Severe Hearing Loss

What Is Severe Hearing Loss?

Severe hearing loss means you can hear some sounds, but very poorly. You may not be able to hear someone speaking, even if they are using a normal voice. You may be able to hear only very loud sounds.

Hearing loss can happen in many different ways to people of all ages. It’s different for everyone. The key is to work with your health care team to find ways help you make the most of the hearing you have. There are many treatments that can help you or your child enjoy life.

Causes

Babies can be born with severe hearing loss, and children and adults can get it at any point in their lives. It can happen suddenly or over many years, in one or both ears, and be brief or long-lasting.

To understand how hearing loss happens, it helps to know how your ear works. Noise travels through the air as sound waves, which vibrate your eardrum and move three tiny bones inside your ear. That causes waves in the fluid that fills your inner ear. Those waves bend tiny hair cells, which are attached to nerves. They pass electrical signals to the main hearing nerve, called the cochlear nerve, which leads to the brain.

Your DNA has many genes that help build the structures involved in hearing. A problem with any of them can mean a baby is born without this sense. Over half of babies born with severe hearing loss, it’s because of a faulty gene. About 20% of babies born with it also have another genetic condition, like Down syndrome.

Babies also can lose their hearing because of a problem in the womb. Pregnant women who take certain medicines, such as the cancer drug thalidomide or drugs for tuberculosis, may have a baby with severe hearing loss. It can also happen if a woman has certain infections, like cytomegalovirus.

You also may lose your hearing as you get older. It can happen because of:

  • Noise. A single very loud noise, like a gunshot or explosion, can damage hearing. So can being around loud noises for a long time, like living next to an airport runway.
  • Diseases. Different conditions can put the ears or nerves involved in hearing at risk, including ear infections, brain tumors, rheumatoid arthritis and other autoimmune diseases, or Meniere’s disease, a disorder of the inner ear.
  • Clogs. Ear canals stopped up with earwax or an object stuck inside can keep you from hearing well. You can also damage your ear if you try to remove it the wrong way.
  • Injury. Head trauma can damage the inside of the ears. So can some sports, like scuba diving or sky diving.
  • Medications. Certain drugs, including some that treat cancer, heart disease, and serious infections, can damage your ear and cause hearing loss. Sometimes, it’s permanent, but in other cases, the problem goes away after you stop taking the medicines.

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Symptoms

If your child has trouble hearing, you’ll probably notice it by the way she behaves. Some signs include:

  • A hard time learning to speak, or speaking later than other children her age
  • Not paying attention to sounds or when people call her
  • Poor school work

If hearing loss is affecting you, you may notice that you have a hard time interacting with others because you can’t hear them. Signs include:

  • Trouble hearing people speak in groups or in noisy places
  • Can’t hear someone behind you speaking
  • Think other people are mumbling when they are speaking
  • Trouble hearing people on the phone
  • Listen to the television or car radio at a very loud volume
  • Don’t hear the alarm clock

Getting a Diagnosis

It’s important to diagnose severe hearing loss as early as possible, especially for children. In some states, laws require medical staff to screen newborns for hearing before they leave the hospital. If your baby doesn’t get a test, ask hospital staff where you can get one.

There are two types of newborn hearing tests:

  • Automated auditory brain response. Medical staff will put soft earphones on your baby’s ears and sensors on her head. A machine measures her hearing nerve’s response to soft clicks or tones.
  • Otoacoustic emissions. A tiny probe inside your baby’s ear canal measures the echo from soft noises played into her ears.

If your child shows any symptoms of severe hearing loss as she gets older, talk to your pediatrician. If you have trouble hearing, you can talk to your doctor. Testing is usually about the same for children and adults.

Your doctor may refer you to a hearing specialist called an otolaryngologist or otologist. She’ll ask about your medical history and do a physical exam of your ears. She may ask you:

  • Do you often feel as if people are speaking too quietly or mumbling?
  • How hard is it to hear someone speak in a loud or noisy place?
  • How long have you noticed a problem?
  • Do you have any other medical conditions?
  • Have any members of your family had hearing loss?

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For a child with hearing problems, the doctor may ask you:

  • How does your child respond when you call her name or make sounds at home?
  • When did she start talking?
  • Was there ever a time when she was around very loud noises?
  • Has she ever had an accident that hurt her head?

The doctor may tell you to see an audiologist, a professional trained in treating hearing problems. He can measure how much hearing you or your child has lost using different tests.

  • Pure tone audiometry. You’ll sit in a soundproof booth and wear headphones and a special headband. The audiologist will play different pitches of sound and ask you what you can hear.
  • Speech audiometry. Also in the booth with headphones, you’ll hear different words at different volumes and repeat them to the audiologist. The test measures how softly and how clearly you can understand speech.
  • Transtympanic electrocochleography (ECOG). You’ll lie down in the sound booth, and the audiologist will put a recording sensor in your ear. It measures the electric signals made by the nerves in your inner ear in response to sound.

For a child with hearing loss, the audiologist may want to see how well she responds to instructions. He may tell her how to play a game to see how she understands speech. He may ask her to look at the sources of sounds.

Questions to Ask Your Doctor

If you have severe hearing loss, you’ll want to ask your doctor questions about your condition, like:

  • What caused my hearing loss?
  • Will it go away?
  • Do I need to see any other doctors?
  • What kind of treatments are there?
  • Will they cure my hearing loss?

If your child has severe hearing loss, you’ll also want to ask:

  • What will my child need at school to deal with hearing loss?
  • What can we do for her at home?
  • How can she learn to speak?
  • Will my other children have hearing loss?
  • Will the hearing loss continue to get worse?

 

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Treatment

Treatment for severe hearing loss usually means using different technology to improve the hearing you have. There’s no single therapy that’s best for every person with the condition. Your doctor will recommend one based on how much hearing you’ve lost, how healthy you are, your lifestyle, and how your ears were damaged.

Your treatment options include:

Hearing aids. They make low sounds louder or easier to hear. Some may help cut background noise.

Some hearing aids fit inside the ears. They can be so small that other people may not notice them. Others have clips that fit over the top of the ears to hold them steady. You can take them out when it’s time to sleep, swim, or shower.

Extended-wear hearing aids are made of soft material. An audiologist will place them in your or your child’s ears, and they can stay there for months. People who are active may wear them while playing sports or swimming.

Implants. Middle ear implants are devices that vibrate inside your ear. Your doctor will place them there for you. You can use them for long periods of time.

Cochlear implants help people who have such severe hearing loss that hearing aids don’t help. They trigger the nerves inside the ears. They don’t cure hearing loss, but they can give children and adults the sensation of sound.

In the hospital, a surgeon places the cochlear implant inside the ear, tiny electrodes next to your cochlear nerve, and a receiver right under the skin behind your ear. About 4 weeks later, you’ll see the doctor again to get the outside parts of the implant, including a microphone, a transmitter, and a small computer called a speech processor. These parts send signals to the device in your ear to translate the sounds around you. You can wear them behind your ear like a hearing aid.

It can take a long time after the surgery to get cochlear implants to work best for you or your child. You’ll need support from hearing specialists and language therapists to learn how to use the device and respond to the sounds you can hear. The process takes a lot of time and effort. Your doctor will help you decide if you or your child are a good fit for this therapy.

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Taking Care of Yourself or Your Child

It can be hard to lose your hearing, whether suddenly or over many years. If your child has severe hearing loss, you may worry about the parts of life that she’ll miss. Remember that there are many things that can help people of all ages with the condition enjoy life. As you work with your doctor to pick a treatment plan, you can take steps to make life easier for you or your child.

For your child:

  • Therapy can help your child learn to use a hearing aid and find ways to communicate. A family or a speech therapist can teach her to say words clearly so others understand. She can also learn how to use other techniques, including sign language, natural gestures, and speech reading. A therapist can help you decide which approach is right for your child and how your family can help.
  • Talk to your child’s school and see what they can do to help her in the classroom. She may be able to use HATS, or Hearing Assistive Technology Systems, small FM radio frequency devices that make sounds easier to hear. Teachers speak into a special microphone that sends sounds to a small receiver that your child uses to hear the lessons.

For yourself:

  • Work with an audiologist to learn ways to deal with noisy places or group conversations.
  • Use HATS that make your alarms, phone, or television easier to hear. Some devices can let you know when someone is ringing the doorbell.
  • Style your home to make sounds easier to hear. Place carpet or rugs on floors to cut down on noise. Arrange chairs so you can sit across from your friends during visits.

It may help to talk to a therapist or counselor to deal with any worry or sadness you or your child may feel. Also, support groups of other families dealing with severe hearing loss are great places to get advice and understanding.

What to Expect

Severe hearing loss is different for everyone. It may affect you or your child for many years. But it doesn’t have to keep you from enjoying life. You should be able to live independently, go to school or college, and have a career with severe hearing loss. Work with your health care team to find treatments and strategies that work for you.

It’s important to diagnose and treat severe hearing loss as soon as possible. The sooner you or your child can begin therapy, the better you’ll be able to adapt.

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Getting Support

Connect with others who are living with severe hearing loss or get helpful information from patient groups like the Hearing Loss Association of America.

WebMD Medical Reference Reviewed by Melinda Ratini, DO, MS on December 06, 2016

Sources

SOURCES:

CDC: “Types of Hearing Loss” and “Hearing Loss in Children: Data and Statistics.”

American Speech-Language Hearing Association: “Hearing Aids, Cochlear Implants and Assistive Technology;” “Audiologic Rehabilitation;” “FM Systems;” and "Degree of Hearing Loss."

Rehabilitation Engineering Research Center on Hearing Enhancement: “Dr. Ross on Hearing Loss: Understanding and Managing a Severe Hearing Loss.”

Merck Manuals: “Evaluation of Hearing Loss” and “Drug-Induced Ototoxicity.”

Hearing Loss Association of America: “Symptoms of Hearing Loss;” “Diagnosing Hearing Loss;” and "Living With Hearing Loss.”

First Signs Organization: “Audiological Screening.”

American Speech Language Hearing Association: “Untreated Hearing Loss in Adults.”

American Hearing Research Foundation: "Congenital Deafness."

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