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Hearing loss, autosomal recessive 94(DFNB94)

MedGen UID:
1679077
Concept ID:
C5193096
Disease or Syndrome
Synonym: Deafness, autosomal recessive 94
 
Gene (location): NARS2 (11q14.1)
 
Monarch Initiative: MONDO:0032749
OMIM®: 618434

Definition

DFNB94 is characterized by prelingual profound sensorineural hearing loss (Simon et al., 2015). [from OMIM]

Clinical features

From HPO
Bilateral sensorineural hearing impairment
MedGen UID:
96788
Concept ID:
C0452138
Disease or Syndrome
A bilateral form of sensorineural hearing impairment.
Abnormal vestibular function
MedGen UID:
334848
Concept ID:
C1843865
Finding
An abnormality of the functioning of the vestibular apparatus.

Recent clinical studies

Etiology

Mu Q, Bai L, Xu B, Du H, Jiang Z, Huang S, Gao B, Wu Q, Zhao H, Dai P, Jiang Y
Prenat Diagn 2024 Aug;44(9):1043-1052. Epub 2024 Mar 15 doi: 10.1002/pd.6550. PMID: 38488843
Leal B, Lopes AC, Peixoto D, Correia L, Almiro MM, Vilar J, Azevedo ML, Bicho MA
Acta Med Port 2023 May 2;36(5):336-342. Epub 2023 Feb 17 doi: 10.20344/amp.18607. PMID: 36799720
Mosrati MA, Fadhlaoui-Zid K, Benammar-Elgaaied A, Gibriel AA, Ben Said M, Masmoudi S
Mol Genet Genomic Med 2021 Oct;9(10):e1810. Epub 2021 Sep 13 doi: 10.1002/mgg3.1810. PMID: 34514748Free PMC Article
Macielak RJ, Mattingly JK, Findlen UM, Moberly AC, Malhotra PS, Adunka OF
Int J Pediatr Otorhinolaryngol 2019 May;120:25-29. Epub 2019 Jan 25 doi: 10.1016/j.ijporl.2019.01.034. PMID: 30753978
Lu YJ, Yao J, Wei QJ, Xing GQ, Cao X
Medicine (Baltimore) 2015 Dec;94(50):e2248. doi: 10.1097/MD.0000000000002248. PMID: 26683941Free PMC Article

Diagnosis

Mu Q, Bai L, Xu B, Du H, Jiang Z, Huang S, Gao B, Wu Q, Zhao H, Dai P, Jiang Y
Prenat Diagn 2024 Aug;44(9):1043-1052. Epub 2024 Mar 15 doi: 10.1002/pd.6550. PMID: 38488843
Köse E, Kasapkara ÇS, İnci A, Yıldız Y, Sürücü Kara İ, Kahraman AB, Tümer L, Dursun A, Eminoğlu FT
Eur J Med Genet 2024 Apr;68:104927. Epub 2024 Feb 19 doi: 10.1016/j.ejmg.2024.104927. PMID: 38382588
Leal B, Lopes AC, Peixoto D, Correia L, Almiro MM, Vilar J, Azevedo ML, Bicho MA
Acta Med Port 2023 May 2;36(5):336-342. Epub 2023 Feb 17 doi: 10.20344/amp.18607. PMID: 36799720
de Muijnck C, Brink JBT, Bergen AA, Boon CJF, van Genderen MM
Surv Ophthalmol 2023 Jul-Aug;68(4):641-654. Epub 2023 Feb 9 doi: 10.1016/j.survophthal.2023.01.012. PMID: 36764396
Richard EM, Santos-Cortez RLP, Faridi R, Rehman AU, Lee K, Shahzad M, Acharya A, Khan AA, Imtiaz A, Chakchouk I, Takla C, Abbe I, Rafeeq M, Liaqat K, Chaudhry T, Bamshad MJ, Nickerson DA; University of Washington Center for Mendelian Genomics, Schrauwen I, Khan SN, Morell RJ, Zafar S, Ansar M, Ahmed ZM, Ahmad W, Riazuddin S, Friedman TB, Leal SM, Riazuddin S
Hum Mutat 2019 Jan;40(1):53-72. Epub 2018 Nov 18 doi: 10.1002/humu.23666. PMID: 30303587Free PMC Article

Therapy

Lu YJ, Yao J, Wei QJ, Xing GQ, Cao X
Medicine (Baltimore) 2015 Dec;94(50):e2248. doi: 10.1097/MD.0000000000002248. PMID: 26683941Free PMC Article

Prognosis

Kabahuma RI, Schubert WD, Labuschagne C, Yan D, Blanton SH, Pepper MS, Liu XZ
Genes (Basel) 2021 Feb 15;12(2) doi: 10.3390/genes12020274. PMID: 33671976Free PMC Article
Lu YJ, Yao J, Wei QJ, Xing GQ, Cao X
Medicine (Baltimore) 2015 Dec;94(50):e2248. doi: 10.1097/MD.0000000000002248. PMID: 26683941Free PMC Article
Marshall BA, Permutt MA, Paciorkowski AR, Hoekel J, Karzon R, Wasson J, Viehover A, White NH, Shimony JS, Manwaring L, Austin P, Hullar TE, Hershey T; Washington University Wolfram Study Group
Orphanet J Rare Dis 2013 Apr 27;8:64. doi: 10.1186/1750-1172-8-64. PMID: 23981289Free PMC Article
Bruce IA, Broomfield SJ, Henderson L, Green KM, Ramsden RT
Cochlear Implants Int 2011 Feb;12(1):60-3. doi: 10.1179/146701010X486534. PMID: 21756462
Winata S, Arhya IN, Moeljopawiro S, Hinnant JT, Liang Y, Friedman TB, Asher JH Jr
J Med Genet 1995 May;32(5):336-43. doi: 10.1136/jmg.32.5.336. PMID: 7616538Free PMC Article

Clinical prediction guides

Köse E, Kasapkara ÇS, İnci A, Yıldız Y, Sürücü Kara İ, Kahraman AB, Tümer L, Dursun A, Eminoğlu FT
Eur J Med Genet 2024 Apr;68:104927. Epub 2024 Feb 19 doi: 10.1016/j.ejmg.2024.104927. PMID: 38382588
Kabahuma RI, Schubert WD, Labuschagne C, Yan D, Pepper MS, Liu XZ
Mol Genet Genomic Med 2022 Oct;10(10):e2015. Epub 2022 Aug 27 doi: 10.1002/mgg3.2015. PMID: 36029164Free PMC Article
Kabahuma RI, Schubert WD, Labuschagne C, Yan D, Blanton SH, Pepper MS, Liu XZ
Genes (Basel) 2021 Feb 15;12(2) doi: 10.3390/genes12020274. PMID: 33671976Free PMC Article
Lu YJ, Yao J, Wei QJ, Xing GQ, Cao X
Medicine (Baltimore) 2015 Dec;94(50):e2248. doi: 10.1097/MD.0000000000002248. PMID: 26683941Free PMC Article
Bruce IA, Broomfield SJ, Henderson L, Green KM, Ramsden RT
Cochlear Implants Int 2011 Feb;12(1):60-3. doi: 10.1179/146701010X486534. PMID: 21756462

Recent systematic reviews

de Muijnck C, Brink JBT, Bergen AA, Boon CJF, van Genderen MM
Surv Ophthalmol 2023 Jul-Aug;68(4):641-654. Epub 2023 Feb 9 doi: 10.1016/j.survophthal.2023.01.012. PMID: 36764396
Acke FR, Dhooge IJ, Malfait F, De Leenheer EM
Orphanet J Rare Dis 2012 Oct 30;7:84. doi: 10.1186/1750-1172-7-84. PMID: 23110709Free PMC Article

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