artykuł oryginalny / original research article

Vertigo in cerebellopontine angle tumor Patients Zaburzenia równowagi u pacjentów z guzem kąta mostowo-móżdżkowego

Paulina Zarębska-Karpieszuk, Robert Bartoszewicz, Katarzyna Pierchała, Kazimierz Niemczyk Department of Otolaryngology, Medical University of Warsaw Head of the department: Prof. Kazimierz Niemczyk

Article history: Received: 10.10.2015 Accepted: 26.11.2015 Published: 15.12.2015

ABSTRACT: Cerebellopontine angle tumors are a group of tumors that originate from the posterior cranial fossa. Most of them are acoustic neuromas arising from the Schwann cell sheath of the vestibular branch of VIII cranial nerve. Those benign tumors usually present with unilateral loss (53–57%), tinnitus (79–82%), and /imbalance (18–50%). The objective of this study was documentation-based evaluation of 53 out of 96 patients that received surgical treat- ment in the ENT Department of Medical University in Warsaw during the period from May 2012 to May 2014, with spe- cial consideration of vertigo/imbalance in relation to other symptoms. Statictical analysis has shown larger frequency of vertigo/imbalance in patients with mild to moderate hearing loss and positive corelation between the largest diameter of the tumor and the vestibular deficit in VNG (videonystag- mography). The corelation of vestibular deficit in VNG and imbalance symptoms was only estabilished in the group with intrameatal localization of the tumor (T1). KEYWORDS: acoustic neuroma, vestibular schwannoma, vertigo, imbalance; cerebellopontine angle tumor; symptoms

STRESZCZENIE: Guzy kąta mostowo-móżdżkowego to wspólna nazwa szeregu nowotworów lokalizujących się w tylnym dole czaszki pomiędzy tylną powierzchnią piramidy kości skroniowej (z uwzględnieniem przewodu słuchowego wewnętrznego) a brzuszną powierzchnią mostu oraz dolną powierzchnią móżdżku. Najczęstsze zmiany lokalizujące się w tej okolicy to osłoniaki części przedsionkowej n. VIII (Vestibular Schwannoma). Te łagodne, rzadkie w strukturze guzy wywołują zespół kąta mostowo-móżdżkowego. Ich charakterystycznymi objawami są: postępujący jednostronny niedosłuch odbiorczy (53–57%), szum uszny (79–82%), zawroty głowy (18–50%). Celem pracy było opracowanie profilu pacjentów z guzem kąta mostowo-móżdżkowego zakwalifikowanych do lec- zenia operacyjnego w Klinice Otolaryngologii WUM, a także analiza występowania w badanej grupie zaburzeń równowagi w odniesieniu do innych objawów. Dane pozyskano na podstawie retrospektywnego opracowania 53 historii chorób 96 pacjentów operowanych od maja 2012 r. do maja 2014 r. Analizie w programie Statistica poddano: maksymalny wymiar guza w osi PSZ, zaawansowanie guza w skali Koos’a i Perneczky’ego, ubytek słuchu według skali Gardnera-Robertsona, uszkodzenie narządu przed- sionkowego (na podstawie badania VNG), oraz występowanie zawrotów głowy w wywiadzie. Analizując wyniki, stwierdzono wyższą częstotliwość występowania zawrotów głowy u pacjentów z dobrze zachow- anym słuchem, a także dodatnią korelację pomiędzy wymiarem guza a patologicznym wynikiem VNG. Występowanie zaburzeń równowagi w wywiadzie współistniało z patologicznym wynikiem VNG tylko w grupie pacjentów z guzami wewnątrzprzewodowymi w stadium zaawansowania T1 wg Koos’a i Perneczky’ego. SŁOWA KLUCZOWE: nerwiak nerwu słuchowego, guzy kąta mostowo-móżdżkowego, zaburzenia równowagi, zawroty głowy, objawy guza kąta mostowo-móżdżkowego

POLSKI PRZEGLĄD OTORYNOLARYNGOLOGICZNY, TOM 4, NR 4 (2015), s. 27-31 DOI: 10.5604/20845308.1184568 27 artykuł oryginalny / original research article

INTRODUCTION

Cerebellopontine angle tumor is a common name for the who- le group of neoplasms located in the posterior cranial fossa between the rear surface of the petrous part of the tempo- ral bone, including the internal auditory meatus, and ventral surface of the pons and lower surface of the cerebellum. This region is the starting point for from VII to XII [1]. The greatest significance for consideration of pathology in the abovementioned region has facial nerve (cranial nerve VII) running with intermediate nerve and vestibulocochlear nerve (cranial nerve VIII) located laterally to it. From the star- ting point below the middle cerebellar peduncle and above the upper margin of the olive, a functional division of the cranial nerve VIII into its vestibular part starting with the upper root and cochlear part containing fibres from the lower root appro- Fig. 1. Distribution of patients with (1) and without (0) vertigo in relation to aching the inferior cerebellar peduncle is visible. The vestibular hearing impairment according to the Gardner-Robertson scale (AT- part is then divided into an upper branch (ramus superior) le- average tone) ading afferent fibers from the utriclenervus ( utriculo-ampul- laris) and a lower branch (ramus inferior) leading fibers from headaches, vomiting and disturbances of consciousness. These the saccular macula (nervus sacculo-ampullaris). The cochlear are directly related to oppression or indirectly to an increase part is the same as the (nervus cochleae) and is in intracranial pressure. the most prone to damage nervous structure passing through the internal auditory meatus [2]. For the past few years a gradual shift of approach to treatment of cerebellopontine angle tumors has been observed. According Most common changes located in the cerebellopontine angle are to research by Stangerup and Caye-Thomasen [3] from 2012 schwannomas of the vestibular part of cranial nerve VIII. They for the past 40 years almost a threefold increase in cases dia- constitute more than 80% of cerebellopontine angle tumors and gnosed annually has been reported. It results from the develop- about 6% of all intracranial tumors. These are rare and benign ment of diagnostic methods including MRI as a gold standard tumors developing from Schwann’s myelin sheath, most often in diagnosing posterior fossa tumors, more widespread specia- vestibular part of the vestibulocochlear nerve, occasionally fa- lized healthcare facilities and increasing social awareness. At cial or cochlear nerve and the incidence rate is approximately the same time the mean size of diagnosed tumors decreased 1-10 cases per 1 000 000 population/year [3-5]. A characteristic from about 30 mm to 10 mm [3]. This tendency, combined place of their origin is so called Redlich-Obersteiner’s zone that with therapeutic decisions aimed not only at radical removal is the most medially located sections of the nerve covered in the of the illness but also at preservation of possibly high quality Schwann’s sheath where it comes into contact with glial frame- of life (QoL) motivates further teams to determine factors in- work protecting nerve fibers {Janczewski, 2007, Otolaryngolo- fluencing the rate of growth of newly-discovered tumors and gia Praktyczna}[4, 6]. Schwannomas are in prevalent proportion facilitating the choice of the optimal therapeutic option [9]. isolated (sporadic) and discovered in the fifth and sixth decade of life. Multiple, bilateral tumors coexisting with meningioma The best-studied parameter so far which may suggest refra- are correlated with genetically caused neurofibromatosis type ining from observation of small tumors (< 15-20 mm) and 2 and due to an early manifestation of symptoms they are typi- attempting radiotherapy or removal by microsurgery may be cally diagnosed between the second and third decade of life [7]. their growth rate within the first year of diagnosis. Yet this is not a sufficient factor to influence the decision, which is -sup Irrespective of the structure from which a given tumor origi- ported by great discrepancies between results provided by va- nated, the direction and rate of its growth determine charac- rious authors. Even though majority of researchers agree that teristic clinical manifestation which includes: progressive uni- a mean annual growth rate of tumor is 0.9-1.9 mm in the axis lateral sensorineural hearing loss (53-57%), tinnitus (79-82%), of the internal auditory meatus [9, 10], research by Stangerup vertigo (18-50%) and much less often facial or trigeminal nerve and Caye-Thomasen cited above revealed that within the first dysfunction [8]. Large tumors oppressing the brainstem and year of observation more than 60% of tumors increased their IV ventricle cause typically neurosurgical symptoms such as size by 10.3 mm on average. It did not necessarily advocate for

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Medical University of Warsaw between 05.2012 and 05.2014 was analyzed. A selected group of 53 patients underwent the final analysis: 31 females and 22 males aged 20 to 74 years (mean 47.3). Comparisons were made based on: sex, age, tumor size, stage in the Koos-Perneczky’s scale, hearing loss in the Gard- ner-Robertson scale, presence of facial nerve paralysis, history of vertigo, and vestibular deficit in VNG testing.

RESULTS

A mean tumor size was 15.78 mm (largest 40 mm, smallest 4.6 mm). According to Koos-Perneczky’s scale a tumor stage of T1 was found in 13 cases (24.5%), T2 stage in 25 (47%), T3 stage in 11 (21%) and T4 (7.5%) in 4. Fig. 2. Patients with (1) and without (0) vertigo in relation to tumor staging according to the Koss and Perneczky’s scale (t1-t4) Patients’ hearing directly before the operation was evaluated with the use of the Gardner-Robertson scale: group 1 (hearing ability within 0-30 dB, speech discrimination score >70%) inc- a need of surgical intervention because in the fourth year of luded 22 (41.5%) patients; group 2 (hearing ability within 31-50 observation the mean increase in tumor size was just 0.9 mm dB, speech discrimination score 50-69%) 12 and group 3 (hearing and after five years no growth of observed tumors was noted ability within 51-90 dB, speech discrimination score 5-49%) also [3]. Moreover, basing the decision upon audiological symp- 12 (22.6%) patients; group 4 (hearing ability within 91-100 dB, toms is unreliable. Although the hearing loss increases with speech discrimination score 1-4%) included 3 (5.6%) patients the duration of symptoms and a positive correlation between and in the group of patients with total deafness there were 4 the size of tumor and the occurrence of hearing loss was pro- (7.5%) persons. Significant hearing loss (groups 2-5 according ven, cases of significant hearing loss in patients with a small, to Gardner-Robertson) was found in 58.5% of individuals qu- intrameatal schwannoma as well as relatively well-preserved alified for an operation. In 7 (13.2%) patients a sudden senso- hearing ability T2 an T3 tumors are also very common [9, 11]. ry hearing loss was experienced as the first symptom of illness.

In order to improve the accuracy of prognosis in patients with In the examined sample 27 (51%) patients declared at least one cerebellopontine tumors, vestibular symptoms have become episode of vertigo in the period preceding the diagnosis and more and more often analyzed as they not only correlate with up to 69.8% were diagnosed with a loss of vestibular function the size of the tumor [11] but also influence patients’ quality ipsilateral to tumor location in VNG. of life in a significant way [12]. In two cases a loss of function of the facial nerve was discove- red at time of diagnosis. In one of those cases (palsy of 4/6 in AIM House-Brackmann scale) a tumor originating from the geni- culum of the facial nerve was revealed intraoperatively – it was The aim of this report was to provide clinical characteristics of a patient suffering from neurofibromatosis type 2. The second patients suffering from cerebellopontine angle tumor treated patient presented with a slight paresis in the lower branch of at the Department of Otolaryngology of Medical University cranial nerve VII (2/6 in House-Brackmann scale). (Table 1) of Warsaw with a particular emphasis on manifestations of vertigo and their connection to other symptoms in a group of In 26 (49%) cases an approach through the labyrinth was used participants qualified for surgical treatment. and in 27 (51%) cases the middle fossa approach. In 51 (96%) patients histological examination confirmed the diagnosis of schwannoma, and in the remaining two meningioma and lipo- MATERIAL AND METHODS ma of the internal auditory meatus were recognized.

Medical documentation of 96 cerebellopontine angle tumor The obtained data were processed in Statistica software. Mann- patients operated on at the Department of Otolaryngology of -Whitney U test confirmed more frequent (p=0.036) occurren-

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Tab. I. Symptoms in patients in relation to tumor staging according to the Koss and Perneczky’s scale (T1-T4) GARDNER-ROBERTSON SCALE VESTIBULAR N VERTIGO 1 2 3 4 5 DEFICIT T1 13 7 7 7 2 3 0 1 T2 25 13 17 11 3 7 2 2 T3 11 6 9 3 6 1 1 0 & Perneczky

Stage acc. to Koos Koos to acc. Stage T4 4 1 4 1 1 1 0 1

ces of vertigo in patients with useful hearing (groups 1 and 2 on gradually compensated during slow ingrowth of the tumor into Gardner-Robertson scale). There was also a marked tendency the cerebellopontine angle, without further clinical manifesta- to vertigo in patients with a tumor of lower stage according tion in a majority of cases. to Koos-Perneczky’s scale. This tendency was not statistically significant though. (Figure 1) (Figure 2). A research in the Norwegian population proved that the oc- currence of vertigo was an important predictor of future disa- In the examined sample no differences in the incidence of vertigo bility in a group of patients diagnosed with vestibular schwan- according to sex, age, pathology in VNG or absolute tumor size nomas [13], yet it is not clear whether it is connected with a were discovered. Next a search for correlations using Spearman’s potentially greater size of a growing tumor which increases the model was performed. In analysis without division into groups vestibular deficit, with its disadvantageous location in relation a weak positive correlation between absolute tumor size and to the or a lack of compensatory capacity in pathological VNG results was found as well as a weak negative a particular patient. correlation between the extent of hearing loss (on Gardner-Ro- bertson scale) and vertigo in the examined group was discovered. The significance of understanding the abovementioned issues Interestingly, a correlation between abnormal VNG results and for optimal patient management may be supported by descrip- symptoms of vestibular dysfunction was revealed just in a sub- tion in professional literature of attempts at controlled dama- group of patients with small intrameatal tumors (stage T1). No ge of the vestibule of the in patients before a sudden loss relationship between abnormal VNG results and incidence of of vestibular function related to surgery on the cerebellopon- vertigo in history in any other analyzed group was revealed. On tine angle. Exercises and progressive damage of the labyrinth the other hand, a positive correlation between abnormal VNG with transtympanic gentamicin infusions are a special sort of result and absolute size and stage of the tumor exists. preoperative rehabilitation which is conducted to enable re- creation of the best (according to the most actual knowledge) conditions for future compensation of the balance system in DISCUSSION every patient [14-17]. These data support more and more so- phisticated otoneurological profiling of patients diagnosed Patients qualified to removal of cerebellopontine angle tumor with cerebellopontine tumors in hope for singling out tho- at the Department of Otolaryngology of Medical University se for whom surgical intervention would be most beneficial. of Warsaw do not differ significantly as far as sex, the extent of hearing impairment, incidence rate of vertigo or sudden it is also worth mentioning that a lot of important information hearing loss in history from samples described in the cited would be provided by enriching data used in the analysis above studies. Younger age at diagnosis is worth noting (mean of 47 with the results of posturographic examinations and VEMP to years compared to 57-60) [9-11]. Statistical analysis showed make the physical examination more objective and to assess that symptoms due to vestibular damage occur most often in the function of the lower vestibular nerve [18-20]. patients with small, intrameatal tumors. Research also reve- aled a statistically significant correlation between clinically manifested vestibular disorders and the rate of tumor growth CONCLUSIONS [11]. Such a clinical presentation combined with the fact that the number of abnormal VNG results increases with tumor Symptoms of imbalance in patients diagnosed with pathology advancement suggests that vestibular deficit develops at in- of the cerebellopontine angle constitute an interesting and to- itial stages of tumor development due to its compression on pical issue. A more profound understanding of relationships nervous structures in the internal auditory meatus. It is then and mechanisms responsible for the development and com-

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pensation of damage of the vestibular part of cranial nerve VIII ublesome symptoms for the patient. Due to the development may enable to predict the rate of tumor growth in the future. of modern, based upon knowledge of pathophysiology of ve- It may influence the evolution of strategies of preparing the stibular nerve damage, rehabilitation techniques there would patient to surgical treatment by a planned decrease in vesti- be a chance for a significant increase in the quality of life in bular excitability which would enable control over most tro- the described group of patients.

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Corresponding author: Paulina Zarębska-Karpieszuk, [email protected], Department of Otolaryngology, Medical University of Warsaw, 1a Banacha Street, 02-097 Warsaw, phone: +48 (22) 5991021, fax 22 599 25 23

Copyright © 2015 Polish Society of Otorhinolaryngologists Head and Neck Surgeons. Published by Index Copernicus Sp. z o.o. All rights reserved

Competing interests: The authors declare that they have no competing interests.

Cite this article as: Zarębska-Karpieszuk P., Bartoszewicz R., Pierchała K., Niemczyk K.: Vertigo in cerebellopontine angle tumor Patients. Pol Otorhino Rev 2015; 4(4): 27-31

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