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Hearing loss, Y-linked 1(DFNY1)

MedGen UID:
854748
Concept ID:
C3888076
Disease or Syndrome
Synonyms: Deafness, Y-linked 1; DFNY1
 
Monarch Initiative: MONDO:0010764
OMIM®: 400043

Definition

Y-linked deafness-1 (DFNY1) is characterized by male-limited postlingual progressive sensorineural hearing loss of variable severity, with onset in the first to third decades of life (Wang et al., 2009). Genetic Heterogeneity of Y-Linked Deafness DFNY2 (400047) is caused by mutation in the TBL1Y gene (400033). [from OMIM]

Clinical features

From HPO
Sensorineural hearing impairment
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Tinnitus
MedGen UID:
52760
Concept ID:
C0040264
Disease or Syndrome
Tinnitus is an auditory perception that can be described as the experience of sound, in the ear or in the head, in the absence of external acoustic stimulation.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

Fu S, Yan J, Wang X, Dong J, Chen P, Wang C, Chen G
Int J Pediatr Otorhinolaryngol 2011 Feb;75(2):202-6. Epub 2010 Dec 3 doi: 10.1016/j.ijporl.2010.10.035. PMID: 21130505

Prognosis

Fu S, Yan J, Wang X, Dong J, Chen P, Wang C, Chen G
Int J Pediatr Otorhinolaryngol 2011 Feb;75(2):202-6. Epub 2010 Dec 3 doi: 10.1016/j.ijporl.2010.10.035. PMID: 21130505

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