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Congenital conductive hearing impairment

MedGen UID:
867179
•Concept ID:
C4021537
•
Congenital Abnormality
Synonyms: Congenital conductive deafness; Congenital conductive hearing loss
SNOMED CT: Congenital conductive hearing loss (737344003)
 
HPO: HP:0008591

Definition

A type of conductive deafness with congenital onset. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital conductive hearing impairment

Conditions with this feature

Ossicular malformations, familial
MedGen UID:
371652
•Concept ID:
C1833790
•
Disease or Syndrome
See: Condition Record
Thickened earlobes-conductive deafness syndrome
MedGen UID:
343676
•Concept ID:
C1851896
•
Finding
Thickened earlobes-conductive deafness syndrome is characterized by microtia with thickened ear lobes, micrognathia and conductive hearing loss due to congenital ossicular anomalies. It has been described in two families. The mode of inheritance is autosomal dominant.
See: Condition Record
Ossicular malformations, familial
Thickened earlobes-conductive deafness syndrome

Professional guidelines

PubMed

Congenital cholesteatoma clinical and surgical management.
Wei B, Zhou P, Zheng Y, Zhao Y, Li T, Zheng Y
Int J Pediatr Otorhinolaryngol 2023 Jan;164:111401. Epub 2022 Dec 8 doi: 10.1016/j.ijporl.2022.111401. PMID: 36512880
Middle ear effusion and newborn hearing screening.
Karawani H, Matanis W, Na'ara S, Gordin A
Eur Arch Otorhinolaryngol 2023 Feb;280(2):643-649. Epub 2022 Jul 15 doi: 10.1007/s00405-022-07524-2. PMID: 35838783
Universal newborn hearing screening.
Wrightson AS
Am Fam Physician 2007 May 1;75(9):1349-52. PMID: 17508530

Recent clinical studies

Etiology

Bone-anchored hearing aid system application for unilateral congenital conductive hearing impairment: audiometric results.
Kunst SJ, Leijendeckers JM, Mylanus EA, Hol MK, Snik AF, Cremers CW
Otol Neurotol 2008 Jan;29(1):2-7. doi: 10.1097/mao.0b013e31815ee29a. PMID: 18199951

Diagnosis

A family with autosomal dominant inherited dysmorphic small auricles, lip pits, and congenital conductive hearing impairment.
Koch SM, Kumar S, Cremers CW
Arch Otolaryngol Head Neck Surg 2000 May;126(5):639-44. doi: 10.1001/archotol.126.5.639. PMID: 10807332

Clinical prediction guides

Asymmetry in Cortical Thickness of the Heschl's Gyrus in Unilateral Ear Canal Atresia.
Siegbahn M, Jörgens D, Asp F, Hultcrantz M, Moreno R, Engmér Berglin C
Otol Neurotol 2024 Apr 1;45(4):e342-e350. Epub 2024 Feb 15 doi: 10.1097/MAO.0000000000004137. PMID: 38361347
Bone-anchored hearing aid system application for unilateral congenital conductive hearing impairment: audiometric results.
Kunst SJ, Leijendeckers JM, Mylanus EA, Hol MK, Snik AF, Cremers CW
Otol Neurotol 2008 Jan;29(1):2-7. doi: 10.1097/mao.0b013e31815ee29a. PMID: 18199951

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