Cochlear Implantation During Vestibular Schwannoma Removal or During Labyrinthectomy Surgery for Treatment of Meniere's Disease
Phase: NA
Conditions: Vestibular Schwannoma, Meniere Disease
Sponsor: Ohio State University

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Title: Cochlear Implantation During  
Vestibular Schwannoma Removal or During Labyrinthectomy Surgery 
for Treatment of Meniere’s Disease 
 
 
 
 
 
Version 1.1; 05/2/2018 
 
 
 
 
 
 
 
 
 
Principal Investigator:   
Oliver Adunka, M.D., FACS 
Professor and Director  
Division of Otology, Neurotology and Cranial Base Surgery 
Department of Otolaryngology-Head and Neck Surgery 
The Ohio State University 
915 Olentangy River Road, Suite 4000 
Columbus, OH  43212 
 
   
 

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Table of Contents 
 
I. 
Objective 
II. 
Background and Rationale 
III. 
Procedures 
a. Research Design 
b. Sample 
c. Measurement/Instrumentation 
d. Detailed Study Procedures 
e. Internal Validity 
f. Risk Analysis 
g. Data Analysis 
h. Device Description 
i. Monitoring Procedures 
IV. 
Bibliography 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

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I. Objectives 
The purpose of this study is to determine longitudinal benefits of listening with a 
cochlear implant placed during the time of tumor removal for patients with a 
vestibular schwannoma and/or with patients undergoing a labyrinthectomy for 
treatment of Meniere’s disease 
 
II. Background and Rationale 
 
Cochlear implantation for patients with single-sided deafness is increasingly being 
performed. Combined electric stimulation in one ear with normal acoustic stimulation 
of the other ear has been shown to improve speech perception and sound 
localization abilities for patients with single-sided deafness, particularly in situations 
with background noise.  
 
In patients with vestibular schwannomas, which grow on the balance nerves 
adjacent to the auditory nerve, hearing loss often occurs. Moreover, during surgical 
removal of the vestibular schwannoma through a translabyrinthine approach, the 
balance portion of the inner ear is destroyed (to access the tumor), which inevitably 
leads to profound hearing loss in that ear, a particular cause of single-sided 
deafness. However, after surgical removal, a viable cochlear portion of the inner ear 
remains, and often times the auditory nerve itself can be preserved anatomically, 
even if partially compromised by tumor compression or surgical manipulation. This 
leaves the potential to deliver signals through a cochlear implant electrode array 
placed into the cochlea at the time of tumor resection. 
 
Meniere’s disease is a disorder of the inner ear in which severe dizziness and 
hearing loss occur. One surgical treatment option for these patients is a 
labyrinthectomy. Labyrinthectomies, frequently performed for Meniere’s disease, are 
very effective for treating the debilitating balance symptoms associated with this 
disease. However, as these surgeries involve opening of the inner ear by drilling of 
the labyrinth, they always result in single sided deafness. However, the cochlear 
portion of the inner ear and the auditory nerve is left intact. This leaves the potential 
to deliver signals through a cochlear implant electrode array placed into the cochlea 
at the time of the labyrinthectomy. 
 
Evidence of the longitudinal effects of electric stimulation of a compromised inner 
ear and/or eighth nerve is limited. Tran Ba Huy et al. (2008) discussed the benefits 
of cochlear implantation in three neurofibromatosis type 2 (NF2) patients who 
underwent cochlear implantation after receiving treatment for their vestibular 
schwannomas. They reported each patient had improved postoperative speech 
perception with the cochlear implant, and that follow-up CT imaging studies did not 
show additional schwannoma growth.  Zanetti et al. (2007) reported a single case 
study, where the patient underwent cochlear implantation at the same time as the 
vestibular schwannoma removal. They reported an improvement in nerve responses 
after extended listening experience with the cochlear implant.  Hansen et al. (2013) 
reported 10 subjects who underwent cochlear implantation at the time of a 

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labyrinthectomy for Meniere’s disease all had improved auditory function with the CI. 
Doobe et al. (2015) describe five patients who were simultaneously implanted with a 
cochlear implant during the same labyrinthectomy surgery for treatment of Meniere’s 
disease and the patients were able to achieve up to 69% speech recognition in the 
implanted ear. The benefits of binaural hearing, including improved localization and 
speech understanding in noise, are well-known and documented in the literature. 
This study will more systematically assess whether this patient population will also 
receive these benefits. 
 
Potential benefits to individual participants in this study include improvement in 
detection and speech perception of the surgical ear.  Participants may also 
experience improved localization abilities and speech perception in noise as the 
result of having binaural hearing.  Society may benefit from a better understanding of 
longitudinal outcomes of electric stimulation of a compromised inner ear and 
auditory nerve. This study will also help to determine if it is safe and effective to use 
cochlear implants in these patient populations. 
 
III. Procedures 
 
 
A. 
Research Design 
 
 
 
This is a prospective, quantitative clinical study. 
 
 
 
B. 
Sample 
Fifteen adult participants, aged 18 – 70 years of age, with a diagnosed vestibular 
schwannoma or Meniere’s disease will be recruited for this study.  Eligible 
participants will meet the following criteria:   
 
Inclusion Criteria: 
• Have a diagnosis of a vestibular schwannoma confirmed by a physician 
with an MRI and/or CT scan; Or have a diagnosis of Meniere’s disease by 
a physician 
• be scheduled to undergo surgery to remove the vestibular schwannoma 
through  translabyrinthine approach;  Or be scheduled to undergo a 
labyrinthectomy 
• Be English-speaking due to objective speech perception tasks. Non-
English speakers may show a reduced speech perception score due to 
language differences 
• For patients undergoing tumor removal, tumor removal must allow 
preservation of the auditory division of the VIIIth cranial nerve 
 
 
 
 
Exclusion Criteria: 

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• Subjects with bilateral Meniere’s disease or bilateral vestibular 
schwannomas 
• Inability to preserve the auditory division of the VIIIth cranial nerve 
during removal of vestibular schwannoma 
• Ossification or fibrosis of the cochlear found on preoperative imaging 
(CT or MRI) that precludes cochlear implantation 
• Active middle ear disease 
• Greater than 70 years of age 
• Vestibular schwannoma greater than 2 cm 
• Patient refusal of receiving pneumococcal vaccine 
• Any contra-indication(s) for undergoing surgery. 
 
There are no exclusion criteria related to race, gender or ethnicity, and 
recruitment will be directed to all participants that meet the criteria stated above. 
 
 
 
C. 
Measurement/Instrumentation 
 
In addition to the cochlear implant activation appointment, participants will have 
follow-up procedures/visits similar to the standard of care clinical 
procedures/visits of traditional cochlear implant recipients.  Participants will return 
to the clinic for speech perception testing and programming at intervals of 2 
weeks, 1 month, 3 months, 6 months, 9 months, and 12 months post-
implantation.  Testing will include both detection and speech perception testing in 
quiet and in noise, along with assessment of sound localization. Following the 
first 12 months the subjects will then be routinely seen on an annual or as 
needed basis for routine follow-up care. No further testing as it pertains to this 
study will be performed at these intervals. However, clinical data obtained in 
typical routine cochlear implant visits will be collected by the investigators for four 
years from the time of subject enrollment.  
 
 
D. 
Detailed Study Procedures 
 
Potential participants will be identified by the study investigators, who are also 
the treating physicians, during a clinical visit for discussion of management of 
their vestibular schwannomas or Meniere’s disease.  The study will be described 
during the clinical visit only after the decision has been made by the treating 
physician and patient on the clinical management of the vestibular schwannoma 
or Meniere’s disease. Potential participants will be provided the contact 
information of the study coordinator, who will review the consent form with the 
participant.  Continued contact will be dictated by the patient, whether they prefer 
phone, mail, or email correspondence.  All discussions regarding the study and 
possible participation will be conducted in a private, closed clinical exam room or 
office to protect the privacy of potential participants.   
 
 
Informed consent will be obtained as part of a preoperative encounter. Interested 
participants will have the study, including all risks and benefits, described to them 

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by a research team member other than the patient’s physician. The potential 
participant will be given a copy of the consent form and be given ample time to 
read it and ask questions that may arise.  If the individual agrees to  participate in 
the study, the consent form will be signed.  The participant will be given a copy of 
the signed consent form.  At this time, the participant will also be asked to 
complete the Tinnitus Handicap Inventory (THI) as a pre-operative measurement 
of his or her tinnitus perception.  
 
The subjects will be patients who have been identified as having a vestibular 
schwannoma, diagnosed via a MRI or CT scan or diagnosed with Meniere’s 
disease and is undergoing a labyrinthectomy surgery. They will receive extensive 
counseling regarding the goal of the surgery, and associated risks. It is 
recommended by the Centers for Disease Control (CDC) and is OSU’s standard 
protocol that all patients’ receiving a cochlear implant, due to the small risk of 
meningitis, be up-to-date on their pneuomococcal vaccinations. Meningitis is an 
infection in the fluid and tissue that surrounds the brain and spinal cord.  If the 
patient is not up-to-date, then he/she will receive the pneumococcal vaccinations 
prior to undergoing surgery. All implantations will be performed under general 
anesthesia. The risks associated with this study are related to the surgery itself, 
which is already routinely performed by the treating physicians for patients with 
hearing loss. Implantation of these devices and subsequent management are not 
experimental. 
 
Intraoperatively, following the procedure of translabyrinhthine approach and 
tumor removal, the surgeon will determine via visual microscopy whether there 
was preservation of the auditory division of the VIIIth cranial nerve. If 
determination is made that the auditory division is intact, then the surgeon will 
proceed with cochlear implantation only on the ipsilateral (operated) side. If the 
surgeon determines the auditory division is more likely than not to remain intact, 
then the subject with be discharged from the study and cochlear implantation will 
not be performed. 
 
Intraoperatively, following the procedure of labyrinthectomy for treatment of 
Meniere’s disease, the surgeon will proceed with cochlear implantation only on 
the ipsilateral (operative) side.  
 
Approximately 4 weeks after surgery, subjects will be fit with an external speech 
processor to stimulate the cochlear implant. Prior to activating the cochlear 
implant, subjects will be asked to complete the THI as a post-operative 
measurement of his or her perception of tinnitus.  Once activated, subjects will be 
encouraged to listen with the device to determine if electrical stimulation of a 
compromised auditory nerve offers improvements in detection and speech 
perception on the implanted side. Subjects will return to clinic for programming of 
the external speech processor and postoperative testing at the following intervals 
after the initial processor fitting: 2 weeks, 1 month, 3 months, 6 months, 9 
months, and 12 months post-implantation. 

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The following test batteries and questionnaires will be presented at each test 
interval: 
• Detection Testing  Determination of softest sound subjects can hear with 
the cochlear implant. 
o Sounds that vary in pitch (frequency) and loudness (intensity) will 
be presented in the sound field. The subject will then be asked to 
indicate when he/she detects/perceives a sound when listening with 
the cochlear implant. 
 
• Speech Perception Testing - Subject listens and responds to recorded 
sentence and word lists in quiet and in noise. 
o AZ Bio sentences and CNC word lists will be presented through a 
sound field speaker and the subject will be asked to repeat back 
the sentences and/or word he or she heard. Background noise will 
be presented in conjunction with the sentences/words at various 
signals to noise levels. 
 
• Sound Localization Testing - Subject listens to sounds presented from a 
number of speakers and identifies which speaker presented the sounds. 
o The subject will sit in the center of the sound testing booth. 
Speakers will be located at various degrees in the horizontal field 
varying from 0 to +/- 180 degrees. Sounds representing varying in 
acoustic properties will be presented at random through one 
speaker at a time. The subject is this asked to identify which 
speaker the sound came was generated from. 
 
• The Speech, Spatial and Qualities of Hearing Scale (SSQ): 
o A subjective questionnaire which allows the user to report 
measures of a range of realistic hearing contexts, spatial hearing 
components (direction and distance judgements), and other 
listening qualities (listening effort, clarity/naturalness).  
 
• Nijmegen Cochlear Implant Questionnaire (NCIQ) 
o The NCIQ encompasses hearing and speech, psychological, and 
social domains. Individual subdomain scores, as well as total 
scores across subdomains are used to evaluate the subject’s 
quality of life. 
 
• Tinnitus Handicap Inventory (THI) 
o A subjective questionnaire that identifies, qualifies, and evaluates 
the difficulties a subject may be experiencing due to tinnitus. 
 
The data collected during the study will be kept in a filing cabinet in the locked 
office of the principal investigator, or on a password-protected medical center 
computer drive, and access to the data will be limited to authorized study 

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personnel.  Data related to the research will be coded with a study number, and 
the code key will be stored separately from the research data. Only coded data 
will be transmitted between the research team. The data will be retained on file 
for at least seven years after the study is closed. The data will then be destroyed 
in accordance with the shredding/recycling procedures at The Ohio State Wexner 
Medical Center.   
 
 
 
 
E.  
Internal Validity 
All English speaking patients with a diagnosed vestibular schwannoma who have 
elected surgical resection by a translabyrinthine approach will be invited to 
participate in the study.  Participants must be English speaking due to objective 
speech perception tasks. Non-English speakers may show a reduced speech 
perception score due to language differences.  Eligible participants will not be 
selected based on gender, race, or ethnicity. 
F.  
Risk Analysis 
There is no significant added risk involved with performing a cochlear implant at 
that time of vestibular schwannoma resection or labyrinthectomy other than those 
risks associated with anesthesia due to the increased surgical time (maximum of 
15-20 minutes additional). Given that hearing is believed to be sacrificed as part 
of translabyrinthine approach for tumor resection or treatment of Meniere’s 
symptoms, the usual concerns of hearing loss, vertigo or dizziness or 
cerebrospinal fluid leak are already encompassed by vestibular 
schwannoma/labyrinthectomy surgery. Of the preliminary reports of simultaneous 
cochlear implantation, there have been no added or unforeseen complications. 
As such, the potential benefits of the knowledge that would be yielded from this 
proposal outweigh any added risks. 
Risks Associated with Surgery  
Risks associated with ALL operations include pain, scarring, bleeding, and 
infection. Anesthesia medication used during the operation also has risks for the 
heart, lungs, kidneys, liver, and brain and, in rare cases, can result in death.  
 
Possible risks associated with ALL ear operations including cochlear 
implantation: 
• damage to the facial nerve  
• dizziness  
• infection  
• bleeding  
• numbness or stiffness around the ear  
• things may taste different  
• increased ringing in the ear (tinnitus)  
• neck pain  
• skin reactions  

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• leakage of inner ear fluid, which may result in an infection called meningitis 
 
Meningitis is a known risk of inner ear surgery. There are two main types of 
meningitis, viral and bacterial. Bacterial meningitis is the most serious type. It is 
the type that has been reported in some patients with cochlear implants.  
 
Some conditions may increase the risk of meningitis. These conditions include:  
• Mondini’s malformation, an inner ear malformation  
• cerebral spinal fluid (CSF) shunt or drain  
• recurrent episodes of bacterial meningitis prior to cochlear implant surgery  
• presence of a perilymph fistula  
• some types of skull fracture and defects  
 
The Centers for Disease Control (CDC) recommends that patients planning to 
receive a cochlear implant should be up-to-date on age-appropriate 
pneumococcal vaccinations 2 or more weeks before surgery (more information 
about the CDC’s recommendations can be found by calling the CDC’s National 
Immunization Information Hotline at 1-800-232-2522). 
 
 
G. 
Data Analysis 
 
Statistical analysis will be undertaken using standard repeated measures of 
analyses of variance.  Fifteen participants will allow for the comparison of 
average speech perception performance with a cochlear implant over the course 
of 12 months. The fifteen patients will not have a “baseline” audiogram that is 
comparable pre- to post- surgery since the nature of the two surgeries result in 
profound hearing loss. However, the preoperative audiogram will be used during 
analysis since it will provide the investigators insight into the extent of neural 
compromise (as measured by word recognition scores) at the time of surgery. 
 
Although the number of subjects is low we will employ descriptive statistics to 
measure variability and central tendency among the entire group as well as 
within the two groups when separated by etiology (Meniere’s disease or 
vestibular schwannoma) at each interval on all performance measures including 
questionnaires. Other variables known to influence outcomes (age, duration of 
hearing loss, pre-operative audiogram) as well as new factors (size of tumor, 
duration of primary disease) will also be used in multiple analyses of the 
variance. 
 
The primary effectiveness endpoint will include a statistically significant 
improvement in the AZ Bio sentences and CNC word recognition scores (in 
quiet) at the 12 month testing interval compared to the 6 month interval in the 
implanted ear. The secondary effectiveness endpoint will include significant 
device benefit for detection of sounds defined as a detection of sounds greater 
than 50% correct on correctly localizing location of the sound in space when 
listening through the implant at the 12 month testing interval.  
 

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The primary safety endpoint will be defined as the number and proportion of 
participants experiencing an adverse event, defined as any medical/surgical (i.e. 
explant due to infection, chronic dizziness) at the conclusion of the study. The 
secondary safety endpoint will be defined as the number and proportion of 
participants experiencing any adverse device events (i.e any open/short circuits, 
facial nerve tingling during stimulation) at the conclusion of the study. 
 
 
 
H. 
Device Description 
 
Cochlear implants (CIs) electronically process sound and transmit information to 
the auditory nerve, allowing dramatically improved perception of speech and 
environmental sounds for many individuals with severe to profound hearing loss. 
Approved by the United States Food and Drug Administration (FDA) in the 
1980’s, CIs have become widely used. For patients with sporadic vestibular 
schwannomas or neurofibromatosis Type 2 (NF2), an autosomal dominant 
condition prone to development of vestibular schwannomas, cochlear 
implantation is often performed as a separate surgery following tumor resection if 
there is preservation of the cochlear nerve (i.e., cranial nerve 8). Cochlear 
implants consist of an internal component, the receiver-stimulator, which receives 
a signal from the externally-worn, magnetically attracted head piece, and 
transduces this into an electrical signal delivered via an electrode inserted 
directly into the cochlea to interface with the cochlear hair cells and the spiral 
ganglion. 
 
For this study all subjects will be implanted with an Advanced Bionics HiRes Ultra 
cochlear implant device for the internal portion. Subjects will also receive an 
Advanced Bionics Naida Kit which will include the Naida CIQ90 sound processor, 
1 universal head piece (UHP), 1 UHP color cap, 1 T-mic, 1 charger, 1 power 
supply, 1 carrying case, 1 Dry ‘n store, 1 brick, and 1 backpack. 
 
I. 
Monitoring Procedures 
 
The risks involved in cochlear implantation are not beyond the risks normally 
encountered in the intraoperative or postoperative course for vestibular 
schwannoma surgery or labryinthectomy for Meniere’s disease, which all study 
participants will be undergoing. Standard intraoperative precautions and safety 
monitoring including, but not limited to nerve integrity monitoring and time outs, 
will be performed as per routine.  
 
An Adverse Event will be considered any unfavorable or unintended change in 
structure, function, signs, or symptoms temporally associated with the use of the 
medicinal device, whether or not a causal relationship with the product has been 
established.  Clinically significant laboratory abnormalities may be considered 
AEs if deemed appropriate by the Investigator.  Worsening of a pre-existing 

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condition is also considered an AE as is the discovery of an abnormal finding 
during physical exam that was not included in the medical history. 
 
Subjects will be encouraged to spontaneously report any AE.  Study personnel 
will ask open-ended questions to obtain information about AEs at every visit.  
Date and time of onset and resolution (if applicable) of the AE will be 
documented.  All adverse events occurring after the initiation of the study 
treatment (treatment emergent adverse events) will be reported, including events 
present at baseline that worsened during the study. 
 
Additionally, any serious adverse event considered by an investigator to be 
possibly, probably, or definitely related to the investigational device that is 
brought to the attention of the investigator at any time will be reported 
immediately to the IRB. 
 
The Data and Safety Monitoring Board (DSMB) within the Clinical Trials Office at 
The Ohio State University Comprehensive Cancer Center (OSUCCC), will serve 
as the independent data and safety monitoring board for this study. The DSMB 
will meet quarterly to review progress of the study and will be available for 
emergent meetings if serious or unanticipated adverse events occur.  The 
serious adverse events and responses will be reviewed by the DSMB. 
 
IV. 
Bibliography 
 
Ahsan S, Telischi F, Hodges A, Balkany T. Cochlear implantation concurrent with 
translabyrinthine acoustic neuroma resection. Laryngoscope. 2003 
Mar;113(3):472-4. 
 
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 Mar;26(2):205-10. 
 
Arriaga MA, Marks S. Simultaneous cochlear implantation and acoustic neuroma 
resection: imaging considerations, technique, and functional outcome. 
Otolaryngol Head Neck Surg. 1995 Feb;112(2):325-8. 
 
Bento RF, Monteiro TA, Bittencourt AG, Goffi-Gomez MV, de Brito 
R.Retrolabyrinthine approach for cochlear nerve preservation in 
neurofibromatosis type 2 and simultaneous cochlear implantation. Int Arch 
Otorhinolaryngol. 2013 Jul;17(3):351-5. doi: 10.7162/S1809-
977720130003000018. 
 
Colletti V, Fiorino F, Carner M, Cumer G, Giarbini N, Sacchetto L. Intraoperative 
monitoring for hearing preservation and restoration in acoustic neuroma surgery.  
Skull Base Surgery. 2000 Nov 10 (4):187-196. 

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Doobe G, Ernst A, Mittmann P, Todt I. Simultaneous Labyrinthectomy and 
Cochlear Implantation for Patients with Single-Sided Ménière’s Disease and 
Profound Sensorineural Hearing Loss. BioMed Res Inter. 2015; 
doi:10.1155/2015/457318 
 
Lloyd SK, Glynn FJ, Rutherford SA, King AT, Mawman DJ, O'Driscoll MP, Evans 
DG, Ramsden RT, Freeman SR.Ipsilateral cochlear implantation after cochlear 
nerve preserving vestibular schwannoma surgery in patients with 
neurofibromatosis type 2. Otol Neurotol. 2014 Jan;35(1):43-51. doi: 
10.1097/MAO.0000000000000185. 
 
Hansen MR, Gantz BJ, Dunn C. Outcomes following cochlear implantation for 
patients with single-sided deafness, including those with recalcitrant Meniere’s 
disease. Oto Neurotol. 2013; 34(9) doi: 10.1097/MAO.0000000000000102. 
 
Tran Ba Huy P, Kania R, Frachet B, Poncet C, Legac MS. Auditory rehabilitation 
with cochlear implantation in patients with neurofibromatosis type 2. Acta 
Otolaryngol. 2009 Sep;129(9):971-5. doi: 10.1080/00016480802510202. 
 
Zanetti D, Campovecchi CB, Pasini S, Nassif N. Simultaneous translabyrinthine 
removal of acoustic neuroma and cochlear implantation. Auris Nasus Larynx. 
2008 Dec;35(4):562-8. doi: 10.1016/j.anl.2007.11.011. Epub 2008 Feb 19.