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Inner ear malformation

MedGen UID:
906478
•Concept ID:
C4231418
•
Finding

Professional guidelines

PubMed

Prenatal diagnosis of vermian cyst: a new type of posterior fossa cyst.
Chanclud J, Valence S, Perre SV, Guilbaud L, Moutard ML, Jouannic JM, Ducou Le Pointe H, Blondiaux E, Garel C
Pediatr Radiol 2023 Mar;53(3):461-469. Epub 2022 Oct 24 doi: 10.1007/s00247-022-05531-3. PMID: 36274068
A comprehensive genotype-phenotype evaluation of eight Chinese probands with Waardenburg syndrome.
Li S, Qin M, Mao S, Mei L, Cai X, Feng Y, He C, Song J
BMC Med Genomics 2022 Nov 3;15(1):230. doi: 10.1186/s12920-022-01379-6. PMID: 36329483Free PMC Article
Analysis between phenotypes and genotypes of inner ear malformation.
Wang S, Ding W, Chen C, Xu B, Liu X, Bian P, Guo Y
Acta Otolaryngol 2019 Mar;139(3):223-232. Epub 2019 Feb 14 doi: 10.1080/00016489.2018.1554262. PMID: 30762457

Recent clinical studies

Etiology

Feasibility of day-case pediatric cochlear implantation.
Hugel M, Ayari-Khalfallah S, Fieux M, Coudert A, Truy E, Hermann R
Eur Arch Otorhinolaryngol 2022 Nov;279(11):5123-5133. Epub 2022 Apr 27 doi: 10.1007/s00405-022-07353-3. PMID: 35476132
Subtotal petrosectomy and cochlear implantation.
D'Angelo G, Donati G, Bacciu A, Guida M, Falcioni M
Acta Otorhinolaryngol Ital 2020 Dec;40(6):450-456. doi: 10.14639/0392-100X-N0931. PMID: 33558774Free PMC Article
New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review.
Hong R, Du Q, Pan Y
AJNR Am J Neuroradiol 2020 Jun;41(6):1076-1080. Epub 2020 May 28 doi: 10.3174/ajnr.A6576. PMID: 32467182Free PMC Article
X-linked Malformation and Cochlear Implantation.
Smeds H, Wales J, Asp F, Löfkvist U, Falahat B, Anderlid BM, Anmyr L, Karltorp E
Otol Neurotol 2017 Jan;38(1):38-46. doi: 10.1097/MAO.0000000000001253. PMID: 27779564
Auditory brainstem implant program development.
Schwartz MS, Wilkinson EP
Laryngoscope 2017 Aug;127(8):1909-1915. Epub 2016 Sep 26 doi: 10.1002/lary.26312. PMID: 27716925

Diagnosis

Impact of inner ear malformation and cochlear nerve deficiency on the development of auditory-language network in children with profound sensorineural hearing loss.
Wang Y, Jiang M, Zhu Y, Xue L, Shu W, Li X, Chen H, Li Y, Chen Y, Chai Y, Zhang Y, Chu Y, Song Y, Tao X, Wang Z, Wu H
Elife 2023 Sep 12;12 doi: 10.7554/eLife.85983. PMID: 37697742Free PMC Article
Imaging Guide to Inner Ear Malformations: An Illustrative Review.
Yu FF, Feltrin FS, Bathla G, Raj K, Agarwal A, Lee WC, Booth T, Singh A
Curr Probl Diagn Radiol 2023 Nov-Dec;52(6):576-585. Epub 2023 Jul 4 doi: 10.1067/j.cpradiol.2023.06.010. PMID: 37500297
Acute myeloid leukemia associated with CHARGE syndrome.
Shuto M, Hirano N, Oguri S, Itonaga T, Inoue M, Suenobu S, Ihara K
Am J Med Genet A 2023 Mar;191(3):878-881. Epub 2022 Dec 21 doi: 10.1002/ajmg.a.63087. PMID: 36543163
Analysis between phenotypes and genotypes of inner ear malformation.
Wang S, Ding W, Chen C, Xu B, Liu X, Bian P, Guo Y
Acta Otolaryngol 2019 Mar;139(3):223-232. Epub 2019 Feb 14 doi: 10.1080/00016489.2018.1554262. PMID: 30762457
Otologic disorders causing dizziness, including surgery for vestibular disorders.
Bertholon P, Karkas A
Handb Clin Neurol 2016;137:279-93. doi: 10.1016/B978-0-444-63437-5.00020-0. PMID: 27638078

Therapy

Cerebrospinal Fluid Gusher in Cochlear Implantation and Its Association with Inner-Ear Malformations.
Dalgic A, Atsal G, Ceylan ME, Aydın E, Adıbelli ZH, Edizer DT, Olgun L
J Int Adv Otol 2022 Nov;18(6):478-481. doi: 10.5152/iao.2022.21441. PMID: 36349668Free PMC Article
Cochlear Implantation in Inner Ear Malformations: Considerations Related to Surgical Complications and Communication Skills.
Karamert R, Tutar H, Altinyay Ş, Düzlü M, Yildiz M, Akdulum İ, Uğur MB, Çolak M, Cebeci S, Şahin MM, Orçan E, Bayazit YA
ORL J Otorhinolaryngol Relat Spec 2022;84(3):211-218. Epub 2021 Sep 9 doi: 10.1159/000517562. PMID: 34500448
The misplaced cochlear implant electrode array.
Mehanna AM, Gamaleldin OA, Fathalla MF
Int J Pediatr Otorhinolaryngol 2019 Feb;117:96-104. Epub 2018 Nov 23 doi: 10.1016/j.ijporl.2018.11.027. PMID: 30579097
X-linked Malformation and Cochlear Implantation.
Smeds H, Wales J, Asp F, Löfkvist U, Falahat B, Anderlid BM, Anmyr L, Karltorp E
Otol Neurotol 2017 Jan;38(1):38-46. doi: 10.1097/MAO.0000000000001253. PMID: 27779564
Cochlear implant and inner ear malformation. Proposal for an hyperosmolar therapy at surgery.
Loundon N, Leboulanger N, Maillet J, Riggouzzo A, Richard P, Marlin S, Garabedian EN
Int J Pediatr Otorhinolaryngol 2008 Apr;72(4):541-7. Epub 2008 Feb 20 doi: 10.1016/j.ijporl.2008.01.004. PMID: 18282614

Prognosis

Non-Syndromic Sensorineural Hearing Loss in Children.
Robson CD, Lewis M, D'Arco F
Neuroimaging Clin N Am 2023 Nov;33(4):531-542. Epub 2023 Jul 11 doi: 10.1016/j.nic.2023.05.005. PMID: 37741656
Impact of inner ear malformation and cochlear nerve deficiency on the development of auditory-language network in children with profound sensorineural hearing loss.
Wang Y, Jiang M, Zhu Y, Xue L, Shu W, Li X, Chen H, Li Y, Chen Y, Chai Y, Zhang Y, Chu Y, Song Y, Tao X, Wang Z, Wu H
Elife 2023 Sep 12;12 doi: 10.7554/eLife.85983. PMID: 37697742Free PMC Article
Feasibility of day-case pediatric cochlear implantation.
Hugel M, Ayari-Khalfallah S, Fieux M, Coudert A, Truy E, Hermann R
Eur Arch Otorhinolaryngol 2022 Nov;279(11):5123-5133. Epub 2022 Apr 27 doi: 10.1007/s00405-022-07353-3. PMID: 35476132
A novel cochlear measurement that predicts inner-ear malformation.
Khurayzi T, Almuhawas F, Alsanosi A, Abdelsamad Y, Doyle Ú, Dhanasingh A
Sci Rep 2021 Apr 1;11(1):7339. doi: 10.1038/s41598-021-86741-x. PMID: 33795738Free PMC Article
Inner-ear malformations as a cause of single-sided deafness.
Tahir E, Bajin MD, Jafarov S, Yıldırım MÖ, Çınar BÇ, Sennaroğlu G, Sennaroğlu L
J Laryngol Otol 2020 Jun;134(6):509-518. Epub 2020 Jun 8 doi: 10.1017/S0022215120001036. PMID: 32508296

Clinical prediction guides

A clinical epidemiological study on congenital ear malformation (CEM).
Zhang M, Lin J, Zhang J, Han Z, Liu C, Zou Y
Acta Otolaryngol 2023 Nov;143(sup1):S17-S24. Epub 2024 Feb 13 doi: 10.1080/00016489.2023.2276348. PMID: 38071650
Feasibility of day-case pediatric cochlear implantation.
Hugel M, Ayari-Khalfallah S, Fieux M, Coudert A, Truy E, Hermann R
Eur Arch Otorhinolaryngol 2022 Nov;279(11):5123-5133. Epub 2022 Apr 27 doi: 10.1007/s00405-022-07353-3. PMID: 35476132
A novel cochlear measurement that predicts inner-ear malformation.
Khurayzi T, Almuhawas F, Alsanosi A, Abdelsamad Y, Doyle Ú, Dhanasingh A
Sci Rep 2021 Apr 1;11(1):7339. doi: 10.1038/s41598-021-86741-x. PMID: 33795738Free PMC Article
Enlarged vestibular aqueduct and Mondini Malformation: audiological, clinical, radiologic and genetic features.
Forli F, Lazzerini F, Auletta G, Bruschini L, Berrettini S
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2305-2312. Epub 2020 Sep 10 doi: 10.1007/s00405-020-06333-9. PMID: 32910226Free PMC Article
X-linked Malformation and Cochlear Implantation.
Smeds H, Wales J, Asp F, Löfkvist U, Falahat B, Anderlid BM, Anmyr L, Karltorp E
Otol Neurotol 2017 Jan;38(1):38-46. doi: 10.1097/MAO.0000000000001253. PMID: 27779564

Recent systematic reviews

Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.
Corazzi V, Fordington S, Brown TH, Donnelly N, Bewick J, Ehsani D, Pelucchi S, Bianchini C, Ciorba A, Borsetto D
Eur Arch Otorhinolaryngol 2024 Jul;281(7):3397-3421. Epub 2024 Feb 27 doi: 10.1007/s00405-024-08527-x. PMID: 38411671

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