The Laryngoscope
Lippincott Williams & Wilkins, Inc.
© 2006 The American Laryngological,
Rhinological and Otological Society, Inc.
Auditory Brainstem Implant in a Child with
Severely Ossified Cochlea
Mario Sanna, MD; Tarek Khrais, MD, DHS, FRCS; Maurizio Guida; Maurizio Falcioni, MD
Objective: The hearing outcome after implanting
a severely ossified cochlea has always been less satis-
factory than implanting a patent one. The aim of our
study is to present a case where brainstem implanta-
tion was successfully performed as an alternative to
cochlear implantation in a child with bilateral severe
ossification of the cochlea. Study Design: Case presen-
tation. This study was conducted at Gruppo Oto-
logico, Rome, Italy, a private referral center for neu-
rotology and skull base surgery. Methods: The subject
of our study was a 12-year-old female child with post-
meningitic deafness and bilaterally ossified cochleae.
This case is the first brainstem implantation per-
formed at our center with the indication of severe
ossification of the cochlea. Results: Successful brain-
stem implantation of a device was carried out, and the
hearing of the patient was restored to the degree that
she can freely use the telephone after 8 months of
implantation. Conclusion: Although more cases are
needed before establishing the exact outcome of
brainstem implantation in cases of deafness in the
presence of severe bilateral cochlear ossification, pre-
liminary results show the superiority of brainstem
implants to conventional or even customized co-
chlear implants. Key Words: Ossified cochlea, cochlear
implant, brainstem implant, child.
Laryngoscope, 116:1700 –1703, 2006
INTRODUCTION
Since its introduction, the results of cochlear implan-
tation for the rehabilitation of bilateral sensorineural
hearing loss (HL) have improved significantly. Indications
for implantation have also been expanded to include pa-
tients with severe to profound HL. Unfortunately, how-
ever, in cases of severe cochlear ossification, the results
have not been as satisfying. Various techniques have been
proposed by different surgeons to deal with this problem,
examples include electrode insertion in the scala vestibuli,1
insertion of electrodes through the middle cranial fossa,2 the
From Gruppo Otologico Piacenza (M.S., M.G., M.F.), Rome, Italy, and
Jordan University of Science and Technology (T.K.), Irbid, Jordan.
Editor’s Note: This manuscript was accepted for publication May
26, 2006.
Send correspondence to Tarek Khrais, P.O. Box 3710, Irbid 21110,
Jordan. E-mail: khraistarek@hotmail.com
DOI: 10.1097/01.mlg.0000231739.79208.97
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1700
drillout circummodiolar technique,3 the use of a double elec-
trode array,4 or even a triple array implant.5 Regardless of
the technique used, however, in severe cochlear ossification,
the reported results are not satisfactory.6,7 Explanations pro-
posed include an inadequate number of inserted electrodes,8
the distance between the electrodes and the nerve fibers
within the modiolus,9 the involvement of the spiral ganglion
cells by ossification,10 and peripheral nerve degeneration
associated with ossification or even mere fibrosis.11 Recently,
there have been a few reports describing the use of auditory
brainstem implant (ABI) for management of such cas-
es.9,12,13 In this paper, we report an additional case of ABI
use for rehabilitation of a child with complete deafness and
severe bilateral cochlear ossification after an attack of
meningitis.
CASE REPORT
A 12-year-old female patient was referred to us 7 months
after the onset of meningitis, which has led to an immediate
bilateral deafness. The patient did not have any additional neu-
rologic sequel. Five months after the onset of HL, an attempt at
right cochlear implantation was performed at another center.
During surgery, however, the cochlea was found to be completely
ossified, and implantation was judged impossible by the surgeon.
Our assessment of the patient’s condition revealed a normal
tympanic membrane bilaterally. Audiologic examination con-
firmed a total bilateral HL. With lip reading, the patient had 20%
word recognition and 18% sentence recognition. She also had
good language development. Radiologic examination revealed an
ossified cochlea bilaterally with computed tomographic (CT) scan-
ning (Fig. 1), wheras magnetic resonance imaging showed the
presence of the 7th and 8th cranial nerves bilaterally.
The decision was taken to implant the left side. Because the
CT showed severe ossification of the left cochlea and failure of the
earlier implant attempt on the right side, an informed consent
was also obtained from the family to shift for an ABI if ossifica-
tion prevented cochlear implantation.
We chose a subtotal petrosectomy with blind-sac closure of
the external auditory canal to provide a wide surgical field to deal
with the ossification. As expected, during surgery, the cochlea
was found completely ossified, so we decided to shift to brainstem
implantation. The subtotal petrosectomy was transformed into a
translabyrinthine approach. Once the posterior fossa dura was
opened, adhesions were encountered in the cerebellopontine an-
gle that were a result of the meningitis. The nerves and the
vessels within the cerebellopontine angle were matted together,
obliterating access to the lateral recess of the 4th ventricle. Dis-
section proceeded slowly to identify the 8th and 9th cranial
Sanna et al.: ABI in Ossified Cochlea

Fig. 1. Axial (A) and coronal (B) computed tomography scan show-
ing the ossification of cochlea.
nerves. Following these nerves medially led to the foramen of
Luschka and consequently the lateral recess, both of which were
free of adhesions. The correct identification of the foramen of
Fig. 2. Electrical auditory brain stem responses elicited at positioning of
array.
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Fig. 3. Mapping of electrodes immediately after positioning of array.
Luschka was further confirmed by the cerebrospinal fluid exiting
from the foramen with Valsalva maneuvers. An ABI24 mol/L
implant (Nucleus, Lone Cove, Australia) was easily inserted un-
der direct monitoring of the 7th, 9th, and 11th cranial nerves and
indirect monitoring of the 10th cranial nerve through electrocar-
diographic recording. Intraoperative impedance telemetry and
electrical auditory brainstem responses (EABR) were performed
to ascertain the correct position and function of the implant. An
example of the obtained EABR waves is shown on Figure 2.
During surgery, it was not possible to obtain any EABR from the
most medial four electrodes, which, on the contrary, produced
some stimulation of the 11th cranial nerve (Fig. 3).
At the end of the operation, the eustachian tube was oblit-
erated with periosteum and the surgical cavity with long strips of
abdominal fat inserted deeply into the cerebellopontine angle
through the dural defect. The surgical wound was then sutured in
layers. The postoperative period was uneventful. The position of
the implant was radiologically confirmed by a postoperative CT
(Fig. 4), and the patient was discharged on the fourth postoper-
ative day.
Activation of the implant was performed, after 1 month, in the
operating room under electrocardiographic monitoring and in the
presence of an anesthesiologist. The spectral peak (SPEAK) strategy
was adopted. During activation, the number of electrodes stimulat-
ing the 11th cranial nerve without eliciting any auditory sensation
was reduced to only 2, thus, overall, we had 19 functioning elec-
trodes (Fig. 5). Immediately after activation, the hearing assessment
tests resulted in 40% sound identification, whereas recognition of
bisyllable words and sentences was 0%. Audiologic rehabilitation
Fig. 4. Correct position as confirmed by soft tissue coronal com-
puted tomography.
Sanna et al.: ABI in Ossified Cochlea
1701
was instituted, and follow-up was performed at 1, 3, 5 and 8 months
after the surgery. There was a marked improvement with each visit
(Fig. 6) so that during the last follow-up, the results were very
satisfying. The patient scored 100% sound identification, 90% rec-
ognition of bisyllable words, and 100% sentences recognition, with
31 words per minute at speech tracking. All the tests reported were
performed in open set auditory-only mode. In addition, she is now
able to communicate with the telephone.

DISCUSSION
The consistency of the tonotopic orientation of the nerve
fibers in the modiolus has made it easy to produce good
hearing results after cochlear implantation. However, to
achieve this aim, one of the important prerequisites is hav-
ing a cochlea with patent turns. These turns act as a canal
that can house the electrode array for the stimulation of the
nerve fibers at the modiolus. In cases of severe cochlear Fig. 6. Results of audiologic testing according to time.
ossification, the absence of this canal has led some authors to
try to drill a neocanal for accommodation of the array. Al-
though the results were discouraging, various techniques
our case, access to the cerebellopontine angle was gained
have been developed with the hope of obtaining results sim-
through the translabyrinthine approach, which, unlike the
ilar to those in patent cochlea.1–5 Unfortunately, none of
retrosigmoid approach, provides a more direct approach to
these techniques could markedly improve the outcome in
the foramen of Luschka, without any cerebellar retraction.
cases of severe ossification, and only a few patients were able
In addition, in this specific case, the translabyrinthine ap-
to obtain some open set discrimination.
proach offered the possibility of performing the ABI insertion
Tonotopic organization has also been demonstrated
during the same stage as the cochlear exploration, with a
at the level of the cochlear nucleus; however, in most
simple extension of the approach.
patients with bilateral acoustic neuromas (NF2), hearing
The results obtained in this case after 8 months of
results using ABI have been unsatisfactory. In these
rehabilitation were 100% sound recognition, 90% bisyl-
cases, ABI is used when the cochlear nerve cannot be
lable word recognition, and 100% sentence recognition,
preserved, and hearing is lost during surgery. This is more
with 31 words per minute speech tracking (open set,
frequent with larger schwannomas. In these cases, the
auditory-only mode). In addition, the patient is now able
tumor usually stretches the cochlear nerve and com-
to conduct regular telephone conversations. In evaluating
presses the area of the brainstem where the cochlear
our results and those reported in literature, one can notice
nucleus is located. Distortion of the cochlear nucleus usu-
a general trend toward better hearing results with the
ally results in reduction and disorganization of the audi-
increase of the number of active electrodes.
tory fibers and, as a consequence, alteration of the complex
three-dimensional tonotopic orientation.14,15 Theoretically, CONCLUSIONS
then, if the implant stimulates an undisturbed cochlear The preliminary results of ABI in bilateral ossified
nucleus, there would be a better chance of achieving a cochlea appear promising when compared with those ob-
good result, which is the case if ABI is performed in cases tained with cochlear implantation in similar cases. We
of cochlea ossification. Grayeli et al.9 were the first to need a large number of cases to prove the effectiveness of
report a case of ABI insertion for the bypass of a severely ABI. We should also take into consideration that the sur-
ossified cochlea. Colletti et al.12,13 then reported four sim- gery is more difficult and risky when compared with co-
ilar cases. Four of five patients reached at least 60% chlear implantation and, as a consequence, must be re-
sentence recognition in open set, auditory-only mode. Our served for selected referral centers. In every case, our
case, which to the best of our knowledge is the sixth reported policy has been to first attempt a cochleostomy to evaluate
in the literature and the first in a child, represents living the possibility of cochlear implantation and reserve the
evidence of the accuracy of the aforementioned theory. In ABI for situations in which there is no practical possibility
to obtain satisfactory results from cochlear implantation.

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