Documentos de Académico
Documentos de Profesional
Documentos de Cultura
Objective: The hearing outcome after implanting drillout circummodiolar technique,3 the use of a double elec-
a severely ossified cochlea has always been less satis- trode array,4 or even a triple array implant.5 Regardless of
factory than implanting a patent one. The aim of our the technique used, however, in severe cochlear ossification,
study is to present a case where brainstem implanta- the reported results are not satisfactory.6,7 Explanations pro-
tion was successfully performed as an alternative to posed include an inadequate number of inserted electrodes,8
cochlear implantation in a child with bilateral severe
the distance between the electrodes and the nerve fibers
ossification of the cochlea. Study Design: Case presen-
tation. This study was conducted at Gruppo Oto- within the modiolus,9 the involvement of the spiral ganglion
logico, Rome, Italy, a private referral center for neu- cells by ossification,10 and peripheral nerve degeneration
rotology and skull base surgery. Methods: The subject associated with ossification or even mere fibrosis.11 Recently,
of our study was a 12-year-old female child with post- there have been a few reports describing the use of auditory
meningitic deafness and bilaterally ossified cochleae. brainstem implant (ABI) for management of such cas-
This case is the first brainstem implantation per- es.9,12,13 In this paper, we report an additional case of ABI
formed at our center with the indication of severe use for rehabilitation of a child with complete deafness and
ossification of the cochlea. Results: Successful brain- severe bilateral cochlear ossification after an attack of
stem implantation of a device was carried out, and the meningitis.
hearing of the patient was restored to the degree that
she can freely use the telephone after 8 months of CASE REPORT
implantation. Conclusion: Although more cases are A 12-year-old female patient was referred to us 7 months
needed before establishing the exact outcome of after the onset of meningitis, which has led to an immediate
brainstem implantation in cases of deafness in the bilateral deafness. The patient did not have any additional neu-
presence of severe bilateral cochlear ossification, pre- rologic sequel. Five months after the onset of HL, an attempt at
liminary results show the superiority of brainstem right cochlear implantation was performed at another center.
implants to conventional or even customized co- During surgery, however, the cochlea was found to be completely
chlear implants. Key Words: Ossified cochlea, cochlear ossified, and implantation was judged impossible by the surgeon.
implant, brainstem implant, child. Our assessment of the patient’s condition revealed a normal
Laryngoscope, 116:1700 –1703, 2006 tympanic membrane bilaterally. Audiologic examination con-
firmed a total bilateral HL. With lip reading, the patient had 20%
INTRODUCTION word recognition and 18% sentence recognition. She also had
Since its introduction, the results of cochlear implan- good language development. Radiologic examination revealed an
tation for the rehabilitation of bilateral sensorineural ossified cochlea bilaterally with computed tomographic (CT) scan-
hearing loss (HL) have improved significantly. Indications ning (Fig. 1), wheras magnetic resonance imaging showed the
for implantation have also been expanded to include pa- presence of the 7th and 8th cranial nerves bilaterally.
tients with severe to profound HL. Unfortunately, how- The decision was taken to implant the left side. Because the
ever, in cases of severe cochlear ossification, the results CT showed severe ossification of the left cochlea and failure of the
have not been as satisfying. Various techniques have been earlier implant attempt on the right side, an informed consent
was also obtained from the family to shift for an ABI if ossifica-
proposed by different surgeons to deal with this problem,
tion prevented cochlear implantation.
examples include electrode insertion in the scala vestibuli,1 We chose a subtotal petrosectomy with blind-sac closure of
insertion of electrodes through the middle cranial fossa,2 the 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
From Gruppo Otologico Piacenza (M.S., M.G., M.F.), Rome, Italy, and implantation. The subtotal petrosectomy was transformed into a
Jordan University of Science and Technology (T.K.), Irbid, Jordan. translabyrinthine approach. Once the posterior fossa dura was
Editor’s Note: This manuscript was accepted for publication May opened, adhesions were encountered in the cerebellopontine an-
26, 2006.
gle that were a result of the meningitis. The nerves and the
Send correspondence to Tarek Khrais, P.O. Box 3710, Irbid 21110,
vessels within the cerebellopontine angle were matted together,
Jordan. E-mail: khraistarek@hotmail.com
obliterating access to the lateral recess of the 4th ventricle. Dis-
DOI: 10.1097/01.mlg.0000231739.79208.97 section proceeded slowly to identify the 8th and 9th cranial
Fig. 2. Electrical auditory brain stem responses elicited at positioning of Fig. 4. Correct position as confirmed by soft tissue coronal com-
array. puted tomography.
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.
BIBLIOGRAPHY
1. Steenerson RL, Gary LB, Wynens MS. Scala vestibuli co-
chlear implantation for labyrinthine ossification. Am J
Otol 1990;11:360 –363.
2. Colletti V, Fiorino FG, Carner M, et al. New approach for
cochlear implantation: cochleostomy through the middle
fossa. Otolaryngol Head Neck Surg 2000;123:467– 474.
3. Balkany T, Gantz B, Nadol JB Jr. Multichannel cochlear
implants in partially ossified cochleas. Ann Otol Rhinol
Laryngol Suppl 1988;135:3–7.
4. Lenarz T, Buchner A, Tasche C, et al. The results in patients
Fig. 5. Mapping of electrodes at follow-up. implanted with the nucleus double array cochlear implant: