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What Is The Most Common Cause Of Congenital Hearing Loss

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Hearing Preservation And Restoration

Congenital hearing loss: Diagnosis and treatment

Emerging therapies for hearing loss can be broadly sub-classified as hearing preservation or hearing restoration strategies. , Hearing preservation would probably be easier to achieve than hearing restoration; preservation strategies seek to promote hair cell survival and correct protein defects before complete and irreversible hair cell damage occurs. Once hair cells have died, hearing restoration strategies are required, with options depending on the condition of the remaining supporting cells.

Thanks to progress in genetic testing, it is possible to identify groups of patient with gene-specific mutations. Studies aimed at elucidating the broader effects of mutations at the gene level are needed to identify pathways interactions and crosstalk, which in turn are likely to provide novel insights into hearing preservation strategies.

What Are Signs Of Autism In Babies

Recognizing signs of autism

  • May not keep eye contact or makes little or no eye contact.
  • Shows no or less response to a parents smile or other facial expressions.
  • May not look at objects or events a parent is looking at or pointing to.
  • May not point to objects or events to get a parent to look at them.

Acquired Sensorineural Hearing Loss

Acquired means the hearing loss develops;after a person is born, usually later in life. Causes can include:

  • Aging: One of the most common conditions of growing older is;presbycusis, or age-related hearing loss, affecting one in three Americans between the ages of 65-74. Because this type of loss occurs over time, typically in both ears, its sometimes difficult to notice.
  • Noise: Noise-induced hearing loss; can be caused by exposure to a one-time loud noise, such as an explosion;or gunfire, or from;sounds louder than 85 decibels over an extended period of time. If you have to shout to be heard or your ears ring after attending a live concert or;ballgame, your hearing health is at risk.
  • Disease and infections: Viral infectionsincluding measles, meningitis and mumpscan cause;sensorineural;hearing loss.;
  • Head or acoustic trauma: Damage to your inner ear also can be caused by a blow to the head or exposure to extremely loud noise, such as an explosion. Many;veterans;suffer from;sensorineural;hearing loss due to time they spent around firearms, artillery and jet engines.
  • Tumors: Examples of common tumors that can affect hearing include acoustic;neuroma;and cholesteatoma, an abnormal skin growth in the middle ear.
  • Medications: More than 200 medications and chemicals are;ototoxic, or damaging to your hearing health. Some of those known to cause permanent damage include certain types of antibiotics, anti-inflammatory medications and cancer chemotherapy drugs.

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Symptoms Of Congenital Hearing Loss

The sensorineural aspect of congenital hearing loss is most often credited to developmental issues at birth. The inner ear houses the cochlea, which contains the nerves that help take all the information from the ear to the brain, where sound is interpreted.;

This means that the symptoms of congenital hearing loss will be moderately severe to severe. However, this is not always the case!;

How Can Hearing Loss Affect Your Baby

(PDF) Hearing Loss and Congenital CMV Infection: A ...

Early screening, diagnosis and treatment can help children with hearing loss develop speech, language and social skills. Without early treatment, hearing loss can lead to:

  • Delayed or limited language and speech development. For example, babies and children with hearing loss may have trouble understanding things that other people say, learning new words and saying words the right way. Children with untreated hearing loss may have poor communication skills.
  • Learning and social problems. Without early treatment, children with hearing loss may have problems learning in school. Hearing loss also may make it hard for them to be social with other children.

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How Is Hearing Loss Managed Or Treated

Hearing loss treatments often depend on the type and degree of hearing loss. Treatments include:

  • Hearing assist devices: These devices help restore hearing. Hearing aids are devices worn on or inside the ear to amplify sound. Healthcare providers surgically implant cochlear implants into the inner ear to treat inner ear hearing loss.
  • Hearing rehabilitation: Also called audiologic rehabilitation, this therapy helps you adjust to hearing loss and hearing aids. A therapist also can help you learn to use visual cues and lip reading to improve communication.
  • Listening devices: Devices can make it easier to hear the telephone, television or videos on your computer.
  • Medications: Hearing loss caused by ear infections may improve with antibiotics. Corticosteroids can ease the swelling of cochlear hair cells after exposure to loud noise. If medications are causing your hearing loss, your provider may prescribe a different drug.
  • Surgery: Your provider may place ear tubes in the eardrum. Ear tubes treat chronic middle ear infections that contribute to hearing loss. Providers also perform surgeries to remove tumors, repair birth defects and place cochlear implants.

How Do Genes Work

Genes are highly complex and form a roadmap of sorts for the synthesis of proteins, which are the building blocks for everything in the body: hair, eyes, ears, heart, lungs, etc. Every child inherits half of their genes from one parent and half from the other parent. If the inherited genes are abnormal or defective, a health disorder such as hearing loss or deafness can occur. Hearing disorders are commonly inherited in one of four ways:

  • Autosomal Dominant InheritanceFor autosomal dominant disorders, only one bad copy of the gene is needed to affect the child. A heterozygous parent has two types of the same gene , each with a 50 percent chance of affecting the child. An affected homozygous parent will pass an abnormal copy to all their children, and they will all be affected. Autosomal dominant traits usually affect males and females equally.
  • Autosomal Recessive InheritanceFor autosomal recessive disorders, the child must inherit a bad copy of the gene from both parents. If there is one good copy of the gene, the child will not be affected. Its possible for two unaffected heterozygous parents to have an affected child , which means that this disorder can skip generations.
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    Using Hearing Protection Whenever Necessary

    Hearing protection aims to reduce the surrounding noise from a potentially dangerous level to a safe listening level for you to prevent noise-induced hearing loss. It is essential to ensure these are implemented in work environments with high levels of noise, such as construction sites, mining and shooting ranges.

    Symptoms Of Hearing Loss

    Genetics of hearing loss

    The signs of hearing loss you may experience are based on different factors like the type and cause of your deafness. While some experience deafness during birth, others experience hearing loss as they age or due to trauma.;

    Hearing loss may be a symptom of an underlying health condition like tinnitus or infectious disease. So, early detection and diagnosis are crucial and will help you decide what to do next.

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    Variable Inheritance Congenital Deafness

    Wolfram Syndrome, first described in 1938, is also known as DIDMOAD . According to Plantinga et al , all patients had similar sensorineural hearing loss with a gently downsloping pattern. It does not progress with age.

    Plantinga et al . Hearing impairment in genotyped Wolfram syndrome pateints. Ann ORL 117 474-500

    Hearing Loss And Deafness

    A person who is not able to hear as well as someone with normal hearing hearing thresholds of 20 dB or better in both ears is said to have hearing loss. Hearing loss may be mild, moderate, severe, or profound. It can affect one ear or both ears, and leads to difficulty in hearing conversational speech or loud sounds.

    ‘Hard of hearing’ refers to people with hearing loss ranging from mild to severe. People who are hard of hearing usually communicate through spoken language and can benefit from hearing aids, cochlear implants, and other assistive devices as well as captioning.

    ‘Deaf’ people mostly have profound hearing loss, which implies very little or no hearing. They often use sign language for communication.

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    Drug Delivery And Therapeutic Targets

    Pharmacological treatment to enhance self-repair of hair cells and trans-differentiation of supporting cells will require high throughput, rapid screening systems to optimize rational drug design and the development of drug delivery systems for the cochlea. Studies have demonstrated the efficiency of zebra-fish models in drug design and middle ear infused, gelatine-based hydrogels appear promising as a means to deliver drugs to the cochlea. A novel intra-tympanic polymer gel delivery system has been evaluated and tested as a strategy for antiviral drug delivery in a guinea pig model and holds promise for providing antiviral therapy to the CMV-infected cochlea while sparing the patient the substantial toxicities associated with systemic delivery of antiviral agents.

    Causes Of Congenital Hearing Loss


    A congenital condition is indicative of a health condition or impairment that is present from birth. This means that the condition is not acquired after birth but instead is the result of some kind of development defect.;

    In congenital hearing loss, this condition is mostly the result of developmental issues that present at birth in the form of hearing impairment. The level of hearing loss that an infant can experience from congenital hearing loss varies. Early detection of hearing loss and assessment of hearing loss severity may improve an infant’s chances of correcting or improving hearing.

    Congenital hearing loss has multiple causes, all of which affect a baby during development. These causes can range from an infection to premature birth, low birth weight, or even maternal diabetes. It is possible in rare cases for the infant to acquire hearing loss as a result of traumatic injury at birth; however, that would not fall under a developmental problem.;

    Here are more possible causes for congenital hearing loss:

    • Anoxia
    • Jaundice;
    • Maternal high blood pressure;
    • Genetics

    According to the American Speech-Language-Hearing Association , the main reason for congenital hearing loss is due to genetic abnormalities. If a baby is born with a genetic disposition to having hearing loss, this can appear at birth or develop later in life.;

    This condition would be caused by a parent of an infant passing on a gene that impairs the normal development of the hearing system.;

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    What Are Common Types Of Hearing Loss

    Your babys auditory system is the system in the body that helps her hear. It makes sense of sound information as it travels from the ear to the brain. Problems in these parts of the auditory system may cause hearing loss:

    • Outer ear. This includes the part of the ear on the outside of the head, the ear canal and the outside of the eardrum. The eardrum separates the outer and middle ear.
    • Middle ear. This is made up of the inside of the eardrum and three small bones called ossicles. Sound coming into the ear moves through the ear canal to the eardrum, causing the eardrum to vibrate . When the eardrum vibrates, it moves the ossicles. This helps sound move to the inner ear.
    • Inner ear. This is made up of the cochlea and canals that help with balance. The inner ear also has nerves that change sound vibrations into signals that travel to the brain through the auditory nerve . The auditory nerve sends sound information from the ear to the brain.

    Common types of hearing loss include:

    How Can I Tell If My Baby Has Hearing Problems

    Signs of hearing loss in your baby can include:

  • Not being startled by loud sounds.
  • Not turning toward a sound after hes 6 months old.
  • Not saying single words like mama or dada by the time hes 1 year old.
  • Turns his head if he sees you, but not if you only call out his name.
  • Seems to hear some sounds but not others.
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    What Causes Hearing Loss

    Were not sure what causes all forms of hearing loss. Possible causes include:

    Genes. Genes are parts of your bodys cells that store instructions for the way your body grows and works. Genes are passed from parents to children. Genes may play a role in about half of the cases of hearing loss in babies and children. If you or your partner has a family history of hearing loss, you may want to talk with a genetic counselor before getting pregnant. This is a person who is trained to help you understand about how genes, birth defects and other medical conditions run in families, and how they can affect your health and your babys health.

    Sometimes the instructions in genes change. This is called a gene change or a mutation. Gene changes can cause two kinds of hearing loss:

  • Syndromic. The hearing loss happens with other health problems, like blindness.
  • Non-syndromic. This is when hearing loss is the only condition a baby has. About 7 in 10 mutations causing hearing loss are non-syndromic.
  • Premature birth or low birthweight. Premature birth is birth that happens too early, before 37 weeks of pregnancy. Premature babies often have more health problems at birth and later in life than babies born full term.; Low birthweight is when a baby is born weighing less than 5 pounds, 8 ounces. Learn more about things you can do to help reduce your risk for premature birth.

    Infections your baby has after birth. Infections that can cause hearing loss include:

    Hearing Loss Signs And Symptoms

    What is Sensorineural Hearing Loss? – Ear Problems

    Everyone has experienced changes in sensitivity to sound at one point or another. Changes in hearing are a common part of everyday life. You may have found yourself speaking louder or even yelling unintentionally, or maybe youve been in a room and wondered why everyone was speaking so softly or quietly.;

    Hearing loss is nothing to be ashamed of, and it is a common experience for many people. So, how do you know when changes in your hearing are a genuine medical concern?

    Most likely, you are missing out. You are either not hearing something due to loudness or intensity of sound or missing certain sounds, such as people’s voices in crowded environments due to loss of hearing at different frequencies. Not being able to hear can be isolating.

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    Auditory Processing Disorder In Kids With Normal Hearing

    Some kids may pass traditional hearing tests, but still struggle to listen and understand speech, particularly in noisy environments. They may ask you to repeat what you’ve said a lot, and struggle to differentiate similar sounding words. If this sounds like your child, it’s important to talk to your doctor, as it could be auditory processing disorder. This means the ears can detect sound adequately, but the brain struggles to interpret the sound correctly.;

    Mondini And Michel Dysplasia

    Temporal Bone CT scans are done routinely in persons with childhood sensorineural hearing loss. About 25% of patients with congenital hearing loss will have bony inner ear malformations .

    The normal cochlea has two and one-half turns. A cochlear malformation consists of a membranous; abnormality, a bony abnormality, or a combination of these two. If cochlear development is arrested in the embryo, a common cavity may occur instead of the snail like cochlea. A complete labyrinthine and cochlelar aplasia is called the Michel deformity .

    An incomplete partition is called the Mondini dysplasia or malformation. This furthermore consists of a cystic apex, a dilated vestibule and a large vestibular aqueduct.

    Patients with the common Down’s syndrome , often have inner ear malformations.

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    How Does It Affect How You Hear

    Sensorineural hearing loss affects both the loudness and the clarity of the sounds you hear. You may also experience a reduced range of sounds you find comfortable. Meaning, soft and normal sounds are too soft, but loud sounds very quickly get too loud and may really bother you.

    Sensorineural hearing loss can affect all ranges of hearing. For people with age-related hearing loss, however, it’s typical to experience what’s known as high-frequency hearing loss, which results in the reduced ability to hear high-pitched sounds.

    Many people with sensorineural hearing loss report that they can hear but struggle to understand speech. This is especially true in the presence of background noise, and it can be frustrating and exhausting to deal with.

    Economic And Financial Impact

    Congenital hearing loss

    The financial and economic implications of hearing loss are immense. According to the World Health Organization, the global cost of unaddressed hearing loss is about $750 billion every year. This estimate encompasses:

    • Health sector costs

    • Loss of productivity

    • Educational support costs;

    • Social costs

    Sadly, the situation is worse in developing countries. Children suffering from hearing loss rarely receive educational and social support. Even some countries with provisions for children in this category have poorly equipped and poorly managed facilities.;

    Adults with hearing loss find it difficult to get employment. Among those who have jobs, a more significant percentage of them are stuck in the lower cadres of work than others at their level.;

    Although efforts are being made to raise awareness, improve access to education, provide vocational support services, and create jobs, there’s still much more to be done.

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    Being Conscious Of Loud Noise Consumption

    Be aware of your surroundings and listening environment. If you find yourself in an environment where you have to raise your voice to speak to someone whos just a meter apart, the listening environment is likely to be above 85 decibels. Whenever possible, make sure to turn down the volume of music, television, radio or speakers to a safe listening level below 80 decibels.

    What Healthcare Providers Diagnose And Treat Hearing Loss

    If you suspect hearing loss, you may see an:

    • Audiologist: These specialists conduct hearing exams and hearing needs assessments to discuss your unique listening and communication needs. They help with determining the appropriate hearing devices, which often include hearing aids, in addition to other types of hearing device technology . Most audiologists have doctorates in audiology . Audiologists are not medical doctors.
    • Hearing aid specialist: These specialists pass a state exam and receive state licenses to conduct hearing tests. They can fit you for hearing aids.
    • Otolaryngologist: These medical doctors are also known as ear, nose and throat specialists. They prescribe medications and perform surgeries to treat ear problems and hearing loss.

    Audiologists or hearing aid specialists often work with otolaryngologists. As a team, they can address all your hearing issues to help improve your hearing.

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