Will you go deaf with Alport syndrome?

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asked 4 days ago in Diseases Conditions by TTTu9k13 (880 points)
Will you go deaf with Alport syndrome?

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answered 4 days ago by Caldecott (32,240 points)
You will sometimes go deaf with Alport syndrome.

Sensorineural hearing loss is a common symptom of Alport syndrome and can progress to deafness in some cases.

The likelihood and the progression of the hearing loss with Alport syndrome can vary depending on the type of Alport syndrome as well as the gender of the person.

Alport syndrome does always affect the kidneys and sometimes affects a persons eyes but it also can cause hearing loss in many people.

The age that people with Alport syndrome lose hearing is during the ages of late childhood or even early adolescence for males that have the X linked form of Alport syndrome.

And hearing loss with Alport syndrome is slightly later for females that have XLAS and for people with the autosomal recessive form of ARAS Alport syndrome.

People with autosomal dominant Alport syndrome tend to have hearing loss later in life, after childhood.

Alport syndrome is a progressive disease that is characterized by a gradual decline in your kidney function, which leads to hearing loss and eye abnormalities.

The progression of the symptoms in Alport syndrome can result in end stage renal disease and can also impact hearing and vision over time.

Alport syndrome mainly affects your kidneys and leads to a progressive loss of the kidneys ability to filter waste from the blood which can result in kidney failure.

Alport syndrome is a pretty rare genetic kidney disease.

It's estimated that Alport syndrome affects around 1 in 5,000 to 1 in 10,000 people worldwide.

The most common form of Alport syndrome is X linked Alport syndrome which affects around 80 percent of cases and is estimated to occur in 1 in 50,000 male births.

Autosomal recessive Alport syndrome affects around 15 percent of cases and occurs in around 1 in 40,000 people.

And autosomal dominant Alport syndrome which is the rarest form of Alport syndrome affects less than 5 percent of cases of Alport syndrome.

The most common manifestation of Alport syndrome is blood in the urine which is also known as hematuria.

The blood in the urine in Alport syndrome is often microscopic which means that the blood can only be detected with a microscope or urine dipstick.

Although some people with Alport syndrome may have episodes of gross hematuria which is visible blood in their urine which is often triggered by infections.

Alport syndrome can cause a variety of different eye problems and eye abnormalities but rarely causes blindness.

The most common problems with the eye that are caused by Alport syndrome are anterior lenticonus which is a cone shaped lens, cataracts and retinal thinning.

These conditions can affect your vision, but they often don't cause any significant or permanent vision loss, especially if you receive treatment.

The eye signs of Alport are retinal abnormalities, lens abnormalities, corneal problems and some people may experience dot and fleck retinopathy, corneal erosion's, and anterior lenticonus.

Even Macular holes and thinning can also occur and affect your central vision.

The cause of Alport syndrome are genetic mutations that affect the type IV collagen protein, which is crucial as a component of the basement membranes in the kidneys, eyes and ears.

The mutations affecting the type IV collagen disrupt the normal structure and the function of the tissues, which lead to the symptoms of Alport syndrome like kidney disease, vision problems and hearing loss.

The first signs of Alport syndrome are hematuria also known as blood in your urine.

The blood in the urine with Alport syndrome is often microscopic which means that the blood can only be detected using a microscope or a urine dipstick test.

Other early signs of Alport syndrome are high blood pressure, protein in your urine and even hearing loss, most particularly in males.

Although not always present at the very beginning of Alport syndrome, the Alport syndrome can lead to eye abnormalities such as anterior lenticonus which is an abnormally shaped lens or even retinal changes which can also be an early sign of Alport Syndrome.

Alport syndrome is a genetic disorder which mainly affects your kidneys and causes your kidneys to lose their ability to filter out waste properly and leads to protein and blood in the urine.

Alport syndrome can also cause hearing loss and eye problems and is caused by mutations in the genes which produce type IV collagen which is a key component of glomerular basement membrane in the kidneys.

Alport syndrome affects around 1 in 5,000 to 10,000 children and can also affect adults.

Alport syndrome often manifests in childhood or early adulthood and most particularly in males that have X linked Alport syndrome.

Alport syndrome is a rare kidney disease and genetic disorder which affects your kidneys as well as your eyes and your ears.

While there's no cure for Alport syndrome, there are treatments available that can help to manage the symptoms.

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