PMID- 30055722
OWN - NLM
STAT- MEDLINE
DCOM- 20181026
LR  - 20181026
IS  - 1872-8464 (Electronic)
IS  - 0165-5876 (Linking)
VI  - 112
DP  - 2018 Sep
TI  - Outcomes after cochlear implant provision in children with cochlear nerve
      hypoplasia or aplasia.
PG  - 132-140
LID - S0165-5876(18)30282-9 [pii]
LID - 10.1016/j.ijporl.2018.06.038 [doi]
AB  - INTRODUCTION: Cochlear nerve aplasia or hypoplasia is found in up to a half of
      patients with unilateral or bilateral hearing loss. There is an ongoing
      discussion regarding the indication of cochlear implants for hearing
      rehabilitation in cases with radiologically-defined aplasia or hypoplasia of the 
      cochlear nerve in those patients, especially in children. At present there is
      conflicting evidence whether the audiological outcomes of those children with a
      CI are comparable to those of children with a CI and a radiologically-normal
      cochlear nerve. The primary aim of this study was to assess the audiological
      abilities before and after CI provision in children with cochlear nerve
      hypoplasia or aplasia. Additionally, we aimed to determine if audiological
      outcomes differed in children with aplasia from those with hypoplasia. Such data 
      should be helpful in determining if CI provision is appropriate for such
      children. METHODS: This retrospective study presents 7 children who were
      diagnosed with cochlear nerve aplasia or hypoplasia and received a CI. The pre-
      and postoperative audiological performance and the hearing and speech development
      of the children were examined. RESULTS: 4 children were unilateral CI users and 3
      were bilateral CI users. Hearing reactions could be detected in all children.
      Already at first fitting, prompt responses and reactions to songs were observed. 
      The aided thresholds in free field in children with hypoplasia were between 30
      and 60dB. Even in children with aplasia, the results in free field with CI
      averaged between 30 and 70dB. Therefore the aided thresholds in children with
      hypoplasia and in children with aplasia of the CN are similar. It could be
      demonstrated that hearing reactions improve with the long term use of the
      implant. Improvement in general development could be observed in all children
      despite the very heterogeneous conditions and the accompanying handicaps.
      CONCLUSION: The results of this study support the hypothesis that children with
      radiologically-defined CN hypoplasia or aplasia and detectable responses to
      electrical or acoustical stimuli can improve their sound detection thresholds and
      their awareness of sound when provided with a CI.
CI  - Copyright (c) 2018 Elsevier B.V. All rights reserved.
FAU - Ehrmann-Muller, Desiree
AU  - Ehrmann-Muller D
AD  - Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and 
      Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080 Wuerzburg, 
      Germany. Electronic address: ehrmann_d@ukw.de.
FAU - Kuhn, Heike
AU  - Kuhn H
AD  - Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and 
      Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080 Wuerzburg, 
      Germany.
FAU - Matthies, Cordula
AU  - Matthies C
AD  - Department of Neurosurgery, University of Wuerzburg, Josef-Schneider-Str. 11,
      97080 Wuerzburg, Germany.
FAU - Hagen, Rudolf
AU  - Hagen R
AD  - Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and 
      Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080 Wuerzburg, 
      Germany.
FAU - Shehata-Dieler, Wafaa
AU  - Shehata-Dieler W
AD  - Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and 
      Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080 Wuerzburg, 
      Germany.
LA  - eng
PT  - Journal Article
DEP - 20180623
PL  - Ireland
TA  - Int J Pediatr Otorhinolaryngol
JT  - International journal of pediatric otorhinolaryngology
JID - 8003603
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - *Cochlear Implantation
MH  - Cochlear Nerve/*abnormalities
MH  - Female
MH  - Follow-Up Studies
MH  - Hearing Loss, Sensorineural/etiology/*surgery
MH  - Humans
MH  - Infant
MH  - Male
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Vestibulocochlear Nerve Diseases/complications/congenital/*surgery
OTO - NOTNLM
OT  - Cochlear implantation
OT  - Cochlear nerve aplasia
OT  - Hearing development
EDAT- 2018/07/30 06:00
MHDA- 2018/10/27 06:00
CRDT- 2018/07/30 06:00
PHST- 2018/02/06 00:00 [received]
PHST- 2018/06/22 00:00 [revised]
PHST- 2018/06/23 00:00 [accepted]
PHST- 2018/07/30 06:00 [entrez]
PHST- 2018/07/30 06:00 [pubmed]
PHST- 2018/10/27 06:00 [medline]
AID - S0165-5876(18)30282-9 [pii]
AID - 10.1016/j.ijporl.2018.06.038 [doi]
PST - ppublish
SO  - Int J Pediatr Otorhinolaryngol. 2018 Sep;112:132-140. doi:
      10.1016/j.ijporl.2018.06.038. Epub 2018 Jun 23.