Introduction: There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, cerebrospinal fluid (CSF) leaks, erroneous electrode insertion, or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of inner ear malformations (IEMs) on surgical complications and outcomes of cochlear implantation. Methods: In order to assess the impact of IEMs on cochlear implant (CI) outcomes, 2 groups of patients with similar epidemiological parameters were selected from among 863 patients. Both the study group (patients with an IEM) and control group (patients with a normal inner ear) included 25 patients who received a CI and completed at least 1 year of follow-up. Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Types of surgical complications and rates of revision surgeries were determined in each group. Results: In the study group, the most common malformation was an isolated enlarged vestibular aqueduct (EVA) (44.8%). Overall, the patients with IEMs showed significant improvement in auditory-verbal skills. In general, the patients who had normal cochleae scored significantly better compared to patients with IEMs (p < 0.05). The complication rate was significantly lower in the control group compared to the study group (p = 0.001), but the rate of revision surgeries did not differ significantly (p = 0.637). Conclusion: It is possible to improve communication skills with CIs in patients with IEMs despite the variations in postoperative performances. Patients with EVA, incomplete partition type 2, and cochlear hypoplasia type 2 were the best performers in terms of auditory-verbal skills. Patients with IEMs scored poorly compared to patients with normal cochleae. CSF leak (gusher or oozing) was the most common complication during surgery, which is highly likely in cases of incomplete partition type 3.

1.
Jackler
RK
,
Luxford
WM
,
House
WF
.
Congenital malformations of the inner ear: a classification based on embryogenesis
.
Laryngoscope
.
1987
;
97
:
2
14
. .
2.
Mondini
C
.
Anatomia surdi nedi section
.
De Bononiensi Sci Artum Inst Atque Acad Commentarii Bol
.
1791
;
7
(
28
):
419
.
3.
Sennaroglu
L
,
Saatci
I
.
A new classification for cochleovestibular malformations
.
Laryngoscope
.
2002
;
112
:
2230
41
. .
4.
Papsin
BC
.
Cochlear implantation in children with anomalous cochleovestibular anatomy
.
Laryngoscope
.
2005
;
115
:
1
26
. .
5.
Sennaroğlu
L
,
Bajin
MD
.
Classification and current management of inner ear malformations
.
Balkan Med J
.
2017
;
34
:
397
411
.
6.
Farhood
Z
,
Nguyen
SA
,
Miller
SC
,
Holcomb
MA
,
Meyer
TA
,
Rizk
HG
.
Cochlear implantation in inner ear malformations: systematic review of speech perception outcomes and intraoperative findings
.
Otolaryngol Head Neck Surg
.
2017
;
156
:
783
93
. .
7.
Van Wermeskerken
GK
,
Dunnebier
EA
,
Van Olphen
AF
,
Van Zanten
BA
,
Albers
FW
.
Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations
.
Acta Otolaryngol
.
2007
;
127
:
252
7
. .
8.
Karamert
R
,
Düzlü
M
,
Tutar
H
,
Eravcı
FC
,
Türkcan
AK
,
Zorlu
ME
,
Assessment of cochlear implant revision surgeries in a cohort of 802 patients
.
Otol Neurotol
.
2019
;
40
:
464
70
. .
9.
Moeller
MP
.
Early intervention and language development in children who are deaf and hard of hearing
.
Pediatrics
.
2000
;
106
:
E43
. .
10.
Dettman
S
,
Sadeghi-Barzalighi
A
,
Ambett
R
,
Dowell
R
,
Trotter
M
,
Briggs
R
.
Cochlear implants in forty-eight children with cochlear and/or vestibular abnormality
.
Audiol Neurootol
.
2011
;
16
:
222
32
. .
11.
Buchman
CA
,
Copeland
BJ
,
Yu
KK
,
Brown
CJ
,
Carrasco
VN
,
Pillsbury
HC
 3rd
.
Cochlear implantation in children with congenital inner ear malformations
.
Laryngoscope
.
2004
;
114
:
309
16
. .
12.
Arnoldner
C
,
Baumgartner
WD
,
Gstoettner
W
,
Egelierler
B
,
Czerny
C
,
Steiner
E
,
Audiological performance after cochlear implantation in children with inner ear malformations
.
Int J Pediatr Otorhinolaryngol
.
2004
;
68
:
457
67
. .
13.
Eisenman
DJ
,
Ashbaugh
C
,
Zwolan
TA
,
Arts
HA
,
Telian
SA
.
Implantation of the malformed cochlea
.
Otol Neurotol
.
2001
;
22
:
834
41
. .
14.
Valvassori
GE
,
Clemis
JD
.
The large vestibular aqueduct syndrome
.
Laryngoscope
.
1978
;
88
:
723
8
. .
15.
Sennaroglu
L
,
Sarac
S
,
Ergin
T
.
Surgical results of cochlear implantation in malformed cochlea
.
Otol Neurotol
.
2006
;
27
:
615
23
. .
16.
Lemmerling
MM
,
Mancuso
AA
,
Antonelli
PJ
,
Kubilis
PS
.
Normal modiolus: CT appearance in patients with a large vestibular aqueduct
.
Radiology
.
1997
;
204
:
213
9
. .
17.
Au
G
,
Gibson
W
.
Cochlear implantation in children with large vestibular aqueduct syndrome
.
Am J Otol
.
1999
;
20
:
183
6
.
18.
Aschendorff
A
,
Marangos
N
,
Laszig
R
.
Large vestibular aqueduct syndrome and its implication for cochlear implant surgery
.
Am J Otol
.
1997
;
18
:
S57
.
19.
Phelps
PD
,
Reardon
W
,
Pembrey
M
,
Bellman
S
,
Luxom
L
.
X-linked deafness, stapes gushers and a distinctive defect of the inner ear
.
Neuroradiology
.
1991
;
33
:
326
30
. .
20.
Munro
KJ
,
George
CR
,
Haacke
NP
.
Audiological findings after multichannel cochlear implantation in patients with Mondini dysplasia
.
Br J Audiol
.
1996
;
30
:
369
79
. .
21.
Miyamoto
RT
,
Robbins
AJ
,
Myres
WA
,
Pope
ML
.
Cochlear implantation in the Mondini inner ear malformation
.
Am J Otol
.
1986
;
7
:
258
61
.
22.
Incesulu
A
,
Adapinar
B
,
Kecik
C
.
Cochlear implantation in cases with incomplete partition type III (X-linked anomaly)
.
Eur Arch Otorhinolaryngol
.
2008
;
265
:
1425
30
. .
23.
Berrettini
S
,
Forli
F
,
De Vito
A
,
Bruschini
L
,
Quaranta
N
.
Cochlear implant in incomplete partition type I
.
Acta Otorhinolaryngol Ital
.
2013
;
33
:
56
62
.
24.
Ito
J
,
Sakota
T
,
Kato
H
,
Hazama
M
,
Enomoto
M
.
Surgical considerations regarding cochlear implantation in the congenitally malformed cochlea
.
Otolaryngol Head Neck Surg
.
1999
;
121
:
495
8
. .
25.
Jackler
RK
,
Luxford
WM
,
House
WF
.
Sound detection with the cochlear implant in five ears of four children with congenital malformations of the cochlea
.
Laryngoscope
.
1987
;
97
:
15
7
. .
26.
Shelton
C
,
Luxford
WM
,
Tonokawa
LL
,
Lo
WW
,
House
WF
.
The narrow internal auditory canal in children: a contraindication to cochlear implants
.
Otolaryngol Head Neck Surg
.
1989
;
100
:
227
31
. .
27.
Incesulu
A
,
Vural
M
,
Erkam
U
,
Kocaturk
S
.
Cochlear implantation in children with inner ear malformations: report of two cases
.
Int J Pediatr Otorhinolaryngol
.
2002
;
65
:
171
9
. .
28.
Casselman
JW
,
Offeciers
FE
,
Govaerts
PJ
,
Kuhweide
R
,
Geldof
H
,
Somers
T
,
Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging
.
Radiology
.
1997
;
202
:
773
81
. .
29.
Kawasaki
A
,
Fukushima
K
,
Kataoka
Y
,
Fukuda
S
,
Nishizaki
K
.
Using assessment of higher brain functions of children with GJB2-associated deafness and cochlear implants as a procedure to evaluate language development
.
Int J Pediatr Otorhinolaryngol
.
2006
;
70
:
1343
9
. .
30.
Hamzavi
J
,
Baumgartner
WD
,
Egelierler
B
,
Franz
P
,
Schenk
B
,
Gstoettner
W
.
Follow up of cochlear implanted handicapped children
.
Int J Pediatr Otorhinolaryngol
.
2000
;
56
:
169
74
. .
31.
Wiley
S
,
Jahnke
M
,
Meinzen-Derr
J
,
Choo
D
.
Perceived qualitative benefits of cochlear implants in children with multi-handicaps
.
Int J Pediatr Otorhinolaryngol
.
2005
;
69
:
791
8
. .
32.
Fallon
M
,
Harris
M
.
Training parents to interact with their young children with handicaps: professional-directed and parent-oriented approaches
.
Infant Toddler Intervent
.
1991
;
1
:
297
313
.
33.
Hadadian
A
,
Merbler
J
.
Parents of infants and toddlers with special needs: sharing views of desired services
.
Infant-Toddler Interv: J Transdiscipl
.
1995
;
5
:
141
51
.
34.
Hoffman
RA
,
Downey
LL
,
Waltzman
SB
,
Cohen
NL
.
Cochlear implantation in children with cochlear malformations
.
Am J Otol
.
1997
;
18
:
184
7
.
35.
Phelps
PD
,
King
A
,
Michaels
L
.
Cochlear dysplasia and meningitis
.
Am J Otol
.
1994
;
15
:
551
7
.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.