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Monogenic hearing loss v6.20 CD164 Ida Ertmanska Classified gene: CD164 as Amber List (moderate evidence)
Monogenic hearing loss v6.20 CD164 Ida Ertmanska Added comment: Comment on list classification: There are now 3 unrelated families various ancestries with the same heterozygous CD164 variant (p.Arg192Ter), which segregates in a dominant fashion with bilateral progressive hearing loss. Hence, this gene can be promoted to Green for Monogenic hearing loss.
Monogenic hearing loss v6.20 CD164 Ida Ertmanska Gene: cd164 has been classified as Amber List (Moderate Evidence).
Monogenic hearing loss v6.19 CD164 Ida Ertmanska Phenotypes for gene: CD164 were changed from ?Deafness, autosomal dominant 66 616969 to ?Deafness, autosomal dominant 66, OMIM:616969; autosomal dominant nonsyndromic hearing loss 66, MONDO:0014854
Monogenic hearing loss v6.18 CD164 Ida Ertmanska Publications for gene: CD164 were set to 26197441
Monogenic hearing loss v6.17 CD164 Ida Ertmanska Mode of inheritance for gene: CD164 was changed from MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Monogenic hearing loss v6.16 CD164 Ida Ertmanska Tag Q2_26_promote_green tag was added to gene: CD164.
Monogenic hearing loss v6.16 CD164 Ida Ertmanska changed review comment from: PMID: 35254497 Oziębło et al., 2022
Family 1 - large Polish pedigree with AD progressive hearing loss; CD164: c.574C>T, p.Arg192Ter segregated in affected individuals
Family 2 - Korean family, 25 yo proband with bilateral progressive hearing loss onset around 22 years old; also het for CD164 p.Arg192Ter
Same variant as reported in PMID 26197441 Nyegaard et al (2015) in a Danish family with hearing loss - recurrent hot spot mutation?
CD164: c.574C>T, p.Arg192Ter - only 2 hets reported in gnomAD v4.1.1, MAF = 0.000001696
Age of onset of hearing loss for all 3 families ranged from newborn stage to adulthood.

PMID: 36454368 Moresco et al., 2023
Family 7 - AD nonsyndromic hearing loss, affected individuals harboured het CD164: c.574C>T, p.Arg192Ter variant - segregated with disease. Seq method: Trio WES.; to: PMID: 35254497 Oziębło et al., 2022
Family 1 - large Polish pedigree with AD progressive hearing loss; CD164: c.574C>T, p.Arg192Ter segregated in affected individuals
Family 2 - Korean family, 25 yo proband with bilateral progressive hearing loss onset around 22 years old; also het for CD164 p.Arg192Ter
Same variant as reported in PMID 26197441 Nyegaard et al (2015) in a Danish family with hearing loss - recurrent hot spot mutation?
CD164: c.574C>T, p.Arg192Ter - only 2 hets reported in gnomAD v4.1.1, MAF = 0.000001696
Age of onset of hearing loss for all 3 families ranged from newborn stage to adulthood.

PMID: 36454368 Moresco et al., 2023
Family 7 - AD nonsyndromic hearing loss, affected individuals harboured het CD164: c.574C>T, p.Arg192Ter variant - segregated with disease. Seq method: Trio WES.

The assocation between CD164 and autosomal dominant nonsyndromic hearing loss has been classified as Moderate by ClinGen Hearing Loss GCEP in 2024.
Monogenic hearing loss v6.16 CD164 Ida Ertmanska reviewed gene: CD164: Rating: GREEN; Mode of pathogenicity: None; Publications: 26197441, 35254497, 36454368; Phenotypes: ?Deafness, autosomal dominant 66, OMIM:616969; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Monogenic hearing loss v1.79 CD164 Eleanor Williams Mode of inheritance for gene: CD164 was changed from Unknown to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Monogenic hearing loss v1.78 CD164 Eleanor Williams Phenotypes for gene: CD164 were changed from to ?Deafness, autosomal dominant 66 616969
Monogenic hearing loss v1.77 CD164 Eleanor Williams Publications for gene: CD164 were set to
Monogenic hearing loss v1.59 CD164 Emma Ashton reviewed gene: CD164: Rating: RED; Mode of pathogenicity: ; Publications: 26197441; Phenotypes: ; Mode of inheritance:
Monogenic hearing loss v1.57 CD164 Eleanor Williams gene: CD164 was added
gene: CD164 was added to Hearing loss. Sources: Expert list
Mode of inheritance for gene: CD164 was set to Unknown
Added comment: Added at suggestion of Emma Ashton, GOSH
Sources: Expert list