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Deafness, neural, congenital moderate

MedGen UID:
347425
•Concept ID:
C1857337
•
Disease or Syndrome
Synonym: Deafness, Neural, Congenital Moderate
 
Monarch Initiative: MONDO:0009087
OMIM®: 221500

Clinical features

From HPO
Sensorineural hearing loss disorder
MedGen UID:
9164
•Concept ID:
C0018784
•
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
See: Feature record | Search on this feature

Recent clinical studies

Etiology

Mild-moderate congenital hearing loss: secular trends in outcomes across four systems of detection.
Carew P, Mensah FK, Rance G, Flynn T, Poulakis Z, Wake M
Child Care Health Dev 2018 Jan;44(1):71-82. Epub 2017 Jun 14 doi: 10.1111/cch.12477. PMID: 28612343
Identification of hearing loss in newborns by transient otoacoustic emissions.
Jakubíková J, Kabátová Z, Závodná M
Int J Pediatr Otorhinolaryngol 2003 Jan;67(1):15-8. doi: 10.1016/s0165-5876(02)00285-9. PMID: 12560144

Diagnosis

Cochlear Synaptopathy due to Mutations in OTOF Gene May Result in Stable Mild Hearing Loss and Severe Impairment of Speech Perception.
Santarelli R, Scimemi P, Costantini M, Domínguez-Ruiz M, Rodríguez-Ballesteros M, Del Castillo I
Ear Hear 2021 Nov-Dec 01;42(6):1627-1639. doi: 10.1097/AUD.0000000000001052. PMID: 33908410Free PMC Article
Comorbidity and cognitive overlap between developmental dyslexia and congenital amusia.
Couvignou M, Peretz I, Ramus F
Cogn Neuropsychol 2019 Feb-Mar;36(1-2):1-17. Epub 2019 Feb 20 doi: 10.1080/02643294.2019.1578205. PMID: 30785364
Mild-moderate congenital hearing loss: secular trends in outcomes across four systems of detection.
Carew P, Mensah FK, Rance G, Flynn T, Poulakis Z, Wake M
Child Care Health Dev 2018 Jan;44(1):71-82. Epub 2017 Jun 14 doi: 10.1111/cch.12477. PMID: 28612343
The pattern of hearing impairment among schoolboys in an Institute for deaf subjects.
Abolfotouh MA, Al-Ghamdi SA
Saudi Med J 2000 Sep;21(9):873-6. PMID: 11376367

Therapy

Mild-moderate congenital hearing loss: secular trends in outcomes across four systems of detection.
Carew P, Mensah FK, Rance G, Flynn T, Poulakis Z, Wake M
Child Care Health Dev 2018 Jan;44(1):71-82. Epub 2017 Jun 14 doi: 10.1111/cch.12477. PMID: 28612343

Prognosis

Mild-moderate congenital hearing loss: secular trends in outcomes across four systems of detection.
Carew P, Mensah FK, Rance G, Flynn T, Poulakis Z, Wake M
Child Care Health Dev 2018 Jan;44(1):71-82. Epub 2017 Jun 14 doi: 10.1111/cch.12477. PMID: 28612343
The pattern of hearing impairment among schoolboys in an Institute for deaf subjects.
Abolfotouh MA, Al-Ghamdi SA
Saudi Med J 2000 Sep;21(9):873-6. PMID: 11376367

Clinical prediction guides

Mild-moderate congenital hearing loss: secular trends in outcomes across four systems of detection.
Carew P, Mensah FK, Rance G, Flynn T, Poulakis Z, Wake M
Child Care Health Dev 2018 Jan;44(1):71-82. Epub 2017 Jun 14 doi: 10.1111/cch.12477. PMID: 28612343

Supplemental Content

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