Acoustic Neuroma &

Acoustic Neuroma
Hearing Loss

K. Kevin Ho, M.D.

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

Medieval Times

1912 Acoustic Neuroma Surgery

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

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

Tumor confined to IAC

I (small)

< 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

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 (%)

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

AN
patients
Normal
hearing

Selesnick
1993

Shaan
1993

Lustig
1998

Magdziarz
2000

126

100

546

369

5
(4%)

6
(6%)

29
(5%)

10
(3%)

Tumor Size and Hearing

Normal Hearing
(29 Patients)

All ANs
(126 Patients)

% Small
(< 1cm)

45

24

% Medium

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

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

> 20 mm

Tumor Growth Rate

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

Tumor Growth: Studies

Bederson

Follow-up

70

26 mo

Selesnick 558

+
No
Growth Growth Growth
(%)
(%)
(%)
40
7
53

3 yr

54

Charabi

126

3.8 yr

12

82

Raut

72

80 mo

42

19

39

Walsh

72

3.2 yr

50

14

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; 86-92

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
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
Vertigo
Dysequilibrium
Tinnitus
Facial nerve
Trigeminal nerve
Headache
Visual symptoms

(85-97% ; 94% )
(5-70 % ; 39% )
(46-70% ; 56 %)
(56-70% ; 64 %)
(10-77% ; 38 %)
(16-63% ; 26 %)
(12-38% ; 25% )
(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

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 Neuroma. 1992

ABR: Diagnostic Efficiency

Generally, Efficiency increases with Size

Sensitivity: > 90 % for tumor > 3 cm
No response for severe/ profound SNHL
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)

(Rupa 2003)

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 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
Risk stratification for hearing preservation surgery

Preoperative TEOAE

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

MRI Brain w. & w/o GAD

T1 pre-Gad

T1:
T2:
T1+Gad:

T2

T1 post-Gad

Isointense to brain, hyperintense to CSF

Hyperintense to brain, hypointense to CSF
Enhancing

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
Translabyrinthine
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); 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