Relapsing Polychondritis and Inner Ear Involvement - An Overview

Introduction:

Relapsing inflammation, deformity, and malfunction are the main effects of the uncommon autoimmune condition known as polychondritis, which mostly affects the body's cartilage-rich structures. The influence of RP on the inner ear is one of its less-examined effects, despite the fact that it is well known for its involvement in several anatomical locations. There is a critical need for prompt diagnosis and treatment since inner ear involvement in RP can result in a wide range of symptoms, including hearing loss and vestibular problems.

What Is Relapsing Polychondritis?

Relapsing polychondritis (RP) is a rare and chronic autoimmune disorder marked by recurrent episodes of inflammation primarily affecting cartilage-rich structures throughout the body. Cartilage, the flexible connective tissue located in regions such as the ears, nose, joints, and more, becomes the target of the immune system's attack in individuals with RP. This condition has the potential to impact individuals across all age groups, although it is most commonly seen in middle-aged adults. The appearance of symptoms may be gradual, with instances of inflammation recurring periodically. The intensity and frequency of these occurrences can significantly differ among those who are affected. Auricular involvement is the most common presentation of relapsing polychondritis, occurring in the majority of cases (89 percent). Bilateral engagement is observed in nearly all instances (95 percent). The earlobe often exhibits characteristics such as pain, redness, and lobulation. As the earlobe lacks cartilaginous composition, it remains unaffected by the condition.

What Are the Clinical Features of Relapsing Polychondritis With Inner Ear Involvement?

Monolateral, or more frequently, bilateral inflammation of the ear cartilage is the most common presentation in RP, affecting about 90 % of patients at some point during the disease course. It can even be the initial symptom in around 20 % of cases. The onset is sudden, marked by painful reddish-purple skin and swelling limited to the cartilaginous portion of the ear. Interestingly, the earlobe, which lacks cartilage, is usually unaffected. These acute inflammatory episodes tend to naturally resolve within a few days or weeks, with recurrences happening at varying intervals.

Over time, the repeated bouts of inflammation lead to significant cartilage damage. The original cartilage matrix is progressively replaced by fibrous connective tissue. Consequently, the ear's normal appearance undergoes changes, appearing nodular, bumpy, and even calcified in some cases. Occasionally, the ear deformation can resemble the ‘cauliflower ear’ often seen in professional boxers.

Hearing loss is a common issue in RP, affecting up to 46 percent of patients. This loss can either be conductive or sensorineural. Conductive hearing loss can result from the collapse of auricular cartilage, canal edema, closure of the ear canal leading to serous otitis media, or fixation of the stapedial footplate. On the other hand, sensorineural hearing loss might be caused by inflammation of vestibular structures or vasculitis of the internal auditory artery. RP patients may experience otitis externa (inflammation of the external ear canal, which connects the external ear to the eardrum), chronic myringitis (inflammation of the eardrum, also known as the tympanic membrane), and tinnitus (perception of sound, such as ringing, buzzing, or clicking, in the ears when no external sound source is present) as well.

How Can Relapsing Polychondritis With Inner Ear Involvement Be Diagnosed?

A detailed medical history is essential to identify characteristic symptoms and assess the progression of the disease. During the physical examination, healthcare providers will focus on identifying signs of inflammation in the ears, nose, joints, and other cartilage-rich structures. The steps involved in diagnosing RP with inner ear involvement include:

• Audiological Assessments: Audiometric tests are crucial for evaluating hearing function. These tests, such as pure-tone audiometry and tympanometry, measure the patient's ability to hear different frequencies and assess middle ear function.

• Imaging Studies -

  1. Magnetic Resonance Imaging (MRI): MRI scans of the head and inner ear can provide detailed images of cartilaginous structures, revealing any signs of inflammation, damage, or deformities.

  2. Computed Tomography (CT) Scan: CT scans may also be used to visualize the inner ear structures and assess for abnormalities.

  3. High-Resolution Temporal Bone CT: This specialized CT scan focuses specifically on the temporal bone and can provide detailed images of the inner ear anatomy.

• Laboratory Tests:

  1. Inflammatory Markers: Blood tests measuring markers of inflammation, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), can provide supportive evidence of ongoing immune activity.

  2. Autoantibody Testing: Some RP cases are associated with the presence of specific autoantibodies, such as anti-collagen type II antibodies. The detection of these antibodies can help with diagnosis.

  3. Rheumatoid Factor (RF): RF may be elevated in some RP cases, contributing to the diagnostic evaluation.

• Biopsy: At times, a biopsy of affected cartilage, such as the auricular cartilage of the ear, may be performed to confirm the presence of inflammation and rule out other conditions.

Given the complex nature of RP with inner ear involvement, consultation with various specialists may be necessary. This includes rheumatologists, otolaryngologists (ear, nose, and throat specialists), and audiologists who can collectively contribute to the diagnostic process.

What Is the Treatment for Relapsing Polychondritis With Inner Ear Involvement?

The treatment strategies for Relapsing polychondritis with inner ear involvement are listed below:

• Symptomatic Relief: For cases of mild relapsing polychondritis affecting the ear, treatment options include nonsteroidal anti-inflammatory drugs (NSAIDs) or Dapsone.

• Immunosuppressive Medications: Corticosteroids, such as Prednisone, are often the first line of treatment to suppress the immune system's abnormal response and reduce inflammation.

  1. A common approach involves initiating a gradually decreasing dose of the corticosteroid Prednisone as symptoms subside.

  2. In situations where symptoms persist without improvement, corticosteroid reduction might be challenging. In these instances, Methotrexate could be administered to minimize corticosteroid reliance.

  3. In more severe scenarios, highly potent immunosuppressive medications such as Cyclosporine, Cyclophosphamide, or Azathioprine might be considered.

  4. Medications can be prescribed to manage specific symptoms associated with inner ear involvement. For instance, anti-dizziness medications like antihistamines or vestibular suppressants can help alleviate vertigo and dizziness.

• Biologic Therapies: For individuals with refractory RP, biologic therapies designed to focus on specific elements within the immune system, such as tumor necrosis factor (TNF) inhibitors, may be considered. These medications aim to dampen the immune response and curb inflammation. Drugs targeting a chemical called tumor necrosis factor, such as Infliximab or Etanercept, could be employed. It is important to note that while these treatments address symptoms, they have not demonstrated the ability to alter the underlying progression of the disorder.

• Hearing Aids and Assistive Devices: For individuals experiencing sensorineural hearing loss, hearing aids can be beneficial in improving auditory function and enhancing communication. Assistive devices may also be recommended to help with tinnitus management.

• Vestibular Rehabilitation: Physical therapy and vestibular rehabilitation exercises can assist in improving balance, reducing vertigo, and enhancing overall vestibular function.

• Surgical Intervention: In severe cases where cartilage damage is extensive and function is significantly compromised, surgical interventions may be considered. Cochlear implants, which provide auditory signals directly to the auditory nerve, can be an option for individuals with profound hearing loss.

Regular follow-up appointments with healthcare providers, including rheumatologists, otolaryngologists, and audiologists, are essential to track disease progression, adjust treatment strategies, and manage any emerging complications. Making lifestyle changes, such as reducing stress, maintaining a healthy diet, and avoiding triggers that exacerbate inflammation, can complement medical treatment and promote overall well-being.

Conclusion:

Relapsing polychondritis (RP) with inner ear involvement exhibits a complex interplay between autoimmune dysfunction and inner ear structures, resulting in a variety of symptoms such as hearing loss, vestibular disturbances, and tinnitus. Although this rare disorder can be difficult to diagnose and manage, a thorough approach involving medical, audiological, and imaging assessments, as well as focused therapeutic interventions, can help patients' lives be less negatively impacted by it. Patients suffering from RP and its inner ear manifestations must receive diagnosis and treatment as soon as possible to maximize outcomes and improve quality of life.