acoustic neuroma slides 061206

8/2/2019 Acoustic Neuroma Slides 061206

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Acoustic NeuromaAcoustic Neuroma

K. Kevin Ho, M.D.Vicente A. Resto, M.D., Ph.D.

Department of Otolaryngology

University of Texas Medical Branch

Acoustic Neuroma &

Hearing Loss
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Medieval TimesMedieval Times
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1912 Acoustic Neuroma1912 Acoustic Neuroma

SurgerySurgery

Jackler RK. 2000, p. 173: Tumors of the Ear and Temporal Bone
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Historical PerspectivesHistorical Perspectives

(contd)(contd)

1905 Dr. Harvey Cushing1905 Dr. Harvey Cushing Meticulous dissectionMeticulous dissection Hemostasis: silver clips, boneHemostasis: silver clips, bone

wax, electrocauterywax, electrocautery Mortality: 20 % (1917)Mortality: 20 % (1917) 4%4%

(1931)(1931)

1916 Dr. Walter Dandy1916 Dr. Walter Dandy Complete removal of ANComplete removal of AN

Mortality: 10%Mortality: 10%

Early 1960s Dr. William HouseEarly 1960s Dr. William House Translabyrinthine approach usingTranslabyrinthine approach using

surgical drill and operatingsurgical drill and operatingmicroscopemicroscope
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AnatomyAnatomy
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Cerebellopontine Angle:Cerebellopontine Angle:

AnatomyAnatomy
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EpidemiologyEpidemiology

6 % of all Intracranial tumors6 % of all Intracranial tumors

80 - 90% of CPA tumors80 - 90% of CPA tumors

Incidence in US: 10 per million /Incidence in US: 10 per million /yearyear

Vast majority in adulthoodVast majority in adulthood

95% Sporadic (unilateral)95% Sporadic (unilateral) 5% Neurofibromatosis type 25% Neurofibromatosis type 2

(bilateral)(bilateral)

No known race, genderNo known race, gender
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PathogenesisPathogenesis

Neither Neuroma or AcousticNeither Neuroma or Acoustic

(auditory)(auditory)

Schwannoma arising fromSchwannoma arising fromvestibular nervevestibular nerve

Benign tumor. MalignantBenign tumor. Malignant

degeneration exceedingly rare.degeneration exceedingly rare.

Majority originate within theMajority originate within the

IACIAC

Equal frequency on SuperiorEqual frequency on Superior
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Jackler Staging SystemJackler Staging System

Stage Tumor Size

Intracanalicular Tumor confined toIAC

I (small) < 10 mm

II (medium) 11-25 mm

III (Large) 25-40 mm

IV (Giant) > 40 mm


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Phases of Tumor GrowthPhases of Tumor Growth

Intracanalicular:Intracanalicular: Hearing loss, tinnitus, vertigoHearing loss, tinnitus, vertigo

Cisternal:Cisternal: Worsened hearing and dysequilibriumWorsened hearing and dysequilibrium

Compressive:Compressive: Occasional occipital headacheOccasional occipital headache

CN V: Midface, corneal hypesthesiaCN V: Midface, corneal hypesthesia Hydrocephalic:Hydrocephalic:

Fourth ventricle compressed andFourth ventricle compressed and

obstructedobstructed

Headache, visual changes, altered mentalHeadache, visual changes, altered mental
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Phases of Tumor GrowthPhases of Tumor Growth

Jackler RK. 2000, p. 180: Tumors of the Ear and Temporal Bone

ntracanalicular Cisternal

Compressive Hydrocephali
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Hearing LossHearing Loss

Most frequent initial symptomMost frequent initial symptom

Most common symptom ~ 95% ANMost common symptom ~ 95% AN

patientspatients Asymmetric SNHLAsymmetric SNHL

Down-sloping / High FrequencyDown-sloping / High Frequency

Decreased Speech DiscriminationDecreased Speech Discrimination
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Serviceable HearingServiceable Hearing

100 70 50 00

30

50

A

DB

C

P

TT

(dB)

SDS (%)
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Distribution of HearingDistribution of Hearing

in ANin AN

Myrseth: Neurosurgery, Volume 59(1).July 2006.67-76

P th h i l f H iP th h i l f H i
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Pathophysiology of HearingPathophysiology of Hearing

LossLoss

in Acoustic Neuromain Acoustic Neuroma Exact etiology is unknownExact etiology is unknown

Compressive effect on cochlearCompressive effect on cochlearnervenerve

Vascular occlusion of internalVascular occlusion of internalauditory arteryauditory artery

Biochemical alterations inner earBiochemical alterations inner earfluidsfluids
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Hearing in AcousticHearing in Acoustic

NeuromaNeuromaSelesnick 1993 Shaan1993

Lustig1998

Magdziarz

2000

ANpatients

126 100 546 369

Normalhearing

5(4%)

6(6%)

29(5%)

10(3%)
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Tumor Size and HearingTumor Size and Hearing

NormalHearing

(29 Patients)

All ANs

(126 Patients)

% Small(< 1cm)

45 24

% Medium

(1-3 cm)

42 59

% Large

(> 3 cm)

12 16

Lustig LR. Am J Otology 1998: 19; 212-8
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Tumor size & HearingTumor size & Hearing

Lack of conclusive correlationLack of conclusive correlation

between tumor size and hearingbetween tumor size and hearing

< 20 mm > 20 mm

Stipkovits EM et al. Am. J. Otology 1998: 19; 834-9


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Tumor Growth RateTumor Growth Rate

Battaglia et al. Otol Neurotol. 2006 Aug;27(5):705-712
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Tumor Growth: StudiesTumor Growth: Studies

N Follow-up

NoGrowth (%)

-Growth (%)

+Growth (%)

Bederson

70 26 mo 40 7 53

Selesnick

558 3 yr - - 54

Charabi 126 3.8 yr 12 6 82

Raut 72 80 mo 42 19 39

Walsh 72 3.2 yr 50 14 37
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Tumor Growth & HearingTumor Growth & Hearing

A

D BA

B

D

Massick DD. Laryngoscope 2000: 110; 1843-9

Change in Tumor Volume (mm3)

Change in Tumor Volume (mm3)

PTA SDS
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Predicting Tumor GrowthPredicting Tumor Growth

Herwadker A. Otology and Neurotology 2005: 26; 86-92

Side Gender

InitialVolume

Age
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24/49

Estimating TumorEstimating Tumor

GrowthGrowth

Serial MRI with andSerial MRI with and

without GADwithout GAD

The only reliable studyThe only reliable studyto estimate tumorto estimate tumor

growth rategrowth rate

T G thT G th
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Tumor Growth:Tumor Growth:

BiomarkersBiomarkers

O Reilly BF et al. Otol Neurotoloty 2000: 25; 791-6


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Fibroblast Growth FactorFibroblast Growth Factor

ReceptorReceptor

O Reilly BF et al. Otol Neurotoloty 2000: 25; 791-6


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Delayed DiagnosisDelayed Diagnosis

Duration of Symptoms Prior toDuration of Symptoms Prior to

DiagnosisDiagnosisSymptoms Years

Hearing Loss 3.9Vertigo 3.6Tinnitus 3.4

Headache 2.2Dysequilibrium 1.7Trigeminal 0.9Facial 0.6

Jackler RK. 2000. Tumors of the Ear and Temporal Bone
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History and PhysicalHistory and Physical

Hearing LossHearing Loss VertigoVertigo DysequilibriumDysequilibrium

TinnitusTinnitus HeadacheHeadache NystagmusNystagmus

Early small lesion: Horizontal (vestibular)Early small lesion: Horizontal (vestibular)

Late large: Vertical (brainstemLate large: Vertical (brainstemcompression)compression) Cranial neuropathyCranial neuropathy

CN V, VIICN V, VII Lower cranial nerves (IX-XII)Lower cranial nerves (IX-XII)
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Frequency of SymptomsFrequency of Symptoms

Hearing LossHearing Loss ((85-97%85-97% ;;94%94% )) VertigoVertigo ((5-70 %5-70 % ;;

39% )39% ) DysequilibriumDysequilibrium ((46-70%46-70% ; 56 %); 56 %) TinnitusTinnitus ((56-70%56-70% ; 64; 64

%)%)

Facial nerveFacial nerve ((10-77%10-77% ; 38; 38%)%) Trigeminal nerveTrigeminal nerve ((16-63%16-63% ; 26; 26

%)%)

HeadacheHeadache ((12-38%12-38% ;;Jackler RK. 2000, p. 182: Tumors of the Ear and Temporal Bone
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Symptoms in AN patientsSymptoms in AN patients

with Normal Hearingwith Normal Hearing

Lustig LR. Am J Otology 1998: 19; 212-8


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Sudden SensorineuralSudden Sensorineural

Hearing lossHearing loss

IdiopathicIdiopathic

1-2 % SSNHL patients have AN1-2 % SSNHL patients have AN

10- 26 % AN patients have a history10- 26 % AN patients have a historyof SSNHLof SSNHL

Most experts advocate obtaining MRIMost experts advocate obtaining MRIin all patients who present within all patients who present withSSNHLSSNHL
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DiagnosisDiagnosis

History and Physical ExamHistory and Physical Exam Audiology testing:Audiology testing:

AudiogramAudiogram

ABRABR OAEOAE

Vestibular testings (eg. ENG, rotaryVestibular testings (eg. ENG, rotarychair, posturography) all lackchair, posturography) all lack

diagnostic valuediagnostic value RadiographyRadiography

MRIMRI Gold StandardGold Standard CTCT
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Pure Tone and SpeechPure Tone and Speech

AudiometryAudiometry


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ABR: RetrocochlearABR: Retrocochlear

PathologyPathology Increased interpeak intervals

I-to-III interval of 2.5 ms, III-to-Vinterval of 2.3 ms, and I-to-V interval

of 4.4 ms Interaural wave V latency

difference (IT5)

Greater than 0.2 ms Poor waveform morphology ie. only

some of the waves are discernible

Absent waveform
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ABR patterns in ANABR patterns in AN

10-20 %10-20 % withwithonly wave I andonly wave I andnothingnothingthereafterthereafter

40-60 %40-60 % withwithwave V latencywave V latencydelaydelay

10-15 %10-15 % havehavenormal findingsnormal findings

Fraysse B et al. First International Conf. on Acoustic Neuroma. 1992
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ABR: DiagnosticABR: Diagnostic

EfficiencyEfficiency Generally, Efficiency increases withGenerally, Efficiency increases with

SizeSize

Sensitivity: > 90 % for tumor > 3 cmSensitivity: > 90 % for tumor > 3 cm

No response for severe/ profoundNo response for severe/ profoundSNHLSNHL (Rupa 2003)(Rupa 2003)

False negative Rate:False negative Rate:

15 % (Wilson 1992 6/40)15 % (Wilson 1992 6/40) 33 % (5/15) for Intracanalicular Tumor33 % (5/15) for Intracanalicular Tumor

False positive Rate:False positive Rate: > 80 % (Jackler 2005)> 80 % (Jackler 2005)

Positive predictive value:Positive predictive value:
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ABR: Sensitivity & TumorABR: Sensitivity & Tumor

sizesize

Gordon ML. American Journal of Otology. 1995; 16: 136-9


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IT 5 & Tumor SizeIT 5 & Tumor Size

Chandrasekhar SS et al. Am J Otol

1995;16:63-7


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Stacked ABRStacked ABR Attempt toAttempt to

improveimprovedetection rate indetection rate insmall < 1 cm ANssmall < 1 cm ANs

Stacking ofStacking ofderived bandderived bandresponseresponse

Out of 25 ANs, 5Out of 25 ANs, 5tumors less thantumors less than1 cm missed in1 cm missed inStandard ABRStandard ABR

were picked upwere picked upDon M et al. Am J. Otology; 1997: 21; 148-151

OAEOAE
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OAEOAE ReflectReflect cochlearcochlear/ OHC / sensory/ OHC / sensory

hearinghearing

Not primarily used as screening toolNot primarily used as screening tool

Presence of OAE in SNHL Presence of OAE in SNHL

RetrocochlearRetrocochlear However, 50 % AN demonstrate bothHowever, 50 % AN demonstrate both

cochlear and retrocochlear hearingcochlear and retrocochlear hearing

lossloss

Risk stratification for hearingRisk stratification for hearingpreservation surgerypreservation surgery

Kim AH. Otol Neurotol. 2006 Apr;27(3):372-9

PreoperativeTEOAE
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MRI Brain w. & w/o GADMRI Brain w. & w/o GAD

T1:T1: Isointense to brainIsointense to brain,, hyperintense thyperintense t

CSFCSF

T2:T2: Hyperintense to brain,Hyperintense to brain, hypointenshypointens

to CSFto CSF

T1 pre-Gad T1 post-GadT2
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CT Brain with contrastCT Brain with contrast

HeterogeneousHeterogeneous

enhancement onenhancement on

contrastcontrast

Rare calcificationRare calcification Contraindication toContraindication to

MRI (metallicMRI (metallic

implants),implants),

claustrophobicclaustrophobic

patientspatients

May not be able toMay not be able to

detect small tumordetect small tumor
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Treatment optionsTreatment options

ObservationObservation

SurgerySurgery TranslabyrintTranslabyrint

hinehine

RetrosigmoidRetrosigmoid

Middle fossaMiddle fossa

RadiotherapyRadiotherapy ConventionalConventional

StereotacticStereotactic

C iC ti
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ConservativeConservative

ManagementManagement Advanced age (> 65 )Advanced age (> 65 )

Short life expectancy (< 10 years)Short life expectancy (< 10 years)

Slow growth rateSlow growth rate Poor surgical candidate / poorPoor surgical candidate / poor

general healthgeneral health

Minimal symptomsMinimal symptoms Only hearing earOnly hearing ear

Patience preferencePatience preference
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Observation: Raut 2004Observation: Raut 2004 Prospective cohort study of 72Prospective cohort study of 72

patientspatients Age at presentation: 60.8 yearsAge at presentation: 60.8 years Mean follow-up: 80 monthsMean follow-up: 80 months

Mean tumor size at diagnosis: 9.4 mmMean tumor size at diagnosis: 9.4 mm

Mean tumor growth rate: 1Mean tumor growth rate: 1 mmmm/ year/ year 87% growth rate < 287% growth rate < 2 mmmm/ year/ year Tumor growthTumor growth

+ : 39 %+ : 39 %

0: 42%0: 42% - : 19%- : 19%

No correlation between growth andNo correlation between growth andage, gender, size at presentation, orage, gender, size at presentation, orpresenting symptomspresenting symptoms

32 % failed conservative mana ement32 % failed conservative mana ementRaut V et a.: Clin Otolaryngol29:505514, 2004.

Preop Predictive factors forPreop Predictive factors for
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Preop Predictive factors forPreop Predictive factors forHearing PreservationHearing Preservation

SurgerySurgery

Rohit MS et al. Ann. Oto. Rhino. Laryng. 2006: 115 (1); 41-6

bL f S i bl H i


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Loss of Serviceable HearingLoss of Serviceable Hearing

during Observationduring Observation

Walsh RM et al. Laryngoscope 2000: 110; 250-5


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ConclusionsConclusions

Tumor size has no correlation withTumor size has no correlation withaudiovestibular symptoms inaudiovestibular symptoms in

Acoustic neuromaAcoustic neuroma

Understanding tumor growth rateUnderstanding tumor growth rateis important for predictingis important for predicting

symptom progression andsymptom progression and

treatment planningtreatment planning The study-of-choice to estimateThe study-of-choice to estimate

tumor growth is serial MRItumor growth is serial MRI
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Thank You
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