Cochlear implantation in children with congenital inner ear
anomalies: challenges and outcomes

J 2026

Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes

URÍK, Milan; Dagmar HOŠNOVÁ; Vít KRUNTORÁD a Jan SIMA

Základní údaje

Originální název

Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes

Autoři

URÍK, Milan; Dagmar HOŠNOVÁ; Vít KRUNTORÁD a Jan SIMA

Vydání

European Archives of Oto-Rhino-Laryngology, New York, Springer, 2026, 0937-4477

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30206 Otorhinolaryngology

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 2.200 v roce 2024

Označené pro přenos do RIV

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

Cochlear Implantation; Inner Ear Malformations; Children

Štítky

14110319

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 10. 3. 2026 10:39, Mgr. Tereza Miškechová

Anotace

V originále

Purpose Cochlear implantation (CI) is the state-of-the-art treatment option for sensorineural hearing loss condition including patients with congenital inner ear malformations. Recently our centre started treating children with CI who were diagnosed with inner ear malformations. Objectives of this retrospective study are to create three-dimensional (3D) model of malformed inner ears, identification of cochlear nerve bundle from pre-operative images, visualize electrode placement inside the cochlear portion from post-operative images, and evaluate hearing benefits post-operatively received from CI. Methods Slicer software was used to 3D segment the inner ear and the electrode from the pre-, and post of CT scans. Using the same software, cross-section of internal auditory canal (IAC) was navigated to visualize the presence or absence of cochlear nerve. Speech intelligibility rating (SIR) and pure tone average (PTA) thresholds were evaluated for the benefits received from CI. Results Our database showed a total of 12 children radiologically diagnosed with inner ear malformation and out of which 19 ears were treated with MED-EL CI devices. Enlarged vestibular aqueduct (EVA, incomplete partition (IP) types I, and II, cochlear hypoplasia (CH), cochlear aperture stenosis, common cavity (CA) were the different malformation types found in this cohort. Intra-operative gusher was observed in EVA and IP type II malformation types. Higher SIR scores and lower PTA thresholds were seen for less severe malformation types and vice-versa. Conclusions Severity of malformation have a negative effect on the hearing outcomes with CI. MRI is an important tool in the identification of cochlear nerve bundle in subjects diagnosed with malformed inner ear anatomies.

Návaznosti

MUNI/A/1543/2024, interní kód MU

Název: Personalizovaný přístup v léčbě dětí se středoušním zánětem vzhledem k analýze bakteriomu a rezistence na antibiotika

Investor: Masarykova univerzita, Personalizovaný přístup v léčbě dětí se středoušním zánětem vzhledem k analýze bakteriomu a rezistence na antibiotika

Citovat

URÍK, Milan; Dagmar HOŠNOVÁ; Vít KRUNTORÁD a Jan SIMA. Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes. European Archives of Oto-Rhino-Laryngology. New York: Springer, 2026. ISSN 0937-4477. Dostupné z: https://doi.org/10.1007/s00405-025-09825-8.

@article{2534002,
   author = {Urík, Milan and Hošnová, Dagmar and Kruntorád, Vít and Sima, Jan},
   article_location = {New York},
   doi = {https://doi.org/10.1007/s00405-025-09825-8},
   keywords = {Cochlear Implantation; Inner Ear Malformations; Children},
   language = {eng},
   issn = {0937-4477},
   journal = {European Archives of Oto-Rhino-Laryngology},
   title = {Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes},
   url = {https://link.springer.com/article/10.1007/s00405-025-09825-8},
   year = {2026}
}

TY  - JOUR
ID  - 2534002
AU  - Urík, Milan - Hošnová, Dagmar - Kruntorád, Vít - Sima, Jan
PY  - 2026
TI  - Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes
JF  - European Archives of Oto-Rhino-Laryngology
PB  - Springer
SN  - 09374477
KW  - Cochlear Implantation
KW  - Inner Ear Malformations
KW  - Children
UR  - https://link.springer.com/article/10.1007/s00405-025-09825-8
N2  - Purpose Cochlear implantation (CI) is the state-of-the-art treatment option for sensorineural hearing loss condition including patients with congenital inner ear malformations. Recently our centre started treating children with CI who were diagnosed with inner ear malformations. Objectives of this retrospective study are to create three-dimensional (3D) model of malformed inner ears, identification of cochlear nerve bundle from pre-operative images, visualize electrode placement inside the cochlear portion from post-operative images, and evaluate hearing benefits post-operatively received from CI. Methods Slicer software was used to 3D segment the inner ear and the electrode from the pre-, and post of CT scans. Using the same software, cross-section of internal auditory canal (IAC) was navigated to visualize the presence or absence of cochlear nerve. Speech intelligibility rating (SIR) and pure tone average (PTA) thresholds were evaluated for the benefits received from CI. Results Our database showed a total of 12 children radiologically diagnosed with inner ear malformation and out of which 19 ears were treated with MED-EL CI devices. Enlarged vestibular aqueduct (EVA, incomplete partition (IP) types I, and II, cochlear hypoplasia (CH), cochlear aperture stenosis, common cavity (CA) were the different malformation types found in this cohort. Intra-operative gusher was observed in EVA and IP type II malformation types. Higher SIR scores and lower PTA thresholds were seen for less severe malformation types and vice-versa. Conclusions Severity of malformation have a negative effect on the hearing outcomes with CI. MRI is an important tool in the identification of cochlear nerve bundle in subjects diagnosed with malformed inner ear anatomies.
ER  -

URÍK, Milan; Dagmar HOŠNOVÁ; Vít KRUNTORÁD a Jan SIMA. Cochlear implantation in children with congenital inner ear anomalies: challenges and outcomes. \textit{European Archives of Oto-Rhino-Laryngology}. New York: Springer, 2026. ISSN~0937-4477. Dostupné z: https://doi.org/10.1007/s00405-025-09825-8.

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