| Acoustic
Neuroma |
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Acoustic
neuromas are benign tumors arising from the vestibular nerve
in the base of the skull. These tumors grow slowly, expanding
into the cranial cavity, compressing the brainstem and causing
cranial nerve dysfunction. Affected individuals develop progressive
hearing loss in one ear, ringing in the ear, a sense of imbalance,
loss of sensation in the face and weakness of facial muscles.
Very large tumors cause headaches, double vision and hydrocephalus.
Interestingly, the surgical treatment of acoustic neuromas
parallels the history of the development of neurosurgery.
Acoustic
neuromas are rare. The incidence is 10 per million population
per year or about 2,800 new cases in the US each year. These
tumors are only rarely associated with genetic errors which
are passed to succeeding generations.
In
Neurofibromatosis2 a genetic error is passed as
an autosomal dominant trait ( one half of children maybe affected)
and this error results in acoustic neuromas on both sides.
Microsurgical
removal and radiosurgery are effective methods of treating
AN. Detailed information regarding the effects of treatment,
the risks and complications, long term effects and natural
history of untreated tumors form an important basis
for every patient's informed decision regarding their own
treatment.
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Natural
History
Several
studies have detailed the growth pattern of untreated AN.
About 50% of tumors grow slowly... 1 to 2 millimeters per
year. For each patient the growth rate is constant and can
be predicted after 1 to 2 years of observation with serial
MR scans. In about 20% the growth rate is more rapid: more
than 2 mm per year. Finally tumors seem not grow in more than
30% of patients (some even become smaller). Malignancy
is extremely rare.
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Microsurgical
Removal
The
success of surgical removal of these tumors is a testament
to the skills and resourcefulness of neurosurgeons and otologists.
At the turn of the century, open operation was a risky proposition.
The mortality rate was 80%. Now the operative mortality rate
is 1% and the likelihood of surgical cure greater than 95%.
In recent years surgeons have turned the emphasis from reduction
of the mortality rate to reduction in complication rates and
preservation of cranial nerve function. Now a days patients
can expect to have near normal facial movement, sensation
and sometimes even hearing preservation following microsurgical
removal.
A
meta-analysis of the results of various microsurgical series
is difficult as it is difficult to control for the many risk
factors which influence the outcome of surgery. For example,
the preservation of facial movement and hearing is highly
influenced by tumor size.
The
following table summarizes several contemporary studies of
microsurgery:
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Year
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#Pts.
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Cntrl.
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Recur.
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Facial
Movement
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Hearing
Preserve
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Comp.
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Death
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OJEMANN
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1993
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410
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97%
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3%
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96%
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36%
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10.5%
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0.5%
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HOUSE
|
1982
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216
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99.5%
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83%
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40%
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10.6%
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0.4%
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HARDY
|
1989
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100
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97%
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0%
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82%
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16%
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18%
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3%
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SAMII
|
1997
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1000
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98%
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0.7%
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15%
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20%
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1.1%
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TOS
|
1988
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300
|
|
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87%
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10.5%
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2%
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EBERSOLD
|
1992
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256
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97%
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3%
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92%
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49%
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28%
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0.7%
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SCRIPPS
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1994
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11
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91%
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9%
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91%
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18%
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9%
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0%
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AVERAGE
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2293
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98%
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1.7%
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90%
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27%
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17%
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1.1%
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The
series of 1,000 patients personally operated by Professor
Samii is a notable example of microsurgical results in the
best of hands. He achieved total removal in 97.9% with a recurrence
rate of 0.7% (without NF-2). Few patients had significant
facial weakness before operation, while 27% had significant
post op weakness. Most patients had reduced hearing prior
and no hearing after operation. The mortality rate was 1.1%.
Total complications were approximately 20% included cerebrospinal
leak, meningitis, hemorrhage, hydrocephalus and about 4% with
severe complications leading to permanent disability.
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Gamma
Knife Radiosurgery
Gamma
Knife radiosurgery
has been used to treat acoustic neuromas for the past quarter
century. Focused, high dose radiation was conceived by Professor
Lars Leksell as a means of non-invasive treatment of movement
disorders and pain. Shortly after its introduction as a surgical
instrument in 1968, it was used to treat brain tumors and
vascular malformations. The first report of GK treatment of
AN was in 1971 and by the end of 1997 more than 8,000 patients
have been treated worldwide. Modern computer graphics and
brain imaging technology have resulted in a very effective
and safe method of treating acoustic neuromas on an outpatient
basis.
Unlike
microsurgery, GK radiosurgical results are less dependent
on the individual skills of the surgeon, since the conformal
treatment planning software, dose schedules and even the machine
are virtually identical among the 112 units worldwide. The
results in San Diego, Pittsburgh, Guadalajara, Stockholm,
Beijing, Tokyo, New Delhi and Singapore will be the same.
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The
series reported by Pollock from the University of Pittsburgh
is interesting because they had a similar group of 47
patients treated by microsurgery for comparison. After 3 years
the control rate was 94% for radiosurgery and 98% for microsurgery.
There were no deaths. Complications occurred in 38% of operated
patients, and 33% required further surgery. Complications
occurred in 28% of radiosurgery patients, and 13% needed more
surgery. Radiosurgery compared favorably with microsurgery
in terms of preservation of useful hearing and facial movement.
After 3 years 63% of surgical and 83% of radiosurgical patients
had normal facial movement. Among those with useful hearing
before operation, 14% of operated and 75% of radiosurgical
patients had serviceable hearing. Hospital stay, charges were
lower for radiosurgery patients, while patient acceptance
was greater.
Finally,
long term results have been reported for Gamma Knife radiosurgery
in the treatment of acoustic neuromas. Kondziolka and others
reported the results of 162 consecutive patients who underwent
radiosurgery between 1987 and 1992 (NEJM 1998;339:1426-1433)
yielding a 5 to 10 year follow-up. Control rate was 98%. Two
thirds of the tumors became smaller. Four patients were operated
within four years and the surgeon described the resection
as no more difficult than unradiated tumors in three of the
four. No further growth was noted after 4 years. Normal facial
movement was found in 79 percent, and no patient with normal
facial movement before radiation developed complete paralysis.
There was no change in hearing in 51% and 47% of those with
useful hearing before treatment preserved useful hearing.
There
are no reports of radiosurgery causing malignancy, and anecdotal
reports of the difficulty of operated individuals previously
treated by radiosurgery are unsubstantiated. Needless to say,
the unusual complications of open surgery...infection, cerebrospinal
fluid leak, hemorrhage etc. can be avoided. In the rare case
of recurrent growth radiosurgery can be repeated.
Our
surgeons have extensive experience in both the microsurgery
and Gamma Knife radiosurgery of acoustic neuromas. We welcome
your inquiries regarding your care.
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