Ear, Nose, and Throat Diseases

Nervous System Diseases

Symptoms and General Pathology

All Conditions

Hearing Loss

Deafness

Hearing Loss, Bilateral

Hearing Disorders

Ear Diseases

Otorhinolaryngologic Diseases

Sensation Disorders

Neurologic Manifestations

All subjects will be part of a single arm involving placement of the ABI541 Auditory Brainstem Implant (ABI) device. The Nucleus 24 was discontinued and is no longer available.

Nucleus ABI541 Auditory Brainstem Implant (ABI) surgery followed by device activation, testing, and clinical assessment for five years following surgery.

Nucleus 24 (discontinued) and ABI541 Auditory Brainstem Implants (ABI)

Daniel J Lee, MD FACS

The purpose of this research study is to determine whether Auditory Brainstem Implant (ABI) can improve hearing in children who are deaf and cannot receive a cochlear implants.

The goal of this MEEI Auditory Brainstem Implant (ABI) research study is to find new ways to improve hearing in children who are deaf and cannot receive a cochlear implant. The ABI is a surgically placed bionic implant that converts sounds into electrical signals that are directly transmitted to the cochlear nucleus, the first auditory center of the brain. For many years, ABIs have improved the hearing of patients who are deaf due to brain tumors associated with a genetic syndrome called Neurofibromatosis Type 2 (NF2). However, a number of recent studies suggest that deaf patients who do not have NF2 and are not eligible for a cochlear implant may also benefit from placement of an ABI. These preliminary studies suggest that these non-NF2 or "nontumor" patients may actually have better outcomes after ABI surgery than patients suffering from NF2. Children appear to be particularly good candidates because of their developmental plasticity and in many studies, outcomes are more favorable in children that adults. Patients who do not have NF2 and are deaf due to abnormalities in their hearing nerves or inner ears from congenital malformations, infection, disease, or injury are not cochlear implant candidates and there are no other options to improve hearing in these cases except for the ABI. Thus, the purpose of our study is to carefully analyze whether ABI surgery improves the hearing and quality of life of non-NF2 children based on subjective and objective measures of their hearing before and after ABI surgery. In particular, we plan to study ABI outcomes in non-NF2 pediatric patients, characterize the parameters used on their devices, and determine the safety profile of ABIs in these patients.

Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects

Study of the Nucleus 24 and ABI541 Auditory Brainstem Implants in Pediatric Non-Neurofibromatosis Type 2

We will track any major and minor complications peri-operatively and post-operatively in all study participants. Major complications include mortality, cerebellar contusion, facial palsy, meningitis, lower cranial neuropathy, hydrocephalus, pseudomeningocele and CSF leak. Minor complications include transient hydrocephalus, wound seroma, minor infections, balance problems, transient nerve palsies, transient dysphonia or difficulty swallowing, headache, flap problems, and non-auditory side effects. These complications will be tracked through serial clinical exams as well as patient recorded questionnaires to determine the safety profile of ABI surgery.

We will complete audiometric threshold testing and speech based tests to determine performance before and after ABI surgery.

The measure data value was calculated as mean threshold shift in dB HL from pre-surgery (or baseline) to postoperatively (following activation of the ABI) across subjects analyzed. Only those subjects that had auditory perception abilities were included. 2/9 subjects did not have auditory perception and are not using their devices.

The outcome measure time frame varies across subjects based on available followup data as this study was stopped early.

Audiometric testing will include:

• Behavioral pure tone threshold average (PTA) in both aided (with ABI) and unaided (without ABI)

We will measure and record the specific programmed parameters of the ABI device. Intraoperatively, evoked Auditory Brainstem Response (eABRs) will be recorded and saved. These tracings and data will be compared to the settings on activation to determine whether the same electrodes are appropriately positioned. By following these measures, we will be able to capture how electrophysiologic parameters vary (if at all) over time.

The outcome measure data are the number of subjects whose eABR morphology was similar on the day of activation (4 to 8 weeks following surgery) as recorded intraoperatively during ABI surgery.

Massachusetts Eye and Ear Infirmary

Principal Investigator

Implantation with Auditory Brainstem Implant (ABI) device and clinical follow-up

Non-NF2 ABI Surgery

Age, Categorical

Age, Continuous

Sex: Female, Male

Race and Ethnicity were not collected from any participant.

Race and Ethnicity Not Collected

United States

Region of Enrollment

As all participants have (1) absent cochleae or absent cochlear nerves or (2) anatomic conditions of the inner ear that preclude the use of a traditional cochlear implant array, it was expected that participants would have profound hearing loss at baseline and no residual hearing. Therefore, it was hypothesized that sound detection would be at the upper limit of clinical testing.

The decibel unit is plotted on a logarithmic scale; higher values represents worse hearing. 120 dB HL represents the upper limit of clinical testing.

Sound detection via pure-tone threshold audiometry

Dr. Daniel Lee

Major Complications

Minor Complications

Number of Participants With Major or Minor ABI Complications

2/9 subjects required revision surgery for device issues and were not included in this analysis as they did not complete the study. An additional 2/9 subjects did not have auditory perception and are not using their device and were also not included in this analysis.

Change in Pure Tone Threshold (dB HL) Using the ABI

Number of Participants With Similar eABR Morphology on the Day of ABI Activation Compared to Intraoperative Responses

Overall Study

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant in prelingiustically deaf children ages 18 months - 5 years

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant in postlinguistically deaf children \< 21 years of age

Surgical implantation of the Nucleus ABI541 Auditory Brainstem Implant device

Nucleus ABI541 Auditory Brainstem Implant

Susan B Waltzman, PhD

Kristin Montella

John T. Roland, MD

Susan B. Waltzman, PhD

John T. Roland, Jr., MD

This study is a feasibility study of the Nucleus 24 ABI in children without NF2. It will be conducted as a repeated-measures, single subject experiment in order to accommodate the known variability in cochlear implant clinical results. Data obtained in the course of this study will be compared with the existing published outcome data from children with normal hearing and with cochlear implants (CIs). These comparisons may provide additional useful information about the progress of ABI subjects' performance over time. Also, this may allow the results of the current study to be utilized to design future pivotal studies of the device in the pediatric population.

The purpose of this study is to evaluate the clinical safety and efficacy of the Nucleus™ 24 Auditory Brainstem Implant (ABI) in pediatric patients who do not have Neurofibromatosis Type 2 (NF2); specifically, children with total hearing loss due to severe cochlear anomalies, cochlear nerve disorders or failed cochlear implantation. These conditions can include: developmental or acquired cochlear nerve deficiency (CND), cochlear aplasia, post-meningitis cochlear ossification, or cochlear malformation. This study proposes to implant up to 10 pre-linguistic young children (18 mos to 5 yrs. of age) and up to 10 post-linguistic children (\<21 yrs of age) with the Nucleus 24 Multichannel ABI in an attempt to demonstrate safety of the surgical procedure and device stimulation and the potential for auditory benefit beyond that experienced with a CI. These children would not qualify for the Nucleus ABI device, as their etiology is not an approved indication such as neurofibromatosis Type 2(NF2). Further, these children do not benefit from conventional hearing aids or cochlear implants.

Two groups of children will be included: Group 1 will include prelingiustic deaf children ages 18 months - 5 years and Group 2 will include postlinguistic deaf children \< 21 years of age. Post-operative evaluations will be conducted at the initial activation and at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals post-activation in order to demonstrate the emergence of open-set speech perception abilities prior to study cessation. Experience in the CI literature has shown that a minimum of 2 years of device usage is required for children with pre-lingual hearing impairment to develop some open-set speech perception abilities. While Group 2 is post-linguistic and some may have had significant hearing prior to becoming deaf, the same time intervals will be used for consistency. Blinding or masking procedures are not included in the design, as the presence/absence of a brainstem implant cannot be easily concealed from the device recipients and/or clinical investigators.

Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves

Study participants will be monitored on a case-by-case basis for ongoing or unanticipated medical complications. Adverse events will be tracked on a case-by-case basis and recorded at the time of occurrence and followed up at resolution. Any adverse events will be reported to the NYUSOM IRB. Should there be concern for the safety of the subjects because of their participation in the study by the investigators or the IRB, the study would be halted at least temporarily and a detailed discussion with the investigators and the IRB would be undertaken.

The following audiometry and speech perception tests will be administered as age-appropriate:

* Detection audiogram, aided and unaided individual ears
* IT-MAIS or MAIS
* LING 6 sound test
* ESP Low Verbal or Standard- MLV @ 50dB HL
* PB-k words and phonemes- MLV @ 50dB HL
* MLNT- recorded @ 60dB SPL
* LNT- recorded @ 60dB SPL
* HINT-C- 2 lists of 10 @ 60dB SPL
* CNC- 50 words @ 60dB SPL

Each child will receive speech/language intervention using the Comprehensive Assessment of Spoken Language (CASL, Elizabeth Carrow-Woolfolk, 1999) The following tests will be administered when appropriate:

* The Oral and Written Language Scales (OWLS)
* Goldman-Fristoe Test of Articulation
* The Peabody Picture Vocabulary Test (PPVT)
* The Rossetti Infant-Toddler Language Scale
* Pre-School Language Scale, (PLS-5)
* The Expressive Vocabulary Test, 2nd Ed
* The Peabody Picture Vocabulary Test, 4th Ed

NYU Langone Health

Neoplasms

Rare Diseases

Neurilemmoma

Neuroma, Acoustic

Neuroma

Neuroendocrine Tumors

Neuroectodermal Tumors

Neuroectodermal Tumors, Primitive

Neoplasms, Germ Cell and Embryonal

Neoplasms by Histologic Type

Nerve Sheath Neoplasms

Neoplasms, Nerve Tissue

Nervous System Neoplasms

Peripheral Nervous System Neoplasms

Vestibulocochlear Nerve Diseases

Retrocochlear Diseases

Otorhinolaryngologic Neoplasms

Cranial Nerve Diseases

Schwannoma

Acoustic Neuroma

Neuroendocrine Tumor

Neuroepithelioma

Electrical stimulation of the cochlear nerve will be tested intraoperatively in patients undergoing vestibular schwannoma resection.

Intraoperative electrical stimulation of the cochlear nerve in patients undergoing surgery for resection of vestibular schwannoma. Activation of the auditory system monitored with scalp recording of auditory brainstem response.

Electrical stimulation of cochlear nerve

Harrison W Lin, MD

John C Middlebrooks, PhD

Harrison M Lin, MD

This is a study of the feasibility of activating the auditory system by an electrode in direct contact with the cochlear nerve.

This is a feasibility study being conducted prior to development of a penetrating cochlear nerve stimulating electrode. Experiments will be conducted in patients who are undergoing surgery for resection of a vestibular schwannoma (VS). In the normal course of such a surgical procedure, the cochlear nerve is exposed and, in many cases, the cochlear nerve is resected or function of the cochlear nerve is otherwise disrupted. Typically, recording electrodes are placed on the scalp for the purpose of recording sound-evoked auditory brainstem responses (ABRs), which serve as a measure of the functional status of the cochlear nerve. In this intraoperative experimental procedure, a conventional bipolar nerve stimulating electrode will be placed in contact with the cochlear nerve and the nerve will be stimulated with electrical pulses. Activation of the auditory system by the electrical pulses will be evaluated by measurement of electrically-evoked ABRs, using the same scalp electrodes that are typically used for measurement of sound-evoked ABRs. The cochlear-nerve stimulating electrode will be in place only during the actual intraoperative test. No device will remain in the patient. The intraoperative experimental procedure is not intended to as a therapeutic measure for the patient's VS.

Test of feasibility of electrical stimulation of the cochlear nerve with a conventional bipolar nerve stimulator

Intraoperative Electrically-evoked ABRs in Patients Undergoing Vestibular Schwannoma Surgery

Electrical potentials recorded with scalp electrodes in response to electrical stimulation of the cochlear nerve

University of California, Irvine

Associate Professor

Respiratory Tract (Lung and Bronchial) Diseases

Substance Related Disorders

Wounds and Injuries

Head and Neck Neoplasms

Lung Neoplasms

Tinnitus

Ototoxicity

Drug-Related Side Effects and Adverse Reactions

Chemically-Induced Disorders

Radiation Injuries

Antineoplastic Agents

All Drugs and Chemicals

Anti-Inflammatory Agents

Antirheumatic Agents

Analgesics

Neuroprotective Agents

Gastrointestinal Agents

Carboplatin

Cisplatin

Ebselen

Anti-Inflammatory Agents, Non-Steroidal

Analgesics, Non-Narcotic

Antacids

Anti-Ulcer Agents

Antioxidants

Protective Agents

Cyclooxygenase Inhibitors

Enzyme Inhibitors

200 mg SPI-1005, capsule, po, bid, x3d surrounding each cycle of chemotherapy

400 mg SPI-1005, capsule, po, bid, x3d surrounding each cycle of chemotherapy

600 mg SPI-1005, capsule, po, bid, x3d surrounding each cycle of chemotherapy

0 mg SPI-1005, capsule, po, bid, x3d surrounding each cycle of chemotherapy

Oral capsules, 200 mg ebselen, twice daily, 3 days for each cycle of chemotherapy

Arms: Low Dose

Other Names:

200 mg Ebselen

SPI-1005 Low Dose

Oral capsules, 400 mg ebselen, twice daily, 3 days for each cycle of chemotherapy

SPI-1005 Middle Dose

Oral capsules, 600 mg ebselen, twice daily, 3 days for each cycle of chemotherapy

SPI-1005 High Dose

Oral capsules, 0 mg ebselen, twice daily, 3 days for each cycle of chemotherapy

Placebo

Eric Lynch, PhD

Tony S. Quang, M.D.

Tony S Quang, M.D.

Jonathan Kil, MD

Chemotherapy treatment with platinum based agents is well noted to cause ototoxicity. It is the objective of this study to determine the safety and efficacy of SPI-1005 at three dose levels when delivered orally twice daily for 3 days, surrounding each cycle of platinum chemotherapy in head and neck or non-small cell lung cancer patients to prevent and treat chemotherapy induced hearing loss and tinnitus.

Chemotherapy treatment with the platinum containing chemotherapies (e.g. cisplatin, carboplatin) are well noted and studied for their ability to cause ototoxicity which includes hearing loss, tinnitus, vertigo, or dizziness. It is the objective of this study to determine the safety and efficacy of SPI-1005 at three dose levels when delivered orally twice daily for 3 days, surrounding each cycle of platinum chemotherapy for head and neck or non-small cell lung cancer patients to prevent and treat chemotherapy induced hearing loss and tinnitus.

SPI-1005, a proprietary oral formulation of ebselen is a small molecule mimic and inducer of the enzyme Glutathione Peroxidase. GPx reduces reactive oxygen species (ROS) by reacting with glutathione. SPI-1005 has been shown to reduce cisplatin induced hearing threshold shift in animal studies.

SPI-1005 for Prevention and Treatment of Chemotherapy Induced Hearing Loss

Safety and Efficacy Study of SPI-1005 for Prevention of Chemotherapy Induced Hearing Loss

VA Puget Sound Health Care System

Sound Pharmaceuticals, Incorporated

Hearing Loss, Sensorineural

Anti-Infective Agents

Respiratory System Agents

Amino Acids

Acetylcysteine

N-monoacetylcystine

Antiviral Agents

Expectorants

Antidotes

Cysteine

One ear will be randomly chosen for the experimental treatment and receive intratympanic NAC injections 60 minutes prior to their scheduled chemotherapy sessions

The control ear will not receive any injections

One ear will be randomly chosen for the experimental treatment of intratympanic NAC. The other ear will serve as the control ear which will not receive any injection.

N-Acetyl Cysteine

Varia Sajeniouk

Trung N Le

Although many intratympanic agents have been attempted, N-Acetylcysteine (NAC) appears to be the most promising and is a powerful, commonly used anti-oxidant. The goal of this prospective phase 2 randomized controlled trial is to determine the optimal dosage and effectiveness of intratympanic NAC injection in reducing hearing loss in head \& neck cancer patients receiving cisplatin chemotherapy with curative intent.

A prospective randomized controlled trial phase 1 using a standard 3+3 design, followed by a randomized controlled phase 2 to determine dosage, safety, and efficacy of intratympanic NAC to treat hearing loss in head \& neck patients receiving high-dose cisplatin chemotherapy. Participants will complete various pre-treatment hearing tests. One ear will be randomly chosen for the experimental treatment and the other ear will serve as the control ear. Participants will receive intratympanic NAC injections 60 minutes prior to their scheduled chemotherapy sessions in the experimental ear. The control ear will not receive any injection. Follow up hearing tests will be performed 2 months following completion of their primary cancer treatment.

Intratympanic N-Acetylcysteine for Prevention of Cisplatin-induced Ototoxicity.

Intratympanic N-Acetylcysteine (NAC) Injections for Prevention of Cisplatin-induced Ototoxicity in Head and Neck Cancer Patients: A Multi-centre Phase II Randomized Controlled Trial.

The primary end-point of the phase 1 part of this study is the safety and tolerability of intratympanic NAC injections, by determining the causality of adverse events and serious adverse events and grading according to National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE) v.5.0

The primary outcome measure of this study will be a comparison of hearing loss between the control and NAC-injected ears. More specifically, each ear will be scored as having hearing loss if there is a greater than 10dB hearing loss in three contiguous frequencies. This will be measured via threshold hearing tests.

The secondary outcome measure of this study will be a comparison of hearing discrimination, subjective tinnitus, otoacoustic emission, speech spatial and quality of hearing between the control and NAC-injected ears.

Toronto Sunnybrook Regional Cancer Centre

London Regional Cancer Program, Canada

Sunnybrook Health Sciences Centre

Trung N. Le MD PhD

All subjects will be part of a single arm involving placement of the Nucleus ABI541 Auditory Brainstem Implant (ABI) device. The Nucleus 24 was discontinued and is no longer available.

Daniel J Lee, MD, FACS

The purpose of this research study is to determine whether Auditory Brainstem Implant (ABI) can improve hearing in persons who are deaf in both ears and are not candidates for cochlear implants.

The goal of this MEEI Auditory Brainstem Implant (ABI) research study is to find new ways to improve hearing in patients who are deaf and cannot receive a cochlear implant. The ABI is a surgically placed bionic implant that converts sounds into electrical signals that are directly transmitted to the cochlear nucleus, the first auditory center of the brain. For many years, ABIs have improved the hearing of patients who are deaf due to brain tumors associated with a genetic syndrome called Neurofibromatosis Type 2 (NF2). However, a number of recent studies suggest that deaf patients who do not have NF2 and are not eligible for a cochlear implant may also benefit from placement of an ABI. These preliminary studies suggest that these non-NF2 or "nontumor" patients may actually have better outcomes after ABI surgery than patients suffering from NF2. Patients who do not have NF2 and are deaf due to damage to the hearing nerves or inner ears from infection, disease or injury are not cochlear implant candidates and there are no other options to improve hearing in these cases except for the ABI. Thus, the purpose of our study is to carefully analyze whether ABI surgery improves the hearing and quality of life of non-NF2 patients based on subjective and objective measures of their hearing before and after ABI surgery. In particular, we plan to study ABI outcomes in non-NF2 patients, characterize the parameters used on their devices, and determine the safety profile of ABIs in these patients.

Auditory Brainstem Implant (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects

Study of Nucleus 24 and ABI541 Auditory Brainstem Implants (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects

Average pure tone threshold measured 12 months post-operatively. Measured in dB HL, where a lower threshold indicates more sensitive hearing and a higher threshold indicates less sensitive hearing.

We only included data from subjects that had auditory percept of any magnitude following ABI surgery (1/3 subjects).

Director of Auditory Brainstem Implant Progra, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary

Placement of an Auditory Brainstem Implant (ABI) device

Pure Tone Threshold Average (dB)

We only included data from subjects that had auditory percept of any magnitude following ABI surgery (1/3 subjects).

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Auditory Brainstem Implant (ABI) in Pediatric Non-Neurofibromatosis Type 2 Subjects	NCT01864291	Bilateral Heari...	Nucleus 24 (dis...	18 Months - 17 Years	Massachusetts Eye and Ear Infirmary
Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves	NCT02310399	Hearing Loss, B...	Nucleus ABI541 ...	18 Months - 21 Years	NYU Langone Health
Intraoperative Electrically-evoked ABRs in Patients Undergoing Vestibular Schwannoma Surgery	NCT05248113	Deafness	Electrical stim...	18 Years - 99 Years	University of California, Irvine
SPI-1005 for Prevention and Treatment of Chemotherapy Induced Hearing Loss	NCT01451853	Lung Cancer Head and Neck C... Hearing Loss Ototoxicity Tinnitus Neuropathy	SPI-1005 Low Do... SPI-1005 Middle... SPI-1005 High D... Placebo	19 Years - 80 Years	Sound Pharmaceuticals, Incorporated
Auditory Brainstem Implant (ABI) in Children With No Cochleae or Auditory Nerves	NCT02310399	Hearing Loss, B...	Nucleus ABI541 ...	18 Months - 21 Years	NYU Langone Health
Intratympanic N-Acetylcysteine for Prevention of Cisplatin-induced Ototoxicity.	NCT04291209	Ototoxic Hearin...	N-Acetyl Cystei...	18 Years -	Sunnybrook Health Sciences Centre
Auditory Brainstem Implant (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects	NCT01736267	Bilateral Heari...	Nucleus 24 (dis...	18 Years -	Massachusetts Eye and Ear Infirmary
Endolymphatic Sac Tumors in a Population of Patients With Von Hippel-Lindau Disease:The Natural History and Pathobiology, and a Prospective Non-Randomized Clinical Trial of Hearing Preservation Surgery in Patients With Early Stage Endolymphatic Sac Tumors	NCT00001668	Deafness Kidney Diseases Kidney Neoplasm... Neoplasms Retinal Disease...		-	National Institutes of Health Clinical Center (CC)

Spots Global Cancer Trial Database for deafness

Every month we try and update this database with for deafness cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Spots Global Cancer Trial Database for deafness

Every month we try and update this database with for deafness cancer trials from around the world to help patients and their families find trials that might be right for them. We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.

Every month we try and update this database with for deafness cancer trials from around the world to help patients and their families find trials that might be right for them.
We offer this 100% free of charge and do not endorse any of the trials listed here, we hope it helps you or a loved one.