Richard D. Kopke, M.D., FACS, medical director of the INTEGRIS Cochlear Implant Clinic and CEO of the Hough Ear Institute

Q: What is nerve deafness?
A: Nerve deafness involves the loss of the tiny sensory hair cells and nerve endings in the inner ear organ for hearing known as the cochlea. It is the most common form of deafness and the most difficult to treat. The other, less common type of hearing loss is known as conductive hearing loss. It is caused by a problem with the ear canal, ear drum or tiny bones of hearing, and is often treated with surgery.

Q: Does it affect people of all ages or does it develop later in life?
A: Nerve deafness affects people of all ages. Three of 1,000 babies born in Oklahoma have significant nerve deafness, and workplace noise places 30 million workers in the U.S. at risk for noise-induced deafness. One of three of our senior citizens older than 65 and 50 percent of our seniors older than 75 have some degree of nerve deafness. The economic impact on the U.S. is about $56 billion annually.

Q: What causes nerve deafness?
A: In adults, the aging process, loud noise exposure and hereditary genetic problems are common causes. In children, most common causes are hereditary genetic problems, followed by problems during birth, or severe infections in childhood such as meningitis, or the virus known as cytomegalovirus. Serious or life-threatening problems such as a brain tumor can also be the cause of the hearing loss.

Q: How does nerve deafness impact people?
A: Adults with nerve deafness retire five to six years before normal-hearing peers on average. Seniors with untreated nerve deafness are more prone to depression, anxiety, social isolation and family problems. Children have difficulty with speech, language and communication ability, which can impact education, social development and career opportunities.

Q: How should nerve deafness be evaluated?
A: Fortunately, state-mandated newborn hearing screenings identify most babies with hearing loss at birth. Hearing testing by an audiologist is important to identify the type and degree of hearing loss. It is also important that a medical doctor who is an ear specialist evaluate both children and adults to look for the cause of the hearing loss and rule out other serious disorders. Genetic testing, which is now more widely available, can be very helpful in children. CT or MRI is also very important and helpful. At times, blood, urine or EKG testing as well as an eye exam are important to rule out other serious health problems associated with childhood deafness. While aging and noise are common causes of nerve deafness in adults, a thorough medical evaluation is important as well to rule out other serious conditions that may be the cause of hearing loss.

Q: How can nerve deafness be treated?
A: In general, there is no “cure” for nerve deafness. Once the hair cells and nerve endings have been lost, they cannot be restored and hearing loss is permanent. An important exception is a condition known as sudden sensorineural hearing loss, in which a person may suddenly develop nerve deafness. These cases can be successfully treated with steroids and other medications if the patient is seen within a day or two. Hearing aids are the most commonly prescribed treatment for nerve deafness. Hearing aid technology has improved tremendously. They are more comfortable, more discreet, more effective, and deliver more patient satisfaction compared with 10 years ago. Cochlear implant technology is exploding in such a way that babies, most senior citizens and people of all ages with the most severe hearing loss can now hear well.

Q: What is the latest research on the treatment of nerve deafness?
A:There are four exciting advancements to mention:

1. Cochlear implant technology is expanding and has significant advantages for the use of implants in both ears. New devices using both the electrical stimulation of the cochlear implant, along with the amplifi ed sound energy of a conventional hearing aid, are showing great promise in preliminary studies.
2. For the first time, an antioxidant nutritional supplement has been shown to be effective in decreasing sudden nerve deafness due to loud noise. This may have wider application for other causes of nerve deafness as well.
3. Continuing research on nanotechnology shows promise in finding new ways to effectively target medicine right to the inner ear, where it's needed most.
4. Hair cell regeneration with restoration of hearing may one day be possible. Recent experiments have shown that hair cell regeneration with recovery from deafness was possible in guinea pigs. We are trying to discover ways to make this technology safe and effective for humans. That might allow many people with chronic nerve deafness to recover some useful hearing. Our hope is to cure deafness in this generation.