Deafness Sensineuronal

8/7/2019 Deafness Sensineuronal

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IntroductionIntroduction

What dilemmas?What dilemmas? Increasing number and complexity of testsIncreasing number and complexity of tests

Increasing costs of medical careIncreasing costs of medical care

What do tests results mean?What do tests results mean?

At what point does cost of testing outweighAt what point does cost of testing outweigh

value of rule out diagnosis?value of rule out diagnosis? How far do we go to diagnose etiology?How far do we go to diagnose etiology?


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Finical PressuresFinical Pressures

Increasing cost of medical careIncreasing cost of medical care Third party payers pressure to contain costThird party payers pressure to contain cost

Government pressure to contain costsGovernment pressure to contain costs Patient pressure to contain costPatient pressure to contain cost

Personal Medical savings accountsPersonal Medical savings accounts

Higher deductiblesHigher deductibles Higher coHigher co--payspays

Services not coveredServices not covered


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OverviewOverview

Diagnosis of the following:Diagnosis of the following: Unilateral Childhood SNHL of unknownUnilateral Childhood SNHL of unknown

durationduration

Sudden sensorineural hearing lossSudden sensorineural hearing loss

Unilateral inner ear complaintsUnilateral inner ear complaints


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Unilateral Childhood SNHLUnilateral Childhood SNHL

Unilateral SNHL in moderate to profoundUnilateral SNHL in moderate to profoundrange correlates with:range correlates with:

Poor academic performancePoor academic performance

Increased chance of repeating a gradeIncreased chance of repeating a grade

Behavioral problemsBehavioral problems

Intervention prior to 6 months results inIntervention prior to 6 months results inadvantages in communicationadvantages in communication


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Universal infant screening has led to earlierUniversal infant screening has led to earlierdiagnosisdiagnosis

Congenital hearing loss may be delayedCongenital hearing loss may be delayed Joint Committee on Infant Hearing guidelines forJoint Committee on Infant Hearing guidelines forpersistent periodic screening:persistent periodic screening: Child with family history of early onset SNHLChild with family history of early onset SNHL

Prenatal infection that may lead to SNHLPrenatal infection that may lead to SNHL Neurofibromatosis IINeurofibromatosis II

Persistent pulmonary hypertensionPersistent pulmonary hypertension


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EpidemiologyEpidemiology Average age of diagnosis unilateral SNHL isAverage age of diagnosis unilateral SNHL is

8.7 yo.8.7 yo.

Bilateral SNHL grater than 50dB, 1Bilateral SNHL grater than 50dB, 1--2/10002/1000

Unilateral SNHL greater than 50 dB, 3/1000Unilateral SNHL greater than 50 dB, 3/1000

Genetic cause in 50% of individualsGenetic cause in 50% of individuals


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Initial referral because of failedInitial referral because of failedaudiogram, parental concerns.audiogram, parental concerns.

Confirmed with audiogram, OAE, ABRConfirmed with audiogram, OAE, ABR In younger children, otolaryngologist mustIn younger children, otolaryngologist must

screen for conductive etiologyscreen for conductive etiology


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Differential diagnosisDifferential diagnosis Syndrome with hearing lossSyndrome with hearing loss

Other genetic causeOther genetic cause MeningitisMeningitis

Intrauterine infectionIntrauterine infection

Trauma to cochlea, vestibule, VIIITrauma to cochlea, vestibule, VIII

Exposure to ototoxic drugExposure to ototoxic drug PrematurityPrematurity

Autoimmune diseaseAutoimmune disease


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Differential DiagnosisDifferential Diagnosis HyperbilirubinemiaHyperbilirubinemia

Neurofibromatosis type IINeurofibromatosis type II Anoxic Brain injuryAnoxic Brain injury

MumpsMumps

Neurodegenerative disorderNeurodegenerative disorder

Malignant infiltrationMalignant infiltration Ischemic insult of cochleaIschemic insult of cochlea

Cochlear hydropsCochlear hydrops


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Usher syndromeUsher syndrome Autosomal recessive disorderAutosomal recessive disorder

Retinitis pigmentosa and SNHLRetinitis pigmentosa and SNHL

Early intervention for future visual andEarly intervention for future visual andauditory impairmentauditory impairment

Bilateral cochlear implant should beBilateral cochlear implant should beconsideredconsidered


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Waardenburg syndromeWaardenburg syndrome SNHLSNHL

White forelockWhite forelock

Multicolored iriesMulticolored iries

Dystropia canthorumDystropia canthorum

hypertelorismhypertelorism


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Jervell and LangeJervell and Lange--NielsenNielsen Autosomal dominantAutosomal dominant

Seen in 1.6 to 6 per millionSeen in 1.6 to 6 per million

Prolonged QT interval and increased risk ofProlonged QT interval and increased risk ofsudden deathsudden death

First degree relatives need screening withFirst degree relatives need screening withEKGEKG


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Neurofibromatosis Type IINeurofibromatosis Type II Autosomal dominantAutosomal dominant

Bilateral acoustic neuromasBilateral acoustic neuromas

MeningiomasMeningiomas

Spinal schwannomasSpinal schwannomas

Posterior capsular lens opacitiesPosterior capsular lens opacities


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Alports syndromeAlports syndrome Autosomal dominant or recessive, or xAutosomal dominant or recessive, or x--linkedlinked

Slowly progressive bilateral SNHL, can beSlowly progressive bilateral SNHL, can beasymmetricalasymmetrical

Progressive renal failureProgressive renal failure

Persistent microscopic hematuriaPersistent microscopic hematuria Episodic gross hematuria precipitated by URIEpisodic gross hematuria precipitated by URI


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BranchioBranchio--otooto--renal syndromerenal syndrome 1/40,000 live births1/40,000 live births

60% with branchial cleft cysts60% with branchial cleft cysts

20% with pure SNHL, majority mixed20% with pure SNHL, majority mixed

Shared antigen between the stria vascuularisShared antigen between the stria vascuularis

and glomeruliand glomeruli


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Noonans syndromeNoonans syndrome 1/2500 live births1/2500 live births

Webbed neckWebbed neck

Pinna abnormalitiesPinna abnormalities

Short statureShort stature

1010--15% with SNHL15% with SNHL


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CHARGE SyndromeCHARGE Syndrome ColobomaColoboma

Heart DefectsHeart Defects Atresia choanaeAtresia choanae

Retarded growthRetarded growth

Genital hypoplasiaGenital hypoplasia

Ear abnormalities and deafnessEar abnormalities and deafness

Associated with Mondini malformationAssociated with Mondini malformation


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Fechners syndromeFechners syndrome High frequency SNHLHigh frequency SNHL

ProteinuriaProteinuria

MacrothrombocytopeniaMacrothrombocytopenia

Ocular diseaseOcular disease

Extremely rareExtremely rare


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Pendred syndromePendred syndrome Autosomal recessiveAutosomal recessive

Congenital SNHLCongenital SNHL

GoiterGoiter

Hypothyroidism (varies)Hypothyroidism (varies)

Abnormal perchlorate discharge testAbnormal perchlorate discharge test Associated with Mondini and Large VestibularAssociated with Mondini and Large Vestibular

aqueductaqueduct


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Other causesOther causes TORCHS (Toxoplasmosis, Others, Rubella,TORCHS (Toxoplasmosis, Others, Rubella,

Cytomegalovirus, Herpes simplex, Syphilis)Cytomegalovirus, Herpes simplex, Syphilis) MeningitisMeningitis

Ototoxic drug exposureOtotoxic drug exposure

Maternal drug alcohol useMaternal drug alcohol use Maternal use of teratogenic drugMaternal use of teratogenic drug

(thalidomide)(thalidomide)


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Diagnostic TestingDiagnostic Testing

CBC with DifferentialCBC with Differential Platelet studiesPlatelet studies ANA, ESR, RFANA, ESR, RF BUN, Creatinine, UrinalysisBUN, Creatinine, Urinalysis Serum glucoseSerum glucose Thyroid function tests, Perchlorate testThyroid function tests, Perchlorate test RPR, TTPARPR, TTPA

GJB2 (Connexin 26)GJB2 (Connexin 26) EKGEKG CT, MRICT, MRI


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CBC with differentialCBC with differential Used to screen for leukemia or lymphomaUsed to screen for leukemia or lymphoma

Hearing loss due to hyperviscosity or temporalHearing loss due to hyperviscosity or temporalbone infiltrationbone infiltration

1 case of leukemia with hearing loss as initial1 case of leukemia with hearing loss as initial

manifestation in literaturemanifestation in literature Low yieldLow yield


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Platelet studiesPlatelet studies Drawn to exclude Fechner syndromeDrawn to exclude Fechner syndrome

Patients with family history, ocular disease,Patients with family history, ocular disease,history of diagnosed proteinuria should behistory of diagnosed proteinuria should bescreenedscreened

Test is low yieldTest is low yield


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ANA, ESR, RFANA, ESR, RF Drawn to screen for autoimmune disorders,Drawn to screen for autoimmune disorders,

Lupus, Cogan's, juvenile rheumatoid arthritisLupus, Cogan's, juvenile rheumatoid arthritis Test nonspecificTest nonspecific

Positive RF is 0.7% sensitive in detectingPositive RF is 0.7% sensitive in detectingjuvenile RAjuvenile RA

Testing without history of joint pain or otherTesting without history of joint pain or othersigns of systemic autoimmune disorders is lowsigns of systemic autoimmune disorders is lowyieldyield


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BUN, Creatinine, UrinalysisBUN, Creatinine, Urinalysis Used to screen for concurrent kidney diseaseUsed to screen for concurrent kidney disease

such as Alports diseasesuch as Alports disease Should be drawn with history of grossShould be drawn with history of gross

hematuria, family history of kidney disease,hematuria, family history of kidney disease,family history of slowly progressive hearingfamily history of slowly progressive hearinglossloss

Routine screening is low yieldRoutine screening is low yield


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Serum glucoseSerum glucose Used to screen for Alston syndrome (ImpairedUsed to screen for Alston syndrome (Impaired

glucose tolerance, retinal degeneration,glucose tolerance, retinal degeneration,neurosensory deafness, acanthosis nigricans,neurosensory deafness, acanthosis nigricans,hepatic dysfunction)hepatic dysfunction)

Only 50 cases reported since 1959Only 50 cases reported since 1959

Very low yieldVery low yield


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RPR, TTPARPR, TTPA Drawn to look for syphilis.Drawn to look for syphilis.

Hearing loss with tertiary syphilis usuallyHearing loss with tertiary syphilis usuallyassociated with other manifestations of theassociated with other manifestations of thedisease, but can be sole manifestationdisease, but can be sole manifestation

History of maternal syphilis exposure, signs ofHistory of maternal syphilis exposure, signs oftertiary syphilis make testing higher yieldtertiary syphilis make testing higher yield

Testing low yieldTesting low yield


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GJB2 gene (Connexin 26)GJB2 gene (Connexin 26) Responsible for as much as 50% of autosomalResponsible for as much as 50% of autosomal

recessive nonsyndromic hearing lossrecessive nonsyndromic hearing loss Use most important in genetic counseling forUse most important in genetic counseling for

parents of hearing impaired childrenparents of hearing impaired children

Homozygous children have a 25% chance ofHomozygous children have a 25% chance of

having siblings with hearing loss. Negative orhaving siblings with hearing loss. Negative orheterozygous results gives siblings 14%heterozygous results gives siblings 14%chancechance


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CT and MRICT and MRI Used to scan for inner ear malformationsUsed to scan for inner ear malformations

Mondini malformation associated withMondini malformation associated withperilymphatic fistulas and recurrent meningitisperilymphatic fistulas and recurrent meningitis

Large vestibular aqueduct associated withLarge vestibular aqueduct associated withhearing loss as a result of minor head traumahearing loss as a result of minor head trauma

MRI used for screening for acoustic neuromaMRI used for screening for acoustic neuromain children with neurofibromatosis type IIin children with neurofibromatosis type II


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Mafong et al, Laryngoscope 2002Mafong et al, Laryngoscope 2002

EKG positive in 1 of 15 patients tested.EKG positive in 1 of 15 patients tested. ESR and ANA were positive in 22% ofESR and ANA were positive in 22% of

patients. No correlation with clinicalpatients. No correlation with clinicaldisease. Occasional nonspecificdisease. Occasional nonspecificabnormalities in CBC.abnormalities in CBC.

All other laboratory testing negative.All other laboratory testing negative.


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CT scanCT scan 39% with abnormality39% with abnormality

Large vestibular aqueduct in 13%Large vestibular aqueduct in 13%

Cochlear dysplasia in 7%Cochlear dysplasia in 7%

MRI added to diagnosis in 4, one of whichMRI added to diagnosis in 4, one of which

related to hearing loss (fistulous connectionrelated to hearing loss (fistulous connectionfrom IAC to temporal bone)from IAC to temporal bone)


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Authors concluded that routine laboratoryAuthors concluded that routine laboratoryevaluation should be reconsidered givenevaluation should be reconsidered given

its low diagnostic yieldits low diagnostic yield They supported routine use of EKG and CTThey supported routine use of EKG and CT

scan.scan.


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ConclusionsConclusions

EKGEKG sudden childhood deathsudden childhood death CTCT High incidence of abnormalityHigh incidence of abnormality

Large vestibular aqueductLarge vestibular aqueduct avoid contactavoid contactsportssports

Used for preoperative information shouldUsed for preoperative information should

bilateral SNHL developbilateral SNHL develop Mondini malformation lead to further testingMondini malformation lead to further testing


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ConclusionConclusion

SyphilisSyphilis Rare without classical stigmata (interstitialRare without classical stigmata (interstitial

keratitis, Hutchinsons teeth, mulberry molars,keratitis, Hutchinsons teeth, mulberry molars,bilateral painless knee effusions, nasal septalbilateral painless knee effusions, nasal septalperforation, saddle nose deformity)perforation, saddle nose deformity)

Simple treatment, potentially fatalSimple treatment, potentially fatal

Recommend testing with RPR, confirmationRecommend testing with RPR, confirmationwith TTPAwith TTPA


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GJB2 (Connexin 26)GJB2 (Connexin 26) Screening only if result will affect futureScreening only if result will affect future

childhood planningchildhood planning Genetics consult warranted for counseling ofGenetics consult warranted for counseling of

resultsresults


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-- Fever or illness more than 3 weeks,Fever or illness more than 3 weeks,gingival bleeding, bone or joint pain, signsgingival bleeding, bone or joint pain, signs

of autoimmune disease, order CBC andof autoimmune disease, order CBC andANA, ESR, RFANA, ESR, RF

-- Family history of progressive early onsetFamily history of progressive early onset

hearing loss in first or second degreehearing loss in first or second degreerelative, order urinalysis, genetics consultrelative, order urinalysis, genetics consult


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Family history of progressive vision loss or visualFamily history of progressive vision loss or visualcomplaints, order ophthalmology consultcomplaints, order ophthalmology consult

History of hematuria or family history of kidneyHistory of hematuria or family history of kidneyfailure, order urinalysis. If urinalysis positivefailure, order urinalysis. If urinalysis positiveorder BUN, creatinineorder BUN, creatinine

Thyroid goiter, signs of hypothyroidism, MondiniThyroid goiter, signs of hypothyroidism, Mondini

malformation, large vestibular aqueduct by CTmalformation, large vestibular aqueduct by CTscan, order thyroid function test, considerscan, order thyroid function test, considerperchlorate testperchlorate test


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History of frequent urination, excessive thirst,History of frequent urination, excessive thirst,order serum glucoseorder serum glucose

History of progressive hearing loss, gait orHistory of progressive hearing loss, gait orvestibular symptoms, focal neurologicalvestibular symptoms, focal neurologicalsymptoms, order MRI of brain and IACssymptoms, order MRI of brain and IACs

History of neurofibroma, meningioma, glioma,History of neurofibroma, meningioma, glioma,schwannoma, juvenile posterior subcapsularschwannoma, juvenile posterior subcapsularlenticular opacity or family history oflenticular opacity or family history ofNeurofibromatosis type II, order MRI of brainNeurofibromatosis type II, order MRI of brainand IACs.and IACs.


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Sudden Sensorineural Hearing LossSudden Sensorineural Hearing Loss

Incidence estimated between 5 and 20 perIncidence estimated between 5 and 20 per100,000100,000

Most common between ages of 40 and 54Most common between ages of 40 and 54 Loss of at least 30dB in 3 contiguousLoss of at least 30dB in 3 contiguous

frequencies in 72 hours or lessfrequencies in 72 hours or less

65% diagnosed will spontaneously recover65% diagnosed will spontaneously recoverwithin 20dB or greater than 50% of totalwithin 20dB or greater than 50% of totallossloss


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EtiologyEtiology Vascular compromise from hyperviscosity, embolicVascular compromise from hyperviscosity, embolic

event, vasospasm.event, vasospasm.

Intracochlear membrane rupture or perilymph fistulasIntracochlear membrane rupture or perilymph fistulas

Viral infectionViral infection

Autoimmune inner ear disease, systemic autoimmuneAutoimmune inner ear disease, systemic autoimmunedisease (Cogans, Wegners, polyarteritis nodosa,disease (Cogans, Wegners, polyarteritis nodosa,temporal arteritis, Berger's, SLE)temporal arteritis, Berger's, SLE)


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Diagnostic testingDiagnostic testing CBCCBC

ESR, ANA, RFESR, ANA, RF Serum GlucoseSerum Glucose

T3, T4, TSHT3, T4, TSH

PT, PTTPT, PTT RPR, TTPARPR, TTPA


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Diagnostic testingDiagnostic testing HIVHIV

Lyme titerLyme titer Cholesterol/TriglyceridesCholesterol/Triglycerides

AntiAnti--hsp 70 (68KD heat shock protein)hsp 70 (68KD heat shock protein)

MRIMRI


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CBCCBC ESR, ANA, RFESR, ANA, RF

Serum GlucoseSerum Glucose Thyroid function studiesThyroid function studies RPR, TTPARPR, TTPA


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PT, PTTPT, PTT Used to look for hemorrhagic etiology ofUsed to look for hemorrhagic etiology of

SSNHLSSNHL Patients likely to have other manifestations ofPatients likely to have other manifestations of

coagulopathycoagulopathy

Higher yield if patient currently on anHigher yield if patient currently on ananticoagulantanticoagulant


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HIVHIV Shown to decrease hearing in up to 30% ofShown to decrease hearing in up to 30% of

patients with active infection compared topatients with active infection compared tocontrols.controls.

Literature shows 3 cases of SSNHL withLiterature shows 3 cases of SSNHL withpositive HIVpositive HIV

Low yieldLow yield

HIV treatment early improves life expectancyHIV treatment early improves life expectancy


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Gagnebin 2000 (French study)Gagnebin 2000 (French study) Analyzed screening HIV, Lyme, syphilisAnalyzed screening HIV, Lyme, syphilis

102 charts reviewed102 charts reviewed Two patients with positive Lyme titers, notTwo patients with positive Lyme titers, not

responding to treatmentresponding to treatment

All HIV negativeAll HIV negative

Latent syphilis without signs of neurosyphilis in oneLatent syphilis without signs of neurosyphilis in onepatientpatient

Concluded screening is low yield without historyConcluded screening is low yield without history


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AntiAnti--hsp70 (68KD heat shock protein)hsp70 (68KD heat shock protein) Thought to be a marker of autoimmune inner earThought to be a marker of autoimmune inner ear

etiologyetiology

Early studies have shown steroid responsiveness inEarly studies have shown steroid responsiveness inpatients with positive testspatients with positive tests

Samuelsson screened 27 with SSNHL and 100Samuelsson screened 27 with SSNHL and 100controls. 19% and 14% respectively (not significant)controls. 19% and 14% respectively (not significant)

Yeom in 2003 tested for antiYeom in 2003 tested for anti--hsp 70 in 20 patientshsp 70 in 20 patientswith rapidly progressive SNHL and 20 controls. Nowith rapidly progressive SNHL and 20 controls. Nosignificant differencesignificant difference


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AntiAnti--hsp70 (68KD heat shock protein)hsp70 (68KD heat shock protein) Testing is high yieldTesting is high yield

Question as to validity of resultsQuestion as to validity of results Question of utility of positive resultsQuestion of utility of positive results

(most patients treated with steroids)(most patients treated with steroids)


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Cholesterol and TriglyceridesCholesterol and Triglycerides Labyrinthine artery atherosclerosis as anLabyrinthine artery atherosclerosis as an

etiologyetiology FriedrichFriedrich 49 patients with neurootologic49 patients with neurootologic

symptoms. Increased LDL and LDL/HDL ratiosymptoms. Increased LDL and LDL/HDL ratioas compared to controlsas compared to controls

NutiNuti No significant difference in cholesterolNo significant difference in cholesteroland LDL/HDL ratio with controlsand LDL/HDL ratio with controls


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Cholesterol and TriglyceridesCholesterol and Triglycerides UllrichUllrich tested lipids and triglycerides in 24tested lipids and triglycerides in 24

patients with SSNHL. No significant differencepatients with SSNHL. No significant differencefrom controlsfrom controls

KojimaKojima 12 patients with SSNHL12 patients with SSNHL Event was at least 1 month prior studyEvent was at least 1 month prior study

Patients with total cholesterol greater thanPatients with total cholesterol greater than230mg/dL treated with diet and medications230mg/dL treated with diet and medications Significant improvement in 125Significant improvement in 125--2000 Hz2000 Hz


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Cholesterol and TriglyceridesCholesterol and Triglycerides American College of Physicians recommendsAmerican College of Physicians recommends

screening for lipid abnormalities in men 35screening for lipid abnormalities in men 35--6565and women 45and women 45--65 years of age using total65 years of age using totalcholesterol level only.cholesterol level only.

Cholesterol or triglyceride levels as an etiologyCholesterol or triglyceride levels as an etiology

of SSNHL likely low probabilityof SSNHL likely low probability Testing in this age range is high yieldTesting in this age range is high yield


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MRIMRI Used to screen for acoustic neuromaUsed to screen for acoustic neuroma

Patients with acoustic neuroma present withPatients with acoustic neuroma present withSSNHL 10% of the timeSSNHL 10% of the time

As high as 2.5% of all patients with SSNHLAs high as 2.5% of all patients with SSNHLhave an acoustic neuromahave an acoustic neuroma


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MRIMRI Able to screen for acoustic neuroma and otherAble to screen for acoustic neuroma and other

causes of hearing losscauses of hearing loss AronzonAronzon treated patients with SSNHL andtreated patients with SSNHL andMRI proven acoustic neuroma with high doseMRI proven acoustic neuroma with high doseof steroidsof steroids Improvement in hearing of all patientsImprovement in hearing of all patients Response to steroids does not exclude acousticResponse to steroids does not exclude acoustic

neuromaneuroma


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All patients should be screened with RPR,All patients should be screened with RPR,confirmed with TTPA (treatable, lifeconfirmed with TTPA (treatable, life

threatening, low cost)threatening, low cost) MRI in all patients, regardless of responseMRI in all patients, regardless of responseto steroidsto steroids

Total cholesterol in men aged 35Total cholesterol in men aged 35--65,65,women aged 45women aged 45--65 if no testing in the last65 if no testing in the lastyearyear


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HIV testing in all patients with high risk ofHIV testing in all patients with high risk ofSTD, +/STD, +/-- in all patientsin all patients

ESR, ANA, RF in patients with history andESR, ANA, RF in patients with history andphysical findings of autoimmune etiology,physical findings of autoimmune etiology,response to steroid, but relapse afterresponse to steroid, but relapse after

tapertaper


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CBC, Thyroid function tests, PT, PTT,CBC, Thyroid function tests, PT, PTT,Lyme titers based on history or physicalLyme titers based on history or physical

exam findings onlyexam findings only 68 KD protein in research settings, +/68 KD protein in research settings, +/-- inin

response to steroids with relapse afterresponse to steroids with relapse after

steroid tapersteroid taper


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Acoustic NeuromaAcoustic Neuroma

12 per million per year12 per million per year MRI can detect tumors as small as 3mmMRI can detect tumors as small as 3mm

Gold standard for diagnosis is MRI ofIACsGold standard for diagnosis is MRI ofIACswith gadoliniumwith gadolinium

Test cost at this institution is $3200Test cost at this institution is $3200


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ABRABR Alternative to MRI for diagnosisAlternative to MRI for diagnosis

MRI generally 5 times more expensive thanMRI generally 5 times more expensive thanABRABR

Decreased sensitivityDecreased sensitivity

Cost at this institution is $500Cost at this institution is $500


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Sensitivity of ABRSensitivity of ABR Wilson 1992Wilson 1992 sensitivity of 85%, 67% forsensitivity of 85%, 67% for

small tumorssmall tumors Chandrasekhar 1995Chandrasekhar 1995 sensitivity of 92%,sensitivity of 92%,83% for small tumors83% for small tumors

Gordon 1995Gordon 1995 sensitivity 88%, 69% for smallsensitivity 88%, 69% for small

tumorstumors Ruckershern 1996Ruckershern 1996 sensitivity of 63%, PPVsensitivity of 63%, PPV26%26%


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Robinette and BauchRobinette and Bauch Retrospective review to identify 95 patients withRetrospective review to identify 95 patients with

acoustic neuromasacoustic neuromas

Divided into 3 groupsDivided into 3 groups

High riskHigh risk asymmetric hearing loss of greater than 20 DBasymmetric hearing loss of greater than 20 DBover three frequencies, greater than 30% decrease of wordover three frequencies, greater than 30% decrease of wordrecognitionrecognition

Intermediate riskIntermediate risk SSNHL or unexplained persistentSSNHL or unexplained persistentunilateral tinnitusunilateral tinnitus

Low riskLow risk isolated vertigo or historically explainedisolated vertigo or historically explainedintermittent tinnitus or historically explained SNHLintermittent tinnitus or historically explained SNHL


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Used probability of acoustic in each groupUsed probability of acoustic in each groupas (30%, 5%, 1%)as (30%, 5%, 1%)

Sensitivity of detecting tumors with ABRSensitivity of detecting tumors with ABRbased on size (100% large, 93% medium,based on size (100% large, 93% medium,82% small)82% small)

Used PPV of 12% in ABRUsed PPV of 12% in ABR


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Calculated cost of MRI for patients in eachCalculated cost of MRI for patients in eachgroupgroup

Calculated cost of ABR, and MRI for allCalculated cost of ABR, and MRI for allABRs suggestive of acoustic neuromaABRs suggestive of acoustic neuroma Calculated cost difference of two groupsCalculated cost difference of two groups

based on risk from history and physicalbased on risk from history and physical

Calculated number acoustic neuromasCalculated number acoustic neuromasmissed if first screened with ABRmissed if first screened with ABR


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High riskHigh risk -- $40,000, no missed tumors$40,000, no missed tumors Intermediate riskIntermediate risk -- $900,000, clinician would$900,000, clinician would

miss 4 tumors in 900 patients screenedmiss 4 tumors in 900 patients screened Low riskLow risk $1.7 million, 1 tumor missed out of$1.7 million, 1 tumor missed out of

1600 screened1600 screened

Authors recommended clinical decision makingAuthors recommended clinical decision makingwith consideration of cost savings inwith consideration of cost savings inintermediate to low risk groupsintermediate to low risk groups


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MRI in:MRI in: All patients with unilateral SNHL greater thanAll patients with unilateral SNHL greater than

20dB difference from unaffected side not20dB difference from unaffected side notexplained by historyexplained by history

Word discrimination difference of 30% orWord discrimination difference of 30% orgreater from asymptomatic sidegreater from asymptomatic side


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MRIMRI Unilateral persistent tinnitus not explained byUnilateral persistent tinnitus not explained by

historyhistory Persistent vertigoPersistent vertigo

Unilateral sudden SNHL, regardless ofUnilateral sudden SNHL, regardless ofresponse to steroidsresponse to steroids


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ABR vs. MRIABR vs. MRI Historically explained unilateral tinnitusHistorically explained unilateral tinnitus

Historically explained hearing lossHistorically explained hearing loss Isolated vertigoIsolated vertigo


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SummarySummary

Number of diagnostic tests expandingNumber of diagnostic tests expanding Physicians asked more and more toPhysicians asked more and more to

evaluate medical necessity and costevaluate medical necessity and costbenefit of diagnostic testingbenefit of diagnostic testing

Research projects based on cost analysisResearch projects based on cost analysis

Balance of cost containment andBalance of cost containment anddiagnostic accuracydiagnostic accuracy

Documents

Deafness Sensineuronal