Antiphospholipid syndrome (APS) is an autoimmune disease of hypercoagulability state in the presence of antibodies against phospholipids. Many neurological deficits related to APS have been described. Sensory neural hearing loss has been described only anecdotally, mostly in case reports. This study investigated a possible association between hearing disorders and APS.
Methods
Seventeen patients (aged 20–80 years) diagnosed with primary APS were evaluated for evidence of hearing disorders. All had otoscopy, full audiometry test, brainstem evoked response audiometry and otoacoustic emissions testing, and completed a questionnaire. Hearing levels were compared to the standard hearing levels of the general population by age group.
Results
Of the 17 APS patients, 10 (age groups 20–29 and 50–59) had significantly reduced hearing levels in all or part of the tested frequencies compared to standard levels. Three patients (age group 60–69) had better hearing levels at low frequencies compared to standard hearing levels.
Conclusion
These findings support the existence of hearing disorders in primary APS.
CaldarelliDDRejowskiJECoreyJP.Sensorineural hearing loss in lupus erythematosus. Am J Otol1986;
7: 210–213.
7.
WolfovitzELevyYBrookJG.Sudden deafness in a patient with temporal arteritis.J Rheumatol1987;
14: 384–385.
8.
LevineJSBranchDWRauchJ.The antiphospholipid syndrome. N Engl J Med2002;
346: 752–763.
9.
MiyakisSLockshinMDAtsumiT, et al.
International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost2006;
4: 295.
10.
LevineSRWelchKMA.The spectrum of neurologic disease associated with antiphospholipid antibodies. Lupus anticoagulants and anticardiolipin antibodies. Arch Neurol1987;
44: 876–883.
11.
KatzavAChapmanJShoenfeldY.CNS dysfunction in the antiphospholipid syndrome. Lupus2003;
12: 903–907.
12.
ChapmanJRandJHBreyRL, et al.
Non-stroke neurological syndromes associated with antiphospholipid antibodies: evaluation of clinical and experimental studies. Lupus2003;
12: 514–517.
13.
LampropoulosCEHughesGR.The antiphospholipid (Hughes’) syndrome: changing the face of neurology. European J Int Med2004;
15: 147–150.
14.
NaarendorpMSpieraH.Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupus-like syndromes and antiphospholipid antibodies.J Rheumatol1998;
25: 589–592.
15.
CompadrettiGCBrandoliniCTascaI.Sudden sensorineural hearing loss in lupus erythematosus associated with antiphospholipid syndrome: case report and review. Ann Oto Rhino Laryngol2005;
114: 214–218.
16.
Jian-GangWQi-BingXNan-PingYGengY. Primary antiphospholipid antibody syndrome; a case with bilateral sudden sensorineural hearing loss. Rheumatol Int2009;
29: 467–468.
17.
KangK-TYoungY-H.Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol2008;
122: 204–206.
18.
VyseTLuxonLWalportMJ.Audiovestibular manifestations of the antiphospholipid syndrome. J Laryngol Otol1994;
108: 57–59.
19.
WilesNMHuntBJCallananVChevrettonEB.Sudden sensorineural hearing loss and antiphospholipid syndrome. Hematologica2006;
91: 124–127.
ToubiEBen-DavidJKesselAPodoshinLGolanTD.Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies. Lupus1997;
6: 540–542.
22.
ToubiEBen-DavidJKesselAHalasKSaboELuntzM.Immune-mediated disorders associated with idiopathic sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol2004;
113: 445–449.
23.
BowmanCALinthicumFHJrNelsonRAMikamiKQuismorioF.Sensorineural hearing loss associated with systemic lupus erythematosus. Otolaryngol Head Neck Surg1986;
94: 197–203.
24.
AndonopoulosAPNaxakisSGoumasPLygatsikasC.Sensorineural hearing disorders in systemic lupus erythematosus. A controlled study. Clin Exp Rheumatol1995;
13: 137–141.
25.
GreenLMillerEB.Sudden sensorineural hearing loss as a first manifestation of systemic lupus erythematosus: association with anticardiolipin antibodies. Clin Rheumatol2001;
20: 220–222.
26.
BainbridgeKEWallhagenMI.Hearing loss in an aging American population: extent, impact, and management. Annu Rev Public Health2014;
35: 139–152.
27.
SchuknechtHF.Pathology of the ear. 2nd ed.Malvern, PA:
Lea & Febiger, 1993, p.64.
28.
Di StadioARalliM.Systemic lupus erythematosus and hearing disorders: literature review and meta-analysis of clinical and temporal bone findings. J Int Med Res2017;
45: 1470–1480.
29.
RautVVCullenJCathersG.Hearing loss in rheumatoid arthritis. J Otolaryngol2001;
30: 289.
30.
AgrupCLuxonLM.Immune-mediated inner-ear disorders in neuro-otology. Curr Opin Neurol2006;
19: 26–32.
