U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Childhood onset sensorineural hearing impairment

MedGen UID:
868928
Concept ID:
C4023340
Disease or Syndrome
HPO: HP:0011474

Definition

Sensorineural hearing impairment with childhood onset. [from HPO]

Term Hierarchy

Conditions with this feature

Otosclerosis 7
MedGen UID:
409738
Concept ID:
C1969044
Disease or Syndrome

Professional guidelines

PubMed

Gilbey P, Kraus C, Ghanayim R, Sharabi-Nov A, Bretler S
Int J Pediatr Otorhinolaryngol 2013 Jan;77(1):97-100. Epub 2012 Nov 2 doi: 10.1016/j.ijporl.2012.10.004. PMID: 23122541

Recent clinical studies

Etiology

Carlson RJ, Walsh T, Mandell JB, Aburayyan A, Lee MK, Gulsuner S, Horn DL, Ou HC, Sie KCY, Mancl L, Rubinstein J, King MC
JAMA Otolaryngol Head Neck Surg 2023 Mar 1;149(3):212-222. doi: 10.1001/jamaoto.2022.4463. PMID: 36633841Free PMC Article
Moein M, Vlassis IM, Saidi R
Transplant Proc 2022 Dec;54(10):2744-2747. Epub 2022 Nov 8 doi: 10.1016/j.transproceed.2022.10.020. PMID: 36369142
Vos B, Noll D, Whittingham J, Pigeon M, Bagatto M, Fitzpatrick EM
Ear Hear 2021 Nov-Dec 01;42(6):1447-1461. doi: 10.1097/AUD.0000000000001055. PMID: 33928914
Tsang SH, Aycinena ARP, Sharma T
Adv Exp Med Biol 2018;1085:167-170. doi: 10.1007/978-3-319-95046-4_32. PMID: 30578505
Riga M, Korres G, Chouridis P, Naxakis S, Danielides V
Int J Pediatr Otorhinolaryngol 2018 Dec;115:156-164. Epub 2018 Oct 4 doi: 10.1016/j.ijporl.2018.10.005. PMID: 30368378

Diagnosis

Möller B, Becker LL, Saffari A, Afenjar A, Coci EG, Williamson R, Ward-Melver C, Gibaud M, Sedláčková L, Laššuthová P, Libá Z, Vlčková M, William N, Klee EW, Gavrilova RH, Lévy J, Capri Y, Scavina M, Körner RW, Valivullah Z, Weiß C, Möller GM, Frazier Z, Roberts A, Gener B, Scala M, Striano P, Zara F, Thiel M, Sinnema M, Kamsteeg EJ, Donkervoort S, Duboc V, Zaafrane-Khachnaoui K, Elkhateeb N, Selim L, Margot H, Marin V, Beneteau C, Isidor B, Cogne B, Keren B, Küsters B, Beggs AH, Sveden A, Chopra M, Genetti CA, Nicolai J, Dötsch J, Koy A, Bönnemann CG, von der Hagen M, von Kleist-Retzow JC, Voermans NC, Jungbluth H, Dafsari HS
Brain 2025 Feb 3;148(2):597-612. doi: 10.1093/brain/awae183. PMID: 38848546Free PMC Article
Reda Del Barrio S, García Fernández A, Quesada-Espinosa JF, Sánchez-Calvín MT, Gómez-Manjón I, Sierra-Tomillo O, Juárez-Rufián A, de Vergas Gutiérrez J
Acta Otorrinolaringol Esp (Engl Ed) 2024 Mar-Apr;75(2):83-93. Epub 2024 Jan 13 doi: 10.1016/j.otoeng.2023.07.002. PMID: 38224868
Dia Y, Loum B, Dieng YJKB, Diop JPD, Adadey SM, Aboagye ET, Ba SA, Touré AA, Niang F, Diaga Sarr P, Tidiane Ly CA, Sène ARG, Kock C, Bassier R, Popel K, Ndiaye Diallo R, Wonkam A, Diallo BK
Genes (Basel) 2023 Feb 23;14(3) doi: 10.3390/genes14030562. PMID: 36980833Free PMC Article
Carlson RJ, Walsh T, Mandell JB, Aburayyan A, Lee MK, Gulsuner S, Horn DL, Ou HC, Sie KCY, Mancl L, Rubinstein J, King MC
JAMA Otolaryngol Head Neck Surg 2023 Mar 1;149(3):212-222. doi: 10.1001/jamaoto.2022.4463. PMID: 36633841Free PMC Article
Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article

Therapy

Moein M, Vlassis IM, Saidi R
Transplant Proc 2022 Dec;54(10):2744-2747. Epub 2022 Nov 8 doi: 10.1016/j.transproceed.2022.10.020. PMID: 36369142
Vos B, Noll D, Whittingham J, Pigeon M, Bagatto M, Fitzpatrick EM
Ear Hear 2021 Nov-Dec 01;42(6):1447-1461. doi: 10.1097/AUD.0000000000001055. PMID: 33928914
Carreau C, Lenglet T, Mosnier I, Lahlou G, Fargeot G, Weiss N, Demeret S, Salachas F, Veauville-Merllié A, Acquaviva C, Nadjar Y
Ann Clin Transl Neurol 2020 Feb;7(2):250-253. Epub 2020 Feb 5 doi: 10.1002/acn3.50977. PMID: 32022482Free PMC Article
Rizzo F, Ramirez A, Compagnucci C, Salani S, Melzi V, Bordoni A, Fortunato F, Niceforo A, Bresolin N, Comi GP, Bertini E, Nizzardo M, Corti S
Sci Rep 2017 Apr 6;7:46271. doi: 10.1038/srep46271. PMID: 28382968Free PMC Article
Bay A, Keskin M, Hizli S, Uygun H, Dai A, Gumruk F
Int J Hematol 2010 Oct;92(3):524-6. Epub 2010 Sep 11 doi: 10.1007/s12185-010-0681-y. PMID: 20835854

Prognosis

Möller B, Becker LL, Saffari A, Afenjar A, Coci EG, Williamson R, Ward-Melver C, Gibaud M, Sedláčková L, Laššuthová P, Libá Z, Vlčková M, William N, Klee EW, Gavrilova RH, Lévy J, Capri Y, Scavina M, Körner RW, Valivullah Z, Weiß C, Möller GM, Frazier Z, Roberts A, Gener B, Scala M, Striano P, Zara F, Thiel M, Sinnema M, Kamsteeg EJ, Donkervoort S, Duboc V, Zaafrane-Khachnaoui K, Elkhateeb N, Selim L, Margot H, Marin V, Beneteau C, Isidor B, Cogne B, Keren B, Küsters B, Beggs AH, Sveden A, Chopra M, Genetti CA, Nicolai J, Dötsch J, Koy A, Bönnemann CG, von der Hagen M, von Kleist-Retzow JC, Voermans NC, Jungbluth H, Dafsari HS
Brain 2025 Feb 3;148(2):597-612. doi: 10.1093/brain/awae183. PMID: 38848546Free PMC Article
Huang D, Song X, Ma J, Li X, Guo Y, Li M, Luo H, Fang Z, Yang C, Xie L, Jiang L
Eur J Pediatr 2023 Feb;182(2):825-836. Epub 2022 Dec 9 doi: 10.1007/s00431-022-04744-w. PMID: 36484864
Pavone P, Pappalardo XG, Ruggieri M, Falsaperla R, Parano E
Medicine (Baltimore) 2022 Aug 5;101(31):e29413. doi: 10.1097/MD.0000000000029413. PMID: 35945798Free PMC Article
Glick H, Sharma A
Hear Res 2017 Jan;343:191-201. Epub 2016 Sep 6 doi: 10.1016/j.heares.2016.08.012. PMID: 27613397Free PMC Article
Gedalia A, Khan TA, Shetty AK, Dimitriades VR, Espinoza LR
Clin Rheumatol 2016 Jul;35(7):1879-84. Epub 2015 Jan 24 doi: 10.1007/s10067-015-2870-9. PMID: 25616361

Clinical prediction guides

Möller B, Becker LL, Saffari A, Afenjar A, Coci EG, Williamson R, Ward-Melver C, Gibaud M, Sedláčková L, Laššuthová P, Libá Z, Vlčková M, William N, Klee EW, Gavrilova RH, Lévy J, Capri Y, Scavina M, Körner RW, Valivullah Z, Weiß C, Möller GM, Frazier Z, Roberts A, Gener B, Scala M, Striano P, Zara F, Thiel M, Sinnema M, Kamsteeg EJ, Donkervoort S, Duboc V, Zaafrane-Khachnaoui K, Elkhateeb N, Selim L, Margot H, Marin V, Beneteau C, Isidor B, Cogne B, Keren B, Küsters B, Beggs AH, Sveden A, Chopra M, Genetti CA, Nicolai J, Dötsch J, Koy A, Bönnemann CG, von der Hagen M, von Kleist-Retzow JC, Voermans NC, Jungbluth H, Dafsari HS
Brain 2025 Feb 3;148(2):597-612. doi: 10.1093/brain/awae183. PMID: 38848546Free PMC Article
Kalinousky AJ, Luperchio TR, Schrode KM, Harris JR, Zhang L, DeLeon VB, Fahrner JA, Lauer AM, Bjornsson HT
Genes (Basel) 2023 Dec 28;15(1) doi: 10.3390/genes15010048. PMID: 38254937Free PMC Article
Dia Y, Loum B, Dieng YJKB, Diop JPD, Adadey SM, Aboagye ET, Ba SA, Touré AA, Niang F, Diaga Sarr P, Tidiane Ly CA, Sène ARG, Kock C, Bassier R, Popel K, Ndiaye Diallo R, Wonkam A, Diallo BK
Genes (Basel) 2023 Feb 23;14(3) doi: 10.3390/genes14030562. PMID: 36980833Free PMC Article
Carlson RJ, Walsh T, Mandell JB, Aburayyan A, Lee MK, Gulsuner S, Horn DL, Ou HC, Sie KCY, Mancl L, Rubinstein J, King MC
JAMA Otolaryngol Head Neck Surg 2023 Mar 1;149(3):212-222. doi: 10.1001/jamaoto.2022.4463. PMID: 36633841Free PMC Article
Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article

Recent systematic reviews

Pollick SA, Mansour Y, Pesch MH
Curr Opin Otolaryngol Head Neck Surg 2024 Oct 1;32(5):329-338. Epub 2024 Jul 26 doi: 10.1097/MOO.0000000000000996. PMID: 39146216Free PMC Article
Roesch S, O'Sullivan A, Zimmermann G, Mair A, Lipuš C, Mayr JA, Wortmann SB, Rasp G
Laryngoscope 2022 Dec;132(12):2459-2472. Epub 2022 Feb 21 doi: 10.1002/lary.30067. PMID: 35188226Free PMC Article
Vos B, Noll D, Whittingham J, Pigeon M, Bagatto M, Fitzpatrick EM
Ear Hear 2021 Nov-Dec 01;42(6):1447-1461. doi: 10.1097/AUD.0000000000001055. PMID: 33928914
Riga M, Korres G, Chouridis P, Naxakis S, Danielides V
Int J Pediatr Otorhinolaryngol 2018 Dec;115:156-164. Epub 2018 Oct 4 doi: 10.1016/j.ijporl.2018.10.005. PMID: 30368378
Goderis J, De Leenheer E, Smets K, Van Hoecke H, Keymeulen A, Dhooge I
Pediatrics 2014 Nov;134(5):972-82. doi: 10.1542/peds.2014-1173. PMID: 25349318

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...