Cochlear Implantation in Pediatric Cases of Unilateral Hearing Loss (CIPUHL)

This feasibility study evaluates whether children with unilateral, moderate to profound sensorineural hearing loss experience an improvement in speech perception, hearing in noise, localization, and quality of life with a cochlear implant as compared to an unaided listening condition.

Study Overview

• Status: Completed
• Conditions: Hearing Loss, Unilateral
• Intervention / Treatment: Device: Cochlear implant

Detailed Description

Unilateral hearing loss (UHL) is a term used to describe a substantial hearing loss in one ear and normal hearing in the contralateral ear. Despite normal hearing in one ear, these individuals experience reduced speech perception in noise, variable abilities on localization tasks, increased reports of hearing handicap, reduced quality of life, and often, they obtain limited or no benefit from conventional amplification. In the United States, the prevalence of UHL in children ranges from 0.03% to 3%, depending on the age of the child.

Cochlear implantation performed for children with bilateral, severe to profound deafness has significant impacts on several aspects of child development.

The practice of providing cochlear implants to children who have significant hearing loss in one ear is of great interest and is occurring with greater frequency as reported in case studies and small set clinical reports. The primary purpose of this feasibility study is to demonstrate the effectiveness of cochlear implantation in children, age 3 years 6 months to 6 years, 6 months, with moderate to profound UHL. Postoperative results will be evaluated with speech perception measures, localization tasks, hearing in noise tasks, and subjective reports.

• Study Type: Interventional
• Enrollment (Actual): 20
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • North Carolina
    • Chapel Hill, North Carolina, United States, 27599-7600
      • University of North Carolina At Chapel Hill

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 3 years to 6 years (CHILD)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

Description

i) Inclusion Criteria

1. Unilateral moderate-to-profound sensorineural hearing loss.

   1. Unaided residual hearing thresholds that yield a pure tone average (PTA) at frequencies 500 Hz, 1 kHz and 2 kHz of ≥70 dB HL in the ear to be implanted. It is possible that subjects may have hearing at other frequencies not included in this average.
   2. Hearing thresholds in the contralateral ear of ≤25 dB HL
2. Between 3 years, 6 mos and 6 years, 6 mos of age at implantation.
3. Anatomically normal cochlear nerve

4. Cochlear anatomy that is amenable to cochlear implantation as evaluated by imaging (modality at the physician's discretion) including:

   1. Normal cochlear anatomy or
   2. Incomplete Partition Type II (IP2) with or without Enlarged Vestibular Aqueduct (EVA) or
   3. EVA with normal partitioning
5. No evidence of progressive hearing loss.
6. Willing to undergo 4 week hearing aid trial as warranted based on achieving desired audibility when fitted via real ear desired sensation level (DSL) method.

7. Aided word recognition in the ear to be implanted of 30% or less as measured with Consonant Nucleus Consonant (CNC) words (50-word list)

   1. When listening with an appropriately fit hearing aid and masking applied to the contralateral ear (Turner, 2004).
   2. Aided testing will be conducted in a sound-proof booth with the participant seated 1 meter from the sound source, facing 0° azimuth. Recorded materials will be presented at 60 dB SPL.
   3. The hearing aid output will be measured using DSL targets.
8. Realistic parental expectations: a verbal acknowledgement of the potential benefits and risks, and postoperative variation in performance. For instance, cochlear implantation will not restore normal hearing.
9. Willing to obtain recommended meningitis vaccinations per CDC recommendations.

(9) Development and cognition within the normal range as measured by the Leiter-R test of nonverbal intelligence and cognitive abilities and the Bracken Basic Concept Scale -Revised.

(10) Parental commitment to study parameters including being able and willing to participate in evaluation schedule, involvement in prescribed therapy, and travel to investigational site and study-related activities.

ii) Exclusion Criteria

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. Compromised auditory nerve
4. Ossification of the cochlea
5. Inability to participate in follow-up procedures (i.e., unwillingness, geographic location)
6. History of condition that contraindicates middle or inner ear surgery or anesthesia (i.e. otitis media refractory to treatment)
7. Case of sudden sensorineural hearing loss that has not been first evaluated by a physician

Study Plan

How is the study designed?

Design Details

• Primary Purpose: TREATMENT
• Allocation: NA
• Interventional Model: SINGLE_GROUP
• Masking: NONE

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
EXPERIMENTAL: Cochlear Implant; Pediatric patients with single sided deafness will receive a cochlear implant in the ear of loss	Device: Cochlear implant; Implantation; Other Names: MED-EL Cochlear Implant; MED-EL Synchrony with FLEX 28 array; MED-EL Synchrony with FLEX 24 array

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change Over Time in the Ability to Perceive Single Words as Assessed by Consonant-Nucleus-Consonant (CNC) Word Recognition Testing	Recorded, open set word recognition presented pre-operatively in the soundfield when using a traditional hearing aid (contralateral ear masked) and at 3-, 6-, 9-, 12-, 18-, and 24-month post-activation intervals by direct input to the speech processor using the Consonant-Nucleus-Consonant (CNC) words. Scores are reported as percent words correct and a higher score is better. Change is measured from the pre-operative to the 3-month post-activation interval, and from the 3-month post-activation interval to each subsequent interval.	up to 24 months post-activation
Mean Change in the Signal-to-noise Ratio 50 (SNR-50) From Device Off to Device on as Measured With the Bamford-Kowal-Bench Speech in Noise Test (BKB-SIN) at 6 Months Post-activation	Two ½ lists of the BKB-SIN were presented in each condition device off and device on after 6-months of device use: Speech and masker in front, speech in front and masker directed 90 degrees to the normal ear, speech in front and masker directed 90 degrees to the affected ear. The SNR-50 represents the signal-to-noise ratio required to perceive 50% of the sentence. A lower score is better.	6 months post-activation
Mean Change in the SNR-50 From Device Off to Device on as Measured With the BKB-SIN at 12 Months Post-activation	Two ½ lists of the BKB-SIN were presented in each condition device off and device on after 12-months of device use: Speech and masker in front, speech in front and masker directed 90 degrees to the normal ear, speech in front and masker directed 90 degrees to the affected ear. The SNR-50 represents the signal-to-noise ratio required to perceive 50% of the sentence. A lower score is better.	12 months post-activation
Mean Change in the SNR-50 From Device Off to Device on as Measured With the BKB-SIN at 24 Months of Device Use	Two ½ lists of the BKB-SIN were presented in each condition device off and device on after 24-months of device use: Speech and masker in front, speech in front and masker directed 90 degrees to the normal ear, speech in front and masker directed 90 degrees to the affected ear. The SNR-50 represents the signal-to-noise ratio required to perceive 50% of the sentence. A lower score is better.	24 months post-activation
Median Change in Localization Error From Device Off to Device on at 3 Months Post-activation	Soundfield localization of 200-ms speech-shaped noise, presented from 11 speakers at 70 decibel (dB) sound pressure level (SPL) in a sound treated room. Error measures are made device on and device off after 3 months of device use. Overall root-mean-square (RMS) error is the difference between the sound source azimuth and the response azimuth and a lower score indicates more accurate localization of the sound source. Random error is the average of the standard deviation of the responses for each source and a lower score reflects a more consistently accurate response. Constant error is a measure of side bias, and a lower score indicates less response bias to either side. Adjusted constant error is a measure of reliability in the response taking side bias into account, and a lower score indicates a more reliable response.	3 months post-activation
Median Change in Localization Error From Device Off to Device on at 9 Months Post-activation	Soundfield localization of 200-ms speech-shaped noise, presented from 11 speakers at 70 decibel (dB) sound pressure level (SPL) in a sound treated room. Error measures are made device on and device off after 9 months of device use. Overall root-mean-square (RMS) error is the difference between the sound source azimuth and the response azimuth and a lower score indicates more accurate localization of the sound source. Random error is the average of the standard deviation of the responses for each source and a lower score reflects a more consistently accurate response. Constant error is a measure of side bias, and a lower score indicates less response bias to either side. Adjusted constant error is a measure of reliability in the response taking side bias into account, and a lower score indicates a more reliable response.	9 months post-activation
Median Change in Localization RMS Error From Device Off to Device on at 18 Months Post-activation	Soundfield localization of 200-ms speech-shaped noise, presented from 11 speakers at 70 decibel (dB) sound pressure level (SPL) in a sound treated room. Error measures are made device on and device off after 18 months of device use. Overall root-mean-square (RMS) error is the difference between the sound source azimuth and the response azimuth and a lower score indicates more accurate localization of the sound source. Random error is the average of the standard deviation of the responses for each source and a lower score reflects a more consistently accurate response. Constant error is a measure of side bias, and a lower score indicates less response bias to either side. Adjusted constant error is a measure of reliability in the response taking side bias into account, and a lower score indicates a more reliable response.	18 months post-activation
Median Change in Localization Error From Device Off to Device on at 24 Months Post-activation	Soundfield localization of 200-ms speech-shaped noise, presented from 11 speakers at 70 decibel (dB) sound pressure level (SPL) in a sound treated room. Error measures are made device on and device off after 24 months of device use. Overall root-mean-square (RMS) error is the difference between the sound source azimuth and the response azimuth and a lower score indicates more accurate localization of the sound source. Random error is the average of the standard deviation of the responses for each source and a lower score reflects a more consistently accurate response. Constant error is a measure of side bias, and a lower score indicates less response bias to either side. Adjusted constant error is a measure of reliability in the response taking side bias into account, and a lower score indicates a more reliable response.	24 months post-activation
Change in Mean Response Scores on the Speech Spatial & Qualities (SSQ) Questionnaire	The Speech Spatial Qualities Questionnaire requires participants to rate their perceived hearing ability for 49 scenarios using a 10-point scale, ranging from 0 (Not at all) to 10 (Perfectly). A score higher than 0 for each listening scenario shows some benefit of the device, while a score of 10 indicates the device was extremely beneficial. The SSQ questionnaire assesses performance in 3 domains, hearing speech in quiet and noise environments (9 items), spatial or directional hearing (5 items) and sound qualities (8 items). Domain scores represent an average of item ratings, therefore each domain score ranges from 0 to 10 with a higher score indicating a better outcome. The pediatric version of this test was used and parents reported as proxy pre-operatively and at at 3-, 6-, 9-, 12-, 18-, and 24-month post-activation intervals. Change is measured from the pre-operative to the 3-month post-activation interval and between the 3-month interval to all subsequent intervals.	Pre-operatively through 24 months post-activation
Change in Mean Response Scores as Measured by the Bern Single Sided Deafness (SSD) Questionnaire	Bern SSD Questionnaire is a 10-item questionnaire used for rating perceived benefit of a hearing technology designed to be used for persons with Unilateral Hearing Loss (UHL). A positive score indicates greater perceived ability with the device and a negative score indicates greater perceived ability without the device. Scores range from -5 meaning much easier listening without the hearing device to +5 indicating much easier listening with the device. Scores across all 10 items are averaged for a single score. A modified version of this test that can be completed by a parent was used pre-operatively and at 3-, 6-, 9-, 12-, 18-, and 24-month post-activation intervals. Change is measured from the pre-operative to the 3-month post-activation interval and from the 3-month interval to each subsequent interval.	Pre-operatively through 24 months post-activation
Change in Mean Fatigue Scores as Measured by the Pediatric Quality of Life (PedsQL) Multidimensional Fatigue Scale	PedsQL Multidimensional fatigue scale is a validated scale for determining fatigue in young children, including general fatigue, sleep/rest fatigue and cognitive fatigue. Scores from this test have been previously demonstrated to be substantially affected by hearing loss in children. A parent report version was used pre-operatively and at 3-, 6-, 9-, 12-, 18-, and 24-month post-activation intervals. Items are scored on a 5-point Likert scale and are then reversed scored and linearly transformed to a 0-100 scale where 100 would indicate no fatigue and 0 would indicate near constant fatigue.	Pre-operatively through 24 months post-activation

Collaborators and Investigators

• Sponsor: University of North Carolina, Chapel Hill
• Collaborators: Med-El Corporation

Publications and helpful links

General Publications

• Arndt S, Aschendorff A, Laszig R, Beck R, Schild C, Kroeger S, Ihorst G, Wesarg T. Comparison of pseudobinaural hearing to real binaural hearing rehabilitation after cochlear implantation in patients with unilateral deafness and tinnitus. Otol Neurotol. 2011 Jan;32(1):39-47. doi: 10.1097/MAO.0b013e3181fcf271.
• Firszt JB, Holden LK, Reeder RM, Waltzman SB, Arndt S. Auditory abilities after cochlear implantation in adults with unilateral deafness: a pilot study. Otol Neurotol. 2012 Oct;33(8):1339-46. doi: 10.1097/MAO.0b013e318268d52d.
• Bess FH, Tharpe AM, Gibler AM. Auditory performance of children with unilateral sensorineural hearing loss. Ear Hear. 1986 Feb;7(1):20-6. doi: 10.1097/00003446-198602000-00005.
• Arndt S, Prosse S, Laszig R, Wesarg T, Aschendorff A, Hassepass F. Cochlear implantation in children with single-sided deafness: does aetiology and duration of deafness matter? Audiol Neurootol. 2015;20 Suppl 1:21-30. doi: 10.1159/000380744. Epub 2015 May 19.
• Kompis M, Pfiffner F, Krebs M, Caversaccio MD. Factors influencing the decision for Baha in unilateral deafness: the Bern benefit in single-sided deafness questionnaire. Adv Otorhinolaryngol. 2011;71:103-111. doi: 10.1159/000323591. Epub 2011 Mar 8.
• Varni JW, Limbers CA, Burwinkle TM. Impaired health-related quality of life in children and adolescents with chronic conditions: a comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL 4.0 Generic Core Scales. Health Qual Life Outcomes. 2007 Jul 16;5:43. doi: 10.1186/1477-7525-5-43.
• PETERSON GE, LEHISTE I. Revised CNC lists for auditory tests. J Speech Hear Disord. 1962 Feb;27:62-70. doi: 10.1044/jshd.2701.62. No abstract available.
• Galvin KL, Noble W. Adaptation of the speech, spatial, and qualities of hearing scale for use with children, parents, and teachers. Cochlear Implants Int. 2013 Jun;14(3):135-41. doi: 10.1179/1754762812Y.0000000014.

Study record dates

Study Major Dates

• Study Start (ACTUAL): April 20, 2017
• Primary Completion (ACTUAL): May 7, 2021
• Study Completion (ACTUAL): May 7, 2021

Study Registration Dates

• First Submitted: October 27, 2016
• First Submitted That Met QC Criteria: November 10, 2016
• First Posted (ESTIMATE): November 15, 2016

Study Record Updates

• Last Update Posted (ACTUAL): August 6, 2021
• Last Update Submitted That Met QC Criteria: August 4, 2021
• Last Verified: June 1, 2021

More Information

Terms related to this study

• Keywords: cochlear implant
• Additional Relevant MeSH Terms: Nervous System Diseases; Neurologic Manifestations; Otorhinolaryngologic Diseases; Ear Diseases; Sensation Disorders; Hearing Disorders; Hearing Loss; Deafness; Hearing Loss, Unilateral
• Other Study ID Numbers: 15-3350

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes