Auditory Neuropathy Spectrum Disorder (ANSD)
About ANSD
Hearing loss is a common problem in newborns. Some cases are due to auditory neuropathy spectrum disorder (ANSD), a problem in the transmission of sound from the inner ear, the sound disorganized when it reaches the brain.
Someone with ANSD has difficulty distinguishing one sound from another, and trouble understanding speech clearly. In some cases, ANSD leads only mild hearing difficulties and is only a problem in noisy situations. In the majority of cases, however, it leads to profound hearing loss.
The causes of ANSD are unknown, but children who are born prematurely or have a family history of the condition are at higher risk for them. The symptoms can develop at any age, but most children with ANSD born with it and diagnosed in the first months of life.
How is ANSD becomes better understood, it is diagnosed more frequently and now around 10% to 15% of cases of hearing loss.
Fortunately, with ANSD strong language and communication skills with the help of medical equipment, therapy, and visual communication techniques to develop children. Proper diagnosis and early intervention are essential, so if you suspect that your child has any difficulty hearing, speak to your doctor as soon as possible.
How Hearing Works
To understand ANSD, it is helpful to review how the ear hears. Hearing begins when sound waves that travel through the air reach the outer ear, or pinna, the part of the ear that is visible. The sound waves go through the ear canal into the middle ear, the eardrum (a thin layer of tissue) and contains three tiny bones called ossicles. The sound causes the eardrum to vibrate. The ossicles amplify these vibrations and carry them to the inner ear.
The inner ear consists of a screw-shaped chamber called the cochlea, which is filled with liquid inside and made with four rows of tiny hair cells. If the vibrations travel through this liquid, the outer hair cells contract to and fro and amplifying the sound. If the vibrations are large enough to translate the inner hair cells into electrical nerve impulses in the auditory nerve that connects the inner ear to the brain. When the nerve impulses reach the brain, they are interpreted as sound.
How ANSD affects hearing
If someone enters ANSD sound the ear normally, but because of the damage to the inner row of hair cells or synapses between inner hair cells and the auditory nerve or damage to the auditory nerve, sound is not right from the transmitted inner ear to the brain.
As a result, the sound that comes not in the brain in a way that the brain organizes understand. It is disorganized and in some cases, the sound never makes it to the brain. In other cases, ANSD is due to a problem with the auditory nerve.
ANSD was only understood and diagnosed in the past years. As a result, many questions remain about it. Can not identify all the newborn hearing screening programs ANSD, so many children and adults could have it, but do not know.
The symptoms of ANSD can range from mild to severe. Some children with ANSD hear sounds but have trouble figuring out what this noise is. For others, all seem to sound the same, like static or white noise. For example, a voice could sound the same as running water, a dog barking might sound the same as a car horn, a bird chirping or can the same banging sound like a frying pan.
For some ANSD improved over time. For other stays the same or gets worse.
Causes of ANSD
The causes of ANSD are unknown. But several factors have put a child at risk, including:
Premature birth
Disease such as jaundice in newborns
low birth weight
Hypoxia and anoxia
Family history of ANSD
Head trauma
The symptoms of ANSD
Even if a child plays early investigation could symptoms of hearing problems in the course of time considerably. Talk to your doctor if you suspect that your child has difficulty hearing or if you notice any of these symptoms:
Not frighten your child when turning it loud or sudden noises or no sound in the direction
Your baby is not cooing, babbling or laughing by 8 months
Your child will not try to imitate sounds and actions of 12 months or does not respond to simple commands
If hearing problems are suspected, your doctor may refer you to an audiologist, someone who specializes in the diagnosis and treatment of hearing loss and balance problems. You may also want to an otolaryngologist (also called otolaryngologist), referred to specialize in the treatment of patients with diseases and disorders of the ears, nose and throat.
ANSD diagnosis
A series of tests help diagnose ANSD and rule out other hearing problems. Many of them are performed as part of routine hearing tests given to newborns. They are also used to diagnose the disease in older children.
The tests will not cause pain or discomfort in most cases not required hospitalization.
Middle ear muscle reflex (MEMR): The MEMR tests how well the ear responds to loud noises. In a healthy ear loud noises trigger a reflex and cause the muscles in the middle ear to contract. Cause in a child with ANSD, I'm not loud noises or the reflex zones much louder sounds are required to trigger it.
For MEMR (also called acoustic reflex test), a soft rubber top is placed in the ear canal. A series of loud noises are sent triggered by the spikes in the ears and a machine records whether the sound has a reflex. Sometimes the test is performed while the child sleeps.
Otoacoustic emissions (OAE): This test measures how well the outer hair cell function within the cochlea. It is done when the child is asleep or, either naturally or by mild sedation. A tiny probe that includes a special microphone is placed in the ear canal, pulsing sounds are sent through it, and a machine, what kind of measures echo the sound causes in the outer hair cells.
Auditory brainstem response (ABR): This test measures whether the auditory sound from the inner ear transmits to the lower part of the brain and how loud sounds need for the brain to be seen. If the information the brain is not obtained in a clear way, this test can show an accurate During the ABR the child is asleep, either naturally or through sedation. The audiologist places tiny earphone in the ear and sends sounds through them while electrodes on the child's head to measure brain activity placed.
If these tests show that the child has ANSD, over time, other tests can determine how severe the condition is. Currently no test can determine the severity of the disease in infants.
Treatment ANSD
Although there is no known cure for ANSD, hearing aids (ALDs), children with ANSD sense of sounds and help develop language skills. You are with a medical team to determine what devices are right for your child to work. Treatment for ANSD depends on how hard it is and the child's age at the time of diagnosis.
A crucial part of making each device effectively running therapy with a speech therapist, the children with hearing loss develop speaking and listening skills helps.
ALDs include:
Frequency modulation (FM) system: An FM system reduces background noise and makes a speaker's voice louder so that the child can understand it. The speaker wears a small microphone and a transmitter which generates an electrical signal to a wireless receiver that the child carries either the ear or elsewhere on the body. It is portable and can be helpful in the classroom.
Hearing Aids: A hearing aid amplifies sounds in the ear. Often it can help if the outer hair cells do not function properly and can not amplify sound. In some cases of ANSD, hearing aids help when they are used with an FM system. In most cases, hearing aids must not only help kids with ANSD because they just make the sound louder disorganized.
Cochlear implant: A cochlear implant is a device that has internal and external components and is designed to replace the malfunction parts of the ear. Parts of the implant positioned within the head during surgery. Another part of the implant on the outside worn behind the ear. With training and therapy a child can learn with a cochlear implant, hear and speak well.
Cochlear implants are not usually considered until children are at least 1 year old and have tried other ALDs without success.
Cochlear implants have helped many people with ANSD, but ongoing therapy after surgery is crucial to be effective for them. Not all children with ANSD are candidates for implants.
Communication and Kids With ANSD
In addition to hearing aids, children with ANSD often benefit from learning visual communication skills. American Sign Language (ASL), Cued Speech and Signed Exact English are three types of visual communication techniques. They will determine with the medical team, which is best suited to work for your child.
ASL is the best known form of visual communication. A clear language with grammar, vocabulary and syntax all its own, ASL is to be used in place of the spoken language.
Cued Speech and Signed Exact English are visual communication techniques that translate directly to what is said. Both should be used with spoken language to help people understand what they can not comprehend through lip reading.
Cued Speech is a technique in which to convey placed in various locations around the face of various forms of hand - or cue - different consonants and vowels. There is a limited number of hand positions and shapes that make it easier to learn than other methods.
Signed Exact English involves the use of certain gestures, sounds and suffixes (such as "-ing") and prefixes (such as "re-") represent.
A parent's role
By learning as much as you can about ANSD and works closely with the medical team, you will play an important role in your child help to manage the condition and play learn to communicate.