Nervus Intermedius Neuralgia is a quite rare but extremely disturbing condition among headache disorders.
This disease is also known among the public as “intra-ear neuralgia” and is characterized by sudden, sharp, electric shock-like pains in or around the ear.

In medical literature, it is also referred to as Hunt’s neuralgia or Wrisberg’s neuralgia.
It occurs as a result of involvement of the nervus intermedius, a branch of the seventh cranial nerve (facial nerve).
Due to its rarity and the complexity of pain distribution, it can often be misdiagnosed and can disturb patients for a long time.

What is Nervus Intermedius Neuralgia?

Nervus intermedius is a nerve located between the facial nerve and the auditory nerve, containing both sensory and autonomic fibers.
This nerve:

• Sends sensory fibers to the posterior wall of the external auditory canal,
• Provides taste sensation in the anterior 2/3 of the tongue,
• Stimulates submandibular and sublingual salivary glands.

Nervus Intermedius Neuralgia, which develops as a result of irritation or compression of this nerve, is characterized by unilateral, sudden onset, paroxysmal intra-ear pains.

The pain is typically:

• Short duration (seconds to minutes),
• Severe and burning,
• Electric shock-like

It is generally seen in individuals between 40-60 years of age and is slightly more common in women.
Due to the rarity of the disease, it can often be confused with trigeminal or glossopharyngeal neuralgia.

What Causes Nervus Intermedius Neuralgia?

Causal factors are examined in two groups as primary (idiopathic) and secondary (due to underlying causes).

1. Primary (Idiopathic) Causes

In some cases, no obvious cause can be found.
In this case, nerve damage generally occurs as a result of age-related degenerative changes, myelin sheath damage, or spontaneous irritation in nerve fibers.

2. Secondary (Underlying) Causes

a. Vascular Compression

It is one of the most common causes.
Pain develops as a result of vessels located in the cerebellopontine angle region (especially AICA or PICA branches) contacting and compressing the nerve.

b. Tumoral Compression

• Acoustic neuroma (vestibular schwannoma)
• Meningioma
• Lesions such as epidermoid cyst can cause neuralgia by compressing the nerve.
  Since these tumors grow slowly, pains generally intensify gradually.

c. Infectious Causes

• Herpes zoster virus (Ramsay Hunt syndrome) is the most important infectious cause.
  The virus can directly affect the nerve, leading to both pain and facial paralysis.
• Additionally, central nervous system infections such as meningitis and encephalitis can also trigger this condition.

d. Traumatic and Surgical Causes

Iatrogenic nerve damage can develop following head trauma, ear surgeries, or temporal bone fractures.

e. Inflammatory and Autoimmune Diseases

• Systemic diseases such as Multiple Sclerosis, sarcoidosis, or vasculitis can affect nerve fibers, leading to demyelination and pain.

Who Gets It?

• Generally between middle age (40-60),
• More common in women,
• It is thought to be more prone in people with a history of migraine or other cranial neuralgias.

Due to its rarity, the diagnostic process may be delayed; this can lead to serious deterioration in quality of life due to pain in patients.

What Are the Symptoms of Nervus Intermedius Neuralgia?

Nervus Intermedius Neuralgia (Wrisberg or Hunt’s neuralgia) is a rare nerve disease that progresses with severe and characteristic pains around the ear.
Since its symptoms are quite typical, it can generally be easily recognized by an experienced neurology or ENT specialist after secondary causes are excluded.

1. Character of Pain

The most prominent feature of the disease is the type and onset of pain.

• Pain is deep in the ear, burning, sharp, and electric shock-like.
• It is generally seen in paroxysmal (sudden onset, short duration) attacks.
• Attacks can last from a few seconds to several minutes and start suddenly, end suddenly.
• Patients often describe the pain as “a knife stabbing inside my ear” or “electric shock in my eardrum.”

These pains can generally repeat several times a day and may become pronounced during waking hours.

2. Localization of Pain

Due to the anatomical distribution of the nervus intermedius nerve, pain is generally felt in the following areas:

• External auditory canal and around the eardrum,
• Back part of the auricle,
• Temporal region (temple),
• Pains spreading to the lower jaw and upper neck can also be seen.

Pain is mostly unilateral; rarely can be in both ears.

3. Triggering Factors

There are some triggers that initiate pain attacks:

• Light touch to the ear or ear cleaning
• Chewing, swallowing, speaking, or laughing
• Water getting in the ear or pressure changes (e.g., air travel, diving)

These factors can trigger short-term but severe pain attacks by stimulating the nerve.

4. Taste and Saliva Disorders

The nervus intermedius nerve also plays a role in taste and salivary gland function.
Therefore, additional symptoms may be seen in some patients:

• Taste disorder:
  Decreased or altered taste sensation may develop in the anterior 2/3 of the tongue.
  Patients generally describe it as “food tastes different.”
• Decreased saliva production:
  Dry mouth may occur due to involvement of submandibular and sublingual glands.
  This condition especially becomes pronounced during meals.

5. Hearing and Other Ear-Related Symptoms

In some cases, accompanying ear-related complaints may also be present:

• Mild hearing loss,
• Tinnitus (ear ringing),
• Pressure or fullness sensation in the ear.

These symptoms may suggest underlying vascular compression or tumoral causes.

6. Attack Frequency and Severity

The frequency of pain attacks varies from person to person:

• In some patients, there are several brief attacks per day,
• In others, infrequent but prolonged pain periods may be observed.

The course of attacks may increase with stress, fatigue, air pressure changes, or ear manipulation.

In Summary

• Pain is sudden, sharp, and electric shock-like in or around the ear.
• It is generally unilateral and comes in brief attacks.
• Taste disorder, dry mouth, and ear ringing may accompany.
• Recognizing triggering factors and avoiding them can reduce attack frequency.

International Classification of Headache Disorders (ICHD-3) Diagnostic Criteria – 13.3.1 Nervus intermedius neuralgia

1. There are paroxysmal (sudden onset, short duration) pain attacks unilaterally in the distribution of the nervus intermedius.
2. Pain meets all of the following characteristics:
   • Can last from a few seconds to minutes.
   • Is of severe intensity.
   • Is shooting, stabbing, or sharp in quality.
   • Can be initiated by touching/stimulating a trigger zone (e.g., posterior wall of the auditory canal and/or around the ear, peri-auricular region).

Notes

• Pain is typically felt in the auditory canal, auricle, or mastoid region; sometimes can also be in the soft palate.
• Disorders related to saliva, taste (gustation) and/or lacrimation (tearing) may accompany.
• This diagnosis should be differentiated from other similar facial/nerve pains because the ear and surrounding area is a neurologically complex region (e.g., auriculotemporal nerve, glossopharyngeal, vagus).

How is Nervus Intermedius Neuralgia Diagnosed?

Nervus Intermedius Neuralgia diagnosis is made with careful evaluation by an experienced neurology specialist due to the nature of this rare and complex nerve pain.
The diagnostic process requires a multidisciplinary approach and includes detailed history taking, physical examination, imaging, and electrophysiological tests.

1. Detailed Anamnesis (Patient History)

The first and most important step of diagnosis is the patient’s pain description.
The specialist physician inquires about the following characteristics:

• Onset, duration, and severity of pain
• Localization of pain (especially intra-ear)
• Triggering factors (touch, chewing, swallowing, etc.)
• Presence of taste disorder or fullness sensation in the ear

This information plays a critical role in distinguishing Nervus Intermedius Neuralgia from trigeminal or glossopharyngeal neuralgia.

2. Neurological Examination

In physical examination, especially cranial nerve examination is of great importance.
The physician evaluates facial muscle movements, taste sensation, and intra-ear sensitivity.

• Triggering of pain with light touch to the external auditory canal is a finding with high diagnostic value.
• Taste sensation function in the anterior 2/3 of the tongue is checked with taste test.
• Facial nerve functions are evaluated to exclude other neuropathies.

3. Differential Diagnosis

Since Nervus Intermedius Neuralgia can be confused with other facial and ear pain causes, differential diagnosis should be done carefully.
Diseases to be considered in differential diagnosis:

• Trigeminal neuralgia
• Glossopharyngeal neuralgia
• Temporomandibular joint disorders
• Middle ear infections (otitis media)
• Atypical facial pain
• Temporal arteritis

Each of these conditions shows different pain patterns. An experienced physician can distinguish these differences through systematic evaluation.

4. Imaging Methods

Magnetic Resonance Imaging (MRI) is the gold standard in diagnosis.
High-resolution and contrast MR techniques are used:

• FIESTA (Fast Imaging Employing Steady-State Acquisition) sequence shows nerve and vascular structures in detail.
• The cerebellopontine angle and internal acoustic canal are carefully examined.
• Tumoral compression (e.g., vestibular schwannoma or meningioma) or vascular compression is detected.

5. Electrophysiological Tests

The following tests can be applied to objectively evaluate nerve functions:

• Blink reflex test
• Stapedius reflex
• Electrogustometry (taste test)

These tests help determine the degree of nerve damage and are guiding in treatment planning.

6. Diagnostic Block (Diagnostic Anesthesia Application)

In some cases, local anesthetic injection can be applied to confirm the diagnosis:

• Local anesthetic to the external auditory canal or
• Stellate ganglion block is applied.

Temporary relief in pain after this procedure is an important finding supporting the diagnosis.

7. Laboratory Tests

Laboratory tests can be done to investigate secondary causes:

• Inflammatory markers (CRP, ESR)
• Autoimmune antibody tests
• Infectious agent investigations (e.g., herpes virus panel)

These tests are helpful in detecting underlying causes such as infection, autoimmune disease, or systemic inflammation.

In Summary

• For correct diagnosis, detailed anamnesis, careful neurological examination, and advanced imaging methods should be used together.
• Diagnostic block and electrophysiological tests are powerful tools supporting the diagnosis.
• Early diagnosis enables avoiding unnecessary treatments and more effective pain control.

Nervus Intermedius Neuralgia Treatment

Nervus Intermedius Neuralgia treatment requires a special approach due to the rarity and complex nature of the disease.
The treatment plan is arranged personalized for each patient, taking into account pain severity, underlying cause, and general health status.

1. Medical (Medication) Treatment

a. Anticonvulsants

They are among the most effective drug groups in controlling neuropathic pain.
Gabapentin, Pregabalin, and Carbamazepine suppress pain signals by regulating abnormal electrical activity in nerve fibers.
These medications are generally used in first-line treatment and provide significant relief in many patients.

b. Tricyclic Antidepressants

Amitriptyline or Nortriptyline is effective both in reducing pain and controlling accompanying depression or anxiety.
These medications reduce the severity of neuropathic pain by balancing neurotransmitters involved in pain transmission.

c. Corticosteroids

In the acute period, especially if there is suspicion of viral infection (e.g., Herpes zoster), corticosteroids such as Prednisolone can be used for a short term.
Steroids can limit nerve damage by reducing inflammation around the nerve.

2. Interventional (Invasive) Treatment Methods

Invasive approaches come into play in patients who do not respond adequately to drug therapy.

a. Stellate Ganglion Block

This method provides pain control by temporarily blocking sympathetic nerve fibers.
It is applied with a combination of local anesthetic and steroid and provides long-term relief in many patients.

b. Radiofrequency Ablation

It is an advanced technology-based treatment method.
Pain-conducting fibers of the nerve are rendered ineffective in a controlled manner with heat energy.
It is a minimally invasive procedure and often does not require general anesthesia.
High success rates have been reported in resistant cases.

c. Botulinum Toxin (Botox) Injections

Can be used in cases accompanied by muscle spasm.
Botulinum toxin reduces pressure on the nerve by providing muscle relaxation and helps alleviate pain.

3. Surgical Treatment Options

Surgical treatment is only considered in selected patients where medical and interventional methods have failed.

• Microvascular Decompression Surgery:
  Aims to eliminate vascular compression on the nerve.
  It is especially applied in cases where vascular compression is detected.
• Gamma Knife Radiosurgery:
  In patients not suitable for surgery, it is a non-invasive alternative that provides relief from nerve compression with focused radiation.

4. Supportive and Rehabilitation Approaches

a. Physical Therapy and Exercise

Exercises that provide relaxation of neck, jaw, and facial muscles support pain control.
Posture correction exercises, relaxation techniques, and TENS (Transcutaneous Electrical Nerve Stimulation) devices can be used.

b. Psychological Support

Chronic pain can create psychological symptoms such as anxiety and depression over time.
Therefore, psychotherapy, cognitive behavioral therapy, or antidepressant treatment if necessary can be applied as supportive.

5. Alternative and Complementary Treatment

In some patients, in addition to conventional treatment, acupuncture, bioresonance, or homeopathic approaches can be applied.
The effectiveness of these methods is supported by limited evidence; however, they may be beneficial in some patients in terms of increasing pain threshold and reducing stress.

6. Lifestyle Modifications

Treatment success should be supported with lifestyle changes:

• Stress management techniques (meditation, breathing exercises)
• Regular sleep and healthy eating habits
• Avoiding factors that trigger pain (cold weather, fatigue, excessive caffeine, alcohol)
• Regular doctor check-ups and medication compliance

These measures reduce the risk of disease flare-up and increase long-term quality of life.

Treatment and Follow-up Process

• Patients should be checked at regular intervals.
• Dose or medication changes can be made by evaluating treatment response.
• The best results are obtained with multidisciplinary approaches where neurology, ENT, pain treatment, and physical therapy specialists work together.

Prognosis (Course of Disease)

Nervus Intermedius Neuralgia responds well to treatment in most patients.
With early diagnosis and appropriate treatment, complete disappearance or significant reduction of pain is possible.
However, in chronic cases, long-term follow-up and treatment may be required.
If the underlying cause is a tumoral lesion, prognosis depends on the treatment of this condition.

Frequently Asked Questions

1. Is Nervus Intermedius Neuralgia a Rare Disease?

Nervus Intermedius Neuralgia is an extremely rare disease that accounts for only 1-2% of all neuralgia (nerve pain) cases.
It is seen in approximately 1-2 out of 100,000 people per year worldwide.
This low incidence can lead to delays and misdiagnoses in the diagnosis of the disease.

The disease most commonly occurs in the 40-60 age range and is seen approximately twice as frequently in women compared to men.
Due to its rarity, many physicians may not encounter this condition in daily practice.
Therefore, it is important for patients experiencing recurrent electric shock-like pain around the ear to be evaluated by an experienced neurology specialist.

Early diagnosis prevents unnecessary treatments and interventions and significantly increases quality of life.

2. Does Nervus Intermedius Neuralgia Cause Facial Paralysis?

Nervus Intermedius Neuralgia does not cause facial paralysis.
The reason for this is that the nervus intermedius does not contain motor fibers.
This nerve consists only of sensory and parasympathetic fibers; therefore, motor nerves that move facial muscles are not affected by this process.

Therefore, in patients with Nervus Intermedius Neuralgia:

• Weakness in facial muscles,
• Asymmetry,
• Facial paralysis findings such as inability to close the eyelid are generally not seen.

When Can Facial Paralysis Develop?

If the patient also has facial paralysis symptoms, this situation indicates a much more serious condition than simple nervus intermedius irritation.
In this case, the physician should investigate the presence of a cause affecting the entire facial nerve complex.
Possible causes include:

• Acoustic neuroma (vestibular schwannoma),
• Meningioma,
• Other tumors located in the cerebellopontine angle region

Such lesions can affect both motor and sensory fibers, leading to a combined pain + facial paralysis picture.

Situations Where You Should Consult a Doctor

If recurrent electric shock-like pain around the ear is accompanied by facial asymmetry, speech disorder, or difficulty closing the eye, this is a situation requiring emergency evaluation.
MRI examination and neurological examination performed early play a critical role in detecting underlying serious causes.

3. How Long Does Treatment Take and Is Permanent Recovery Possible?

Nervus Intermedius Neuralgia treatment generally requires a long-term process.
The treatment plan is personalized according to the cause, severity of the disease, and the patient’s general health status.

How Long is Treatment Duration?

• In approximately 60-70% of patients receiving medical (medication) treatment, significant improvement is achieved within 2-6 months.
• During this period, pain frequency decreases, attacks progress more mildly, and quality of life increases.
• However, in some patients, long-term continuation of medication therapy may be required due to continuation of nerve sensitivity.

In cases where invasive or interventional treatment (e.g., nerve block, radiofrequency ablation) is applied, 80-90% success rate has been reported.

Is Permanent Recovery Possible?

Yes, permanent recovery is possible.
However, this depends on the underlying cause of the disease:

• In idiopathic (unknown cause) cases, prognosis is generally very good.
• In cases due to tumoral or vascular compression, recovery varies depending on the treatment of the underlying cause.

While long-term remission (complete disappearance of attacks) is achieved in some patients, others may experience pain that recurs from time to time.

To Increase Treatment Success

• Not neglecting regular doctor check-ups
• Adhering to medication dose and duration
• Avoiding triggering factors (stress, sleeplessness, alcohol, etc.)
• Continuing rehabilitation and supportive treatments (e.g., physical therapy, relaxation exercises)

The treatment process requires patience.
With early diagnosis and correct treatment, long-term or even permanent recovery is largely possible.

4. How Much Does This Disease Affect Quality of Life?

Nervus Intermedius Neuralgia, although it affects anatomically a small nerve, can significantly reduce quality of life due to the severe and sudden pain attacks it creates.

Effects on Daily Life

• Simple facial movements such as eating, speaking, laughing can trigger pain.
• Patients, due to fear of these attacks recurring, can withdraw from social environments, change eating habits, or experience decreased work performance.
• The constant anxiety of pain triggering can lead to psychological problems such as anxiety, depression, or sleep disorders over time.
• In some patients, alteration in taste sensation occurs, which affects both eating pattern and general life enjoyment.

Quality of Life Can Be Regained with Treatment

When appropriate medical or interventional treatment is applied, quality of life improves significantly in 80-85% of patients.
With pain attacks being controlled:

• Daily activities become doable again,
• Social and professional life returns to normal,
• Psychological well-being increases significantly.

Multidisciplinary approach (cooperation of neurology, pain, psychiatry, physical therapy) is the most important key to this process. Although Nervus Intermedius Neuralgia appears to be a disease of a small nerve, the life area it affects is very large. Early control of pain enables patients to recover both physically and emotionally.

5. Can Recurrence of the Disease Be Prevented?

Although Nervus Intermedius Neuralgia is a disease with chronic tendency, the recurrence risk can be largely reduced with correct measures.
In approximately 20-30% of patients after treatment, symptoms may reappear after a certain period.

Ways to Prevent Recurrence

1. Avoid Triggers:
   • Direct exposure to cold weather,
   • Water getting in the ear,
   • Movements such as prolonged speaking or excessive chewing can trigger pain again.
2. Regular Medication Use:
   Medications used in treatment (e.g., anticonvulsants, antidepressants) should not be stopped suddenly, dose reduction should definitely be done under doctor control.
   Sudden medication discontinuation can increase recurrence risk by increasing nerve sensitivity.
3. Stress Management:
   In chronic pain diseases, stress is an important triggering factor. Meditation, breathing exercises, yoga, or relaxation techniques can reduce recurrence risk.
4. Regular Check-ups:
   When early signs of the disease (stinging in the ear, brief electrification sensation, etc.) are noticed, early intervention can prevent recurrences from progressing.

Is Long-term Well-being Possible?

Yes. In patients applying lifestyle measures in addition to appropriate treatment, 70-80% long-term remission (periods when attacks completely disappear) can be achieved.
Multidisciplinary follow-up (neurology, otolaryngology, pain clinic) plays a decisive role in this success.