Rare diseases · Sign or symptom
Bilateral sensorineural hearing impairment
HP:0008619
What it means
A form of sensorineural hearing impairment that affects both ears.
Rare diseases that can present with this32
Very common80–99%
8- Bartter syndrome type 4
- Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome
- Growth delay due to insulin-like growth factor type 1 deficiency
- Jervell and Lange-Nielsen syndrome
- N syndrome
- Split-foot malformation-mesoaxial polydactyly syndrome
- Superficial siderosis
- Woodhouse-Sakati syndrome
Common30–79%
11- Acitretin/etretinate embryopathy
- Alport syndrome
- Isotretinoin-like syndrome
- Juvenile-onset diabetes mellitus-central and peripheral neurodegeneration syndrome
- Microcephaly-facial dysmorphism-ocular anomalies-multiple congenital anomalies syndrome
- MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome
- Myoclonus-cerebellar ataxia-deafness syndrome
- Non-progressive predominantly posterior cavitating leukoencephalopathy with peripheral neuropathy
- Ramos-Arroyo syndrome
- Sensory ataxic neuropathy-dysarthria-ophthalmoparesis syndrome
- Xq21microdeletion syndrome
Sometimes5–29%
11- Autosomal recessive spastic ataxia with leukoencephalopathy
- Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation
- De Barsy syndrome
- Distal duplication 17q syndrome
- Frontotemporal dementia with motor neuron disease
- Medulloblastoma
- Metachromatic leukodystrophy, adult form
- Metachromatic leukodystrophy, juvenile form
and 3 more in this range
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 nerve deafness · Bilateral sensorineural deafness · Bilateral sensorineural hearing loss · Hearing loss, sensorineural, bilateral
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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.