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How is Usher syndrome diagnosed Because Usher syndrome affects hearing, balance, and vision, diagnosis of the disorder usually includes the evaluation of all three senses. Evaluation of the eyes may include a visual field test to measure a person's peripheral vision, an electroretinogram (ERG) to measure the electrical response of the eye's light-sensitive cells, and a retinal examination to observe the retina and other structures in the back of the eye. A hearing (audiologic...) evaluation measures how loud sounds at a range of frequencies need to be before a person can hear them. An electronystagmogram (ENG) measures involuntary eye movements that could signify a balance problem. Early diagnosis of Usher syndrome is very important. The earlier that parents know if their child has Usher syndrome, the sooner that child can begin special educational training programs to manage the loss of hearing and vision.

What are the characteristics of the three types of Usher syndrome Type 1 Children with type 1 Usher syndrome are profoundly deaf at birth and have severe balance problems. Many of these children obtain little or no benefit from hearing aids. Parents should consult their doctor and other hearing health professionals as early as possible to determine the best communication method for their child. Intervention should be introduced early, during the first few years of life, so th...at the child can take advantage of the unique window of time during which the brain is most receptive to learning language, whether spoken or signed. If a child is diagnosed with type 1 Usher syndrome early on, before he or she loses the ability to see, that child is more likely to benefit from the full spectrum of intervention strategies that can help him or her participate more fully in life's activities. Because of the balance problems associated with type 1 Usher syndrome, children with this disorder are slow to sit without support and typically don't walk independently before they are 18 months old. These children usually begin to develop vision problems in early childhood, almost always by the time they reach age 10. Vision problems most often begin with difficulty seeing at night, but tend to progress rapidly until the person is completely blind. Type 2 Children with type 2 Usher syndrome are born with moderate to severe hearing loss and normal balance. Although the severity of hearing loss varies, most of these children can benefit from hearing aids and can communicate orally. The vision problems in type 2 Usher syndrome tend to progress more slowly than those in type 1, with the onset of RP often not apparent until the teens. Type 3 Children with type 3 Usher syndrome have normal hearing at birth. Although most children with the disorder have normal to near-normal balance, some may develop balance problems later on. Hearing and sight worsen over time, but the rate at which they decline can vary from person to person, even within the same family. A person with type 3 Usher syndrome may develop hearing loss by the teens, and he or she will usually require hearing aids by mid- to late adulthood. Night blindness usually begins sometime during puberty. Blind spots appear by the late teens to early adulthood, and, by mid-adulthood, the person is usually legally blind.

What causes Usher syndrome? Usher syndrome is inherited, which means that it is passed from parents to their children through genes. Genes are located in almost every cell of the body. Genes contain instructions that tell cells what to do. Every person inherits two copies of each gene, one from each parent. Sometimes genes are altered, or mutated. Mutated genes may cause cells to act differently than expected. Usher syndrome is inherited as an autosomal recessive trait. The t...erm autosomal means that the mutated gene is not located on either of the chromosomes that determine a person's sex; in other words, both males and females can have the disorder and can pass it along to a child. The word recessive means that, to have Usher syndrome, a person must receive a mutated form of the Usher syndrome gene from each parent. If a child has a mutation in one Usher syndrome gene but the other gene is normal, he or she is predicted to have normal vision and hearing. People with a mutation in a gene that can cause an autosomal recessive disorder are called carriers, because they "carry" the gene with a mutation, but show no symptoms of the disorder. If both parents are carriers of a mutated gene for Usher syndrome, they will have a one-in-four chance of having a child with Usher syndrome with each birth. Usually, parents who have normal hearing and vision do not know if they are carriers of an Usher syndrome gene mutation. Currently, it is not possible to determine whether a person who does not have a family history of Usher syndrome is a carrier. Scientists at the National Institute on Deafness and Other Communication Disorders are hoping to change this, however, as they learn more about the genes responsible for Usher syndrome.

What is Usher syndrome Usher syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom. The major symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. The retina is a light-sensitive tissue at the back of the eye ...and is crucial for vision. As RP progresses, the field of vision narrows - a condition known as "tunnel vision" - until only central vision (the ability to see straight ahead) remains. Many people with Usher syndrome also have severe balance problems. There are three clinical types of Usher syndrome: type 1, type 2, and type 3. In the United States, types 1 and 2 are the most common types. Together, they account for approximately 90 to 95 percent of all cases of children who have Usher syndrome. Photograph of the retina of a patient with Usher syndrome (left) compared to a normal retina (right). The optic nerve (arrow) looks very pale, the vessels (stars) are very thin and there is characteristic pigment, called bone spicules (double arrows).

How effective are hearing protection devices Hearing protection devices decrease the intensity of sound that reaches the eardrum. They come in two forms: earplugs and earmuffs. Earplugs: Earplugs are small inserts that fit into the outer ear canal. To be effective they must totally block the ear canal with an air-tight seal. They are available in a variety of shapes and sizes to fit individual ear canals and can be custom made. For people who have trouble keeping them in thei...r ears, they can be fitted to a headband. Earmuffs: Earmuffs fit over the entire outer ear to form an air seal. They are held in place by an adjustable band. Earmuffs must be snugly sealed so the entire circumference of the ear canal is blocked. Properly fitted earplugs or muffs reduce noise by 15 to 30 dB of sound. The better earplugs and earmuffs are approximately equally effective in sound reduction. However, earplugs are better protection against low frequency noise(such as noise from a jackhammer), and earmuffs are better protection against high frequency noise (such as noise from an airplane taking off). For high frequency sounds, think of the high-pitched treble keys of the piano, whereas for low frequency sounds, think of the low- or deep-pitched bass keys of the piano.

What is the importance of noise-induced hearing loss? The industrial and technological revolution may have propelled society to higher levels of achievement, but this progress has also made the world a noisier place in which to live. In fact, noise pollution is a growing health hazard and can be found almost everywhere. Car alarms, leaf blowers, gunshots, boom boxes, and traffic congestion fill our cities with decibels (the measure of sound intensity). Escaping to the country... may not provide a quiet refuge, and even farmers are at high risk for exposure to noise from their farm machinery. What's more, potentially harmful noise is not necessarily unpleasant or unwanted. For example, the music at a concert and the pounding of a jackhammer on the street can be equally damaging to the inner ear. The reason for this is that any sounds (acoustic energies) delivered with equal intensity, regardless of their source, are equally dangerous. Eventually, continued or repeated exposures to high intensity sound can cause acoustic trauma to the ear. This trauma can result in hearing loss, ringing in the ears (tinnitus), and occasional dizziness (vertigo), as well as non-auditory effects, such as increases in heart rate and blood pressure. One-third of the 30 million Americans with hearing loss have an impairment that is at least partially attributed to excessive noise exposure. Noise remains the most common preventable cause of irreversible sensorineural (involving the ear's sensory nerve) hearing loss.

How is Meniere's disease diagnosed The diagnosis of Meniere's disease is primarily made from the history and physical examination. Tinnitus or ear fullness (aural fullness) need to be present to make the diagnosis An audiogram is helpful to show a hearing loss, and to rule-out other abnormalities. It is often helpful, if it can be done safely, to have an audiogram during or immediately following an attack of vertigo. This may show the characteristic low frequency hearing loss.... As the disease progresses hearing loss usually worsens. Other tests such as the auditory brain stem response (ABR), a computerized test of the hearing nerves and brain pathways, computer tomography (CT scan) or, magnetic resonance imaging (MRI) may be needed to rule out a tumor occurring on the hearing or balance nerve. These tumors are rare, but they can cause symptoms similar to Meniere's disease. A full neurological evaluation is performed to exclude other causes of vertigo.

What are the symptoms of Meniere's disease? The symptoms of Meniere's disease typically include at least several of the following: Episodic rotational vertigo: Attacks of a spinning sensation accompanied by disequilibrium (an off-balanced sensation), nausea, and sometimes vomiting. This is usually the most troublesome symptom. The vertigo usually last 20 minutes to four hours or longer. During attacks, patients are very disabled, and sleepiness may follow. An off-balanced sen...sation may last for several days. Tinnitus: A roaring, buzzing, machine-like, or ringing sound in the ear. It may be episodic with an attack of vertigo or it may be constant. Usually the tinnitus gets worse or will appear just before the onset of the vertigo. Hearing loss: It may be intermittent early in the onset of the disease, but overtime it may become a fixed hearing loss. It may involve all frequencies, but most commonly occurs in the lower frequencies. Loud sounds may be uncomfortable and appear distorted in the affected ear. Ear fullness: Usually this full feeling occurs just before the onset of an attack of vertigo. See more

What causes Meniere's disease? Although the cause of Meniere's disease is unknown, it probably results from an abnormality in the way fluid of the inner ear is regulated. In most cases only one ear is involved, but both ears may be affected in about 15% of patients. Meniere's disease typically starts between the ages of 20 and 50 years of age (although it has been reported in nearly all age groups). Men and women are equally affected. The symptoms may be only a minor nuisance, or can become disabling, especially if the attacks of vertigo are severe, frequent, and occur without warning. Meniere's disease is also called idiopathic endolymphatic hydrops.

Dry Skin on the Ear Canal Although ears play a key role in allowing people to interact with the world around them, most people usually don't notice their ears until they start developing problems. Dry ear canal skin is a seemingly mild problem that could arise from multiple causes and may lead to more serious ear issues if disregarded. Learning the facts about dry ear canal skin can help you pinpoint the cause of your ear woes and seek appropriate treatment. Facts The ear can...al is a narrow, curving tube that connects the external part of your ear -- called the auricle -- to the eardrum or tympanic membrane, which separates the ear canal from the middle ear. Lined with sensitive skin, the ear canal serves promotes hearing by channeling sound waves to the middle ear. Special glands -- called ceruminous glands -- in the outer third of your ear canal are responsible for the production of earwax, a sticky substance that lubricates and protects the ear canal skin. Causes Dry skin on the ear canal could be cause by a skin condition, lack of earwax or ear cleaning issues. Skin conditions such as dermatitis, psoriasis and eczema could affect the sensitive ear canal skin, potentially leading to skin irritation, especially in individuals with a history of sensitive skin or skin problems. According to the American Academy of Otolarygology-Head and Neck Surgery, lack of earwax in your ear canals could also lead to dry or itchy ears. Similarly, cleaning your ears too frequently, especially with cotton-tipped swabs, could wipe away the earwax lining the ear canals, which may result in dry or irritated ear canal skin. See more

What are forms of hearing loss? Hearing loss, or deafness, can be present at birth (congenital), or become evident later in life (acquired). The distinction between acquired and congenital deafness specifies only the time that the deafness appears. It does not specify whether the cause of the deafness is genetic (inherited). Acquired deafness may or may not be genetic. For example, it may be a manifestation of a delayed-onset form of genetic deafness. Alternatively, acquired ...deafness may be due to damage to the ear due to noise or from other conditions. Congenital deafness similarly may or may not be genetic. For example, it may be associated with a white forelock, and be caused by a genetic disease called Waardenburg syndrome. In fact, more than half of congenital hearing loss is inherited. Alternatively, congenital deafness may be due to a condition or infection to which the mother was exposed during pregnancy, such as the rubella virus

What are risks and complications of ear tubes? While ear tube surgery is common, minor complications can occur in up to half of the children who have them inserted. Complications include: Failure to resolve the ear infections.... Thickening of the eardrum over time, which affects hearing in a small percentage of patients. Persistent perforation after the tube falls out of the eardrum. Chronic ear drainage. Need for further and more aggressive surgery such as tonsil, adenoid, sinus, or ear surgery. Infection Hearing loss Scarring of the eardrum Possible need to keep the ear dry and to use ear plugs Foreign body reaction to the tube itself - for example, an allergic reaction to the tube material (rare) See more