Rare diseases · Sign or symptom
Congenital sensorineural hearing impairment
HP:0008527
What it means
A type of hearing impairment caused by an abnormal functionality of the cochlear nerve with congenital onset.
Rare diseases that can present with this16
Very common80–99%
7- Growth delay due to insulin-like growth factor type 1 deficiency
- Hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome
- Knuckle pads-leukonychia-sensorineural deafness-palmoplantar hyperkeratosis syndrome
- Kyphoscoliotic Ehlers-Danlos syndrome due to FKBP22 deficiency
- Lethal ataxia with deafness and optic atrophy
- Waardenburg syndrome type 1
- Wildervanck syndrome
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Bilateral congenital sensorineural deafness · Congenital neurosensory deafness · Congenital perceptive deafness · Congenital sensorineural deafness · Congenital sensorineural hearing loss · Hearing loss, congenital sensorineural
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.