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

1912 Acoustic Neuroma

Surgery

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

Historical Perspectives
(contd)

1905 Dr. Harvey Cushing

1916 Dr. Walter Dandy

Meticulous dissection
Hemostasis: silver clips, bone wax,
electrocautery
Mortality: 20 % (1917) 4% (1931)

Complete removal of AN
Mortality: 10%

Early 1960s Dr. William House

Translabyrinthine approach using

surgical drill and operating
microscope

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 predilection

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)

Jackler Staging System

Stage

Tumor Size

Intracanalicular
I (small)

Tumor confined to
IAC
< 10 mm

II (medium)

11-25 mm

III (Large)

25-40 mm

IV (Giant)

> 40 mm

Phases of Tumor Growth

Intracanalicular:

Cisternal:

Worsened hearing and dysequilibrium

Compressive:

Hearing loss, tinnitus, vertigo

Occasional occipital headache

CN V: Midface, corneal hypesthesia

Hydrocephalic:

Fourth ventricle compressed and obstructed

Headache, visual changes, altered mental
status

Phases of Tumor Growth

Intracanalicular

Compressive

Cisternal

Hydrocephal

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

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

Serviceable Hearing
SDS (%)

100

70

50

A
P
T
T
(dB)

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
Neuroma
Selesni Shaan
Lustig Magdzi
Selesni Shaan
ck 1993 1993

AN
patient
s
Norma
l
hearin
g

Lustig
1998

Magdzi
arz
2000

126

100

546

369

5
(4%)

6
(6%)

29
(5%)

10
(3%)

Tumor Size and Hearing

% Small
(< 1cm)
% Medium

Normal
All ANs
Hearing
(126 Patients)
(29 Patients)
24
45
42

59

12

16

(1-3 cm)

% 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

Beders
on
Selesni
ck
Charab
i
Raut
Walsh

70
55
8
12
6
72
72

FollowNo
+
up
Growt Growt Growt
h (%) h (%) h (%)
26 mo
40
7
53
3 yr

54

3.8 yr

12

82

80 mo
3.2 yr

42
50

19
14

39
37

Tumor Growth & Hearing

B

A
A
B
D

Change in Tumor Volume (mm3)

PTA

Change in Tumor Volume (mm3)

SDS

Massick DD. Laryngoscope 2000: 110; 1843-9

Predicting Tumor Growth

Side

Initial
Volume

Gender

Age

Herwadker A. Otology and Neurotology 2005: 26; 86-9

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

Hearing Loss
Vertigo
Tinnitus
Headache
Dysequilibrium
Trigeminal
Facial

Years

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)

Cranial neuropathy

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

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 Tempora

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

Interaural wave V latency difference

(IT5)

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

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 Neuro

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)

False positive Rate:

33 % (5/15) for Intracanalicular Tumor

> 80 % (Jackler 2005)

Positive predictive value:

15 % (Weiss 1990 4/26)

12 % (Walsted 1992 23/185)

ABR: Sensitivity & Tumor

size

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

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 by Stacked
ABR.

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 and retrocochlear hearing loss
RiskTEOAE
stratification for hearing
Preoperative
preservation surgery

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

MRI Brain w. & w/o GAD

T1 pre-Gad

T1:
to CSF
T2:
to CSF

T2

T1 post-Gad

Isointense to brain, hyperintense

Hyperintense to brain, hypointense

hypointens

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

Treatment options

Observation
Surgery
Translabyrinth
ine
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

Mean tumor size at diagnosis: 9.4 mm

Mean tumor growth rate: 1 mm/ year
87% growth rate < 2 mm/ year
Tumor growth

Age at presentation: 60.8 years

Mean follow-up: 80 months

+ : 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 29:505514, 2004.

Preop Predictive factors for

Hearing Preservation
Surgery

Rohit MS et al. Ann. Oto. Rhino. Laryng. 2006: 115 (1); 4

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