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Deafness, prelingual, profound (affects all frequencies)

MedGen UID:
387913
•Concept ID:
C1857810
•
Finding

Professional guidelines

PubMed

Epidemiology, aetiology and diagnosis of congenital hearing loss via hearing screening of 153 913 newborns.
Yoshimura H, Okubo T, Shinagawa J, Nishio SY, Takumi Y, Usami SI
Int J Epidemiol 2024 Apr 11;53(3) doi: 10.1093/ije/dyae052. PMID: 38609324Free PMC Article
Usher Syndrome on the Island of Ireland: A Genotype-Phenotype Review.
Stephenson KAJ, Whelan L, Zhu J, Dockery A, Wynne NC, Cairns RM, Kirk C, Turner J, Duignan ES, O'Byrne JJ, Silvestri G, Kenna PF, Farrar GJ, Keegan DJ
Invest Ophthalmol Vis Sci 2023 Jul 3;64(10):23. doi: 10.1167/iovs.64.10.23. PMID: 37466950Free PMC Article
Cost-effectiveness of Universal and Targeted Newborn Screening for Congenital Cytomegalovirus Infection.
Gantt S, Dionne F, Kozak FK, Goshen O, Goldfarb DM, Park AH, Boppana SB, Fowler K
JAMA Pediatr 2016 Dec 1;170(12):1173-1180. doi: 10.1001/jamapediatrics.2016.2016. PMID: 27723885

Recent clinical studies

Etiology

Usher Syndrome on the Island of Ireland: A Genotype-Phenotype Review.
Stephenson KAJ, Whelan L, Zhu J, Dockery A, Wynne NC, Cairns RM, Kirk C, Turner J, Duignan ES, O'Byrne JJ, Silvestri G, Kenna PF, Farrar GJ, Keegan DJ
Invest Ophthalmol Vis Sci 2023 Jul 3;64(10):23. doi: 10.1167/iovs.64.10.23. PMID: 37466950Free PMC Article
Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Ciliopathy: Usher Syndrome.
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
Sign Language and Spoken Language for Children With Hearing Loss: A Systematic Review.
Fitzpatrick EM, Hamel C, Stevens A, Pratt M, Moher D, Doucet SP, Neuss D, Bernstein A, Na E
Pediatrics 2016 Jan;137(1) Epub 2015 Dec 18 doi: 10.1542/peds.2015-1974. PMID: 26684476
Radiotherapy-induced ear toxicity.
Jereczek-Fossa BA, Zarowski A, Milani F, Orecchia R
Cancer Treat Rev 2003 Oct;29(5):417-30. doi: 10.1016/s0305-7372(03)00066-5. PMID: 12972360

Diagnosis

Usher Syndrome on the Island of Ireland: A Genotype-Phenotype Review.
Stephenson KAJ, Whelan L, Zhu J, Dockery A, Wynne NC, Cairns RM, Kirk C, Turner J, Duignan ES, O'Byrne JJ, Silvestri G, Kenna PF, Farrar GJ, Keegan DJ
Invest Ophthalmol Vis Sci 2023 Jul 3;64(10):23. doi: 10.1167/iovs.64.10.23. PMID: 37466950Free PMC Article
Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Genetic hearing impairment.
Ječmenica J, Bajec-Opančina A, Ječmenica D
Childs Nerv Syst 2015 Apr;31(4):515-9. Epub 2015 Feb 17 doi: 10.1007/s00381-015-2628-3. PMID: 25686889
Auditory dysfunction in patients with cerebrovascular disease.
Tabuchi S
ScientificWorldJournal 2014;2014:261824. Epub 2014 Oct 23 doi: 10.1155/2014/261824. PMID: 25401133Free PMC Article
Radiotherapy-induced ear toxicity.
Jereczek-Fossa BA, Zarowski A, Milani F, Orecchia R
Cancer Treat Rev 2003 Oct;29(5):417-30. doi: 10.1016/s0305-7372(03)00066-5. PMID: 12972360

Therapy

Analysis of the vestibular aqueduct development on the risk for suffering from idiopathic sudden sensorineural hearing loss.
Fan X, Zhu Q, Hou H, Hou L, Wang Z, Zhang H, Xu A
Auris Nasus Larynx 2023 Oct;50(5):733-742. Epub 2023 Feb 14 doi: 10.1016/j.anl.2023.01.013. PMID: 36797195
Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.
Ryan P, Huins CT, O'Brien KJ, Misra S, Birman CS
Int J Pediatr Otorhinolaryngol 2023 Feb;165:111445. Epub 2023 Jan 7 doi: 10.1016/j.ijporl.2023.111445. PMID: 36630865
Intratympanic steroids as a salvage therapy for severe to profound idiopathic sudden sensorineural hearing loss.
Covelli E, Al Tabaa K, Verillaud B, Camous D, Hautefort C, Barbara M, Herman P, Kania R
Acta Otolaryngol 2018 Nov;138(11):966-971. Epub 2018 Oct 31 doi: 10.1080/00016489.2018.1497805. PMID: 30380957
Bilateral Deafness as a Complication of the Vaccination-A Case Report.
Rikitake M, Sampei S, Komori M, Sakurai Y, Kojima H
Int Tinnitus J 2018 Jun 1;22(1):19-22. doi: 10.5935/0946-5448.20180003. PMID: 29993212
Cochlear implantation in unilateral deaf subjects associated with ipsilateral tinnitus.
Buechner A, Brendel M, Lesinski-Schiedat A, Wenzel G, Frohne-Buechner C, Jaeger B, Lenarz T
Otol Neurotol 2010 Dec;31(9):1381-5. doi: 10.1097/MAO.0b013e3181e3d353. PMID: 20729788

Prognosis

Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Universal newborn hearing screening in the Italian Region of Sicily in 2018.
Ferlito S, Maniaci A, Cocuzza S, La Mantia I, Di Mauro P, Poli G, Maiolino L, Coco S, Merlino F, Maltese M, Ragliani M, Russo M, Gulino A, Azieli C, Martines F, Galletti F, Bubbico L
Acta Otorhinolaryngol Ital 2021 Aug;41(4):356-363. doi: 10.14639/0392-100X-N1162. PMID: 34533539Free PMC Article
Identification of Hearing Loss-Associated Variants of PTPRQ, MYO15A, and SERPINB6 in Pakistani Families.
Mahmood U, Bukhari SA, Ali M, Ahmed ZM, Riazuddin S
Biomed Res Int 2021;2021:5584788. Epub 2021 Apr 24 doi: 10.1155/2021/5584788. PMID: 33997018Free PMC Article
Frequency Of Congenital Hearing Loss In Neonates.
Ahmed S, Sheraz S, Malik SA, Ahmed NR, Malik SA, Farooq S, Raheem A, Basheer F, Nayyar ZA; Fazal-e-Malik
J Ayub Med Coll Abbottabad 2018 Apr-Jun;30(2):234-236. PMID: 29938425
Radiotherapy-induced ear toxicity.
Jereczek-Fossa BA, Zarowski A, Milani F, Orecchia R
Cancer Treat Rev 2003 Oct;29(5):417-30. doi: 10.1016/s0305-7372(03)00066-5. PMID: 12972360

Clinical prediction guides

Hearing Loss and Its Burden of Disease in a Large German Cohort-Hearing Loss in Germany.
Hackenberg B, Döge J, Lackner KJ, Beutel ME, Münzel T, Pfeiffer N, Nagler M, Schmidtmann I, Wild PS, Matthias C, Bahr K
Laryngoscope 2022 Sep;132(9):1843-1849. Epub 2021 Dec 14 doi: 10.1002/lary.29980. PMID: 34904723
Audiological Features in 63 Patients With Cochlear Nerve Deficiency.
Matsuura K, Yoshimura H, Shinagawa J, Kurozumi M, Takumi Y
Otol Neurotol 2022 Jan 1;43(1):23-28. doi: 10.1097/MAO.0000000000003365. PMID: 34538855
Universal newborn hearing screening in the Italian Region of Sicily in 2018.
Ferlito S, Maniaci A, Cocuzza S, La Mantia I, Di Mauro P, Poli G, Maiolino L, Coco S, Merlino F, Maltese M, Ragliani M, Russo M, Gulino A, Azieli C, Martines F, Galletti F, Bubbico L
Acta Otorhinolaryngol Ital 2021 Aug;41(4):356-363. doi: 10.14639/0392-100X-N1162. PMID: 34533539Free PMC Article
Identification of Hearing Loss-Associated Variants of PTPRQ, MYO15A, and SERPINB6 in Pakistani Families.
Mahmood U, Bukhari SA, Ali M, Ahmed ZM, Riazuddin S
Biomed Res Int 2021;2021:5584788. Epub 2021 Apr 24 doi: 10.1155/2021/5584788. PMID: 33997018Free PMC Article
Frequency Of Congenital Hearing Loss In Neonates.
Ahmed S, Sheraz S, Malik SA, Ahmed NR, Malik SA, Farooq S, Raheem A, Basheer F, Nayyar ZA; Fazal-e-Malik
J Ayub Med Coll Abbottabad 2018 Apr-Jun;30(2):234-236. PMID: 29938425

Recent systematic reviews

Characterization of Sensorineural Hearing Loss in Patients With MYH9-Related Disease: A Systematic Review.
Peddu D, Amin S, Ying YM
Otol Neurotol 2022 Mar 1;43(3):e298-e308. doi: 10.1097/MAO.0000000000003450. PMID: 35147601
Sign Language and Spoken Language for Children With Hearing Loss: A Systematic Review.
Fitzpatrick EM, Hamel C, Stevens A, Pratt M, Moher D, Doucet SP, Neuss D, Bernstein A, Na E
Pediatrics 2016 Jan;137(1) Epub 2015 Dec 18 doi: 10.1542/peds.2015-1974. PMID: 26684476

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