J Neurol Surg B Skull Base 2021; 82(06): 643-651
DOI: 10.1055/s-0040-1715606
Original Article

Cochlear Implantation in Vestibular Schwannoma: A Systematic Literature Review

Kent Tadokoro
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
,
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
,
Nadeem El-Kouri
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
,
Dennis Moore
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
,
Christopher Britt
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
,
Matthew Kircher
1   Department of Otolaryngology, Loyola University Medical Center, Maywood, Illinois, United States
› Author Affiliations
 

Abstract

Objective Ipsilateral cochlear implantation (CI) in vestibular schwannoma (VS) has been an emerging trend over the last two decades. We conducted the first systematic review of hearing outcomes comparing neurofibromatosis 2 (NF2) and sporadic VS undergoing CI. A comparison of the two populations and predictor of outcome was assessed. This is an update to a previously presented study.

Data Sources Systemic data searches were performed in PubMed NCBI and Scopus by an academic librarian. No restrictions based on the year of publication were used.

Study Selection Studies were selected if patients had a diagnosis of NF2 and a CI placed in the affected side with reports of hearing outcome. Two independent reviewers screened each abstract and full-text article.

Data Extraction Studies were extracted at the patient level, and the assessment of quality and bias was evaluated according to the National Institutes of Health Quality Assessment Tool.

Main Outcome Measures Outcome predictors were determined by using the chi-square test and Student's t-test.

Results Overall, most CI recipients functioned in the high-to-intermediate performer category for both sporadic and NF2-related VS. Median AzBio (Arizona Biomedical Institute Sentence Test) was 72% (interquartile range [IQR]: 50) in NF2 patients and 70% (IQR: 7.25) in sporadic patients. Larger tumor size predicted a poorer final audiometric outcome.

Conclusions Categorization of hearing outcome into superior performance and inferior performance based on sentence recognition revealed a generally good hearing outcome regardless of treatment or patient population. Select patients with sporadic and NF2 VS may benefit from CI.


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Introduction

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder with an estimated incidence of 1 in 33,000 people, and characterized by benign tumors of the nervous system.[1] [2] The most common lesions in NF2 are bilateral vestibular schwannomas (VSs), which occur at the cerebellopontine angle (CPA) and affect 95% of patients.[3] The majority of VS patients are non-NF2 and present with sporadic tumor formation in the fourth and fifth decades and predictably slow tumor growth patterns. NF2 patients, unfortunately, can experience a more troublesome clinical course with multiple CNS lesions, early age of onset, and bilateral VS with aggressive growth patterns.[4] [5]

Due to the bilateral nature of the VS in NF2, patients may become functionally deaf at a relatively young age from either a natural course of disease and/or treatment. Generally accepted modes of treatments for VS include observation, stereotactic radiosurgery (SRS), and surgery with, trends shifting toward more conservative measures in recent years.[6] Preservation of hearing may be best achieved with observation; however, radiation and hearing preservation surgical approaches offer some possibility of long-term hearing when neurovascular structures are protected.[7] [8] [9] Historically, hearing restoration in NF2 populations was limited to auditory brainstem implantation (ABI).[10] [11] Recently, accumulating literature suggests that when the cochlear nerve is intact, cochlear implantation (CI) offers superior hearing outcomes.[11] [12] [13] Studies have shown that peak hearing outcome is superior in CI versus ABI if the electrophysiological function is preserved in the cochlear nerve.[14] Also, there is an abundance of new literature available to draw from for an updated systematic literature review of CI outcomes in patients with NF2.

In our previous study, we performed a systematic review examining hearing outcomes in patients with sporadic VS undergoing ipsilateral CI.[15] This study will compare hearing outcomes in patients undergoing ipsilateral CI placement in NF2 and sporadic VS patients.


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Methods

A thorough systematic literature review was performed on CI outcomes in NF2 patients. To compare the hearing outcomes of patients with sporadic VS versus patients with NF2-related VS, we performed a systematic review of all patients with NF2-related VS receiving a CI in the ipsilateral ear. This newly obtained data were compared with our previous data for sporadic VS.

Eligibility Criteria

Inclusion was evaluated for each individual patient in all studies included. Studies were included even if only a portion of the patients met the inclusion criteria. For a study to be included, the data could not be aggregated; it had to be reported at a granular, patient level. The inclusion criteria for each patient were as follows: (1) must have VS with a diagnosis of NF2, (2) must have had a CI placed on the side of a VS, (3) must have reported treatment modality, and (4) cochlear implant auditory outcomes must have been reported. Of note, the VS need not have been resected for inclusion.


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Search Strategy

To identify relevant studies, searches were performed in PubMed NCBI and Scopus by an academic librarian. No restrictions based on the year of publication were used.


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Study Selection and Validation

Two reviewers independently screened each abstract and then evaluated the remaining full articles for eligibility. Discrepancies were resolved by a third reviewer.


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Data Extraction

Information was extracted at two levels: a study level and a patient level. Information extracted from each study included author, year of publication, number of patients, whether it was retrospective or prospective, and the study's level of evidence based on the Oxford Centre for Evidence-Based Medicine 2011 criteria.[14] Information extracted from individual patients, when available, included gender, age, laterality, tumor location, tumor size, preoperative hearing metrics, duration of deafness before CI, CI type, whether VS was resected, approach to tumor resection, timing of CI placement compared with resection, complications, and postoperative auditory outcomes. The data were entered into an electronic research database (REDCap).[16]


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Categorization of Hearing Outcome

A commonly encountered problem during any review of the literature analyzing hearing outcomes is the variability in the method of reporting outcomes. We adapted a method similar to that of Carlson et al of reporting hearing outcome by categorizing a range of hearing outcomes into high performance (HP), intermediate performance (IntP), low performance (LP), environmental only (EO), and no response (NR).[17] Patients with an open set speech recognition (i.e., Arizona Biomedical Institute Sentence Test [AzBio], CNC [Maryland Consonant-Vowel Nucleus-Consonant Test] score, BKB [Bamford–Kowal–Bench test], CUNY [City University of New York Test]) of 67 to 100%, 34 to 66%, and 1 to 33% were categorized as HP, IntP, and, LP respectively. Patients who had only environmental perception and nonresponders were categorized into the EO and NR categories, respectively.


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Assessment of Quality and Bias of Individual Studies

The National Institutes of Health's (NIH) Quality Assessment Tool for of Case Series Studies was used to evaluate quality and bias of individual studies.[18]


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Statistical Methods

Descriptive statistics were used to summarize patient characteristics and outcomes of interest. Associations between predictors and hearing function were tested using regression analysis on the pooled sample. Between-study variation was accounted for by incorporating fixed study effects. Cases with missing data were excluded from the analyses. All analyses were performed using SAS Version 9.4 (SAS Institute Inc., Cary, North Carolina, United States).


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Systematic Review Results

Study Selection

A total of 245 studies were identified from the PubMed and Scopus searches. After duplicates were removed, 170 abstracts were screened. After implementation of our selection criteria, 133 studies were excluded based on their abstracts. The remaining 37 full articles were reviewed and 5 records were excluded because of sporadic VS and no postimplant hearing outcome. A total of 32 articles fulfilled all criteria for inclusion.


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Study Characteristics

The studies that met the inclusion criteria were published between 1992 and 2018. [Table 1] shows studies that reported outcome measures. The assessment of bias and quality for each study was recorded in [Supplementary Table S1], available online only.

Table 1

Study characteristics[48] [49] [50] [51] [52] [53] [54] [55] [56] [57] [58] [59] [60] [61] [62] [63] [64] [65] [66] [67] [68] [69] [70]

Reference

First author

Publication year

Language

Patients in study

Patients used

Reason for patient removal

Outcomes measures

1296

Tan

2018

E

12

12

PTA, SDS, Sent

1298

Peng

2018

E

10

10

CUNY, Iowa

1542

Neto

2018

E

1

1

Ling

1038

Pisa

2017

E

3

3

PTA, SDS, HINT, CNC

1306

Harris

2017

E

12

12

SDS, BKB

1547

DeHart

2017

E

1

1

AzBio, CNC

1040

Costello

2016

E

1

1

CUNY

1310

North

2016

E

13

10

No hearing outcome

CUNY, BKB

1313

Carlson

2016

E

10

7

Sporadic VS

PTA, AzBio, CNC

1316

Pimentel

2015

E

1

1

SDS

1572

Ozdek

2014

E

2

1

Sporadic VS

SDS, Sent

1321

Lassaletta

2013

E

15

8

Sporadic VS

PTA, SDS

1327

Lloyd

2013

E

6

5

No hearing outcome

CUNY, BKB

1331

Pai

2013

E

7

5

Sporadic VS

CUNY, BKB

1050

Mukherjee

2013

E

11

9

Sporadic VS

BKB

1043

Carlson

2012

E

10

9

No speech hearing outcome

AzBio, HINT, CNC, BKB

1045

Amoodi

2012

E

2

2

SDS, SRT, HING, CNC

1334

Monteiro

2012

E

4

1

ABI implanted

PTA, open set phrases

1344

Cruz

2011

E

1

1

PTA, Sent

1345

Odat

2011

E

45

5

No CI implanted

PTA, SDS, Sent

1346

Roehm

2011

E

7

7

PTA, SDS, HINT, CNC, CUNY

1033

Trotter

2010

E

3

2

No hearing outcome

CNC, CUNY

1593

Huy

2009

E

3

3

SDS, Sent

1366

Vincenti

2008

E

9

4

ABI implanted

SDS, Sent

1371

Neff

2007

E

6

6

HINT, Sent, CUNY

1028

Lustig

2006

E

7

7

PTA, SDS

1383

Aristegui

2005

E

2

2

SDS, Sent

1387

Nolle

2003

E

1

1

Sent

1411

Graham

1999

E

1

1

BKB

1412

Temple

1999

E

1

1

Sent

1418

Tono

1996

E

1

1

SDS, Sent

1700

Hoffman

1992

E

1

1

NU-6, MAC open set, CID sent

Abbreviations: ABI, auditory brainstem implantation; AzBio, Arizona Biomedical Institute Sentence Test; BKB, Bamford–Kowal–Bench test; CI, cochlear implant; CNC, Maryland Consonant-Vowel Nucleus-Consonant Test; CUNY, City University of New York Test; E, English; HINT, hearing in noise test; Lang., language; MAC, minimum auditory capabilities; NU-G, Northwestern University Auditory Test No. 6; SDS, speech discrimination score; Sent, sentence discrimination; SF, sound field; THI, tinnitus handicap index; VAS, visual analog scale; VS, vestibular schwannoma.



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Patient Characteristics

Preoperative characteristics and distribution of VS treatment modality from our study of NF2 patients and our previous study of sporadic VS patients are listed in [Table 2]. In NF2 patients, the implanted tumor side was on the left (62.6%) more often than the right (37.4%). The cerebellopontine angle was the most common location of VS (60.8%) and the internal auditory canal was the second most common (37.3%). A single tumor was involved at more than one location in 19.6% of cases. A total of 132 patients were included in this study, and the majority of patients underwent surgery (56.1%). SRS was pursued in 25.8% of patients, and 18.2% of patients were observed. Operative therapy was pursued in 74 (56.1%) of patients, and nonoperative VS therapy was pursued in 58 (44%) of patients. Several different approaches to resect tumors were performed. The most common approach was retrosigmoid (46.9%) and the second most common approach was translabyrinthine (32.8%). A CI was placed at the time of surgical resection of the tumor in 44.1% of patients. Patients were followed for a mean of 39 months after CI placement.

Table 2

Preoperative characteristics

Characteristic

NF2

Sporadic

n

Result

n

Results

Treated side: right

91

34 (37.4%)

39

24 (61.5%)

Tumor location

51

42

 CPA

31 (60.8%)

5 (11.9%)

 IAC

19 (37.3%)

24 (57.1%)

 Intracochlear

7 (13.7%)

11 (26.2%)

 Vestibular structures

4 (8.0%)

12 (28.6%)

 Two locations

10 (19.6%)

10 (23.8%)

Tumor size (mean)

111

16.7 mm

8

10.63 mm

Treatment

132

45

 Resection

74 (56.1%)

39 (86.7%)

 Stereotactic radiosurgery

34 (25.8%)

 Observation

24 (18.2%)

6 (13.3%)

Operative vs. nonoperative therapy

132

45

 Operative

74 (56.1%)

39 (86.7%)

 Nonoperative

58 (44.0%)

6 (13.3%)

Approach for resection

64

39

 Translabyrinthine

21 (32.8%)

24 (61.5%)

 Retrosigmoid

29 (46.9%)

2 (5.1%)

 Middle cranial fossa

10 (17.2%)

 Posterior fossa

1 (1.6%)

 Labyrinthectomy

8 (20.5%)

 Cochleostomy

8 (20.5%)

 Retrolabyrinthine

1 (2.6%)

 Combined approach

3 (7.7%)

CI placed concurrently with resection

59

26 (44.1%)

39

19 (48.7%)

Follow-up time (mean)

88

39.0 mo

41

20.2 mo

Duration of deafness

77

24 mo (IQR: 90)

18

60 mo (IQR: 132)

Abbreviations: CI, cochlear implant; CPA, cerebellopontine angle; IAC, internal auditory canal; IQR, interquartile range; NF2, neurofibromatosis 2.


Note: duration of deafness is reported as median with IQR (%).


In comparison to NF2 patients, the implanted sporadic VS was more commonly located on the right side (61.5%) and at the internal auditory canal. Mean tumor size in sporadic VS was 10.63 ± 4.1 mm, which was smaller than that in the NF2 group (16.7 ± 9.2 mm). The majority of sporadic VS were resected most commonly through the translabyrinthine approach, and we found no studies looking at CI in sporadic VS after SRS.

Preoperative hearing characteristics in NF2 and sporadic VS patients are shown in [Table 2] and [Fig. 1a,b]. NF2 patients presented with a median of 24 (interquartile range [IQR]: 90) months of deafness compared with sporadic patients who presented after a median of 60 (IQR: 132) months. The median preoperative ipsilateral pure tone average (PTA) was 110 db (IQR: 19) and speech discrimination score (SDS) was 0% (IQR: 0) in NF2 patients. Sporadic VS patients presented with a better PTA at 82.5 dB (IQR: 57.5) and SDS of 1% (IQR: 55).

Zoom Image
Fig. 1 (a) median pure tone average (preoperative and postoperative) with interquartile ranges. (b) Median postoperative hearing outcome with interquartile ranges. AzBio, Arizona Biomedical Institute Sentence Test; CNC, Maryland Consonant-Vowel Nucleus-Consonant Test; NF2, neurofibromatosis 2; SP, sporadic.

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Auditory Outcomes

Postoperative hearing outcomes for NF2 and sporadic VS patients are described in [Fig. 1]. NF2 patients had a median PTA of 35 dB (IQR: 27), and sporadic VS patients had a median PTA of 25 dB (IQR: 2.5). Median SDS was 46% (IQR: 60) for NF2 patients and 60% (IQR: 41.5) for sporadic VS patients. AzBio testing showed a median of 72% (IQR: 50) in NF2 patients and 70% (IQR: 7.25) in sporadic patients. NF2 patients had a median CNC score of 37% (IQR: 29) and sporadic VS patients had a score of 60% (IQR: 32).

Hearing outcomes based on previously mentioned categories were analyzed from this study of NF2 patients and our previous study of sporadic VS. There was a higher frequency of HP and IntP compared with lower or poor performance across all groups and treatments ([Fig. 2a]).

Zoom Image
Fig. 2 (a) Hearing outcome (five categories). (b) Hearing outcome (two categories). SP, sporadic; Surg, surgery; Obs, observation; NF2, neurofibromatosis 2; SRS, stereotactic radiosurgery; HP, high performance; IntP, intermediate performance; LP, low performance; EO, environmental only; NR, no response.

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Cochlear Implant Outcome Predictors

In our NF2 cohort, characteristics that predicted hearing outcome were analyzed based on the five aforementioned categories but showed no statistical significance due to the small number in each category. We simplified our categorical scheme down to two by combining high performers and intermediate performers into a superior performance (SP) category (since HP and IP are more typical for uncomplicated CI in non-VS patients), and low performers, EO, and nonresponders into an inferior performance category. A simplified version of the hearing outcomes based on the two category scheme is represented in [Fig. 2b]. Interestingly, our analysis showed that operative VS therapy was not a predictor of outcome. Also, with this two category scheme, a Student's t-test found that larger tumor size predicted a poorer outcome ([Table 3]). In exploration of size threshold for predicting CI performance, we found that for tumors 1 to 10 mm in size, 78% achieved SP, for tumors 11 to 20 mm, 61% achieved SP, for tumors 21 to 30, 39% achieved SP, and tumors > 30 mm had insufficient numbers to analyze ([Table 4]).

Table 3

Outcome predictors for NF2

Predictor

n

Chi-square test

Student's t-test

p-Value

Age

139

1.73

0.086

Tumor size

111

–2.34

0.018

All treatment

132

0.59

0.75

Operative vs. nonoperative

132

0.58

0.45

Timing of CI

59

2.42

0.12

Duration of deafness

77

–0.466

0.64

Ipsilateral preoperative PTA

73

–0.9

0.37

Ipsilateral preoperative SDS

68

0.34

0.74

Abbreviations: CI, cochlear implant; NF2, neurofibromatosis 2; PTA, pure tone average; SDS, speech discrimination score.


Table 4

Distribution of CI performance for size threshold

Size (mm)

Superior performance

Inferior performance

1–10

18 (78%)

5 (22%)

11–20

31 (61%)

20 (39%)

21–30

9 (39%)

14 (62%)

>31

5 (63%)

3 (38%)

Abbreviations: CI, cochlear implant.


In our sporadic tumor population, univariable regression analysis found that a higher preoperative ipsilateral SDS predicted a lower postoperative SDS, and neither tumor resection status, tumor location, duration of deafness, ipsilateral PTA, or timing of CI placement had a significant effect on patient's outcome.[15] Unfortunately, statistical analyses for CI outcome predictors in sporadic patients using the two aforementioned category scheme was not performed due to the small number of data points available. Superior and inferior CI performance for both sporadic and NF2 groups based on tumor location is represented in [Table 5].

Table 5

Tumor location determining hearing outcome

Location

NF2

Sporadic

SP

IP

SP

IP

CPA

74%

26%

67%

33%

IAC

79%

21%

89%

11%

Intracochlear

71%

29%

80%

20%

Vestibular

100%

0%

100%

0%

Multiple locations

80%

20%

88%

13%

Abbreviations: CPA, cerebellopontine angle; IAC, internal auditory canal; IP, inferior performance; NF2, neurofibromatosis 2; SP, superior performance.



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Discussion

Bilateral VS is the hallmark of NF2 patients and is seen in 90 to 95% of affected individuals.[19] The majority of these patients suffer from hearing loss and demonstrate diminished quality of life along with communication difficulties.[20] [21] The presentation, progression, and treatment considerations, including hearing rehabilitation options, differ between NF2 and sporadic VS patients.[22]

ABIs, which directly stimulate the cochlear nucleus, have traditionally been the focus for hearing restoration in NF2 patients. Hearing outcomes with ABI are variable but typically allow patients to detect environmental sounds and improve lip reading.[23] [24] [25] More recent clinical understanding demonstrates that if the cochlear nerve is preserved, then CI should be considered. While there have been studies investigating hearing outcomes, single-institution retrospective chart reviews, and a systematic review on NF2 patients receiving CI in the ipsilateral ear, there has yet to be a review of the literature comparing CI hearing outcomes of patients with NF2 versus sporadic VS.[9] [14] [18] [26] [27] [28]

Increasingly, recent clinical practice trends favor a “watchful waiting” VS management strategy when possible.[5] [6] [29] [30] Many patients being observed can expect to retain serviceable hearing. A recent systematic review showed that approximately half of the patients being observed with stable tumors and that patients with good discrimination at presentation were able to preserve hearing for a long term.[29] Select patients with poor hearing may be considered for CI. An observed VS patient being considered for CI should have a long-term stable tumor with low likelihood for future intervention. Our study showed that 88% of observed sporadic VS patients achieved either intermediate or high, i.e., superior group, CI performance based on our open set sentence recognition category. However; only 67% of observed NF2 patients achieved SP.

A meta-analysis in 2017 demonstrated a hearing preservation rate of 79.1% in patients undergoing CyberKnife SRS treatment for sporadic and NF2 VS.[31] NF2 patients with poor hearing following radiation have shown similar CI outcomes compared with patients who are observed.[32] [33] Remember, no data were available from our review regarding CI function after SRS in sporadic VS. In NF2 patients, our study showed that 64% of patients undergoing CI after radiosurgery scored in the SP group. This number is remarkably similar to the 67% superior CI performance in the observation NF2 group. It is known that radiation produces tissue damage through vascular injury,[34] and it is postulated that the stria vascularis is the site of injury responsible for posttreatment hearing loss.[35] However, the similarity in performance between SRS and observation NF2 groups, at least, demonstrates that CI function does not appear to be largely affected with current SRS protocols.

Hearing conservation VS surgery is an option in select patients, but it may prove technically challenging, with only experienced centers achieving good results.[36] [37] Wilkinson et al showed that hearing-sparing surgery in sporadic VS yielded mean PTA and SDS of 64.4 dB and 60.8% for the middle fossa approach and 81.3 dB and 46.3% for the retrosigmoid approach, respectively. Dead ears resulted in 29% of patients with middle fossa approach and 40.7% of retrosigmoid approach.[38] Patients undergoing surgery for VS may be considered for concurrent or delayed CI placement. Despite concerns for cochlear ossification following labyrinthectomy, this study showed that only half of all patients had CIs placed concurrently with surgery. In addition, 80% of sporadic VS patients after surgery achieved superior group CI performance, with 61.5% of these patients undergoing the translabyrinthine approach. Sixty-one percent of NF2 patients after surgery achieved superior group CI performance with retrosigmoid used in 46.9% and translabyrinthine in 32.8% of cases. Ultimately, CI may be considered simultaneous or delayed after any surgical approach where the cochlea and cochlear nerve are preserved but functional hearing results are poor.

One might expect that injury to the auditory pathway at the level of the cochlea with radiation and at the retrocochlear level with surgery would affect CI performance. However, we observed that hearing outcomes were not dependent on treatment modality, and this finding is consistent with our previous study on sporadic VS.[15] Tumor size did predict hearing outcome as larger tumors tended to have worse CI performance in NF2 patients ([Table 4]). At first glance, this lower success rate with larger tumors may be expected, as NF2 tumors are known to pose a more difficult surgical dissection challenge.[39] However, as stated prior, hearing outcomes are similar between all treatment modalities.

Perhaps, tumor location affects CI performance in all VS patients. There was a noted preponderance of intracanalicular and intralabyrinthine tumor location in the sporadic group compared with a majority cerebellopontine angle location in the NF2 group. Preoperative SDS was predictive of outcome in sporadic patients, and NF2 patients did not show similar results.

A significant challenge in systematic reviews of literature of this nature is the variability of hearing outcomes reported in the studies. Each study reported hearing outcomes that were thought to be relevant. Adunka et al published a detailed minimum reporting standard in adult cochlear implants. They recommended a requirement of postoperative hearing threshold if preoperative low frequency (125, 250, 500Hz) PTA was <80 dB.[40] Committees from the American Academy of Audiology and the American Academy of Otolaryngology - Head and Neck Surgery recommended pre- and postoperative CNC and AzBio or BKB speech-in-noise test for all patients in this group.[40] [41] While these recommendations are helpful, we observed that not all studies followed these guidelines. For the purposes of this study, we used a categorical scheme that combined different hearing outcomes.

Carlson et al created a scheme of three categories based on postimplant open set speech recognition (high performer, intermediate performer, low performer). In our study, we expanded on Carlson et al's three open set levels to include a nonopen set category of EO to indicate sound awareness, which is known to enhance lip-reading ability, and an NR category. In our study, approximately 10% of the sporadic cohort achieved EO compared with approximately 25% of the NF2 cohort. Additionally, none of the sporadic patients had NR performance compared with ∼8% of the NF2 cohort ([Fig. 2a]). These data offer general guidance with which the surgeon can counsel patients about the quality of performance that may be anticipated with CI in VS.

Significant limitations exist within this study including, but not limited to, patient selection bias, heterogeneous audiometric data reporting, and comparison between tumor types. The intimate involvement of neurovascular elements and younger age of diagnosis of NF2 patients compared with sporadic VS patients can change management decisions for the treating physician.[39] At the very least, the data presented in this study demonstrate that select patients with sporadic and NF2 VS benefit from CI placement. At 6 months postimplantation, Sladen et al. showed that in nontumor patients implanted with asymmetric SNHL, mean CNC/AzBio scores of 55.9%/73.6% were achieved.[42] This systematic review demonstrated comparable outcomes with median CNC/AzBio scores of 37%/72% in NF2 and median CNC/AzBio scores of 60%/70% in sporadic tumors.

Clinical challenges in these patient populations include obtaining insurance approval for the device, managing post-CI posterior fossa artifact MRI (magnetic resonance imaging),[43] [44] and achieving maximal tumor removal with preservation of a functional cochlear nerve. Recent attempts at intracochlear electrically evoked auditory brainstem response recording may provide hope for testing intraoperative nerve integrity[45] [46] [47] and ultimately predicting CI function.


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Conflict of Interest

None declared.

Acknowledgments

The author would like to thank Jeanne Sadlik, MLS, from the Loyola University Chicago Health Sciences Library for performing the literature search.

Supplementary Material

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  • 14 Peng KA, Lorenz MB, Otto SR, Brackmann DE, Wilkinson EP. Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2. Laryngoscope 2018; 128 (09) 2163-2169
  • 15 Bartindale MR, Tadokoro KS, Kircher ML. Cochlear implantation in sporadic vestibular schwannoma: a systematic literature review. J Neurol Surg B Skull Base 2019; 80 (06) 632-639
  • 16 Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap)--a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009; 42 (02) 377-381
  • 17 Carlson ML, Breen JT, Driscoll CL. et al. Cochlear implantation in patients with neurofibromatosis type 2: variables affecting auditory performance. Otol Neurotol 2012; 33 (05) 853-862
  • 18 National Institutes of Health. Quality Assessment Tool for Case Series Studies. https://www.nhlbi.nih.gov/health-pro/guidelines/in-develop/cardiovascular-risk-reduction/tools/case_series . Accessed August 28, 2019
  • 19 Evans DG, Huson SM, Donnai D. et al. A clinical study of type 2 neurofibromatosis. Q J Med 1992; 84 (304) 603-618
  • 20 Patel CM, Ferner R, Grunfeld EA. A qualitative study of the impact of living with neurofibromatosis type 2. Psychol Health Med 2011; 16 (01) 19-28
  • 21 Neary WJ, Hillier VF, Flute T, Stephens D, Ramsden RT, Evans DGR. Use of a closed set questionnaire to measure primary and secondary effects of neurofibromatosis type 2. J Laryngol Otol 2010; 124 (07) 720-728
  • 22 Mahboubi H, Maducdoc MM, Yau AY. et al. Vestibular schwannoma excision in sporadic versus neurofibromatosis type 2 populations. Otolaryngol Head Neck Surg 2015; 153 (05) 822-831
  • 23 Otto SR, Brackmann DE, Hitselberger WE, Shannon RV, Kuchta J. Multichannel auditory brainstem implant: update on performance in 61 patients. J Neurosurg 2002; 96 (06) 1063-1071
  • 24 Lenarz T, Moshrefi M, Matthies C. et al. Auditory brainstem implant: part I. Auditory performance and its evolution over time. Otol Neurotol 2001; 22 (06) 823-833
  • 25 Nevison B, Laszig R, Sollmann W-P. et al. Results from a European clinical investigation of the Nucleus multichannel auditory brainstem implant. Ear Hear 2002; 23 (03) 170-183
  • 26 Harris F, Tysome JR, Donnelly N. et al. Cochlear implants in the management of hearing loss in neurofibromatosis type 2. Cochlear Implants Int 2017; 18 (03) 171-179
  • 27 North HJD, Mawman D, O'Driscoll M. et al. Outcomes of cochlear implantation in patients with neurofibromatosis type 2. Cochlear Implants Int 2016; 17 (04) 172-177
  • 28 Lloyd SKW, Glynn FJ, Rutherford SA. et al. Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2. Otol Neurotol 2014; 35 (01) 43-51
  • 29 Reznitsky M, Cayé-Thomasen P. Systematic review of hearing preservation in observed vestibular schwannoma. J Neurol Surg B Skull Base 2019; 80 (02) 165-168
  • 30 Martin TPC, Senthil L, Chavda SV, Walsh R, Irving RM. A protocol for the conservative management of vestibular schwannomas. Otol Neurotol 2009; 30 (03) 381-385
  • 31 Mahboubi H, Sahyouni R, Moshtaghi O. et al. CyberKnife for treatment of vestibular schwannoma: a meta-analysis. Otolaryngol Head Neck Surg 2017; 157 (01) 7-15
  • 32 Pisa J, Sulkers J, Butler JB, West M, Hochman JB. Stereotactic radiosurgery does not appear to impact cochlear implant performance in patients with neurofibromatosis type II. J Radiosurg SBRT 2017; 5 (01) 63-71
  • 33 Costello MS, Golub JS, Barrord JV, Pater L, Pensak ML, Samy RN. Cochlear implantation after radiation therapy for acoustic neuroma. J Radiosurg SBRT 2016; 4 (01) 69-74
  • 34 Adams RD. The neuropathology of radiosurgery. Stereotact Funct Neurosurg 1991; 57 (1-2): 82-86
  • 35 Wackym PA, Runge-Samuelson CL, Nash JJ. et al. Gamma knife surgery of vestibular schwannomas: volumetric dosimetry correlations to hearing loss suggest stria vascularis devascularization as the mechanism of early hearing loss. Otol Neurotol 2010; 31 (09) 1480-1487
  • 36 Buchman CA, Chen DA, Flannagan P, Wilberger JE, Maroon JC. The learning curve for acoustic tumor surgery. Laryngoscope 1996; 106 (11) 1406-1411
  • 37 Mangham Jr CA. Retrosigmoid versus middle fossa surgery for small vestibular schwannomas. Laryngoscope 2004; 114 (08) 1455-1461
  • 38 Wilkinson EP, Roberts DS, Cassis A, Schwartz MS. Hearing outcomes after middle fossa or retrosigmoid craniotomy for vestibular schwannoma tumors. J Neurol Surg B Skull Base 2016; 77 (04) 333-340
  • 39 Linthicum Jr FH, Brackmann DE. Bilateral acoustic tumors. A diagnostic and surgical challenge. Arch Otolaryngol 1980; 106 (12) 729-733
  • 40 Adunka OF, Gantz BJ, Dunn C, Gurgel RK, Buchman CA. Minimum reporting standards for adult cochlear implantation. Otolaryngol Head Neck Surg 2018; 159 (02) 215-219
  • 41 Luxford WM. Ad Hoc Subcommittee of the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery. Minimum speech test battery for postlingually deafened adult cochlear implant patients. Otolaryngol Head Neck Surg 2001; 124 (02) 125-126
  • 42 Sladen DP, Carlson ML, Dowling BP. et al. Cochlear implantation in adults with asymmetric hearing loss: speech recognition in quiet and in noise, and health related quality of life. Otol Neurotol 2018; 39 (05) 576-581
  • 43 Carlson ML, Neff BA, Link MJ. et al. Magnetic resonance imaging with cochlear implant magnet in place: safety and imaging quality. Otol Neurotol 2015; 36 (06) 965-971
  • 44 Edmonson HA, Carlson ML, Patton AC, Watson RE. MR imaging and cochlear implants with retained internal magnets: reducing artifacts near highly inhomogeneous magnetic fields. Radiographics 2018; 38 (01) 94-106
  • 45 Lassaletta L, Polak M, Huesers J. et al. Usefulness of electrical auditory brainstem responses to assess the functionality of the cochlear nerve using an intracochlear test electrode. Otol Neurotol 2017; 38 (10) e413-e420
  • 46 Cinar BC, Yarali M, Atay G, Bajin MD, Sennaroglu G, Sennaroglu L. The role of eABR with intracochlear test electrode in decision making between cochlear and brainstem implants: preliminary results. Eur Arch Otorhinolaryngol 2017; 274 (09) 3315-3326
  • 47 Kasbekar AV, Tam YC, Carlyon RP. et al. Intraoperative monitoring of the cochlear nerve during neurofibromatosis type-2 vestibular schwannoma surgery and description of a “test intracochlear electrode”. J Neurol Surg Rep 2019; 80 (01) e1-e9
  • 48 Tan H, Jia H, Li Y. et al. Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2. Eur Arch Otorhinolaryngol 2018; 275 (11) 2667-2674
  • 49 Dos Santos NetoPH, Zamponi JO, Hamerschmidt R, Wiemes GRM, Rassi MS, Borba LAB. Simultaneous cochlear implantation as a therapeutic option in vestibular schwannoma surgery: case report. Neurosurg Focus 2018; 44 (03) E9
  • 50 DeHart AN, Broaddus WC, Coelho DH. Translabyrinthine vestibular schwannoma resection with simultaneous cochlear implantation. Cochlear Implants Int 2017; 18 (05) 278-284
  • 51 Carlson ML, Neff BA, Sladen DP, Link MJ, Driscoll CL. Cochlear Implantation in Patients With Intracochlear and Intralabyrinthine Schwannomas. Otol Neurotol 2016; 37 (06) 647-653
  • 52 Pimentel PS, Ramos DS, Muniz L, Leal M de C, Caldas Neto S da S. Cochlear implant in a patient with neurofibromatosis type 2 undergoing radiotherapy. Braz J Otorhinolaryngol 2016; 82 (02) 242-243
  • 53 Ozdek A, Bayir O, Dönmez T. et al. Hearing restoration in NF2 patients and patients with vestibular schwannoma in the only hearing ear: report of two cases. Am J Otolaryngol 2014; 35 (04) 538-541
  • 54 Lassaletta L, Aristegui M, Medina M. et al. Ipsilateral cochlear implantation in patients with sporadic vestibular schwannoma in the only or best hearing ear and in patients with NF2. Eur Arch Otorhinolaryngol 2016; 273 (01) 27-35
  • 55 Pai I, Dhar V, Kelleher C. et al. Cochlear implantation in patients with vestibular schwannoma: a single United Kingdom center experience. Laryngoscope 2013; 123 (08) 2019-2023
  • 56 Amoodi HA, Makki FM, Cavanagh J, Maessen H, Bance M. Cochlear implant rehabilitation for patients with vestibular schwannoma: report of two cases. Cochlear Implants Int 2012; 13 (02) 124-127
  • 57 Monteiro TA, Goffi-Gomez MVS, Tsuji RK, Gomes MQT, Brito Neto RV, Bento RF. Neurofibromatosis 2: hearing restoration options. Braz J Otorhinolaryngol 2012; 78 (05) 128-134
  • 58 Mukherjee P, Ramsden JD, Donnelly N. et al. Cochlear implants to treat deafness caused by vestibular schwannomas. Otol Neurotol 2013; 34 (07) 1291-1298
  • 59 Cruz OLM, Vellutini EAS. Cochlear implant in type 2 neurofibromatosis: an option for better hearing rehabilitation. Braz J Otorhinolaryngol 2011; 77 (04) 538
  • 60 Odat HA, Piccirillo E, Sequino G, Taibah A, Sanna M. Management strategy of vestibular schwannoma in neurofibromatosis type 2. Otol Neurotol 2011; 32 (07) 1163-1170
  • 61 Roehm PC, Mallen-St ClairJ, Jethanamest D. et al. Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants. J Neurosurg 2011; 115 (04) 827-834
  • 62 Trotter MI, Briggs RJS. Cochlear implantation in neurofibromatosis type 2 after radiation therapy. Otol Neurotol 2010; 31 (02) 216-219
  • 63 Tran Ba HuyP, Kania R, Frachet B, Poncet C, Legac M-S. Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 2009; 129 (09) 971-975
  • 64 Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M. Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurootol 2008; 13 (04) 273-280
  • 65 Lustig LR, Yeagle J, Driscoll CLW, Blevins N, Francis H, Niparko JK. Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 2006; 27 (04) 512-518
  • 66 Arístegui M, Denia A. Simultaneous cochlear implantation and translabyrinthine removal of vestibular schwannoma in an only hearing ear: report of two cases (neurofibromatosis type 2 and unilateral vestibular schwannoma). Otol Neurotol 2005; 26 (02) 205-210
  • 67 Nölle C, Todt I, Basta D, Unterberg A, Mautner VF, Ernst A. Cochlear implantation after acoustic tumour resection in neurofibromatosis type 2: impact of intra- and postoperative neural response telemetry monitoring. ORL J Otorhinolaryngol Relat Spec 2003; 65 (04) 230-234
  • 68 Graham J, Lynch C, Weber B, Stollwerck L, Wei J, Brookes G. The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2. J Laryngol Otol 1999; 113 (05) 458-463
  • 69 Temple RH, Axon PR, Ramsden RT, Keles N, Deger K, Yöcel E. Auditory rehabilitation in neurofibromatosis type 2: a case for cochlear implantation. J Laryngol Otol 1999; 113 (02) 161-163
  • 70 Tono T, Ushisako Y, Morimitsu T. Cochlear implantation in an intralabyrinthine acoustic neuroma patient after resection of an intracanalicular tumour. J Laryngol Otol 1996; 110 (06) 570-573

Address for correspondence

Matthew Kircher, MD
Department of Otolaryngology, Loyola University Medical Center
2160 S First Avenue, Maywood, IL 60153
United States   

Publication History

Received: 11 January 2020

Accepted: 22 May 2020

Article published online:
08 June 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

  • References

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  • 14 Peng KA, Lorenz MB, Otto SR, Brackmann DE, Wilkinson EP. Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2. Laryngoscope 2018; 128 (09) 2163-2169
  • 15 Bartindale MR, Tadokoro KS, Kircher ML. Cochlear implantation in sporadic vestibular schwannoma: a systematic literature review. J Neurol Surg B Skull Base 2019; 80 (06) 632-639
  • 16 Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap)--a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009; 42 (02) 377-381
  • 17 Carlson ML, Breen JT, Driscoll CL. et al. Cochlear implantation in patients with neurofibromatosis type 2: variables affecting auditory performance. Otol Neurotol 2012; 33 (05) 853-862
  • 18 National Institutes of Health. Quality Assessment Tool for Case Series Studies. https://www.nhlbi.nih.gov/health-pro/guidelines/in-develop/cardiovascular-risk-reduction/tools/case_series . Accessed August 28, 2019
  • 19 Evans DG, Huson SM, Donnai D. et al. A clinical study of type 2 neurofibromatosis. Q J Med 1992; 84 (304) 603-618
  • 20 Patel CM, Ferner R, Grunfeld EA. A qualitative study of the impact of living with neurofibromatosis type 2. Psychol Health Med 2011; 16 (01) 19-28
  • 21 Neary WJ, Hillier VF, Flute T, Stephens D, Ramsden RT, Evans DGR. Use of a closed set questionnaire to measure primary and secondary effects of neurofibromatosis type 2. J Laryngol Otol 2010; 124 (07) 720-728
  • 22 Mahboubi H, Maducdoc MM, Yau AY. et al. Vestibular schwannoma excision in sporadic versus neurofibromatosis type 2 populations. Otolaryngol Head Neck Surg 2015; 153 (05) 822-831
  • 23 Otto SR, Brackmann DE, Hitselberger WE, Shannon RV, Kuchta J. Multichannel auditory brainstem implant: update on performance in 61 patients. J Neurosurg 2002; 96 (06) 1063-1071
  • 24 Lenarz T, Moshrefi M, Matthies C. et al. Auditory brainstem implant: part I. Auditory performance and its evolution over time. Otol Neurotol 2001; 22 (06) 823-833
  • 25 Nevison B, Laszig R, Sollmann W-P. et al. Results from a European clinical investigation of the Nucleus multichannel auditory brainstem implant. Ear Hear 2002; 23 (03) 170-183
  • 26 Harris F, Tysome JR, Donnelly N. et al. Cochlear implants in the management of hearing loss in neurofibromatosis type 2. Cochlear Implants Int 2017; 18 (03) 171-179
  • 27 North HJD, Mawman D, O'Driscoll M. et al. Outcomes of cochlear implantation in patients with neurofibromatosis type 2. Cochlear Implants Int 2016; 17 (04) 172-177
  • 28 Lloyd SKW, Glynn FJ, Rutherford SA. et al. Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2. Otol Neurotol 2014; 35 (01) 43-51
  • 29 Reznitsky M, Cayé-Thomasen P. Systematic review of hearing preservation in observed vestibular schwannoma. J Neurol Surg B Skull Base 2019; 80 (02) 165-168
  • 30 Martin TPC, Senthil L, Chavda SV, Walsh R, Irving RM. A protocol for the conservative management of vestibular schwannomas. Otol Neurotol 2009; 30 (03) 381-385
  • 31 Mahboubi H, Sahyouni R, Moshtaghi O. et al. CyberKnife for treatment of vestibular schwannoma: a meta-analysis. Otolaryngol Head Neck Surg 2017; 157 (01) 7-15
  • 32 Pisa J, Sulkers J, Butler JB, West M, Hochman JB. Stereotactic radiosurgery does not appear to impact cochlear implant performance in patients with neurofibromatosis type II. J Radiosurg SBRT 2017; 5 (01) 63-71
  • 33 Costello MS, Golub JS, Barrord JV, Pater L, Pensak ML, Samy RN. Cochlear implantation after radiation therapy for acoustic neuroma. J Radiosurg SBRT 2016; 4 (01) 69-74
  • 34 Adams RD. The neuropathology of radiosurgery. Stereotact Funct Neurosurg 1991; 57 (1-2): 82-86
  • 35 Wackym PA, Runge-Samuelson CL, Nash JJ. et al. Gamma knife surgery of vestibular schwannomas: volumetric dosimetry correlations to hearing loss suggest stria vascularis devascularization as the mechanism of early hearing loss. Otol Neurotol 2010; 31 (09) 1480-1487
  • 36 Buchman CA, Chen DA, Flannagan P, Wilberger JE, Maroon JC. The learning curve for acoustic tumor surgery. Laryngoscope 1996; 106 (11) 1406-1411
  • 37 Mangham Jr CA. Retrosigmoid versus middle fossa surgery for small vestibular schwannomas. Laryngoscope 2004; 114 (08) 1455-1461
  • 38 Wilkinson EP, Roberts DS, Cassis A, Schwartz MS. Hearing outcomes after middle fossa or retrosigmoid craniotomy for vestibular schwannoma tumors. J Neurol Surg B Skull Base 2016; 77 (04) 333-340
  • 39 Linthicum Jr FH, Brackmann DE. Bilateral acoustic tumors. A diagnostic and surgical challenge. Arch Otolaryngol 1980; 106 (12) 729-733
  • 40 Adunka OF, Gantz BJ, Dunn C, Gurgel RK, Buchman CA. Minimum reporting standards for adult cochlear implantation. Otolaryngol Head Neck Surg 2018; 159 (02) 215-219
  • 41 Luxford WM. Ad Hoc Subcommittee of the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery. Minimum speech test battery for postlingually deafened adult cochlear implant patients. Otolaryngol Head Neck Surg 2001; 124 (02) 125-126
  • 42 Sladen DP, Carlson ML, Dowling BP. et al. Cochlear implantation in adults with asymmetric hearing loss: speech recognition in quiet and in noise, and health related quality of life. Otol Neurotol 2018; 39 (05) 576-581
  • 43 Carlson ML, Neff BA, Link MJ. et al. Magnetic resonance imaging with cochlear implant magnet in place: safety and imaging quality. Otol Neurotol 2015; 36 (06) 965-971
  • 44 Edmonson HA, Carlson ML, Patton AC, Watson RE. MR imaging and cochlear implants with retained internal magnets: reducing artifacts near highly inhomogeneous magnetic fields. Radiographics 2018; 38 (01) 94-106
  • 45 Lassaletta L, Polak M, Huesers J. et al. Usefulness of electrical auditory brainstem responses to assess the functionality of the cochlear nerve using an intracochlear test electrode. Otol Neurotol 2017; 38 (10) e413-e420
  • 46 Cinar BC, Yarali M, Atay G, Bajin MD, Sennaroglu G, Sennaroglu L. The role of eABR with intracochlear test electrode in decision making between cochlear and brainstem implants: preliminary results. Eur Arch Otorhinolaryngol 2017; 274 (09) 3315-3326
  • 47 Kasbekar AV, Tam YC, Carlyon RP. et al. Intraoperative monitoring of the cochlear nerve during neurofibromatosis type-2 vestibular schwannoma surgery and description of a “test intracochlear electrode”. J Neurol Surg Rep 2019; 80 (01) e1-e9
  • 48 Tan H, Jia H, Li Y. et al. Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2. Eur Arch Otorhinolaryngol 2018; 275 (11) 2667-2674
  • 49 Dos Santos NetoPH, Zamponi JO, Hamerschmidt R, Wiemes GRM, Rassi MS, Borba LAB. Simultaneous cochlear implantation as a therapeutic option in vestibular schwannoma surgery: case report. Neurosurg Focus 2018; 44 (03) E9
  • 50 DeHart AN, Broaddus WC, Coelho DH. Translabyrinthine vestibular schwannoma resection with simultaneous cochlear implantation. Cochlear Implants Int 2017; 18 (05) 278-284
  • 51 Carlson ML, Neff BA, Sladen DP, Link MJ, Driscoll CL. Cochlear Implantation in Patients With Intracochlear and Intralabyrinthine Schwannomas. Otol Neurotol 2016; 37 (06) 647-653
  • 52 Pimentel PS, Ramos DS, Muniz L, Leal M de C, Caldas Neto S da S. Cochlear implant in a patient with neurofibromatosis type 2 undergoing radiotherapy. Braz J Otorhinolaryngol 2016; 82 (02) 242-243
  • 53 Ozdek A, Bayir O, Dönmez T. et al. Hearing restoration in NF2 patients and patients with vestibular schwannoma in the only hearing ear: report of two cases. Am J Otolaryngol 2014; 35 (04) 538-541
  • 54 Lassaletta L, Aristegui M, Medina M. et al. Ipsilateral cochlear implantation in patients with sporadic vestibular schwannoma in the only or best hearing ear and in patients with NF2. Eur Arch Otorhinolaryngol 2016; 273 (01) 27-35
  • 55 Pai I, Dhar V, Kelleher C. et al. Cochlear implantation in patients with vestibular schwannoma: a single United Kingdom center experience. Laryngoscope 2013; 123 (08) 2019-2023
  • 56 Amoodi HA, Makki FM, Cavanagh J, Maessen H, Bance M. Cochlear implant rehabilitation for patients with vestibular schwannoma: report of two cases. Cochlear Implants Int 2012; 13 (02) 124-127
  • 57 Monteiro TA, Goffi-Gomez MVS, Tsuji RK, Gomes MQT, Brito Neto RV, Bento RF. Neurofibromatosis 2: hearing restoration options. Braz J Otorhinolaryngol 2012; 78 (05) 128-134
  • 58 Mukherjee P, Ramsden JD, Donnelly N. et al. Cochlear implants to treat deafness caused by vestibular schwannomas. Otol Neurotol 2013; 34 (07) 1291-1298
  • 59 Cruz OLM, Vellutini EAS. Cochlear implant in type 2 neurofibromatosis: an option for better hearing rehabilitation. Braz J Otorhinolaryngol 2011; 77 (04) 538
  • 60 Odat HA, Piccirillo E, Sequino G, Taibah A, Sanna M. Management strategy of vestibular schwannoma in neurofibromatosis type 2. Otol Neurotol 2011; 32 (07) 1163-1170
  • 61 Roehm PC, Mallen-St ClairJ, Jethanamest D. et al. Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants. J Neurosurg 2011; 115 (04) 827-834
  • 62 Trotter MI, Briggs RJS. Cochlear implantation in neurofibromatosis type 2 after radiation therapy. Otol Neurotol 2010; 31 (02) 216-219
  • 63 Tran Ba HuyP, Kania R, Frachet B, Poncet C, Legac M-S. Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 2009; 129 (09) 971-975
  • 64 Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M. Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurootol 2008; 13 (04) 273-280
  • 65 Lustig LR, Yeagle J, Driscoll CLW, Blevins N, Francis H, Niparko JK. Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 2006; 27 (04) 512-518
  • 66 Arístegui M, Denia A. Simultaneous cochlear implantation and translabyrinthine removal of vestibular schwannoma in an only hearing ear: report of two cases (neurofibromatosis type 2 and unilateral vestibular schwannoma). Otol Neurotol 2005; 26 (02) 205-210
  • 67 Nölle C, Todt I, Basta D, Unterberg A, Mautner VF, Ernst A. Cochlear implantation after acoustic tumour resection in neurofibromatosis type 2: impact of intra- and postoperative neural response telemetry monitoring. ORL J Otorhinolaryngol Relat Spec 2003; 65 (04) 230-234
  • 68 Graham J, Lynch C, Weber B, Stollwerck L, Wei J, Brookes G. The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2. J Laryngol Otol 1999; 113 (05) 458-463
  • 69 Temple RH, Axon PR, Ramsden RT, Keles N, Deger K, Yöcel E. Auditory rehabilitation in neurofibromatosis type 2: a case for cochlear implantation. J Laryngol Otol 1999; 113 (02) 161-163
  • 70 Tono T, Ushisako Y, Morimitsu T. Cochlear implantation in an intralabyrinthine acoustic neuroma patient after resection of an intracanalicular tumour. J Laryngol Otol 1996; 110 (06) 570-573

Zoom Image
Fig. 1 (a) median pure tone average (preoperative and postoperative) with interquartile ranges. (b) Median postoperative hearing outcome with interquartile ranges. AzBio, Arizona Biomedical Institute Sentence Test; CNC, Maryland Consonant-Vowel Nucleus-Consonant Test; NF2, neurofibromatosis 2; SP, sporadic.
Zoom Image
Fig. 2 (a) Hearing outcome (five categories). (b) Hearing outcome (two categories). SP, sporadic; Surg, surgery; Obs, observation; NF2, neurofibromatosis 2; SRS, stereotactic radiosurgery; HP, high performance; IntP, intermediate performance; LP, low performance; EO, environmental only; NR, no response.