Introduction

Tympanosclerosis can be described as a condition that develops through a scarring process during a condition called otitis media. This condition can be observed in the middle ear and eardrum. The incidence of tympanosclerosis is around 10%.¹ Damage to the nerve linked to hearing, the main brain areas, or the tiny hair cells in the inner ear can lead to sensorineural hearing loss. This can be considered the most frequent type of hearing loss with a higher number of cases.² 

The ear is made of three major parts: the inner ear, the middle ear, and the external ear. Here we mainly focus on the middle ear. The middle ear consists of the eardrum, also known as the tympanic membrane, ossicles (malleus, incus, and stapes), cochlea, eustachian tube opening, and oval and round windows.^2,3 

Etiology and pathogenesis of tympanosclerosis

The main etiology of tympanosclerosis is unknown. Possible causes may be an infection of the eardrum, diseases where the body attacks itself (autoimmune diseases), and genetic factors identified by genetic tests.⁴ The pathology of tympanosclerosis is known to have four stages. Inflammation and fibrogenesis marking the first stage, followed by degeneration of cells, calcification of the tympanic membrane, and decalcification and healing process marking the final stage of tympanosclerosis.⁵

Sensorineural Hearing Loss (SNHL)

Sensorineural hearing loss (SNHL) happens when there's damage to the inner ear or the nerve pathways responsible for hearing. The pathological features involved include infections, loud noise, trauma, ischemia, aging, drugs that can cause damage to the ears, and autoimmunity. Medications that cause ototoxicity include aminoglycosides, cisplatin, and carboplatin.⁶

Etiology and pathogenesis of Sensorineural Hearing Loss (SNHL)

Etiology 

Common factors leading to SNHL include

• Congenital - syndromic and nonsyndromic
• Presbycusis
• Noise-induced hearing loss
• Head injury
• Meniere's disease
• Ototoxicity due to aminoglycosides, loop diuretics, and some chemotherapeutic agents like cisplatin and carboplatin
• Systemic conditions such as meningitis, diabetes
• Vestibular schwannoma
• Other factors include autoimmune, barotrauma, and perilymphatic fistula

Pathogenesis of SNHL

The pathogenesis of SNHL can occur in various processes such as:

1. Abnormality in the structure of the components of the cochlea, which can be due to trauma or any congenital deformities
2. Uneven metabolic work due to abnormal ion movement in the inner ear space
3. Changes in the vascular distribution to the cochlea in conditions like ototoxicity and noise trauma
4. Diabetes and autoimmune conditions leading to a buildup of basilar membrane² 

Diagnostic Approach

Complete audiometric analysis should be performed 

1. Physical examination: Ear canal examination and visibility of tympanic membrane⁷
2. Weber and Rinne tests: in these tests, diagnosis is made using a tuning fork of 256 or 512 Hz^7,8
3. Otoscopy: Presence of white plaques in the middle ear or tympanic membrane⁹
4. Pneumatic otoscopy, otoacoustic emissions, audiometry, electrophysiological tests, and tympanometry can aid in the process of diagnosis^2,7 
5. Laboratory tests are usually unnecessary, but in some cases of autoimmune SNHL, erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), rheumatoid factor, and anti-microsomal antibodies may be performed² 

Treatment 

Surgical treatment involves removing the tympanosclerosis while preserving normal ossicular continuity. If tympanosclerosis is not treated accordingly, in rare instances, it can lead to sensorineural hearing.^4,10 Infants diagnosed with sensorineural hearing loss may be managed by surgical therapy, as cochlear implantation can be performed in infants as old as 6 months. In severe cases, cochlear implantation can also be done in adults.⁷

Individuals with acute SNHL, if the cause of hearing loss is not ruled out, will be treated with prednisolone 1mg/kg/day as a single dose with a maximum dose of 60 mg/day, which is an oral corticosteroid. In chronic cases, hearing aids are the main standard of care. The duration of treatment usually is from 10 to 14 days.^2,7 If the individual does not respond to systemic corticosteroid therapy, then intratympanic steroids such as dexamethasone at a dose of 10 to 24 mg/ml and solumedrol at 30 mg/ml may be used.⁷

Patients can also use Roger pens or personal frequency modulation systems. These are cordless tools used with hearing aids. This will help the individuals pay attention to the speech they want to hear.²

Complications of hearing loss 

• Delay in speech 
• Depression
• Reduction in quality of life
• Tinnitus
• Dizziness
• In many instances, individuals may isolate themselves from family and friends
• Inability to concentrate 
• Sleep deprivation²

Patient education 

Telling patients about their health helps them get better. This can be done by providing patient information leaflets, communicating with them frequently, educating the friends and family members about their condition, and asking them to go for regular health checkups. Counselling the patients during their visit to the healthcare facility about the complications and treatment effects. 

Recommendations

In nasopharyngeal carcinoma patients who lose hearing after radiotherapy, ear massage can help stop or slow down hearing loss and make their lives better.¹¹ This can be implemented even in case of tympanosclerosis and sensorineural hearing loss patients, so that we can observe any changes in the effect of hearing in these patients.   

Summary 

Hearing loss can occur due to various causes; identifying it and getting treatment in the initial stage is crucial. There are various types of hearing loss, this topic gives detailed information on tympanosclerosis and sensorineural hearing loss. Family members, friends, and peers should help the individuals suffering from hearing loss by accompanying them to visit the health care facility for proper medical treatment. Early diagnosis and management will help individuals with hearing loss get better treatment during the initial stages.  

FAQs

Can we take corticosteroids for a long time? 

No, taking them for too long can lead to side effects like gaining weight, and rhabdomyolysis. 

Does tympanosclerosis and sensorineural hearing loss (SNHL) impact daily activities?

Yes, these conditions can impact your day-to-day activities to some extent, but if treatment is started as soon as the symptoms appear, it won't affect your routine work. 

Can we get our hearing back to normal? 

No, if you lose your hearing, you can't get it back to normal.

Can hearing loss be congenital?

Yes, one of the causes for loss of hearing is congenital. 

When should I see a doctor?

You should see a doctor as soon as you notice that your hearing ability is diminishing.