Clinical trial NCT05775367: Cochlear Implants in Young Children With SSD
Official title: Cochlear Implantation in Infants and Toddlers With Single-Sided Deafness
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Single Sided Deafness
Unilateral Deafness
Hearing Loss, Unilateral
Hearing Loss
Congenital Hearing Loss
Deafness One Ear
Keywords: cochlear implant
uhl
ssd
children
hearing loss
deafness
Lead sponsor: University of North Carolina, Chapel Hill
Sponsor class: OTHER
Collaborators: name: Med-El Corporation
First posted: 2023-03-20
Enrollment: 60
Sex: ALL
Minimum age (years): 7
Maximum age (years): 71
Standard ages: CHILD
Accepts healthy volunteers: yes
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Intervention model description: The study group will receive a cochlear implant under the age of three and be tested at the age of five. Two control groups, both five years old, will be tested as well. The control groups will consist of children with typical hearing in both ears and children with single-sided deafness who have not received a cochlear implant.
Primary purpose: OTHER

Brief summary: The goal of this clinical trial is to investigate the safety and effectiveness of cochlear implantation in infants and toddlers with single-sided deafness.

The main questions it aims to answer are:

* Are cochlear implants an effective treatment of single-sided deafness in infants and toddlers?
* Are cochlear implants a safe treatment for single-sided deafness in infants and toddlers?

Participants will receive a cochlear implant and be followed until they are five years old. During those five years, the investigators will program the device and monitor auditory development.

Children will be asked to:

* Undergo cochlear implantation
* Wear their cochlear implant processor whenever they are awake.
* Participate in traditional hearing tests
* Participate in traditional hearing testing
* Participate in localization testing
* Participate in hearing in noise testing
* Participate in word recognition testing
* Participate in speech, language, and educational evaluations

The researchers will compare results to children with typical hearing in both ears and children with single-sided deafness who have not received an implant to observe any differences between the groups.

Detailed description: Cochlear implantation is an FDA-approved option for children with single-sided deafness (SSD) who are over the age of five years. It has been well established that early implantation is advantageous for children with bilateral hearing loss as it takes advantage of the narrow window of neural plasticity. Research has yet to show the ideal age for implantation in children with SSD but considering the known impacts of age at implantation and duration of deafness on cochlear implant (CI) outcomes, five years is likely a late age for implantation in a child with congenital SSD. The purpose of this prospective clinical trial is to evaluate the safety and effectiveness of cochlear implantation in infants and toddlers with SSD.

Twenty (20) infants and Toddlers with SSD who are under the age of three will receive a cochlear implant. They will be followed until they reach the age of five. A group of typically hearing five-year-olds (n=20) and a group of five-year-olds with congenital or early onset SSD who have not received a cochlear implant (n=20) will also be recruited. All three groups of five-year-olds will be tested on measures of hearing in quiet, localizing, and hearing in spatially separated noise for comparison. Scores from the study group and the SSD control group will be compared to evaluate effectiveness.

The study will also explore the potential effects of SSD and early implantation on language, sensory processing, executive function, fatigue, and cognition.

Eligibility criteria: To be eligible to participate in this trial as a study subject, an individual must meet all of the following criteria:

1. Provision of signed and dated parental permission form
2. Unilateral severe-to-profound sensorineural hearing loss, congenital or acquired prior to the age of 2 years, defined as:

   1. Unaided residual hearing thresholds that yield a 3 frequency pure tone average (PTA; average at 500, 1000, and 2000 Hz) of ≥70 dB hearing loss (HL) in the ear to be implanted
   2. Hearing thresholds in the contralateral ear of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz).

   i. Thresholds should be measured using inserts wherever possible, or in the sound field to record the better-hearing ear alone. Required thresholds will include 250, 500, 1000, 2000, and 4000 Hz for air conduction. All other octave frequencies should be attempted. Bone conduction thresholds should be obtained at 500, 1000, 2000, and 4000 Hz. Masking should be attempted where appropriate. As participants are very young and masking is difficult for this population, suspected shadow audiograms will be sufficient if objective testing has confirmed a severe-to-profound unilateral hearing loss. Testing should confirm a severe-to-profound hearing loss, defined as a 3-frequency PTA (500, 1000, and 2000) \>70 dB HL in one ear and ≤20 dB HL in the contralateral ear. The Primary Investigator and Lead Diagnostic Audiologist must agree on this diagnosis for enrollment.
3. Insufficient functional access to sound with appropriately fit amplification and aural habilitation (based on best practices and standard of care) defined as:

   1. PEACH + parent questionnaire scores below the expected value for children who lack the requisite language to complete open-set word recognition testing or
   2. Recorded Multisyllabic Lexical Neighborhood Test (MLNT) scores ≤ 30% in the ear to be implanted for children with the requisite language to complete open-set word recognition testing.
4. Between 7 months and 2 years, 11 months of age at implantation
5. Normal cochlear nerve as evaluated by imaging, required imaging by MRI brain/Internal auditory canal (IAC) without contrast with heavily weighted T2 images. CT optional at the physician's discretion.
6. No malformation of the cochlea
7. No evidence of progressive hearing loss of the contralateral ear
8. Willing to/has undergone hearing aid trial as warranted based on achieving an aided speech intelligibility index (SII) of \> .65 when fitted via Desired Sensation Level (DSL) methods.
9. Realistic parental expectations: a verbal acknowledgment of the potential benefits and risks, and postoperative variation in performance. For instance, cochlear implantation will not restore normal hearing
10. Willing to obtain recommended meningitis vaccinations per Centers for Disease Control (CDC) recommendations
11. Development and cognition within the normal range as measured by the Developmental Assessment of Young Children (DAYC-2).
12. Parental commitment to study parameters including being able and willing to participate in the evaluation schedule, involvement in prescribed therapy, and travel to the investigational site and study-related activities. Parents must be willing to encourage wearing implant during waking hours.

To participate as an SSD control subject, an individual must meet all the following criteria:

1. Provision of signed and dated parental permission form
2. Unilateral severe-to-profound hearing loss - congenital or acquired prior to 2 years of age.

   1. Unaided residual hearing thresholds that yield a 3 frequency PTA (500, 1000, and 2000 Hz) of ≥70 dB HL in the affected ear.
   2. Hearing thresholds in the contralateral ear of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz)
   3. Unable to use or benefit from traditional amplification
3. Five years of age at the time of testing
4. Development and cognition within the normal range as measured by the Early Stanford Binet 2 (SB2).

To participate as a TH control subject, an individual must meet all the following criteria:

1. Provision of signed and dated parental permission form
2. Bilateral hearing thresholds that yield a PTA of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz).
3. Five years of age at the time of testing
4. Development and cognition within the normal range as measured by the Early SB2.

An individual who meets any of the following criteria will be excluded from participation in this study as a study subject:

1. English is not primary language of the home

   1. Speech perception materials are presented in English
   2. Parental questionnaires are administered in English
2. Conductive hearing loss in either ear
3. Cochlear nerve deficiency
4. Ossification/Fibrosis of the cochlea precluding implantation
5. Inability to participate in follow-up procedures (i.e., unwillingness, geographic location)
6. History of refractory chronic otitis media or condition that contraindicates anesthesia

An individual who meets any of the following criteria will be excluded from participation in this study as a control subject:

1. English is not primary language of the home

   1. Speech perception materials are presented in English
   2. Parental questionnaires are administered in English
2. Inability to participate in testing (i.e., unwillingness)

Primary outcomes:
measure: Mean Difference in the Signal-to-Noise Ratio 50 (SNR-50) between the Study Group and SSD Control Group as Measured with the BKB-SIN at 5 Years of Age
timeFrame: 5 years of age
measure: Mean Difference in Localization Error Between the Study Group and the SSD Control group Measured at 5 Years of Age
timeFrame: 5 years of age
Secondary outcomes:
measure: Proportion of Procedural Related Adverse Events 6 months after Surgery
timeFrame: 6 months after surgery
measure: Proportion of Device-Related Adverse Events 6 Months after Surgery
timeFrame: 6 months after surgery
Other outcomes:
Locations:
city: Durham
country: United States
facility: The Children's Cochlear Implant Center at UNC
state: North Carolina
Study officials:
affiliation: University of North Carolina, Chapel Hill
name: Lisa Park, AuD
role: PRINCIPAL_INVESTIGATOR
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