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Prelingual sensorineural hearing impairment

MedGen UID:
867432
Concept ID:
C4021806
Disease or Syndrome
Synonyms: Deafness, sensorineural, prelingual; Prelingual sensorineural deafness
 
HPO: HP:0000399

Definition

A form of sensorineural deafness with either congenital onset or infantile onset, i.e., before the acquisition of speech. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrelingual sensorineural hearing impairment

Conditions with this feature

Autosomal recessive nonsyndromic hearing loss 12
MedGen UID:
330455
Concept ID:
C1832394
Disease or Syndrome
A genetic condition inherited in an autosomal recessive caused by mutation(s) in the CDH23 gene, encoding cadherin-23, characterized by progressive sensorineural hearing loss. Mutation(s) in the CDH23 gene may also cause Usher syndrome 1D.
Autosomal recessive nonsyndromic hearing loss 39
MedGen UID:
374909
Concept ID:
C1842342
Disease or Syndrome
An autosomal recessive disorder caused by mutations in the HGF gene, encoding hepatocyte growth factor. It is characterized by profound deafness.
Autosomal recessive nonsyndromic hearing loss 40
MedGen UID:
334053
Concept ID:
C1842345
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 22q11.21-q12.1.
Autosomal recessive nonsyndromic hearing loss 38
MedGen UID:
330838
Concept ID:
C1842381
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 6q26-q27.
Autosomal recessive nonsyndromic hearing loss 44
MedGen UID:
341854
Concept ID:
C1857809
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the ADCY1 gene.
Autosomal recessive nonsyndromic hearing loss 49
MedGen UID:
346670
Concept ID:
C1857811
Disease or Syndrome
Autosomal recessive deafness-49 (DFNB49) is characterized by prelingual profound sensorineural hearing loss at all frequencies (Riazuddin et al., 2006 and Chishti et al., 2008).
Autosomal recessive nonsyndromic hearing loss 62
MedGen UID:
387916
Concept ID:
C1857820
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 12p13.2-p11.23.
Autosomal recessive nonsyndromic hearing loss 15
MedGen UID:
355626
Concept ID:
C1866094
Disease or Syndrome
This form of autosomal recessive deafness is sensorineural and nonsyndromic, and shows prelingual onset (summary by Charizopoulou et al., 2011).
Autosomal recessive nonsyndromic hearing loss 71
MedGen UID:
411609
Concept ID:
C2748554
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 8p22-p21.3.
Autosomal recessive nonsyndromic hearing loss 85
MedGen UID:
463629
Concept ID:
C3160740
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 17p12-q11.2.
Autosomal recessive nonsyndromic hearing loss 45
MedGen UID:
854732
Concept ID:
C3888030
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 1q43-q44.
Autosomal recessive nonsyndromic hearing loss 83
MedGen UID:
854856
Concept ID:
C3888310
Disease or Syndrome
An autosomal recessive nonsyndromic deafness that has material basis in variation in the chromosome region 9p23-p21.2.
Autosomal recessive nonsyndromic hearing loss 104
MedGen UID:
899775
Concept ID:
C4225298
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the RIPOR2 gene.
Usher syndrome, type 1M
MedGen UID:
1684669
Concept ID:
C5231434
Disease or Syndrome
Usher syndrome type 1M (USH1M) is characterized by prelingual sensorineural hearing loss, vestibular dysfunction, and retinitis pigmentosa (Ahmed et al., 2018). For a general phenotypic description and discussion of genetic heterogeneity of Usher syndrome, see USH1 (276900).

Professional guidelines

PubMed

Han B, Cheng J, Yang SZ, Cao JY, Shen WD, Ji F, Kang DY, Zhang X, Dai P, Yuan HJ
Chin Med J (Engl) 2009 Apr 5;122(7):830-3. PMID: 19493398
Duncan RD, Prucka S, Wiatrak BJ, Smith RJ, Robin NH
Arch Otolaryngol Head Neck Surg 2007 Mar;133(3):231-6. doi: 10.1001/archotol.133.3.231. PMID: 17372079

Recent clinical studies

Etiology

Jerger S, Tye-Murray N, Damian MF, Abdi H
Ear Hear 2016 Nov/Dec;37(6):623-633. doi: 10.1097/AUD.0000000000000334. PMID: 27438867Free PMC Article
Duncan RD, Prucka S, Wiatrak BJ, Smith RJ, Robin NH
Arch Otolaryngol Head Neck Surg 2007 Mar;133(3):231-6. doi: 10.1001/archotol.133.3.231. PMID: 17372079
Stinckens C, Kremer H, van Wijk E, Hoefsloot LH, Huygen PL, Standaert L, Fryns JP, Cremers CW
Ann Otol Rhinol Laryngol 2004 Jul;113(7):587-93. doi: 10.1177/000348940411300714. PMID: 15274422
Haggard MP, McCormick B, Gannon MM, Spencer H
Clin Otolaryngol Allied Sci 1992 Feb;17(1):34-43. doi: 10.1111/j.1365-2273.1992.tb00985.x. PMID: 1555316

Diagnosis

Jerger S, Tye-Murray N, Damian MF, Abdi H
Ear Hear 2016 Nov/Dec;37(6):623-633. doi: 10.1097/AUD.0000000000000334. PMID: 27438867Free PMC Article
Duncan RD, Prucka S, Wiatrak BJ, Smith RJ, Robin NH
Arch Otolaryngol Head Neck Surg 2007 Mar;133(3):231-6. doi: 10.1001/archotol.133.3.231. PMID: 17372079
Stinckens C, Kremer H, van Wijk E, Hoefsloot LH, Huygen PL, Standaert L, Fryns JP, Cremers CW
Ann Otol Rhinol Laryngol 2004 Jul;113(7):587-93. doi: 10.1177/000348940411300714. PMID: 15274422
Haggard MP, McCormick B, Gannon MM, Spencer H
Clin Otolaryngol Allied Sci 1992 Feb;17(1):34-43. doi: 10.1111/j.1365-2273.1992.tb00985.x. PMID: 1555316

Prognosis

Jerger S, Tye-Murray N, Damian MF, Abdi H
Ear Hear 2016 Nov/Dec;37(6):623-633. doi: 10.1097/AUD.0000000000000334. PMID: 27438867Free PMC Article
Stinckens C, Kremer H, van Wijk E, Hoefsloot LH, Huygen PL, Standaert L, Fryns JP, Cremers CW
Ann Otol Rhinol Laryngol 2004 Jul;113(7):587-93. doi: 10.1177/000348940411300714. PMID: 15274422
Haggard MP, McCormick B, Gannon MM, Spencer H
Clin Otolaryngol Allied Sci 1992 Feb;17(1):34-43. doi: 10.1111/j.1365-2273.1992.tb00985.x. PMID: 1555316

Clinical prediction guides

Jerger S, Tye-Murray N, Damian MF, Abdi H
Ear Hear 2016 Nov/Dec;37(6):623-633. doi: 10.1097/AUD.0000000000000334. PMID: 27438867Free PMC Article
Han B, Cheng J, Yang SZ, Cao JY, Shen WD, Ji F, Kang DY, Zhang X, Dai P, Yuan HJ
Chin Med J (Engl) 2009 Apr 5;122(7):830-3. PMID: 19493398
Wajid M, Abbasi AA, Ansar M, Pham TL, Yan K, Haque S, Ahmad W, Leal SM
Eur J Hum Genet 2003 Oct;11(10):812-5. doi: 10.1038/sj.ejhg.5201041. PMID: 14512973Free PMC Article
Ansar M, Ramzan M, Pham TL, Yan K, Jamal SM, Haque S, Ahmad W, Leal SM
Hum Hered 2003;55(1):71-4. doi: 10.1159/000071813. PMID: 12890929Free PMC Article
Haggard MP, McCormick B, Gannon MM, Spencer H
Clin Otolaryngol Allied Sci 1992 Feb;17(1):34-43. doi: 10.1111/j.1365-2273.1992.tb00985.x. PMID: 1555316

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