Cochlear Implant Stimulation for Usher Syndrome

No longer recruiting at 3 trial locations

Age: Any Age

Sex: Any

Trial Phase: Academic

Sponsor: Ohio State University

Disqualifiers: Severe medical comorbidities, electrode malposition, others

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Approved in 4 Jurisdictions This treatment is already approved in other countries

Compensation: Varies

Number of Visits: Unknown

Duration: 2 years

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial examines how cochlear implants function for individuals with Usher syndrome, which affects both hearing and vision. Researchers aim to understand how effectively the cochlear nerve processes sounds when using a cochlear implant. They will compare these results with individuals who have hearing loss from other causes. The trial seeks both adults and children who have used a cochlear implant for at least six months and have been diagnosed with either Usher syndrome or idiopathic hearing loss (hearing loss with no known cause). As an unphased trial, this study offers participants the chance to contribute to groundbreaking research that could enhance understanding and treatment options for those with hearing loss.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications.

What prior data suggests that this cochlear implant stimulation is safe for Usher syndrome patients?

Research has shown that changing the settings in cochlear implants is generally safe for people with Usher syndrome. One study found that patients heard better and understood speech more clearly after these changes, indicating that any side effects from adjusting the settings are manageable. Another study confirmed that these changes are well-tolerated, meaning patients can handle them without major issues. This evidence suggests these adjustments are safe, which is encouraging for those considering joining the trial. ^{1 2 3 4 5}

Why are researchers excited about this trial?

Researchers are excited about the experimental manipulation of cochlear implant stimulation parameters for Usher syndrome because it offers a tailored approach to enhancing hearing. Unlike standard cochlear implants that use fixed settings, this method allows adjustments personalized to each user's needs, potentially improving hearing outcomes. This unique approach aims to maximize the auditory experience for individuals with Usher syndrome, giving them a better quality of life by fine-tuning the implant's performance.

What evidence suggests that this experimental manipulation of stimulation parameters is effective for Usher syndrome?

Research shows that cochlear implants can help people with Usher syndrome hear better and understand speech more clearly. Studies have found that patients often notice improved sound detection and clearer speech after receiving these implants. Many patients report better speech understanding, clearer speech, and an overall improved quality of life. Additionally, those who receive implants at a younger age tend to achieve the best results in hearing and cognitive skills. This trial will compare cochlear implant users with Usher syndrome to those with idiopathic hearing loss, suggesting that adjusting cochlear implant functionality could be a promising way to enhance hearing for people with Usher syndrome. ^{1 3 4 6 7}

Who Is on the Research Team?

Shuman He, MD, PhD

Principal Investigator

Ohio State University

Are You a Good Fit for This Trial?

This trial is for individuals with Usher Syndrome or idiopathic hearing loss who have used a cochlear implant for at least 6 months. It's not suitable for those with severe medical conditions or issues with the position of their cochlear implant electrodes.

Inclusion Criteria

You have had a cochlear implant for at least 6 months.

I have been diagnosed with Usher syndrome or have hearing loss without a known cause.

Exclusion Criteria

The electrodes are not placed correctly or have moved, as shown in the imaging tests.

I have severe health conditions besides my cancer.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Evaluation of local neural health and neural encoding of temporal and spectral cues at the cochlear nerve in implanted patients with Usher syndrome

2 years

Regular visits as per study protocol

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

• Experimental manipulation of stimulation parameters

Trial Overview The study aims to understand how well the auditory nerve functions in patients with Usher Syndrome using cochlear implants by manipulating stimulation settings and measuring responses. It also seeks to develop a tool to assess the connection between electrodes and nerves.

How Is the Trial Designed?

Group I: Usher Syndrome Experimental Treatment 1 Intervention

Adult and pediatric cochlear implant users with Usher syndrome

Group II: Idiopathic Hearing Loss Active Control 1 Intervention

Adult and pediatric cochlear implant users with idiopathic hearing loss

Experimental manipulation of stimulation parameters is already approved in European Union, United States, Canada for the following indications:

Approved in European Union as Cochlear implant for:

• Severe to profound sensorineural hearing loss
• Single-sided deafness
• Bilateral profound sensorineural hearing loss in children

Approved in United States as Cochlear implant for:

• Severe to profound sensorineural hearing loss
• Single-sided deafness
• Bilateral profound sensorineural hearing loss in children

Approved in Canada as Cochlear implant for:

• Severe to profound sensorineural hearing loss
• Single-sided deafness
• Bilateral profound sensorineural hearing loss in children

Who Is Running the Clinical Trial?

Ohio State University

Lead Sponsor

Trials

891

Recruited

2,659,000+

National Institute on Deafness and Other Communication Disorders (NIDCD)

Collaborator

Trials

377

Recruited

190,000+

Published Research Related to This Trial

In a study of 7356 patients with hearing impairment, only 0.71% were found to be candidates for electric-acoustic stimulation (EAS) implants, compared to 2.52% for standard cochlear implants (CIs).

Among the EAS candidates, only 5.83% (2 individuals) actually received the EAS implant surgery, highlighting the need for healthcare professionals to thoroughly evaluate patients with high-frequency hearing loss for potential EAS eligibility.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Prevalence of potential candidates for electric-acoustic stimulation implant in a hearing-impaired population. Saito, K., Fujita, T., Osaki, Y., et al.[2021]

A new 3D-printed cochlea model with integrated sensing wires allows for better understanding of how electrical stimulation spreads in cochlear implants, which is crucial for improving hearing quality in deaf patients.

The study reveals a trade-off between stimulation amplitude and current focusing across different stimulation modes (monopolar, bipolar, tripolar), providing insights that could enhance clinical strategies for delivering clearer speech signals to cochlear implant users.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

An Instrumented Cochlea Model for the Evaluation of Cochlear Implant Electrical Stimulus Spread. Jiang, C., Singhal, S., Landry, T., et al.[2022]

Combined electroacoustic stimulation in residually-hearing ears enhances low-frequency sound perception and expands the dynamic range of hearing compared to pure electrical stimulation alone.

Physiological studies indicate that electrically stimulated hearing ears show less synchronization in evoked activity than deaf ears, which helps reduce hypersynchronization and allows for better integration of acoustic and electric inputs.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Physiological Mechanisms in Combined Electric-Acoustic Stimulation. Sato, M., Baumhoff, P., Tillein, J., et al.[2018]

Out of 712 pediatric cochlear implant recipients, 26 (3.7%) were diagnosed with Usher syndrome, and preoperative imaging showed no cochlear malformations, indicating that cochlear implants can be safely performed in these patients.

Most children with Usher syndrome who received cochlear implants achieved significant speech perception (92%) and primarily used oral communication (69.2%), demonstrating the efficacy of cochlear implantation in improving communication skills despite challenges related to their condition.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Usher syndrome: characteristics and outcomes of pediatric cochlear implant recipients. Jatana, KR., Thomas, D., Weber, L., et al.[2013]

In a study of 115 children with cochlear implants, those using the precurved Nucleus 24RE array showed significantly lower electrically evoked compound action potential (ECAP) and behavioral thresholds, indicating better auditory responsiveness compared to those using the Nucleus 24M and 24RCS arrays.

The 24RE group also achieved higher speech perception scores, suggesting that this cochlear implant design may enhance functional hearing outcomes in profoundly hearing-impaired children.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Evolution of cochlear implant arrays result in changes in behavioral and physiological responses in children. Gordin, A., Papsin, B., James, A., et al.[2022]

Cochlear implants have successfully provided partial hearing to over 120,000 people worldwide, including many children who can develop nearly normal language skills, highlighting their efficacy in treating hearing loss.

The success of cochlear implants is attributed to a collaborative effort among engineers, scientists, and healthcare professionals, focusing on system design, safety, and integration, which sets a model for developing other neural prostheses.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Cochlear implants: system design, integration, and evaluation. Zeng, FG., Rebscher, S., Harrison, W., et al.[2022]

Optogenetic stimulation using multi-channel optical cochlear implants (oCIs) can provide high spectral selectivity in activating the auditory nerve, which may improve speech recognition in noisy environments compared to traditional electrical cochlear implants (eCIs).

In a study involving adult Mongolian gerbils, the oCIs demonstrated effective tonotopic activation of the auditory pathway with modest power requirements, indicating their potential for restoring hearing in both hearing and deaf subjects.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

μLED-based optical cochlear implants for spectrally selective activation of the auditory nerve. Dieter, A., Klein, E., Keppeler, D., et al.[2021]

In a study of 17 children with limited low-frequency hearing who underwent cochlear implantation (CI), electroacoustic stimulation (EAS) rehabilitation showed potential benefits, particularly in noisy environments, enhancing speech perception compared to CI-only mode.

Successful EAS rehabilitation was achieved in 9 out of 21 ears, with the postoperative low-frequency pure-tone average identified as a key predictive factor for better outcomes, suggesting that preserving low-frequency hearing during CI can improve auditory rehabilitation.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Outcomes and Predictive Factors of Electroacoustic Stimulation Rehabilitation in Children With Limited Low-Frequency Hearing. Nam, GS., Song, MH., Choi, JY., et al.[2020]

Cochlear implants are highly effective neural prostheses that can successfully mimic the natural hearing process by electrically stimulating the cochlea in deaf individuals.

Research into how the central auditory system responds to cochlear implants can enhance our understanding of hearing restoration and provide insights into central hearing mechanisms, independent of the cochlea's physical functions.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Cochlear implants: the view from the brain. Middlebrooks, JC., Bierer, JA., Snyder, RL.[2006]

Cochlear implants provide a significant rehabilitative solution for severely deaf individuals, allowing them to experience sound and communicate effectively, with 7 out of 16 patients achieving excellent results by hearing solely through the implant.

The process includes thorough auditory diagnosis, precise tuning of the implant, and an 8-week hearing training program, demonstrating that even patients with moderate outcomes find substantial benefits from the device, enhancing their daily lives.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

[Auditory achievements of cochlear implantation]. Hildesheimer, M., Muchnik, C., Tenne, S., et al.[2006]

In a study involving 20 cochlear implant patients, those with combined electric acoustic stimulation (EAS) showed significant advantages in speech perception when both electrical and acoustic stimulation were used compared to using either stimulation alone.

The EAS group preserved hearing in 9 out of 10 patients post-surgery, while the conventional cochlear implant group lost all residual hearing, highlighting the potential safety and efficacy of EAS in maintaining hearing while improving speech understanding.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Is electric acoustic stimulation better than conventional cochlear implantation for speech perception in quiet? Adunka, OF., Pillsbury, HC., Adunka, MC., et al.[2022]

The earliest sign of Usher syndrome in patients is delayed walking, with an average onset at 20 months, which is significantly later than the average of 14 months in other congenitally deaf children, indicating a need for early diagnosis.

Cochlear implantation in children with Usher syndrome leads to good speech perception outcomes, especially when performed before the age of 9, highlighting the importance of early intervention for optimizing rehabilitation and speech therapy.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Usher syndrome and cochlear implantation. Loundon, N., Marlin, S., Busquet, D., et al.[2019]

Chronic electrical stimulation of the auditory nerve can lead to significant complications, including surgical trauma and electrode displacement, which may occur at various times after insertion.

Histopathological analysis revealed severe damage, such as loss of hair cells and spiral ganglion cells, as well as new bone growth, highlighting the need for improved design and safety measures in cochlear prostheses.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

[Cochlear pathology following chronic electrical stimulation in cats]. Ni, DF.[2007]

Stimulation-Current-Induced Non-Stimulating Electrode Voltage Recordings (SCINSEVs) effectively detect extracochlear electrodes (EEs) in cochlear implants, showing high sensitivity and specificity (91% for CI-A and 100% for CI-B) across different manufacturers' devices.

The study demonstrated that SCINSEVs can generalize to various cochlear implant designs, indicating a promising tool for improving detection of EEs, which can negatively impact speech outcomes in patients.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Detection of Extracochlear Electrodes Using Stimulation-Current- Induced Non-Stimulating Electrode Voltage Recordings With Different Electrode Designs. de Rijk, SR., Hammond-Kenny, A., Tam, YC., et al.[2023]

Citations

1. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC8267700/

The Outcomes of Cochlear Implantation in Usher Syndrome USH patients experienced improvements in PTA and speech perception and expression outcomes after CI, as well as improvements in phonological ...

2. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC10858075/

Outcomes of cochlear implantation in Usher syndrome Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, ...

3. researchgate.net researchgate.net/publication/352827838_The_Outcomes_of_Cochlear_Implantation_in_Usher_Syndrome_A_Systematic_Review

The Outcomes of Cochlear Implantation in Usher Syndrome Most USH patients benefit from CI. USH patients who undergo CI at younger ages generally achieve better hearing, speech, and cognitive outcomes.

4. journals.lww.com journals.lww.com/ear-hearing/fulltext/2022/03000/phantom_stimulation_for_cochlear_implant_users.34.aspx

Phantom Stimulation for Cochlear Implant Users With... This work investigates the use of phantom stimulation for EAS users in terms of speech understanding.

5. mdpi.com mdpi.com/2039-4349/14/2/23

Navigating the Usher Syndrome Genetic Landscape Our review of 95 cochlear-implant patients with US, along with their CI outcomes, revealed the importance of presurgical genetic testing.

6. withpower.com withpower.com/trial/phase-usher-syndromes-9-2021-cb8bd

Cochlear Implant Stimulation for Usher Syndrome ... Experimental manipulation of stimulation parameters will have tolerable side effects & efficacy for patients with Usher Syndrome, Postprandial Fullness ...

7. usher-syndrome.org usher-syndrome.org/what-is-usher-syndrome/science-news/general-news.html

General Research News Navigating the Usher Syndrome Genetic Landscape: An Evaluation of the Associations between Specific Genes and Quality Categories of Cochlear Implant Outcomes.