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7/27/2019 Systemic Lupus Erythematosus Audiological Findings
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KUNNAMPALLIL GEJO
JOHN,
BASLP, MASLP
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SENSORINEURAL
HEARING LOSS
associated with
SystemicLupusErythematosis
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Immunity=Resistance
Immune system=Bodys defence mechanism againstpathogens
Lymphocyte and antibodies:Soldiers of Immune system
INTRODUCTION
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AUTOIMMUNEDISEASE
Immune system uncontrollably attack
bodys own tissue Examples:
SLE
Rheumatoid arthritis
Multiple sclerosis
Scleroderma
Mysthenia gravis
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Multi system connective tissue disorder.
Presence of Auto immune antibodies Circulating immune complexes.
Wide spread immunologically determined tissuedamage.
Synonyms: Lupus, SLE
SYSTEMIC LUPUS
ERYTHEMATOSUS
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Onset: 2nd & 3rd decades
Female- Male Ratio- 9:1
Sex incidence: Equal in children & elderly
EPIDERMIOLOGY
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Exact cause is unknown
Multi factorial disease
3 major mechanisms which develop SLE are;
-Genetic pre disposition
-Environmental triggers
-Drug reaction
AETIOLOGY
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Multifactorial Inheritance
6th chromosome carry SLE gene
1.GENETICS
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They trigger the initial onset. They exacerbate the existing condition.
Extreme stress exposure to sunlight
Hormones
Infection
2.ENVIRONMENTAL
TRIGGERS
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Drugs which induce SLE are: Hydralazine
Procainamide
Phenytoin Isoniazide
3.DRUG REACTION
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Immunologically mediated tissue damage results fromat least two different mechanisms in SLE.
01.Direct antibody mediated cytotoxicity(type2 reactions)
02.Immune complex and complement mediated(type3reactions)
PATHOLOGY
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CLINICAL FEATURES
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Arthritis,arthralgia and fever
Skin lesions:-Malar,Discoid, Butterfly rashes-Photosensitivity
Cardiopulmonary Features:
-Myocarditis and pleurisy,-fibrosing alveolitis,& shrinking lung syndrome
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Review of literature:
- Brian Mc Cabe 1979 :- Auto immune inner ear disease (AIED)
-AIED: Immune system attacks inner ear hair cells
- Major cause of rapidly progressing sensori neuralhearing loss
-Campbell 2000:-Use of immune screening to assist the auditorydiagnosis
CURRENT TRENDS IN AUTO
IMMUNE DISORDER
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.Vestibular symptoms.
-Fluctuating Course-Tinnitus
-Endolymphatic Disturbances
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McCabe2001:
Differential diagnosis of AIED from
-Acoustic neuroma
-Neuro syphillis
-Menieres disese-Metabolic disorders
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Recent studies on SLE:
-A sub clinical features Auto immune mediated
sensori neural hearing loss-Progressive hearing loss
-Unilateral or Bilateral
SLE & SENSORI NEURAL
HEARING LOSS
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Pathogenic circulating Immune complexes activate as
Antineuronal antibodys & anti cochlear antibodies along with
antiphospholipids & anti ribosomal- P
Deposition of Auto immune complex leads to
-Vasculitis & Endartritis of stria vascularis
-Degeneration of outer & inner hair cells
-Sudden SN hearing loss
PATHOPHYSIOLOGY
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Sudden hearing loss in SLE:
-Anti endothelial antibodies
-Vasculitis of stria vascularis
-Anna Rita MD etal ( 1999)
ABR inter wave latency abnormalities in SLE
-Bresnihan etal (2000)
Anti cardiolipin antibodies in SLE: Activate as anticochlearantibodies & resulting in Sensory hearing loss
-Tasca MD etal (2002)
REVIEW OF LITERATURE
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1. SN hearing loss in SLE patients
-High frequency hearing loss- Pre mature aging of inner
ear
2. Low frequency hearing loss- A sub clinical vestibular hydrops
3. 1st & 2nd are due to auto immune causes in the inner ear
-Goumas P etal(2004)
REVIEW OF LITERATURE
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Findings include
-Normocytic Anemia-Mild Thrombocytopenia
-Lupus Nephritis
-Secondary APLA syndrome
-Vasculitic Ulcers-Retinal Vasculitis
-Previous history of recurrent DVT and pulmonary
embolism.
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PTA
Right ear: 86.66dB
Left ear : 15 dB
Immittance findings
Right ear: A Type Tympanogram with absent reflexes
Left ear: A Type tympanogram with absent reflex at 4kHz
AUDIOLOGICAL FINDINGS
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Right Ear: Severe Sensorineural hearing loss
Left Ear : Hearing sensitivity within normal limits.
Detailed audiological counseling was done. Where in thepatient was counselled regarding
-The degree & type of hearing loss
- The progressive nature of loss- The cause of hearing loss(Auto immune mediated
sensori neural hearing loss).
Follow up could not be done due to the poor physicalcondition of the patient.
INTERPRETATION
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Recent studies on Systemic Lupus Erythrematosis haverevealed the presence of a subclinical feature. Auto immune
mediated sensori neural hearing loss. In the present casestudy the case was presented with a combination of APLAsyndrome and mild thrombocytopenia associated with SLE. Asubclinical feature unilateral sensori neural hearing loss wasdetected by detailed audiological evaluation.
Apart from the diagnosis of such a condition, it is also equallyimportant to know the nature of deafness to target better auralrehabilitation approaches.
SUMMARY & CONCLUSION
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PATIENTS WITH SLE
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