Paget’s disease can severely affect hearing, especially when it involves the temporal bone, leading to hearing loss through two primary mechanisms: Mechanical compression: the thickening and reshaping of the temporal bone can compress inner ear structures, obstructing sound wave transmission and limiting the movement of the ossicles (malleus, incus, and stapes), which are essential for carrying sound from the eardrum to the inner ear. Nerve damage: abnormal bone growth may also exert pressure on the auditory nerve, potentially damaging the nerve fibres that transmit sound signals to the brain. This can cause sensorineural hearing loss, a type of hearing impairment where the ability to perceive sound is diminished due to inner ear or auditory nerve damage.

Paget's Disease: symptoms, causes & treatment

Paget's disease: effects on inner ear health

Paget's disease can have a substantial impact on the inner ear by altering the temporal bone, which encloses auditory structures, causing it to thicken and lose shape. This irregular bone development may compress and interfere with components of the inner ear, resulting in symptoms such as hearing loss, constant ear ringing, and issues with balance. Consistent monitoring and prompt treatment are crucial to effectively manage these symptoms, supporting overall auditory and balance health.

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What is Paget's disease?

Paget’s disease, also known as Paget’s disease of bone, is a long-term condition characterised by abnormal and excessive bone remodelling. This disorder causes bones to enlarge and become misshapen, creating a weaker bone structure that is more susceptible to fractures and other complications. Although Paget’s disease can affect any bone, it most commonly involves the pelvis, spine, skull, and the long bones of the legs. The irregular bone growth linked with this disease can lead to pain, deformities, and challenges in mobility and joint function.

Who gets Paget’s disease?

Paget's disease typically affects older adults, particularly those over 50, with an estimated 1-2% of people over 55 experiencing the condition. Men have a slightly higher likelihood of developing Paget's disease than women, and it is more commonly seen in individuals of Anglo-Saxon descent, especially in regions such as North America, Australia, New Zealand, and Europe.

In contrast, the disease is relatively rare in Asia, Africa, and Scandinavia. Age is a key risk factor, as Paget’s disease is uncommon in individuals under 40, and the likelihood of its onset increases with age. Family history also plays a role, as the disease often appears within families.

What causes Paget’s disease?

The exact causes of Paget’s disease are still not fully understood, but it is thought to stem from a mix of genetic and environmental influences.

A genetic predisposition is considered a major risk factor, as the condition frequently appears within families. Specific genetic mutations have been identified that alter normal bone remodelling by impairing osteoclast function, the cells that handle bone resorption, resulting in excessive and uncontrolled bone growth.

Environmental factors may also play a role in the onset of Paget’s disease. One hypothesis suggests that certain viral infections, potentially lying dormant in the body for years, could contribute to its development. Research indicates that viruses such as the measles virus or canine distemper virus might affect osteoclast activity, possibly interacting with genetic susceptibility to trigger the condition.

What are the symptoms and signs of Paget’s disease

Many individuals with Paget’s disease of bone may be unaware of their condition, as symptoms can either be mild or entirely absent. When symptoms do appear, they often resemble those of other bone conditions, such as arthritis.

The symptoms of Paget’s disease of bone vary depending on which bones are affected and typically worsen gradually if left untreated. Signs and symptoms of Paget’s disease of bone may include:

Bone or joint pain: this can result from Paget’s disease itself or from arthritis, a common complication of Paget’s when the disease affects areas near joints. Osteoarthritis may develop in these cases.
Increased head size, limb bowing, or spinal curvature: these types of bone deformities may occur in advanced stages of Paget’s disease.
Bone fractures: Paget’s disease can weaken bones, making fractures (breaks) more likely.
Headaches and hearing loss: if Paget’s disease affects the skull, it may lead to symptoms such as headaches and hearing impairment.

Impact of Paget’s disease on hearing

When Paget’s disease affects the temporal bone—a complex structure of the skull that encloses key parts of the inner ear, such as the cochlea and vestibular system—it can significantly impact hearing and balance. The disease may cause the temporal bone to thicken and become misshapen, compressing inner ear structures and hindering their function. This abnormal bone growth can lead to symptoms such as hearing loss, tinnitus ( ringing in the ears), and dizziness. Additionally, changes in bone structure may disrupt the auditory nerve, impeding sound transmission to the brain and worsening hearing difficulties.

Hearing loss

Paget’s disease can severely affect hearing, especially when it involves the temporal bone, leading to hearing loss through two primary mechanisms:

Mechanical compression: the thickening and reshaping of the temporal bone can compress inner ear structures, obstructing sound wave transmission and limiting the movement of the ossicles (malleus, incus, and stapes), which are essential for carrying sound from the eardrum to the inner ear.
Nerve damage: abnormal bone growth may also exert pressure on the auditory nerve, potentially damaging the nerve fibres that transmit sound signals to the brain. This can cause sensorineural hearing loss, a type of hearing impairment where the ability to perceive sound is diminished due to inner ear or auditory nerve damage.

Tinnitus

Tinnitus, often experienced as a ringing or buzzing in the ears, is another common symptom of Paget’s disease. This condition may result from altered blood flow or pressure in the blood vessels within the temporal bone, which can be impacted by the disease’s bone deformities.

Dizziness and balance issues

Paget’s disease can also disturb balance, as the inner ear plays a crucial role in maintaining equilibrium. Structural changes in the temporal bone may disrupt the inner ear’s balance mechanisms, resulting in dizziness and stability issues that can complicate walking and standing.

How is Paget's disease diagnosed?

The diagnosis of Paget’s disease involves several essential steps:

Clinical assessment: a thorough medical history and physical examination are fundamental. This includes reviewing symptoms, identifying any visible bone deformities, and assessing overall health.
Imaging techniques: Several imaging methods are utilised to confirm the diagnosis:
X-rays: these can reveal typical changes in bone structure, such as thickening and deformities.
CT scans: provide detailed cross-sectional images that help identify bone abnormalities and gauge the disease's extent.
MRI scans: offer high-resolution images of bones and surrounding tissues, useful for detecting changes in bone marrow and adjacent soft tissues.
Blood tests: blood tests measure specific biomarkers associated with bone remodelling. Elevated levels of alkaline phosphatase, an enzyme involved in bone turnover, can indicate increased bone activity, which is characteristic of Paget’s disease.

By combining these diagnostic tools with a comprehensive clinical evaluation, healthcare providers can accurately diagnose Paget’s disease and assess its impact on the bones.

Treatment for Paget's disease in the temporal bone

Medication and bisphosphonates

Bisphosphonates: these medications are commonly prescribed to reduce bone resorption and stabilise bone structure. By decreasing osteoclast activity, bisphosphonates help slow the progression of Paget’s disease, preventing further bone deformity.
Pain relief: analgesics and anti-inflammatory drugs are often used to manage the pain associated with Paget’s disease, improving patient comfort and quality of life.

Surgical interventions

Correction of bone deformities: surgery may be required to address severe bone deformities caused by Paget’s disease. Reconstructive procedures can help restore normal bone shape and function.
Nerve decompression: surgical intervention may be necessary to relieve pressure on the auditory nerve, potentially improving symptoms like hearing loss and tinnitus.
Fracture stabilisation: for fractures resulting from weakened bones, surgical procedures may be needed to repair and stabilise affected areas.

Hearing aids and other aids

Hearing aids: for patients experiencing hearing loss due to Paget’s disease, hearing aids can improve auditory function and daily communication.
Assistive listening devices: devices like amplified telephones or TV listeners can further support hearing, enhancing overall quality of life.

Lifestyle and nutrition

Balanced diet: a nutrient-rich diet, especially one high in calcium and vitamin D, supports bone health and can help manage symptoms of Paget’s disease.
Regular exercise: physical activity contributes to overall health, helping manage symptoms by strengthening muscles and supporting bone integrity.

Exercise and physiotherapy

Physiotherapy and targeted exercises can enhance joint mobility, reduce muscle weakness, and improve balance, which are valuable for managing the effects of Paget’s disease on the temporal bone.

Living with Paget's disease

Paget’s disease can impact many aspects of daily life, from physical mobility to social interactions. Common challenges include difficulty walking, pain during movement, and balance problems. To manage these, individuals may need to adapt their living environments by adding grab bars in bathrooms, using mobility aids, or adjusting workspaces. Occupational therapy can also provide valuable strategies for managing daily tasks and maintaining independence.

Managing Paget’s disease, especially its effects on hearing, requires a comprehensive approach:

Hearing aids and assistive devices:

Hearing aids: these can significantly improve hearing, making it easier to engage in conversations and daily activities
Assistive listening devices: tools such as amplified telephones or TV listening systems can further aid communication and enhance quality of life.

Communication strategies:

Environment: reducing background noise and creating quiet settings can make communication clearer.
Visual aids: techniques like lip reading, visual cues, or even sign language can improve understanding.
Subtitles: using subtitles on television or video calls can aid comprehension.

Regular medical check-ups:

Monitoring progression: consistent medical assessments help track Paget’s disease progression and identify any new complications early on.
Hearing tests: routine hearing evaluations allow for timely interventions if hearing declines.

By incorporating these strategies and maintaining regular medical oversight, individuals with Paget’s disease can more effectively manage their condition and enhance their quality of life.

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Paget's disease