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What Does Progressive Hearing Loss Mean

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Finding A Provider For Your Child

What is Sensorineural Hearing Loss? – Ear Problems

Seek help right away if you suspect your child has hearing loss. Oftentimes, your pediatrician is a good place to start. She may refer you to a pediatric audiologist or an ear, nose and throat doctor. This primer on different types of hearing specialists may come in handy as you navigate your child’s journey to better hearing.

Signs Of Hearing Loss In Toddlers And School

Older children sometimes develop hearing loss that wasn’t present before. Here are some things to look for if you think your toddler or preschool-age child might have hearing loss:

  • Has difficulty understanding what people are saying.
  • Speaks differently than other children her or his age.
  • Doesn’t reply when you call his or her name.
  • Responds inappropriately to questions .
  • Turns up the TV volume incredibly high or sits very close to the TV to hear.
  • Has problems academically, especially if they weren’t present before.
  • Has speech or language delays or problems articulating things.
  • Watches others in order to imitate their actions, at home or in school.
  • Complains of ear pain, earaches or noises.
  • Cannot understand over the phone or switches ears frequently while talking on the phone.
  • Says “what?” or “huh?” several times a day.
  • Watches a speaker’s face very intentlymany children’s hearing loss escapes detection because they are very successful lip readers.

Symptoms Can Be Unpredictable

Symptoms can vary widely in severity among people with the same disorder. And intellectual ability may or may not be affected. For example, children with Carpenter syndrome may be of normal intelligence but it is common for them to have an intellectual disability and sometimes hearing loss.

Such is the case with Myhre syndrome, the condition that Edith has. At least 80 percent of people with it have a hearing impairment, as well as intellectual disability and stiff joints.

Although Ediths hearing did decline, she does not have a profound loss. She happily wears her hearing aids, even at home.

She also takes an art class with 32 students, and as her mother noted, many dont know she has hearing aids.”

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Action List For Parents Regarding Progressive Hearing Loss:

  • Six month hearing tests may be recommended instead of the typical annual exam
  • Compare audiograms over an extended period of time, not just the two most recent audiograms.
  • When considering hearing aids, consider the most flexible gain.
  • Know your child’s audiogram and functional listening level in real life.
  • Discuss the possibility of changes in hearing with an older child.
  • Always be open about communication modes – let your child’s personality and situation lead.

1 Laryngorhinootologie. 1997 Mar 76:123-6. Streppel M Betten T von Wedel H Eckel HE Damm M Klinik und Poliklinik fur Hals-Nasen-Ohrenheilkunde, Kopf-Halschirurgie, Universitat Koln.

2 Ibid

Assistive Strategies And Technologies

What Role Does an Audiologist Play in Diagnosing and ...

Alerting systems that use light let people know when the doorbell is ringing, a smoke detector is sounding, or a baby is crying. Special sound systems transmitting infrared or FM radio signals help people hear in theaters, churches, or other places where competing noise exists. Many television programs carry closed captioning. Telephone communication devices are also available.

Lip-reading or speech-reading is particularly important for people who can hear but have trouble discriminating sounds. Most people get useful speech information from lip-reading even without formal training. Even people with normal hearing can better understand speech in a noisy place if they can see the speaker. To use this information the listener must be able to see the speakerâs mouth. Health care personnel should be sensitive to this issue and always position themselves appropriately when speaking to the hearing-impaired. Observing the position of a speakerâs lips allows recognition of the consonant being spoken, thereby improving speech comprehension in patients with high-frequency hearing loss. Lip-reading may be learned in aural rehabilitation sessions in which a group of age-matched peers meets regularly for instruction and supervised practice in optimizing communication.

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Genetic And Developmental Conditions

Hereditary factors can lead to progressive hearing loss during childhood. For example, mutations of the connexin 26 gene and the PRPS1 gene have been associated with hearing loss.

Genetic conditions such as Pendred syndrome, Alport syndrome, Turner syndrome, and Usher syndrome are linked with progressive childhood hearing loss. Congenital problems such as Mondini syndrome, a malformation of the inner ear, can also result in a progressive hearing loss.

Differential Diagnosis And Treatment Of Hearing Loss

JON E. ISAACSON, M.D., and NEIL M. VORA, M.D., Milton S. Hershey Medical Center, Hershey, Pennsylvania

Am Fam Physician. 2003 Sep 15 68:1125-1132.

Hearing loss is a common problem that can occur at any age and makes verbal communication difficult. The ear is divided anatomically into three sections , and pathology contributing to hearing loss may strike one or more sections. Hearing loss can be categorized as conductive, sensorineural, or both. Leading causes of conductive hearing loss include cerumen impaction, otitis media, and otosclerosis. Leading causes of sensorineural hearing loss include inherited disorders, noise exposure, and presbycusis. An understanding of the indications for medical management, surgical treatment, and amplification can help the family physician provide more effective care for these patients.

More than 28 million Americans have some degree of hearing impairment. The differential diagnosis of hearing loss can be simplified by considering the three major categories of loss. Conductive hearing loss occurs when sound conduction is impeded through the external ear, the middle ear, or both. Sensorineural hearing loss occurs when there is a problem within the cochlea or the neural pathway to the auditory cortex. Mixed hearing loss is concomitant conductive and sensorineural loss.

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Signs Of Fluctuating Hearing Loss

Its important to know the signs of fluctuating hearing loss, especially in children. If a child is experiencing fluctuating hearing loss, its essential to get them medical help as soon as possible to prevent the hearing loss from becoming more serious. Signs to look for in children include:

  • Babies: Not showing interest in music, or not quieting when the mother speaks
  • Children without hearing loss: More easily distracted and less responsive not understanding directions correctly
  • Children with hearing loss: Not wanting to wear hearing aids or acting like hearing aids arent working

What Are The Symptoms Of A Progressive Hearing Loss

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A progressive hearing loss may occur at both low frequency and high frequency.

If your hearing has become worse, you will experience that it becomes more and more difficult to hear and understand what people are saying in more and more situations. Certain high-frequency sounds like women´s and children´s voices have become more difficult to hear. Some sounds may even have disappeared, such as the birds singing. You may turn up the volume more and more on the TV or the radio and maybe you now also have to put on the subtitles to follow a film or a TV-program.

These are some of the symptoms and signs of a progressive hearing loss. Many of the symptoms of a progressive hearing loss are similar to those of a hearing loss in general. Read more about the symptoms of hearing loss.

If you suspect that your child has a progressive hearing loss, you will experience that your child has more and more difficulty hearing in more and more situations and may not even respond to what you are saying. Read more about the signs of hearing loss in children.

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What Do The Colors On An Audiogram Mean

An audiogram represents an individuals hearing ability by frequency and intensity . The softest sounds that a person can hear at a particular frequency is called their hearing threshold. This is usually represented by markings on their graph red represents the right ear and blue represents the left.

How Does The Sense Of Hearing Work

Here is how the ear works normally: The sound waves cause the eardrum to vibrate, which sets the three tiny bones in the middle ear into motion. The motion of the bones causes the fluid in the inner ear or cochlea to move. The movement of the inner ear fluid causes the hair cells in the cochlea to bend.

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Progressive Hearing Loss In Children

Progressive hearing loss in children can manifest with vague symptoms, such as behavioral issues or speech problems. There are many causes of childhood hearing loss, including infections, certain medications, and neurological disorders.

If you suspect your child is losing their sense of hearing, discuss your concerns with their pediatrician. Hearing tests can determine whether your child’s hearing really is diminishing and if so, other tests can be done to determine why so the appropriate treatment can be started as soon as possible. This may involve strategies aimed at improving hearing and speech, as well as management of the underlying cause of the progressive hearing loss.

Hearing Impairment In Infants

Progressive Hearing Loss in Children

The following signs may indicate a hearing problem:

  • Before the age of 4 months, the baby does not turn their head toward a noise.
  • The infant does not appear to be startled by a loud noise.
  • The infant responds to you when they can see you, but respond far less or do not respond at all when you are out of sight and call out their name.
  • The infant only seems to be aware of certain sounds.

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What Your Test Results Mean:

Carriers are not expected to show symptoms. You and your partner would both have to be carriers of nonsyndromic hearing loss, GJB2-related for there to be an increased chance to have a child with symptoms this is known as autosomal recessive inheritance. Carrier testing of your partner or donor is recommended in addition to consultation with a genetic counselor for a more detailed risk assessment.Since this is an inherited gene change, this information may be helpful to share with family members as it may impact their family planning.

Differences Between Normal & Bilateral Hearing Loss

The bilateral hearing loss is different from normal hearing loss in the following aspects :

Normal hearing loss refers to the partial or complete inability to hear. It may affect one or both ears. But bilateral hearing loss refers to the inability of both the ears to hear. Therefore it affects both the ears. As we can say that bilateral loss is a component or part of the normal hearing loss. The normal hearing loss includes both unilateral as well as bilateral hearing loss.

They also differ in their symptoms. Normal hearing loss may affect one or both ears. But in bilateral hearing loss, you will face difficulty in hearing from both sides. Bilateral hearing loss is more threatening, complex as compared to the normal hearing loss. The symptoms, as well as causes, are similar in case of normal hearing loss as well as bilateral.

But they are slightly different. In the normal hearing loss, you will face problems in one or both ears like irritating sounds, muffling or whispering of people, ear pain, the ringing of ears. But in the case of bilateral, you will observe the symptoms in both ears.

You may need a hearing aid for normal hearing loss but in case of bilateral hearing loss, you need bilateral hearing aids for better hearing.

Thus they both are slightly different from each other. But in maximum terms, both are similar in causes, symptoms, and treatment methods.

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Bilateral Hearing Loss Differential Diagnosis

Prior to the treatment, diagnosis is necessary to reveal the actual cause behind your hearing loss and the degree of hearing loss your ears are suffering. An audiologist may refer to the following diagnostic procedures for your ears :

An otoscopic and physical examination along with the questions related to your symptoms and other factors. Afterward, he may refer to the following hearing tests :

  • Laboratory tests

Objectives And Research Questions

Genetics of hearing loss

The overall aim of this systematic review is to synthesize evidence on risk factors related to neonatal, early, and late onset hearing loss as well as progressive hearing loss in children. We will investigate all risk factors that have an immediate or delayed effect on hearing. The resulting information will inform surveillance protocols and will be applicable to EHDI programs worldwide.

The review aims to answer the following primary research question: what risk factors are associated with permanent hearing loss in children? Risk factors related to congenital, early, and late onset hearing loss will be examined.

A secondary objective of this review is to investigate the risk factors associated with progressive hearing loss in children with congenital, early, or late onset hearing loss.

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When A Child Is Born With Hearing Loss

Some babies are born with hearing loss, which is known as congenital hearing loss. Many different things can cause this type of hearing loss, but it’s not always possible to pinpoint the precise cause. In about half of all cases, the cause is geneticmeaning, inherited from a parent.

Genetic factors cause more than 50 percent of all hearing loss in children, whether present at birth or developed later in life.

Genetic factors that might cause congenital hearing loss include:

Non-genetic factors that might cause congenital hearing loss include:

  • Birth complications, including herpes, rubella cytomegalovirus, toxoplasmosis or another serious infection lack of oxygen or the requirement of a blood transfusion for some reason.
  • Premature birth. Babies that have a birth weight of less than 3 pounds or that require certain life-sustaining drugs for breathing due to prematurity are at increased risk for hearing loss.
  • A nervous system or brain disorder.
  • The use of drugs that cause hearing loss by the mother during pregnancy. Known as ototoxic medications, these drugs are usually prescribed medications, including antibiotics and some pain relievers. Ototoxic drugs potentially cause damage to the auditory nerve or other hearing structures in a fetus.
  • The mother had an infection during pregnancy, including diseases like toxoplasmosis, cytomegolavirus, herpes simplex or German measles.
  • Maternal diabetes.
  • Drug or alcohol abuse by the mother or smoking during pregnancy.

Exposure To Therapeutic Drugs

Various chemicals and drugs adversely affect the auditory system the main ones in clinical use are aminoglycoside antibiotics and cisplatin, both of which are toxic to sensory hair cells. Hearing loss develops in approximately 20% of patients receiving aminoglycosides,, and the prevalence is as high as 56% among patients with cystic fibrosis,, a population exposed to repeated courses of aminoglycoside therapy. Among adults who have received cisplatin, clinically significant hearing loss develops in approximately 60% of patients with testicular cancer and 65% of patients with head and neck cancer. Susceptibility to cisplatin-induced hearing loss depends on the cumulative dose of the drug, the age of the patient , and status with respect to concurrent cranial irradiation. Patients who have severe hearing loss caused by ototoxic drugs are likely to be identified and referred for follow-up auditory testing, but many more patients with mild-to- moderate drug-induced hearing loss are not identified and hence do not receive treatment for their hearing loss.

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Why Do We Lose Our Hearing As We Get Older

Many factors can contribute to hearing loss as you get older. It can be difficult to distinguish age-related hearing loss from hearing loss that can occur for other reasons, such as long-term exposure to noise.

Noise-induced hearing loss is caused by long-term exposure to sounds that are either too loud or last too long. This kind of noise exposure can damage the sensory hair cells in your ear that allow you to hear. Once these hair cells are damaged, they do not grow back and your ability to hear is diminished.

Conditions that are more common in older people, such as high blood pressure or diabetes, can contribute to hearing loss. Medications that are toxic to the sensory cells in your ears can also cause hearing loss.

Rarely, age-related hearing loss can be caused by abnormalities of the outer ear or middle ear. Such abnormalities may include reduced function of the tympanic membrane or reduced function of the three tiny bones in the middle ear that carry sound waves from the tympanic membrane to the inner ear.

Most older people who experience hearing loss have a combination of both age-related hearing loss and noise-induced hearing loss.

Causes Of Hearing Loss And Deafness

Understanding the Life and Role of an Audiologist

Although these factors can be encountered at different periods across the life span, individuals are most susceptible to their effects during critical periods in life.

Prenatal Period

  • Genetic factors – Include hereditary and non-hereditary hearing loss
  • Intrauterine infections such as rubella and cytomegalovirus infection

Perinatal period

  • Birth asphyxia (a lack of oxygen at the time of birth
  • Hyperbilirubinemia
  • Low-birth weight
  • Other perinatal morbidities and their management

Childhood and adolescence

  • Chronic ear infections
  • Collection of fluid in the ear
  • Meningitis and other infections

Factors across the life span

  • Cerumen impaction
  • Trauma to the ear or head
  • Loud noise/loud sounds
  • Viral infections and other ear conditions
  • Delayed onset or progressive genetic hearing loss

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Causes And Risk Factors

There are a variety of causes of progressive childhood hearing loss, including infections, congenital malformations, neurological disorders, tumors, toxins, medications, trauma, and nerve damage. Your child may have a genetic predisposition to hearing loss as well.

Babies who are born prematurely or at a low birth weight are at an increased risk of developing progressive hearing loss. And if a mother has certain infections during pregnancy, that too can result in a child’s progressive hearing loss.

Childhood hearing loss can be progressive because any damage to the structures that control hearing can worsen over time. Furthermore, as a child’s hearing is developing, an impairment can prevent normal auditory development from occurring as it should.

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