Acoustic Neuroma

Author: Joe Walter Kutz Jr, MD, FACS; Chief Editor: Arlen D Meyers, MD,
MBA
Udated: Mar !", #$!%
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Background
A(ousti( neuro'as are intra(ranial, e0tra-a0ial tu'ors that arise fro' the S(h/ann
(ell sheath in.estin1 either the .esti2ular or (o(hlear ner.e) As a(ousti( neuro'as
in(rease in size, they e.entually o((uy a lar1e ortion of the (ere2elloontine an1le)
A(ousti( neuro'as a((ount for aro0i'ately *$3 of tu'ors found /ithin the
(ere2elloontine an1le) 4he re'ainin1 #$3 are rin(ially'enin1io'as) 5n rare
(ases, a fa(ial ner.e neuro'a, .as(ular tu'or, lio'a, or 'etastati( lesion is found
/ithin the (ere2elloontine an1le)
History of the Procedure
6.er the last *$-"$ years, the oerati.e 'ortality rate has droed dra'ati(ally fro'
%$3 at the 2e1innin1 of the (entury to less than !3 in the last de(ade)
7ostoerati.e fa(ial aralysis, on(e the rule, is no/ an un(o''on er'anent
se8uelae of a(ousti( tu'or sur1ery) Atte'ts at hearin1 (onser.ation, uni'a1ina2le at
the 2e1innin1 of the #$th (entury, are in(reasin1ly su((essful)
4hese .ery dra'ati( i'ro.e'ents are the result of the (on.er1en(e of se.eral
fa(tors) 9astly i'ro.ed i'a1in1 te(hni8ues er'ittin1 early dia1nosis, adatation of
the 'i(ros(oe to the oeratin1 theater, de.elo'ent of fa(ial and auditory ner.e
'onitorin1 te(hni8ues, i'ro.ed anesthesia, and i'ro.ed erioerati.e 'ana1e'ent
ha.e all (ontri2uted to i'ro.ed out(o'es
Epidemiology
Frequency
Clini(ally dia1nosed a(ousti( neuro'as o((ur in $)+-!)$ eole er !$$,$$$
oulation) 4he in(iden(e 'ay 2e risin1, a refle(tion of the in(reasin1 fre8uen(y /ith
/hi(h s'all tu'ors are 2ein1 dia1nosed /ith the 'ore /idesread use of M:5) A
#$$; study 2y <in et al su11ested the re.alen(e of in(idental a(ousti( neuro'as to
2e # in !$,$$$ eole)
=!>
Careful autosy studies (an dete(t s'all .esti2ular
s(h/anno'as in a hi1her er(enta1e of elderly atients, /hi(h su11ests that 'any
a(ousti( neuro'as ne.er 2e(o'e (lini(ally aarent)
Etiology
Most atients dia1nosed /ith an a(ousti( neuro'a ha.e no aarent ris? fa(tors)
E0osure to hi1h-dose ionizin1 radiation is the only definite en.iron'ental ris? fa(tor
asso(iated /ith an in(reased ris? of de.eloin1 an a(ousti( neuro'a) Multile studies
ha.e deter'ined (ell hone use is not asso(iated /ith an in(reased ris? of de.eloin1
an a(ousti( neuro'a, althou1h data on the effe(ts of lon1-ter' (ell hone use are still
endin1)
@eurofi2ro'atosis tye 55 o((urs in indi.iduals /ho ha.e defe(ti.e tu'or suressor
1ene lo(ated on (hro'oso'e ##8!#)#) 4he defe(ti.e rotein rodu(ed 2y the 1ene is
(alled 'erlin or s(h/anno'in) Bilateral a(ousti( tu'ors are a rin(ile (lini(al
feature of neurofi2ro'atosis tye 55, althou1h other 'anifestations, in(ludin1
eriheral neurofi2ro'ata, 'enin1io'a, 1lio'a, and Au.enile osterior su2(asular
lenti(ular oa(ities, are often resent as /ell) 7eriheral neurofi2ro'ato'a and (afe
au lait sots, ho/e.er, are 'u(h less fre8uently o2ser.ed than is tyi(al in
neurofi2ro'atosis tye 5) Many atients /ith neurofi2ro'atosis tye 55 resent in late
adoles(en(e or early adulthood 2ut o((asionally 'ay resent later in the fifth to
se.enth de(ade /ith slo/ly 1ro/in1 tu'ors)
Pathophysiology
4he .ast 'aAority of a(ousti( neuro'as de.elo fro' the S(h/ann (ell in.est'ent of
the .esti2ular ortion of the .esti2ulo(o(hlear ner.e) <ess than ;3 arise fro' the
(o(hlear ner.e) 4he suerior and inferior .esti2ular ner.es aear to 2e the ner.es of
ori1in /ith a2out e8ual fre8uen(y) 6.erall, B searate 1ro/th atterns (an 2e
distin1uished /ithin a(ousti( tu'ors, as follo/s: C!D no 1ro/th or .ery slo/ 1ro/th,
C#D slo/ 1ro/th Cie, $)# ('&y on i'a1in1 studiesD, and CBD fast 1ro/th Cie, E !)$ ('&y
on i'a1in1 studiesD) Althou1h 'ost a(ousti( neuro'as 1ro/ slo/ly, so'e 1ro/ 8uite
8ui(?ly and (an dou2le in .olu'e /ithin , 'onths to a year)
Althou1h so'e tu'ors adhere to one or another of these 1ro/th atterns, others
aear to alternate 2et/een eriods of no or slo/ 1ro/th and raid 1ro/th) 4u'ors
that ha.e under1one (ysti( de1eneration Cresu'a2ly 2e(ause they ha.e out1ro/n
their 2lood sulyD are so'eti'es (aa2le of relati.ely raid e0ansion 2e(ause of
enlar1e'ent of their (ysti( (o'onent) Be(ause a(ousti( tu'ors arise fro' the
in.estin1 S(h/ann (ell, tu'or 1ro/th 1enerally (o'resses .esti2ular fi2ers on the
surfa(e) Destru(tion of .esti2ular fi2ers is slo/; (onse8uently, 'any atients
e0erien(e little or no dise8uili2riu' or .erti1o) 6n(e the tu'or has 1ro/n
suffi(iently lar1e to fill the internal auditory (anal, it 'ay (ontinue 1ro/th either 2y
e0andin1 2one or 2y e0tendin1 into the (ere2elloontine an1le) Fro/th /ithin the
(ere2elloontine an1le is 1enerally sheri(al)
A(ousti( tu'ors, li?e other sa(e-o((uyin1 lesions, rodu(e sy'to's 2y any of %
re(o1niza2le 'e(hanis's: C!D (o'ression or distortion of the sinal fluid sa(es, C#D
disla(e'ent of the 2rain ste', CBD (o'ression of .essels rodu(in1 is(he'ia or
infar(tion, or C%D (o'ression and&or attenuation of ner.es)
Be(ause the (ere2elloontine an1le is relati.ely e'ty, tu'ors (an (ontinue to 1ro/
until they rea(h B-% (' in size 2efore they (onta(t i'ortant stru(tures) Fro/th is
often suffi(iently slo/ that the fa(ial ner.e (an a((o''odate to the stret(hin1
i'osed 2y tu'or 1ro/th /ithout (lini(ally aarent deterioration of fun(tion)
4u'ors that arise /ithin the internal auditory (anal 'ay rodu(e relati.ely early
sy'to's in the for' of hearin1 loss or .esti2ular distur2an(e 2y (o'ressin1 the
(o(hlear ner.e, .esti2ular ner.e, or la2yrinthine artery a1ainst the 2ony /alls of the
internal auditory (anal)
As the tu'or aroa(hes #)$ (' in dia'eter, it 2e1ins to (o'ress the lateral surfa(e
of the 2rain ste') Further 1ro/th (an o((ur only 2y (o'ressin1 or disla(in1 the
2rain ste' to/ard the (ontralateral side) 4u'ors 1reater than % (' often e0tend
suffi(iently far anteriorly to (o'ress the tri1e'inal ner.e and rodu(e fa(ial
hyesthesia) As the tu'or (ontinues to 1ro/ 2eyond % (', ro1ressi.e effa(e'ent of
the (ere2ral a8uedu(t and fourth .entri(le o((urs /ith e.entual de.elo'ent
of hydro(ehalus)
Presentation
Unilateral hearin1 loss is o.er/hel'in1ly the 'ost (o''on sy'to' resent at the
ti'e of dia1nosis and is 1enerally the sy'to' that leads to dia1nosis) Assu'e that
any unilateral sensorineural hearin1 loss is (aused 2y an a(ousti( neuro'a until
ro.en other/ise) 4he tu'or (an rodu(e hearin1 loss throu1h at least # 'e(hanis's,
dire(t inAury to the (o(hlear ner.e or interrution of (o(hlear 2lood suly)
7ro1ressi.e inAury to (o(hlear fi2ers ro2a2ly a((ounts for slo/ ro1ressi.e
neurosensory hearin1 loss o2ser.ed in a si1nifi(ant nu'2er of atients /ith a(ousti(
neuro'as) Sudden and flu(tuatin1 hearin1 losses are 'ore easily e0lained on the
2asis of disrution of (o(hlear 2lood suly)
Consistent /ith dire(t inAury to (ranial ner.e 9555, a si1nifi(ant nu'2er of indi.iduals
/ith a(ousti( neuro'a ha.e see(h dis(ri'ination s(ores redu(ed out of roortion to
the redu(tion in the ure-tone a.era1eGa feature tyi(al for retro-(o(hlear lesions)
4his (an often 2e de'onstrated throu1h audiolo1i(al testin1 2y a heno'enon (alled
Hrollo.er,H in /hi(h see(h dis(ri'ination s(ores de(rease as the .olu'e of the
see(h sti'ulus in(reases) Su(h 'ar?ed redu(tions in see(h dis(ri'ination s(ores
are not in.aria2le, ho/e.er) A nor'al see(h dis(ri'ination s(ore does not rule out
an a(ousti( tu'or) 7atients /ith a(ousti( tu'ors 'ay ha.e nor'al or near-nor'al
hearin1 and see(h dis(ri'ination s(ores)
Iearin1 loss asso(iated /ith a(ousti( neuro'a (an 2e sudden or flu(tuatin1 in ;-!;3
of atients) Su(h hearin1 loss 'ay i'ro.e sontaneously or in resonse to steroid
theray) Conse8uently, a 1adoliniu' enhan(ed M:5 should 2e ordered in anyone /ith
a sudden or flu(tuatin1 loss e.en if hearin1 returns to nor'al
@ot surrisin1ly, the dis(o.ery of a(ousti( neuro'as in ersons /ith nor'al hearin1
has 2een in(reasin1 as 1adoliniu'-enhan(ed M:5 is 2e(o'in1 'ore (o''on) 5n
addition, tu'or size oorly (orrelates /ith hearin1 status as atients /ith lar1e tu'ors
'ay ha.e nor'al hearin1 and atients /ith s'all tu'ors 'ay 2e rofoundly deaf in
the affe(ted ear) 4he resen(e of unilateral tinnitus alone is a suffi(ient reason to
e.aluate an indi.idual for a(ousti( tu'or) Althou1h tinnitus is 'ost (o''only a
'anifestation of hearin1 loss, a fe/ indi.iduals /ith a(ousti( tu'ors Caround !$3D
see? treat'ent for unilateral tinnitus /ithout asso(iated su2Ae(ti.e hearin1 loss)
9erti1o and dise8uili2riu' are un(o''on resentin1 sy'to's a'on1 atients /ith
a(ousti( tu'ors) :otational .erti1o Cthe illusion of 'o.e'ent or fallin1D is un(o''on
and is o((asionally seen in atients /ith s'all tu'ors) Dise8uili2riu' Ca sense of
unsteadiness or i'2alan(eD, on the other hand, aears to 2e 'ore (o''on in lar1er
tu'ors) 6.erall, if (arefully 8uestioned, aro0i'ately %$-;$3 of atients /ith an
a(ousti( neuro'a reort so'e 2alan(e distur2an(e) Io/e.er, 2alan(e distur2an(e is
the resentin1 sy'to' in less than !$3 of atients) 4he destru(tion of .esti2ular
fi2ers aarently is suffi(iently slo/ as to er'it (o'ensation)
Ieada(hes are resent in ;$-,$3 of atients at the ti'e of dia1nosis, 2ut fe/er than
!$3 of atients ha.e heada(he as their resentin1 sy'to') Ieada(he aears to
2e(o'e 'ore (o''on as tu'or size in(reases and is a ro'inent feature in atients
/ho de.elo o2stru(ti.e hydro(ehalus asso(iated /ith a .ery lar1e tu'or)
Fa(ial nu'2ness o((urs in a2out #;3 of atients and is 'ore (o''on at the ti'e of
resentation than fa(ial /ea?ness) 62Ae(ti.e hyoesthesia in.ol.in1 the teeth, 2u((al
'u(osa, or s?in of the fa(e is asso(iated /ith lar1er tu'ors, 2ut a su2Ae(ti.e redu(tion
in sensation that (annot 2e do(u'ented on o2Ae(ti.e e0a'ination o((urs (o''only
/ith 'ediu'-sized and s'all tu'ors) De(rease in the (orneal refle0 1enerally o((urs
earlier and 'ore (o''only than o2Ae(ti.e fa(ial hyoesthesia) Althou1h
aro0i'ately ;$-+$3 of indi.iduals /ith lar1e tu'ors ha.e o2Ae(ti.ely
de'onstra2le fa(ial hyoesthesia, they are often una/are of it, and it is un(o''only
the resentin1 sy'to')
4he 'otor fi2ers in the fa(ial ner.e (an a((o''odate .ery su2stantial stret(hin1 as
lon1 as it o((urs slo/ly and are 'u(h 'ore resistant to inAury than sensory fi2ers of
the tri1e'inal ner.e) Fa(ial /ea?ness is suffi(iently un(o''on CJ !3D that fa(ial
/ea?ness asso(iated /ith a s'all- or 'ediu'-size tu'or should raise susi(ion that it
is not an a(ousti( neuro'a) 6ther dia1nosis should 2e (onsidered in(ludin1 fa(ial
neuro'a, he'an1io'a, 'enin1io'a, 1ranulo'a,arterio.enous 'alfor'ation CA9MD,
or lio'a) <ar1e tu'ors CK% ('D (an o2stru(t the flo/ of sinal fluid throu1h the
.entri(ular syste' 2y distortin1 and o2stru(tin1 the fourth .entri(le) 5n the early
de(ades of this (entury, +;3 of atients resented /ith hydro(ehalus)
Indications
4reat'ent deends on 'ultile fa(tors in(ludin1 the a1e and 'edi(al status of the
atient, tu'or size and lo(ation, hearin1 status, and atient referen(e) 5n older
atients /ith s'all tu'ors, (areful o2ser.ation 'ay 2e ele(ted (onsistin1 of serial
M:5s) 5n older atients /ith a 1ro/in1 tu'or, radiosur1ery 'ay 2e an aroriate
otion) Loun1 atients, lar1e tu'ors C1reater than #); to B ('D and atients /ith s'all
tu'ors and inta(t hearin1 'ay (hoose sur1ery) See Sur1i(al theray)
Relevant Anatomy
4he (ere2elloontine an1le is a sa(e filled /ith sinal fluid) 5t has the 2rain ste' as
its 'edial 2oundary, the (ere2ellu' as its roof and osterior 2oundary, and the
osterior surfa(e of the te'oral 2one as its lateral 2oundary) 4he floor of the
(ere2elloontine an1le is for'ed 2y the lo/er (ranial ner.es C5M-M5D and their
surroundin1 ara(hnoid in.est'ents) 4he flo((ulus of the (ere2ellu' 'ay lie /ithin
the (ere2elloontine an1le and 'ay 2e (losely asso(iated /ith (ranial ner.es 9555 and
955 as they (ross the (ere2elloontine an1le to enter the internal auditory (anal)
4he fa(ial ner.e arises #-B '' anterior to the root entry zone of the .esti2ulo(o(hlear
ner.e) 4he fora'en of <us(h?a Cie, the oenin1 of the lateral re(ess of the fourth
.entri(leD is lo(ated Aust inferior and osterior to the root entry zones of the fa(ial and
.esti2ulo(o(hlear ner.e) A tuft of (horoid le0us (an fre8uently 2e o2ser.ed
e0trudin1 fro' it) 5nferior and a 2it anterior to the fora'en of <us(h?a is the oli.e,
and Aust osterior to the oli.e lie the rootlets of ori1in for (ranial ner.es 5M, M, and
M5) 4he hyo1lossal ner.e e0its the 2rain ste' throu1h a series of s'all rootlets
anterior to the oli.e)
Vascular structures within the cerebellopontine angle
4he 'ost i'ortant .as(ular stru(ture /ithin the (ere2elloontine an1le is the anterior
inferior (ere2ellar artery CA5CAD) 5t arises 'ost (o''only as a sin1le trun? fro' the
2asilar artery 2ut (an arise as # searate 2ran(hes) 5n rare (ases, it ori1inates as a
2ran(h of the osterior inferior (ere2ellar artery C75CAD) As the A5CA 'o.es fro'
anterior to osterior, it first follo/s the .entral surfa(e of the 2rain ste', 2ut /ithin
the (ere2elloontine an1le it ta?es a lon1 loo laterally to the orus a(ousti(us) 5n !;-
#$3 of (ases, the A5CA a(tually asses into the lu'en of the internal auditory (anal
2efore turnin1 2a(? on itself to/ard the osterior surfa(e of the 2rain ste') 4he A5CA
(an thus 2e di.ided into the re'eatal, 'eatal, and ost'eatal se1'ents)
4he 'ain 2ran(h of the A5CA asses o.er (ranial ner.es 955 and 9555 in only !$3 of
(ases) 4he re'ainder of the ti'e, it either asses 2elo/ the 955 and 9555 (ranial
ner.es or, in #;-;$3 of indi.iduals, a(tually asses 2et/een the') 4hree 2ran(hes
that re1ularly arise fro' the 'eatal se1'ent of the A5CA (an 2e identified) S'all
erforatin1 arteries suly 2lood to the 2rain ste') 4he su2ar(uate artery asses
throu1h the su2ar(uate fossa into the osterior surfa(e of the te'oral 2one, and the
third re1ular 2ran(h is the internal auditory artery Cla2yrinthine arteryD) Cranial ner.es
955 and 9555 re(ei.e their 2lood suly fro' s'all 2ran(hes of A5CA)
4/o .enous stru(tures 'ust 2e ?et in 'ind durin1 sur1i(al ro(edures in.ol.in1 the
(ere2elloontine an1le) 4he etrosal .ein Cof DandyD 2rin1s returnin1 .enous 2lood
fro' the (ere2ellu' and lateral 2rain ste' to the suerior or inferior etrosal sinus) 5t
is 1enerally en(ountered in the area of the tri1e'inal ner.e anterior to the orus
a(ousti(us) 4he etrosal .ein often (arries enou1h .enous 2lood that its o2stru(tion
(an lead to .enous infar(tion and (ere2ellar ede'a, and it should 2e reser.ed if at all
ossi2le) Additional .enous 2lood rea(hes the suerior etrosal sinus throu1h a series
of 2rid1in1 .eins that (ross the (ere2elloontine an1le) Althou1h e.ery atte't should
2e 'ade to reser.e these .eins, their sa(rifi(e is 1enerally in(onse8uential)
4he .ein of <a22N (arries returnin1 .enous 2lood fro' the inferior and lateral surfa(e
of the te'oral lo2e to the suerior etrosal sinus, tentorial .enous la?es, or the
trans.erse sinus) 5ts (onfi1uration and anato'y is 8uite .aria2le) Io/e.er,
o2stru(tion, o2literation, or o((lusion of the suerior etrosal sinus 'ay, in so'e
(ases, result in o((lusion of the .ein of <a22N) Sudden o((lusion of the .ein of <a22N
(arries /ith it hi1h ris? of .enous infar(tion of the te'oral lo2e and raid life-
threatenin1 (ere2ral ede'a)
Nerves
4he fa(ial ner.e lea.es the 2rain ste' anterior to the fora'en of <us(h?a) As it lea.es
the 2rain ste', the fi2ers are sheathed in oli1odendro1lia deri.ed fro' the (entral
ner.ous syste') Within a fe/ 'illi'eters of lea.in1 the 2rain ste', ho/e.er, the
ner.e loses its oli1odendro1lial ensheath'ent and 2e(o'es ensheathed instead 2y
S(h/ann (ells) 4hrou1hout the re'ainder of its eriheral (ourse, it re'ains /ithin
its S(h/ann (ell in.est'ent) 5t asses dire(tly a(ross the (ere2elloontine an1le for
a2out !; '', a((o'anied 2y the .esti2ulo(o(hlear ner.e) 5t (onsistently enters the
internal auditory (anal 2y (rossin1 the anterior suerior 'ar1in of the orus a(usti(us)
4he .esti2ulo(o(hlear ner.e arises fro' the 2rainste' sli1htly osterior to the fa(ial
ner.e) 5t re'ains sheathed in oli1odendro1lia for aro0i'ately !; '' Cal'ost to the
oint at /hi(h it asses into the internal auditory (analD) 5t has the lon1est
oli1odendro1lial in.est'ent of any eriheral ner.e) 4he Aun(tion 2et/een
oli1odendro1lia and S(h/ann (ells Cie, the 62ersteiner-:edli(h zoneD o((urs Aust
'edial to the orus a(ousti(us) Be(ause a(ousti( neuro'as arise fro' S(h/ann (ells,
they arise 'ost (o''only /ithin the 'ost lateral ortions of the (ere2elloontine
an1le or the internal auditory (anal)
4he ner.us inter'edius Cner.e of Wris2er1D lea.es the 2rain ste' to1ether /ith the
.esti2ulo(o(hlear ner.e) At so'e oint /ithin the (ere2elloontine an1le, the ner.us
inter'edius (rosses o.er to 2e(o'e asso(iated /ith the fa(ial ner.e) 5t 'ay do so as
se.eral searate rootlets) 4he oint /here the ner.us inter'edius (rosses to 2e(o'e
asso(iated /ith the fa(ial ner.e sho/s (onsidera2ly .ariation, 2ut in ##3 of
indi.iduals, it is adherent to the .esti2ulo(o(hlear ner.e for !% '' or 'ore) As the
.esti2ulo(o(hlear and fa(ial ner.e rea(h the orus a(ousti(us C'edial oenin1 of the
internal auditory (analD they ass to1ether /ith the ner.us inter'edius and so'eti'es
a loo of A5CA)
Internal auditory canal
4he internal auditory (anal is aro0i'ately *); '' in len1th Cran1e ;);-!$); ''D,
lined /ith dura, and filled /ith sinal fluid) 5ts 'edial end is o.al in shae and is
referred to as the orus a(ousti(us) 5ts lateral end is a (o'li(ated stru(ture referred to
as the fundus or la'ina (ri2rosa) 4he fundus is di.ided into a suerior and inferior
half 2y the trans.erse (rest) 4he uer half is further su2di.ided into an anterior and
osterior se1'ent 2y a .erti(al (rest, often referred to as BillOs Bar, na'ed after
Willia' Iouse, /ho oularized its i'ortan(e as a sur1i(al land'ar?) 4he .erti(al
(rest searates the 'a(ula (ri2rosa suerior, a series of .ery s'all oenin1s throu1h
/hi(h the ter'inal fi2ers of the .esti2ular ner.e ass in order to rea(h the (uula of
the suerior se'i(ir(ular (anal, fro' the 'eatal fora'en, /hi(h 'ar?s the oint at
/hi(h the fa(ial ner.e lea.es the internal auditory (anal and enters the falloian (anal
as the la2yrinthine se1'ent)
Be(ause the 'ost lateral ortion of the internal auditory (anal is %-; '' inferior to
the le.el of the 1eni(ulate 1an1lion, the la2yrinthine se1'ent of the fa(ial ner.e 'ust
ta?e a .erti(ally oriented (ourse u/ard to rea(h it) 4he la2yrinthine se1'ent 'ay 2e
less than a 'illi'eter /ide as it asses 2et/een the (o(hlea and the anterior end of the
suerior se'i(ir(ular (anal) 4he inferior ortion of the fundus is a sin1le o.al-shaed
sa(e, the anterior ortion of /hi(h is o((uied 2y a rounded deression Ctra(tus
siralis fora'inosusD filled /ith s'all oenin1s to a((o''odate the ter'inal
2ran(hes of the (o(hlear ner.e) 4he osterior ortion is filled /ith a 'a(ula (rista
inferior throu1h /hi(h ass the ter'inal ends of the inferior .esti2ular ner.e)
Temporal bone
4he anato'y of the suerior surfa(e of the te'oral 2one 'ust 2e 'astered if 'iddle
fossa aroa(hes are to 2e underta?en su((essfully) <aterally, the irre1ular suerior
surfa(e of the te'oral 2one transitions relati.ely s'oothly to the te'oral s8ua'osa)
4he free ed1e of the tentoriu' and the suerior etrosal sinus atta(h to the 'edial
ed1e of the suerior surfa(e of the te'oral 2one) 4he ar(uate e'inen(e, a 2ony
ro'inen(e that is erendi(ular to the etrous rid1e and lies t/o (enti'eters 'edial
to the s8ua'ous te'oral 2one, often o.erlies the suerior se'i(ir(ular (anal) 4he
ar(uate e'inen(e is often diffi(ult to identify, ese(ially in /ell-neu'atized
te'oral 2ones)
4he 1eni(ulate 1an1lion 1enerally lies /ithin the su2stan(e of the te'oral 2one Aust
'edial to and a fe/ 'illi'eters anterior to the head of the 'alleus) 4he 1eni(ulate
1an1lion 'ay 2e dehis(ent, or alternati.ely, it 'ay lie se.eral 'illi'eters 2eneath the
suerior surfa(e of the 2one) 4he head of the 'alleus is 1enerally easy to identify if
the thin 2one of the te1'en ty'ani is re'o.ed so as to enter into the 'iddle ear
sa(e) 5n diffi(ult sur1i(al situations, the head of the 'alleus (an 2e used to identify
the 1eni(ulate 1an1lion) 4he 1reater suerfi(ial etrosal ner.e ori1inates fro' the
1eni(ulate 1an1lion and (ourses antero'edially, assin1 o.er the suerior surfa(e of
the te'oral 2one at the fa(ial hiatus) 4he fa(ial hiatus is 1enerally %-* '' anterior to
the 1eni(ulate 1an1lion) 4he 1reater suerfi(ial etrosal ner.e (an 1enerally 2e
identified in this area) 5t (an then 2e follo/ed retro1rade to the 1eni(ulate 1an1lion)
4he 'iddle 'enin1eal artery and asso(iated .eins tra.erse the fora'en sinosu',
/hi(h is lo(ated aro0i'ately ! (' anterolaterally to the 1reater suerfi(ial etrosal
ner.e) 4he 'andi2ular di.ision of the tri1e'inal ner.e tra.erses the fora'en o.ale,
/hi(h lies a fe/ 'illi'eters anterior and 'edial to the fora'en sinosu') 4he
horizontal ortion of the (arotid (anal (ourses throu1h the anterior te'oral 2one
'edial to the fora'en sinosu' and fora'en o.ale) 4he (o(hlea (annot 2e identified
fro' the surfa(e aearan(e of the suerior te'oral 2one) 5t lies Aust anterior and
inferior to the la2yrinthine se1'ent of the fa(ial ner.e 2ut is dee to the 1eni(ulate
1an1lion)
Contraindications
Fe/ a2solute (ontraindi(ations to the sur1i(al re'o.al of an a(ousti( tu'or e0ist)
Serious 'edi(al illness 'ay 'a?e sur1i(al re'o.al in so'e atients too ris?y)
Sur1ery 'ust often 2e erfor'ed for lar1e tu'ors /ith 2rainste' shift and o2stru(ti.e
hydro(ehalus, e.en in the resen(e of si1nifi(ant 'edi(al illness) 4he
transla2yrinthine aroa(h is (ontraindi(ated in a atient /ith (hroni( otitis 'edia)
4he de(ision to oerate should 2e (arefully (onsidered /hen the tu'or is /ithin the
internal auditory (anal of a atientPs only hearin1 ear) 5n so'e (ases, o2ser.in1 the
tu'or until hearin1 has 2een lost is 2est, /hile in other (ases, atte'tin1 sur1i(al
re'o.al /ith hearin1 (onser.ation is 'ore rudent)
Laboratory tudies
:outine la2 studies are 1enerally not re8uired)
Imaging tudies
4he definiti.e dia1nosti( test for atients /ith a(ousti( tu'ors is 1adoliniu'-
enhan(ed M:5)
Well-erfor'ed s(annin1 (an de'onstrate tu'ors as s'all as !-# ''
in dia'eter) 6n the other hand, thin-(ut C4 s(annin1 (an 'iss tu'ors as lar1e as !);
(' e.en /hen intra.enous (ontrast enhan(e'ent is used)
Fadoliniu' (ontrast is (riti(al 2e(ause nonenhan(ed M:5 (an 'iss
s'all tu'ors)
Fast-sin e(ho te(hni8ues do not re8uire 1adoliniu' enhan(e'ent and
(an 2e erfor'ed .ery raidly and relati.ely ine0ensi.ely) Io/e.er, su(h hi1hly
tar1eted te(hni8ues ris? 'issin1 other i'ortant (auses of unilateral sensory hearin1
loss, in(ludin1 intra-a0ial tu'ors, de'yelinatin1 disease, and infar(ts)
M:5 is (ontraindi(ated in indi.iduals /ith ferro'a1neti( i'lants)
Fine-(ut C4 s(annin1 of the internal auditory (anal /ith (ontrast (an rule out a
'ediu'-size or lar1e tu'or 2ut (annot 2e relied uon to dete(t a tu'or s'aller than
!-!); (')
5f susi(ion is hi1h and M:5 is (ontraindi(ated, air-(ontrast (isterno1rahy
has hi1h sensiti.ity and (an dete(t relati.ely s'all intra(anali(ular tu'ors)
!iagnostic Procedures
A .ariety of audio'etri( tests /ere de.eloed in the 'iddle of the (entury in
an atte't to identify atients /ith in(reased li?elihood of ha.in1 an a(ousti(
neuro'a) 4hat /as a /orth/hile underta?in1 /hen definiti.e radio1rahi( i'a1in1
(onsisted of so'e for' of either neu'oen(ehalo1rahy or for'al arterio1rahy)
Su(h testin1 is no lon1er used) E.en the auditory 2rainste' e.o?ed resonse CAB:D is
no/ infre8uently used as a s(reenin1 test for a(ousti( neuro'a) AB: s(reenin1
te(hni8ues 'iss #$-B;3 of a(ousti( tu'ors s'aller than ! (') Moreo.er, AB: is
li?ely to 'iss those tu'ors in atients /ith e0(ellent hearin1, /hi(h are the (ases
'ost fa.ora2le for hearin1 (onser.ation ro(edures)
Histologic "indings
4/o histolo1i( tyes of tissue ha.e 2een identified in a(ousti( tu'ors) Antoni A
tissue (onsists of elon1ated sindle (ells /ith a alisadin1 attern) Antoni B tissue, on
the other hand, has a loose son1y te0ture and 'ar?edly redu(ed (ellularity) A 1i.en
a(ousti( neuro'a 'ay (ontain areas /ith 2oth Antoni A and Antoni B tissue) Another
histolo1i( feature (hara(teristi( of s(h/anno'as are ro/s of alisadin1 nu(lei (alled
9ero(ay 2odies) Althou1h the histolo1i( aearan(e of a(ousti( tu'ors is fairly
strai1htfor/ard, they (an o((asionally 2e diffi(ult to distin1uish fro' 'enin1io'as)
5''unohisto(he'i(al stainin1 (an distin1uish s(h/anno'as fro' 'enin1io'as in
diffi(ult (ases) S(h/anno'as are i''unorea(ti.e to S-!$$ anti2ody /hile
'enin1io'as are i''unorea(ti.e to eithelial 'e'2rane anti2ody CEMAD)
taging
As a rule, a(ousti( neuro'as are 2eni1n tu'ors, althou1h rare (ases of 'ali1nant
a(ousti( neuro'as ha.e 2een reorted in the literature) @o /idely a((eted sta1in1
syste' e0ists for a(ousti( neuro'as)
#edical $herapy
A(ousti( neuro'as are 'ana1ed in one of the follo/in1 B /ays: C!D sur1i(al e0(ision
of the tu'or, C#D arrestin1 tu'or 1ro/th usin1 stereota(ti( radiation theray, or CBD
(areful serial o2ser.ation)
Observation
Si'le o2ser.ation /ithout any theraeuti( inter.ention has 2een used in the
follo/in1 1rous of atients:
Elderly atients
7atients /ith s'all tu'ors, ese(ially if their hearin1 is 1ood
7atients /ith 'edi(al (onditions that si1nifi(antly in(rease the ris? of
oeration
7atients /ho refuse treat'ent
7atients /ith a tu'or on the side of an only hearin1 ear or only seein1 eye
o 5n a nu'2er of series reorted to date, the indi.iduals /ho are 2ein1 o2ser.ed
ulti'ately re8uire theraeuti( inter.ention in 2et/een !;-%$3)
o Durin1 an o2ser.ation eriod, 'ost C+$3 or 'oreD atients /ho are eli1i2le
for hearin1 (onser.ation sur1ery initially lost their eli1i2ility)
o 4elian has analyzed the i'ortant .aria2les that should 2e e.aluated /hen
o2ser.ation is (onsidered, and these in(lude the follo/in1:
=#>
!D reoerati.e hearin1
in 2oth ears, #D the ris? of i''ediate hearin1 loss as a (onse8uen(e of sur1ery, BD the
ris? of fa(ial ner.e aralysis, %D the ris? of other sur1i(al (o'li(ations and their
seriousness, ;D the atientPs life e0e(tan(y, ,D the size of the tu'or, +D tu'or 1ro/th
rate, and *D atients /ith neurofi2ro'atosis tye # C@F#D or 2ilateral tu'ors
Stereotactic radiotherapy
Stereota(ti( radiotheray has e'er1ed /ithin the last #$ years as an alternati.e to
'i(rosur1ery for sele(ted atients /ith a(ousti( neuro'a)
Stereota(ti( radiation theray 'a?es use of one of se.eral radiation sour(es
and is ad'inistered usin1 a .ariety of different 'a(hines /ith rorietary na'es Ce1,
Fa''a Knife, Cy2erKnife, Brain<ABD)
Stereota(ti( theray uses radiation deli.ered to a re(ise oint or series of
oints to 'a0i'ize the a'ount of radiation deli.ered to tar1et tissues /hile
'ini'izin1 the e0osure of adAa(ent nor'al tissues) 5t (an 2e deli.ered as a sin1le
dose or as 'ultile fra(tionated doses)
4he effe(ts of radiation deli.ered at the (urrent lo/ dose li?ely re.ents
further tu'or 1ro/th 2y (ausin1 o2literati.e endarteritis of the .essels sulyin1 the
tu'or) :adiosur1ery 'ay affe(t tu'or (ells under1oin1 'itosis 2y (ausin1 dou2le
strand D@A 2rea?s) Iansen et al de'onstrated a(ousti( neuro'a (ells are
radioresistant at the (urrent lo/-dose radiation used /ith radiosur1ery)
=B>
Co'arison of 'i(rosur1ery and stereota(ti( radiation is diffi(ult for the
follo/in1 reasons:
4u'or size is in(onsistently reorted in the literature)
Data usin1 the lo/er radiation dosa1es are a.aila2le for only the ast
!$ years)
Be(ause the 1oal of radiotheray is (ontrol of tu'or 1ro/th,
understandin1 /hether osttreat'ent neuroi'a1in1 refle(ts ade8uate
treat'ent or 'erely the natural history of .esti2ular s(h/anno'as is
diffi(ult)
@o data (on(ernin1 the ris? for se(ondary tu'or indu(tion 2y
radiotheray are a.aila2le)
Ad.anta1es of radiation theray in(lude the follo/in1:
De(reased len1th of stay
De(reased (ost
:aid return to full e'loy'ent
<o/er i''ediate osttreat'ent 'or2idity and 'ortality
Disad.anta1es of stereota(ti( radiation in(lude the follo/in1:
o @e(essity for re1ular 'onitorin1 and fre8uent res(annin1 C5n the end, (osts
asso(iated /ith lon1-ter' 'onitorin1 (ould e0(eed those of sur1ery)D
o Does not eli'inate the tu'or and 'ay fail to (ontrol tu'or 1ro/th, so'eti'es
re8uirin1 sal.a1e sur1ery)
o Ii1her in(iden(e of tri1e'inal ner.e inAury)
o Un?no/n lon1-ter' in(iden(e of se(ondary 'ali1nan(ies) 4he 2est (urrent
esti'ates of de.eloin1 a se(ondary 'ali1nan(y fro' the radiosur1ery are ! in a
!$$$ atients o.er B$ years)
o Does not address dise8uili2riu' and 'ay lead to lon1-ter' 2alan(e
dysfun(tion)
Fra(tionated stereota(ti( radiotheray ro.ides .ery 1ood tu'or (ontrol of a(ousti(
neuro'a, 2ut it also (arries a ris? of the atient de.eloin1 hydro(ehalus) 5t is
ne(essary 2efore treat'ent to (losely 'onitor atients at hi1h ris? Cie, those /ith
lar1er tu'ors /ith artial effa(e'ent of the fourth .entri(leD and to 'onitor the'
'ore (losely durin1 follo/-u) Before tu'or dia'eter 1ro/s to lar1er than # (', it
/ould 2e 2enefi(ial to offer treat'ent to atients /ith ro1ressi.e a(ousti( neuro'a
/hile the ris? of hydro(ehalus is lo/)
=%>
Stereota(ti( radiosur1ery and fra(tionated stereota(ti( radiotheray ha.e the otential
for hearin1 reser.ation, at least in the short-ter') Iearin1 reser.ation is deendent
on 'ultile fa(tors in(ludin1 tu'or size, tu'or lo(ation, and radiation dose) Most
(enters use a dose of !#-!B Fy at the ;$3 isodose line /hen (onsiderin1 hearin1
reser.ation) Iearin1 reser.ation is also deendent on the radiation dose to the
(o(hlea, (o(hlear ner.e, and (o(hlear nu(leus) Ki' et al re(ently noted transient
.olu'e e0ansion that is (o''only seen after radiosur1ery ortends the /orse
ro1nosis for hearin1 reser.ation)
=;>
urgical $herapy
Sur1i(al re'o.al re'ains the treat'ent of (hoi(e for tu'or eradi(ation) 9arious
sur1i(al aroa(hes (an 2e used to re'o.e a(ousti( tu'ors) Ea(h aroa(h is
dis(ussed in detail in the follo/in1 se(tions)
Preoperative !etails
4hree different aroa(hes are used in the 'ana1e'ent of a(ousti( neuro'as, the
retrosi1'oid, transla2yrinthine, and 'iddle fossa aroa(hes) All ha.e ad.anta1es
and disad.anta1es as indi(ated 2elo/)
Advantages of the retrosigmoid approach
4he retrosi1'oid aroa(h (an 2e alied to all a(ousti( tu'ors and to 'any
other histolo1i( tu'or tyes) 5t (an 2e used for oerations that sa(rifi(e hearin1 and
oerations that atte't to (onser.e hearin1) 5ts only li'itation in this rese(t is its
inali(a2ility for s'all tu'ors that o((uy the far-lateral ortions of the internal
auditory (anal)
4he retrosi1'oid aroa(h ro.ides the 2est /ide-field .isualization of the
osterior fossa) 4he inferior ortions of the (ere2elloontine an1le and the osterior
surfa(e of the te'oral 2one anterior to the orus a(usti(us are 'u(h 'ore (learly
o2ser.ed than .ia the transla2yrinthine aroa(h) 7anora'i( .isualization is
ese(ially helful /hen disla(e'ent of ner.es is not redi(ta2le, /hi(h o((urs
(o''only /ith 'enin1io'as)
Iearin1 (onser.ation sur1ery (an 2e atte'ted e.en for relati.ely lar1e
tu'ors .ia the retrosi1'oid aroa(h) Destru(tion of the la2yrinth is not re8uired as
art of the retrosi1'oid aroa(h)
isadvantages of the retrosigmoid approach
4he retrosi1'oid aroa(h 'ay re8uire (ere2ellar retra(tion or rese(tion)
Maniulation of the (ere2ellu' ro.ides oortunities for ostoerati.e ede'a,
he'ato'a, infar(tion, and 2leedin1)
5n(reased in(iden(e of (ere2rosinal fluid lea? o((urred in so'e series)
4he retrosi1'oid aroa(h is asso(iated /ith 1reater li?elihood of se.ere
rotra(ted ostoerati.e heada(he)
4he hi1hest in(iden(e of tu'or re(urren(e or ersisten(e o((urs /ith
retrosi1'oid aroa(hes)
Advantages of the translabyrinthine approach
4he transla2yrinthine aroa(h ro.ides the 2est .ie/ of the lateral 2rain ste'
fa(in1 the a(ousti( tu'or)
:etra(tion of the (ere2ellu' is al'ost ne.er ne(essary)
4he fundus and lateral end of the internal auditory (anal are (o'letely
e0osed; the fa(ial ner.e (an 2e identified at a lo(ation /here it is undistorted 2y
tu'or 1ro/th and (o'ressed into the la2yrinthine se1'ent, de(reasin1 the ris? of
delayed ostoerati.e fa(ial ner.e alsy)
5n(iden(e of (ere2rosinal fluid lea? is de(reased in so'e series)
5f the fa(ial ner.e has 2een di.ided or sa(rifi(ed, the transla2yrinthine
aroa(h 'ay allo/ restoration of the fa(ial ner.e (ontinuity 2y reroutin1 the fa(ial
ner.e and erfor'in1 a ri'ary anasto'osis) Conse8uently, interosition 1raft (an
so'eti'es 2e a.oided)
Fa(ial fun(tion is 'ore fre8uently reser.ed in so'e series)
isadvantages of the translabyrinthine approach
Iearin1 sa(rifi(e is (o'lete and una.oida2le)
4he inferior ortions of the (ere2elloontine an1le and (ranial ner.es are not
as /ell .isualized as they are in the retrosi1'oid aroa(h) 4he te'oral 2one
anterior to the orus a(usti(us is also less /ell .isualized)
A fat 1raft is re8uired) :e'o.al of fat fro' the a2do'en (reates oortunities
for donor site (o'li(ations, in(ludin1 he'ato'a, 2leedin1, and infe(tion)
4he si1'oid sinus is 'ore .ulnera2le to inAury) Bleedin1 fro' the si1'oid
sinus (an 2e diffi(ult to (ontrol and (an si1nifi(antly in(rease oerati.e 2lood loss) 5f
a do'inant si1'oid sinus is o((luded durin1 the oeration, ostoerati.e intra(ranial
ressure ele.ation or .enous infar(t (an o((ur)
A hi1h Au1ular 2ul2 or anteriorly la(ed si1'oid sinus (an su2stantially
(o'ro'ise the sa(e a.aila2le for tu'or re'o.al) 6((asionally, the sa(e is so
(ontra(ted that another aroa(h has to 2e sele(ted)
Advantages of the middle cranial fossa approach
5t is the only ro(edure that fully e0oses the lateral third of the internal
auditory (anal /ithout sa(rifi(in1 hearin1)
5t is e0tradural)
isadvantages of the middle cranial fossa approach
4he fa(ial ner.e 1enerally (ourses a(ross the anterior suerior ortion of the
tu'or) Conse8uently, it is in the /ay durin1 tu'or re'o.al and is 'ore .ulnera2le to
inAury) Althou1h lon1-ter' fa(ial ner.e out(o'es are as 1ood /ith the 'iddle (ranial
fossa aroa(h as /ith other aroa(hes, te'orary ostoerati.e aresis is 'ore
(o''on)
4he ris? of dural la(eration and a.ulsion 2e(o'es in(reasin1ly 'ore li?ely as
atients 2e(o'e older) 4he dura 'ater in elderly atients is 'ore fria2le) 4his
2e(o'es ese(ially noti(ea2le durin1 the si0th and se.enth de(ades of life)
4he aroa(h ro.ides only .ery li'ited e0osure of the osterior fossa)
4he oeration is te(hni(ally diffi(ult and de'andin1)
So'e atients in(ur ostoerati.e tris'us related to 'aniulation and&or
inAury to the te'oralis 'us(le)
4he te'oral lo2e 'ust 2e retra(ted, resentin1 the oortunity for te'oral
lo2e inAury, usually in the for' of a he'ato'a that is asy'to'ati( and,
therefore, ro2a2ly o((urs 'ore fre8uently than is realized) S(attered reorts
e0ist of seizure disorder follo/in1 'iddle (ranial fossa sur1ery, resu'a2ly
due to te'oral lo2e inAury)
Approach Selection
A .ariety of different (onsiderations 1o into de(idin1 /hi(h aroa(h should 2e used
for any indi.idual atient) 4hese .aria2les are detailed 2elo/)
!reoperative hearing level
5f the atient has no useful hearin1, either the transla2yrinthine or the retrosi1'oid
aroa(h is sele(ted, deendin1 uon the e0erien(e and trainin1 of the sur1eon) 5n
'ost (enters erfor'in1 lar1e nu'2ers of sur1eries for a(ousti( tu'ors, the
transla2yrinthine aroa(h is referred) 6inions .ary (onsidera2ly a2out /hat
(onstitutes useful hearin1) 4he ;$&;$ rule is fre8uently 8uoted) 4he rule su11ests that
indi.iduals /ith a ure-tone a.era1e 1reater than ;$ dB and see(h dis(ri'ination
less than ;$3 do not ha.e useful or sal.a1ea2le hearin1) 6ther sur1eons ha.e stri(ter
(riteria and (onsider only indi.iduals /ith 2etter than a B$-dB ure-tone a.era1e and
'ore than +$3 dis(ri'ination for hearin1 (onser.ation oerations)
Auditory brainstem response
@or'al reoerati.e AB: findin1s fa.or hearin1 (onser.ation) Mar?ed a2nor'alities
of AB: /a.e 'orholo1y or in(reased /a.e 5-555 and 5-9 laten(ies 'a?e hearin1
(onser.ation less feasi2le)
"lectronystagmography
An a2nor'al (alori( test on ele(tronysta1'o1rahy CE@FD in(reases the li?elihood of
su((essful hearin1 (onser.ation sur1ery) 4he E@F tests the horizontal se'i(ir(ular
(anal, /hi(h is inner.ated 2y the suerior .esti2ular ner.e) A nor'al E@F findin1
ar1ua2ly de'onstrates that the suerior .esti2ular ner.e is nor'al) Conse8uently, the
a(ousti( tu'or 'ust ha.e ori1inated fro' the inferior .esti2ular ner.e, /hi(h is
dire(tly adAa(ent to the (o(hlear ner.e) Sur1i(al re'o.al, then, is 'ore li?ely to
dire(tly inAure the (o(hlear ner.e or interfere /ith (o(hlear 2lood suly) 9esti2ular
e.o?ed 'yo1eni( otential C9EM7D testin1 is a2nor'al /hen the inferior .esti2ular
ner.e is affe(ted) As a result, an a2nor'al 9EM7 /ith nor'al (alori( testin1 on E@F
stron1ly su11ests an inferior .esti2ular ner.e tu'or /ith oorer hearin1 reser.ation)
Tumor si#e
6ortunities for hearin1 (onser.ation de(rease as tu'ors 2e(o'e lar1er) Iearin1 is
'u(h 'ore diffi(ult to (onser.e /hen tu'ors are !);-#)$ (' in dia'eter than if they
are s'all intra(anali(ular tu'ors) Conse8uently, so'e sur1eons li'it hearin1
(onser.ation sur1ery to s'aller tu'ors, referrin1 to use a transla2yrinthine aroa(h
to 'a0i'ize the (han(e of fa(ial ner.e (onser.ation for lar1er tu'ors)
Tumor position
5f hearin1 (onser.ation is to 2e atte'ted and the tu'or lies /ithin the lateral ortions
of the internal auditory (anal, 'any sur1eons refer a 'iddle fossa aroa(h) 4he
'iddle fossa aroa(h er'its dire(t e0osure of the lateral end of the internal
auditory (anal /ithout sa(rifi(in1 hearin1) 4he aroa(h is fre8uently used for any
tu'or lyin1 (o'letely /ithin the internal auditory (anal, althou1h tu'ors li'ited to
the 'edial ortions of the internal auditory (anal (an 2e 'ana1ed usin1 a
retrosi1'oid aroa(h) So'e sur1eons e0tend the use of the 'iddle fossa te(hni8ue
to in(lude tu'ors that e0tend as 'u(h as $);-!)$ (' into the (ere2elloontine an1le)
Di.ision of the suerior etrosal sinus 'ay 2e re8uired to 1ain suffi(ient a((ess to the
osterior fossa /ith lar1er tu'ors)
Fenerally, ho/e.er, tu'ors that ha.e si1nifi(ant .olu'e 'edial to the lane of the
orus a(ousti(us are e0tirated usin1 a retrosi1'oid aroa(h if hearin1 is to 2e
(onser.ed) 5f hearin1 (onser.ation is not an issue, the retrosi1'oid aroa(h is
so'eti'es referred for tu'ors /ith si1nifi(ant inferior e0tension sin(e the lo/er
(ranial ner.es are 2etter .isualized /ith a retrosi1'oid aroa(h) 6((asionally, the
retrosi1'oid aroa(h is (o'2ined /ith a transla2yrinthine aroa(h for su(h lar1e
a(ousti( neuro'as
$elevant anatomy
4he follo/in1 anato'i( .ariations (an 'a?e the transla2yrinthine aroa(h 'u(h
'ore diffi(ult and at ti'es i'ossi2le)
Ii1h-ridin1 Au1ular 2ul2: 5n so'e indi.iduals, the Au1ular 2ul2 'ay a(tually
ride u a2o.e the le.el of the inferior internal auditory (anal)
Anteriorly la(ed si1'oid sinus: 5n su(h (ir(u'stan(es, the distan(e 2et/een
the si1'oid sinus and the e0ternal auditory (anal 'ay 2e a fe/ 'illi'eters or less)
Su(h a dra'ati( li'itation of the sa(e /ithin /hi(h the sur1eon has to oerate not
only 'a?es a su((essful tu'or e0tiration 'u(h 'ore diffi(ult 2ut uts the fa(ial
ner.e and the disla(ed sinus itself at si1nifi(antly in(reased ris? of inAury)
Contra(ted s(leroti( 'astoid: Su(h 'astoid (a.ities ro.ide little roo' for
tu'or re'o.al) Moreo.er, they are often asso(iated /ith suurati.e otitis 'edia, in
itself a (ontraindi(ation to the transla2yrinthine aroa(h)
:edu(ed or a2sent flo/ in the (ontralateral sinus: 7re.ious oeration, trau'a,
(on1enital ano'alus de.elo'ent, and re.ious or (on(urrent disease (an all result
in 'ar?edly redu(ed or a2sent .enous outflo/ throu1h the (ontralateral sinus) 5n
su(h (ases, (onsideration 'ay 2e 1i.en to a retrosi1'oid aroa(h 'erely 2e(ause it
redu(es the ris? of inAury to the re'ainin1 sinus, o((lusion of /hi(h /ould result in
(atastrohi( .enous infar(tion)
Surgeon preference
So'e sur1eons ha.e 'ore e0erien(e and are 'u(h 'ore (o'forta2le /ith one
aroa(h relati.e to another) Fenerally, su(h referen(es should 2e follo/ed)
Io/e.er, if hearin1 (onser.ation is a realisti( otion usin1 an aroa(h unfa'iliar to
the ri'ary sur1eon, (onsideration should 2e 1i.en to referrin1 the atient to so'eone
/ho is fa'iliar /ith the aroriate aroa(h)
!atient preference
7atient referen(es should 2e (arefully (onsidered e.en /hen they do not (onfor' to
the sur1eonPs Aud1'ent) So'e atients are ada'ant a2out 1oin1 to any len1ths for
hearin1 (onser.ation e.en /hen the treatin1 hysi(ian is 8uite (on.in(ed that the
atientPs hearin1 is so oor as to 2e of little or no ra(ti(al utility) So'e atients
/illin1ly sa(rifi(e e.en 1ood hearin1 if doin1 so e.en sli1htly enhan(es the ossi2ility
of su((essful fa(ial ner.e reser.ation) So'e atients ha.e .ery (lear-(ut oinions
a2out one tye of in(ision .ersus another Cso'eti'es 2ased on (os'eti(
(onsiderationD)
Intraoperative !etails
Translabyrinthine approach
4he transla2yrinthine aroa(h is the 'ost .ersatile of the B (o''on aroa(hes to
the (ere2elloontine an1le) 4he 'ain disad.anta1e is rofound deafness in the
oerated ear due to .iolation of the 'e'2ranous la2yrinth) 5n 1eneral, e.en the lar1est
a(ousti( neuro'as (an 2e re'o.ed throu1h a transla2yrinthine (ranioto'y) 5n
addition, the fa(ial ner.e is found at the fundus of the internal auditory (anal /here
the .erti(al (rest CBillOs 2arD ro.ides a natural lane for fa(ial ner.e disse(tion fro'
the suerior .esti2ular ner.e) At the authorOs institution, the transla2yrinthine
aroa(h is referred /ith any a(ousti( neuro'a o.er # (' or in an ear /ith oor
hearin1)
4he atient is laid suine and a Mayfield head fra'e 'ay 2e used) An in(ision is then
'ade t/o fin1er-2readths fro' the ostauri(ular sul(us) 4he te'oralis 'us(le and
'astoid eriosteu' are identified) 4he s?in fla is then ele.ated anteriorly, lea.in1 as
'u(h eriosteu' do/n as ossi2le) 4he eriosteu' is then in(ised alon1 the linea
te'oralis and then to/ards the 'astoid ti in a 4-shaed fashion) 4his /ill allo/ a
/ater-ti1ht se(ond layer for (losure to re.ent ostoerati.e (ere2rosinal fluid
lea?a1e) 4he 'astoid eriosteu' is then ele.ated fro' the underlyin1 'astoid 2one)
6ften, the e'issary .ein is en(ountered and this (an 2e (ontrolled /ith 2iolar
(oa1ulation and&or 2one /a0)
A /ide (orti(al 'astoide(to'y is erfor'ed) 4he 'iddle and osterior fossa dura are
identified as /ell as the si1'oid sinus) 4he 2one is re'o.ed fro' these stru(tures to
allo/ retra(tion of the te'oral lo2e dura and si1'oid sinus) @e0t, the antru', lateral
se'i(ir(ular (anal, and .erti(al fa(ial ner.e are identified)
4he in(us is re'o.ed and a fa(ial re(ess is erfor'ed) 4he in tensor ty'ani tendon
is se(tioned and the eusta(hian tu2e is a(?ed /ith o0idized (ellulose a(?in1) 4he
'iddle ear sa(e is then a(?ed /ith te'oralis 'us(le)
A la2yrinthe(to'y is erfor'ed and the Au1ular 2ul2 is identified) 4he internal
auditory (anal is su2se8uently identified and trou1hs are de.eloed 2oth sueriorly
and inferiorly around the internal auditory (anal until aro0i'ately #+$Q of internal
auditory (anal is e0osed) 4he re'ainin1 2one is then re'o.ed fro' the internal
auditory (anal and the fa(ial ner.e is found as it turns into the la2yrinthine se1'ent)
4he suerior .esti2ular ner.e is then follo/ed out to the a'ullated end of the
suerior se'i(ir(ular (anal)
At this oint, the trans.erse (rest and .erti(al (rest CBillOs 2arD are identified) 4he
suerior .esti2ular ner.e is then refle(ted inferiorly fro' the a'ullated end of the
suerior se'i(ir(ular (anal) 4he fa(ial ner.e (an often 2e found suerior 'edial to
this and is (onfir'ed usin1 a fa(ial ner.e sti'ulator) At this oint, the tu'or is
1enerally de2ul?ed and the fa(ial ner.e is lo(ated at the ori1in fro' the 2rain ste')
6n(e the tu'or is ade8uately de2ul?ed, the a(ousti( neuro'a is then disse(ted fro'
the fa(ial ner.e) 6ften, the fa(ial ner.e is .ery adherent to the a(ousti( neuro'a
around the orus of the internal auditory (anal)
6n(e the tu'or has 2een re'o.ed, the osterior fossa dura is then re-aro0i'ated)
Fat is har.ested fro' the a2do'en and a(?ed into the sur1i(al defe(t) 4he eriosteal
and s?in layers are (losed in a /ater-ti1ht fashion) 4he atient /ears a ressure
dressin1 for B days)
$etrosigmoid approach
4he atient 'ay 2e la(ed in the suine osition on the oeratin1 ta2le and /ith the
head to/ard the (ontralateral shoulder) 4he true lateral or ar?-2en(h osition is still
used 2y so'e sur1eons 2e(ause it er'its the o((iut to 2e rotated a little 2it 'ore
sueriorly) 4his allo/s a sli1htly 'ore dire(t .ie/ of the internal auditory (anal)
4he oeration is erfor'ed throu1h either a .erti(ally oriented linear in(ision or an
anteriorly 2ased U-shaed fla) An o((iital (ranioto'y is then erfor'ed) Any
'astoid air (ells are (arefully /a0ed off to re.ent ostoerati.e (ere2rosinal fluid
lea?) 4he dura is oened and the ara(hnoid in(ised) 4he (ere2ellu' fre8uently falls
a/ay fro' the osterior surfa(e of the te'oral 2one after the (isterna 'a1na has
2een oened) Iyer.entilation, steroids, and intraoerati.e diureti(s Crin(ially
'annitolD are used to redu(e intra(ranial ressure and to ro.ide additional e0osure
/ith a li'ited a'ount of retra(tion) @onetheless, 1entle (ere2ellar retra(tion is
o((asionally re8uired ese(ially in lar1er tu'ors)
6n(e ade8uate e0osure has 2een o2tained, the tu'or is (learly .isualized alon1 /ith
the 2rain ste' and lo/er (ranial ner.es) Io/e.er, (ranial ner.es 955 and 9555 are
rarely o2ser.ed 2e(ause they are al'ost al/ays ushed for/ard and lie a(ross the
anterior surfa(e of the tu'or, /hi(h (annot 2e .isualized) De2ul?in1 of the tu'or is
the ne0t ste and 'ust 2e (arefully erfor'ed so as to 'aintain the anterior ortions
of the (asule in order to re.ent inAury to (ranial ner.e 955 and&or 9555) 6n(e the
tu'or has 2een su2stantially de2ul?ed, the osterior /all of the internal auditory
(anal (an 2e re'o.ed usin1 a hi1h-seed drill)
Freat (are 'ust 2e ta?en to a.oid inAurin1 the la2yrinth /hile re'o.in1 the osterior
/all of the internal auditory (anal) 7ortions of the la2yrinth 8uite (o''only are
'edial to the lateral end of the internal auditory (anal) Althou1h no sin1le anato'i(
land'ar? is (o'letely relia2le for re.ention of inAury to the la2yrinth, the sin1ular
ner.e and its (anal, and the oer(ulu' of the .esti2ular a8uedu(t, are used as
i'ortant sur1i(al land'ar?s) Careful 'easure'ents ta?en fro' reoerati.e C4
s(ans (an ro.ide useful infor'ation durin1 drillin1 of the osterior /all of the
internal auditory (anal)
4he len1th of the internal auditory (anal .aries (onsidera2ly, and ?no/in1 e0a(tly
ho/ 'u(h osterior (anal /all needs to 2e re'o.ed to ade8uately e0ose the tu'or
(an hel li'it inad.ertent inAury to the la2yrinth) Blind e0tra(tion of tu'or fro' the
internal auditory (anal /ithout re'o.in1 the osterior /all oses a si1nifi(ant ris? to
the fa(ial and&or auditory ner.e inte1rity and in(reases the (han(e of lea.in1 tu'or at
the fundus) Use of intraoerati.e an1led endos(oes has 2een reorted as an adAun(t
in erfor'in1 this hase of the oeration)
E.ery effort should 2e 'ade to re.ent 2one dust fro' enterin1 the su2ara(hnoid
sa(e durin1 the intradural drillin1 of the internal auditory (anal) 6ne ro2a2le (ause
for se.ere and intra(ta2le ostoerati.e heada(he is silla1e of 2one dust into the
su2ara(hnoid sa(e durin1 tu'or re'o.al) Sur1i(el, Felfoa', 4elfa ads, and&or
(ottonoid stris are la(ed around the oerati.e site so that 2one dust adheres to the'
and is re'o.ed as they are re'o.ed) 6n(e the internal auditory (anal is e0osed, the
dura is oened and the tu'or is re'o.ed) Althou1h ne.er ro.en, disse(tion fro'
'edial to lateral is thou1ht to 2e less trau'ati( to 2oth the (o(hlear ner.e and to the
.as(ular suly of the inner ear) 4he .esti2ular ner.es are 1enerally sa(rifi(ed, and
unless hearin1 is to 2e reser.ed, the (o(hlear ner.e is sa(rifi(ed as /ell)
E.entually, the sur1eon is left /ith the anterior ortions of the (asule adhered to the
2rain ste' and (ranial ner.e 955) As the tu'or (asule is (arefully re'o.ed fro' the
2rain ste', the root entry zone of (ranial ner.e 955 (an 2e identified) 4he (asule is
then (arefully re'o.ed fro' the fa(ial ner.e /ith as little trau'a as ossi2le)
4he fa(ial ner.e 'onitor fa(ilitates this ortion of the disse(tion) A 'eanin1ful
a'ount of data no/ sho/s that results are i'ro.ed /hen fa(ial ner.e 'onitorin1 is
e'loyed) A .ariety of te(hni8ues ha.e 2een used to 'onitor the (o(hlear ner.e /hen
hearin1 reser.ation is desired) 4he 'ost (o''only used 'ethod is intraoerati.e
AB:, 2ut it has a nu'2er of disad.anta1es) Most i'ortantly, it re8uires su''in1 a
lar1e nu'2er of reetitions in order to e0tra(t a resonse fro' 2a(?1round noise)
Conse8uently, a delay o((urs 2et/een sur1i(al 'aniulations and AB: (han1es)
Dire(t (o(hlear ner.e 'onitorin1 offers the ad.anta1e of real-ti'e feed2a(?, 2ut a
fully satisfa(tory 'ethod of la(in1 and se(urin1 the ele(trode still is la(?in1)
6n(e tu'or re'o.al is (o'lete and he'ostasis is a2solute, the dura is (losed and the
(ranioto'y defe(t is reaired, either 2y rela(in1 the ori1inal 2one fla or /ith
'ethyl'etha(rylate or hydro0yaatite)
%iddle cranial fossa approach
Althou1h so'e sur1eons use an e0tended 'iddle (ranial fossa aroa(h for tu'ors
that e0tend a (enti'eter or 'ore outside the orus a(usti(us into the (ere2elloontine
an1le, the 'iddle (ranial fossa aroa(h is 'ost fre8uently used for intra(anali(ular
tu'ors) 5t is, 2y (onsensus, the aroa(h of (hoi(e for s'all tu'ors that lie /ithin the
lateral ortions of the internal auditory (anal /hen hearin1 (onser.ation is desired)
4he head 'ust 2e in the true lateral osition) 5n youn1 indi.iduals /ith a sule ne(?,
this (an often 2e a((o'lished 2y turnin1 the head to the side /ith the atient in the
suine osition) But if ne(? 'o2ility is li'ited or (on(ern e0ists that for(ed head
turnin1 /ill li'it osterior fossa (ir(ulation or a11ra.ate (er.i(al sine disorders, then
a true lateral Car?-2en(hD osition should 2e used)
E0osure 'ust 2e (entered o.er a .erti(ally oriented line that asses aro0i'ately !
(' anterior to the e0ternal auditory 'eatus) 4his is 'ost easily a((o'lished throu1h
a linear in(ision) A osteriorly 2ased U-shaed or (ur.ilinear S-shaed in(ision (an
2e used if (on(ern e0ists a2out s(ar (ontra(ture) Deendin1 uon the in(ision used,
the te'oralis 'us(le is in(ised or refle(ted inferiorly) A te'oral (ranioto'y
Caro0i'ately ; (' 2y ; ('D is erfor'ed /ith its 2ase at the root of the zy1o'a)
4he dura is ele.ated fro' the floor of the 'iddle (ranial fossa, and os'oti( diureti(s,
head ele.ation, hyer.entilation, and steroids are used to li'it (ere2ral ede'a)
4he dura of the te'oral lo2e is then ele.ated off the suerior surfa(e of the te'oral
2one) 4he anterior e0tent of su(h ele.ation is usually the fora'en sinosu', 2ut the
'iddle 'enin1eal artery (an 2e di.ided 2et/een (lis and ele.ation (ontinued
anteriorly to the fora'en o.ale if additional e0osure is desired) Dural ele.ation
should ro(eed fro' osterior to anterior to a.oid inAury to an e0osed 1reater
suerfi(ial etrosal ner.e or 1eni(ulate 1an1lion) Bleedin1 fro' the .eins asso(iated
/ith the 'iddle 'enin1eal artery is often 8uite 2ris? 2ut (an 1enerally 2e (ontrolled
/ith o0idized (ellulose a(?in1) Medial disse(tion (ontinues to the free ed1e of the
te'oral 2one)
4he suerior etrosal sinus is atta(hed to the osterior surfa(e of the te'oral 2one
2ut not al/ays at its suerior ed1e) Care 'ust 2e ta?en to a.oid inAurin1 it) 5f
inad.ertent inAury o((urs, 2leedin1 (an 1enerally 2e (ontrolled /ith intralu'inal
o0idized (ellulose a(?in1, ele(tro(autery, or he'o(lis) When e0tended 'iddle
(ranial fossa aroa(hes are e'loyed, the suerior etrosal sinus is deli2erately
di.ided 2et/een (lis)
When it (an 2e identified easily, the ar(uate e'inen(e is an e0tre'ely helful
land'ar?) Careful drillin1 (an often identify the 2lue line of the suerior (anal
inferior to it) Be(ause the 'ost diffi(ult e0osure to a(hie.e durin1 'iddle fossa
sur1ery is the lateral osterior end of the internal auditory (anal, disse(tion is
erfor'ed as (lose to the suerior se'i(ir(ular (anal as ossi2le) 4he 1reater
suerfi(ial etrosal ner.e is 1enerally easy to .isualize and (an 2e follo/ed retro1rade
to the 1eni(ulate 1an1lion) 5t lies aro0i'ately !)$ (' dire(tly 'edial to the fora'en
sinosu') 6n(e the area of the 1eni(ulate is identified, s'all dia'ond 2urrs are used
to (o'letely e0ose it) 5f the 1reater suerfi(ial etrosal ner.e (annot 2e lo(ated and
no other land'ar?s are a.aila2le, the 'iddle ear sa(e (an 2e entered fro' a2o.e and
the head of the 'alleus (an 2e identified) 4he 1eni(ulate 1an1lion lies aro0i'ately
#-B '' anterior and 'edial to the head of the 'alleus)
6n(e the 1eni(ulate 1an1lion has 2een (o'letely e0osed, the la2yrinthine ortion
of the ner.e (an 2e identified and follo/ed 'edially and inferiorly into the internal
auditory (anal) 4he la2yrinthine ortion of the ner.e ta?es a 'ar?edly .erti(al and
'edial (ourse as it 'o.es fro' the lateral 1eni(ulate 1an1lion to the ro0i'al fundus
of the internal auditory (anal, /hi(h lies ; or 'ore 'illi'eters dee to the 1eni(ulate
1an1lion) So'e sur1eons refer to identify the internal auditory (anal 'edially) 6n(e
the 'edial end of the (anal is (o'letely identified, they follo/ the (anal laterally to
the fundus of the internal auditory (anal)
4he 2one o.erlyin1 the internal auditory (anal should 2e re'o.ed until aro0i'ately
#+$ R of the internal auditory (anal is e0osed) 4he 'ost diffi(ult area to e0ose is the
oint at /hi(h the suerior .esti2ular ner.e enetrates the la2yrinthine 2one to
inner.ate the a'ulla; ho/e.er, e0osure in this area is (riti(al if the anato'y of the
lateral end of the internal auditory (anal is to 2e /ell .isualized) 5f the suerior
.esti2ular ner.e (hannel is identified, tu'or re'o.al is 1enerally su((essful and
relati.ely strai1htfor/ard)
<ar1er tu'ors fre8uently ha.e the fa(ial ner.e slayed out o.er the anterior suerior
ortions of the tu'or) 4u'or re'o.al 2e1ins, as /ith other aroa(hes, 2y (areful
de2ul?in1) 6n(e the tu'or is de2ul?ed, enou1h roo' is (reated /ithin the internal
auditory (anal to (arefully re'o.e the tu'or (asule fro' the inferior surfa(e of the
fa(ial ner.e) A1ain, (are 'ust 2e ta?en to a.oid torsion or t/istin1 of the ner.e durin1
tu'or re'o.al)
6n(e the tu'or has 2een (o'letely re'o.ed, the inte1rity of the fa(ial ner.e is
tested usin1 the intraoerati.e fa(ial ner.e 'onitor) 7resu'a2ly, the 'onitor has 2een
in use throu1hout the (ase) 5f the fa(ial ner.e (an 2e sti'ulated /ith lo/ sti'ulus
intensities, (han(es of 1ood ostoerati.e fa(ial ner.e fun(tion in(rease) Fat is then
a(?ed into the internal auditory (anal after usin1 2one /a0 to fill o2.ious air (ells to
re.ent ostoerati.e (ere2rosinal fluid lea?) 4he fa(ial ner.e 'onitor 1enerally
alerts the hysi(ian if fat is 2ein1 a(?ed in too ti1htly that the inte1rity of the fa(ial
ner.e is 2ein1 (o'ro'ised) :etra(tors are re'o.ed, and the te'oral lo2e dura is
allo/ed to rela0) 4he 2one late is rela(ed usin1 'inilates, and the /ound is (losed
in 'ultile layers
Postoperative !etails
Unless a (o'li(ation de.elos, ostoerati.e (are is strai1htfor/ard) 4he atient is
1enerally ?et in the 5CU o.erni1ht so that raid inter.ention is a.aila2le if
ostoerati.e intra(ranial ressure in(reases or 2leedin1 o((urs) 9esti2ular
reha2ilitation should 2e1in on the first ostoerati.e day and (ontinues t/i(e daily
throu1hout the hosital stay) Most atients (an 2e dis(har1ed on the third or fourth
ostoerati.e day)
"ollo%&up
A follo/-u M:5 is o2tained /ithin ,-!# 'onths after sur1i(al e0(ision to do(u'ent
the (o'leteness of tu'or re'o.al and to ser.e as a 2aseline for further follo/-u
s(ans) Assu'in1 (o'lete tu'or re'o.al, follo/-u M:5 should 2e o2tained at ;
years and at !$ years) 5f the findin1s on the !$-year s(an are nor'al, further i'a1in1
should 2e erfor'ed only if (lini(al (ir(u'stan(es re8uire it) 7ostoerati.e M:5
s(ans 'ust 2e erfor'ed /ith fat-suressed te(hni8ues if fat /as used to o2literate
the sur1i(al site)
Complications
Arterial in&ury
5nAury to the A5CA C'u(h less (o''only to the 75CAD fortunately o((urs .ery rarely)
Althou1h the A5CA 'ay 2e loosely atta(hed to the tu'or (asule, searatin1 it fro'
the tu'or is 1enerally fairly easy) Sa(rifi(in1 the A5CA itself has .aria2le
(onse8uen(es deendin1 uon the details of indi.idual atient anato'y) 5t (an 2e
(atastrohi( and lead to de.astatin1 neurolo1i( inAury or death)
4he 2ran(hes of A5CA 'ost .ulnera2le to inAury are, of (ourse, the la2yrinthine artery
and 2ran(hes sulyin1 the fa(ial ner.e) So'e other/ise erle0in1 (ases of
ostoerati.e fa(ial ner.e /ea?ness 'ay 2e related to interrution of fa(ial ner.e
.as(ular suly due to (oa1ulation of s'all 2ran(hes of the A5CA) Failure to (onser.e
hearin1 'ay 2e due to the disrution of (o(hlear 2lood suly) Be(ause the
la2yrinthine artery 'ay 2e inti'ately asso(iated /ith the tu'or, sa(rifi(e often (annot
2e a.oided) Conser.ation of the internal la2yrinthine artery 2e(o'es 'ore diffi(ult as
tu'or size in(reases, dou2tlessly a((ountin1 in so'e 'easure for the redu(ed su((ess
in hearin1 (onser.ation /ith lar1er tu'ors) @eurolo1i( inAury or (ere2ral ede'a
se(ondary to .enous inAury usually o((urs as a result of inAury to the si1'oid sinus
itself, the etrosal .ein of Dandy, or to the .ein of <a22N)
6((lusion of the si1'oid sinus has .aria2le effe(ts deendin1 lar1ely uon the
atientPs uni8ue .enous anato'y) 5f the (ontralateral .enous outflo/ tra(t is atent
and (o''uni(ation throu1h the tor(ula herohili is ade8uate, (o'lete o((lusion 'ay
2e asy'to'ati() 4he size of the # si1'oid sinuses is usually asy''etri(al, /ith a
1reater .olu'e of 2lood flo/in1 throu1h the ri1ht-sided sinus) Deendin1 on ho/
'u(h 'ore 2lood flo/s throu1h the do'inant sinus, o((lusion of the do'inant sinus
(an result in (atastrohi( in(reases in intra(ranial ressure, .enous infar(tion, and
e.en death) Be(ause a nu'2er of otential (ollaterals e0ist 2et/een the tor(ula
herohili and the Au1ular 2ul2, o((lusion of the si1'oid sinus (lose to the tor(ula
herohili is 'u(h 'ore li?ely to ha.e si1nifi(ant ad.erse effe(ts than its o((lusion
(lose to the Au1ular 2ul2)
4he etrosal .ein of Dandy is a sin1le lar1e outflo/ tra(t in so'e atients 2ut (onsists
of series of se.eral lar1e .eins in others) 5ts o((lusion (an result in ede'a and
infar(tion of either the te'oral lo2e or the 2rain ste') Althou1h neurolo1i( inAury
se(ondary to o((lusion of the Dandy .ein is not ine.ita2le, se.ere inAury (an o((ur,
and the etrosal .ein should 2e (arefully reser.ed)
6((lusion of the .ein of <a22N results in se.ere ede'a of the te'oral lo2e and
te'oral infar(t) 4he ede'a (an 2e suffi(iently se.ere to (ause 2rain herniation and
death) 4he .ein of <a22N 1enerally enters the suerior etrosal or trans.erse sinus
2et/een the tor(ula herohili and the oint at /hi(h the suerior etrosal sinus Aoins
the trans.erse sinus) 4hus, it is 1enerally not dire(tly in the field durin1 a(ousti(
tu'or sur1ery) 6((asionally, ho/e.er, inAury to the suerior etrosal sinus results in
its o2literation, and in so'e instan(es, the .ein of <a22N is also inAured or o2stru(ted)
5ts resen(e and i'ortan(e should 2e ?et in 'ind durin1 a(ousti( tu'or sur1ery)
Ie'orrha1e into the osterior fossa in the i''ediate ostoerati.e eriod (an
rodu(e 2rainste' (o'ression and death 8uite raidly) Death (an o((ur /ithin a fe/
'inutes) :aid neurolo1i( deterioration in the first #% hours ostoerati.ely should
raise susi(ion of osterior fossa he'orrha1e and 'andates raid and de(isi.e
inter.ention) 5f ti'e er'its, a raid unenhan(ed C4 s(an should 2e o2tained to
se(ure the dia1nosis /hile the oeratin1 roo' is reared for an i''ediate return to
sur1ery) 5f neurolo1i( deterioration is raid, for1o C4 s(annin1 and ta?e the atient
dire(tly 2a(? to the oeratin1 theatre) 5f deterioration is .ery raid /ith loss of
(ons(iousness, de(ere2rate osturin1, and si1ns of i''inent death, oen the /ound at
the 2edside to er'it a osterior fossa de(o'ression rior to e'er1ent transortation
of the atient to the oeratin1 roo' for /ound e0loration, de2ride'ent, and
e0tensi.e irri1ation)
'erebellar in&uries
5nAury to the (ere2ellu' /as (o''on in the early de(ades of the (entury, 2ut its
in(iden(e has dra'ati(ally di'inished in re(ent de(ades) Cere2ellar inAuries still
o((ur 2ut are 1enerally not trou2leso'e) 4he rotatin1 shaft of the sur1eonPs 2urr is
often the (ulrit 2e(ause sur1eons usually loo? ast the shaft to the head of the 2urr to
(on(entrate on 2one re'o.al) 4he shaft is often outside the sur1i(al field of .ie/)
Su(h s'all areas of inAury rarely ha.e noti(ea2le se8uelae) Bleedin1 (an 2e (ontrolled
/ith o0idized (ellulose, (autery, or 1elatin son1es, and ede'a is li'ited) Dire(t
inAury to the (ere2ellar he'ishere fro' (o'ression and retra(tion, intra(ere2ral
he'orrha1e, infar(tion due to alteration of the arterial inflo/, or .enous en1or1e'ent
/ith our /ithout infar(tion (an rodu(e se.ere ede'a of the entire (ere2ellu')
Brainste' (o'ression and&or intra(ranial herniation (an rodu(e death) 62stru(tion
of the fourth .entri(le and (ere2ral a8uedu(t (an rodu(e si1nifi(ant hydro(ehalus)
Mana1e'ent should (onsist of a11ressi.e use of os'oti( diureti(s, hyer.entilation,
and steroids) 5f 'edi(al 'ana1e'ent is unsu((essful, rese(tion of art of the in.ol.ed
(ere2ellar he'ishere 'ay 2e re8uired)
Facial paralysis
5n so'e (ases, ostoerati.e fa(ial aralysis is una.oida2le) 4he tu'or 'ay si'ly 2e
atta(hed too inti'ately to the thin attenuated fa(ial ner.e) So'eti'es the tu'or has
en.eloed the fa(ial ner.e, and tu'or re'o.al (annot 2e a((o'lished /ithout
rese(tion of a ortion of the fa(ial ner.e)
Eye (are is (riti(al to su((essful 'ana1e'ent of ostoerati.e fa(ial aralysis) Ma?e
li2eral use of artifi(ial tears se(ifi(ally adated to deal /ith dry eye Ce1, Bion 4earsD)
At ni1ht, la(e o(ular lu2ri(ants Ce1, <a(ri-<u2eD in the eye) 5f a11ressi.e use of
artifi(ial tears durin1 the day C8!;-B$'inD and oint'ent at ni1ht is insuffi(ient to
'aintain (orneal hydration and e0osure ?eratitis 2e1ins to de.elo, then (onsider use
of an eye at(h, la(e'ent of a 1old /ei1ht, and lo/er lid shortenin1 ro(edures)
4arsorrhahy should 2e used only as a last resort and is only .ery rarely re8uired)
Coe0istin1 inAury to (ranial ner.e 9 /ith (orneal hyesthesia or anesthesia .astly
in(reases the ro2le' in 'ana1e'ent) 4he la(? of (orneal sensation ro.ides the
atient no relia2le 1uide as to the se.erity of (orneal eithelial disrution) 5n su(h
(ases, tarsorrhahy is 'u(h 'ore li?ely to 2e re8uired)
'erebrospinal fluid complications
4ransient a2nor'ality of (ere2rosinal fluid resortion 'ay lead to 'ild te'orary
ostoerati.e hydro(ehalus) Althou1h ostoerati.e shuntin1 (an fa(ilitate
(ontrollin1 (ere2rosinal fluid fistula, it is no/ rarely, if e.er re8uired) E.en /hen
hydro(ehalus is resent in the reoerati.e eriod, it 1enerally resol.es /ithout
diffi(ulty in the first fe/ ostoerati.e /ee?s)
7ostoerati.e 'enin1itis o((urs in # for's) Ba(terial 'enin1itis is otentially life
threatenin1 and o((urs in less than !3 of atients in the ostoerati.e eriod) 5t (an
o((ur /ithin the first #%-B, hours ostoerati.ely, or its aearan(e 'ay 2e delayed
for a (oule of /ee?s) 6n(e initiated, it (an ro1ress .ery raidly, and indi.iduals (an
lase fro' a nor'al le.el of (ons(iousness into a dense (o'a in a 'atter of a fe/
hours)
Conse8uently, inter.ention 'ust 2e raid) Dia1nosis deends uon the resen(e of
fe.er and, in the alert atient, the resen(e of heada(he, stiff ne(?, nu(hal ri1idity, and
de(reasin1 le.el of (ons(iousness) 5f 'enin1itis is suse(ted, erfor' an
i''ediate lu'2ar un(ture to o2tain fluid for (ulture, 2ut only after a C4 s(an has
e0(luded the ossi2ility of si1nifi(ant hydro(ehalus, /hi(h (ould lead to 2rain
herniation)
62tain sinal fluid for Fra' stain, 1lu(ose, rotein, and /hite 2lood (ell (ount) 5f the
Fra' stain is ositi.e, sinal fluid 1lu(ose is less than %$ '&d<, or the sinal fluid
/hite 2lood (ell (ount is hi1her than #;$$ (ells&''
B
, 2e1in anti2ioti(s i''ediately
endin1 (ulture results) 5f the sinal fluid does not 'eet any of these (riteria, (losely
o2ser.e the atient /ith the understandin1 that any deterioration of the (ondition
re8uires a reeat lu'2ar un(ture for additional sinal fluid)
Aseti( 'enin1itis has 2een reorted in +-+$3 of ostoerati.e neurosur1i(al
atients) 5t shares /ith 2a(terial 'enin1itis the (lini(al si1ns of in(reasin1 heada(he,
fe.er, nu(hal ri1idity, and ele.ation of (ere2rosinal fluid ressure) Sinal fluid
rofile in su(h atients sho/s 'ar?ed ele.ation of /hite 2lood (ell (ount and
(ere2rosinal fluid rotein le.els, 2ut (ere2rosinal fluid 1lu(ose re'ains /ithin the
referen(e ran1e, and (ulture results C/hen they are finally (o'leteD are nor'al)
Corti(osteroids are e0tre'ely helful in 'ana1in1 aseti( 'enin1itis, and their
ro't ad'inistration often results in 'ar?ed de(rease in heada(he and nu(hal
ri1idity /ithin a fe/ hours)
Sinal fluid lea? throu1h either the /ound or the eusta(hian tu2e and 'iddle ear
o((urs in #-#$3 of atients) 5t (an o((ur after transla2yrinthine or retrosi1'oid
aroa(hes and is less (o''on after 'iddle fossa (ranioto'y) When it follo/s
retrosi1'oid aroa(hes, the ath of e1ress is 1enerally throu1h neu'atized air (ell
tra(ts)
Cere2rosinal fluid is rodu(ed /ithin the .entri(ular syste' at a rate of $)B '<&'in
or at a2out ;$$ '<&day) 5t enters the su2ara(hnoid sa(e in the osterior fossa .ia the
'idline and lateral fora'en of the fourth .entri(le) Conta'ination of the
(ere2rosinal fluid (ir(ulation 2y 2lood, 2one dust, and ne(roti( de2ris at the ti'e of
sur1ery often i'airs (ere2rosinal fluid a2sortion dire(tly 2y 'e(hani(al
interferen(e in the ara(hnoid .illi or indire(tly 2y in(itin1 an infla''atory resonse
/ithin the su2ara(hnoid sa(e) 4he syndro'e 'ay .ary fro' 2rief asy'to'ati(
ele.ation of (ere2rosinal fluid ressure to (lini(ally 'anifested aseti( 'enin1itis
Cdis(ussed a2o.eD) Cere2rosinal fluid es(ain1 throu1h the /ound (an initially 2e
'ana1ed 2y resuturin1 the /ound) So'eti'es this results in eli'ination of the
diffi(ulty, /hile at other ti'es it 'erely rodu(es (ere2rosinal fluid rhinorrhea, as
the sinal fluid finds an alternate 'eans of e1ress)
5f the (ere2rosinal fluid lea? ersists for 'ore than !#-#% hours after initiation of
(onser.ati.e 'ana1e'ent, in(ludin1 ressure dressin1 and (onsistent head ele.ation,
then (onsider redu(in1 the (ere2ral sinal fluid ressure 2y ! of B 'easures, in(ludin1
C!D 'ultile lu'2ar un(tures, C#D (ontinuous or inter'ittent draina1e .ia lu'2ar
intradural (atheter, or CBD er'anent (ere2rosinal fluid di.ersion 2y 'eans of an
ind/ellin1 shunt)
When (ere2rosinal fluid di.ersion is sele(ted, the 'ost (o''on 'ethod is an
ind/ellin1 su2ara(hnoid (atheter la(ed into the lu'2ar su2ara(hnoid sa(e) 4he
drain is oened eisodi(ally so as to re'o.e #$$-%$$ '< of sinal fluid in any 1i.en
#%-hour eriod) So'e sur1eons o2ser.e a 'ini'u' draina1e eriod of # days, others
; days) Feneral (onsensus is that, if the drain has 2een in la(e for 'ore than ; days,
it should 2e rela(ed to a.oid infe(tion) 9ariation a'on1 sur1eons is (onsidera2le as
to /hen ree0loration is re8uired) So'e (enters ree0lore after #%-%* hours of
draina1e; other (enters use as 'any as t/o ;-day trials of (ontinuous lu'2ar draina1e
2efore (onsiderin1 a se(ond oeration)
Se.ere ostoerati.e heada(he has lon1 2een asso(iated /ith retrosi1'oid
ro(edures) 4his ro2le' aears to ha.e di'inished (onsidera2ly sin(e the
introdu(tion of # intraoerati.e stes: C!D 1reat (are is ta?en to a.oid (onta'inatin1
the sinal fluid and su2ara(hnoid sa(e /ith 2one dust, and C#D the 2one fla is
rela(ed and any residual 2ony defe(t is eli'inated /ith 'ethyl'etha(rylate or
hydro0yaatite) 4he latter ste eli'inates the dire(t atta(h'ent of osterior (er.i(al
'us(ulature to the dura) When ostoerati.e heada(hes do o((ur, they should 2e
'ana1ed /ith relati.ely hi1h-dose nonsteroidal anti-infla''atory a1ents and
a11ressi.e re1i'ens of 'aniulati.e hysi(al theray)
'utcome and Prognosis
Tinnitus
4innitus 2e(o'es /orse in only ,-#$3 of indi.iduals after tu'or re'o.al) 5n a
su2stantial nu'2er of indi.iduals, the tinnitus re'ains un(han1ed) 5n a2out #;-,$3
of atients, tinnitus is eli'inated or i'ro.ed) Althou1h B$-;$3 of atients /ho had
no reoerati.e tinnitus de.elo it in the i''ediate ostoerati.e eriod, su(h
tinnitus only rarely 2e(o'es trou2leso'e)
$ecurrence(residual tumor
:e(urren(e is un(o''on after a(ousti( tu'or re'o.al) 6.erall, the re(urren(e rate is
less than ;3) 4he .ast 'aAority of re(urren(es follo/ retrosi1'oid re'o.al)
7resu'a2ly, a s'all a'ount of tu'or is left in the lateral end of the internal auditory
(anal /here intraoerati.e .isualization is diffi(ult) 4u'or re(urren(e 'ay 2e
suse(ted 2y re(urrin1 heada(he, altered sensation to the fa(e, or dysarthria and
dyshasia if the lo/er (ranial ner.es 2e(o'e in.ol.ed)
5nfla''ation in the tu'or 2ed 'ay ersist for 'onths and e.en years after a(ousti(
tu'or re'o.al, and (onse8uently, areas of (ontrast enhan(e'ent are resent on
ostoerati.e 1adoliniu' M:5) Distin1uishin1 tu'or re(urren(e fro' ostoerati.e
infla''ation (an 2e 8uite diffi(ult) 4u'or re(urren(es tend to 2e 1lo2ular /hile
ostoerati.e infla''atory enhan(e'ent tends to 2e linear) 6ften, ho/e.er, one 'ust
.ie/ serial s(ans to dete(t tu'or re(urren(e) Fat suression te(hni8ues are essential
for ostoerati.e sur.eillan(e to distin1uish re(urren(e fro' fat a(?in1) Sur.eillan(e
for ostoerati.e tu'or re(urren(e should ersist for *-!$ years ostoerati.ely)
Facial function
7reser.ation of fa(ial fun(tion (ontinues to i'ro.e, ese(ially /ith the /idesread
use of fa(ial ner.e 'onitorin1) Io/e.er, fa(ial ner.e out(o'es (ontinue to .ary
a((ordin1 to tu'or size) When tu'ors are s'aller than !); (', 1ood fa(ial ner.e
fun(tion (an 2e e0e(ted CIouse-Bra(?'ann 1rade 5-55D in 'ore than "$3 of atients)
5n addition to tu'or size, reoerati.e ele(trohysiolo1i( testin1 (an hel redi(t
ostoerati.e out(o'e, althou1h this testin1 is not (o''only used) De'onstra2le
ele(trohysiolo1i( a2nor'alities on ner.e (ondu(tion studies, ele(tro'yo1rahy, and
2lin? refle0 testin1 (orrelate /ell /ith ostoerati.e fa(ial ner.e defi(its) Arria1a has
sho/n that atients /ith oor fa(ial ner.e fun(tion at the ti'e of dis(har1e CIouse-
Bra(?'ann 9-95D had a #;3 (han(e of re(o.ery of nor'al fun(tion CIouse-
Bra(?'ann 5-55D) <ess oti'isti( is the reort of Ster?ers) 5n his series of atients,
anyone /ho had Iouse-Bra(?'ann 555 fun(tion or /orse at a %- to ,-/ee?
ostoerati.e e.aluation /as left /ith si1nifi(ant defi(it and 1enerally had so'e
syn?inesis)
Fa(ial ner.e aralysis 'ay 2e delayed and 'ay de.elo /ithin a fe/ hours to a /ee?
or 'ore after a(ousti( neuro'a re'o.al) 5n(iden(e of delayed fa(ial alsy .aries fro'
!$-B$3) 4he 'e(hanis' of a(tion is un(lear) 5s(he'ia se(ondary to .asosas',
.as(ular inAury, tra(tion, ner.e ede'a, stret(hin1, and e.en a .iral rea(ti.ation ha.e
2een roosed) Unli?e final fa(ial ner.e out(o'e, in(iden(e of delayed fa(ial
aralysis does not aear to 2e related to tu'or size)
4he .ast 'aAority of indi.iduals /ho ha.e delayed onset of fa(ial aralysis 'a?e
(o'lete and total re(o.eries) 5f deterioration is se.ere C'ore than B Iouse-
Bra(?'ann 1radesD, so'e (han(e of oor lon1-ter' out(o'e e0ists) 7erioerati.e
steroids are /idely used in an atte't to enhan(e 2oth i''ediate and lon1-ter'
ostoerati.e fa(ial ner.e fun(tion, 2ut une8ui.o(al e.iden(e for their effe(ti.eness is
la(?in1) 4he use of erioerati.e anti.iral a1ents is used 2y so'e (enters to re.ent
delayed aralysis fro' .iral rea(ti.ation, as in Bell alsy)
)earing outcome
4he a2ility to reser.e hearin1 has in(reased su2stantially o.er the last de(ade or t/o)
Deendin1 on (riteria for su((essful hearin1 (onser.ation, hearin1 (an 2e reser.ed in
B$-*$3 of roerly sele(ted atients)
Stereota(ti( radiation Cthe 1a''a ?nifeD does not aear to ha.e a si1nifi(antly hi1her
rate of hearin1 (onser.ation than does roerly (ondu(ted sur1ery /hen lon1-ter'
results are (o'ared) Chora, et al de'onstrated a hearin1 reser.ation rate of %%3 at
!$-year follo/-u in #!, atients re(ei.in1 Fa''a Knife radiosur1ery)
:osen2er1 et al and 4u((i et al ha.e 2oth sho/n reasona2le sta2ility of hearin1 o.er
ti'e after sur1ery)
=,>
6n the other hand, SheltonPs study aears to sho/ si1nifi(ant
hearin1 deterioration in B$-;$3 of atients /ho ori1inally had su((essful hearin1
reser.ation)
$ehabilitation
4he hearin1 defi(it after the re'o.al of an a(ousti( neuro'a (an ha.e a si1nifi(ant
i'a(t on 8uality of life) :eha2ilitation otions in(lude a (ontralateral routin1 of
si1nals CC:6SD hearin1 aid or 'ore re(ently a 2one an(hored hearin1 aid CBAIAD)
4he BAIA (onsists of a sur1i(al i'lanted titaniu' a2ut'ent that osseointe1rates
into the (al.aria) A see(h ro(essor is then snaed onto the a2ut'ent, allo/in1
sound to trans'it throu1h the s?ull to the nor'al (ontralateral ear)
5n atients /ith neurofi2ro'atosis tye #, all atients de.elo 2ilateral a(ousti(
neuro'as and 'ost /ill e.entually lose hearin1 in 2oth ears) 4he auditory 2rainste'
i'lant CAB5D (an 2e inserted durin1 a transla2yrinthine (ranioto'y and ro.ide
atients the 2enefit of auditory inut to assist /ith (o''uni(ation)
5f a atient e0erien(es fa(ial ner.e /ea?ness and not total aralysis after sur1i(al
re'o.al, often eye (are (onsistin1 of artifi(ial tears and lu2ri(ant /ill 2e suffi(ient
until fa(ial ner.e fun(tion returns) 5f the fa(ial ner.e is se.ered intraoerati.ely, the
ner.e (an 2e aro0i'ated at the ti'e of sur1ery) 5f the atient has fa(ial ner.e
aralysis ! year after sur1ery, the (han(e of further re(o.ery is re'ote) At this ti'e, a
hyo1lossal-fa(ial ner.e 1raft 'ay 2e (onsidered) 4his /ill often result in i'ro.ed
tone and ossi2ly eye (losure)
"uture and Controversies
4he relati.e roles of stereota(ti( radiation and sur1ery /ill re'ain (ontro.ersial until
the lon1-ter' results of radiation treat'ent roto(ols are (learly defined)