Rare diseases · Sign or symptom
Progressive sensorineural hearing impairment
HP:0000408
What it means
A progressive form of sensorineural hearing impairment.
Rare diseases that can present with this14
Very common80–99%
10- Alström syndrome
- Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome
- Cochleosaccular degeneration-cataract syndrome
- Cockayne syndrome
- Deafness-hypogonadism syndrome
- DIAPH1-related sensorineural hearing loss-thrombocytopenia syndrome
- Flynn-Aird syndrome
- Muckle-Wells syndrome
- Otodental syndrome
- Progeria-short stature-pigmented nevi syndrome
Common30–79%
2The 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 progressive sensorineural hearing loss · Hearing loss, progressive sensorineural · Hearing loss, sensorineural, bilateral, progressive · Hearing loss, sensorineural, progressive · Progressive bilateral sensorineural hearing loss · Sensorineural hearing loss, progressive
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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.