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Objective: This study was designed to investigate the derstanding, two subjects from this series (7.4%) did
perceptual benefits and potential risks of implant- receive sufficient benefit to be able to use the ABI in
ing the Nucleus® multichannel auditory brainstem conversation without lipreading.
implant.
Conclusions: Although the medical risks and surgi-
Design: Between September 1992 and October cal complexity associated with ABI device implan-
1997 a total of 27 subjects received a Nucleus 20- or tation are far greater than those for a cochlear
21-channel Auditory Brainstem Implant (ABI). All implant, the clinical results from this trial show
subjects involved in the trial had bilateral acoustic that the Nucleus multichannel ABI is capable of
tumour as a result of neurofibromatosis type 2 providing a significant patient benefit over risk
(NF2) resulting in complete dysfunction of the ratio for subjects suffering loss of hearing due to
VIIIth nerve. The study used each subject as their bilateral retrocochlear lesions.
own control without a preoperative baseline be-
(Ear & Hearing 2002;23;170–183)
cause residual hearing, if existing, was destroyed at
surgery by tumour removal. A battery of speech
tests was conducted to evaluate each patient’s per-
formance and communication abilities. Tests were The Nucleus Cochlear Implant System has long
conducted, where possible, in the auditory-only, been proven to provide a safe and effective restoration
visual-only, and auditory-visual conditions at 3 of hearing (NIH Consensus Statement, 1995; NPCIP
days postoperatively (baseline), at 3-mo intervals Progress Report, Reference Note 4; Summerfield &
for the first year and every 12 mo thereafter. A Marshall, 1995) to those with a profound or total
subjective performance questionnaire was adminis- sensorineural hearing loss. Although effective for
tered together with an extensive neurological ex- many cochlear pathologies, it is ineffective for those
amination at each test interval. with dysfunction of both VIIIth nerves. In 1984, Hit-
Results: 27 subjects involved in this trial were suc- selberger first reported the successful application of
cessfully implanted with a Nucleus ABI. One subject electrical stimulation of the cochlear nucleus (CN)
died 2 days postoperatively due to a lung embolism within the lateral recess of the 4th ventricle to restore
unrelated to the device. Twenty-six subjects under- a degree of auditory sensation after the removal of an
went device activation and all but one patient re- acoustic tumour in a patient with NF2 (Hitselberger et
ceived auditory sensation at initial stimulation
al., 1984). This subject was stimulated by simple
(96.2%). On average 8.6 (ⴞ4.2) of the available 21
electrodes were used in the patients’ MAPs. Perfor-
bipolar electrodes across the CN and connected to a
mance evaluation measures showed that the major- single channel cochlear implant.
ity of users had access to auditory information such Due to advancements in cochlear implant design, in
as environmental sound awareness together with 1992 a pilot study was initiated by Laszig et al., based
stress and rhythm cues in speech that assist with on a multichannel ABI design with 20 electrodes
lipreading. Although most subjects did not achieve manufactured by Cochlear Limited, Australia (Laszig
any functional auditory-alone, open-set speech un- et al., 1991). The first subject, a 52-yr-old lady with
NF2, was implanted in September of that year. Re-
Cochlear Europe Ltd (B.N.), London, United Kingdom; Albert-
Ludwigs Universität (R.L.), Freiburg, Germany; Städtisches
sults from this subject have been reported elsewhere
Klinikum (W.-P.S.), Braunschweig, Germany; Medizinische (Laszig et al., 1995; Laszig, Reference Note 2) and
Hochschule (T.L.), Hannover, Germany; L’Hôpital Beaujon (O.S.), demonstrated that the ABI could provide auditory
Paris, France; Manchester Royal Infirmary (R.R.), Manchester, sensations that restore a sense of environmental
United Kingdom; CHU Purpan (B.F.), Toulouse, France; Clinica awareness and assist lipreading. During the pilot
Universitaria de Navarra (M.M.), Pamplona, Spain; University of
Uppsala (H.R.-A.), Uppsala, Sweden; Instituto de Otologia (E.G.-
study a total of 10 subjects were implanted and several
I.), Barcelona, Spain; University of Verona (V.C.), Verona, Italy; reports of performance have been presented (Laszig et
and Cochlear AG (E.v.W.), Basel, Switzerland. al., 1994; Laszig, Reference Note 1; Marangos et al.,
0196/0202/02/2303-0170/0 • Ear & Hearing • Copyright © 2002 by Lippincott Williams & Wilkins • Printed in the U.S.A.
170
EAR & HEARING, VOL. 23 NO. 3 171
Subjects
Selection criteria for subjects in this trial in-
cluded cerebello-pontine angle tumors or head
trauma leading to complete, bilateral VIIIth nerve
dysfunction. Subjects were required to be 18 yr of
Figure 1. The Nucleus 21-channel Auditory Brainstem Implant age or older and could be implanted on either their
(ABI) based on the Nucleus 22 receiver/stimulator package. first or second tumour side, but not both. Surgery
was recommended to be performed concurrent with
tumour removal, although in two subjects this took
1997; Shin et al., 1997). The pilot study allowed a
place as a separate procedure. Subjects were re-
period for modifications to the design of the ABI to
quired not to have undergone any preoperative ste-
accommodate intra-subject variations in the anatomy
reotactic radiosurgery (e.g., gamma knife) due to
of the lateral recess. The final design was a 21-channel
concerns expressed by some investigators about the
device. Both the 20 and 21-channel ABI designs were
degenerative effect this may have had on the struc-
based on the Nucleus CI22M cochlear implant with a
ture of the cochlear nucleus and the reduced likeli-
modified electrode array. A clinical investigation based
hood of electrically induced auditory sensation.
around an 8-channel ABI electrode attached to a
Implantation of the ABI (Brackmann et al., 1993) is
CI22M cochlear implant was initiated in 1992 and has
an extension to the procedure used for the removal of
also been reported (Ebinger et al., 2000; Otto et al.
an acoustic tumour combined with a mastoid bed
1998).
excavation for seating the implant’s receiver/stimula-
After the pilot study, a multi-center clinical inves-
tor body as per a regular Nucleus CI22M surgery.
tigation was initiated in February 1996, involving
Access to the lateral recess required to place the ABI
10 centers from six European countries. This report
was achieved by either the translabyrinthine or ret-
details the results and the associated surgical expe-
rosigmoid/suboccipital approaches used for tumour
riences and risks from both the 10 pilot study and 17
removal; however, the former approach is favored by
clinical trial subjects.
many surgeons as the angle of access to the CN may
serve for better visualization and positioning of the
METHODS array. Verification of correct electrode placement and
function of the ABI device was confirmed by intra-
Device Description operative electrically evoked auditory brainstem re-
The Nucleus 21-channel auditory brainstem im- sponses (EABR) (Waring 1995a, 1995b).
plant (CI21⫹1M) used for the clinical trial (Fig. 1) Initial stimulation took place around 6 wk after
incorporated a silicone elastomer electrode carrier, surgery and would normally occur over the course of 2
8.5 mm long ⫻ 3.0 mm wide ⫻ 0.6 mm thick. On one to 3 days. Auditory threshold and comfort levels were
face of this carrier were 21 platinum disk electrodes established together with any associated nonauditory
0.7 mm in diameter, arranged in three diagonally sensations. Only electrodes with purely auditory sen-
offset rows. The electrodes were connected to a sations were included in the final MAPs these being
Nucleus CI22M receiver-stimulator by a silicone ranked from lowest to highest pitch as reported by the
elastomer lead 1.2 mm diameter and 11 cm long patient or, if not possible, in numerical order (2 ⫽
containing 21 individually insulated, helically lowest pitch, 22 ⫽ highest pitch). Electrical stimula-
wound, 25 micron platinum/iridium (90%/10%) tion levels were monitored according to a published
wires. Attached to the rear surface of the carrier was model for safe charge and charge density (McCreery et
a special narrow-weave mesh cut into a “T.” The al., 1990; Shannon 1992).
mesh promotes fibrous tissue growth helping to fix To assess clinical performance the study used
the array in situ. each subject as their own control. The study itself
The receiver/stimulator was identical to the Nu- did not prescribe a preoperative baseline as the
cleus CI22M cochlear implant although the final surgery to remove the tumour, more commonly by
172 EAR & HEARING / JUNE 2002
Number test
SWEDISH
Not tested
Not tested
25% (R)
Spondees
residual hearing. As a consequence, without the
5% (R)
6.3% (L)
11% (L)
ABI, all open-set speech perception scores would be
expected to be at or close to zero and closed-set
speech perception scores close to the random level.
Nevertheless, wherever hearing-preservation sur-
gery was a viable management option this was
Innsbrucker sentences
MTS words (patterns)
multisyllabic words
(open-set) or TAPs
MTS words 8.3% or
⬃1% (L)
line was established 3 days after initial device acti-
vation. Subsequent evaluations were conducted at 3
mo intervals for the first postoperative year then
every 12 mo thereafter.
Postoperative performance assessment was based
Number test ⬃1% (L)
MTS words (patterns)
(open-set) (L)
10% (L)
8.3% (L)
20% (L)
25% (R)
12.5% (R)
4% (R)
8.3% (R)
(open-set) (R)
BKB sentences
Sentence recognition
Word identification
removal to be tracked.
Owing to detail differences between the tests in
different languages and also to the general poor
health of many ABI patients, which prevented a full
EAR & HEARING, VOL. 23 NO. 3 173
Subject Age at Tumor Size Implant 1st or 2nd Operation Surgical Follow-up
(Nationality) Sex Implantation (yr) Etiology (largest diameter) Side Side Date Approach Clinic
1 HK (D) F 52 NF2* 1.5 cm L 1st 7 Sep 92 T.lab Freiburg
2 GS (D) M 58 NF2 1.2 cm L 1st 23 Nov 93 T.lab Freiburg
3 AL (F) M 31 NF2 7.0 cm L 2nd 25 Nov 93 T.lab Freiburg
4 SG (D) M 30 NF2 1.5 cm R 1st 10 Feb 94 T.lab Freiburg
5 JB1 (GB) F 33 NF2 2.0 cm R 1st 25 Feb 94 T.lab Manchester
6 RF (D) F 34 NF2 2.0 cm R 1st 27 Jun 94 T.lab Freiburg
7 PF (F) ✞ M 34 NF2 3.0 cm R 2nd 28 Jun 94 T.lab Freiburg
8 CH1 (D) M 29 NF2 5.0 cm L 2nd 14 Feb 95 T.lab Freiburg
9 FU (D) M 41 NF2 1.5 cm L 2nd 16 Feb 95 T.lab Freiburg
10 MP1 (Ru) M 17 NF2** 5.0 cm R 1st 14 Dec 95 T.lab Toulouse
11 ID (D) M 40 NF2 5.0 cm R 2nd 7 Feb 96 T.lab Freiburg
12 MK (GB) M 32 NF2 3.0 cm L 2nd 8 Feb 96 T.lab Manchester
(imp. Freiburg)
13 AT (GB) M 24 NF2 2.5 cm R 2nd 9 Feb 96 T.lab Manchester
(imp. Freiburg)
14 TL (D) M 29 NF2 1.0 cm L 2nd 7 May 96 R.sig Hannover
15 BC (D) F 35 NF2 4.5 cm R 2nd 25 Jul 96 R.sig Hannover
16 AA (D) M 51 NF2 0 cm R 2nd 25 Jul 96 R.sig Hannover
17 IG (F) F 30 NF2 0 cm R 2nd 23 Sep 96 T.lab Paris
18 MP2 (D) F 24 NF2 3.5 cm R 2nd 7 Nov 96 R.sig Hannover
19 TK (D) F 31 NF2 4.0 cm L 2nd 12 Nov 96 R.sig Hannover
20 NW (D) F 13 NF2** NR R 1st 24 April 97 T.lab Freiburg
21 CH2 (D) M 39 NF2 NR L 2nd 24 April 97 T.lab Freiburg
22 FD (F) ⫹ F 44 NF2 1 cm R 1st 5 May 97 T.lab Paris
blind
23 LP (N) F 42 NF2* 2.5 cm L 1st 21 May 97 T.lab Uppsala
24 JB2 (S) M 24 NF2* 3 cm R 1st 22 May 97 T.lab Uppsala
25 CR (E) F 20 NF2 2.5 cm L 1st 23 June 97 T.lab Pamplona
26 CM (F) M 30 NF2 3 cm R 2nd 30 June 97 T.lab Paris
27 SM (I) F 27 NF2 2.5 cm R 2nd 23 Aug 97 R.sig Verona
* Irradiated.
** Under 18 yr old.
test battery from being completed in many cases, all The period covering the pilot study subjects incor-
data in this report represent individual subject’s porates one major and two minor changes to the
performance and not group means and standard original ABI design. Only the first three subjects
deviations. were implanted with a significantly different device
This paper combines both pilot study and clinical comprising 20 rather than 21 electrodes and with a
trial subjects without exclusion so that it represents smaller diameter (0.5 mm c.f. 0.7 mm). All other
a comprehensive assessment of results and nonau- changes affected only the presupplied shape of the
ditory responses from the first 27 subjects in Europe Dacron®/PET (polyethylenetetratylate) mesh back-
to have received an ABI. The demographics of this ing on the electrode array. All subjects in the formal
group are shown in Table 2. clinical trial were implanted with the 21-channel
ABI design with modified mesh backing.
RESULTS
Between September 1992 and October 1997, 27 Surgery (N ⴝ 27)
subjects received a Nucleus 20-channel or 21-chan- The 27 subjects implanted with an ABI comprised
nel Auditory Brainstem Implant. Of this population, 12 females (44.4%) and 15 males (55.6%) with an
17 were implanted after February 1996, following average age of 33.1 yr (SD ⫽ 10.5 yr). The oldest
commencement of the multi-center clinical trial, of patient was 58 yr, the youngest was 13 yr (out of
which three patients were implanted out-of-protocol protocol). All subjects had an etiology of neurofibro-
for specific reasons detailed. Those subjects falling matosis type II. The average tumour size was 2.71
out-of-protocol or forming part of the pilot study cm (SD ⫽ 1.69 cm). Eleven subjects (40.7%) were
population are indicated accordingly. implanted on their first operated tumour side. Only
174 EAR & HEARING / JUNE 2002
Monopolar (MP) stimulation mode was used in 14 and scored either 100% or very close to it, the
(53.8%) subjects’ MAPs. Five (19.2%) subjects could lowest score being 80%. In the auditory-only condi-
only be programmed in bipolar (BP) mode without tion, scores were lower but still well above chance
nonauditory sensations and a further six subjects and often approaching the auditory-visual score.
(23.1%) found that a mixed monopolar/bipolar mode Vowel and consonant confusion tests were com-
provided a greater variety in pitch sensations for pleted by 16 subjects (Figs. 5 and 6). Thirteen
their MAP compared with either MP or BP mode subjects (86.6%) from 15 tested in the auditory-only
alone. condition showed scores well above chance levels in
Performance (N ⴝ 26)
All individual subjects scores are shown as a
mean value over the data collection period. Because
performance generally improved over time, indi-
cated scores generally underestimate the ultimate
benefit experienced by most subjects.
Of the 26 attempted initial stimulations 25 indi-
cated detection of sound from their processor in
response to either speech or environmental sounds
across the frequency spectrum (e.g., Ling 5 sounds)
presented at conversational levels (typically 65 to 70
dB SPL).
Sixteen subjects were tested using a closed-set
MTS word test scored by correct identification of
syllable patterns (Fig. 4). Fifteen of the 16 sub- Figure 4. Results of stress-pattern test (16 of 26 subjects tested
jects were tested in the auditory-visual condition in this condition)
176 EAR & HEARING / JUNE 2002
Figure 5. Results of vowel confusion test (17 of 26 subjects Figure 7. Results of closed-set word identification test (20 of
tested in this condition) 16 subjects tested in this condition)
vowel confusion. As expected, the overall level of and demonstrating a small decrement when com-
performance for consonant confusion in this condi- bined with lipreading, scoring 70% auditory visual.
tion was lower than for vowels. Nevertheless, eight In most other tests, this patient shows good lipread-
subjects (57.1%) from 14 show scores significantly ing enhancement indicating that the lower auditory-
above chance for this test. A further nine subjects visual score may be due to random error or a
(75.0%) of 12 tested in both the visual and auditory- reflection of the difficulty some patients face in
visual conditions on vowels, and 11 subjects from 12 adapting to the ABI alone.
(91.7%) on consonants, demonstrated benefit, some Open-set sentence recognition tests were con-
markedly, when using their ABI in conjunction with ducted by 17 subjects as shown in Figure 8. Eight
lipreading. subjects scored at or above 50% with four scoring
Closed-set word identification tests were con- above 80% in the auditory-visual condition, with a
ducted on 20 subjects as shown in Figure 7. For further six subjects achieving at least some open-set
those subjects tested in the auditory-visual condi- score. Furthermore for nine subjects out of 11 who
tion (18 subjects), nine scored 100% correct with the were also tested in the visual condition, there is
remainder scoring not worse than 66% correct. Au- clear lipreading enhancement. Three subjects tested
ditory alone scores collected for 16 subjects were were unable to achieve any score in any condition.
lower than for stress-pattern perception but still As this test represents a more real-life communica-
significantly above chance except for one patient RF. tion condition, clearly the majority of ABI users are
Only two subjects were tested in the visual-only benefiting from using their device in conjunction
condition, one scoring at chance level and demon- with lipreading. Two subjects even show scores in
strating great benefit from lipreading enhancement the auditory-only condition, one scoring an average
with the ABI, the other scoring at 80% visual-only of 68% and obtaining significant functional benefit
Figure 6. Results of consonant confusion test (17 of 26 Figure 8. Results of open-set sentence recognition test (17 of
subjects tested in this condition) 26 subjects tested in this condition)
EAR & HEARING, VOL. 23 NO. 3 177
Figure 11. Results from neurological assessment (completed for 16 of 17 subjects in formal trial)
From these results it can be seen that only the surgery, especially for a large tumour, reflected in
reflex response (from both top and bottom of the one occurrence.
spinal cord) and facial nerve function (commonly a In all cases, deficits observed postoperatively
facial palsy accompanied by eyelid, mouth and fore- were directly attributed to the tumour removal sur-
head muscle deficit, even taste) scored, on average, gery and not to the passive or active ABI.
worse than a mild deficit. In this population, all
patients exhibited reduced reflex responses as a
result of their tumour or previous surgery and not as Adverse Events
a result of the surgery performed at the time of ABI
An important aspect of the clinical investigation
placement. Hence only facial nerve function was
was to monitor both acute and chronic changes in
really affected—a common side effect from this type
either the patient’s health or performance after
of tumour removal. There were also three cases out
receiving their ABI. All performance data were ac-
of four of reduced function of the hypoglossal nerve
affecting movement of the tongue, probably due to crued over time and a formal record made of any
previous hypoglossal facial anastomosis because unexpected changes.
acoustic tumour removal via the translabyrinthine Two subjects (HK, RF) were observed to have
approach does not significantly risk damage to this experienced an increased sensitivity of the facial
nerve. nerve to electrical stimulation eventually leading to
Four single occurrences of a deficit in upper body nonuse. For subject HK, preoperative gamma knife
strength, lower body strength, lower body sensation, therapy (out of protocol) is thought to have contrib-
and glossopharyngeal nerve function (gag reflex and uted to a gradual decline in responsiveness of the
palate symmetry) were observed. Upper and lower CN to electrical stimulation. Over a period of 2 to 3
body strength deficit is most likely to be a result of a mo auditory thresholds steadily increased until the
reduced cerebrovascular supply caused by pressure patient no longer obtained auditory sensations be-
of the tumour on the cerebellum or manipulation fore the facial nerve side effect was sensed. A very
during its removal. Likewise for reduced sensory gradual recovery in the function of the facial nerve
function. The glossopharyngeal nerve, being further was observed in patient RF who, 6 mo after surgery
away from the tumour than the facial nerve, is less started to experience a reduction in her threshold for
likely to be affected but is still at risk during facial-stimulation until eventually it was below the
EAR & HEARING, VOL. 23 NO. 3 179
auditory thresholds that had remained substan- nant confusion tests further show that the ABI
tially constant throughout this change. provides most subjects with a limited ability to
One subject (AL) died 1 yr after initial device discriminate some basic temporal and spectral pat-
fitting. Death was due to subsequent surgery on terns. The particular improvement seen in the au-
spinal tumors and was unrelated to the use of the ditory-visual condition on these tests reveal that the
ABI. majority of subjects tested are able to combine both
One subject (MK) experienced a gradual but sig- the sound from their ABI with visual cues giving an
nificant increase in the frequency of epileptic sei- overall lipreading enhancement.
zures over a period of 6 mo from one to two per week As test materials start to get more complex,
to one to two per day. The occurrence of fits was performance with the ABI declines especially in the
unrelated to periods of ABI use. It was concluded auditory-only condition, and especially in compari-
that this increase was unlikely to be related to the son with cochlear implants. For example, although
ABI as no cranial nerve is known to affect epilepsy. closed set word identification tests show that limited
It is believed that these fits were a result of the auditory-alone word identification is possible, with
patient’s general medical condition and progres- average scores around the 50% mark, it would
sively worsening health as a result of a large and clearly not be possible for users to expect to rely on
developing supra-tentorial tumour. The patient had their ABI alone. Word identification is a rather
a history of epilepsy before receiving his ABI. artificial test and the advantages of the ABI in
A further patient (AT) experienced a temporary real-communication scenarios are better illustrated
threshold shift with adaptation 3 mo after initial with tests such as open-set sentence recognition
stimulation resulting in reduced sound quality. The where there is a lipreading enhancement seen for
cause for this threshold shift, adaptation and subse- most users. Because the results show key words
quent sound quality reduction has never been fully scored correctly, it is reasonable to assume that the
established. This subject was not on medication at ABI in combination with lipreading helps to identify
this time and the problem resolved itself over a those key words in real conversation that are impor-
period of 1 mo. This event has not been repeated and tant to establish context and thus make following a
this subject is currently one of the better performers. conversation easier. It is not unreasonable to as-
Finally, a single patient had a relatively rapid sume that word scores on related sentences would be
decline in performance after approximately 3 yr of further increased.
good device use. Combined CT and MRI investiga- The hardest tests performed were those looking
tion did not reveal any obvious movement of the at open-set word recognition for which few subjects
device, which would have been unexpected as ani- achieved any score using the ABI alone. Five sub-
mal studies (Manrique et al., 2000) have clearly jects achieved a lipreading enhancement but two
shown the effectiveness of the PET mesh at fixing subjects were recorded as showing a lipreading
the ABI array within the lateral recess. Further- distraction as a result of their ABI. In this respect it
more, electrical investigation did not suggest any is possibly relevant to note that these subjects were
device malfunction. It is believed that this decline in good lipreaders before receiving their ABI. It is
performance was attributable to the subject’s dete- suggested that with subjects such as these there
riorating medical condition. could be expected a transition period from an initial
dependence on lipreading alone to a dependence on
their ABI to assist lipreading.
DISCUSSION In the harder tests, two subjects (CH1, IG) in
The performance obtained from both the pilot particular stand out as showing significant levels of
study and the formal clinical trial show that the auditory-only sentence and word recognition. This is
Nucleus multichannel ABI is capable of providing a quite remarkable and shows that for a few subjects,
functionally beneficial level of auditory information the ABI even provides sufficient information to
to assist subjects in their communication abilities. understand speech without lipreading. Neverthe-
At the most elementary level the ABI provides the less, this performance level cannot generally be
ability to detect sound at conversational levels. In expected.
itself, this provides the user with basic access to any It has to be borne in mind in each of these tests
surrounding sound. that a number of subjects’ results are not quantified.
Stress-pattern tests show that the ABI alone, The absence of specific data was generally due to
without lipreading, can provide access to acoustic subjects who were less fit and thus less able to
features within speech that are necessary for dis- attend testing sessions. Unfortunately this group
criminating rhythm, stress, simple phonemes and comprised a prevalence of those whose performance
words. The slightly more difficult vowel and conso- was below the average inferred from the data and
180 EAR & HEARING / JUNE 2002
thus the scores may not truly reflect the average cepts and furthermore that changing the stimulus
ABI performance of the group. intensity may also change the pitch sensation. This
Although no formal assessment was made of observation would be consistent with the notion that
environmental awareness, the performance ques- groups of axons representing different characteristic
tionnaire rated environmental awareness and com- frequencies, travel parallel with the surface of the
munication ability with familiar speakers in quiet as CN. In this way, when the surface if the CN is
being the most significant benefits received from the stimulated, the current penetrates to a depth depen-
ABI in the view of recipients. This accounts for why dent on the stimulus intensity and thus groups of
most ABI users informally reported to clinic staff axons representing several characteristic frequen-
that they were happy to have received the implant. cies are stimulated.
Even those who did not score highly on objective Despite this situation it would be wrong to inter-
tests found the ABI provided increased awareness of pret poor sound quality as evidence that pitch infor-
their surroundings. The aspect of benefit from envi- mation over the CN is not useful, but rather that the
ronmental awareness is likely related to the reduced full potential has yet to be achieved. It should be
sense of social isolation so often experienced as a taken as the starting point for further investigation
result of a profound or total hearing loss. Overall, into designs of ABI that might better exploit the
almost all subjects completing the questionnaire felt tonotopic arrangement within the CN such as pen-
that the ABI was at least of some benefit, many etrating electrodes (El-Kashlan 1999; El-Kashlan et
benefiting greatly. al., 1991; McCreery et al., 1992, 1998, 2000). Having
The impression of all subjects when “switching- said this, the current ABI design has many advan-
on” their ABI for the first time was that the sound tages; its smooth surface is relatively easy to insert
sensations experienced were extremely unusual. Re- into the lateral recess and the design is robust. No
actions varied considerably between those who ac- incidents of electrode fault or device failure have yet
cepted that this was now their hearing and they been encountered. But more immediate benefits
must learn to use it, to others who were bemused by might be gained by investigating speech encoders
the strange sound. This reaction may be influenced that better represent temporal information. This
by the patient’s hearing level before implantation, has lead to the development of an enhanced ABI
as some had already been profoundly deaf for a time design available since April 1999 and based around
and may have better come to terms with their the Nucleus CI24M cochlear implant. This newer
deafness. Others lost their final functional hearing receiver/stimulator package is capable of supporting
at surgery. For these subjects there are many more higher-rate coding strategies that may provide bet-
adjustments and psychological influences to accom- ter access to temporal cues in speech.
modate immediately after the operation and per- A further factor that influences the final perfor-
haps there is the hope that the ABI will be some mance is, of course, the success of the surgery itself.
kind of cure. This leads, inevitably, to disappoint- Whether via the translabyrinthine or suboccipital
ment. Extensive preoperative counseling is clearly route, an excellent technique is required. Although
necessary. It should be noted that the one subject the translabyrinthine approach is recommended due
receiving her ABI but getting no auditory sensations to a better angle onto the lateral recess into which
was bitterly disappointed despite extensive counsel- the ABI is inserted, the approach per se is not a
ing that the device might not work. Three years major factor in surgical success. A larger tumour
after surgery, this subject regrets having her hopes (⬎4 cm), on the other hand, presents a worse prog-
raised by the prospect of the ABI and declined the nosis; not only can the tumour itself have caused
opportunity for reoperation or to have an ABI on the damage within the brainstem but its removal is
other side at a later stage. more complex and increases risks of damage to the
It is clear that the level of performance and the surrounding cranial nerves. A larger tumour also
quality of sound from the ABI does not reach that distorts the brainstem and may, once it is removed,
obtained with the latest multichannel cochlear im- make location of the lateral recess using known
plants (Nevison et al., Reference Note 3) where the landmarks more difficult. This probably accounts for
tonotopicity of the cochlea is exploited and useful the one nonstimulating patient. Although a good
open-set speech understanding can be expected in understanding of the normal anatomy of the IVth
the majority of subjects. The reason for reduced ventricle is essential to carry out a successful ABI
performance is almost certainly due to the stimula- operation, a distorted anatomy can seriously ham-
tion of an auditory structure that does not permit per orientation. In these cases, the role of EABR
good access to the tonotopic arrangement of neu- becomes especially important because the results
rons. Initial stimulation reports indicate that sub- obtained could be critical in locating the correct
jects experience mixed rather than clear pitch per- electrode position. It is therefore necessary that the
EAR & HEARING, VOL. 23 NO. 3 181
electrophysiologist is confident in recording and in- An additional benefit of EABR can be establishing
terpreting EABRs generated by electrical stimula- optimal electrode placement. The bipolar electrode
tion of the CN. combinations used to stimulate the CN permit
Tumour size has yet another effect, namely that a EABRs to be generated from different regions of the
heavily distorted brainstem, after tumour removal, array and different parts of the CN. By comparing
must resettle into a new position and will probably responses from the distal, central and proximal
do so relatively soon after surgery. During this time, portions of the array, the ABI can be positioned to
before fibrous tissue has surrounded the device, the get the best responses over as much of the array as
ABI array is most at risk of migrating from its possible. This was used to good effect on one subject,
original position, especially if there is a large lateral SM, where despite an apparent optimal visual place-
recess. In two cases (JB, TK) despite obtaining ment, EABRs showed poor responses from the more
intraoperative EABRs, no auditory sensation were lateral part of the array. Initially, inserting the
obtained at device activation and postoperative array deeper failed to improve the responses; how-
EABRs were absent. In one case (TK) the neurosur- ever, after inspecting the anatomy in more detail it
geon elected to reoperate some 4 mo later, success- was decided that the ABI array was not in the
fully repositioning the ABI with EABRs again ob- optimal orientation. Rotating the array accordingly
tained and resulting in auditory sensation at the gave excellent responses from the entire array and
second device activation. In this case it is believed this subject, when activated, exhibited auditory sen-
that the subject’s large lateral recess had prevented sations from all 21 electrodes.
stable contact between the ABI electrode array and An encouraging finding from this study was the
the CN. During the second surgery the electrode fact that there were no reports of any serious com-
array was stabilized by packing the lateral recess plications attributable to the ABI alone. This is an
with additional gelfoam and abdominal fat. important fact when considering the risks and the
Although reoperation is not undertaken lightly, safety of the device versus its intended performance.
the main risks are really associated with the tumour Potential postoperative clinical side effects of the
removal and not with the ABI placement (or replace- ABI were determined via the neurological assess-
ment) as witnessed by few minor problems to date. ment questionnaire. No subject suffered any com-
Reoperation does not require tumour removal, but promise in neurological function as a direct conse-
simply a reopening of the previous route—albeit that quence of the implantation of an ABI in the
scar tissue will have formed. There is the same risk brainstem. All postoperative neurological symptoms
again to the main cranial nerves once within the reported were consistent with those generally seen
vicinity of the brainstem, but the greater risk is prob- as a result of a cerebello-pontine angle tumour or its
ably damage to the ABI itself, which must be freed removal (including some unsteadiness, decrease in
from the fibrous capsule that surrounds it. Primate limb strength, facial deficit, etc.). During the early
studies (Manrique et al., 2000) using an ABI con- research, concern was expressed that placement of
structed with identical materials showed that the the “foreign” ABI within the lateral recess (together
electrode face is easily freed but that the mesh on the with associated scar tissue formation) might block
rear becomes extremely well integrated into the sur- drainage of CSF through the foramen of Luschka
rounding tissue and may be impossible to free intact. resulting in increased intracranial pressure. How-
It has already been mentioned that EABRs can ever, there have been no reports of any such increase
form an important part of the ABI placement— espe- and indeed the absence of complication has added
cially when the brainstem is distorted. EABR re- fuel to the debate over the importance of the fora-
sponses typically comprise two or three visible waves men of Luschka in CSF circulation. Nevertheless,
rather than the traditional five waves from an acoustic bilateral ABIs would not normally be advised.
ABR. The 3-wave responses probably represent wave In no cases were disturbances of any cranial
III (the CN itself), wave IV and wave V, whereas nerves responsible for unexpected adverse effects
2-wave responses may be wave IV and V or III and IV. although the majority of subjects did suffer tempo-
In only five cases were poor EABRs obtained, yet three rary stimulation of various cranial nerves during
of these five cases were good performers. A further 18 device activation. In all cases, except one, these
cases of good EABRs correspond to a whole range of could be eliminated through programming— either
performance and thus it appears that latencies, by removing the electrode, reprogramming in a
thresholds or response type (2 or 3 peak) do not different stimulation mode or by widening the stim-
correlate with performance. However, it is true that ulus pulse width and reducing the stimulation cur-
subjects getting good EABRs intraoperatively (not- rent. As a precaution an emergency resuscitation
withstanding device movement postoperatively) also team should be available in case the patient suffers
get auditory sensations postoperatively. disturbance to any vital signs due to the small,
182 EAR & HEARING / JUNE 2002
theoretical risk that a misplaced device could stim- received CE-mark in April 1999 and Food and Drug
ulate the vagus nerve (N.X) controlling the heart. In Administration approval in July 2000.
practice no disturbance to any vital sign has ever
been seen and all nonauditory responses observed
during initial stimulation have been transitory. CONCLUSION
In all but one subject undergoing an initial stim- The clinical trial of the Nucleus multichannel
ulation, some auditory-only sensation has been Auditory Brainstem Implant has demonstrated that
achieved. In most subjects this was achieved simply this device is effective at restoring a sense of hearing
by creating a MAP from electrodes that, in monopo- to subjects with bilateral retrocochlear lesions for
lar mode, gave purely auditory sensations. However, whom a cochlear implant will not be effective. In this
in six subjects, optimal sensations were obtained by study, 96.2% of subjects received auditory sensa-
using a combination of monopolar and bipolar tions. Provided good placement of the ABI is
modes. A further five subjects could be programmed achieved over the cochlear nucleus, ideally con-
only in bipolar mode due to low nonauditory re- firmed by intraoperative EABR, users can expect
sponse thresholds in monopolar mode. This suggests the ABI to provide them access to auditory informa-
that any ABI device should be capable of supporting tion such as environmental sound awareness to-
variable mode programming in order not to risk a gether with information regarding patterns of stress
higher incidence of nonusers due to an inherent and rhythm in speech that will assist with lipread-
limitation in the device. ing. Most subjects cannot expect significant, func-
The above design feature of the Nucleus 21- tional auditory-alone, open-set speech understand-
channel ABI gives it the flexibility required to max- ing although this was achieved by two subjects
imize the chance of postoperative success for all (7.4%) in the trial. Due to the nature of the NF2
subjects. In addition, the physical design of the disease, performance may decline due to worsening
current ABI appears to be optimal for surgical physical health although no deterioration in perfor-
success in the hands of a trained and experienced mance has been seen that was attributable to the
surgeon: The dimensions of the ABI electrode array ABI device. Although the level of performance
fit well within the lateral recesses and the excellent achieved with an ABI does not reach that typical of
flexibility of the electrode lead does not hamper a cochlear implant recipient, the performance at-
manipulation. tained is sufficient for most subjects to receive a
To date there have been no failures of the ABI functional benefit from their ABI similar to the first
stimulator. In the unlikely event of failure, experi- single channel cochlear implants. Furthermore the
ence suggests that reoperation would be possible. device helps with the subject’s psychological well-
The risk-benefit for placing an ABI in candidates being through improved awareness of the environ-
already requiring an operation for a cerebello-pon- ment and reduced isolation. The ABI therefore is a
tine angle tumour removal is low. However, for beneficial intervention.
those subjects not explicitly requiring this surgery To achieve good placement of the ABI electrode
such as a reoperation, or for alternate indications array a thorough anatomical understanding of the
such as bilateral skull base fractures or possibly 4th ventricle combined with good surgical technique
even cochlea agenesis, the risk-benefit increases due are essential. Placement of the ABI can be success-
to an extensive surgery being undertaken for no fully achieved via the translabyrinthine or suboccip-
reason other than the placement or replacement of ital route although the former route is generally
the ABI. Such subjects should certainly not be ex- preferred due to the improved access angle to the
cluded from treatment or revision surgery but this lateral recess. Nevertheless, a surgeon who is well
should be approached with extreme caution based experienced with the suboccipital approach is better
on the psychological state and expectations of the advised to continue with what is familiar than to
subject and after full understanding by the patient change to the translabyrinthine approach simply for
of the risks involved. ABI surgeries. Increased confidence in placement is
Since completion of the European clinical trial, achieved by intraoperative EABRs, which were ob-
the Nucleus 22 auditory brainstem implant received tained in 75% of subjects where they were at-
the European CE-mark in August 1998. With the tempted. The risks associated with the extended
introduction of the Nucleus 24 cochlear implant procedure for ABI placement are small. In no cases
system in 1997, the receiver-stimulator package of were disturbances of any cranial nerves responsible
the ABI was upgraded to allow brainstem implant for unexpected adverse effects and all postoperative
recipients the possibility of high-rate speech coding neurological symptoms reported were consistent
strategies (such as CIS and ACE) together with with those generally seen as a result of a cerebello-
advanced telemetry functions. The Nucleus 24 ABI pontine angle tumour or its removal.
EAR & HEARING, VOL. 23 NO. 3 183
The ultimate purpose of this study was to establish Manrique, M., Jauregui, I., et al. (2000). Experimental study
following inactive implantation of an auditory brainstem im-
that the ABI is safe and that the risks for the patient
plant in nonhuman primates. Annals of Otology, Rhinology
are acceptable given the expected performance. The and Laryngology, 109, 163–169.
study shows that the risks of placing the ABI are Marangos, N., Laszig, R., et al. (1997). Langzeitergebnisse der
relatively small compared with the risks of tumour Mehrkanalstimulation des Nucleus cochlearis mit audito-
removal and that the risks of nonauditory responses rischen Hirnstammprothesen. Wiener Medizinische Wochen-
schrift: Themenheft: Prothetische Versorgung von Hörstörun-
from electrical stimulation, although high during pro-
gen (10), 259 –264.
gramming, are minimal after proper processor fitting. McCreery, D. B, Agnew, W. F., et al. (1990). Charge density and
It is possible, however, that due to ongoing neurologi- change per phase as cofactors in neural injury induced by
cal and physiological changes that additional nonau- electrical stimulation. IEEE Transactions on Biomedical Engi-
ditory responses may develop that cannot always be neering, 37, 10: 996 –1001.
McCreery, D. B, Shannon, R. V., et al. (1998). Accessing the
eliminated by further programming.
tonotopic organization of the ventral cochlear nucleus by in-
The data presented in this report represent a tranuclear microstimulation. IEEE Transactions on Biomedi-
total of 27 subjects—all of whom had NF2. No cal Engineering, 6, 391–399.
subject was excluded from this report although not McCreery, D. B, Yuen, T. G., et al. (2000). Chronic microstimu-
all subjects (especially before the clinical trial) had lation in the feline ventral cochlear nucleus: Physiologic and
histologic effects. Hearing Research, 149, 223–238.
extensive performance data for reasons previously
McCreery, D. B, Yuen, T. G. H., et al. (1992). Microstimulation in
described. This population represents 5 yr of expe- the cochlear nucleus of the cat with chronically implanted
rience and active use of the ABI. It is felt that the microelectrodes: histologic and physiologic effects. Hearing
data obtained regarding the ABI to date indicate Research, 62, 42–56.
that it is both a safe and effective intervention for National Institutes of Health (1995). Cochlear implants in adults
subjects losing hearing as a result of NF2. and children. NIH Consensus Statement, 13, 1–30.
Otto S., et al. (1998). The multichannel auditory brainstem
implant: Performance in 20 patients. Otolaryngology and Head
ACKNOWLEDGMENTS: and Neck Surgery, 118, 291–303.
Shannon, R. V. (1992). A model of safe levels for electrical
This clinical investigation was supported by Cochlear Europe stimulation. IEEE Transactions on Biomedical Engineering,
Limited. 39, 424 – 426.
Shin, Y.-J., Deguine, O., et al. (1997). Implant du Tronc Cerebral:
Address for correspondence: Dr. Barry L. Nevison, Cochlear
A propos d’un patient atteint de Nuerofibromatose type 2.
Europe Ltd, 22-24 Worple Road, Wimbledon, London SW19 4DF,
JFORL, 46.
UK.
Skinner, M. W., Clark, G. M., et al. (1994). Evaluation of a new
Received February 20, 2001; accepted January 30, 2002 spectral peak coding strategy for the Nucleus 22 channel
cochlear implant system. American Journal of Otology,
15(Suppl. 2), 15–27.
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