Overview
Cochlear nerve deficiency (CND), also called cochlear nerve aplasia or cochlear nerve hypoplasia, is a condition where the cochlear nerve — the nerve that carries sound signals from the inner ear to the brain — is either missing, very small, or underdeveloped. This nerve is essential for hearing, so when it is absent or too thin to work properly, it causes significant hearing loss, usually from birth. The hearing loss is typically severe to profound, meaning a child may hear very little or nothing at all in the affected ear or ears. CND can affect one ear (unilateral) or both ears (bilateral). The condition is usually discovered during newborn hearing screening or when parents notice their child is not responding to sounds. Doctors confirm the diagnosis using special MRI scans that can show whether the cochlear nerve is present and how large it is. CND is one of the more common causes of permanent hearing loss found on inner ear imaging in children. Treatment focuses on helping the child communicate and develop language. Hearing aids are often tried first, but because the nerve itself is the problem, they may not help much. Cochlear implants are a common option, though their success depends on how much nerve tissue is present. Auditory brainstem implants (ABIs) may be considered in some cases. Early speech therapy and educational support are very important for language development.
Key symptoms:
Severe to profound hearing loss from birthLittle or no response to sounds or voicesDelayed speech and language developmentDifficulty understanding spoken wordsHearing loss in one or both earsFailure of newborn hearing screeningNo improvement with hearing aids in some cases
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Cochlear nerve deficiency.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Cochlear nerve deficiency.
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Caregiver Resources
NORD Caregiver Resources
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Family & Caregiver Grants
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Social Security Disability
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Questions for your doctor
Bring these to your next appointment
- Q1.Is the cochlear nerve completely absent or just smaller than normal, and what does that mean for my child's treatment options?,Would my child benefit from a cochlear implant, and how do we predict how well it might work?,Should we consider genetic testing to find out why this happened and whether it could affect other family members?,What communication approach — spoken language, sign language, or both — is best for my child right now?,What early intervention services and therapies should we start as soon as possible?,Are there any other health conditions we should watch for that are sometimes linked to cochlear nerve deficiency?,What does the long-term educational and communication outlook look like for a child with this level of cochlear nerve involvement?
Common questions about Cochlear nerve deficiency
What is Cochlear nerve deficiency?
Cochlear nerve deficiency (CND), also called cochlear nerve aplasia or cochlear nerve hypoplasia, is a condition where the cochlear nerve — the nerve that carries sound signals from the inner ear to the brain — is either missing, very small, or underdeveloped. This nerve is essential for hearing, so when it is absent or too thin to work properly, it causes significant hearing loss, usually from birth. The hearing loss is typically severe to profound, meaning a child may hear very little or nothing at all in the affected ear or ears. CND can affect one ear (unilateral) or both ears (bilateral).
At what age does Cochlear nerve deficiency typically begin?
Typical onset of Cochlear nerve deficiency is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Cochlear nerve deficiency?
2 specialists and care centers treating Cochlear nerve deficiency are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.