Bilateral Cochlear Aplasia, Bilateral Cochlear Nerve Deficiency, Bilateral Cochlear Ossification Secondary to Meningitis
Conditions
Brief summary
Current treatment options for bilateral profoundly deaf children, diagnosed with inner ear anatomical abnormalities are limited, and in the case of absent cochleas, non-existent. An auditory brainstem implant (ABI) places an electrode close to the auditory nucleus in the brainstem. Children aged 2 - 5 who are not candidates for a cochlear implant, or who did not demonstrate benefit from a cochlear implant, will be implanted with an ABI and followed for 1 year for safety and a total of 3 years for preliminary efficacy. This is a feasibility study to determine the safety of the ABI.
Interventions
Sponsors
House Clinic, Inc.
Children's Hospital Los Angeles
House Research Institute
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
2 Years to 5 Years
Healthy volunteers
No
Inclusion criteria
* Bilateral profound deafness due to cochlear aplasia, cochlear nerve deficiency, or ossification secondary to meningitis * If previously received a cochlear implant, must demonstrate lack of benefit from that device
Exclusion criteria
* Medical contraindication to craniotomy/intracranial surgery * Severe cognitive or developmental delays
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Number of Serious Adverse Events | 12-month post-activation | Count of the number of expected serious adverse events must not exceed that known to occur in pediatric retrosigmoid craniotomy and Count of the number of unexpected serious adverse events must not exceed 2 in the first 5 children. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Access to sound at a level (dB) and within the frequency range (500-4000 Hz), known to be associated with speech | 3 years post-activation | ability to detect sounds |
Countries
United States
Outcome results
None listed