General Information of Disease (ID: DIS3U2K4)

Disease Name Postlingual non-syndromic genetic hearing loss
Synonyms postlingual non-syndromic genetic deafness; isolated postlingual genetic deafness
Definition
Postlingual non-syndromic genetic deafness is a rare, genetically highly heterogeneous otorhinolaryngologic disease, resulting from inner and/or middle ear or hearing nerve anomalies, typically characterized by progressive, bilateral, moderate to profound hearing loss (mean sensorineural hearing impairment equal to 40 dB or more for 500-, 1,000-, and 2,000-Hz frequency tones in the better ear) which occurs after the onset of speech development and is not associated with visible external ear abnormalities or any other medical problems. Language development is not initially significantly delayed.
Disease Hierarchy
DISZX61P: Nonsyndromic genetic hearing loss
DIS3U2K4: Postlingual non-syndromic genetic hearing loss
Disease Identifiers
MONDO ID
MONDO_0016298
UMLS CUI
C4706678
MedGen ID
1641874
Orphanet ID
216452
SNOMED CT ID
764097002