Ear, Nose, Throat, Head and Neck Surgery Specialist
Prof. Dr. Murat Topdağ

Born in Malatya in 1978, Murat Topdağ completed his primary and secondary education, then attended high school in Istanbul. He graduated from the English program of Cerrahpaşa Tıp Fakültesi. Performing his surgeries at Acıbadem Altunizade Hospital, Prof. Dr. Murat Topdağ is married and has two children.

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What Is an Inner Ear Tumor?

A vestibular schwannoma is a benign tumor originating from the balance and hearing nerves in the inner ear. This slowly growing tumor forms due to the uncontrolled production of Schwann cells, known for their role in surrounding nerve fibers. As the tumor grows, its effects on hearing and balance functions increase. Hearing loss is usually unilateral and can cause symptoms such as tinnitus. Balance problems and dizziness are also commonly observed. In advanced stages, the tumor may press on the trigeminal nerve, leading to numbness in the face. Additionally, serious issues such as facial paralysis or paralysis of the facial muscles may occur. When the tumor grows larger:

• It can exert pressure on the brainstem,
• and on nearby brain structures such as the cerebellum, posing life-threatening risks. Schwannomas usually occur individually and in 90% of cases appear this way. The presence of multiple tumors may indicate genetic syndromes, which include:
• Neurofibromatosis Type 2,
• Schwannomatosis,
• Carney Complex.

These types of tumors are classified as Grade 1 (benign) by the World Health Organization. Schwannomas are nerve sheath tumors that can arise from any myelinated nerve.

Types and Characteristics of Tumors in the Inner Ear

Tumors in the inner ear can affect hearing and balance functions. Inner ear tumors typically develop around the endolymphatic sac and the facial nerves. These tumors may cause various symptoms such as hearing loss, ear pain and numbness, and dizziness. The treatment of inner ear tumors varies depending on the location and type of tumor.

Treatment options include surgical intervention, radiotherapy, and chemotherapy. Known to be benign, these tumors generally originate from the nerve sheath and are treated surgically. However, in some cases, surgical intervention can be risky.

In particular, tumors originating from the facial nerve may remain untreated unless they cause facial paralysis. If facial paralysis occurs, the tumor is removed, and the nerve is repaired. Some of the tumors in the inner ear include:

1. Acoustic tumor or vestibular schwannoma: These tumors develop from nerves related to balance and originate from the nerve sheath.
2. Endolymphatic sac tumors: These are malignant, typically have a poor prognosis, and require extensive removal of the ear bone.

Early diagnosis and proper treatment methods greatly affect the quality of life of patients.

Variety and Characteristics of Inner Ear Tumors

Inner ear tumors are primarily categorized into two main groups, endolymphatic and acoustic tumors. Each tumor type develops in different parts of the inner ear and possesses distinct characteristics. The growth process and effects of these tumors in the inner ear directly influence the treatment approach.

Acoustic Tumors:

1. Benign tumors.
2. They originate from the sheaths of the balance nerves.
3. Their average annual growth rate is around 1.5-2 mm.
4. There are two main areas of development:

• Those growing in the internal auditory canal, where bony walls cause slow growth.
• They press on the hearing nerve, leading to early hearing loss.

1. Despite being close to the facial nerve, the facial nerve generally resists chronic compression, so facial paralysis usually appears in the later stages.

Endolymphatic Tumors:

1. They are malignant and follow a more aggressive course.
2. They originate in a region called the endolymphatic sac.
3. Due to their proximity to the brainstem, surgical intervention can be challenging.
4. They are generally treated with radiotherapy and chemotherapy.

These tumors directly affect inner ear functions. In particular, in acoustic tumors, those extending outside the internal auditory canal may grow faster. In such cases, since the pressure on the hearing nerve is less, hearing loss develops at a later stage.

These silently progressing tumors may show no symptoms until they press on the brainstem. Both types of tumors seriously affect inner ear health, lowering a person’s quality of life. Early diagnosis and appropriate treatment methods play a significant role in reducing the impact of these tumors.

The Importance of Early Diagnosis in Inner Ear Tumors

Early diagnosis is critical in the treatment of inner ear tumors. Detecting tumors in the early stage expands treatment options and increases the chance of recovery. Initial symptoms are often mild and can be overlooked; however, careful observation and early intervention can prevent serious health problems.

Common symptoms of inner ear tumors include persistent hearing loss, ear-related pain and numbness, imbalance, and dizziness. Seeking medical help promptly when these symptoms are observed is essential.

1. If you experience persistent hearing loss
2. If you feel pain and numbness around the ear
3. If frequent imbalance and dizziness occur

These signs may indicate the presence of an inner ear tumor, suggesting the need to consult a healthcare professional right away. Thanks to early diagnosis, it is possible to prevent tumor growth and preserve the patient’s quality of life. Therefore, the first signs of an inner ear tumor should be taken seriously, and a specialist doctor should be consulted without delay.

What Are the Causes of Inner Ear Tumors?

The main causes of inner ear tumors arise from genetic factors. Schwannomas are particularly common tumor types found in the inner ear. Most schwannomas appear sporadically, but certain special cases link these tumors to specific genetic syndromes.

• Neurofibromatosis Type 2 accounts for 3% of schwannomas. This syndrome is characterized by NF2 gene mutations that inhibit the production of the merlin protein.
• Schwannomatosis accounts for 2% of schwannomas. Similar genetic influences can be observed in this syndrome as well.
• Carney complex rarely leads to schwannomas, and changes in the PRKAR1A gene are significant in such cases.

Additionally, the NF2 gene located on chromosome 22 plays a central role in the development of both sporadic and syndromic schwannomas. Inactivation of this gene contributes to schwannoma formation in both types of cases. Along with these genetic factors, mutations in the SMARCB1 gene can also trigger tumor development in spinal schwannomas.

These genetic influences are critically important in understanding how inner ear tumors develop. Although most schwannomas are sporadic, genetic predisposition is more noticeable in syndromic cases.

Stages of Inner Ear Tumors

It is important to note that the AJCC does not provide an official TNM staging system for inner ear tumors. Instead, the Koos and Hannover grading systems are used. Both systems define tumor stages based on extracanalicular spread and the extent of brainstem compression.

The Koos Grading System consists of four stages:

• Grade 1: The tumor is completely intracanalicular.
• Grade 2: The tumor extends into the cerebellopontine angle.
• Grade 3: The tumor is in contact with the brainstem.
• Grade 4: The tumor has displaced the brainstem.

The Hannover Grading System is more detailed:

• T1: The tumor is intracanalicular.
• T2: The tumor is intrameatal and/or extrameatal.
• T3a: The tumor is within the cerebellopontine cistern.
• T3b: The tumor is in contact with the brainstem.
• T4a: The tumor is compressing the brainstem.

These grading systems provide a basis for evaluating tumor size and spread, thereby guiding treatment planning. Consequently, both systems are regarded as reliable in medical practice and are widely employed. Each system offers different criteria for assigning a stage to the tumor, and medical decisions are made according to these criteria.

Diagnosis and Treatment Methods for Inner Ear Tumors

Various methods are used to diagnose inner ear tumors. Audiometry measures hearing levels and determines hearing loss. Magnetic Resonance Imaging (MRI) offers a detailed view of the tumor’s location and size. These methods assist in accurately diagnosing the tumor.

Among the treatment options are:

1. Surgery: Aims to completely remove the tumor. It is the most effective method but carries the risk of complications.
2. Radiotherapy: Uses high-energy rays to shrink or destroy the tumor. Its side effects are fewer, but it may not achieve complete cure.
3. Chemotherapy: Uses drugs that target cancer cells. It can be effective but may cause strong side effects.

The advantages and disadvantages of each treatment method must be evaluated according to the patient’s condition and the tumor’s characteristics. In treating inner ear tumors, a multidisciplinary approach ensures that the patient’s overall health and quality of life are taken into account in determining the most appropriate treatment plan.

Postoperative Recovery Process

After the operation, the patient typically spends one night in intensive care. Light mobilization begins the following day. The patient is helped to sit up within the first few days. The tight head dressing placed initially is kept for five days. During this period, the head dressing is crucial to reduce the risk of CSF leakage and swelling. Once the dressing is removed, the patient may shower using the waterproof wound covering.

• Recovery usually involves a hospital stay of five to ten days.
• After returning home, some physical and psychological setbacks can occur.

Paper tapes on the surgical site are left in place for two weeks. Headache is common during the first few weeks, and painkillers are administered regularly. Vestibular rehabilitation is recommended for dizziness. If the patient’s condition is not under control, the doctor can directly send a prescription to the pharmacy.

• Sometimes temporary facial nerve palsy may occur.
• If the eye does not fully close, drops and gels are used instead of tears.

Any fever, increased swelling, or tenderness around the wound should be reported immediately. It is important to contact the doctor with any concerns until the first follow-up appointment. The first postoperative visit usually takes place about 2-3 weeks later.

Dietary and Lifestyle Changes for Postoperative Patients

After vestibular schwannoma surgery, patients are advised to make key dietary and lifestyle changes to support the recovery process and maintain overall health. Certain fundamental steps should be followed so that the body remains healthy and the healing process accelerates.

Hydration is very important; therefore, drinking plenty of water is essential. A balanced and nutritious diet is also critical in supporting recovery. Eating nutrient-rich foods supplies the vitamins and minerals that the body needs.

Additionally, reducing salt intake can help prevent fluid retention and high blood pressure. Limiting caffeine and alcohol can also be beneficial during recovery, as these substances may negatively affect sleep patterns and the effectiveness of certain medications.

Rest is the most significant factor in the recovery process. Adequate sleep is vital for the body’s repair. Adjusting physical activity according to the doctor’s recommendations and participating in physical therapy can aid in improving balance, coordination, and muscle strength.

Stress management techniques are equally crucial. Methods like meditation, deep breathing, or yoga help reduce stress, thus positively supporting the healing process. Doctor visits enable regular checkups on health status and early detection of any issues.

Additionally:

• Keeping the head elevated during rest can reduce swelling and enhance comfort.
• Avoiding strenuous activities in the initial weeks is important.
• Protecting the ears from loud noise and water minimizes the risk of infection.

These recommendations help patients who undergo inner ear tumor surgery recover more quickly and effectively.

Frequently Asked Questions about Inner Ear Tumors

Are ear tumors dangerous?

Most ear tumors are benign and generally not dangerous. However, some can affect hearing or balance, in which case treatment may be necessary. Cancerous ear tumors are rarer and often present as skin cancer. Such tumors must be treated, as they can lead to serious health problems if left untreated. Therefore, not every ear tumor is dangerous, but some do require treatment.

What happens if an ear tumor is not treated?

If cholesteatoma is not treated, it can cause serious health issues. Initially, it erodes the ear bone and damages the structures in the middle ear responsible for hearing. Over time, this damage can lead to permanent hearing loss. In advanced stages, facial paralysis may occur. More severe conditions can involve a risk of meningitis. An abscess may also form in the brain or cerebellum. Damage may occur in the inner ear, which can cause permanent balance problems.

What is a glomus tumor in the ear?

A glomus tumor in the ear is a slow-growing, vascular-origin tumor. These tumors are more commonly seen in women over 40. Glomus tympanicum is found in the middle ear, while glomus jugulare may enter the ear from the neck area. Early symptoms may include hearing loss and a pulsating sound similar to a pulse. In more advanced stages, dizziness and facial paralysis may occur. On examination, a red mass behind the eardrum is typically seen.

Can an ear tumor damage the brain?

Yes, an ear tumor can damage the brain. When the tumor grows, complete hearing loss may result. Large tumors can injure the nerve that controls facial muscles, potentially causing permanent facial paralysis. Very large tumors can press on the brainstem and disrupt the normal flow of cerebrospinal fluid. If left untreated, a growing acoustic neuroma can lead to dangerous fluid buildup in the brain. This situation can put pressure on the cerebellum and brainstem, posing a life-threatening risk.

Which doctor should I see for an ear tumor?

Patients with serious disorders like an ear tumor should definitely consult an ENT (Ear, Nose, and Throat) specialist. First and foremost, you need to visit an ENT doctor for the diagnosis and treatment of such conditions. However, oncology specialists may also be involved in the treatment process. Therefore, you should begin with an ENT doctor. If treatment is needed, referrals to the relevant specialists can be made. For the most accurate and effective treatment, visiting an ENT specialist is the first step.

How long does ear tumor surgery take?

Ear tumor surgery generally lasts 6-12 hours. The exact duration depends on the size of the tumor. The tumor’s location also influences the length of the procedure. The surgery time may vary for each patient, but these types of surgeries usually last a long time. The surgeon’s experience and the tumor’s accessibility can also affect the surgery duration.

Is there a chance of survival for a Stage 4 inner ear tumor?

For patients with a fourth-stage inner ear tumor, the two-year overall survival rate has been identified as 67.7%. Additionally, these patients have a 57.8% disease-free survival rate. This data indicates that patients with Stage 4 inner ear tumors have a certain likelihood of survival for a period of time. Therefore, there is a chance of recovery for such patients.

It can be misleading to self-diagnose by looking at images or photos of people with ear cancer; if in doubt, please consult your doctor.