European Annals of Otorhinolaryngology, Head and Neck diseases 133S (2016) S12–S14

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Original article

Cochlear implantation in children with congenital unilateral deafness:

Mid-term follow-up outcomes
D. Távora-Vieira a,b,∗ , G.P. Rajan a,b
a
Otolaryngology, Head & Neck Surgery, School of Surgery, University of Western Australia, Perth, Australia
b
Fiona Stanley Hospital, Perth WA 6, Australia

a r t i c l e i n f o a b s t r a c t

Keywords: Objectives: Although cochlear implantation is widely used to treat unilateral deafness in adults, very
Cochlear implant little literature exists on its use and effects on the paediatric population. This report adds to the literature
Paediatric unilateral deafness showing the mid-term follow-up outcomes achieved by these children.
Binaural hearing
Material and methods: Three children with congenital unilateral deafness were studied after implanta-
tion. Speech perception in noise, and sound localization ability were evaluated using age-appropriate
materials.
Results: The preliminary data of our small group of 3 children with congenital unilateral profound hearing
loss revealed that up to 3 years post-implantation, congenitally deaf children who received a cochlear
implant after 4 years of age do not demonstrate binaural hearing benefits.
Conclusion: Early intervention in the prelingual phase may be crucial for the development of binaural
hearing.
© 2016 Elsevier Masson SAS. All rights reserved.

1. Introduction
Several studies have demonstrated that cochlear implant (CI)
use significantly improves the speech perception in noise, sound
localization ability, and subjective hearing performance of adults
with unilateral deafness (UD) [1–8]. Very limited literature, how-
ever, is available for the paediatric population.
Hassepass et al. [9] presented 3 children who received a CI as a
treatment for postlingual UD. They were aged 4, 10, and 11 years; 2
of the children had a short duration of deafness (5 and 18 months).
All children were reported to wear the speech processor more than
8 hours a day. Results indicated binaural hearing benefits for speech
recognition in noise and sound localization. Similarly, Plontke et al.
[10] reported a case of an 8-year-old boy who received a CI for UD 5
months after a lateral skull-base fracture. Six months following ini-
tial activation, he also showed significant improvement for speech
understanding in noise and sound localization.
The preliminary data of our small group – 4 children: 3 with
congenital hearing loss and 1 with acquired hearing loss – were
published early 2015. At 12 months follow-up, the 3 children
with congenital hearing loss presented poor outcomes, with the
∗ Corresponding author at: Fiona Stanley Hospital, Perth WA 6, Australia.
Tel.: +61 8 9431 2144.
E-mail address: dayse.tavora@gmail.com (D. Távora-Vieira).
exception of the youngest recipient [11]. Arndt et al. [12] also
presented the 12-month follow-up results on a cohort of children:
2 with congenital deafness, 2 with perilingual UD, and 9 with
postlingual UD. The 2 children with congenital deafness had
outcomes very similar to those in our cohort, with no evidence
of binaural benefits at the 12-month review. Arndt et al. [12] also
reported that the younger of the 2 children with congenital deaf-
ness, although he/she was too young to be tested, showed clinical
evidence of CI benefit, which is again similar to our youngest child,
who was implanted at 17 months old [11].
Although these findings are from a very small number of sub-
jects, they are in line with recent research demonstrating that
monaural hearing beyond a period of approximately 1.5 years
drives abnormal reorganization of the auditory pathway with con-
sequent restriction to binaural hearing development [13–15]. If UD
occurs early in life, a massive reorganization of aural preference
in favour of the hearing ear takes place, resultsing in asymmetric
auditory brain development [15,16].
Stigmatization and natural compensation mechanisms are
important factors to be considered in the pre-surgical assessment
of a patient [17]. Nevertheless, it is very likely that children with
congenital UD have a short window of opportunity for cochlear
implantation in order to attain bilateral input and possible binaural
hearing development.
In view of this management dilemma, prior to recruiting
patients for a study involving a larger number of participants with

http://dx.doi.org/10.1016/j.anorl.2016.04.016
1879-7296/© 2016 Elsevier Masson SAS. All rights reserved.
 D. Távora-Vieira, G.P. Rajan / European Annals of Otorhinolaryngology, Head and Neck diseases 133S (2016) S12–S14 S13

Table 1
Demographic data.

ID Gender Duration of deafness Age at implantation Ear Aetiology Pure tone average (0.5, 1, 2,
and 4 kHz) – non-implanted ear
in dB

S1 M 17 months 17 months R Unknown 15

S2 M 4.5 years 4.5 years R Unknown 10
S3 F 6.8 years 6.8 years R Unknown 5

M: male; F: female; R: right.

congenital UD, our group has decided to prepare this follow-up

report and show the results of our cohort of 3 children who received
CI to treat their congenital UD.

2. Methods

2.1. Study design

This prospective long-term follow-up study of children with UD

was designed and conducted in accordance with the Declaration of
Helsinki. Ethical approval was obtained from the relevant ethics
and institutional review committee. The inclusion criteria were
published in Távora-Vieira and Rajan [11].

2.2. Subjects Fig. 1. Speech perception in noise scores [adaptive Bamford-Kowel-Bench adap-
tive speech-in-noise test (BKB-SIN)] preoperatively, 6, 12, 24, and 36 months after
The 3 children included in this study were referred to our CI cochlear implantation for S3.
centre by the audiologists who were involved in their early inter-
vention and hearing rehabilitation together with their families
since the diagnosis of UD. Speech and language development were
2.4. The subjects’ motivation
age-appropriate for 3 children according to medical records.
The children were aged 17 months to 6.8 years when they
S1 had had a few episodes of middle ear infections in his normal-
received a OPUS 2 (MED-EL, Innsbruck, Austria) with a FLEXSOFT
hearing ear, which triggered his parents’ concerns regarding the
electrode array. All arrays were inserted completely. Mapping ses-
future hearing ability of that ear and the possible futility of any
sions followed the standard paediatric protocol used in the implant
late intervention for the deaf ear. S2 and S3’s parents sought inter-
centre. Each child was fitted with a FM system to assist in auditory
vention advice based upon their desire to provide their child with
training at home, ensuring input from the CI alone. Subject #1 (S1)
the benefits of binaural hearing. They were apprehensive about the
has been implanted for 36 months, S2 for 40 months, and S3 for 42
implications of UD later in their children’s future and concerned
months (Table 1).
that a late intervention would be of no benefit because of long
auditory deprivation.
2.3. Audiological evaluation

The preoperative audiological evaluation included acoustic 3. Results

immittance measures, otoacoustic emissions (OAE), and auditory
brainstem responses (ABR).
The postoperative evaluation differed according to subject. For
S1, speech perception testing was performed using the Northwest-
ern University-Children’s Perception of Speech (NU-CHIPS, [18])
in a soundproof room with the normal-hearing ear masked with
speech noise at 65 dB.
For S3, speech perception in noise was evaluated using:
• the Bamford-Kowel-Bench adaptive speech-in-noise test (BKB-
SIN; [19]) in 3 spatial configurations: speech and noise from the
front (S0/N0), speech from the front and noise from the normal-
hearing ear (S0/Nhe), and speech from the CI side and noise from
the normal-hearing ear (Sci/Nhe);
• the CNC words test. Testing took place in a soundproof room with
the normal-hearing ear masked with speech noise at 65 dB.
Results are presented individually for each subject as the proto-
col for evaluation was determined by each child’s age.
3.1. Acceptance
S1 is a full-time user.
S2 wore the sound processor for 5 weeks and found the electri-
cal stimulation too unpleasant. He is currently a non-user and his
parents decided to withdraw him from the hearing rehabilitation
program. No postoperative audiological data were collected.
S3 is a full-time user.
3.2. Audiological evaluation

Sound localization ability was measured using the auditory
speech sounds evaluation (A§E® ) localization test (PJ Govaerts,
Antwerp, Belgium) as described by Tavora-Vieira et al. [8].
Data collection occurred prior to implantation, and at several
postoperative intervals.
S1 scored a 100% in the NU-CHIPS speech perception testing.
S3 perceives auditory stimulation as vibration-only 3 years post-
implantation. Scores for speech perception in noise with CI-on did
not differ from those without CI (Fig. 1). S3 could not correctly
repeat any words in the CNC words testing.
S14 D. Távora-Vieira, G.P. Rajan / European Annals of Otorhinolaryngology, Head and Neck diseases 133S (2016) S12–S14
Fig. 2. Localization ability at 6, 12, 24, and 36 months post-cochlear implantation
with cochlear implant (CI) on and CI off for S3.
3.3. Sound localization
S1 is, according to his parents, able to localize sounds in his own
environment. Several trials of sound lateralization in a soundproof
room showed that the child is able to correctly lateralize sounds
presented through speakers located at 90◦ and −90◦ . Spatial acuity
has not yet been tested because of the child’s young age.
S3 has not shown any improvement in sound localization ability
(Fig. 2).
4. Discussion
Very little is known regarding the outcomes of cochlear implan-
tation in children with UD, particularly those with congenital
deafness. Apart from our own preliminary results, Arndt et al. [12],
who reported the outcomes of 4 children with congenital UD, found
that early intervention with a CI might be the way to allow these
children to develop binaural hearing.
Although a study involving a large number of children is neces-
sary, this follow-up report adds to the literature, as it demonstrates
the results obtained in unilaterally deaf children up to 3 years post-
implantation.
S1 had 17 months of unilateral auditory stimulation and was
implanted prelingually. He is now 57 months old and has been
wearing his implant on full-time basis for over 3 years. His speech
perception scores show that he can hear in the CI alone condi-
tion. Binaural hearing benefits remain to be tested when he is old
enough.
S2 had 4.5 years and S3 had 6.8 years of monaural hearing at the
time of implantation. S2 could not tolerate electrical stimulation
and is currently a non-user. S3 has not achieved any measurable
hearing benefits after implantation. However, S3’s family is hoping
that the long-term results might change. She is currently full-time
wearer.
The results achieved by these children may be explained by
monaural stimulation during the critical period of binaural hearing
development leading to permanent asymmetric development of
auditory pathways and subsequent loss of binaural abilities, which,
it seems, cannot be reversed after restoration of auditory stimula-
tion via CI.
It is possible that early cochlear implantation would avoid the
permanent re-routing of auditory pathways. In other words, the
early establishment of bilateral stimulation with CI in the deaf
ear would avoid this abnormal strengthening of the only hearing
ear pathway. This interpretation, however, cannot be generalized
because this study is based on a very small cohort.
In our report of the preliminary results of this group of children,
we suggested that early intervention for children with congenital
UD could be the best approach to regain binaural hearing function.
Our current 3-year follow-up outcomes and the early outcomes
reported by Arndt et al. [12] support this approach because they
indicate that the hearing outcomes at 36 months post-CI do not
differ from those obtained at 6 and 12 months post-CI. Further
investigation involving a larger number of participants and includ-
ing children with perilingual UD is crucial in the development of CI
candidacy criteria for children with UD.
5. Conclusion
Children with congenital unilateral deafness might be good
candidates for cochlear implantation. Early intervention in the
prelingual-perilingual phase might be crucial for the develop-
ment of binaural hearing. The optimal age for enhanced outcomes
remains to be investigated. Is there a time window for implantation
in congenital unilateral deafness? Is the time window, if it exists,
different for children with pre- or perilingual deafness?
Disclosure of interest
The authors declare that they have no competing interest.
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