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E. R. Anson and Y. Gimmon
Chapter 15
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Vestibular Implants
E.Loos, N.Verhaert, E.Devocht, N.Guinand, A.Perez-Fornos, C.Desloovere, andR.van de Berg
Introduction
During the last few decades, much research has been performed to develop an implant­able vestibular prosthesis to articially restore vestibular function. The concept is simi­lar to a cochlear implant (CI), which has already been used for many years for treating severe sensorineural hearing loss. The vestibular implant (VI) aims to provide the
E. Loos (*) Department of Neurosciences, Research Group ExpORL, KU Leuven, University of Leuven, Leuven, Belgium
Department of Otorhinolaryngology-Head and Neck Surgery, University Hospitals Leuven, Leuven, Belgium
Department of ENT/Audiology, School for Mental Health and Neuroscience (MHENS), Maastricht University Medical Center, Maastricht, The Netherlands e-mail: elke.loos@kuleuven.be
N. Verhaert · C. Desloovere Department of Neurosciences, Research Group ExpORL, KU Leuven, University of Leuven, Leuven, Belgium
Department of Otorhinolaryngology-Head and Neck Surgery, University Hospitals Leuven, Leuven, Belgium e-mail: elke.loos@kuleuven.be; Nicolas.verhaert@kuleuven.be; christian.desloovere@
uzleuven.be
E. Devocht · R. van de Berg Department of ENT/Audiology, School for Mental Health and Neuroscience (MHENS), Maastricht University Medical Center, Maastricht, The Netherlands e-mail: Elke.devocht@mumc.nl; raymond.vande.berg@mumc.nl
N. Guinand · A. Perez-Fornos Division of Otorhinolaryngology and Head-and-Neck Surgery, Department of Clinical Neurosciences, Geneva University Hospitals, Geneva, Switzerland e-mail: Nils.Guinand@hcuge.ch; Angelica.PerezFornos@hcuge.ch
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 B. T. Crane et al. (eds.), Disorders of the Vestibular System,
https://doi.org/10.1007/978-3-031-40524-2_15
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central nervous system with information about head spatial orientation and movement. The pioneering work of Bernard Cohen and Jun-Ichi Suzuki in the 1960s, who rst described eye movements originating from electrically stimulating the ampullary nerves of rabbits, pigeons, cats, and monkeys [1], inspired Gong and Merfeld, who described the rst vestibular prosthesis that was tested in guinea pigs [2]. Those animal experiments paved the way for human research. In 2004, the rst human patients under­went vestibular stimulation before undergoing surgery for cochlear implantation or sur­gical labyrinthectomy [3]. In these trials, it was conrmed that electrical stimulation of the ampullary nerves could produce a nystagmic response aligned with the plane of the stimulated canal. After this proof of concept, Guyot etal. performed the rst vestibular implantation in humans in 2007 [4]. This implant was a modied CI (MED-EL, Innsbruck, Austria), in which one electrode was removed from the cochlear array and implanted in the vicinity of the posterior ampullary nerve. It was demonstrated that a patient could adapt to electrical stimulation of the vestibular system without too much discomfort and that, once the adaptation was completed, the electrical stimulation could be modulated to articially evoke smooth eye movements [5]. As these prerequisites for developing a VI were fullled, the implant was modied into a multichannel prosthesis and implanted for the rst time in 2012 by the Geneva-Maastricht Group [6]. At the moment, two main types of VIs exist: the semicircular canal implants and the otolith implants. There are two subtypes of semicircular canal (SCC) implants: pure VIs and cochleovestibular implants, which combine a CI with a VI.
In this chapter, we provide an overview of current knowledge concerning VI candidacy, operative techniques, device programming, clinical outcomes, possible complications, and a future outlook.
E. Loos et al.
Candidacy
To date, VIs are only available in a research setting, and therefore no reimbursement criteria exist. They are mainly developed to treat patients with bilateral vestibulopa­thy (BV). This is a very debilitating disorder that can lead to a broad spectrum of symptoms like, for example, postural instability, impairment of spatial orientation, and distorted vision in dynamic conditions (i.e., while walking), commonly known as oscillopsia. It has been conservatively estimated that more than 1.8 million peo­ple worldwide have severe bilateral vestibular loss, but this is probably an underes­timation [7]. To date, BV has a poor prognosis, as more than 80% of the patients experience no signicant improvement despite vestibular rehabilitation [8]. Vestibular implantation could be the solution for treating this debilitating condition.
To facilitate comparison of results from different studies, VI-implantation criteria were proposed by van de Berg etal. [9] (Table15.1) in cooperation with all current research groups. The diagnostic criteria for BV, according to the Bárány Society, were modied and extended because they are solely based on the horizontal vestibular­ocular reex (VOR) function. As vestibular implantation is practically irreversible and can cause a deterioration of residual vestibular functions, it was important to include
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Table 15.1
VI implantation criteria for BV as described by van de Berg etal. [9]
A.Chronic vestibular syndrome with the following disablinga symptoms:
• Unsteadiness when walking or standing plus at least one of the following: – Movement-induced blurred vision or oscillopsia during walking or quick head/body
movements, and/or
– Worsening of unsteadiness in darkness and/or on uneven ground B.Symptoms greatest during head movement C.Bilaterally reduced or absent angular VOR function documented by at least one of the
following major criteria:
• Bilaterally pathological horizontal angular VOR gain ≤0.6 and at least bilaterally one vertical angular VOR gain <0.7, documented by the vHIT or scleral-coil technique
• Reduced caloric response (sum of bithermal max. peak slow-phase velocity on each side ≤6°/s for 30-s water stimuli or <10°/s for 60-s water or air stimuli)
• Decreased horizontal angular VOR gain ≤0.1 upon sinusoidal stimulation on a rotatory chair (0.1Hz, V
=50°/s) and a phase lead >68° (time constant <5s)
max
C′. Obligatory only in case of implantation of otolith structures: Bilaterally absent cVEMP and oVEMP responses
D.In case only one or two criteria from C are met (and also criterion C′ is met in case of otolith stimulation), the remaining test(s) should comply with the following minor criteria:
• Bilaterally pathological VOR gains of at least two SSCs <0.7, measured by the vHIT or scleral-coil technique
• Reduced caloric response (sum of bithermal max. peak slow phase velocity on each side <10°/s for water and air stimuli of ≥30s)
• Reduced horizontal angular VOR gain <0.2 upon sinusoidal stimulation on a rotatory chair (0.1Hz, V
=50°/s)
max
E.Symptoms are not better accounted for by another disease F.Fitting the additional requirements relevant to initial preclinical trials
• Age 18years and above
• BV results most likely from a peripheral origin
• Vestibular function and symptoms are unlikely to signicantly improve, according to the duration of symptoms and clinicians’ estimations
• Patent vestibular end-organ and intact vestibular nerve
b
c
d
• Ability to use the device and follow a personalized rehabilitation program
• Ability to undergo the surgery
e
G.No current psychological or psychiatric disorder that could signicantly interfere with the use or evaluation of the VI
a
Disabling is dened as “interfering with activities of daily living”. It is up to the patient and clini­cian to determine whether symptoms are disabling. A Dizziness Handicap Inventory total score of >30 can be considered
b
A disorder of peripheral origin means the etiology is localized to the inner ear (e.g., hair cell loss or dysfunction due to gentamicin ototoxicity, a genetic abnormality, infection, ischemia, trauma, or other labyrinthine injuries). Patients with obvious central vestibular involvement (e.g., tumors of the vestibular nerve, brainstem lesions, and cerebellar ataxia) should be excluded. An idiopathic BV patient with a normal MRI of the brain and internal auditory canals and without any central vestibular signs on physical examination including ocular motor testing can be regarded as “most likely from a peripheral origin”
c
If possible, we advise waiting at least 6months before implantation
(continued)
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Table 15.1 (continued)
d
 We recommend performing a CT scan for evaluation of the anatomy. In conditions with an increased risk of a nonpatent labyrinth, for example, meningitis with concern for developing laby­rinthitis ossicans, patency should be checked with MRI and/or CT.There should be no clear signs of structural nerve pathology
e
No allergy to the material of the prosthesis, no anesthetic contraindication
E. Loos et al.
the function of all three SSCs. For the otolith implants, cervical and ocular vestibular myogenic potentials (cVEMP and oVEMP) were included in the criteria as well.
Possibly, more extensive criteria, including patients with uctuating vestibular disorders, elderly patients with presbyvestibulopathy, pediatric patients, or chroni­cally uncompensated unilateral vestibulopathy, might be considered in the future. Additionally, bilateral VIs could also be considered. Interestingly, research has already demonstrated that VI information is able to overrule residual natural ves­tibular information [10, 11]. This could lead to using a VI as a “vestibular pace­maker” in cases of a uctuation of the vestibular function and vertigo attacks in disorders like Meniere’s disease [10]. Former research, however, demonstrated a loss of residual vestibular function as a consequence of the implantation surgery [11]. Therefore, at the moment, the use of the VI as a pacemaker should be consid­ered only in patients with no “useful” vestibular function.
SSC Implants
To date, most research has been done on SSC implants. Each research group has a specic subtype. The group of Geneva-Maastricht uses a vestibulocochlear implant manufactured by the company MED-EL (Innsbruck, Austria) (Fig.15.1).
The system comprises 12 stimulation contacts. The three most basal electrodes from the cochlear array were taken out and replaced by three individual electrode branches. These three individual branches were designed to be inserted into the SSCs to allow for stimulation of the vestibular ampullary nerves. The remaining nine stimulation contacts make up the cochlear array, designed to be inserted into the cochlea like a CI [12]. At the moment, the Geneva-Maastricht Group only uses the VI on patients with severe hearing loss, as the chances of hearing loss from implanting the vestibular organs are high. All patients were candidates for CI sur­gery, which reduced the surgical risks and increased the benet for the patients.
The Baltimore group uses a VI without a CI, also manufactured by the company MED-EL (Innsbruck, Austria) [13, 14]. This implant consists of three electrode arrays (each for one SSC) with three stimulating contacts per array and one refer­ence electrode.
The implant of the Washington group is similar to the vestibulocochlear implant of the Geneva-Maastricht Group but consists of a combined 16-channel cochlear and six-channel vestibular prosthesis (two stimulating contacts in each vestibular branch) (Cochlear Ltd., Sydney, Australia) [15, 16]. The initial design did not include a cochlear array [11], which was added to the second generation of the implant.
15 Vestibular Implants
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Fig. 15.1 The cochleovestibular implant of the Maastricht-Geneva Group
Fig. 15.2 Right ear. Cortical mastoidectomy and posterior tympanotomy have been performed. Blue lining of all SSCs. sSSC superior semicircular canal, lSCC lateral semicircular canal, pSCC posterior semicircular canal. *=posterior tympanotomy
305
*
sSCC
lSCC
Surgical Techniques
Two surgical techniques were developed over the past years: the extralabyrinthine and the intralabyrinthine approaches [6].
The Intralabyrinthine Approach
The rst step of the intralabyrinthine approach is a cortical mastoidectomy and posterior tympanotomy. Subsequently, the anterior ends of the anterior and lateral canals are blue-lined by drilling cranially at the dome of the lateral canal and fol­lowing it until a V-shape appears.
Then, the SCCs are carefully fenestrated [12, 13], and the electrodes are inserted as close as possible to the ampullary nerves (Figs.15.2, 15.3 and 15.4).
The posterior canal is located medially to the facial nerve, at an imaginary hori­zontal line through the stapes footplate, between the sigmoid sinus and the facial
pSCC