Systemic Lupus Erythematosus Audiological Findings

7/27/2019 Systemic Lupus Erythematosus Audiological Findings

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KUNNAMPALLIL GEJO

JOHN,

BASLP, MASLP

KUNNAMPALLIL GEJO JOHN


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SENSORINEURAL

HEARING LOSS

associated with

SystemicLupusErythematosis



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3KUNNAMPALLIL GEJO JOHN


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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

http://images.google.com/imgres?imgurl=http://oto.wustl.edu/bbears/norm-oc.jpg&imgrefurl=http://oto.wustl.edu/bbears/bohne1.htm&h=429&w=600&sz=260&hl=en&start=5&tbnid=sjvTnmLjiDaKcM:&tbnh=97&tbnw=135&prev=/images%3Fq%3Dimages%2Bof%2Bdegeneration%2Bof%2Borgan%2Bof%2Bcorti%26svnum%3D10%26hl%3Den%26sa%3DG


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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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Systemic Lupus Erythematosus Audiological Findings

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