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Autosomal dominant non-syndromic genetic…

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Autosomal dominant non-syndromic genetic deafness

ORPHA:90635Etiological subtype

Also called Autosomal dominant non-syndromic neurosensory hearing loss · Autosomal dominant non-syndromic sensorineural deafness · Autosomal dominant non-syndromic sensorineural hearing loss · Non-syndromic genetic DFNA · Autosomal dominant isolated neurosensory deafness · Autosomal dominant isolated neurosensory hearing loss · Autosomal dominant isolated sensorineural deafness · Autosomal dominant isolated sensorineural hearing loss · Autosomal dominant non-syndromic genetic hearing loss · Autosomal dominant non-syndromic neurosensory deafness

What it is

Orphanet has not published a description for this disease yet. The identifiers, classification and cross-references below are still current.

Key facts

Age of onset
Childhood
Inheritance
Autosomal dominant
Classified as
Etiological subtype

Genes

ABCC1Disease-causing germline mutation(s)
ACTG1Disease-causing germline mutation(s)
ATP11ADisease-causing germline mutation(s)
CCDC50Disease-causing germline mutation(s)
CD164Disease-causing germline mutation(s)
CEACAM16Disease-causing germline mutation(s)
CENPPDisease-causing germline mutation(s)
COCHDisease-causing germline mutation(s)
COL11A1Disease-causing germline mutation(s)
COL11A2Disease-causing germline mutation(s)
CRYMDisease-causing germline mutation(s)
DIABLODisease-causing germline mutation(s)
DIAPH3Disease-causing germline mutation(s)
DMXL2Disease-causing germline mutation(s)
ESPNDisease-causing germline mutation(s)
EYA4Disease-causing germline mutation(s)
GJB2Disease-causing germline mutation(s)
GJB3Disease-causing germline mutation(s)
GJB6Disease-causing germline mutation(s)
GRHL2Disease-causing germline mutation(s)
GSDMEDisease-causing germline mutation(s)
HOMER2Disease-causing germline mutation(s)
KCNQ4Disease-causing germline mutation(s)
KITLGDisease-causing germline mutation(s)
MAP1BDisease-causing germline mutation(s)
MCM2Disease-causing germline mutation(s)
MIR96Disease-causing germline mutation(s)
MYH14Disease-causing germline mutation(s)
MYH9Disease-causing germline mutation(s)
MYO1CDisease-causing germline mutation(s)
MYO6Disease-causing germline mutation(s)
MYO7ADisease-causing germline mutation(s)
OSBPL2Disease-causing germline mutation(s)
P2RX2Disease-causing germline mutation(s)
PDE1CDisease-causing germline mutation(s)
PLS1Disease-causing germline mutation(s)
POU4F3Disease-causing germline mutation(s)
PTPRQDisease-causing germline mutation(s)
SIX1Disease-causing germline mutation(s)
SLC17A8Disease-causing germline mutation(s)
SLC44A4Disease-causing germline mutation(s)
SSBP1Major susceptibility factor
TBC1D24Disease-causing germline mutation(s)
TECTADisease-causing germline mutation(s)
TJP2Disease-causing germline mutation(s)
TMC1Disease-causing germline mutation(s)
TNCDisease-causing germline mutation(s)
TRRAPDisease-causing germline mutation(s)
USP48Disease-causing germline mutation(s)
WFS1Disease-causing germline mutation(s)
MYO1ACandidate gene tested

ICD-10 codes

H90.3filed under a broader ICD-10 category — shared with 9 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Hearing Disorders and Deafness

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Benefit programs to look at

Deaf Access ProgramCaliforniaDeaf and Hard of Hearing ProgramWashingtonDeaf and Hard of Hearing ServicesMinnesotaDelaware Deaf and Hard of Hearing ProgramDelawareEarly Hearing Detection and InterventionHEALTH RESOURCES AND SERVICES ADMINISTRATIONFlorida Telecommunications Relay ServicesFloridaHearing Impaired ServicesRhode IslandIllinois Relay ServiceIllinois

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

MONDO 0019587OMIM 600101OMIM 600652OMIM 600965OMIM 600994OMIM 601316OMIM 601317OMIM 601369OMIM 601412OMIM 601543OMIM 601544OMIM 601868OMIM 602459OMIM 603622OMIM 603964OMIM 604717OMIM 605192OMIM 605583OMIM 606012OMIM 606282OMIM 606346OMIM 606451OMIM 606705OMIM 607017OMIM 607197OMIM 607453OMIM 607841OMIM 608224OMIM 608372OMIM 608394OMIM 608641OMIM 608645OMIM 608652OMIM 609129OMIM 609965OMIM 612431OMIM 612642OMIM 612643OMIM 612644OMIM 613074OMIM 613558OMIM 614152OMIM 614211OMIM 614614OMIM 615629OMIM 615649OMIM 615654OMIM 616044OMIM 616340OMIM 616357OMIM 616697OMIM 616707OMIM 616968OMIM 616969OMIM 617605OMIM 617606OMIM 617663OMIM 618094OMIM 618140OMIM 618410OMIM 618533OMIM 618778OMIM 618787OMIM 618915OMIM 619081OMIM 619086OMIM 619274OMIM 620227OMIM 621556UMLS C5779548

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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