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.
University of Texas
UTMB Medical Branch
Otolaryngology
Medieval Times
1912 Acoustic Neuroma Surgery

Jackler RK. 2000, p. 173: Tumors of the Ear and Temporal Bone
 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
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:
 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
 Phases of Tumor Growth

Intracanalicular Cisternal

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 (%)
100 70 50 0
0
A
P
T 30
T
(dB) B
D
50

C
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
Selesnick Shaan Lustig Magdziar
1993 1993 1998 z
2000

AN 126 100 546 369

patients
Normal 5 6 29 10
hearing (4%) (6%) (5%) (3%)
 Tumor Size and Hearing
Normal Hearing All ANs
(29 Patients) (126 Patients)
% Small 45 24
(< 1cm)
% Medium 42 59
(1-3 cm)
% Large 12 16
(> 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 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
 Tumor Growth & Hearing

D B
A

B
D

Change in Tumor Volume (mm3)

Change in Tumor Volume (mm3)

PTA SDS

Massick DD. Laryngoscope 2000: 110; 1843-9

Predicting Tumor Growth

Side Gender

Initial Age
Volume

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 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
 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 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 Gold Standard
 CT
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:
 15 % (Weiss 1990 – 4/26)
 12 % (Walsted 1992 – 23/185)
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 T2 T1 post-Gad
 T1: Isointense to brain, hyperintense to CSF
 T2: Hyperintense to brain, hypointense to CSF
 T1+Gad: 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
 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.
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