorineural hearing loss , which is usually a dramatic condition , has been seldom reported in systemic lupus erythematosus patients ( table 1 ) . three pathophysiological hypotheses are proposed for sudden sensorineural hearing loss at the beginning of systemic lupus erythematosus manifestations:.t - cell autoimmunity : as shown patients with sudden sensorineural hearing loss presented significant proliferation of t lymphocytes specifically responsive to human inner ear antigens , as well as increased interferon - gamma and other inflammatory intracellular cytokines levels in peripheral blood.humoral autoimmunity : specific autoantibodies directed against inner - ear antigens have been detected in this context and may be involved in sudden hearing loss.antiphospholipid syndrome : case reports have highlighted the possible association of anticardiolipin antibodies with sudden sensorineural hearing loss in systemic lupus erythematosus . antiphospholipid antibodies ( i.e. , anticardiolipin antibody , lupus anticoagulant , and anti-2 gp1 ) are known to be associated with microcirculation thrombosis and histological proof of microinfarctions has been reported in systemic lupus erythematosus patients with sudden sensorineural hearing loss . high concentrations of antiphospholipid antibodies have been reported in case reports of lupus - associated sudden sensorineural hearing loss , with satisfying clinical response to anticoagulant therapy . whether the presence of antiphospholipid antibodies in patients presenting sudden sensorineural hearing loss is sufficient to establish a diagnosis of secondary antiphospholipid syndrome in the absence of other symptoms ( i.e. , thromboembolic or obstetrical complications ) remains to be determined . t - cell autoimmunity : as shown patients with sudden sensorineural hearing loss presented significant proliferation of t lymphocytes specifically responsive to human inner ear antigens , as well as increased interferon - gamma and other inflammatory intracellular cytokines levels in peripheral blood . humoral autoimmunity : specific autoantibodies directed against inner - ear antigens have been detected in this context and may be involved in sudden hearing loss . antiphospholipid syndrome : case reports have highlighted the possible association of anticardiolipin antibodies with sudden sensorineural hearing loss in systemic lupus erythematosus . antiphospholipid antibodies ( i.e. , anticardiolipin antibody , lupus anticoagulant , and anti-2 gp1 ) are known to be associated with microcirculation thrombosis and histological proof of microinfarctions has been reported in systemic lupus erythematosus patients with sudden sensorineural hearing loss . high concentrations of antiphospholipid antibodies have been reported in case reports of lupus - associated sudden sensorineural hearing loss , with satisfying clinical response to anticoagulant therapy . whether the presence of antiphospholipid antibodies in patients presenting sudden sensorineural hearing loss is sufficient to establish a diagnosis of secondary antiphospholipid syndrome in the absence of other symptoms ( i.e. , thromboembolic or obstetrical complications ) remains to be determined . when reviewing the english and french literature in the medline database , we found 22 documented cases of sudden sensorineural hearing loss in association with systemic lupus erythematosus ( table 1 ) . hearing loss was the first manifestation of systemic lupus erythematosus in four cases . in 11 cases major systemic involvement of systemic lupus erythematosus such as renal , cardiac , or neurological involvement was also reported . besides hearing loss , other reported aural symptoms included : middle ear involvement , otitis , and in 9 cases , vestibular symptoms . when tested , anticardiolipin antibodies , and/or lupus anticoagulant and/or isolated syphilis reaginic antibody proved positive in 11 out of 15 cases . ten patients had had a brain ct - scan or mri : among them , 3 had signs of central nervous system ischemia . however , pathological data were available in three cases , showing middle ear lesions such as vasculitis and nonspecific inflammation . twelve patients were treated with steroids ( 0.51  mg / kg equivalent prednisone , 1 was treated with 500  mg methylprednisonolone on 3 consecutive days ) , 4 patients were prescribed immunosuppressive therapy with azathioprine or cyclophosphamide , 3 were treated with plasmapheresis and 2 with hydroxychloroquine . clinical outcome was reported for 13 patients : recovery from hearing loss was complete in only 4 patients and partial in 2 other cases . a recent publication of a retrospective cohort study from the taiwan national health insurance research database reported 27 cases of sudden sensorineural hearing loss associated with systemic lupus erythematosus , but no detail on clinical and biological data was available . we conclude that sudden sensorineural hearing loss can be the presenting clinical feature of systemic lupus erythematosus and may be associated with antiphospholipid antibodies . although management of such cases remains empirical , the high risk of permanent hearing loss seems to justify emergency treatment with high dose corticosteroids . if the laboratory criteria of antiphospholipid syndrome are present , antiplatelets agents or anticoagulation therapy shall be considered .
abstractintroduction : sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus.case report : we report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement , thrombocytopenia , and consistent autoantibodies . 
 favorable outcome was obtained on high - dose corticosteroids , and the hearing fully recovered.discussion:sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present . 
 although management remains empirical , the high risk of permanent hearing impairment seems to justify emergency treatment with high - dose corticosteroids . when the clinical and laboratory criteria of antiphospholipid syndrome are met , antiplatelets agents or anticoagulation therapy shall be considered .