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Why Does Paget’s Disease Cause Hearing Loss

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Hearing Loss In Pagets Disease

Pagets Disease

When Pagets disease affects the skull and the temporal bone , severe and progressive loss of hearing may occur. This may involve both sides or one side predominantly. If the loss of hearing is progressive and due to Pagets disease, treating the underlying Pagets disease may slow or stop the progression of the hearing loss. Hearing aids may sometimes be helpful.

Infusion Therapy Treatment For Pagets Disease

When a persons bones are healthy, they gradually replace old bone tissue. Doing so maintains adequate calcium levels, as well as preserves bone density. The entire process occurs gradually, completely regenerating itself every decade. This is known as bone remodeling. However, Pagets disease interferes with this process. Over time, it causes a persons bones to become weak. Eventually, they may also become misshapen and prone to fractures. While treatment often involves oral medications , some people fail to obtain relief from them. This is when infusion therapy may be a more viable option.

Pagets Disease Of The Temporal Bone And Hearing Loss

Pagets disease may present with unexplained hearing loss, especially in one ear. In patients with skull involvement, 30-50% may experience hearing loss. According to Deep and co-workers the most common pattern of hearing loss is a down-sloping, high frequency sensorineural hearing loss, often with a low frequency conductive loss. It is typically bilateral and progressive, occuring at a rate that is greater than observed in age-matched healthy patients. The exact mechanism of hearing loss is not known. Tinnitus occurs in 20% of patients with Pagets disease of the temporal bone and is believed to co- incide with the hearing loss. Tinnitus may be pulsatile in nature.

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Pagets Disease Of Bone Symptoms

Many patients do not know they have Pagets disease of bone because they have no symptoms. Sometimes the symptoms may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after the patient has developed complications.

Pagets disease of bone symptoms can include:

  • Pain, which can occur in any bone affected by the disease or result from arthritis, a complication that develops in some patients
  • Headaches and hearing loss, which may occur when Pagets disease affects the skull
  • Pressure on nerves, which may occur when Pagets disease affects the skull or spine
  • Increased head size, bowing of a limb, or curvature of the spine, which may occur in advanced cases
  • Hip pain, which may occur when Pagets disease affects the pelvis or thighbone.
  • Damage to cartilage of joints, which may lead to arthritis.

Any bone or bones can be affected, but Pagets disease of bone occurs most frequently in the spine, pelvis, legs, or skull. Generally, symptoms progress slowly, and the disease does not spread to normal bones.

Causes And Risk Factors Of Paget’s Disease

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Paget’s disease is a bone disorder that usually progresses at a slow rate. It’s the second most common type of bone disease, and it affects the process of bone renewal. This gradual recycling process involves the replacement of old bone tissue with new bone tissue. The new tissue is generated too quickly in individuals with Paget’s disease, and the resulting bone is weak and fragile. The disease can lead to bone pain, fractures, and deformities. The most commonly affected bones are located in the pelvis, skull, spine, and legs. Complications of Paget’s disease include hearing loss, arthritis, osteoarthritis, heart failure, and bone cancer.

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Audiometry And Auditory Brainstem Responses

Pure-tone behavioral audiometric thresholds for air and bone conduction were determined at 2.5 dB increments. Because of reports that a high-frequency sensorineural HL and a low-frequency air-bone gap are characteristic in Paget’s disease, ,,, audiometric frequencies that reflected these two effects were selected. A high pure-tone average was calculated as the arithmetic mean of pure tone thresholds at 1, 2, and 4 kHz. The air-bone gap was calculated as the mean of air-conduction thresholds at 0.5 and 1 kHz minus the bone-conduction thresholds at those frequencies.

The ABR was recorded with a commercially available clinical evoked potentials system . Electrodes were applied with a conductant paste to the vertex , left and right mastoids , and at the midfrontal location . Simultaneous ipsilateral and contralateral recordings were conducted to optimize the identification of component wave V. The bioelectric activity was differentially amplified , filtered , and averaged .

Pagets Disease Of Bone Diagnosis

Pagets disease of bone is almost always diagnosed using x rays but may be discovered initially by either of the following tests:

  • Alkaline phosphatase blood test. An elevated level of alkaline phosphatase in the blood can be suggestive of Pagets disease of bone.
  • An X-ray can show whether your bones have become enlarged as a result of Pagets disease of bone.
  • Bone scans. Bone scans are useful in determining the extent and activity of the condition. For this scan, a small amount of a radioactive substance is injected into your blood. This collects in areas where theres a lot of bone renewal taking place. For this scan, a small amount of a radioactive substance is injected into your blood. This collects in areas where theres a lot of bone renewal taking place.

If a blood test or bone scan suggests Pagets disease of bone, the affected bone should be x rayed to confirm the diagnosis.

Early diagnosis and treatment are important to minimize complications. Siblings and children of people with Pagets disease of bone may wish to have an alkaline phosphatase blood test every 2 or 3 years starting around the age of 40. If the alkaline phosphatase level is higher than normal, a bone scan may be used to identify which bone or bones are affected and an x ray of these bones is used to verify the diagnosis of Pagets disease of bone.

Further tests

In these cases, you may be advised to have a:

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Pagets Disease Of Bone Causes

Bone cells regenerate in a similar way to skin old bone is removed and replaced by new bone. This is known as bone remodeling.

Two cells are responsible for this:

  • osteoclasts cells that absorb old bone
  • osteoblasts cells that make new bone

In Pagets disease of bone, something goes wrong with the osteoclast cells and they start to absorb bone at a much faster rate than usual.

The osteoblasts then try to produce new bone more quickly, but the new bone is larger and weaker than normal.

Its not clear what triggers this, but youre at a higher risk if you have a family history of Pagets disease of bone. You may inherit a genetic fault that means youre much more likely to develop the condition.

What Are The Treatments For Paget’s Disease Of Bone

Paget Disease of the Bone

To avoid complications, it is important to find and treat Paget’s disease early. The treatments include:

  • Medicines. There are several different medicines to treat Paget’s disease. The most common type is bisphosphonates. They help reduce bone pain and stop or slow down the progress of the disease.
  • Surgery is sometimes needed for certain complications of the disease. There are surgeries to
  • Allow fractures to heal in a better position
  • Replace joints such as the knee and hip when there is severe arthritis
  • Realign a deformed bone to reduce the pain in weight-bearing joints, especially the knees
  • Reduce pressure on a nerve, if enlargement of the skull or spine injuries effects the nervous system

Diet and exercise do not treat Paget’s, but they can help to keep your skeleton healthy. If you do not have kidney stones, you should make sure to get enough calcium and vitamin D through your diet and supplements. Besides keeping your skeleton healthy, exercise can prevent weight gain and maintain the mobility of your joints. Talk with your health care provider before you start a new exercise program. You need to make sure that the exercise does not put too much stress on the affected bones.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Treatment Of Pagets Disease Of Bone

Not all patients may require active treatment. In asymptomatic patients follow up will usually be advised. Depending on the activity, severity and site of the involvement, treatment may be necessary even if a patient is not symptomatic. This is indicated in a preventative sense, to avoid later complications. The medical treatment of choice is biphosphonates, commonly used in osteoporosis. It may cause side effects in which case calcitonin, a natural occuring hormone in calcium metabolism, may be used as an alternative. Surgery may be indicated in patients with fractures, joint problems, deformed bones and in those where nerves are compressed. Surgery may improve hearing loss and some patients may benefit from bone conducton hearing devices such as the BONEBRIDGE from MED-EL and the PONTO or even cochlear implantation .

What Are Complications Of Paget’s Disease

  • Complications of Paget’s disease include
  • bone pain,
  • sarcoma, a form of bone cancer.
  • Sarcoma of bone is also referred to as osteosarcoma and is rare .
  • Abnormalities of calcium and phosphorus balance are common.
  • Bleeding can occur during surgery, when affected bone is operated on, because of an increased blood supply to the bone.
  • When Paget’s disease affects the area of the skull adjacent to the nerves of hearing, it can sometimes lead to hearing loss due to pressure on the nerves.
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    Treatment Of Pagets Syndrome

    Even if there is none therapy for Pagets disease, prescription can assist reduce discomfort besides relieve pain and other symptoms.

    Remedies are effective in combating this disease, especially if the problems have not yet begun, and it is expected that complex therapies will be based on the understanding of the sickness.

    Complaints of bone pain when symptoms occur.

    Because this phenomenon reasons your body to produce bone marrow faster than normal, faster repair results in soft and weak bone than standard bone, that can cause bone ache and rupture.

    The syndrome can influence or spread to one or all parts of the corpse. Since your bones are weak, they can bend and turn into wells. Too much stiff bones and mixing can put too much pressure on the closed joints that can reason .

    Pagets syndrome affects 1.5-9.0% of the inhabitants and is widespread in people of British inhabitants. This is more common in the elderly as well as less common in populace under the age of 55.

    Men are treated more habitually than women . The syndrome was characterized after the British physician Sir James Paget, who expresses this in 1877.

    Pagets bone disease affects the immune system in which the bone marrow replaces the old bone.

    This can cause damaged bones to break down and harden over time. Pagets bone disease is most common within the hip, head, back and leg.

    Although his findings were false and always encouraging, he believed that tumors and hypertrophy were the same.


    Diseases That Can Cause Hearing Loss


    We tend to associate hearing loss with ageing or something caused by genetic predisposition. Disease can however also lead to hearing loss.

    In fact, anything that leads to damage of the complicated structure of the ear can lead to deafness.

    1. Otosclerosis

    What it is: Otosclerosis refers to abnormal bone growth in the ears. It is often genetic and can cause a gradual onset of hearing loss. This is a relatively common cause of hearing loss.

    Symptoms: Symptoms can include dizziness, a ringing in one or both ears and gradual hearing loss.

    How it leads to hearing loss: Theabnormal growth of bone within the inner ear interferes with the movement of the bones and makes it difficult for sound waves to be transmitted.

    Treatment: There are a couple of methods doctors use for otosclerosis. A surgical procedure called stapedectomy, as well as a cochlear implant may help reverse hearing loss.

    2. Ménières disease

    What it is: Ménières disease is an illness that interferes with the flow of fluid in the inner ear.

    Symptoms: A loss of balance, a feeling of fullness in one or both ears, dizziness, nausea and ringing in the ear.

    How it leads to hearing loss: Hearing loss is caused by an extreme buildup of fluid in the ear. The buildup occurs in the part of the ear that is called the labyrinth leading to disturbed balance and distorted sound waves.

    Treatment: Doctors may prescribe medication such as prochlorperazine and antihistamines to help control symptoms.

    3. Ushers syndrome

    5. Mumps

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    What Conditions Are Linked To Hearing Loss

    If youve noticed that your hearing is worse than it used to be, its probably the case that youre suffering from some sort of hearing loss. But whats causing that loss? Thats the question you have to ask yourself because there are many different reasons why your hearing could be suffering. Its always important to get to the bottom of these problems so you can treat the cause. Your audiologist or hearing instrument specialist will be able to help you with this. Here are some conditions linked to hearing loss.

    Symptoms Of Pagets Disease Of Bone

    Most patients with Pagets disease will have no symptoms. The most common symptom is pain in the affected bone/bones. Nearby joints may also be painfull. The new weaker bone may cause deformities and fractures. The spesific symptoms will depend on which bones are affected. Other symptoms include tingling and weakness in extremities. Nerves can also be pinched by the abnormal bone. Luckily the facial nerve is not commonly involved.

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    What About Side Effects Of Bisphosphonates

    You should discuss your individual circumstances with your doctor, but if bisphosphonate treatment is required, the benefits often outweigh the risk of any potential side effects.

    A comprehensive review on the effects of bisphosphonates in the treatment of Pagets disease, published in 2017, concluded that serious side effects were rare and that the most common side effects experienced were a flu-like illness in people given bisphosphonates by infusion, and stomach upset in those given tablets. Severe side effects causing treatment discontinuation were rare.

    The most common side effect with zoledronic acid is a flu-like reaction that lasts a day or two in most people who experience it. It is usually of mild or moderate severity, but more severe reactions can sometimes occur. Pamidronate can also cause a flu-like reaction but there is less information on the frequency with which this occurs or its severity. If a flu-like reaction does occur with either drug, it can usually be controlled with paracetamol or ibuprofen.

    Both zoledronate and pamidronate may cause a decrease in calcium levels. The risk of this is reduced in people who have a good dietary calcium intake and those with normal levels of vitamin D. Sometimes vitamin D levels might be measured before treatment to determine if supplements are required. Depending on the results, calcium and vitamin D supplements may be given prior to treatment as a preventative measure.

    When should bisphosphonates be avoided?

    How Is Paget’s Disease Diagnosed

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    Pagets disease is often diagnosed by chance during tests for an unrelated medical condition, or when a bone is fractured.

    If you show certain signs of the disease , your doctor may refer you to a specialist. You may be asked to have blood or urine tests, an x-ray or a bone scan. In some cases, imaging tests such as a CT scan or MRI scan or a bone biopsy may be needed to confirm the diagnosis.

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    Pagets Disease Of The Breast Causes

    Doctors do not fully understand what causes Pagets disease of the breast. The most widely accepted theory is that cancer cells from a tumor inside the breast travel through the milk ducts to the nipple and areola. This would explain why Pagets disease of the breast and tumors inside the same breast are almost always found together 5).

    A second theory is that cells in the nipple or areola become cancerous on their own 6). This would explain why a few people develop Pagets disease of the breast without having a tumor inside the same breast. Moreover, it may be possible for Pagets disease of the breast and tumors inside the same breast to develop independently 7).

    Who Gets Pagets Disease Of The Breast

    Pagets disease of the breast occurs in both women and men, but most cases occur in women. Approximately 1 to 4 percent of all cases of breast cancer also involve Pagets disease of the breast. The average age at diagnosis is 57 years, but the disease has been found in adolescents and in people in their late 80s 3).

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    Pagets Syndrome : Causes Symptoms Diagnosis Treatment

    Pagets Syndrome or Pagets illness of bone meddles with your bodys typical reusing measure, in which new bone tissue continuously replaces old bone tissue. Over the long run, bones can get delicate and deformed. The pelvis, skull, spine, and legs are most normally influenced.


    Paget Schoettler condition is exertion actuated apoplexy of the axillary and subclavian veins related with pressure of the subclavian vein at the thoracic outlet.

    It is the venous variation of thoracic outlet condition , the disorder of side effects related with pressure of the subclavian vein, subclavian supply route or brachial plexus as they go through the thoracic outlet.

    The depiction of this element traces all the way back to 1875 when Sir James Paget detailed an instance of unconstrained apoplexy of the subclavian vein in a patient.

    In 1884, von Schoettler guessed that the condition was an outcome of injury to the vein from dreary musculoskeletal movement.

    Pagets disease affects 1.5 to 8.0% of the inhabitants as well as is widespread in populace of descent of British.

    It is more common in the elderly and less common in people. Pagets virus of the bone affects the immune system, where the new bone marrow replaces the old jawbone.

    Done period, this condition can reason damaged bones to break as well as strengthen. Pagets bone virus is mainly frequently seen in the waist, skull, back and legs.


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