Auditory Brainstem Implantation in Young Children

N/ACompletedNCT01904448

University of North Carolina, Chapel Hill5 enrolled

Overview

To purpose of this feasibility study is to demonstrate the safety and efficacy of the Nucleus 24 Multichannel Auditory Brainstem Implant (ABI, Cochlear Corp, Sydney, AUS) in children without the diagnosis of neurofibromatosis type II (NFII) that have either experienced failed cochlear implantation (CI) or have been unable to receive a CI secondary to cochlear or cochlear nerve disorders. These conditions can include: developmental or acquired cochlear nerve deficiency (CND), cochlear aplasia (Michel), post-meningitic cochlear ossification or cochlear malformation. This study proposes to implant up to 10 young children (\<5 yrs. of age) with the Nucleus 24 Multichannel ABI (Sydney, AUS) in an attempt to demonstrate safety of the surgical procedure, tolerance of device stimulation, and the potential for auditory benefit beyond that experienced with their CI. This study will provide the preliminary experience for a larger scale clinical trial. Aim 1: Demonstrate the safety of ABI surgery in children. Aim 2: Demonstrate the development of sound awareness and improved speech understanding among children implanted with the ABI when compared to their baseline skills. Aim 3: Demonstrate the development of oral language skills following the use of the ABI that were not evident prior to its use.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Developmental Cochlear Nerve Deficiency

Eligibility

Sex: ALLMin age: 18 MonthsMax age: 17 Years

Medical Language ↔ Plain English

Inclusion Criteria: * Pre-linguistic hearing loss (birth-5 yrs.; age at ABI 18 months-5yrs) with both: * MRI +/- CT evidence of one of the following: Cochlear nerve deficiency, Cochlear aplasia or severe hypoplasia, Severe inner ear malformation, Post-meningitis ossification * When a cochlea is present or patent, lack of significant benefit from CI despite consistent use (\>6 mo.) * Post-linguistic hearing loss (\<18 yrs. of age) with both: * Loss or lack of benefit from appropriate CI without the possibility for revision or contralateral implantation. Examples might include: Post-meningitis ossification, Bilateral temporal bone fractures with cochlear nerve avulsion, Failed revision CI without benefit * Previously developed open set speech perception and auditory-oral language skills * No medical contraindications * Willing to receive the appropriate meningitis vaccinations * No or limited cognitive/developmental delays which would be expected to interfere with the child's ability to cooperate in testing and/or programming of the device, in developing speech and oral language, or which would make an implant and subsequent emphasis on aural/oral communication not in the child's best interest * Strong family support including language proficiency of the parent(s) in the child's primary mode of communication as well as written and spoken English. * Reasonable expectations from parents including a thorough understanding: * of potential benefits and limitations of ABI * of parental role in rehabilitation * that the child may not develop spoken language as a primary communication mode or even sufficient spoken language to make significant academic progress in an aural/oral environment * Involvement in an educational program that emphasizes development of auditory skills with or without the use of supplementary visual communication. * Able to comply with study requirements including travel to investigation sites. * Informed consent for the procedure from the child's parents/legal guardian. Exclusion Criteria: * Pre- or post-linguistic child currently making significant progress with CI * MRI evidence of one of the following: * normal cochlea and cochlear nerves or NFII * brainstem or cortical anomaly that makes implantation unfeasible * Clear surgical reason for poor CI performance that can be remediated with revision CI or contralateral surgery rather than ABI. * Intractable seizures or progressive, deteriorating neurological disorder * Unable to participate in behavioral testing and mapping with their CI. If this appears to be an age effect, ABI will be delayed until we can be assured that the child will be able to participate, as reliable objective measures of mapping are currently not available for mapping these devices. * Lack of potential for spoken language development. This will be considered the case when evidence of the following exist: * Severe psychomotor retardation, autism, cerebral palsy, or developmental delays beyond speech that would preclude usage of the device and oral educational development. Autism is a special case where there is the potential for delayed presentation. When early signs are considered present, our group routinely requests a comprehensive developmental assessment for further evaluation prior to considering routine evaluation. A specialized group is readily available at our institution for such an evaluation. * Unable to tolerate general anesthesia (cardiac, pulmonary, bleeding diathesis, etc.). * Need for brainstem irradiation * Unrealistic expectations on the part of the subject/family regarding the possible benefits, risks and limitations that are inherent to the procedure and prosthetic device. * Unwilling to sign the informed consent. * Unwilling to make necessary follow-up appointments.

Outcomes

Primary Outcomes

Number of ABI Complications

Complications and related outcomes will be tracked and recorded. These include bleeding, infection, neural injury, cerebrospinal fluid (CSF) leakage, brain bleeding or bruising, stroke, death. Also, complications and sequelae related to the device such as non-auditory stimulation and device failure will be tracked.

Time frame: Year 3

Secondary Outcomes

The Oral and Written Language Scales (OWLS) Listening Comprehension Score

OWLS-II: Listening Comprehension measures oral language reception, which is the understanding of spoken language. The examiner orally presents increasingly difficult words, phrases, and sentences to the participant and he/she responds by pointing to or stating which of four pictures is correct. The derived mean standard score for this test is 100 with a standard deviation of 15. Higher scores indicate better listening comprehension. Scores of 85 to 115 are within normal limits. Scores that fall 2 standard deviations below the mean are considered to be severely delayed.

Time frame: Year 3

The Oral and Written Language Scales (OWLS) Oral Expression Score

OWLS-II: Oral Expression measures oral language expression, which is the use of spoken language. The examiner presents a verbal prompt along with a picture and the participant must respond orally to the prompt with increasingly difficult language. The derived mean standard score for this test is 100 with a standard deviation of 15. Higher scores indicate better oral expression. Scores of 85 to 115 are within normal limits. Scores that fall 2 standard deviations below the mean are considered to be severely delayed.

Time frame: Year 3

Goldman-Fristoe Test of Articulation Scores (GFTA)

The GFTA-2 is a standardized, norm-based articulation measure that samples spontaneous sound production. Children are asked to respond to picture plates and verbal cues from the examiner with single words that test consonant accuracy in initial, medial, and final positions. This measure has norms based on the performance of normal-hearing children from age 2 years to 21 years.

Time frame: Year 3

MacArthur-Bates Communicative Development Inventories (CDIs) Score

MacArthur-Bates Communicative Development Inventories (CDIs)

Time frame: Year 3

Percentage of Correctly Produced Items From the Identifying Early Phonological Needs in Children With Hearing Loss (IEPN) Test-Vocalize on Demand

This is a measure of the child's ability to vocalize on demand elicited by pictures of objects. Scores are derived from the percentage of time the child vocalized for 25 words.