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Deafness, sensorineural, with neurologic features

MedGen UID:
463325
Concept ID:
C3151975
Finding
Synonym: DEAFNESS, SENSORINEURAL, WITH NEUROLOGIC FEATURES
 
OMIM®: 590080

Professional guidelines

PubMed

Na JH, Lee YM
Biomolecules 2024 Nov 28;14(12) doi: 10.3390/biom14121524. PMID: 39766231Free PMC Article
Bose S, Papathanasiou A, Karkhanis S, Appleton JP, King D, Batra R, Mollan SP, Jacob S
J Neurol 2023 Dec;270(12):6193-6206. Epub 2023 Aug 22 doi: 10.1007/s00415-023-11891-z. PMID: 37608221Free PMC Article
Ortigoza-Escobar JD, Molero-Luis M, Arias A, Martí-Sánchez L, Rodriguez-Pombo P, Artuch R, Pérez-Dueñas B
Expert Rev Neurother 2016 Jul;16(7):755-63. Epub 2016 May 23 doi: 10.1080/14737175.2016.1187562. PMID: 27191787

Recent clinical studies

Etiology

Garcia-Moreno H, Langbehn DR, Abiona A, Garrood I, Fleszar Z, Manes MA, Morley AMS, Craythorne E, Mohammed S, Henshaw T, Turner S, Naik H, Bodi I, Sarkany RPE, Fassihi H, Lehmann AR, Giunti P
Brain 2023 Dec 1;146(12):5044-5059. doi: 10.1093/brain/awad266. PMID: 38040034Free PMC Article
Ng YS, Lax NZ, Blain AP, Erskine D, Baker MR, Polvikoski T, Thomas RH, Morris CM, Lai M, Whittaker RG, Gebbels A, Winder A, Hall J, Feeney C, Farrugia ME, Hirst C, Roberts M, Lawthom C, Chrysostomou A, Murphy K, Baird T, Maddison P, Duncan C, Poulton J, Nesbitt V, Hanna MG, Pitceathly RDS, Taylor RW, Blakely EL, Schaefer AM, Turnbull DM, McFarland R, Gorman GS
Brain 2022 Apr 18;145(2):542-554. doi: 10.1093/brain/awab353. PMID: 34927673Free PMC Article
Abeti R, Zeitlberger A, Peelo C, Fassihi H, Sarkany RPE, Lehmann AR, Giunti P
Br J Pharmacol 2019 Nov;176(22):4293-4301. Epub 2019 Jan 23 doi: 10.1111/bph.14557. PMID: 30499105Free PMC Article
Karikkineth AC, Scheibye-Knudsen M, Fivenson E, Croteau DL, Bohr VA
Ageing Res Rev 2017 Jan;33:3-17. Epub 2016 Aug 6 doi: 10.1016/j.arr.2016.08.002. PMID: 27507608Free PMC Article
Rizzo WB, Jenkens SM, Boucher P
Semin Neurol 2012 Feb;32(1):75-84. Epub 2012 Mar 15 doi: 10.1055/s-0032-1306390. PMID: 22422210Free PMC Article

Diagnosis

Kaler A, Couser N
J AAPOS 2025 Feb;29(1):104102. Epub 2024 Dec 31 doi: 10.1016/j.jaapos.2024.104102. PMID: 39746537
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
Abeti R, Zeitlberger A, Peelo C, Fassihi H, Sarkany RPE, Lehmann AR, Giunti P
Br J Pharmacol 2019 Nov;176(22):4293-4301. Epub 2019 Jan 23 doi: 10.1111/bph.14557. PMID: 30499105Free PMC Article
Karikkineth AC, Scheibye-Knudsen M, Fivenson E, Croteau DL, Bohr VA
Ageing Res Rev 2017 Jan;33:3-17. Epub 2016 Aug 6 doi: 10.1016/j.arr.2016.08.002. PMID: 27507608Free PMC Article
Pingault V, Ente D, Dastot-Le Moal F, Goossens M, Marlin S, Bondurand N
Hum Mutat 2010 Apr;31(4):391-406. doi: 10.1002/humu.21211. PMID: 20127975

Therapy

Ortigoza-Escobar JD, Molero-Luis M, Arias A, Martí-Sánchez L, Rodriguez-Pombo P, Artuch R, Pérez-Dueñas B
Expert Rev Neurother 2016 Jul;16(7):755-63. Epub 2016 May 23 doi: 10.1080/14737175.2016.1187562. PMID: 27191787
Bradberry SM, Wilkinson JM, Ferner RE
Clin Toxicol (Phila) 2014 Sep-Oct;52(8):837-47. Epub 2014 Aug 16 doi: 10.3109/15563650.2014.944977. PMID: 25132471
Pfeffer G, Majamaa K, Turnbull DM, Thorburn D, Chinnery PF
Cochrane Database Syst Rev 2012 Apr 18;2012(4):CD004426. doi: 10.1002/14651858.CD004426.pub3. PMID: 22513923Free PMC Article
Chinnery P, Majamaa K, Turnbull D, Thorburn D
Cochrane Database Syst Rev 2006 Jan 25;(1):CD004426. doi: 10.1002/14651858.CD004426.pub2. PMID: 16437486
Román GC
J Neurol Sci 1994 Dec 1;127(1):11-28. doi: 10.1016/0022-510x(94)90130-9. PMID: 7699385

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
Garcia-Moreno H, Langbehn DR, Abiona A, Garrood I, Fleszar Z, Manes MA, Morley AMS, Craythorne E, Mohammed S, Henshaw T, Turner S, Naik H, Bodi I, Sarkany RPE, Fassihi H, Lehmann AR, Giunti P
Brain 2023 Dec 1;146(12):5044-5059. doi: 10.1093/brain/awad266. PMID: 38040034Free PMC Article
Taketani T
Subcell Biochem 2015;76:309-22. doi: 10.1007/978-94-017-7197-9_14. PMID: 26219717
Sathasivam S
Orphanet J Rare Dis 2008 Apr 17;3:9. doi: 10.1186/1750-1172-3-9. PMID: 18416855Free PMC Article
Sclafani AP, DeDio RM, Hendrix RA
Ear Nose Throat J 1991 Apr;70(4):208-12. PMID: 1874153

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
Garcia-Moreno H, Langbehn DR, Abiona A, Garrood I, Fleszar Z, Manes MA, Morley AMS, Craythorne E, Mohammed S, Henshaw T, Turner S, Naik H, Bodi I, Sarkany RPE, Fassihi H, Lehmann AR, Giunti P
Brain 2023 Dec 1;146(12):5044-5059. doi: 10.1093/brain/awad266. PMID: 38040034Free PMC Article
Bose S, Papathanasiou A, Karkhanis S, Appleton JP, King D, Batra R, Mollan SP, Jacob S
J Neurol 2023 Dec;270(12):6193-6206. Epub 2023 Aug 22 doi: 10.1007/s00415-023-11891-z. PMID: 37608221Free 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
Ng YS, Lax NZ, Blain AP, Erskine D, Baker MR, Polvikoski T, Thomas RH, Morris CM, Lai M, Whittaker RG, Gebbels A, Winder A, Hall J, Feeney C, Farrugia ME, Hirst C, Roberts M, Lawthom C, Chrysostomou A, Murphy K, Baird T, Maddison P, Duncan C, Poulton J, Nesbitt V, Hanna MG, Pitceathly RDS, Taylor RW, Blakely EL, Schaefer AM, Turnbull DM, McFarland R, Gorman GS
Brain 2022 Apr 18;145(2):542-554. doi: 10.1093/brain/awab353. PMID: 34927673Free PMC Article

Recent systematic reviews

Pfeffer G, Majamaa K, Turnbull DM, Thorburn D, Chinnery PF
Cochrane Database Syst Rev 2012 Apr 18;2012(4):CD004426. doi: 10.1002/14651858.CD004426.pub3. PMID: 22513923Free PMC Article
Chinnery P, Majamaa K, Turnbull D, Thorburn D
Cochrane Database Syst Rev 2006 Jan 25;(1):CD004426. doi: 10.1002/14651858.CD004426.pub2. PMID: 16437486

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