Acta Oto-Laryngologica

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ABI-auditory brainstem implant

Anandhan Dhanasingh & Ingeborg Hochmair

To cite this article: Anandhan Dhanasingh & Ingeborg Hochmair (2021) ABI-auditory brainstem
implant, Acta Oto-Laryngologica, 141:sup1, 63-81, DOI: 10.1080/00016489.2021.1888486

To link to this article: https://doi.org/10.1080/00016489.2021.1888486

© 2021 The Author(s). Published by Informa

UK Limited, trading as Taylor & Francis
Group.

Published online: 03 Apr 2021.

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ACTA OTO-LARYNGOLOGICA
2021, VOL. 141, NO. S1, S63–S81
https://doi.org/10.1080/00016489.2021.1888486

REVIEW ARTICLE

ABI-auditory brainstem implant

Anandhan Dhanasingh and Ingeborg Hochmair
MED-EL Elektromedizinische Geraete Gesellschaft m.b.H., Innsbruck, Austria

ABSTRACT ARTICLE HISTORY

The Auditory Brainstem Implant (ABI) is based on the classic cochlear implant (CI) but uses a different stimula- Received 13 December 2020
tion electrode. At MED-EL, the early development activities on ABI started in the year 1994, with the sugges- Accepted 6 January 2021
tion coming from J. Helms and J. Mu €ller from Wu€rzburg, Germany in collaboration with the Univ. of
KEYWORDS
Innsbruck Austria. The first ABI surgery in a neuro-fibromatosis (NF2) patient with the MED-EL device took
Neuro-fibromatosis; non-
place in the year 1997. Later, the indication of ABI was expanded to non-NF2 patients with severe inner-ear tumor; cochlear nerve
malformation, for whom a regular CI will not be beneficial. Key translational research activities at MED-EL in deficiency; non-auditory
collaboration with numerous clinics investigating the factors that affect the hearing performance amongst side effects
ABI patients, importance of early ABI implantation in children, tools in pre-operative assessment of ABI candi-
dates and new concepts that were pursued with the MED-EL ABI device. The CE-mark for the MED-EL ABI to
be used in adults and children down to the age of 12 months without NF-2 was granted in 2017 mainly
based on two long-term clinical studies in the pediatric population. This article covers the milestones of trans-
lational research from the first concept to the widespread clinical use of ABI in association with MED-EL.

3.1. Introduction nerve due to tumour presence or its removal – all such con-
ditions preclude CI to act as a connection between the inner
Cochlear implants (CI) have been clinically proven to be
effective in restoring hearing in sensorineural hearing loss ear and CN. CN being positioned anatomically in the near
(SNHL). The electric stimulation from the CI electrode is vicinity of the cochlear nerve and directly on the auditory
picked up by the peripheral neural fibres of the spiral gan- brainstem has proven to be a surgically viable location for
glion cell bodies and transmitted to the cochlear nerve the application of direct electric stimulation.
which then leads it to the cochlear nucleus (CN) of the This article will introduce the history of ABI at MED-EL
auditory brainstem to reach the brain. and the translational science path it took from a university
Conditions such as the absence or nonfunctional cochlear laboratory to the patients in restoring hearing. This article also
nerve or suspected to be rendered nonfunctional cochlear covers the MED-EL sponsored/supported/site-initiated studies

CONTACT Anandhan Dhanasingh Anandhan.dhanasingh@medel.com MED-EL Elektromedizinische Geraete Gesellschaft m.b.H., Fuerstenweg 77a, 6020,
Innsbruck, Austria.
This article is a part of the compendium entitled ‘Thirty years of Translational Research behind MED-EL’ authored by Anandhan Dhanasingh (Director)
(Anandhan.dhanasingh@medel.com) and Ingeborg Hochmair (CEO, CTO) (Ingeborg.hochmair@medel.com).
ß 2021 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (http://creativecommons.org/licenses/by-nc-nd/4.0/),
which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way.
S64 A. DHANASINGH AND I. HOCHMAIR

Figure 1. Scheme showing acoustic neuroma on the auditory nerve. (www.healthdirect.gov.au/acoustic-neuroma).

that reported on the hearing performance of the ABI implant- Mark and Dr Herzog who are now appointed at MED-EL in dif-
ees. Those studies were of great support to MED-EL with ferent roles – and at the time were MSc and PhD students,
bringing forward its Auditory Brainstem Implant (ABI) respectively – began their exploration on the development of
approved by the notified bodies and consequently commercial- ABI paddle electrode for human application (Figure 2).
ising it within the European Union (EU) and other countries.

3.2. MED-EL’s journey in the development of ABI

In 1994, the journey of ABI started at MED-EL. Prof. Helms
and Prof. M€ uller who had heard about ABI activities in Los
Angeles, suggested that MED-EL should develop an ABI sys-
tem which would restore hearing in patients diagnosed with
neurofibromatosis type 2 (NF2) and needing tumour removal
surgery as these patients if deaf after the surgery did not have
any possibility for hearing restoration. An excellent co-oper- Figure 2. Master- and PhD- students at the University of Innsbruck (Prof. Erwin
Hochmair) involved in the early development of the ABI electrode.
ation between the University clinics for ENT (Prof. Jan Helms)
and neurosurgery (Prof. Klaus Roosen) proved helpful.
Acoustic neuromas in the internal auditory meatus (IAM) is The first version of the ABI electrode had penetrating needles
a pathological signature in NF2 patients, as shown in Figure 1. made of Hysol (epoxy material) as a base, and platinum-iridium
These patients are bound to lose their natural hearing during (90:10) wires of 75 mm thickness, which projected perpendicularly
their life as it causes severe damage to the cochlear nerve. NF2 to the surface of the paddle as shown in Figure 3(A).
is a genetic disorder characterised by the growth of non-cancer- Prof. Steffen Rosahl from Hannover around 1995, sup-
ous Schwann cell tumours in the nervous system, with an esti- ported evaluating the ABI electrode with penetrating needle
mated incidence rate of 1 in 40,000 people worldwide [1]. contacts in non-human subjects. On the one hand, the pen-
The most common tumours associated with NF2 are called etrating needles may be suitable for self-anchoring on the
vestibular schwannomas (VS) or acoustic neuromas (AN), and
CN, but on the other hand, no scientific evidence with
they develop along the auditory nerve, leading to SNHL and
regards to the traumatic effects in penetrating the CN dir-
deafness. VS and AN may also cause brainstem compression
ectly with such needles existed at the time. That resulted in
resulting in neurologic morbidity and mortality that requires a
flatting the contacts decision, and distribution over the pad-
surgical procedure for its removal [2]. The underlying reason
for the neuromas to develop are the mutations in the NF2 dle surface along with a polyester mesh on the bottom side
gene. Schwann cells surround the neurons and act as support- of the paddle for natural fixation over the CN (Figure 3(B)).
ing structure. NF2 gene, which is present in Schwann cells, pro- ABI paddle electrode with flat contacts was mainly the work
vides instructions for making a protein called merlin that of Dr Kovacs – another PhD student at the time at the
regulates the multiplication of the Schwann cells. The muta- University of Innsbruck who was funded by MED-EL.
tions in the NF2 gene lead to the production of a nonfunctional MED-EL’s ABI system looks identical to the CI system in
version of merlin protein that cannot regulate the growth and every aspect, other than in the design of the electrode array,
division of Schwann cells, resulting in the formation of which is placed close to the neural elements. Whilst the CI
tumours – a characteristic of NF2 [3,4]. electrode array has the stimulating contacts distributed lon-
In 1995, the first ABI paddle electrode research and develop- gitudinally along the array length, enabling the electrode to
ment activities that resulted in MED-EL’s ABI product with the be placed inside the cochlear lumen to match the tonotopic
scientific collaboration between the University of Innsbruck in frequency distribution (Figure 4(A)) closely, the ABI elec-
Austria and the University of W€ urzburg in Germany. Mag. trode has to be in a paddle format with the stimulating
 ACTA OTO-LARYNGOLOGICA S65

Figure 3. ABI paddle electrode with penetrating needle contacts (A) and flat contacts (B) (image courtesy of MED-EL).

Figure 4. CI system, with a straight longitudinal electrode placed inside the cochlea (black arrow) (A). ABI system, with the pad electrode placed on the CN (black
arrow) (B) (image courtesy of MED-EL).

contacts distributed within the paddle, enabling it to cover electric circuit is in the shape of three-leaf clover, surgically posi-
the rostral surface of the CN (Figure 4(B)). tioned under the periosteum of temporalis muscle on the tem-
poral bone. The telemetry measurements confirm the proper
functioning of the implant. Implant’s stimulator houses a magnet
3.3. Description of the first ABI system to attract the external antenna coil from the audio processor. The
MED-EL’s first commercially available ABI system was developed external components of MED-EL’s first ABI system consist of
with C40þ implant technology, a ceramic-based implant housing TEMPO þ BTE audio processor or the CIS PRO þ body-worn
that measured 33.5 mm  23.4 mm  3.95 mm. The ABI implant device. Figure 5 shows MED-EL’s first ABI system along with
electronics are identical to those of C40þ CI. They include twelve BTE audio processor.
individual capacitors for every output, safeguarding the neuronal
elements by blocking out any direct current components. ABI
active electrode array is connected to the stimulator, and the array
consists of twelve active platinum contacts which are partially
embedded within a silicone paddle that measures 5.5 mm 
3.0 mm  0.6 mm. A polyester mesh embedded in silicone
exceeds the size of the paddle – allowing the fibrous tissue growth
– which eventually stabilises the paddle over CN. The electrode
lead diameter increases from 0.7 mm at the silicone paddle to
1.3 mm over the length of 10 cm up until its connection with the Figure 5. MED-EL’s C40þ ABI system with paddle electrode and externally
stimulator housing. The reference electrode for closing the worn TEMPO þ BTE speech processor (image courtesy of MED-EL).
S66 A. DHANASINGH AND I. HOCHMAIR

3.4. MED-EL’s first ABI implantation in patients

In 1997, on 10 June, the first MED-EL ABI implantation
took place at the Julius Maximilian University of W€ urzburg
in Germany as part of a clinical trial aiming at a CE-marked
device. The ABI implantations were performed following
the study protocol prepared by Mrs. Darcy Ochs from
MED-EL together with surgeons. The surgery was per-
formed by two doctors, Prof. Behr, the neurosurgeon and
Prof. M€uller, the otologic surgeon creating a milestone in
MED-EL’s journey with scientific exploration and develop-
ment of the ABI system (Figure 6).

Figure 7. MED-EL’s ABI placement/test electrode with four stimulating contacts

and waves in eABR recording (image courtesy of Prof. Behr) [5].

lip-reading (LR). Also, some patients did exceptionally well

Figure 6. Surgeons from Julius-Maximilian University of W€
urzburg, Germany (in
the year 1994) who suggested and supported MED-EL in developing the with ABI only. Figure 8(C) shows the loudness scaling of ABI
ABI system. patients that are similar to normal hearing participants. Figure
8(D) compares the number, sentence and word test results at
The placement of the ABI paddle electrode at the best
1 and 2 years postoperatively where the results show a gradual
anatomical location on the CN is essential in bringing
increase in the hearing scores with time.
effective hearing sensation to patients. Such landmark is
In 2003, MED-EL obtained the CE mark for its COMBI
identified by placing a temporary ABI placement electrode
40þ ABI system to be implanted in over 15-year-old NF2
intraoperatively, which comprises four stimulating electrode
patients. To include the COMBI 40þ ABI system to its hearing
channels to electrically stimulate the auditory brainstem
implant solution portfolio was a historic moment in MED-EL’s
(Figure 7, inner picture). If such electric stimulation is
journey. In 2005, MED-EL upgraded its ABI system to
applied at the best anatomical location of the CN, then the
PULSARCI 100 implant type hardware, including the CE mark-
response from the CN can be recorded by the recording
ing. The difference between COMBI 40þ and PULSARCI 100
electrodes which are fixed on the head’s surface.
is in the implant electronics and the mode of communication
If recordings show waves as shown in Figure 7, then this
between the latter and the externally worn audio processor.
is a proof that the CN is functional, and this way of electric-
In 2007, September 26th, the first paediatric patient of age
ally stimulating the auditory brainstem and recording its
responses is known as electrically evoked auditory brainstem 3.5 years was implanted with MED-EL ABI system by Prof.
response (eABR). This method helps surgeons with placing Levent Sennaroglu from Hacettepe University, Ankara Turkey.
the actual ABI paddle electrode at the anatomical location
from where the satisfactory responses were recorded. 3.5. Nonauditory side effects with ABI stimulation
In 2001, there were already sixteen patients implanted
with MED-EL’s ABI system. Patients’ age at the time of sur- It is not unusual for NF2 patients with ABI to experience
gery ranged from eighteen and a half to 63 years, and all of nonauditory sensations [6]. Typically, nonauditory side
them had NF2 clinical history of deafening. Patients’ hearing effects are produced via inadvertent stimulation of cerebellar
performance was evaluated by the number and sentence tests, flocculus, the cerebellar peduncle, the long sensory tracts, or
performed at different time points and in patients’ mother the facial nerve. Up to 42% of ABI users experienced non-
tongue (Figure 8). In 2002, the audiological test results of auditory sensations with electric stimulation which are
patients implanted with MED-EL’s ABI were published in the benign but could cause considerable patient discomfort, and
American Journal of Audiology by Prof. Behr and his col- this was reported in 2007 [7]. In 2018, Dr Polak (MED-EL)
leagues [5]. There was another study that took place in paral- and Prof Colletti reported a small incidence rate of nonau-
lel which evaluated the audiological performances of twenty ditory side effects with only 6 out of 17 patients [8].
patients implanted with MED-EL’s ABI, although it was pub- Nonauditory side effects are generally minimised by deacti-
lished at a later time, in the year 2007 [6]. vating/configuration of electrode channels.
Figure 8 summarises the audiological test results from both In 2007, Prof. Behr and his colleagues published their
studies. Figure 8(A,B) show the hearing benefits in number experience with MED-EL’s ABI system, especially with the
and sentence test, respectively, with ABI in combination with nonauditory side effects and the number of deactivated
 ACTA OTO-LARYNGOLOGICA S67

Figure 8. Number (A) and sentence test (B) under various conditions with ABI, LR and ABI þ LR [5]. Loudness scaling of the ABI subjects is similar to the normal
hearing subjects when tested in the free sound field at a test frequency of 4KHz (C). Number, sentence, and word test scores of ABI patients at 1 and 2 years post-
operatively, showing an increase in scores with time (D) [6]. Reproduced by permission of Georg Thieme Verlag KG.

channels to minimise those, in a cohort of twenty patients All side effects were resolved with deactivating electrode
[6]. Side effects included sensations such as twitching in the channels randomly in a trial and error fashion. In terms of
arm and abdomen, pressure in the ear, and diplopia the number of individual electrodes used after the first fit-
(Figure 9). ting process by these patients (n ¼ 20), an average of 70.2%

Figure 9. Nonauditory sensations on different parts of the body as felt by the ABI implanted patients (image courtesy of MED-EL).
S68 A. DHANASINGH AND I. HOCHMAIR

Table 1. Number of patients and the auditory channels after the first fitting
and used channels [6].
Number of Auditory Number of Used
patients channels patients channels
13 12 3 12
2 10 3 10
1 11 1 9
3 9 3 8
1 0 3 6
4 5 Figure 10. Prof. Vittorio Colletti from the University of Verona and Prof. Robert
1 4 Shannon from the University of Southern California, evaluated the effectiveness
1 3 of ABI in non-NF2 patients.
1 0

to define the presence and functionality of CN is by electric

was used for sound sensation (Table 1), and the remainder
stimulation on the area corresponding to CN upon surgical
relates to the abovementioned side effects. In comparison to
this study, Dr Polak and his colleagues also reported on ABI exposure of brainstem. Such electric stimulation may be
side effects, but they used eABR to determine which chan- effectively done by placing the temporary ABI placement
nels are giving sound sensation and which are causing non- electrode on the anatomical landmark of the CN, as estab-
auditory side effects. An average of 79.7% of all electrode lished by the surgeon, followed by the recording of eABR
channels in their twenty patients was used for sound sensa- responses [5].
tion, and showed eABR responses without any non-auditory In 2009, Prof. Colletti and his colleagues from Italy
sensations [8]. together with Prof. Shannon from the House Ear Institute in
Table 2 presents the cumulative average of postoperative Los Angeles, USA published their 10-year experience
audiological results. Compared to ABI only mode, the of monitoring the hearing performance of NF2 and non-
audiological results were higher with the assistance of LR. tumour patients implanted with ABI [10]. In the non-tumour
Also, at 12 months, the results showed improvement from patient population were included those with head-trauma,
the sixth month. auditory neuropathy, cochlear malformations and altered
cochlear patency. Sentence recognition score in auditory-only
Table 2. Postoperative audiological results in terms of LR improvements with mode ranged from 10% to 100% (mean ¼ 59%, median ¼
the ABI device [6]. 53%, SD ¼ 21.34) in the non-tumour groups, compared to
6 months 12 months 2 years the NF2 patient group where the results ranged between 5%
LR þ ABI Sentences (%) 59.6 (n ¼ 9) 79.3 (n ¼ 5) 67.5 (n ¼ 9) and 31% (mean ¼ 10%, median ¼ 16%, SD ¼ 15.21). The
Numbers (%) 62.9 (n ¼ 9) 87.5 (n ¼ 5) 81.3 (n ¼ 5) authors of the study grouped their non-tumour patients to
ABI only Sentences (%) 46.0 (n ¼ 4) 48.4 (n ¼ 6) 42.7 (n ¼ 8)
Numbers (%) 40.1 (n ¼ 8) 69.5 (n ¼ 5) 51.7 (n ¼ 9) three cohorts based on the hearing scores.
Both groups Sentences (%) 58.5 (n ¼ 12) 81.3 (n ¼ 7) 71.1 (n ¼ 12) Rapid improvement in performance was mainly associ-
Numbers (%) 53.3 (n ¼ 9) 84.3 (n ¼ 7) 78.5 (n ¼ 11) ated with head trauma and severely altered cochlear patency
while the patients with auditory neuropathy and neurologic
The study demonstrated and reconfirmed that ABI in
disorders were the group with slow performance improve-
NF-2 patients is a safe and promising procedure for those
ment as shown in Figure 11.
who would otherwise remain deaf. Almost all patients were
able to perceive environmental sounds, and for many
patients, comprehension of open speech was restored to a
useful level.

3.6. ABI in non-tumour cases

Although the ABI was initially developed as a solution for
NF2 affected patients only, in 2002, Prof. Colletti from the
University of Verona in Italy and Prof. Shannon from the
University of Southern California in the USA were keen on
expanding it to indications other than the NF2 (Figure 10).
The indications included patients with cochlear nerve apla- Figure 11. Open set speech per cent correct in NF2 and non-tumour patients
sia (absence) – in such cases, a CI may not be beneficial – [10]. Statistical analysis: 2-tailed paired Student’s t-test. Reproduced by permis-
and patients with complete cochlear ossification – in such sion of Wolters Kluwer Health, Inc.
cases, the CI implantation may be challenging.
Prof. Colletti and his colleagues performed first ABI In 2010, MED-EL upgraded its ABI system from ceramic
implantation in cochlear nerve aplasia patients [9], and one implant housing to titanium housing (Figure 12) and CE
of the surgical challenges associated with such surgery is the marked it under the name CONCERTO ABI system for 15-
validation of the presence or absence of CN. The only way years and older NF2 patients.
 ACTA OTO-LARYNGOLOGICA S69

3.7. Consensus on ABI for children and non-

NF2 patients
Earlier studies that reported on excellent hearing benefits
with ABI in non-NF2 patients [5–10] encouraged clinicians
to widen the ABI indications to non-NF2 candidates offi-
cially. A consensus statement on indications, surgical pro-
cedure, electrophysiological tests and postoperative
rehabilitation was established. Moreover, two consensus
meetings took place in the years 2009 and 2013 to bring a
unified message from the experienced clinicians (Figure 13)
across the world [11,12].

(i) Which children and non-NF2 patients are candidates

for ABI?
Figure 12. CONCERTO ABI implant system with titanium housing encasing the Prelingually deaf patients with inner ear malformation,
implant electronics (image courtesy of MED-EL). cochlear nerve hypoplasia/aplasia and children with bilateral

Figure 13. Experienced ABI clisnicians with MED-EL ABI system. 1 Hacettepe Medical University, Turkey; 2 University of Verona, Italy; 3 Klinikum Fulda, Germany;
4
Madras ENT Research Foundation, India; 5 University of W€ urzburg, Germany; 6 Uppsala University Hospital, Sweden; 7 King Saud University, Saudi Arabia; 8 Yonsei
University Seoul, South Korea; 9 Central Manchester University Hospital, UK; 10 Institute of Physiology and Pathology of Hearing, Poland; 11 Hannover Medical
School, Germany; 12 Medipol University, Turkey; 13 University College London Hospitals, UK; 14 ENT and Hearing Care New Delhi, India; 15 University of Tokyo,
Japan; 16 Antwerp Medical University; 17 Pitie-Salpetriere Hospital, France; 18 MEDers Speech and Hearing Center, Turkey.
S70 A. DHANASINGH AND I. HOCHMAIR

and total inner ear ossification shall be considered candi- (vii) What is the age limit for ABI in children?
dates. Also, individuals deafened postlingually due to men-
Children younger than 1 year have less relative blood volume
ingitis, temporal bone fractures with cochlear nerve
and cerebrospinal fluid in the posterior fossa, and there is a
avulsion, otosclerosis with gross cochlear destruction, or
risk of brain swelling. Therefore, the optimum age for elective
unmanageable facial nerve stimulation with CI may be con-
intracranial surgery in children is between 18 and 24 months.
sidered as ABI candidates.
However, depending on the experience of the surgical team, the
minimum age for ABI in children may be as early as 1 year.
(ii) Which healthcare team is the best to undertake the
ABI intervention?
The team should comprise of otologist or neuro-otologist, (viii) Surgical approach
radiologist, paediatric neurosurgeon, implantation-experi- Retro-sigmoid approach is the preferred route to implant
enced audiologist, electrophysiologist, speech and language ABI in children and non-NF2 cases.
habilitation specialist, experienced neuroradiologist, and
experienced paediatric anesthesiologist with intensive care
unit facilities for children. (ix) Importance of electrophysiologic tests before and
after ABI activation
Electrophysiologic (EP) testing may provide two levels of
(iii) What are the radiological indications?
guidance: firstly, the optimal electrode positioning during
There are three patient categories under radiologic indications. ABI surgery and secondly, it may be used to decide which
Well defined congenital indications are Michel aplasia (absence electrode delivers sufficient auditory response levels. The
of both, inner ear and auditory nerve), cochlear aplasia preparation for activating the device should permit observa-
(absence of cochlea), cochlear nerve aplasia (absence of coch- tion of the heart rhythm as the vagus nerve is potentially
lear nerve), cochlear aperture aplasia (missing structure close to the intended location of the ABI array.
between IAC and the cochlea) as defined from the radio-
graphs. Possible congenital indications include hypoplastic
cochleae with cochlear aperture hypoplasia, common cavity (x) Rehabilitation
and incomplete partition type-I cases with cochlear nerve apla- Auditory verbal therapy, along with total communication
sia and ears with CI that did not result in satisfactory out- and speech reading, should be encouraged to convey more
comes in the first attempt. Acquired indications include linguistic and language information to these children.
postlingually deaf children with severe meningitis-related coch-
lear ossification – as viewed from computed tomography (CT)
and magnetic resonance imaging (MRI). Other indications
3.8. MED-EL’s ABI implantations across the world
include bilateral temporal bone transverse fractures with coch-
lear nerve avulsion, cochlear otosclerosis with gross destruction Over time, treatment of NF2 patients with MED-EL’s ABI
of the cochlea, which is readily diagnosed in CT and MRI, system extended to many countries, including Poland,
and with facial nerve stimulation limiting the CI use. Japan, China, Philippines and South Africa.
In 2013, a multicentric international study reported on
the nonauditory side effects and the number of deactivated
(iv) Which ear side should be selected for ABI? channels from 32 patients implanted with MED-EL’s ABI
system [7] (Figure 14).
The side with better developed lateral recess for optimal
At the time of first fitting, 8.8 ± 2.2 out of 12 available
placement of the ABI paddle electrode and which has an
electrode channels were activated to provide auditory stimuli
entrance to the lateral recess is more favourable as less cere-
to the patients. The reasons for deactivating the remaining
bellar retraction is expected.
contacts varied from no auditory sensation, unpleasant sound
sensation (faint, scratchy or persistent), contacts with same
(v) ABI revision pitch rank, electrode contacts with mixed auditory and non-
auditory sensations, and electrode contacts with only nonau-
The first ABI surgery shall be performed in an experienced
ditory responses. Different pitch perceptions are generated by
centre that minimises or avoids revision surgery as it could
the electric stimulation of different electrode contacts from
involve excessive damage to the vascularisation and scarring
the ABI paddle electrode. Deactivation of electrode channels
around the implant paddle array.
due to nonauditory sensations could be theoretically thought
to affect the hearing performance of patients with ABI; how-
ever, there was no apparent existence in the correlation
(vi) What are the contraindications?
between the number of active electrodes and patient’s hearing
Auditory neuropathy is contraindicated with the ABI surgery. performance and this is given in section 3.11.
 ACTA OTO-LARYNGOLOGICA S71

3.9. ABI versus CI in patients with cochlear

nerve deficiency
Children with cochlear nerve deficiency (CND) are a dis-
tinct patient population which generally performs below
average amongst paediatric CI recipients, with some excep-
tions. This raises medical and ethical considerations when
selecting a device and intervention that prove most benefi-
cial. In the ABI consensus meetings that took place in 2009
and 2013, the ABI experts collectively agreed that ABI
should be indicated for children with CND.
In 2014, Dr Liliana Colletti and her colleagues from the
University of Verona in Italy evaluated the auditory percep-
tual ability of children (n ¼ 40) with CND. The cohort was
implanted with CI (n ¼ 20) and ABI (n ¼ 20) (either MED-
EL or CochlearV) and evaluated for auditory perceptual abil-
R

ity using Category of Auditory Performance (CAP) index

[13]. Children with any degree of cochlear malformation
and either absence/smaller cochlear nerve fitted with ABI
demonstrated significantly earlier and better perceptual out-
comes of CAP test than children implanted with CI
(Figure 15).
In 2020, Prof. Hagr, Prof. Alsanosi, Prof. Alzhrani and
their colleagues from King Saud University in Saudi Arabia
and other centres in the Middle East, investigated CI out-
comes in children with nerve deficiency [14] (Figure 16).

Figure 14. Expert ABI surgeons who implanted MED-EL’s ABI implant systems:
1
University of W€urzburg, Germany; 2National Tokyo Medical Center, Japan; 3NTT
Medical Center Tokyo, Japan; 4Toranomon Hospital Tokyo, Japan; 5Institute of
Physiology and Pathology of Hearing, Poland; 6Free State University, South
Africa; 7University of Hong Kong Medical Centre, Hong Kong; 8University of the Figure 16. Clinicians from King Saud University, Saudi Arabia, investigated the
Philippines Manila, Philippines. CI outcomes in children with auditory nerve deficiency.

Figure 15. Categories of auditory perception scores of children with CND who were implanted with ABI/CI [13]s. Statistical analysis: Wilcoxon Mann-Whitney test
(p < .05). Histogram created from data given in Colletti et al. [13].
S72 A. DHANASINGH AND I. HOCHMAIR

A total of seven children with prelingual profound deaf- In 2014, while the research community was committed
ness with auditory nerve deficiency and a control group of to evaluating the hearing performance of ABI implanted
ten children with no cochlear nerve anomalies were patients and developing a consensus statement for ABI to
included in the study. Patients from both groups were be implanted in non-tumour cases, MED-EL continued its
implanted with MED-EL SYNCHRONY CI device. Speech dedication of bringing the best implant solutions to patients.
skills ratings using Meaningful Auditory Integration Scale MED-EL further upgraded its ABI system to SYNCHRONY
(MAIS) and Meaningful Use of Speech Scale (MUSS) were implant platform which is known for its unique diametric-
compared across the groups. In general, patients with audi- ally magnetized magnet that self-aligns in response to the
tory nerve deficiency performed poorer than those without external magnetic field in the MRI machine
nerve deficiency, as it is reflected in Figure 17. It was con- (Figure 18(A,B)).
cluded that CI outcome in children with auditory nerve The SYNCHRONY ABI implant system enables patients
deficiency is poorer than those without nerve deficiency. to undergo a 1.5 tesla MRI without magnet removal. Also,
This was a similar observation reported by Colletti et al., as the single unit audio processor (RONDOV) was made avail-
R

mentioned above [14]. able in combination with the ABI hearing system. This
upgraded system was CE marked for implantation in
patients who are 12 years and older. It is of fundamental
value to mention that Prof. Hochmair, Dr Zimmerling and
Dr Jamnig from MED-EL invented the concept of diametric
implant magnet that allows MRI scanning without the need
for implant magnet removal (Figure 19). MED-EL was the
first hearing implant company to have such implant magnet
in its CI and ABI hearing systems (US patent numbers:
6348070 and 8634909 [15]).

Figure 17. Average scores of MAIS and MUSS scales are compared for the three
groups of patients. Histogram created from data given in Yousef et al. [14].

3.10. Importance of MRI compatible implant Figure 19. Engineers from MED-EL who invented the diametric magnet for MRI
compatible implant.
NF2 patients, following the tumour removal and the ABI
surgery, may need to undergo a follow-up MRI scans to
check for any new tumour growths, and in such situation, In 2017, a case study by Prof. Staecker and his colleagues
MRI compatible hearing implant system prevails over the from Kansas in the USA demonstrated the clinical import-
MRI non-compatible hearing implant systems. ance of MRI compatible implant magnet in ABI patient
[16]. The latter was a 27-year-old female with a bilateral

Figure 18. SYNCHRONY ABI system showing both single unit and BTE audio processor (A). The implant has a 1.5 T conditional MRI compatible magnet that self-
aligns to the external magnetic field (B) (image courtesy of MED-EL).
 ACTA OTO-LARYNGOLOGICA S73

vestibular schwannoma, and she was under observation for between tumour size and hearing outcomes [19]. The
some time before opting for MED-EL’s SYNCHRONY ABI latter was further confirmed with a study conducted by
implant system for her left side. After surgery, the patient Prof. Behr and his colleagues [18] in which they –
underwent postoperative MRI scans for tumour growth based on the tumour size – grouped MED-EL’s ABI
observation on her right side. The MRI compatible implant implanted patients (n ¼ 26) to one group with tumour
magnet allowed the MRI scans without the need for implant size 3 cm and the second group with tumour size of
magnet removal and attainment of clear and quality images >3 cm. The sentence recognition score did not show
of the contralateral side (Figure 20). any significant difference between these two groups (p

Figure 20. MRI with MED-EL’s SYNCHRONY ABI implant demonstrated clear and quality images of the contralateral side. The ABI created moderate metallic artefact
distortion that limits evaluation of the ipsilateral cerebral and cerebellar hemispheres. Image adapted from Shew et al. [16].

3.11. Factors which affect hearing performance ¼ .83, two-tailed t-test). Some patients with a tumour
amongst ABI patients [17–19] size of >4 cm were able to obtain >80% sentence rec-
ognition test scores correctly.
The ABI has shown acceptable hearing outcomes in 2. Tumour stage is another factor that was investigated,
patients, including in children with both, NF2 and non- and it showed no difference in hearing outcomes
tumour conditions. Children implanted at 2–3 years of age between patients with tumour stage III or IV. Tumour
have developed open-set speech recognition and have stage IV corresponds to tumour size >5 cm, and which
attended general education [17]. Although these results are compresses the brainstem – this suggests that patients
encouraging for implantation of ABI for various indications, with large tumours (>5 cm) may reach open-set scores.
there is considerable variability in hearing outcomes, rang- 3. Length of deafness before ABI surgery is seen as another
ing from high levels in open-set speech recognition to only contributing factor towards variability in hearing out-
sound awareness and a supplement of lip-reading. The comes amongst ABI patients. Group of patients who
aforementioned makes it challenging for clinicians to discuss became deaf 1 year or less before implantation (n ¼ 18)
the benefits of ABI during preoperative patient counselling obtained significantly higher speech recognition scores
sessions. There could be several factors contributing to the (65% correct scores), compared to the group of patients
variability in hearing outcomes. In order to have a clear whose deafness duration lasted more than 1 year before
understanding of these factors, Prof. Behr and clinicians ABI surgery (n ¼ 7; 45% correct scores; p ¼ .03, two-
from different centres across the world investigated the tailed t-test).
hearing outcomes on all patients implanted with MED-EL’s 4. The surgical effect on minimising the CN damage is
ABI hearing system with open-set speech understanding another contributing factor to the hearing outcomes.
scores included [18] (Figure 21). Open-set speech under- Semi-sitting position showed better speech recognition
standing is defined as speech understanding is 30% with- scores compared to the lying position. In a semi-sitting
out lip-reading on sentence test. One-third of the NF2 position, the intraoperative bleeding is minimised due
patients implanted with MED-EL’s ABI system reached to reduced intracranial venous pressure. Bipolar elec-
open-set speech understanding. trocautery and associated excitotoxic effects on the
neural elements in CN could be another explanation
1. NF2 tumour size of larger than 2–3 cm in diameter for the variations in hearing outcomes with ABI in
would impinge on the brainstem surface, and the surgi- general. Surgical techniques of exposing auditory
cal removal of the tumour would cause some damage brainstem (translabyrinthine (TL) vs retrosigmoid (RS)
on the brainstem. However, earlier reports on the ana- approach) could also contribute to a certain degree in
lysis of hearing outcomes related to tumour size with the hearing outcomes, as it was reported by Prof. Behr
excluding other factors have shown no correlation and his colleagues [18] with findings that the RS
S74 A. DHANASINGH AND I. HOCHMAIR

and his colleagues [17]. Prof. Behr and his colleagues

have also obtained similar findings with MED-EL’s ABI
implanted patients [18]. They categorised patients into
three groups based on the number of activated elec-
trode contacts. Group I had five to seven active elec-
trode contacts, group II had eight to nine, and group
III had ten to twelve electrode contacts active. The cor-
responding mean sentence recognition scores were 52%,
70.3% and 54% correct, respectively. Two-tailed t-test
comparisons revealed no significant differences amongst
the three groups, with p values ranging from 0.08
to 0.90.
6. Not all activated channels produce distinct pitch sensations.
During the ABI audio processor fitting, multiple channels
with similar pitches may be included hoping that they will
contribute to independent spectral information, even if
they are perceived to be of similar pitch. Interestingly,
with MED-EL’s ABI device, none of the patients who had
distinct pitch sensation on all 12 channels reached open-
set speech understanding scores. Patients with better
speech understanding (60%) have a higher number of
distinct pitch electrodes (9.1 contacts) than patients with
poorer speech understanding (<60%) with 7.4 contacts.
The minimum number of distinct pitch electrodes to
reach the open set was found to be five.
7. Maximum comfort level (MCL) may be an indicator of
distance between the electrode channel and stimulated
nerve, and in that sense, higher current levels are
required with increased distance. With MED-EL’s ABI
system, Prof. Behr and his colleagues [18] showed that
the patient group with MCL <28 nC reached higher
sentence recognition scores (67.1%) than patients with
higher MCLs (>28 nC) and which are typically seen in
CI patients. This result suggests that the ABI paddle
electrodes placed proximal to neural structures posi-
tively relate to the hearing performance.
8. During ABI surgeries, intraoperative recordings of eABR
would assist the surgeon in optimal placement of the
Figure 21. Clinicians from different centres investigated the factors contribu-
ting to variability in ABI outcomes: 1 Klinikum Fulda, Germany; 2 University of paddle electrode on the CN. Auditory responses are
W€urzburg, Germany; 3 University of Verona, Italy; 4 University of Tokyo, Japan; 5 peaks in the recorded responses with a latency of five to
NTT Medical Center Tokyo, Japan; 6 University of Bordeaux, France; 7 Institute of six milliseconds, and the amplitude of the peaks grows
Physiology and Pathology, Poland.
with increased stimulation level. In a typical hearing
mechanism, the number of peaks in eABR ranges from
approach results in 30% higher speech recognition one to five, corresponding to the mixture of responses
scores in comparison with the TL approach. from the auditory nerve and brainstem nucleus. With the
Nevertheless, such a comparison was not possible due ABI paddle electrode stimulating the brainstem nucleus by
to the small number of patients with open-set scores bypassing the auditory nerve, there is no auditory nerve
with the TL method. response as a result, and the latency of peaks related to
5. Another factor that may be thought to influence hear- brainstem nucleus is shorter due to no mechanical delays
ing outcomes is the number of stimulating channels in of the cochlea or its nerve. The eABR recordings may be
the paddle electrode. MED-EL’s ABI paddle has twelve categorised by having one, two or three distinct peaks,
active stimulating channels, whereas the ABI paddle depending on the optimal placement of the paddle elec-
from CochlearV has twenty-two. If the number of active
R
trode, as well as they relate to the health of neural ele-
stimulating channels acts as the deciding factor in better ments in the brainstem nucleus. The study by Prof. Behr
hearing outcomes, then theoretically, all the CochlearV
R
and his colleagues of using MED-EL’s ABI device showed
ABI implantees should outperform MED-EL’s ABI two peaks in most eABR recordings, and these recordings
implanted patients. However, scientific facts reveal the were obtained from patients who had good hearing out-
opposite, according to the report from Prof. Colletti comes. However, there was no significant correlation
 ACTA OTO-LARYNGOLOGICA S75

between the number of peaks and the level of speech rec- mainly due to the supply of sensory information through CI
ognition [18]. This data is inconclusive due to the fact to the developing brain while the developmental plasticity is
that not all ABI patients in this cohort had eABR meas- still strong [20]. However, children with the absent auditory
ured from the implant directly as this was only possible nerve – and therefore not CI candidates – are a more chal-
after the year 2008. lenging patient population. If early CI implantation in chil-
9. The rate of stimulation on each electrode is related to dren could bring better hearing outcomes, then this should
the MCL levels, and the stimulation rate is determined also happen with ABI in children who are CI
by the number of stimulating electrode channels and contraindicated.
the duration of each stimulation pulse. MED-EL’s ABI In 2014, Prof. Colletti and his colleagues reported on
device used either CIS þ or HDCIS signal processing hearing outcomes from a consecutive group of sixty-four
strategy until the year 2011, and the default program- children who they followed up for 12 years postimplantation
ming mode of applying biphasic pulses had a min- with ABI. The children had a variety of aetiologies, includ-
imum pulse width of 7ms, which allowed stimulation ing cochlear nerve aplasia, auditory neuropathy (AN), coch-
rate to up to 4225 pulses/second/electrode. For lear malformations with dysplasia of the eight nerve,
patients who required higher thresholds and current and other cochlear abnormalities. The patients were initially
levels, the pulse duration was lengthened, and in fitted with a CI but with no sound detection outcomes [21].
patients with open set, the overall stimulation rate Their findings revealed a positive trend toward better
started as low as 377 pulses/second/electrode. outcomes with a CAP score of seven in children implanted
However, grouping patients with stimulation pulses with an ABI at a very young age – at 2 years old
higher or lower than 1200 pulses/second/electrode, the (Figure 22(A)) – and particularly no other disorders
speech recognition scores differed significantly (67.1% (Figure 22(B)).

Figure 22. High CAP score of seven was recorded in patients implanted with ABI as young as two years of age (A). High CAP score was identified in patients with
cochlear ossification and trauma, which is not considered as a disorder when compared with a condition like NF2 and AN (B) [21]. Reproduced by permission of
Karger AG, Basel.

vs 45.7%; p ¼ .03, two-tailed t-test) [18]. This suggests In 2015–2016, MED-EL sponsored a study to evaluate
that higher stimulation rate positively influences the safety and efficacy of ABI in children, and the study
speech performance. In contrast to the MED-EL’s sys- took place in Chennai in India, at the Madras ENT
tem, SPEAK strategy from CochlearV that is typically Research Foundation (MERF) [22], led by Prof.
R

used in ABI patients has the mean stimulation rate of Kameswaran and Dr Rajeswaran (Figure 23).
only 250 pulses/second/electrode.

While these are the widely studied factors which contrib-

ute to the variability in hearing performances of ABI
patients with NF2, the optimal identification of the CN, its
size variations and the tonotopic arrangement of neural ele-
ments – which are hard to understand in patients in-situ –
may also be other contributing factors. Figure 23. Team of CI/ABI 1 surgeon and 2 audiologist from Madras ENT
Foundation (MERF) evaluated the safety and effectiveness of MED-EL’s ABI hear-
ing system in young children with various forms of inner ear malformations.

3.12. The importance of early ABI implantation

Ten children were included in the study, with the mean
in children
age at implantation of 3.5 ± 1.3 years. Michel’s aplasia, coch-
Prelingually deaf children make remarkable advances in lear nerve hypoplasia and cochlear aplasia were the causes
auditory perception following CI implantation, and this is of their deafness, and all children received either MED-EL’s
S76 A. DHANASINGH AND I. HOCHMAIR

PULSAR ABI or CONCERTO ABI implant with OPUS 2 3.13.1. Bilateral ABI with a single implant
audio processor. As far as the safety of the device was con-
It is known from the CI field that bilateral CI implantation
cerned, there were no reports on device failure other than
one adverse event related to device or surgical procedure. brings binaural hearing benefits in patients with bilateral
The hearing performance was measured through different deafness. If bilateral CI bring binaural benefits, then bilateral
assessment methods, including Listening Progress Profile ABI should result in the same. MED-EL thought that if two
(LiP), Meaningful Auditory Integration Scale (MAIS), independent ABI electrode arrays could be implanted to
Meaningful Use of Speech Scale (MUSS), Monosyllabic- Foraminae of Luschkae (Figure 24(A)) in one surgery bilat-
Trochee-Polysyllabic (MTP), Categories of Auditory erally, that would be highly beneficial in terms of reducing
Perception (CAP), Speech Intelligibility Rating (SIR) scale, the surgical complications, as well as of associated surgical
the LittlEARS Auditory Questionnaire (LEAQ), and the costs. Under the Custom Made Device (CMD) council dir-
Checklist of Auditory Communication Skills. Tests were ective 93/42/European Economic Community (EEC) [24], a
performed preoperatively and compared with the twelfth modified ABI implant with a SPLIT electrode lead with two
postoperative month results. Significant improvements in branches of ABI paddle electrode was designed and devel-
LiP (p ¼ .012), MAIS (p ¼ .008), MUSS (p ¼ .011), MTP 3 oped by MED-EL to be implanted in a patient who suffered
(p ¼ .042), CAP (p ¼ .011), SIR (p ¼ .008), LEAQ (p ¼ .008) from tumours in the midline of the posterior fossa, and
and in the Checklist of Auditory Communication Skills who was also expecting to undergo spinal cord surgery.
(p ¼ .012) results were observed from preoperation to 12 Prof. Behr from Klinikum Fulda in Germany performed the
months postoperatively. Performance from 12 months to 24 surgery to implant this CMD ABI device in the year 2009,
months after first fitting significantly improved or remained although its results were published only in 2018 [25]. The
stable for every test. A significant improvement was reached intraoperative eABR recordings and postoperative CT
for MAIS (p ¼ .028), MUSS (p ¼ .041), SIR (p ¼ .046), LEAQ images confirmed the proper placement of the two ABI pad-
dle electrodes (Figure 24(B)).

Figure 24. The surgical image of SPLIT ABI electrode showing two branches of electrode lead that goes into both Foraminae of Luschkae (A). Postoperative CT
image showing the proper placement of ABI paddle electrodes bilaterally [23].

(p ¼ .019), and the Checklist of Auditory Communication The left side paddle electrode had dislocated after 3
Skills (p ¼ .021). Performance remained stable for LiP months, but due to patient’s precondition, it was decided
(p ¼ .090), MTP 3 (p ¼ .0141). The study concluded that not to perform revision surgery to reposition it. The authors
ABI provision and use is safe and allows significant auditory concluded that the special device design and the surgical
development in children with cochlear (nerve) aplasia or experience of placing two paddle electrodes in a single sur-
hypoplasia and without NF2. gical procedure is feasible and safe for the patient.
In 2017 September, MED-EL was finally granted the CE
mark for its SYNCHRONY ABI system to be implanted in
3.13.2. Midbrain implant
non-tumour children as young as 12 months and older.
This was a significant milestone in MED-EL’s journey with Depending on schwannoma size and the surgical procedure,
its ABI system, and the approval was obtained based on the some damage may be induced to the CN upon schwannoma
two abovementioned studies along with an expert opinion removal. Prof. Colletti attempted to place MED-EL’s ABI
that was given by Prof. Behr from Klinikum Fulda in paddle electrode on the inferior colliculus (IC) as shown in
Germany to the notified body. Figure 25 – which is one step higher in the auditory path-
way [25] – of a patient previously treated with ABI with the
paddle electrode placed on the CN, but with limited speech
3.13. New concepts tried with MED-EL ABI system
recognition results.
This section will describe the key novel concepts that were The results of the first patient with IC implant showed
tried with the MED-EL ABI system. substantial benefit from electric stimulation with no
 ACTA OTO-LARYNGOLOGICA S77

Figure 25. Schematic of the surgical approach and electrode placement on the IC (A). X-ray image of the implant placed on the IC taken at a slightly oblique
angle. The red circle points to the ABI paddle electrode [26]. Reproduced by permission of Wolters Kluwer Health, Inc.

complications or side effects. All twelve electrodes produced processor based on postoperative eABR [8]. From a group of
auditory sensations with no non-auditory responses. The children with a mean age of 2.4 years (n ¼ 17), the postopera-
threshold levels were as low as 5nC, indicating the array in tive eABR recordings showed differences in the number of
excellent position over auditory pathway structures. The waves. This is also the first study detailing the morphology of
patient was able to discriminate multisyllabic words at 80% eABR responses in congenitally deaf children implanted with
correct in a five-choice test that was based on prosodic and ABI. The most robust was the wave P2 that appeared from
syllabic cues. Face-to-face communication with the implant 1 ms to 2.5 ms, and which was present in all measured eABRs.
demonstrated a significant improvement in speech under- Wave P3 occurred between 1.7 ms and 4.5 ms and wave P4
standing over lip-reading alone. Recognition of words and between 3.5 ms and 5 ms. Another key finding was that waves
sentences increased from 5% and 10% to 80% and 90%, P3 and P4 were present only in the presence of P1 and P2,
respectively, after the revision surgery. These results indicate and the nonauditory responses that were confirmed by the
that the stimulation on the surface if the inferior colliculus observation, subjective responses, or both, were recordable
in the auditory midbrain could provide an alternative possi- only after 2.5 ms. These findings make the wave P2, which
bility for auditory prosthetic devices. appeared between 1 ms and 2.5 ms, a prominent element and
its presence in the postoperative eABR could be used during
the ABI audio processor fitting in small children. This data
3.13.3. A Novel method in the audio processor fitting of
suggests that postoperative eABR fitting could help toddlers
children with ABI
implanted with ABI to achieve auditory perception and devel-
One of the main challenges in providing small children with opment quickly.
an ABI is the fitting of their audio processor, as reliable
subjective feedback from them is challenging to obtain.
3.13.4. Tools in preoperative assessment
With the ABI indication criteria expanding in toddlers,
there was a need to develop an easy and reliable method to The goal of the preoperative assessment tools is to minimise
perform the fitting of the audio processor. eABR is an estab- the time of hearing deprivation in questionable candidates,
lished method and the gold standard in ABI, and it is com- who would typically not be implanted or implanted with a
monly used during the ABI paddle electrode positioning on question mark, and to help the implant team to decide
the CN during surgery. Until the end of the year 2008, which implant is the best choice for each candidate. Both
eABR recording was only possible with the ABI placement tools discussed below were developed for the MED-EL’s
electrode, which meant that immediately after selecting a MAESTRO 9.0 clinical system and required only a dedicated
location with the best response, the surgeon had to place evoked potential measuring system [26].
the ABI paddle electrode precisely on such spot on the CN. In some instances, candidates show no response or a ques-
The method represented a challenge to the operating sur- tionable response to sound whilst diagnostic imaging tests sug-
geon, but at the beginning of 2009, MED-EL’s ABI implant gest normal or abnormal anatomy. This may occur in patients
was enhanced with the eABR feature within the paddle elec- with a narrow internal auditory canal or patients with either
trode, which ensured validation of the best placement. malformed or patent cochlea. For such cases, the preoperative
From literature, it is known that nonauditory sensation by Promontory Stimulation System was developed. Its benefits
some electrode contacts in the ABI paddle electrode is always were evidenced in initial studies with a success rate of 80–90%
present and therefore finding these specific electrode contacts in CI implanted children. The system intends the transtym-
is also challenging in small children. In the year 2018, Prof. panic electrode to be placed on the round window niche, and
Colletti from the University of Verona and Dr Polak from biphasic electric pulses are delivered to the transtympanic elec-
MED-EL developed a novel method of fitting the audio trodes. At the time of stimuli, the MAX interface triggers the
S78 A. DHANASINGH AND I. HOCHMAIR

evoked potential device, and the eABR response is obtained 3.14. Star performance with ABI
from the surface electrodes, as shown in Figure 26. If the
eABR shows a positive response, the implant team may decide It is known from the literature that NF2 patients implanted
to proceed with cochlear implantation. If no responses are with ABI, their hearing performance may unfortunately not
obtained, the candidate may be considered for an ABI, or fur- be reported as excellent mainly due to complications associ-
ther tests may be required. ated with the tumour itself and the surgical removal of it

Figure 26. Transtympanic rounded-bent tip electrode that facilitates easy placement at the RW niche (A). Illustrative representation of the transtympanic electrode
placement at the RW niche (B). PromStim eABR responses for all 11 patients (C) [27] (image courtesy of MED-EL).

In situations where an individual shows no response or affecting the cochlear nucleus. This section showcases few
is expected to have no response to the sound, and where NF2 subjects implanted with MED-EL ABI devices, who are
imaging tests show normal or abnormal anatomy, or where star performers with their hearing abilities.
the individual has already been selected for either a CI or In 2000, Skarzynski et al. reported implanting MED-EL
an ABI, an intraoperative test of nerve functionality may Combi40þ ABI device in a 28-year-old woman with bilat-
be used. This test includes placement of the cochlear test eral deafness caused by NF2 [28]. This was the first ever
electrode into the scala tympani (ST). ABI surgery in Poland. The surgery took place at the
The intra-cochlear test electrode contains four electrode Institute of physiology and pathology of hearing, Warsaw,
contacts. It is intended to be inserted into the ST during Poland with the support of neurosurgeons from the
surgery. The length of the electrode is 18 mm, as indicated University of W€ urzburg. Limited migration of the ABI pad
by the marker ring. Three of the electrode contacts are electrode was observed a few weeks after surgery and eight
placed directly into the ST, and the fourth electrode con- channels were finally stimulated using a CIS speech coding
tact is placed under the temporalis muscle. Biphasic pulses strategy. She was able to detect and identify most environ-
are generated using the MAX interface and delivered to mental sounds and was able to hear music. There was a
the cochlea. At the time of stimulation, the MAX interface continuous improvement of her auditory skills and very
triggers the evoked potential, and eABR response is importantly, no changes in the stimulation parameters nor
obtained from the surface electrode as depicted in in the electrode placement. She continued her profession as
Figure 27. a Polish to German language translator, was taking care of
This tool is suitable for individuals with questionable her children, speak over the telephone and was able to learn
functionality of the auditory nerve, individuals with a nar- Italian as a third language using tapes and books
row internal auditory canal and patent or malformed simultaneously.
cochlea, in tumour patients to monitor nerve functionality In 2009, Skarzynski et al. reported sequential bilateral
during the tumour removal, or in situations where ABI (MED-EL Combi40þ) in a 27-year-old man with NF2
any other tests/methods failed to show CI candidacy, [29]. The first implantation took place on 4 April 2006 and
including the use of eABR with the Promontory the second implantation on 26 June 2008. Both surgeries
Stimulation System. were led by Prof. Robert Behr from the University of
 ACTA OTO-LARYNGOLOGICA S79

Figure 27. Intracochlear test electrode and test set-up in recording the eABR responses (image courtesy of MED-EL).

W€urzburg, Germany. This patient continued his profession  Implantation concurrent with tumour removal surgery
as a singer even after the ABI surgery, owned a business,
and remained very active.
While these two studies are published evidences, there 3.16. Reimbursement from the healthcare system
are a lot more ABI star performers who were implanted Reimbursement from the healthcare system is a topic of
with MED-EL ABI device. commercial importance, as well as it serves as a direct
acknowledgement of the acceptance by the medical society.
In 2020, Health Quality Ontario declared that compared
3.15. Current (the year 2021) eligibility criteria for with no intervention, ABI provides benefit for completely
MED-EL’s SYNCHRONY ABI system deaf adults with NF2 or severe inner ear abnormalities, con-
The following conditions are indicated safe for treatment traindicated for CI [30]. Surprisingly, in well-developed
with MED-EL’s SYNCHRONY ABI system: countries like Australia, Belgium and the USA, ABI is not
reimbursed by the healthcare systems in general. In contrast,
 Twelve months or older in European countries such as in Austria, Denmark,
 Cannot benefit from a cochlear implant Finland, France, Germany, Netherlands, Norway, Poland,
 Nonfunctional auditory nerve: Portugal, Russia, Slovakia, Spain, Sweden, Switzerland and
 Auditory nerve aplasia in countries like Iran and Turkey, the cost of ABI
 Auditory nerve hypoplasia is reimbursed.
 Head trauma By 2021, more than three-hundred and fifty non-NF2
 Non-NF2 tumour children of age down to 1 year, were implanted with MED-
 Severe cochlear ossification EL ABI system in 30 countries world-wide involving
 Neurofibromatosis type 2 (NF2) 57 surgeons.
S80 A. DHANASINGH AND I. HOCHMAIR

3.17. MED-EL’s commitment towards ABI candidates and the topics that were discussed were prelingual deafness
indication, possible congenital ABI indications, ABI out-
As ABI surgery involves certain complications and risks as
comes and surgical procedure in ABI reimplantation.
discussed throughout this chapter, MED-EL has dedicated
The translational science path with the ABI paddle elec-
electrophysiological assessment experts to attend every ABI
trode design originated in the laboratories of Innsbruck and
surgery as to ensure optimal placement of ABI paddle elec-
W€ urzburg universities to later reach the patients in restoring
trode over the CN and optimal eABR measurements. Also,
hearing and has culminated in the regulatory approval of
MED-EL covers all financial costs associated with bringing
the MED-EL ABI, which is the only ABI system with CE
in an expert paediatric electrophysiologist during ABI sur-
geries in countries where such experts are lacking. While mark and other regulatory approval for not only NF2
these overhead costs are relatively high and, in many cases, patients but for non-tumour patients including children
exceed the break-even price of the device, MED-EL contin- down to 12 months of age.
ues its mission of providing hearing to every individual, and
especially to children. Up to date (2021), MED-EL has sup- Acknowledgments
ported more than six hundred ABI cases worldwide, and
this journey has just started as many more milestones are to The authors would gratefully like to acknowledge the key contributors
to the development of the subject matter. Their contributions are out-
be achieved in the years to come for MED-EL (Figure 28). lined in this article. The authors further acknowledge Marek Polak
from MED-EL for his valuable input and comments during several
rounds of review meetings that contributed to the final version of
this article.

Disclosure statement
This article is sponsored by MED-EL and has not undergone the regu-
lar peer-review process of Acta Oto-Laryngologica. Both the authors
are affiliated with MED-EL.

Figure 28. Electrophysiological experts from MED-EL who attend every MED-EL
ABI surgery. References
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[2] Blakeley JO, Evans DG, Adler J, et al. Consensus recommenda-
3.18. Conclusion
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cians globally and to strive to deliver the best hearing 2012;158A(1):24–41.
[3] Morrow KA, Shevde LA. Merlin: the wizard requires protein
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However, one-third of MED-EL implantees have shown to dren with auditory brainstem implants. Eur Ann
have more than 30% correct open-set speech scores. MED- Otorhinolaryngol Head Neck Dis. 2018;135(6):403–409.
EL continues with technologically advancing and further [9] Colletti V, Carner M, Fiorino F, et al. Hearing restoration with
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improving its ABI implant system, stimulation strategies
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