Doctors find cochlear implants restore hearing in rare disorder

Clinical-researchers from University Hospitals (UH) Case Medical Center report that cochlear implantation provides an effective and safe way of restoring hearing in patients with far advanced otosclerosis (FAO), a hereditary condition that can lead to severe hearing loss.

"This is the first study to demonstrate that cochlear implants provide robust and long-term restoration for patients with FAO," said lead author Maroun T. Semaan, M.D., an with UH Case Medical Center and an Assistant Professor at Case Western Reserve University School of Medicine. "This is an important new treatment option for this challenging group of patients."

FAO causes abnormal growth of bone in the middle and inner ear. This bone prevents structures within the ear from working properly and it diminishes hearing. Therapies have included fluoride pills to potentially prevent development of hearing loss, but they will not improve hearing loss that has already developed. Hearing aids are commonly prescribed for patients with FAO. Surgery is an option with an operation called a stapedectomy, or stapedotomy, where an otologist (ear surgeon) bypasses the diseased bone with a that allows sound waves to be passed to the . However, in cases with advanced disease, stapedectomy surgery is not effective in restoring hearing, and the patient is essentially deaf, even with the strongest hearing aids.

The authors studied the records of 30 patients with FAO with age-matched controls who suffered from from other causes besides FAO. The researchers found that previous concerns about possible complications thought to potentially affect cochlear implantation in FAO patients were not seen using newer surgical techniques.

Cliff A. Megerian, MD, senior author on the study, noted, "Although a significant percentage of these patients required the additional operative step of 'round window drill out,' this finding in no way diminished the excellent hearing outcomes enjoyed using cochlear implantation. In addition, the study showed that the presence of abnormalities on radiographic imaging did not affect hearing following implantation." Dr. Megerian is Director, Otology, Neurotology, and , UH Case Medical Center, and a Professor at Case Western Reserve University School of Medicine.

The findings were presented at the115th Annual Meeting of the Triological Society, the most prestigious meeting for ear, nose and throat specialists. Gail S. Murray, PhD, Director of Audiology Services at UH Case Medical Center, was also an author on the paper.

Related Stories

Ear tubes appear safe for children with cochlear implants

date Jun 21, 2010

A history of ear tubes to treat infections does not appear to adversely affect children with cochlear implants, regardless of whether the tubes are left in place or removed before implantation, according to a report in the ...

Can you hear me now? Stem cells enhance hearing recovery

date Jun 25, 2007

Tokyo, Japan -- Researchers have shown that bone marrow stem cells injected into a damaged inner ear can speed hearing recovery after partial hearing loss. The related report by Kamiya et al, “Mesenchymal stem cell transplantation ...

Cochlear implants slightly less beneficial in older patients

date May 17, 2010

Older adults appear to benefit significantly from cochlear implants, but not as much as younger patients who had similar levels of hearing impairment before surgery, according to a new study by researchers at The Medical ...

Recommended for you

A high-fat diet may alleviate mitochondrial disease

date 5 hours ago

Mice that have a genetic version of mitochondrial disease can easily be mistaken for much older animals by the time they are nine months old: they have thinning grey hair, osteoporosis, poor hearing, infertility, ...

Cheek muscles hold up better than leg muscles in space

date 5 hours ago

It is well known that muscles need resistance (gravity) to maintain optimal health, and when they do not have this resistance, they deteriorate. A new report published in the July 2015 issue of The FASEB Journal, however, sugges ...

Sialic acid: A key to unlocking brain disorders

date 8 hours ago

A new report published in the July 2015 issue of The FASEB Journal suggests that a common molecule found in higher animals, including humans, affects brain structure. This molecule may play a significant role in how brain ...

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.