labyrinth it is slides 040225 (1)

8/8/2019 Labyrinth It Is Slides 040225 (1)

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Infections of theInfections of the

LabyrinthLabyrinthSam J. Cunningham, MD, PhDSam J. Cunningham, MD, PhD

Arun Gadre, MD Arun Gadre, MDUTMB Dept of OtolaryngologyUTMB Dept of Otolaryngology

February 2004February 2004


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Infections of the LabyrinthInfections of the Labyrinth

Labyrinthitis: inflammation of the innerLabyrinthitis: inflammation of the innerearear

Multiple etiologies: infectious,Multiple etiologies: infectious,autoimmune, systemic disease, traumaautoimmune, systemic disease, trauma

Infectious agents include bacteria,Infectious agents include bacteria,viruses, fungus and protozoa.viruses, fungus and protozoa.


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LabyrinthitisLabyrinthitis

Vestibular manifestations (vertigo) Vestibular manifestations (vertigo)

Cochlear manifestations (hearing loss)Cochlear manifestations (hearing loss)

BothBoth


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LabrynthitisLabrynthitis

Infection usually occurs by one of Infection usually occurs by one of three routes:three routes:From the meningesFrom the meningesFrom the middle ear spaceFrom the middle ear spaceHematogenous spreadHematogenous spread


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B acterial InfectionsB acterial Infections

Two types of labyrinthitis associatedTwo types of labyrinthitis associatedwith bacterial infections:with bacterial infections:

Toxic LabyrinthitisToxic LabyrinthitisSuppurative LabyrinthitisSuppurative Labyrinthitis


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B acterial InfectionsB acterial Infections

Toxic Labyrinthitis: results from aToxic Labyrinthitis: results from asterile inflammation of the inner earsterile inflammation of the inner earfollowing an acute or chronic otitisfollowing an acute or chronic otitismedia or early bacteria meningitis.media or early bacteria meningitis.Toxins penetrate the round window,Toxins penetrate the round window,IAC, or cochlear aqueduct and causeIAC, or cochlear aqueduct and causean inflammatory reaction in thean inflammatory reaction in theperilymph space.perilymph space.


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B acterial InfectionB acterial Infection

Toxic Labyrinthitis produces mild highToxic Labyrinthitis produces mild highfrequency hearing loss or mildfrequency hearing loss or mildvestibular dysfunctionvestibular dysfunction

Treatment: Antibiotics for precipitatingTreatment: Antibiotics for precipitatingotitis, possible myringotomy.otitis, possible myringotomy.


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Suppurative Labyrinthitis: direct Suppurative Labyrinthitis: direct invasion of the inner ear by bacteria.invasion of the inner ear by bacteria.From otitis or meningitisFrom otitis or meningitis


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Suppurative Labyrinthitis: 4 stagesSuppurative Labyrinthitis: 4 stagesSerous or irritative: production of Ig richSerous or irritative: production of Ig rich

exudates in the perilymphexudates in the perilymph Acute or purulent: bacterial and leukocyte Acute or purulent: bacterial and leukocyteinvasion of the perilymphatic scalainvasion of the perilymphatic scala- -end organend organnecrosisnecrosisFibrous or latent: proliferation of fibroblastsFibrous or latent: proliferation of fibroblastsand granulation tissue in the perilymphand granulation tissue in the perilymphOsseous or sclerotic: new bone depositionOsseous or sclerotic: new bone depositionthroughout the involved labyrinththroughout the involved labyrinth


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Purulent Labyrinthitis: medicalPurulent Labyrinthitis: medicalemergencyemergency

Meningitis or Otitis symptomsMeningitis or Otitis symptomsHospitalization, hydration, vestibularHospitalization, hydration, vestibular

suppressants and iv antibioticssuppressants and iv antibiotics


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B acterial MeningitisB acterial Meningitis

H. influenza B, N. meningitidis, S.H. influenza B, N. meningitidis, S.pneumoniaepneumoniae

Hib vaccine: 55% decrease in casesHib vaccine: 55% decrease in cases

Pneumococcus now predominant org.Pneumococcus now predominant org.


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B acterial MeningitisB acterial Meningitis

Postmeningitis hearing loss 10Postmeningitis hearing loss 10- -20%20%Bilateral, severe to profound,Bilateral, severe to profound,permanent permanent


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S yphilisS yphilis

Treponema pallidumTreponema pallidumDiagnosis by FTADiagnosis by FTA- -ABS and confirmed ABS and confirmedby Western Blot.by Western Blot.Congenital or AcquiredCongenital or Acquired


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V iral Infections V iral Infections

Congenital InfectionCongenital InfectionSystemic viral illnessSystemic viral illnessIsolated involvement of inner earIsolated involvement of inner ear


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C ytomegalovirusC ytomegalovirus

Most common congenital infection in USMost common congenital infection in USMost common infectious cause of congenitalMost common infectious cause of congenitaldeafnessdeafnessLow birth weight, jaundice,Low birth weight, jaundice,hepatosplenomegaly, petechiae,hepatosplenomegaly, petechiae,microcephaly and psychomotor retardation.microcephaly and psychomotor retardation.65% w SNH L65% w SNH L--bilateral, severe to profoundbilateral, severe to profound


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C M VC M V

Diagnosis by isolating virus from urineDiagnosis by isolating virus from urineduring first few weeks of life.during first few weeks of life.Virus isolation form cord blood Virus isolation form cord blood

No treatment: acyclovir may decreaseNo treatment: acyclovir may decrease

amount of shedding, gancyclovir &amount of shedding, gancyclovir &foscarnet not approved duringfoscarnet not approved duringpregnancy.pregnancy.


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R ubellaR ubella

1969 58 /1000001969 58 /1000001983 0.5 /1000001983 0.5 /100000Decline due to vaccineDecline due to vaccineCongenital rubella: cataracts, heart Congenital rubella: cataracts, heart malformations and SNH L, othersmalformations and SNH L, othersDx by viral cultureDx by viral cultureNo treatment; prevention onlyNo treatment; prevention only


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MumpsMumps

ParamyxovirusParamyxovirusParotitis, orchitis, meningoencephalitis,Parotitis, orchitis, meningoencephalitis,and in 0.05% of casesand in 0.05% of cases- -hearing loss.hearing loss.Hearing loss at end of first week of Hearing loss at end of first week of parotitis, unilateral and range fromparotitis, unilateral and range from

mild, high frequency SNHL

to profoundmild, high frequency SNHL

to profoundSNHL.SNHL.Vestibular involvement is uncommon Vestibular involvement is uncommon


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MeaslesMeasles

R ubeola virusR ubeola virusSystemic illness w rash, conjunctivitis,Systemic illness w rash, conjunctivitis,and mucosal Koplik spots.and mucosal Koplik spots.Measles induced hearing loss is 1 /1000Measles induced hearing loss is 1 /1000casescasesMeasles less common 2Measles less common 2 ndnd to vaccineto vaccine


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MeaslesMeasles

Encephalitis in 0.1% of cases w overallEncephalitis in 0.1% of cases w overallmortality rate of 15%, with 25% of mortality rate of 15%, with 25% of

survivors with SNH L.survivors with SNH L.SNHL seen in conjunction with rash.SNHL seen in conjunction with rash.Sudden onset Sudden onset Varies from mild to profound HF SNH L Varies from mild to profound HF SNH L

Unilateral or bilateralUnilateral or bilateralPER MANENTPER MANENT70% have vestibular losses also70% have vestibular losses also


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Varicella Varicella- -zosterzoster

Primary vzv=chicken poxPrimary vzv=chicken poxHL w chicken pox = CH L 2HL w chicken pox = CH L 2ndnd to MEEto MEER eactivation=zosterR eactivation=zosterHerpes zoster oticus= R amsay Hunt Herpes zoster oticus= R amsay Hunt syndrome, reactivation from the geniculatesyndrome, reactivation from the geniculate

ganglion of CN V

II. Painful vesicles.ganglion of CN V

II. Painful vesicles.1 /3 have auditory or vestibular symptoms1 /3 have auditory or vestibular symptoms- -HFHL, hyperacusis, tinnitus, vertigoHFHL, hyperacusis, tinnitus, vertigo


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H erpes simplexH erpes simplex

Labyrinthine infection by:Labyrinthine infection by: R eactivation in the spiralR eactivation in the spiral

ganglion=SSNH Lganglion=SSNH L Extension of the meningoencephalitisExtension of the meningoencephalitis

along CN VIII to the labyrinth=acquiredalong CN VIII to the labyrinth=acquired

SNHL

SNHL


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H umanH uman

Immunodeficiency virusImmunodeficiency virus Auditory and vestibular complaints Auditory and vestibular complaintsrare in AIDS patientsrare in AIDS patientsSome w hearing loss, tinnitus andSome w hearing loss, tinnitus andvertigovertigoThought to be result of opportunisticThought to be result of opportunisticinfections (CM V, HS V), ototoxic drugs,infections (CM V, HS V), ototoxic drugs,neoplasm of inner ear.neoplasm of inner ear.


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F ungal InfectionsF ungal Infections

Fungal labyrinthitis is exceedingly rareFungal labyrinthitis is exceedingly rareoutside the context of host outside the context of host

immunocompromise.immunocompromise.High risk: diabetics, chemo therapy, organHigh risk: diabetics, chemo therapy, organtransplant recipients, AIDS patientstransplant recipients, AIDS patients

Agents include Mucor, Cryptococcus, Agents include Mucor, Cryptococcus,Candida, Aspergillus, and BlastomycesCandida, Aspergillus, and BlastomycesHearing loss is severe and permanent Hearing loss is severe and permanent


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P rotozoaP rotozoa

Toxoplama gondii most commonToxoplama gondii most common Acquired infection usually asymptomatic Acquired infection usually asymptomatic

Congenital infection may lead to severeCongenital infection may lead to severemalformations of fetusmalformations of fetusTriad of chorioretinitis, hydrocephalus,Triad of chorioretinitis, hydrocephalus,intracranial calcificationsintracranial calcifications

May also have microcephaly, cataracts,May also have microcephaly, cataracts,micropthalmia, jaundice, and hsm.micropthalmia, jaundice, and hsm.3000 cases annually3000 cases annually


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T oxoplasmaT oxoplasma

75% asymptomatic at birth75% asymptomatic at birth15% ocular problems15% ocular problems10% severe malformations10% severe malformations85% of symptomatic infants at birth85% of symptomatic infants at birthwill later develop decreasing visualwill later develop decreasing visualacuity, decreased intellectual function,acuity, decreased intellectual function,hearing loss or precocious puberty.hearing loss or precocious puberty.


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Screening test to determine fetalScreening test to determine fetalinfectioninfection PCR analysis of amniotic fluidPCR analysis of amniotic fluid IgM assaysIgM assays Quantitative maternal / fetal IgG analysisQuantitative maternal / fetal IgG analysis

of cord bloodof cord blood


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Treatment Treatment Prenatal tx reduces both transmission andPrenatal tx reduces both transmission and

severity of illness in the fetusseverity of illness in the fetus Combination of pyrimethamine andCombination of pyrimethamine and

sulfonamidesulfonamide

Neonates with documented infectionNeonates with documented infectionshould be given tx for 1should be given tx for 1 st st year of life + year of life + folic acid supplementsfolic acid supplements


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C linical P resentationC linical P resentation

Pts present with only auditoryPts present with only auditorydysfunctiondysfunction- -acute cochlear labyrinthitisacute cochlear labyrinthitisPts present with only vestibularPts present with only vestibulardysfunctiondysfunction- -acute vestibularacute vestibularlabyrinthitislabyrinthitis

BothBoth--acute cochleovestibularacute cochleovestibularlabyrinthitislabyrinthitis


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C linical presentationC linical presentation

Acute cochlear labyrinthitis, aka idiopathic Acute cochlear labyrinthitis, aka idiopathicsudden sensory neural hearing losssudden sensory neural hearing loss(ISSNHL)(ISSNHL)Defined as minimum of 30dB deficit in threeDefined as minimum of 30dB deficit in threecontiguous frequencies over a period of lesscontiguous frequencies over a period of lessthan 3 days in a previously healthy person.than 3 days in a previously healthy person.3 pathologic theories: viral infection,3 pathologic theories: viral infection,

vascular phenomenon, intralabyrinthinevascular phenomenon, intralabyrinthinemembrane rupture.membrane rupture.Much circumstantial evidence of viralMuch circumstantial evidence of viraletiologyetiology


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A cute cochlear A cute cochlear

labyrinthitislabyrinthitisTreatment is steroids. Studies have shownTreatment is steroids. Studies have shownno benefit of steroids and antivirals.no benefit of steroids and antivirals.

3030--70% have complete recovery of hearing.70% have complete recovery of hearing.Prognosis related to age, time from onset toPrognosis related to age, time from onset topresentation, type of audiogram, presencepresentation, type of audiogram, presenceof vestibular symptomsof vestibular symptoms


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C linical presentationC linical presentation

Acute vestibular labyrinthitis, aka Acute vestibular labyrinthitis, akavestibular neuritisvestibular neuritisDefined as sudden unilateral vestibularDefined as sudden unilateral vestibularweakness in the absence of weakness in the absence of concomitant auditory or CNSconcomitant auditory or CNS

dysfunction in a previously healthydysfunction in a previously healthypersonperson


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A cute vestibular A cute vestibular

labyrinthitislabyrinthitisDiagnostic criteria:Diagnostic criteria: An acute, unilateral, peripheral vestibular An acute, unilateral, peripheral vestibular

disorder w /o associated hearing lossdisorder w /o associated hearing loss Occurrence predominantly in middle ageOccurrence predominantly in middle age A single episode of severe, prolonged vertigo A single episode of severe, prolonged vertigo Decreased caloric response in the involved ear.Decreased caloric response in the involved ear.

Complete subsidence of the symptoms within 6Complete subsidence of the symptoms within 6monthsmonths


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A cute vestibular A cute vestibular

labyrinthitislabyrinthitisTreatment is supportive and includesTreatment is supportive and includeshydration, antiemetics, and vestibularhydration, antiemetics, and vestibularsuppressants.suppressants.


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R eferencesR eferences

Gulya, AJ Infections of the Labyrinth. Head and Neck SurgeryGulya, AJ Infections of the Labyrinth. Head and Neck Surgery- -Otolarygology, BJ BaileyOtolarygology, BJ Baileyed. Philadelphia. 2001.ed. Philadelphia. 2001.R osen, EJ Infections of the Labyrinth. UTMB Dept of Otolaryngology Web site in Dr.R osen, EJ Infections of the Labyrinth. UTMB Dept of Otolaryngology Web site in Dr.Quinns Online Textbook and Grand R ound Archives. 2000.Quinns Online Textbook and Grand R ound Archives. 2000.

Stokroos,R

J Antiviral treatment of idiopathic sudden sensorineural hearing loss: aStokroos,R

J Antiviral treatment of idiopathic sudden sensorineural hearing loss: aprospective, randomized, doubleprospective, randomized, double- -blinded clinical trial. Acta Otoblinded clinical trial. Acta Oto- -Laryngologica.Laryngologica.118(4):488118(4):488- -95, Jul 1998.95, Jul 1998.Stokroos, R J The etiology of idiopathic sudden sensorineural hearing loss. ExperimentalStokroos, R J The etiology of idiopathic sudden sensorineural hearing loss. Experimentalherpes simplex virus infection of the inner ear. Am J of Otology. 19(4): 447herpes simplex virus infection of the inner ear. Am J of Otology. 19(4): 447- -52, Jul52, Jul1998.1998.Paparella, MM. Labyrinthitis. Pp 81Paparella, MM. Labyrinthitis. Pp 81- -92. June 12, 1978.92. June 12, 1978.Satoh, H. Proinflammatory cytokine expression in the endolymphatic sac during earSatoh, H. Proinflammatory cytokine expression in the endolymphatic sac during earinflammation. Jaro. 4(2): 139inflammation. Jaro. 4(2): 139- -47, Jun 2003.47, Jun 2003.

Westerlaken, BO. Treatment of idiopathic sudden sensorineural hearing loss withWesterlaken, BO. Treatment of idiopathic sudden sensorineural hearing loss withantiviral therapy: a prospective, randomized, double blind clinical trial. Ann Oto, R hino,antiviral therapy: a prospective, randomized, double blind clinical trial. Ann Oto, R hino,Laryn. 112(11):993Laryn. 112(11):993- -1000. Nove 2003.1000. Nove 2003.

Arbusow V. HS V Arbusow V. HS V--1 not only in human vestibular ganglia but also in the vestibular1 not only in human vestibular ganglia but also in the vestibularlabyrinth. Audiology and Neurolabyrinth. Audiology and Neuro- -Otology. 6(%):259Otology. 6(%):259--62, Sept. 2001.62, Sept. 2001.Furman, J. Vestibular Disorders. 2nd ed. New York. Oxford Pub. 2003.Furman, J. Vestibular Disorders. 2nd ed. New York. Oxford Pub. 2003.

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