GenTIGSA Gene Database on Rare Genetic Disorders

Select a RGD Category for details 

RGDs registry under the 'Ear disorders'
Disorder Name (Total=4) Aliases Sub-Category Gene Indian Reports Link
Meniere disease
Autosomal dominant
•Meniere's disease
•Ménière Disease
FAM136A family with sequence similarity 136 member A
DTNA dystrobrevin alpha
MYO7A myosin VIIA
CDH23 cadherin related 23
SHROOM2 shroom family member 2
TECTA tectorin alpha
Reports
Updated as of Sep 05, 2023
PubMed
Autosomal recessive nonsyndromic hearing loss 1A
Autosomal recessive, Digenic dominant
•GJB6-Related DFNB 1 Nonsyndromic Hearing Loss and Deafness
•deafness, digenic GJB2/GJB6, Autosomal recessive, Digenic dominant
•deafness, digenic, GJB2/GJB3, Autosomal recessive, Digenic dominant
•Connexin 26 deafness
•Deafness nonsyndromic, Connexin 26 linked
•Deafness, autosomal recessive 1A
•Nonsyndromic hearing loss and deafness, DFNB1
•DFNB 1 Nonsyndromic Hearing Loss and Deafness
Hearing disorder GJB2 gap junction protein beta 2
GJB3 gap junction protein beta 3
Reports
Updated as of Feb 26, 2025
PubMed
Autosomal recessive nonsyndromic hearing loss 12
Autosomal recessive
•DFNB12 Nonsyndromic Hearing Loss and Deafness
•DEAFNESS, AUTOSOMAL RECESSIVE 12
Hearing disorder CDH23 cadherin related 23
PKHD1L1 PKHD1 like 1
Reports
Updated as of Feb 10, 2026
PubMed
Autosomal recessive nonsyndromic hearing loss 9
Autosomal recessive
•OTOF-related hearing loss
•Auditory neuropathy, autosomal recessive, 1, temperature-sensitive
•Deafness, autosomal recessive 9
•Neurosensory nonsyndromic recessive deafness 9
Hearing disorder OTOF otoferlin
SERPINB6 serpin family B member 6
TSPEAR thrombospondin type laminin G domain and EAR repeats
CABP2 calcium binding protein 2
Reports
Updated as of Mar 30, 2026
PubMed