Otosclerosis (also called otospongiosis) is a condition in which abnormal bone remodeling occurs in the middle ear, most commonly affecting the stapes bone—the smallest bone in the human body.

How otosclerosis develops:

In a healthy ear, the three ossicles (malleus, incus, and stapes) vibrate freely to transmit sound from the eardrum to the inner ear. In otosclerosis:

1. Abnormal bone tissue begins to grow in the middle ear, typically around the stapes footplate
2. This new bone gradually hardens and "fixes" the stapes to the oval window (the membrane between the middle and inner ear)
3. The stapes loses its ability to move freely and vibrate
4. Sound transmission to the inner ear is disrupted, causing progressive hearing loss
5. In advanced cases, the abnormal bone may also affect the cochlea (inner ear), causing sensorineural hearing loss

Types of hearing loss in otosclerosis:

• Conductive hearing loss (most common): When the stapes can't vibrate, sound doesn't reach the inner ear properly
• Mixed hearing loss: Conductive loss plus sensorineural loss when the cochlea is affected
• Pure sensorineural loss (rare): "Cochlear otosclerosis" affecting only the inner ear

Who gets otosclerosis?

• Age of onset: Typically starts between ages 20-40, though it can begin in childhood or later adulthood
• Gender: Affects women twice as often as men
• Ethnicity: More common in Caucasians, less common in people of Asian or African descent
• Progression: Usually gradual over years, though pregnancy and menopause may accelerate progression in women
• Bilateral involvement: Affects both ears in about 70-80% of cases, though one ear may be worse than the other
• Prevalence: Affects approximately 1 in 200 people in the UK, though only about 10% of those develop symptoms severe enough to seek treatment

The progression timeline:

Otosclerosis is progressive but the rate varies greatly between individuals:

• Some people experience slow progression over decades
• Others may notice more rapid deterioration over a few years
• The condition may stabilize in some cases
• Early diagnosis and treatment can prevent severe hearing loss

Over time, untreated otosclerosis can lead to significant hearing challenges that impact communication, work, and quality of life. However, with appropriate treatment—either hearing aids or surgery—most people maintain excellent hearing and connectivity.

The exact cause of otosclerosis isn't fully understood, but research has identified several contributing factors. It's likely a combination of genetic, hormonal, and environmental influences.

1. Genetic factors (Primary cause):

Heredity plays a significant role in otosclerosis:

• Family history: About 50-60% of people with otosclerosis have a family member with the condition
• Inheritance pattern: Autosomal dominant with incomplete penetrance—meaning if you inherit the genes, you may or may not develop symptoms
• Multiple genes involved: Several genes have been identified that increase susceptibility
• Risk to relatives: If you have otosclerosis, your children have about a 25% chance of developing it

2. Hormonal factors:

Hormones appear to influence the development and progression of otosclerosis:

• Female predominance: Women are affected twice as often as men, suggesting hormonal involvement
• Pregnancy: Many women first notice symptoms during pregnancy, or experience worsening during pregnancy
• Estrogen connection: Estrogen may activate dormant otosclerotic foci (areas of abnormal bone)
• Menopause: Some women experience progression of hearing loss during menopause
• Multiple pregnancies: May increase risk or accelerate progression

3. Autoimmune factors:

• Some research suggests otosclerosis may have an autoimmune component
• The body's immune system may trigger abnormal bone remodeling
• Higher incidence in people with certain autoimmune conditions

4. Viral factors (debated):

• Some studies have suggested measles virus involvement, though this remains controversial
• Measles vaccination has NOT been shown to cause otosclerosis
• More research is needed to confirm any viral connection

5. Environmental and lifestyle factors:

• Vitamin D deficiency: May play a role, though evidence is limited
• Fluoride intake: Some older studies suggested a protective effect, but this is not conclusive
• Stress fractures: Some theories suggest micro-trauma to the temporal bone may trigger abnormal bone growth

Risk factors summary:

• Family history of otosclerosis or unexplained hearing loss
• Being female, especially during reproductive years
• Caucasian ethnicity
• Age 20-40 (typical onset)
• Pregnancy or hormonal changes

Can otosclerosis be prevented?

Unfortunately, because otosclerosis is primarily genetic, there's no known way to prevent it. However, early detection and treatment can prevent severe hearing loss and maintain quality of life. If you have a family history, inform your GP or audiologist so they can monitor your hearing appropriately.

The primary symptom of otosclerosis is gradual, progressive hearing loss. Because it develops slowly, many people don't notice the early changes until hearing difficulties become more pronounced.

Primary symptom - Progressive hearing loss:

• Gradual onset: Hearing loss typically develops slowly over months to years
• Low-frequency first: Often affects ability to hear low-pitched sounds initially (like men's voices, bass notes)
• Difficulty with soft sounds: Trouble hearing whispers, quiet conversations, or soft environmental sounds
• Speech understanding: Particular difficulty hearing conversations in noisy settings like restaurants, parties, or busy care homes
• One ear first: May start in one ear but usually progresses to affect both ears over time
• Variable severity: Can range from mild to profound, depending on the extent of stapes fixation

Tinnitus (Common additional symptom):

• Affects about 75% of people with otosclerosis
• Often described as ringing, buzzing, humming, or roaring sounds
• May be constant or intermittent
• Can be more noticeable in quiet environments
• Sometimes improves after successful treatment
• May worsen with fatigue or stress

Other possible symptoms:

• Fullness or pressure: A sensation that the ear feels "stuffed up" or blocked
• Dizziness or vertigo: Occurs in about 25% of cases, usually when the cochlea is affected (cochlear otosclerosis)
• Balance problems: Mild imbalance, especially in advanced cases affecting the inner ear
• Autophony: Hearing your own voice as unusually loud or echoing

Impact on daily life:

• Difficulty following conversations in groups or meetings
• Missing important information at work or social events
• Struggling to hear on the telephone
• Needing to turn up TV or radio volume significantly
• Asking people to repeat themselves frequently
• Social withdrawal or avoiding situations where hearing is challenging
• Listening fatigue and mental exhaustion from straining to hear
• Safety concerns (not hearing doorbells, alarms, traffic)

Symptoms that warrant urgent evaluation:

• Sudden hearing loss (seek immediate medical attention)
• Severe dizziness or vertigo
• Hearing loss accompanied by ear pain or discharge (suggests another condition)
• Rapid progression of hearing loss

For care home residents:

Symptoms may be more difficult to recognize in residents with cognitive impairment:

• Increased confusion or appearing "not with it"
• Withdrawal from social activities or group settings
• Not responding when called
• Increased agitation or frustration
• Appearing not to follow instructions

If you or a loved one notice these symptoms, especially if there's a family history of hearing loss, consult your GP for a hearing evaluation. Early diagnosis allows for timely intervention and better outcomes.

Otosclerosis is highly manageable with the right care. Treatment options range from observation to sophisticated surgery, depending on the severity of hearing loss, individual circumstances, and patient preferences.

1. Monitoring and Observation:

For early-stage otosclerosis with minimal hearing loss:

• Regular audiological assessments (typically annually or as recommended)
• Tracking progression to determine optimal timing for intervention
• Appropriate for mild hearing loss that doesn't significantly impact quality of life
• Allows patient to make informed decisions about timing of treatment

2. Hearing Aids (Highly Effective Non-Surgical Option):

Hearing aids are excellent for otosclerosis and work very well for conductive hearing loss:

Important note about hearing aids:

• Hearing aids don't stop otosclerosis from progressing
• They treat the symptoms (hearing loss) but not the underlying condition
• As hearing changes, hearing aids can be adjusted to accommodate
• Many people successfully use hearing aids their entire lives without needing surgery

Clear Ear Cheer hearing aid support:

• We provide mobile hearing aid clinics for care home residents
• Free cleaning, filter changes, and battery replacements
• Troubleshooting common issues to ensure optimal performance
• Keeping devices in top condition alongside medical treatment

3. Surgery - Stapedectomy/Stapedotomy (Definitive Treatment):

For more advanced cases or when patients prefer surgical intervention:

Surgical details:

• Procedure time: 1-2 hours
• Anesthesia: Usually performed under local anesthetic with sedation, sometimes general anesthetic
• Approach: Through the ear canal using microscopes or endoscopes
• Hospital stay: Usually day-case surgery or overnight stay
• Recovery: Most people return to normal activities within 1-2 weeks

Success rates and outcomes:

• Hearing improvement: 90-95% of patients experience significant hearing improvement
• Complete restoration: Many achieve hearing levels close to normal
• Long-term stability: Results are generally stable over many years
• Tinnitus: Often improves after successful surgery
• Quality of life: Dramatic improvement in communication and daily functioning

Risks and considerations:

• Hearing loss: Small risk (1-2%) of sensorineural hearing loss or no improvement
• Tinnitus: Rare risk of new or worsened tinnitus
• Vertigo: Temporary dizziness common immediately after surgery; rarely persistent
• Taste changes: Temporary altered taste sensation (resolves within weeks)
• Prosthesis issues: Rare possibility of prosthesis displacement requiring revision
• Infection: Very rare

Recovery and post-operative care:

• Keep ear dry for 4-6 weeks (no swimming, careful showering)
• Avoid nose blowing, heavy lifting, or straining
• Avoid flying for 2-4 weeks
• Hearing may initially be worse due to swelling/packing
• Gradual improvement over 6-12 weeks as healing progresses
• Follow-up hearing test typically at 6-8 weeks to assess results

Which ear to operate on first?

• Usually the worse-hearing ear is operated on first
• If successful and needed, the other ear can be done 6-12 months later
• Some surgeons prefer not to operate on both ears to maintain at least one "untouched" ear

4. Sodium Fluoride (Limited Evidence):

• Some studies suggest sodium fluoride supplements may slow progression
• Evidence is mixed and not conclusive
• Rarely prescribed in modern practice
• Should only be considered under specialist guidance

Choosing between hearing aids and surgery:

Hearing aids may be preferred if:

• Hearing loss is mild to moderate
• Patient prefers non-invasive approach
• Medical conditions make surgery higher risk
• Patient wants to avoid surgical risks
• Only one ear is affected (can keep one ear "natural")

Surgery may be preferred if:

• Hearing loss is moderate to severe
• Patient desires long-term solution without devices
• Both ears significantly affected
• Hearing aids insufficient or patient dissatisfied with them
• Professional or lifestyle reasons favor not wearing hearing aids

Your ENT specialist will discuss potential outcomes, risks, and benefits based on your specific situation to help you make an informed decision. Many people successfully use either option, and the choice is highly personal.

Unique challenges for care home residents with otosclerosis:

• Late-stage presentation: Residents may have had progressive hearing loss for many years before entering care
• Multiple hearing issues: May have both age-related hearing loss (presbycusis) and otosclerosis
• Surgical considerations: Age and comorbidities may make surgery more complex
• Communication barriers: Difficulty participating in care discussions or expressing needs
• Social isolation: Withdrawal from group activities and conversations
• Cognitive concerns: Hearing loss can mimic or worsen dementia symptoms
• Safety issues: Not hearing alarms, warnings, or instructions

Recognition in care home settings:

Care staff should be alert to signs suggesting otosclerosis or progressive hearing loss:

• Gradual worsening of hearing despite hearing aid use
• Family history of early hearing loss or otosclerosis
• Resident reporting tinnitus or ear fullness
• Increasing social withdrawal or confusion
• Difficulty hearing even with properly functioning hearing aids

How Clear Ear Cheer supports care home residents with otosclerosis:

1. Mobile ear health assessments:

• We bring clinical ear health checks directly to care homes
• Otoscopic examination and hearing screening
• Identification of potential middle ear issues warranting specialist referral
• Regular monitoring to track hearing changes over time
• Can distinguish between earwax build-up and more complex hearing loss

2. Hearing aid optimization:

• Free hearing aid clinics: Regular cleaning and maintenance to ensure devices work optimally
• Professional cleaning removes wax and debris that reduces effectiveness
• Filter changes and battery replacements
• Checking fit and function to maximize benefit
• Troubleshooting whistling, poor sound quality, or comfort issues
• Education for care staff on proper insertion and daily care

3. Coordination with medical professionals:

• Working closely with GPs, audiologists, and ENT specialists
• Facilitating appropriate referrals for assessment and treatment
• Providing detailed observations for medical teams
• Supporting follow-up care after medical interventions
• Ensuring seamless care coordination for optimal outcomes

4. Earwax management:

• Safe, professional earwax removal that won't affect middle ear conditions
• Ensuring wax doesn't compound existing hearing difficulties from otosclerosis
• Regular maintenance to keep ear canals clear
• Using appropriate methods safe for residents with various ear conditions

5. Supporting duty of care:

• Helping care homes meet obligations for resident health monitoring
• Documentation for care plans and medical records
• Education for staff about hearing loss impact on wellbeing and cognition
• Person-centered approach respecting residents' dignity and preferences

Quality of life improvements:

Proper hearing health management for residents with otosclerosis can:

• Maximize remaining hearing through optimized hearing aids
• Improve communication with family, friends, and care staff
• Reduce isolation and support mental wellbeing
• Enable better participation in activities and social engagement
• Help maintain cognitive function by reducing sensory deprivation
• Support safety by improving ability to hear warnings and instructions
• Enhance dignity through better communication and connection
• Reduce frustration and agitation related to hearing difficulties

Decision-making about surgery for older residents:

For care home residents, stapedectomy may still be an option if:

• Overall health is good enough for surgery
• Resident can participate in decision-making or has family advocacy
• Hearing aids are insufficient or poorly tolerated
• Quality of life would be significantly improved

However, many older residents do very well with properly maintained hearing aids, avoiding surgical risks. The decision should be made in consultation with family, GP, and ENT specialist based on individual circumstances.

We understand that care home residents deserve accessible, compassionate ear care. Our mobile services ensure that even residents with complex needs receive the hearing health support necessary to stay engaged, connected, and cheerful.

At Clear Ear Cheer, we're passionate about delivering cheerful, professional ear care across the UK, especially in Mid & South Essex and Suffolk & North East Essex, where NHS ear care services may be limited or have extended waiting times.

Our mission includes:

• Providing clear, evidence-based information about ear conditions and hearing health
• Offering expert mobile ear care services that bring professional care directly to you
• Supporting individuals, families, and care homes with personalized guidance
• Campaigning for better access to ear health care for everyone
• Working collaboratively with medical professionals for comprehensive patient care

Our role in otosclerosis care:

While diagnosis and treatment of otosclerosis remain with GPs, audiologists, and ENT specialists, Clear Ear Cheer supports the broader hearing health journey:

• Identification: Recognizing potential progressive hearing loss that warrants specialist referral
• Optimization: Ensuring earwax doesn't compound hearing difficulties
• Hearing aid support: Maintaining and optimizing hearing aids for those using them alongside or instead of surgery
• Ongoing monitoring: Regular ear health checks to track changes and ensure optimal hearing aid function
• Education: Helping patients and caregivers understand otosclerosis and treatment options
• Accessibility: Bringing services to those with mobility challenges or transport difficulties

Who we serve:

• Care home residents with otosclerosis requiring hearing aid maintenance and ear health monitoring
• Individuals with hearing loss needing comprehensive ear care support
• Families seeking information and guidance about hearing conditions
• Anyone in our service areas requiring professional, accessible ear health services

Advocacy for better services:

We're actively campaigning to improve access to ear health and hearing services. We believe everyone deserves timely diagnosis, treatment options, and ongoing support for hearing conditions like otosclerosis. Hearing is fundamental to quality of life, communication, and independence—it should never be compromised by lack of access to appropriate care.

Whether you're considering surgical options, successfully using hearing aids, or supporting a loved one with otosclerosis, we're here to ensure your ears stay as clear and cheerful as possible.