Trials Today

Conditions

• Single Sided Deafness
• Unilateral Deafness
• Hearing Loss, Unilateral
• Hearing Loss
• Congenital Hearing Loss
• Deafness One Ear

Eligibility

Eligible Ages

Between 7 Months and 71 Months

Eligible Sex

All

Accepts Healthy Volunteers

Yes

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)

Study Contact

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.