Acoustic Neuroma & Acoustic Neuroma Hearing Loss

K. Kevin Ho, M.D. Vicente A. Resto, M.D., Ph.D. Department of Otolaryngology University of Texas Medical Branch

Medieval Times

1912 Acoustic Neuroma Surgery

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

1905 Dr. Harvey Cushing

Historical Perspectives (contd)

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

Anatomy

Cerebellopontine Angle: Anatomy

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

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

Jackler Staging System

Stage Intracanalicular I (small) II (medium) III (Large) IV (Giant) Tumor Size Tumor confined to IAC < 10 mm 11-25 mm 25-40 mm > 40 mm

Phases of Tumor Growth

Intracanalicular:

Hearing loss, tinnitus, vertigo Worsened hearing and dysequilibrium Occasional occipital headache CN V: Midface, corneal hypesthesia Fourth ventricle compressed and obstructed Headache, visual changes, altered mental

Cisternal:

Compressive:

Hydrocephalic:

Phases of Tumor Growth

Cisternal

Intracanalicular

Compressive

Hydrocephalic

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

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

Serviceable Hearing
SDS (%)

0
P T T (dB)

100

70

50

A
30

B
50

Distribution of Hearing in AN

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

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

Normal or Symmetrical Hearing in Acoustic NeuromaLustig Magdziar Selesnic Shaan

k 1993 1993 1998 z 2000 369

AN patients Normal hearing

126

100

546

5 (4%)

6 (6%)

29 (5%)

10 (3%)

Tumor Size and Hearing

Normal All ANs Hearing (126 Patients) (29 Patients) % Small (< 1cm) % Medium
(1-3 cm)

45 42 12

24 59 16

% Large (> 3 cm)

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

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

Tumor Growth Rate

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

Tumor Growth: Studies

N FollowNo + up Growt Growt Growt h (%) h (%) h (%) 26 mo 3 yr 3.8 yr 80 mo 3.2 yr 40 12 42 50 7 6 19 14 53 54 82 39 37

Bederso 70 n Selesnic 558 k Charabi 126 Raut Walsh 72 72

Tumor Growth & Hearing

D B A B D Change in Tumor Volume (mm3) A

Change in Tumor Volume (mm3)

PTA

SDS

Massick DD. Laryngoscope 2000: 110; 1843-9

Predicting Tumor Growth

Side Gender

Initial Volume

Age

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

Estimating Tumor Growth

Serial MRI with and without GAD The only reliable study to estimate tumor growth rate

Tumor Growth: Biomarkers

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

Fibroblast Growth Factor Receptor

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

Delayed Diagnosis
Duration of Symptoms Prior to Diagnosis
Symptoms Years Hearing Loss Vertigo Tinnitus Headache Dysequilibrium Trigeminal Facial 3.9 3.6 3.4 2.2 1.7 0.9 0.6

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

History and Physical

Hearing Loss Vertigo Dysequilibrium Tinnitus Headache Nystagmus

Early small lesion: Horizontal (vestibular) Late large: Vertical (brainstem compression) CN V, VII Lower cranial nerves (IX-XII)

Cranial neuropathy

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

Symptoms in AN patients with Normal Hearing

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

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

Diagnosis

History and Physical Exam Audiology testing:

Audiogram ABR OAE

Vestibular testings (eg. ENG, rotary chair, posturography) all lack diagnostic value Radiography

MRI CT

Gold Standard

Pure Tone and Speech Audiometry

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

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

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:

ABR: Sensitivity & Tumor size

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

IT 5 & Tumor Size

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

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

OAE
Reflect cochlear/ OHC / sensory hearing Not primarily used as screening tool Presence of OAE in SNHL Retrocochlear However, 50 % AN demonstrate both cochlear Preoperative TEOAE and retrocochlear hearing loss Risk stratification for hearing preservation surgery

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

MRI Brain w. & w/o GAD

T1 pre-Gad

T2

T1 post-Gad

T1: CSF T2: to CSF

Isointense to brain, hyperintense to t

Hyperintense to brain, hypointense

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

Treatment options

Observation Surgery
Translabyrint hine Retrosigmoid Middle fossa

Radiotherapy
Conventional Stereotactic

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

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 Raut V et a.: conservative management 32 % failed Clin Otolaryngol 29:505514, 2004.

Preop Predictive factors for Hearing Preservation Surgery

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

Loss of Serviceable Hearing during Observation

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

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

Thank You