SEVERE HEARING LOSS

Severe hearing loss is when you struggle to hear most sounds, even if they’re loud. It’s like having the volume turned way down on the world around you. Conversations may be a challenge, even if the speaker is close by and speaking clearly.

Hearing loss can happen to anyone, at any age, and it varies from person to person. The important thing is to team up with your healthcare providers to explore ways to make the most out of your remaining hearing ability. Fortunately, there are various treatments available that can significantly improve your quality of life, whether it’s for yourself or your child.

Causes of severe hearing loss

Severe hearing loss can occur in babies, children, and adults, either from birth or at any stage of life. It can manifest suddenly or gradually over time, affecting one or both ears and persisting for varying durations.

Understanding how hearing loss develops requires insight into the workings of the ear. Sound waves travel through the air, vibrating the eardrum and stimulating three small bones inside the ear. These vibrations create waves in the fluid within the inner ear, which in turn bend tiny hair cells connected to nerves. These hair cells transmit electrical signals to the auditory nerve, which then sends information to the brain.

Genetic factors play a significant role in hearing loss. Certain genes involved in building the auditory structures may be defective, resulting in congenital hearing impairment. In fact, more than half of babies born with severe hearing loss have a genetic mutation. Additionally, about 20% of these babies may have another genetic disorder, such as Down syndrome.

Hearing loss in babies can also be attributed to issues during pregnancy. Exposure to certain medications, like thalidomide or tuberculosis drugs, or infections like cytomegalovirus, can lead to hearing problems in the unborn child.

Risk Factors

As individuals age, they may experience hearing loss due to various factors:

Exposure to Loud Noise: A sudden loud noise or prolonged exposure to loud environments, such as living near an airport, can damage hearing.
Diseases: Various medical conditions, including ear infections, brain tumors, auto-immune diseases like rheumatoid arthritis, and Meniere’s disease, can jeopardize hearing function.
Ear Canal Obstructions: Build-up of earwax or foreign objects lodged in the ear canal can impair hearing. Attempts to remove these obstructions incorrectly can also cause damage.
Head Trauma: Injuries to the head can result in damage to the inner ear structures, leading to hearing loss. Certain sports activities like scuba diving or skydiving also pose risks.
Medications: Some medications used to treat conditions such as cancer, heart disease, and infections can harm the ear and cause hearing loss. In some cases, the hearing loss may be reversible upon discontinuation of the medication, while in others, it may be permanent.

Symptoms of severe hearing loss

Recognizing hearing loss, whether in a child or oneself, involves observing certain behaviors and indicators. Here are some common symptoms to watch for:

Signs in Children:

Delayed Speech Development: Difficulty in learning to speak or speaking later than other children their age.
Inattentiveness to Sounds: Not responding to sounds or when called by name.
Academic Struggles: Poor performance in school, which may be linked to difficulties in hearing instructions or engaging in classroom discussions.

Signs for adults:

Difficulty in Noisy Environments: Trouble understanding conversations in crowded places or when there is background noise.
Difficulty in Localization: Inability to discern where sounds are coming from, such as being unable to hear someone speaking from behind.
Perception of Mumbling: Feeling like others are mumbling when they speak, indicating difficulty in understanding speech clearly.
Phone Communication Challenges: Struggling to hear or understand people during phone conversations.
High Volume Preferences: Frequently setting the volume of the television or car radio at a loud level.
Missed Alarms: Not hearing alarm clocks or other auditory alerts, which can affect daily routines and schedules.

These symptoms can vary in severity and may indicate different degrees of hearing loss. Recognizing these signs early on can prompt individuals to seek professional evaluation and appropriate interventions to address the hearing impairment effectively.

Diagnosis of severe hearing loss

Diagnosing severe hearing loss, particularly in children, is crucial for early intervention. In some states, laws mandate newborn screening for hearing before discharge from the hospital. If your baby doesn’t undergo this screening, inquire with hospital staff about where to get one.

There are two primary types of newborn hearing tests:

Automated Auditory Brain Response: Soft earphones and sensors are placed on the baby’s ears and head. A machine measures the response of the hearing nerve to gentle clicks or tones.
Otoacoustic Emissions: A small probe inserted into the baby’s ear canal measures the echo generated by soft noises played into the ears.

If your child displays symptoms of severe hearing loss as they grow older, discuss your concerns with their pediatrician. Similarly, if you’re experiencing hearing difficulties, consult your doctor. The diagnostic process is typically similar for both children and adults.

Your doctor may refer you to a specialist called an otolaryngologist or otologist, who will conduct a physical examination of your ears and inquire about your medical history. They may ask questions such as:

Do you often feel like people are speaking quietly or mumbling?
How challenging is it to hear in loud or noisy environments?
When did you first notice the problem?
Do you have any other medical conditions?
Has anyone in your family experienced hearing loss?

For children with hearing issues, the doctor may ask additional questions, such as:

How does your child respond to sounds or when you call their name?
When did they start speaking?
Have they been exposed to loud noises?
Have they experienced any head injuries?

The doctor may then recommend seeing an audiologist, who specializes in treating hearing problems. Audiologists employ various tests to assess the extent of hearing loss, including:

Pure Tone Audiometry: Conducted in a soundproof booth, this test involves wearing headphones and indicating when different pitches of sound are heard.
Speech Audiometry: Also performed in a soundproof booth, this test evaluates the ability to understand spoken words at different volumes.
Tympanometry: This test measures how the eardrum responds to air pushed into the ears, helping to identify any abnormalities in the middle ear space.

For children with hearing loss, the audiologist may assess their response to instructions and their ability to locate sources of sound through interactive games.

If you or your child has severe hearing loss, it’s essential to ask your doctor pertinent questions, such as:

What caused the hearing loss?
Is it reversible?
Are there any other specialists I need to see?
What treatment options are available?
Will the treatments restore my hearing?
For children: What accommodations are necessary at school? How can we support them at home? How can they develop their speech skills? Is there a risk of hearing loss in other siblings? Will the hearing loss worsen over time?

Treatment of severe hearing loss

Treatment for severe hearing loss typically involves utilizing various technologies to enhance the existing hearing capabilities. There isn’t a one-size-fits-all approach to therapy; rather, your doctor will recommend a treatment based on factors such as the extent of hearing loss, overall health, lifestyle, and the cause of ear damage.

Here are some treatment options:

1. Hearing Aids: These devices amplify low sounds, making them easier to hear. Some models can also reduce background noise. Hearing aids come in different styles, including those that fit inside the ear and those that clip over the top of the ears. They can be removed for activities like sleeping, swimming, or showering.

2. Extended-Wear Hearing Aids: Made of soft material, these aids can be placed in the ears by an audiologist and remain there for months. They are particularly suitable for active individuals, including athletes.

3. Implants: There are various types of implants used for severe hearing loss:

Middle Ear Implants: These devices vibrate inside the ear and can be worn for extended periods.
Cochlear Implants: Suitable for individuals with profound hearing loss where hearing aids are ineffective. Cochlear implants stimulate the nerves inside the ears and provide the sensation of sound and improved speech recognition.

The process of getting cochlear implants involves surgery to implant the device inside the cochlea, followed by the fitting of external components like a microphone, transmitter, and speech processor. Adjusting to cochlear implants requires support from hearing specialists and language therapists to learn how to interpret the sounds and utilize the device effectively.

Taking Care of Yourself or Your Child

Therapy: For children, therapy can assist in learning to use hearing aids and develop communication skills, including speech therapy or learning sign language.
School Accommodations: Communication with your child’s school can help implement hearing assistive technologies (HATS) and other supportive measures in the classroom.
Environmental Adjustments: In your home, consider using devices like HATS to improve hearing in various situations, and make adjustments such as using carpets or rugs to reduce noise.

Support and Expectations:

Seeking emotional support from therapists, counselors, or support groups can help manage feelings of worry or sadness associated with severe hearing loss.
Remember, severe hearing loss doesn’t have to hinder you or your child’s ability to enjoy life. With the right treatments and support, individuals can lead fulfilling lives, pursuing education, careers, and independence.

Early Intervention:

Prompt diagnosis and treatment are essential for managing severe hearing loss effectively. The earlier therapy begins, the better individuals can adapt to their condition.

Getting Support:

Connecting with patient groups like the Hearing Loss Association of America can provide valuable information and support for individuals living with severe hearing loss.

In summary, while severe hearing loss presents challenges, with the support of healthcare professionals, appropriate treatments, and a supportive environment, individuals can lead fulfilling lives despite their condition.

Sources

Centers for Disease Control and Prevention (CDC)
American Speech-Language-Hearing Association
Rehabilitation Engineering Research Center on Hearing Enhancement
Merck Manuals
Hearing Loss Association of America
First Signs Organization:
American Hearing Research Foundation: