Science Current Events | Science News |

For First Time, Cochlear Implant Restores Hearing To Patient With Rare Genetic Disorder

June 08, 2007

Washington, D.C.--Researchers at Georgetown University Medical Center and the National Institutes of Health (NIH) have, for the first time, used a "bionic" ear to restore hearing in a patient with von Hippel-Lindau disease. They say this advance offers new hope for individuals with the rare disorder, which can produce non-malignant tumors in ears, as well as in the eyes, brain, and kidneys.

The advance was possible, researchers say, because their years of research into the disease showed that these tumors do not affect the cochlear nerve necessary for receipt of sound in the brain. The device they used is known as a cochlear implant, which stimulates the cochlear nerve with electrical impulses. It is predominately used to treat the deaf.

"Based on our understanding of how these tumors affect the inner ear, we felt that a cochlear implant could work, and it did," said the study's lead author, H. Jeffrey Kim, M.D., an assistant professor in the Department of Otolaryngology--Head and Neck Surgery, and a part-time investigator at the NIH, where the surgery was performed. Two years after the surgery, the implant has significantly improved the quality of life of the patient, he said.

Based on this successful surgery, which was published as a case report in the May issue of the journal Otology & Neurology, patients with von Hippel-Lindau disease with hearing loss may be now be candidates for a cochlear implant, Kim said. The disease, caused by inheritance of a mutated tumor suppressor gene, occurs in 1 out of 36,000 live births, and about 30 percent of these patients develop tumors in their ears--often in both. To date, the only option to help control these tumors is repeated surgery, which is often not successful, he said. Loss of hearing is sudden, and hearing aids don't help, Kim said.

These tumors occur in the endolymphatic sac, part of the inner ear labyrinth of fluid passages. They are benign, but are invasive, and can cause hemorrhages that lead to tinnitus, vertigo, and hearing loss. Kim and his colleagues have been following a population of patients with the disorder and are national leaders in characterizing the disorder's effect on the ears. They have published a series of findings in such journals as the New England Journal of Medicine (NEJM) and the Journal of the American Medical Association (JAMA).

Kim's research also sheds light on other ear problems, including Ménière's Disease, a disorder of the inner ear that can affect hearing and balance due to pressure in the same endolymphatic sacs. "This is a much more common condition, so we hope that what we learn from von Hippel-Lindau disease may help in the treatment of hearing problems that affect many of us," he said.

Georgetown University Medical Center

Related Cochlear Implant Current Events and Cochlear Implant News Articles

Engineering music to sound better with cochlear implants
When hearing loss becomes so severe that hearing aids no longer help, a cochlear implant not only amplifies sounds but also lets people hear speech clearly.

Study of cognitive development in deaf children revisits longstanding debate
A team of researchers at the University of Connecticut is reexamining a decades-long debate as to whether deaf children should learn sign language to maximize their potential for optimal development.

New computer program predicts cochlear implant success in hearing-impaired children
A new computer program that analyzes functional brain MRIs of hearing impaired children can predict whether they will develop effective language skills within two years of cochlear implant surgery, according to a study in the journal Brain and Behavior.

Early evidence suggests hybrid cochlear implants may benefit millions with common form of hearing loss
People with a common form of hearing loss not helped by hearing aids achieved significant and sometimes profound improvements in their hearing and understanding of speech with hybrid cochlear implant devices, according to a new multicenter study led by specialists at NYU Langone Medical Center.

Experts map surgical approaches for auditory brainstem implantation
A technique called auditory brainstem implantation can restore hearing for patients who can't benefit from cochlear implants.

Study: Infants Benefit from Implants with More Frequency Sounds
A new study from a UT Dallas researcher demonstrates the importance of considering developmental differences when creating programs for cochlear implants in infants.

Design prototype chip makes possible a fully implantable cochlear implant
Researchers from Massachusetts Eye and Ear, Harvard Medical School, and Massachusetts Institute of Technology (MIT) have designed a prototype system-on-chip (SoC) that could make possible a fully implanted cochlear implant.

Cochlear implants -- with no exterior hardware
Cochlear implants - medical devices that electrically stimulate the auditory nerve - have granted at least limited hearing to hundreds of thousands of people worldwide who otherwise would be totally deaf.

New strategy lets cochlear implant users hear music
For many, music is a universal language that unites people when words cannot. But for those who use cochlear implants - technology that allows deaf and hard of hearing people to comprehend speech - hearing music remains extremely challenging.

New technology will improve neuron activation induced by cochlear implants
Cochlear implants, electrical prosthetic devices that stimulate inner ear neurons of individuals who have lost their cochlear sensory cells, restore usable hearing to deaf patients.
More Cochlear Implant Current Events and Cochlear Implant News Articles

Cochlear Implant Patient Assessment: Evaluation of Candidacy, Performance, and Outcomes (Core Clinical Concepts in Audiology)

Cochlear Implant Patient Assessment: Evaluation of Candidacy, Performance, and Outcomes (Core Clinical Concepts in Audiology)
by Rene H. Gifford (Author)

Cochlear Implant Patient Assessment is an invaluable clinical reference for every cochlear implant audiologist and speech-language pathologist working with the adult or pediatric populations. This unique text is designed to provide information on best practices assessment of implant candidacy as well as the postoperative assessment of performance over the long term. This book is written for audiologists, speech-language pathologists and deaf educators to serve as a clinical handbook on the assessment tools and therapeutic intervention that are critical during the pre- and post-implant periods. Given that the numbers of both adult and pediatric cochlear implant recipients continue to increase, more and more clinicians will be expected to gain and maintain a level of experience surrounding...

Kylie Gets a Cochlear Implant

Kylie Gets a Cochlear Implant
by Marilyn C. Rose (Author)

Kylie Gets a Cochlear Implant is the heartwarming story of a little girl who loses her hearing and is heartbroken about the things she believes she can no longer do. Kylie loves to dance, but fears that without hearing the music, she won't be able to feel the beat or the rhythm. She is very sad until she hears about a way to overcome her hearing loss. See what happens when Kylie undergoes a cochlear implant, which is a surgically implanted device that can help her hear sound and music. In fact, cochlear implants can be so successful that they are often called bionic ears! Written by a teacher who works with deaf children, this story shows insight into what these brave children face. Marilyn C. Rose finished her Ph.D. at the same time she was writing this book. A graduate of Hunter...

Sunny and her Cochlear Implants

Sunny and her Cochlear Implants
by Susanna Dussling (Author)

Follow Sunny as she lives her life as a deaf child, in a hearing world, with hearing aids. Then, her life changes when her aids no longer help - but there is a solution. Sunny is about hope, acceptance and a positive "can do" attitude. This book is the first in a new series. In future books, follow Sunny's adventures as she uses special tools such as a bed-shaker alarm clock, flashing telephone signaler, etc. to live her life.

Programming Cochlear Implants (Core Clinical Concepts in Audiology)

Programming Cochlear Implants (Core Clinical Concepts in Audiology)
by Jace Wolfe (Author), Erin Schafer (Author)

Cochlear implants offer significant benefits for children and adults with severe to profound hearing loss; however, to realize these benefits the device must be carefully and correctly programmed. With current information on cochlear implant technology, Programming Cochlear Implants, Second Edition - a volume in the Core Clinical Concepts in Audiology Series - is a valuable guide for clinicians providing services to cochlear implant users or as a teaching tool for graduate-level students. Programming Cochlear Implants, Second Edition introduces the basics of cochlear implant hardware and programming and continues through advanced programming techniques, with manufacturer-specific information and case studies. The text reviews clinical protocols for cochlear implant management; programming...

Cochlear Implants: Evolving Perspectives

Cochlear Implants: Evolving Perspectives
by Raylene Paludneviciene (Editor), Irene W. Leigh (Editor)

The cochlear implant debate has changed, as evidenced in this cogent collection that presents 13 chapters by 20 experts, including several who communicate through sign language but also utilize cochlear implants. The impetus for this change stems from recognition that both visual and aural input can enhance the education of deaf children.Divided into four sections, Cochlear Implants: Evolving Perspectives first focuses on the impact of implants in the Deaf community. Chapters in this section examine the issues driving the cochlear implant debate, the ethics of genetic engineering, experiences of implanted adult deaf signers, reflections of deaf mothers who have had their children implanted, and the effects of implants on deaf identity. The second section delves into the mechanics of...

Cochlear Implants

Cochlear Implants
by Susan B. Waltzman (Editor), J. Thomas Roland (Editor)

Thieme congratulates author Dr. J. Thomas Roland, Jr. for being chosen by New York magazine for its prestigious ‘Best Doctors 2015’ list.
Praise for the previous edition:
"Overall, the second edition of Cochlear Implants is an excellent resource for professionals and students in various disciplines (otolaryngology, audiology, education of the deaf, and basic sciences) involved with the care or understanding of hearing loss. This edition serves its purpose for learning and reference in a condensed textbook that will be well used." -- The Laryngoscope
Cochlear Implants, Third Edition, has been completely revised to include the most up-to-date information on the clinical and translational sciences related to this rapidly evolving technology. It contains chapters on the...

Made to Hear: Cochlear Implants and Raising Deaf Children (A Quadrant Book)

Made to Hear: Cochlear Implants and Raising Deaf Children (A Quadrant Book)
by Laura Mauldin (Author)

A mother whose child has had a cochlear implant tells Laura Mauldin why enrollment in the sign language program at her daughter’s school is plummeting: “The majority of parents want their kids to talk.” Some parents, however, feel very differently, because “curing” deafness with cochlear implants is uncertain, difficult, and freighted with judgment about what is normal, acceptable, and right. Made to Hear sensitively and thoroughly considers the structure and culture of the systems we have built to make deaf children hear.Based on accounts of and interviews with families who adopt the cochlear implant for their deaf children, this book describes the experiences of mothers as they navigate the health care system, their interactions with the professionals who work with them, and...

Cochlear Implant Research Advances

Cochlear Implant Research Advances
by David Crow (Editor)

This pioneering book consists of research-focused information in respect of cochlear implant. Cochlear implant has been a much sought after field of research for a long time and encompasses various disciplines including surgery, engineering, audiology etc. With growing research in this genre, a better understanding of surgical methods, pre and post-surgery processes, implant technology and other aspects has developed. While a few better technologies have emerged over time, more are expected to come up. This book aims at providing all inclusive information regarding cochlear implantation and hence, covers a wide variety of topics ranging from surgical issues to sound processing strategies. This book presents latest and updated information which is valuable to students, researchers and...

Cochlear Implants: Principles and Practices

Cochlear Implants: Principles and Practices
by John K. Niparko MD (Editor)

Thoroughly updated for its Second Edition, this book provides an in-depth discussion on prosthetic restoration of hearing via implantation. The text succinctly discusses the scientific principles behind cochlear implants, examines the latest technology, and offers practical advice on how to assess candidates, how to implant the devices, and what rehabilitation is most effective. The authors thoroughly examine the outcomes of cochlear implantation, the impact on the patient's quality of life, the benefits in relation to the costs, and the implications of cochlear implants for language and speech acquisition and childhood education.

Cochlear Implants: What Parents Should Know

Cochlear Implants: What Parents Should Know
by Betty Loy (Author), Peter Roland (Author)

Jargon-free and comprehensive in coverage, this handbook has been written by members of the Dallas Cochlear Implant Program to serve the needs of parents considering CI implantation for their children. Their rationale in writing the book was to ensure that parents had a reliable information source to supplement advices from medical and allied health professionals, to help enable them to make informed decisions.

From surgical considerations, through insurance and lifestyle issues, to counseling, this book is an ideal patient hand-out, or should at the very least be recommended as a primary source of information. It further meets the needs of medical and allied health professionals in the communication sciences in understanding and being able to serve the information needs of...

© 2017