*ept+ o& Otolar%ngolog% *ATE: September ,-# .-/- RESI*E0T "12SICIA0: An!rew Coughlin (+* FACULT2 "12SICIA0: Tomo3o (a3ishima (*# "h* *ISCUSSA0T: Tomo3o (a3ishima (*# "h* SERIES E*ITOR: Francis )+ 4uinn# 5r+# (* ARC1IVIST: (elin!a Stoner 4uinn# (SICS "This material was prepared by resident physicians in partial fulfillment of educational requirements established for the Postgraduate Training Program of the UTMB Department of Otolaryngology/ead and !ec" #urgery and was not intended for clinical use in its present form$ %t was prepared for the purpose of stimulating group discussion in a conference setting$ !o warranties& either e'press or implied& are made with respect to its accuracy& completeness& or timeliness$ The material does not necessarily reflect the current or past opinions of members of the UTMB faculty and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion$" Introduction The cerebellopontine angle (CPA) is a dense area of vital neurologic tissue. Tumors growing in this region can cause significant dysfunction and even death if allowed to grow too large. They represent 10 of all intracranial tumors and although the differential is !uite e"tensive# $% of tumors in this region are vestibular schwannomas (1). The second and third most common tumors in this region are meningiomas and epidermoid tumors respectively. Autopsy studies have shown an incidence of CPA tumors to be 1.$ to &.$ however a single '() study loo*ing at 10#000 films obtained for non+otologic reasons showed only $ positive cases (&+,). -ecause of the significant dysfunction these tumors can cause# timely diagnosis and treatment are paramount. History of CPA tumors )n 1%., /ir Charles -alance was the first surgeon to successfully remove a vestibular schwannoma. 0ue to the significant morbidity of the surgery# however# his first patient died shortly after the operation from complications. )n 1.0$# -alance# improved upon his previous outcome and performed a successful surgical e"cision without the patient mortality. 0espite this outcome the patient still had significant facial nerve paralysis complicating the e"cision (1#2). )n 1.1$ 3arvey Cushing pioneered the subtotal resection through bilateral suboccipital craniectomy and described the CPA syndrome ($). The CPA syndrome is the constellation and progression of symptoms as a CPA tumor grows larger. Cushing described it as ipsilateral hearing loss followed by facial hypesthesia# hydrocephalus# and finally brainstem compression and death. 0espite the high morbidity and mortality rates# surgeons continued to improve upon previous results# and in 1.&1 4alter 0andy reported a mortality rate of only 10 using a debul*ing and capsular resection techni!ue (%). 'ost recently in the 1.205s 4illiam house advanced the Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas surgical techni!ue of resecting CPA tumors by introducing the microscope and dental drill# the translabyrinthine approach# and the middle cranial fossa approach (.+11). 'uch of what 3ouse accomplished is still considered standard of care today. Anatomy and Tumor Biology As stated previously# the CPA is an area densely pac*ed with neurologic tissue and any tumor growth can cause significant dysfunction. Cranial nerves 6+6))) are affected with more superiorly based tumors# whereas tumors that e"tend inferiorly can compress cranial nerves )7+ 7)). 8"tension to the cerebellum laterally can cause generali9ed ata"ia and even opto*inetic difficulties with compression on the flocculus. -rainstem compression leading to fourth ventricle obstruction can lead to hydrocephalus# respiratory depression# and death. 6estibular /chwannomas arise from /chwann cells within the )AC and arise from the /chwann cell rich 9one of /carpa5s ganglion (1). There is an e!ual fre!uency of vestibular nerve involvement between the superior and inferior segments# however involvement of the acoustic portion of the nerve is rare. 6estibular schwannoma formation is thought to be due to a defect in the tumor suppressor gene :;& found at &&!1&. The :; & gene prevents /chwann cell proliferation. The sporadic form of unilateral vestibular schwannomas represents .1 of cases (1&). )n this case there are two hits to the normal :; gene. Patients with :eurofibromatosis &# however# inherit one defective gene in an autosomal dominant manner# and then develop a genetic defect in the second normal allele. ;or this reason they are predisposed to developing bilateral vestibular schwannomas. /everal studies have loo*ed at the biochemical effects of different agents on the growth of vestibular schwannomas. :euregulin is a hormone e"pressed by /chwann cells to control proliferation and survival of /chwann cells (1). /everal chemo*ines such as ;<;# T<;+-# 68<;# and P0<; are all chemo*ines that have been evaluated as potential targets to prevent tumor growth (1=+12). /tudies in the past had previously shown increased growth of vestibular schwannomas during pregnancy but more recent studies have failed to show an increased growth rate dependent on se" hormones or their receptors (1$). Symptoms and Signs /ymptoms and signs are highly dependent on the epicenter of the tumor. )ntracanalicular tumors often present with hearing loss# tinnitus# or vertigo. Tumors e"tending into the CPA will li*ely have dise!uilibrium or ata"ia depending on the amount of e"tension on the brainstem. 4ith -rainstem e"tension midface hypesthesia# hydrocephalus# and other cranial neuropathies become more prevalent. 6estibular schwannomas most commonly present with sensorineural hearing loss# followed by tinnitus# dise!uilibrium and facial hypesthesia in decreasing order (1&). >nilateral hearing loss is present in greater than %1 of patients (1%) but 1 of patients with vestibular schwannoma will have no associated hearing loss (1.). )n most instances of retrocochlear losses# despite the sensorineural hearing loss# many patients will have speech discrimination scores proportionally worse than the observed hearing loss. Patients will therefore complain of difficulty when tal*ing on the telephone. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas /udden /:3? will be present in about &0 of patients with a vestibular schwannoma (1&)# but only 1 of patients with sudden /:3? have a vestibular schwannoma (&0). ;ifty percent of patients with vestibular schwannoma and sudden /:3? will have spontaneous recovery and therefore you cannot simply rule out a vestibular schwannoma if they recover. Tinnitus is the second most common presenting symptom and can present in a variety of ways. )t can be present without hearing loss# it can be described as a roar# a high pitch ring# or even hissing# and it can even locali9e to the opposite ear. Therefore any person with a complaint with unilateral hearing loss should at least be evaluated with an audiogram. 6estibular complaints are present in =2+10 of patients and will describe a vague or transient lightheadedness. They often will @ust have some dise!uilibrium. Acute vertigo is the presenting symptom in only about &$ of patients and is associated with smaller tumors (1). ;acial hypesthesia is the presenting symptom in only , of patients# is associated with larger tumors A&cm and commonly will affect the ma"illary division of cranial nerve 6 first. The corneal refle" is often the first symptom but involvement of the muscles of mastication can also occur. ;acial wea*ness is rare with vestibular schwannomas# and if present# should be grounds to assume there is a different type of tumor. Bcular complaints are rare and can range from simple loss of corneal refle"# to true ocular dysfunction. :ystagmus toward the affected side# diplopia from involvement of cranial nerve 6)# and blurry vision from hydrocephalus leading to paapilledema and optic atrophy. Physical e"am findings of an acoustic neuroma can include an absent corneal refle"# wea*ness of the temporalis or masseter muscles# hypesthesia to pinpric* and touch# nystagmus# and other cranial neuropathies. 3istelberger5s sign is present when the sensory portion of cranial nerve seven is absent but the motor portion is intact. The actual sign is hypesthesia of the e"ternal canal (1). ;inally you may identify gait disturbances or difficulties on finger to nose testing. Workup Audiologic testing is paramount when evaluating a patient with a suspected CPA tumor or vestibular schwannoma. A downsloping high fre!uency hearing loss is the most common finding on audiogram in about 21 of patients (&1). ;ive percent of all patients will have no hearing loss (&&). (ollover is a term used to represent retrocochlear hearing loss where patient5s speech discrimination is worse than e"pected based on pure tone averages that become worse as the sound is intensified. There are two classifications used to represent the different stages of hearing loss and can be seen in Table 1. 'any papers use these classifications to compare preoperative and postoperative hearing abilities for !ualitative purposes. 'ost papers recogni9e serviceable hearing as AAB+ 3:/ class A or - and <ardner+(obinson class 1 or &. 3earing classification is also very important when determining what surgical procedure a patient is !ualified for based on their preoperative hearing scores. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas AAO-HNS classifcation Class Pure tone average (0.5, 1, 2, 3 H! "easure# in #$ H%& S'eec( #iscri"ination score ()& A 0*30 +0*100 $ 31*50 50*100 C ,50 50*100 - An. /50 0ar#ner-1o2ertson Classifcation Class Pure 3one4S'eec( 1ece'tion 3(res(ol# (#$ H%& S'eec( -iscri"ination Score ()& 1 0*30 +0*100 2 31*50 50*56 3 51*60 5*76 7 ,60 1*7 Table 1. /howing different classification schema for hearing loss. )n addition to audiologic testing# acoustic refle"es can also be used to show a retrocochlear loss. Acoustic refle" testing has an %1 sensitivity for identifying abnormalities. An abnormal test will be an increased or absent acoustic refle" threshold compared to cochlear norms. This is not considered a reliable screening test. Auditory -rainstem 8vo*ed (esponse testing has a sensitivity of %1 to .0 with a false positive rate of only about 10. ;alse negative rates were much lower in the past but have increased to about 1%+=0 due to improvements of imaging techni!ues to find smaller tumors (&,). 0uring the test five waveforms are produced and a latency of A0.& milliseconds in wave 6 is diagnostic of a retrocochlear loss. )n patients with a low inde" of suspicion for a vestibular schwannoma# this test can be used for screening purposes. 6estibular testing or electroneurography will show some form of abnormality in $0+.0 of patients (&1) but 10 of small tumors will produce no abnormalities (&2). Abnormalities in caloric testing on the ipsilateral side is usually the only abnormal finding# however compression on the cerebellum and flocculus can lead to opto*inetic abnormalities. )nterestingly this test pic*s up .% of tumors involving the superior vestibular nerve but only 20 of patients with tumors involving the inferior vestibular nerve. This is because the superior nerve innervates the lateral semicircular canal which is tested with calorics where the inferior nerve is supplies the other two portions of the vestibule (&$). Therefore this cannot be used to screen for vestibular schwannomas. )maging is really the gold standard for identifying vestibular schwannomas and other CPA tumors. Bn CT imaging .0 of tumors will enhance with contrast but the diagnostic accuracy is only 2= (&%). CT scanning fre!uently misses tumors that are e"tracanalicular and tumors not e"tending A1mm to the CPA. '() imaging on the other hand is the gold standard for vestibular schwannomas. Tumors preferentially ta*e up gadolinium for visuali9ation of smaller tumors as small as =mm in si9e (&.). Contrasted studies will show a hyperintense signal on both T1 and T& images but non+contrasted studies will show hypointense lesions on T1 and isointense lesions on T&. /ome institutions have used T& fast spin+echo '()5s as a screening test in those with contrast allergies# but most patients with abnormalities will re!uire a formal study (=0). Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas -ecause meningiomas are the second most common type of CPA tumor it is important to be able to differentiate it5s characteristics from that of a vestibular schwannoma on '(). 6estibular schwannomas will have more of a globular appearance# centered on the internal auditory canal# and may resemble an ice cream cone. )n contrast# a meningioma will li*ely have more of a sessile appearance with a Cdural tailD at the periphery and will be hypointense on T1 images but hypearintense on T& images. Differential Diagnosis As stated previously# meningiomas are the second most common CPA tumor and represent about = of all CPA tumors (1&). They do not metastasi9e but do recur due to bony invasion. They are formed from cap cells around the arachnoid villi near dural sinus5 and where cranial nerves enter their foramina. <enerally these tumors are not intracanalicular and therefore must be much larger to produce hearing loss. /ymptoms and signs again depend on the location of the tumor. )ntracanalicular tumors usually present similar to vestibular schwannomas. Btherwise they often present with spontaneous nystagamus# facial hypesthesia and gait ata"ia. )f they e"tend inferiorly they can produce hoarseness# dysphagia# shoulder wea*ness and even tongue atrophy (=1). ?i*e vestibular schwannomas# hearing tests will show retrocochlear losses and the A-( will be normal in &1 of patients (=&). Treatment is surgical but you must remove a rim of normal tissue due to the bony invasion to prevent recurrence. 8pidermoid tumors are histologically the same as cholesteatomas and develop from epithelial rest cells. They are slow growing and often do not present until the second or third decade. These tumors will often follow the path of least resistance and therefore will often be irregularly shaped and !uite large before causing symptoms. ;acial twitching has been described as a common finding with these tumors and facial wea*ness is more common than with cranial nerve schwannomas. 4or*up is again consistent with retrocochlear losses and treatment is surgical e"cision. 8pidermoids can often resemble cysts on '() e"amination as they are hypointense on T1 images but are bright on T&. )n order to differentiate these lesions from more cystic lesions li*e an arachnoid cyst# the T1 weighted flair images can be helpful to show the more heterogeneous characteristics of an epidermoid compared to the homogenous loo* of a cyst. ;acial schwannomas are histologically identical to vestibular schwannomas. They are rarely restricted to the internal auditory canal# commonly have multiple s*ip lesions# and generally involve part of the geniculate ganglion. >ni!ue characteristics include aural fullness if distal to the geniculate ganglion and facial wea*ness is not usually present until these tumors are very large. 3earing and impedance testing shows a retrocochlear loss but in addition the stapedial refle" can be lost in these tumors. Treatment is observation until there is significant growth or grade ))) facial nerve dysfunction and nerve graft repair is commonly performed at the time of resection (1). <lomus tumors can present with @ugular foramen syndrome and involvement of cranial nerves )7+7). Treatment is surgery. 3emangiomas are usually centered on the geniculate and lead to a progressive facial wea*ness. Treatment is surgery with primary nerve graft repair. Arachnoid cysts can often be drained without large operations. Cholesterol granulomas are bright on both T1 and T& in contrast to cholesteatomas and can often be drained. 8mbryonic tumors and primary C:/ tumors are resected as appropriate. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas Management: !ser"ation Bnce a CPA tumor is found counseling is of the utmost importance. Bften times resection or treatment of these tumors# especially slow growing vestibular schwannomas can leave the patient with more dysfunction than they had prior to treatment. )t is with this idea in mind that the options of observation# radiation therapy# and surgery can be offered depending on the circumstances and patient preferences. )f patients opt for observation it is important to let them *now what to e"pect. Average growth rates for vestibular schwannomas are &mm per year with a range of 0 to &cm per year (==). A review of multiple studies have shown that about ,0 to %0 of tumors will show some growth by = years after diagnosis (&=) and 1, to &, of patients who are observed will go on to have some form of treatment (11). ?ongstanding hearing loss may represent a slow growing tumor and therefore may help guide treatment options (=,). Age should not be the only determining factor for which patients receive different treatment options. Eounger patients may be able to tolerate a large operation better than the elderly# older patients may outlive any symptoms of their surgery# but all patients regardless of age with life threatening symptoms should be offered some form of treatment. )f observation is chosen# '() should be repeated at least every 2 months if not every = months during the first year and then yearly after that to obtain proper growth surveillance. (anagement: Ra!iation6Stereotactic Ra!iosurger% <amma Fnife (<F) was first introduced by ?es*ell in 1.2. using a cobalt 20 source. )t utili9es &01 ioni9ing beams of radiation to attac* tumors from multiple directions. The ?):AC system uses a linear accelerator as its source of electrons# but has much fewer beams of radiation compared to the gamma *nife. -oth of these modalities re!uire only a single session for treatment. 'ore recently fractionated radiotherapy has been used to catch cells in different stages of the cell cycle for ma"imal effect. The drawbac* to fractionated therapy is that it re!uires multiple sessions for full treatment. >ltimately the goal of radiosurgery is to arrest tumor growth rather than to shrin* it or remove it. ?ocal control is defined as patients treated with radiation# not re!uiring salvage therapy. 4hen combining all three previously mentioned modalities %$ to 100 local control rates have been published. 0espite these numbers appro"imately &= of patients will develop a transient increase in tumor si9e for 2 months up to 1 years due to central necrosis of the tumor (&=). Therefore it is important to differentiate true tumor growth from transient swelling post therapy to prevent unnecessary salvage therapy. )n &001# 3asagawa et al. loo*ed at =1$ patients treated with <F therapy and $.% years of median follow up. 10 year local control rates were A.& and significantly better in patients with tumors G11cm=# with no brainstem compression# and no , th ventricle compression (=1). 'ost tumors that progressed did so within the first = years post+therapy. ;reidman et al. in &002 loo*ed at =.0 patients treated with ?):AC and a median dose of 1&.1<y. 'edian follow up was ,0 months. 1 and 10 year local control rates were .0 and only 1 of patients re!uired salvage therapy. 4ith fractionated therapy being so new# there is a wide variety of study treatments Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas ranging from 11 to 1$.2<y in = to =& fractions. 'edian follow up over multiple studies has been ,% months and the 1 year local control rate is A.0 (&=). 4hen comparing tumor control# tumor growth# and hearing preservation between fractionated therapy and radiosurgery (<F or ?):AC)# fractionated therapy has shown better ranges loo*ing at multiple studies. Tumor shrin*age however is similar between the two modalities (=$). )nitially patients were being treated with 12<y doses with hearing preservation of only ,2. ?ower doses were instituted at 1&+1=<y# which led to hearing preservation of 2% to $% without change in local control rates (&=). A study loo*ing at 11= patients followed for a median of ,.& years showed that useful hearing was preserved in 1% of patients but that tumors G1cm= had significantly more patients with preserved hearing compared to those with tumors A1cm= of $1 vs 1$ respectively. 4hen comparing radiosurgery to fractionated therapy# one study in &001 was able to show serviceable hearing preservation at = years follow up of %1 versus ==# <F versus fractionated therapy respectively (=.). ;acial and trigeminal neuropathy showed similar results compared to hearing preservation. As radiation dose was decreased from 12<y to 1&+ 1=<y complications decreased (=2# ,0)# and fractionated therapy showed better results than radiosurgery for both complications (=$). Bther complications observed with radiation therapy included hydrocephalus (0+11)# tinnitus (0.&+1)# ata"ia (1.,+=.2)# vertigo (1.,+1.$)# malignant transformation (0+0.=)# and dise!uilibrium (==)(,1). (anagement: Surger% 4hen it comes to surgery there are three different options including the translabyrinthine approach (T?)# the middle cranial fossa approach ('C;)# and the retrosigmoidHsuboccipital approach (/). All surgical options re!uire discussion and collaboration with neurosurgery. The T? approach has several advantages. )t provides an option for resecting a tumor of any si9e# it has e"cellent e"posure of the posterior fossa# it utili9es the least cerebellar retraction and the facial nerve is easily identified throughout the case. The ma@or disadvantage is the fact that any residual hearing is sacrificed through this approach (,&). Control rates with total resection range from ...1+...$ (,=+,,). :ear total resection has been described as G&1mm& or &mm thic* tumor left in the operative field. ;ifty percent of lesions can be visuali9ed on '() with near total resection and = of patients will have a recurrence (,1+,2). The 'C; approach is one of the approaches that allow hearing preservation. Additionally it provides good e"posure of the lateral internal acoustic meatus# CPA and clivus# and drilling is e"tradural thereby decreasing morbidity. 0isadvantages include si9e limits of tumors G&cm in greatest dimension# e"tensive temporal lobe retraction# limited e"posure of the posterior fossa# and re!uired facial nerve dissection to access tumors (,&). Control rates for this techni!ue are .% (,$). The (/ approach is another hearing preservation procedure and li*e the T? approach# a tumor of any si9e can be reached as long as there is less than &cm of e"tension into the )AC. Additionally there is wide e"posure of the brainstem and lower cranial nerves with consistent Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas identification of the facial nerve throughout the procedure. 0isadvantages include increased ris* of in@ury to the endolymphatic sac and vestibular labyrinth with lateral tumors# cerebellar retraction leading to occulomotor and dise!uilibrium difficulties# and increased ris* of air embolism with older positioning techni!ues (,&). Control rates using total resection techni!ues are .1 with this approach (,%). /erviceable hearing can be preserved in appro"imately 11 and =1 'C; vs (/ approaches respectively (&=). 'eyer et al in &002 loo*ed at 12& consecutive patients treated with 'C; approach and showed ,1 AAB+3:/ class AH- hearing and that 10 of patients with word recognition scores greater than $0 preoperatively maintained that level (,.). They also showed that as tumor si9e increased serviceable hearing decreased in an e"pected manner. ;acial nerve function for microsurgical treatment is best reported at 2 months or 1& months as the gold standard. Patients treated with the (/ approach had the best facial nerve function (grade )H))) followed by the 'C; and T? approach respectively (.1 vs %% vs $$) (&=). 0elayed paralysis has been described where patients have normal postoperative facial nerve function and develop paralysis A$& hours after surgery. The incidence is appro"imately 1 and $. of patients will regain normal function by 1 year follow up (10). C/; lea* is more common with patients treated with (/ (11) approach compared to 'C; (2) and T? (%) approaches and the overall rate is considered to be about % (11). Postoperative headache of over = months occurs in appro"imately 10 of patients with (/ (&1) approach having the highest incidence compared to 'C; (%) and T? (=) approaches (11). The most li*ely reason is due to intradural drilling where bone dust contacts the meninges and C/; leading to aseptic meningitis. Bther microsurgical complications include a mortality of 1 usually due to neurovascular in@ury. 'eningitis occurs in 1+% of patients with aseptic meningitis being the most common. This can be simply treated with steroids. -acterial meningitis re!uires antibiotics and is most commonly caused by #$ aureus. Tinnitus occurs in about 10 of patients but 10 of patients with preoperative tinnitus will have resolution postoperatively (1&). /ei9ure# hydrocephalus# and stro*e represent G& of cases and are rarely encountered (&=). Microsurgery "ersus #adiosurgery /everal studies have compared radiosurgery to microsurgery for treatment of vestibular schwannomas. )n a retrospective review by 'yrseth et al in &001 with median follow up of 1.. years# local control rates between microsurgery and gamma *nife were not statistically different (%..& versus .,.&) (1&). ;acial nerve function and !uality of life were both significantly worse in the surgery group versus the gamma *nife group. <amma *nife boasted a facial nerve preservation rate of .,.& while surgery only showed $..% 3- grade )H)). To give further clout to 'yrseth5s study# Polloc* et al. in &002 performed a prospective cohort of %& patients treated with surgery versus gamma *nife in %& patients with tumors G=cm in si9e. 'edian follow up was ,& months and local control rates were not significantly different between the groups (.2 versus 100). 0espite these findings# facial nerve functionHhearing Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas preservation were both significantly worse in those treated with surgery versus those treated with gamma *nife ($1H1 versus .2H2=) (1=). Conclusions Any patient with unilateral sensorineural hearing loss or tinnitus must be evaluated for possible CPA tumor. Although the differential diagnosis for CPA tumors is !uite large# the vast ma@ority are vestibular schwannomas. 6estibular schwannomas less than =cm in si9e can be treated with either <F or ?):AC radiosurgery# however fractionated radiation is gaining promise and may become the standard of care. )f a surgical approach is re!uired and the patient has no serviceable hearing the T? approach is the best option but after that either the (/ or 'C; approach can be utili9ed for hearing preservation depending on surgeon preferences. The most important thing to remember when counseling a patient regarding therapy is that treatment of these lesions can often lead to an increase in symptoms or side effects compared to their baseline function. Therefore a candid informed consent is re!uired prior to any therapy of these lesions. DISC$SSA%T: Tomoko Makis&ima' MD' P&D on Cere!ellopontine Angle Tumors (it& )ocus on *esti!ular Sc&(annomas This was a nicely prepared review of CP angle tumors. There was emphasis on management options, especially regarding newer radiotherapy technology. ost cases will undergo conservative management and observation. !owever, for those cases that do undergo surgery, " would li#e to further emphasi$e the importance of pre%operative evaluation and consent with the patients. "t is very important to e&plain and ma#e sure the patient understands e&actly what to e&pect after surgery. any patients have the e&pectation that their symptoms will get better after surgery, or don't e&pect it to get worse. (specially, the postoperative outcome regarding di$$iness is often unpredictable. "t is important to #eep in mind that the tumor itself is non%life threatening, and that by performing surgery, you may be causing the patient to end up with more symptoms than before surgery. And, regarding infections such as cholesterol granulomas, cholesteatomas, and dermoid cysts ) the gold standard of treatment is to remove the infection, or obtain a *drainage* pathway. !owever, given the di+culty reaching the disease, sometimes this will not be feasible, and will result in multiple surgeries. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas #eferences: 1. -ailey -I# Iohnson# IT# :ewlands /0. 3ead and :ec* /urgery J Btolaryngology# ;ourth 8dition. PhiladelphiaK ?ippincott# &002. pp. $1.+20# %==+,# .0. &. 3ardy '# Crowe /I. 8arly asymptomatic acoustic tumorK report of 2 cases. Arch /urg 1.=2L=&K&.&J=01. =. 8c*ermeier ?/# /orenson <0# 'c<avran '3. 3istopathology of =0 non+operated acoustic schwannomas. Arch Btorhinolaryngol 1.$.L&&&K1. ,. Anderson T0# ?oevner ?A# -igelow 0C# et al. Prevalence of unsuspected acoustic neuroma found by magnetic resonance imaging. Btolaryngol 3ead :ec* /urg &000L1&&K2,=J2,2. 1. 3ouse 4;. A history of acoustic tumor surgeryK 1%00J1.00# early history. )nK 3ouse 4;# ?uet@e C'# eds. Acoustic tumors 6ol. 1. -altimoreK >niversity Par* Press# 1.$.K=J%. 2. (osenberg /). :atural history of acoustic neuromas. ?aryngoscope &000L110K,.$J10%. $. Cushing 3. Tumors of the nervus acusticus and the syndrome of the cerebellopontine angle ((eprint of the 1.1$ edition.) :ew Eor*K 3afner# 1.2=. %. 0andy 48. An operation for the total removal of cerebellopontine (acoustic) tumors. /urg <ynecol Bbstet 1.&1L,1K1&.J1,%. .. 3ouse 4;. (eport of casesK monographtranstemporal bone microsurgical removal of acoustic neuromas. Arch Btolaryngol 1.2,L%0K21$J22$. 10. <lasscoc* '8 )))# /teenerson (?. A history of acoustic tumor surgeryK 1.21Jpresent. )nK 3ouse 4;# ?uet@e C'# eds. Acoustic tumors 6ol. 1. -altimoreK >niversity Par* Press# 1.$.K==J,1. 11. 3ouse 4;. 8"posure of the internal auditory canal and its contents through the middle cranial fossa. ?aryngoscope 1.21L$1K1=2=J1=%1. 1&. CummingsK BtolaryngologyK 3ead M :ec* /urgery# ,th ed. 1=. 'urphy P(# 'yal E# /ato E# et al. 8levated e"pression of basic fibroblast growth factor messenger ribonucleic acid in acoustic neuromas. 'ol 8ndocrinol 1.%.L=K&&1J&=1. 1,. 0iensthuber '# -randis A# ?enar9 T# et al. Co+e"pression of transforming growth factor+ N1 and glial cell line derived neurotrophic factor in vestibular schwannomas. Btol :eurotol &00,L&1K=1.J=21. 11. CayO+Thomasen P# -aandrup ?# Iacobsen <F# et al. )mmunohistochemical demonstration of vascular endothelial growth factor in vestibular schwannomas correlates to tumor growth rate. ?aryngoscope &00=L11=K&1&.J&1=,. 12. -rieger I# -edavani@a A# ?ehr 3# et al. 8"pression of angiogenic growth factors in acoustic neuroma. Acta Btolaryngol &00=L1&=K 10,0J10,1. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas 1$. -eatty C4# /cheithauer -4# Fat9mann IA# et al. Acoustic schwannoma and pregnancyK a 0:A flow cytometric# steroid hormone receptor# and proliferation mar*er study. ?aryngoscope 1..1L101K2.=J$00. 1%. /elesnic* /3# Iac*ler (F# Pitts ?4. The changing clinical presentation of acoustic tumors in the '() era. ?aryngoscope 1..=L10=K,=1J,=2. 1.. /ataloff (T# 0avies -# 'yers 0?K Acoustic neuromas presenting as sudden deafness. (m ) Otol 1.%1L 2K=,.. &0. ;riedman (A# Fesser -4# /lattery 43# et al. 3earing preservation in patients with vestibular schwannomas with sudden sensorineural hearing loss. Btolaryngol 3ead :ec* /urg &001L1&1K1,,J111. &1. Iohnson 84. (esults of auditory tests in acoustic tumor patients. )nK 3ouse 4;# ?uet@e C'# eds. Acoustic tumors 6ol. 1. -altimoreK >niversity Par* Press# 1.$.K&0.J&&,. &&. -eatty C4# 8bersold 'I# 3arner /<K (esidual and recurrent acoustic neuromas. *aryngoscope 1.%$L .$K112%. &=. -ac*ous 00# Pham 3TK <uiding patients through the choices for treating vestibular schwannomasK -alancing options and ensuring informed consent. Otolaryngol +lin ! (m &00$L ,0K 1&1+1,0. &,. 4ilson 0;# 3odgson (/# <ustafson ';# et al. The sensitivity of auditory brainstem response testing in small acoustic neuromas. ?aryngoscope 1..&L10&K.21J.2,. &1. Ien*ins 3A. ?ong+term adaptive changes of the vestibulo+ocular refle" in patients following acoustic neuroma surgery. ?aryngoscope 1.%1L.1K1&&,J1&=, &2. :ed9els*i I'# /chessel 0A# Pfleiderer A# et al. Conservative management of acoustic neuromas. Btolaryngol Clin :orth Am 1..&L&1K2.1J$01. &$. ?inthicum ;3. 8lectronystagmography findings in patients with acoustic tumors. /emin 3ear 1.%=L,K,$J1=. &%. 4elling 0-# <lasscoc* '8 )))# 4oods C)# et al. Acoustic neuromaK a cost+effective approach. Btolaryngol 3ead :ec* /urg 1..0L10=K=2,J=$0. &.. Press <A# 3esselin* I(K '( imaging of cerebellopontine angle and internal auditory canal lesions at 1.1T. (), (m ) ,oentgenol 1.%%L 110K1=$1. =0. /helton C# 3arnsberger 3(# Allen (# et al. ;ast spin echo magnetic resonance imagingK clinical application in screening for acoustic neuroma. Btolaryngol 3ead :ec* /urg 1..2L11,K$1J$2. =1. <ranic* '/# 'artu9a (?# Par*er /4# et al. Cerebellopontine angle meningiomasK clinical manifestations and diagnosis. Ann Btol (hinol ?aryngol 1.%1L.,K=,J=%. =&. /elters 4A# -rac*mann 08. Acoustic tumor detection with brain stem electric response audiometry. Arch Btolaryngol 1.$$L10=K1%1J1%$. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas ==. 3ouse 4;K Translabyrinthine approach. )nK 3ouse 4;# ?eut@e C'# 0oyle FI# ed. (coustic Tumors# /an 0iegoK /ingular PublishingL 1..$K1$1+1$2. =,. Charabi /# Thomsen I# 'antoni '# et al. Acoustic neuroma (vestibular schwannoma)K growth and surgical and nonsurgical conse!uences of the wait+and+see policy. Btolaryngol 3ead :ec* /urg 1..1L11=K1J1,. =1. 3asegawa T.# ;u@utani /.# Fatsumata /.# et alK /tereotactic radiosurgery for vestibular schwannomasK analysis of =1$ patients followed more than 1 years. !eurosurgery 1$. &1$+&21.&001 =2. ;riedman 4.A.# -radshaw P.# 'yers A.# et alK ?inear accelerator radiosurgery for vestibular schwannomas. ) !eurosurg 101. 21$+221.&002. =$. ?i*terov )# Allbright ('# /elesnic* /3K ?):AC radiosurgery and radiotherapy treatment of acoustic neuroma. Otolaryngol +lin ! (m &00$L ,0K 1,1+1$0. =%. Prasad 0.# /teiner '.# /teiner ?.K <amma surgery for vestibular schwannoma. ) !eurosurg .&. $,1+$1..&000. =.. Andrews 0.4.# /uare9 B.# <oldman 4.# et alK /tereotactic radiosurgery and fractionated stereotactic radiotherapy for the treatment of acoustic schwannomasK comparative observations of 1&1 patients treated at one institution. %nt ) ,adiat Oncol Biol Phys 10. (1)K 1&21+1&$%.&001. ,0. Fond9iol*a 0.# ?unsford 0.# 'c?aughlin # et alK ?ong+term outcomes after radiosurgery for acoustic neuromas. ! -ngl ) Med 1..%# ==.K 1,&2+1,==. ,1. (oland P/# 8ston 0. /tereotactic radiosurgery for acoustic tumors. Btolaryngol Clin :orth Am &00&L=1K=,=J=11. ,&. -ennett '# 3aynes 0/K /urgical approaches and complications in the removal of vestibular schwannomas. Otolaryngol +lin ! (m &00$L ,0K 1%.+20.. ,=. /helton C. >nilateral acoustic tumorsK how often do theyrecur after translabyrinthine removalP ?aryngoscope 1..1L101K.1%J.22. ,,. Thedinger -A# <lasscoc* '8 )))# Cueva (A# et al. Postoperative radiographic evaluation after acoustic neuroma and glomus @ugulare tumor removal. ?aryngoscope 1..&L10&K&21J&22. ,1. Pace+-al9an A# ?ey (3# (amsden (T# et al. <rowth characteristics of acoustic neuromas with particular reference to the fate of capsule fragments remaining after tumor removalK implications for patient management. )nK Tos '# Thomsen I# eds. Proceedings of the first international conference on acoustic neuroma AmsterdamKFugler. 1..&K$01J$0=. ,2. -loch 0# Bghalai I/# Iac*ler (F# et al. The role of less than complete resection of acoustic neuroma. Btolaryngol 3ead :ec* /urg &00,L1=0K10,J11&. Cerebellopontine Angle Tumors with Focus on Vestibular Schwannomas ,$. ;riedman (A# Fesser -# -rac*mann 08# et al. ?ong+term hearing preservation after middle fossa removal of vestibular schwannoma. Btolaryngol 3ead :ec* /urg &00=L1&.K220J221. ... ,%. 8bersold 'I# 3arner /<# -eatty C4# et al. Current results of the retrosigmoid approach to acoustic neurinoma. I :eurosurg 1..&L$2K.01J.0.. ,.. 'eyer T.A.# Canty P.A.# 4il*inson 8.P.# et alK /mall acoustic neuromasK surgical outcomes versus observation or radiation. Otol !eurotol &002# &$(=)K =%0+=.&. 10. <rant <A# (ostomily ((# Fim F# et al. 0elayed facial palsy after resection of vestibular schwannoma. I :eurosurg &00&L.$K.=J.2. 11. 3arsha 4.I.# -ac*ous 0.0.K Counseling patients on surgical options for treating acoustic neuroma. Otolaryngol +lin !orth (m &001# =%(,)K 2,=+21&. 1&. 'yrseth 8.# 'oller P.# Pederson P.3.# et alK 6estibular schwannomasK clinical results of !uality of life after microsurgery or gamma *nife radiosurgery. !eurosurgery &001# 12(1)K .&$+.=1. 1=. Polloc* -.8.# 0iscoll C.?.4.# ;oote (.?.# et alK Patient outcomes after vestibular schwannoma managementK a prospective comparison of microsurgical resection and stereotactic radiosurgery. !eurosurgery &002# 1.(1)K $$+%1. QQQ