THE AMERICAN JOURNAL OF OTOLOGY/VOLUME 15, NUMBER 1 January 1994

CLINICAL VIGNETTE

In this era of double blinded, statistically valid research, there remains a place for
empiric or even anecdotal observation. This section of the Journal creates a vehicle for
the communication of such observation.
Content should be regarded as interesting or thought provoking or as a novel
"discovery" that is not presented as valid science, statistically or otherwise. The opinions
and observations are those of the author. Any recommendation as to standard of care
or treatment guidelines is neither explicit not implied.
It is our hope that this section will stimulate dialogue and an exchange o f
experience. The perspective of "observation" should not be displaced.
The Editors

ACOUSTIC NEUROMA IN PREGNANCY

Karen Jo Doyle, Ph.D., M.D., and William M. Luxford, M.D.

ABSTRACT
Acoustic neuroma in the pregnant patient has been described infrequently. The
symptoms of acoustic neuroma can commence or worsen during the last 3 or 4 months
of pregnancy. In women, acoustic tumors have been shown generally to be larger and
more vascular, and some acoustic tumors contain estrogen receptors. This is a report of
our management of two patients with acoustic neuroma who presented early in
pregnancy. Surgery was delayed to the second trimester in each, to avoid spontaneous
abortion. Both patients underwent translabyrinthine tumor removal at 18-19 weeks
gestation, and each had an uncomplicated postoperative course. Examination of the
tumor for estrogen receptors was performed for the second patient and was negative.
Uncomplicated acoustic neuroma surgery can be performed in pregnant patients during
the second trimester.

The problems o f diagnosis and treatment o f

acoustic tumors are complicated by pregnancy.
Acoustic tumors are statistically more common in
women, and the larger, more vascular tumors are
twice as common in women.' Though case reports of
known acoustic tumors in pregnant patients are few,
they generally show that the signs and symptoms of
these tumors can dramatically worsen during the last
3 or 4 months of pregnancy." Some acoustic tumors
contain estrogen receptors, and it has been postulated that estrogen may be the factor involved in such
accelerated symptoms i n pregnancy.' One author
previously has recommended termination o f pregnancy to avoid the accelerated growth phase of tumors in neurofibromatosis.5

This report presents two cases o f acoustic

neuroma in pregnancy. The presenting complaints,
work-up, surgical treatment, tumor characteristics,
and outcome are outlined for each patient. The
possible role of estrogen receptors is discussed, and
recommendations are given for the timing of surgery.
CASE REPORTS
Patient 1
A 28-year-old woman was initially seen at the
House Ear Clinic in August 1991. She had developed
a sudden left ear hearing loss in October 1990 that

House Ear Clinic, Los Angeles, California

Presented at the Western Section Triologic Society Meeting, Seattle, Washington, January 9, 1992.
Reprint requests: William L. Luxford, M.D., House Ear Clinic, 2100 W. Third Street, Los Angeles, CA 90057

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THE AMERICAN JOURNAL OF OTOLOGY/VOLUME 15, NUMBER 1 January 1994

persisted. After initial treatment with decongestants

and antibiotics she had an audiogram in January
1991 that showed a left-sided 40 dB HL sensorineural
hearing loss at the frequencies 750, 1000, and 2000
Hz, with normal hearing at both the lower and higher
audiometric frequencies. Speech discrimination was
76 percent. Magnetic resonance imaging (MRI) revealed a 2.0-cm mass filling the left internal auditory
canal and involving the left cerebellopontine angle.
Surgery was scheduled for November 1991, but in
October 1991 she was found to be pregnant. Obstetric consultation was obtained, and pelvic ultrasound
performed on October 28 1991 demonstrated an 8to 9-week single uterine pregnancy. The patient was
desirous o f continuing the pregnancy, and i t was
recommended that she undergo resection o f the
tumor during the second trimester to minimize the
risks of general anesthesia.
On January 6, 1992 she underwent translabyrinthine tumor removal, under general anesthesia. The
tumor originated from the inferior vestibular nerve.
The facial nerve was left intact. Continuous fetal
monitoring was performed and was unremarkable
throughout the procedure. Operating time was 3
hours, 15 minutes. Estimated blood loss was 200 mL.
The tumor was sent for estrogen receptor assay, and
there were no estrogen or progesterone receptors
present in the tissue. The patient had an uncomplicated postoperative course, and on follow-up examination at 1 week she had normal facial nerve function. She delivered a healthy infant at term.
Patient 2
A 29-year-old woman in the eleventh week of her
first pregnancy was referred to the House Ear Clinic
in July 1990, f o r treatment o f a right acoustic
neuroma. She had a 7-year history of gradually decreasing hearing in the right ear with some fluctuation and also noted 4 years of increasing right facial
numbness. Audiometry showed a 65-70 dB H L flat
sensorineural hearing loss on the right, with 20 percent speech discrimination. A 4.5-cm right acoustic
neuroma was found on MRI. High-risk obstetric consultation was obtained and clearance was given for
surgery. She had a translabyrinthine tumor removal
at 18 weeks of pregnancy. The tumor extended laterally in the internal auditory canal and involved the
superior and inferior vestibular nerves. The facial
nerve was left intact. Operating time was 4 hours, 45
minutes, and the estimated blood loss was 500 mL.
The tumor was not assayed for estrogen or progesterone receptors. The patient had an uncomplicated
postoperative course, and had gradual return of facial function/ to House-Brackmann grade I I I / V I at
1 year postoperatively. She delivered a healthy infant at term.

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DISCUSSION
Harvey Cushing, in 1917, was the first to publish
the observation that growth of acoustic neuroma may
be accelerated during pregnancy.' The last published
report of acoustic neuroma in pregnancy, published
in 1974, described eight cases dating from 1912 to
1972, in which acoustic neuroma was unsuspected
and undiagnosed until after delivery.' Four of these
eight cases had onset o f symptoms before the first
pregnancy, and o f these four patients, three had
acceleration of their signs and symptoms during the
last part of the first pregnancy. Three other women
developed the initial symptoms and signs of acoustic
neuroma during the last months of their first pregnancies. The eighth patient had bilateral acoustic
neuromas that were seemingly unaffected by pregnancy. The authors attributed the aggravation o f
tumor symptoms in late pregnancy to either hormonal stimulation of tumor growth during pregnancy or
hormonally induced vascular dilation within the tumor.
The advent of improved diagnostic tests for the
detection of acoustic neuroma has permitted early
diagnosis, as in our two cases. In patient 1, the tumor
was diagnosed before the first pregnancy, and was of
a medium size. Patient 2 had a giant tumor that was
symptomatic, but was not diagnosed until her first
pregnancy. In both patients, diagnosis made early in
pregnancy allowed definitive treatment during the
second trimester, to avoid accelerated tumor growth
that may occur in the last trimester.
Techniques of general anesthesia and monitoring have advanced to the point that non-elective
surgery can be performed safely in pregnant mothers, with some limitations.7 The rate of spontaneous
abortion is greatest during the first trimester with
inhaled anesthetic agents, as is the risk of fetal teratogenicity. Risks to the mother increase during the
last trimester of pregnancy, owing to changes in drug
distribution secondary to expanded blood volume,
hemodilution, reduction in functional residual respiratory capacity, increased oxygen consumption predisposing to hypoxemia, and capillary venous engorgement o f the airway. Surgery with general
anesthesia performed during the second trimester
minimizes risks to both fetus and mother. Our two
patients had general anesthesia without complications.
With the discovery that breast carcinomas contain estrogen receptors and could be treated with
antiestrogen therapy,8 the search began for hormone
receptors in other tumors. Meningiomas, which occur more frequently in women and may also show
accelerated growth in pregnancy,' can exhibit estradiol binding. Martuza et all found estradiol binding
in 7 of 10 meningiomas. Similarly, studies of acoustic
neuroma patients have hinted at a link between sex
hormone activity and tumor behavior.

CLINICAL VIGNETTE

Kasantikul et al found that 58 percent of their

acoustic neuroma patients were women and that
large and giant tumors occurred twice as often in
women.' They also found that the acoustic tumors in
women are more vascularized and collagenized and
are more frequently associated with symptoms o f
short duration. They noted that the only three reports of acoustic neuroma presenting as subarachnoid hemorrhage were all in women.
Several researchers have looked specifically for
estrogen and/or progesterone binding in acoustic
neuromas. The earliest study, by Kasantikul and
Brown in 1981, used a qualitative fluorescent histochemical technique t o look at tissue from eight
acoustic neuromas and concluded that estrogen receptors were present in every tumor studied.' But
since that time, more sophisticated quantitative assays have been developed for estrogen receptor measurement. The most commonly studied method is the
radioimmunoassay dextran-coated charcoal method."
In this method, homogenized tumor tissue is incubated with radiolabeled estrogen or progesterone.
Receptor-bound steroids are then separated by adsorption o f unbound steroids onto dextran-coated
charcoal. Bound hormone radioactivity is then measured, and results expressed in femtomoles (fmol) of
steroid hormone bound to receptors per milligram
of tissue protein. If 10 fmol or greater receptor protein per milligram is found, the tissue is considered
positive for estrogen receptors. Of eight studies using
this assay, four have found estrogen receptors in a
minority of their tumor samples, ranging from 10 to
25 percent.10,12-14 The other four studies found no
evidence of estrogen receptors in a total of 44 tumor
specimens.15-18 Other techniques for measurement
of estrogen receptors in acoustic neuroma tissue include the sucrose gradient method and a monoclonal
antibody method. Two studies using the sucrose gradient method had conflicting results in that one
found that 4 of 8 tumor specimens were positive for
estrogen receptors, whereas the other found 0 o f
2 tumors positive f o r estrogen receptors.190 The
two studies employing monoclonal antibody methods found 0 o f 28 tumors positive f o r estrogen
receptors.15'2I
To summarize the literature on estrogen receptors in acoustic neuromas, there is at most a small
subset of these tumors that exhibit specific estrogen
binding. Our single pregnant patient in whom estrogen receptor assay was negative is in agreement with
this finding. It is unlikely that antiestrogen therapy
will be applicable t o the treatment o f acoustic
neuroma. Previous literature does not support estrogen binding by acoustic neuromas as the explanation
for the clinical observations that acoustic neuromas
are more common, larger, and more vascular in

women. Other factors that contribute to these differences remain to be discovered.

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