Acoustic Neuroma K. Kevin Ho, M.D. Faculty Advisor: Vicente A. - - PowerPoint PPT Presentation

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Acoustic Neuroma K. Kevin Ho, M.D. Faculty Advisor: Vicente A. - - PowerPoint PPT Presentation

Acoustic Neuroma & Hearing Loss Acoustic Neuroma K. Kevin Ho, M.D. Faculty Advisor: Vicente A. Resto, M.D., Ph.D. The University of Texas Medical Branch Department of Otolaryngology Grand Rounds Presentation December 6, 2006 K. Kevin


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Acoustic 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

  • K. Kevin Ho, M.D.

Faculty Advisor: Vicente A. Resto, M.D., Ph.D. The University of Texas Medical Branch Department of Otolaryngology Grand Rounds Presentation December 6, 2006

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Medieval Times

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1912 Acoustic Neuroma Surgery

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

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Historical Perspectives (cont’d)

 1905 Dr. Harvey Cushing

 Meticulous dissection  Hemostasis: silver clips, bone wax,

electrocautery

 Mortality: 20 % (1917)  4% (1931)

 1916 Dr. Walter Dandy

 Complete removal of AN  Mortality: 10%

 Early 1960s Dr. William House

 Translabyrinthine approach using surgical

drill and operating microscope

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Cerebellopontine Angle: Anatomy

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Epidemiology

 6 % of all Intracranial tumors  80 - 90% of CPA tumors  Incidence in US: 10 per million / year  Vast majority in adulthood  95% Sporadic (unilateral)  5% Neurofibromatosis type 2 (bilateral)  No known race, gender predilection

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Pathogenesis

 Neither Neuroma or Acoustic (auditory)  Schwannoma arising from vestibular nerve  Benign tumor. Malignant degeneration

exceedingly rare.

 Majority originate within the IAC  Equal frequency on Superior and Inferior

vestibular nerves (controversial)

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Jackler Staging System

Stage Tumor Size Intracanalicular Tumor confined to IAC 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 Growth

 Intracanalicular:

 Hearing loss, tinnitus, vertigo

 Cisternal:

 Worsened hearing and dysequilibrium

 Compressive:

 Occasional occipital headache  CN V: Midface, corneal hypesthesia

 Hydrocephalic:

 Fourth ventricle compressed and obstructed  Headache, visual changes, altered mental status

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Phases of Tumor Growth

Jackler RK. 2000, p. 180: Tumors of the Ear and Temporal Bone Intracanalicular Cisternal Compressive Hydrocephalic

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Hearing Loss

 Most frequent initial symptom  Most common symptom ~ 95% AN patients  Asymmetric SNHL  Down-sloping / High Frequency  Decreased Speech Discrimination

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Serviceable Hearing

100 70 50 30 50

A D B C

P T T (dB) SDS (%)

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Distribution of Hearing in AN

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

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Pathophysiology of Hearing Loss in Acoustic Neuroma

 Exact etiology is unknown  Compressive effect on cochlear nerve  Vascular occlusion of internal auditory

artery

 Biochemical alterations inner ear fluids

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Normal or Symmetrical Hearing in Acoustic Neuroma

Selesnick 1993 Shaan 1993 Lustig 1998 Magdziarz 2000 AN patients 126 100 546 369 Normal hearing 5 (4%) 6 (6%) 29 (5%) 10 (3%)

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Tumor Size and Hearing

Normal Hearing (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 & Hearing

 Lack of conclusive correlation between 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 Rate

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

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Tumor Growth: Studies

N Follow-up No Growth (%)

  • 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 & Hearing

A D B A 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 Growth

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

Side Gender Initial Volume Age

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Estimating Tumor Growth

 Serial MRI with and without GAD The only reliable study to

estimate tumor growth rate

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Tumor Growth: Biomarkers

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

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Fibroblast Growth Factor Receptor

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

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Delayed Diagnosis

Duration of Symptoms Prior to Diagnosis

Symptoms Years Hearing Loss 3.9 Vertigo 3.6 Tinnitus 3.4 Headache 2.2 Dysequilibrium 1.7 Trigeminal 0.9 Facial 0.6

Jackler RK. 2000. Tumors of the Ear and Temporal Bone

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History and Physical

 Hearing Loss  Vertigo  Dysequilibrium  Tinnitus  Headache  Nystagmus

 Early small lesion: Horizontal (vestibular)  Late large: Vertical (brainstem compression)

 Cranial neuropathy

 CN V, VII  Lower cranial nerves (IX-XII)

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Frequency of Symptoms

 Hearing Loss

(85-97% ; 94% )

 Vertigo

(5-70 % ; 39% )

 Dysequilibrium

(46-70% ; 56 %)

 Tinnitus

(56-70% ; 64 %)

 Facial nerve

(10-77% ; 38 %)

 Trigeminal nerve

(16-63% ; 26 %)

 Headache

(12-38% ; 25% )

 Visual symptoms

(1- 15 % ; 7% )

 Lower cranial nerves: Dysphagia, Hoarseness, Aspiration,

Shoulder weakness (Jugular foramen syndrome)

Jackler RK. 2000, p. 182: Tumors of the Ear and Temporal Bone

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Symptoms in AN patients with Normal Hearing

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

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Sudden Sensorineural Hearing loss

 Idiopathic  1-2 % SSNHL patients have AN  10- 26 % AN patients have a history of SSNHL  Most experts advocate obtaining MRI in all

patients who present with SSNHL

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Diagnosis

 History and Physical Exam  Audiology testing:

 Audiogram  ABR  OAE

 Vestibular testings (eg. ENG, rotary chair,

posturography) all lack diagnostic value

 Radiography

 MRI

Gold Standard

 CT

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Pure Tone and Speech Audiometry

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ABR: Retrocochlear Pathology

 Increased interpeak intervals

 I-to-III interval of 2.5 ms, III-to-V interval 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 AN

 10-20 % with only

wave I and nothing thereafter

 40-60 % with wave V

latency delay

 10-15 % have normal

findings

Fraysse B et al. First International Conf. on Acoustic Neuroma. 1992

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ABR: Diagnostic Efficiency

 Generally, Efficiency increases with Size  Sensitivity: > 90 % for tumor > 3 cm  No response for severe/ profound SNHL (Rupa 2003)  False negative Rate:

 15 % (Wilson 1992 – 6/40)

 33 % (5/15) for Intracanalicular Tumor

 False positive Rate:

 > 80 % (Jackler 2005)

 Positive predictive value:

 15 % (Weiss 1990 – 4/26)  12 % (Walsted 1992 – 23/185)

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ABR: Sensitivity & Tumor size

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

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IT 5 & Tumor Size

Chandrasekhar SS et al. Am J Otol 1995;16:63-7

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Stacked ABR

 Attempt to improve

detection rate in small < 1 cm ANs

 “Stacking” of derived

band response

 Out of 25 ANs, 5

tumors less than 1 cm missed in Standard ABR were picked up by Stacked ABR.

Don M et al. Am J. Otology; 1997: 21; 148-151

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OAE

 Reflect cochlear/ OHC / sensory hearing  Not primarily used as screening tool  Presence of OAE in SNHL ↔ Retrocochlear  However, 50 % AN demonstrate both cochlear and

retrocochlear hearing loss

 Risk stratification for hearing preservation surgery Kim AH. Otol Neurotol. 2006 Apr;27(3):372-9

Preoperative TEOAE

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MRI Brain w. & w/o GAD

 T1:

Isointense to brain, hyperintense to CSF

 T2:

Hyperintense to brain, hypointense to CSF

 T1+Gad: Enhancing

T1 pre-Gad T1 post-Gad T2

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CT Brain with contrast

 Heterogeneous

enhancement on contrast

 Rare calcification  Contraindication to MRI

(metallic implants), claustrophobic patients

 May not be able to detect

small tumor < 1.5cm

 Radiation

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Treatment options

 Observation  Surgery

 Translabyrinthine  Retrosigmoid  Middle fossa

 Radiotherapy

 Conventional  Stereotactic

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Conservative Management

 Advanced age (> 65 )  Short life expectancy (< 10 years)  Slow growth rate  Poor surgical candidate / poor general health  Minimal symptoms  Only hearing ear  Patience preference

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Observation: Raut 2004

 Prospective cohort study of 72 patients

 Age at presentation: 60.8 years  Mean follow-up: 80 months

 Mean tumor size at diagnosis: 9.4 mm  Mean tumor growth rate: 1 mm/ year  87% growth rate < 2 mm/ year  Tumor growth

 + : 39 %  0: 42%  - : 19%

 No correlation between growth and age, gender,

size at presentation, or presenting symptoms

 32 % failed conservative management Raut V et a.: Clin Otolaryngol 29:505–514, 2004.

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Preop Predictive factors for Hearing Preservation Surgery

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

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Loss of Serviceable Hearing during Observation

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

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Conclusions

 Tumor size has no correlation with

audiovestibular symptoms in Acoustic neuroma

 Understanding tumor growth rate is important

for predicting symptom progression and treatment planning

 The study-of-choice to estimate tumor growth

is serial MRI

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Thank You