Dr Paulose

World Class ENT Plastic and Laser Surgeon

Ear

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Tumors of the Middle Ear and Mastoid

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Malignant tumors in the middle ear and mastoid are very rare. Of these very uncommon tumors, the squamous cell cancers are the most common. Squamous cell cancer of the middle ear and mastoid is a fatal disease, if not treated properly. These cancers are usually found in individuals who have neglected chronic infection in the mastoid or middle ear. The exact relationship of the infection to the formation of the squamous cell cancer is unclear. Presumably, it is related to chronic inflammation.

Malignant tumors of the mastoid and middle ear accounts for 5 to 26% of all ear neoplasms. Of these neoplasms, squamous cell carcinoma is the most common, with an age-adjusted incidence of 1 case in 1 million and peak age of 60 years.

Squamous cell cancer of the middle ear is often quite advanced before a correct diagnosis is made. Pain is a significant feature of squamous cell cancer of the middle ear and mastoid. Intermittent hemorrhage, bleeding and drainage for long periods of time are also usual. Hearing loss is significant. Diagnosis depends upon a biopsy of the tissue.

The major etiological factor is chronic suppurative otitis media although irradiation and inverted papilloma of the middle ear have also been reported to be additional risk factors.

Human papilloma virus types 16 and 18 have been associated with squamous cell carcinoma of the middle ear at both tissue and molecular levels, thus providing a good model to explain the pathogenesis of chronic inflammation-related human malignancies.

An examination by the ear surgeon investigating a perforated eardrum which shows suspicious tissue in the middle ear or mastoid that does not heal after appropriate medical therapy would indicate a need for a biopsy. Suspicion should be aroused about any unusual tissue which is seen through a perforated eardrum or mastoid, particularly those that are painful. After topical or local anesthesia is placed into the ear, the tissue to be biopsied is sent off for analysis and a pathologist will be able to determine whether cancer is present in the tissues. During middle ear mastoid surgery any polyps or granulations seen must be sent for histopathology as routine.

CT scan and MRI are necessary in order to help determine the extent of surgery necessary. Even with early surgery and radiation therapy, cure may not be possible if the tumor is deeply invasive. Thus, it is imperative that diagnosis be made as early as possible. Once diagnosis is made, the patient must be prepared for radical surgery.

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Glomus Tumors

Glomus tumors are the most common benign tumors of the middle ear. They arise from glomus bodies. Glomus bodies are tiny, normal structures in the middle ear which serve as baro receptors. These baro receptors sense and help to regulate the oxygen pressure in the middle ear and mastoid.

Benign tumors of glomus bodies can occur within the middle ear or at other sites: the temporal bone and neck, or within the jugular vein (the large vein in the upper neck which drains the head toward the heart). Glomus tumors of the middle ear are more common than glomus tumors of the jugular vein. They can result from abnormal growth of a single glomus body.

Glomus tumors are highly vascular and are primarily composed of blood channels flowing through the tumor itself. They appear red on examination of the eardrum. Most glomus tumors are readily noted by an n ENT Surgeon\’s routine examination of the ear. They appear as a red ball or mass behind the eardrum. The eardrum may pulsate, if the glomus tumor is touching the under surface of the intact eardrum.

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Diagnostic Imaging Studies

CT scan: CT scanning of the mastoid and middle ear determine the extent of bone involvement. As a tumor grows, it will invade and destroy bones. Cancers also appear on a CT scan, and an estimate of the extent of the disease can be obtained.

MRI scanning can demonstrate the infiltration of the tumor into soft tissue, specifically tissue in the front of the ear or parotid gland area. MRI will also help to determine if the tumor has invaded the dura, which is the capsule surrounding the brain. If the tumor has grown through the plate of bone that separates the middle ear and mastoid from the brain, then cure becomes quite difficult.

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Treatment

The classical treatment is combined surgery and radiotherapy, although either method may be used alone. Most authorities agree that a combination of surgery and radiotherapy as opposed to single modality treatment is likely to yield the best results.

Glomus tumors are not highly radiosensitive. However, in older patients, or those who should not undergo sugery, radiation therapy may help to arrest the growth of a glomus tumor. In the younger patient, complete surgical removal of the tumor after emobilzation is the preferred method of treatment.

 

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Chronic Ear Disease-Cholesteatoma and its Complications

cholesteatoma

Cholesteatoma is a benign growth of skin in the middle ear or mastoid that can lead to infection and more serious problems involving the brain and facial nerve. It is not a cancer condition but is important because it can lead to serious complications such as permanent deafness and life-threatening illnesses such as meningitis and brain abcesess.

The cause of the cholesteatoma is not fully understood. It is thought that skin cells from the lining of the ear canal get trapped in the middle ear. Skin cells, including those that line the ear canal, normally multiply regularly to replace those that have died. Usually these skin cells flake off. The dead cells are trapped too and build up. This build-up of dead skin cells over time is what forms the cholesteatoma.

Types of Cholesteatoma

and Congenital cholesteatoma grows behind the eardrum from birth.

and Acquired cholesteatoma develops later, usually in adults. This is often as a result of a chronic or recurring ear infection. The infection causes a blockage of the Eustachian tube. This is the tube that connects the middle ear to the back of the nose and throat. The blockage creates a negative pressure that draws the eardrum inwards. This can result in a small pocket forming, usually at the very top of the eardrum. In this pocket some skin cells collect, get trapped, but continue to multiply to form the cholesteatoma. The pocket is too deep to allow the dead skin cells to escape, so the cholesteatoma gradually expands.

Symptoms

Cholesteatoma is usually unilateral. The most common initial symptoms are a smelly discharge from the ear, and some hearing loss. You are also likely to have had previous problems with ear infections. Other symptoms that may occur include hearing loss and vertigo.

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Complications

Erosion of the ossicles (bones) behind the eardrum can lead to a conductive hearing loss. The bone over the facial nerve can also be destroyed, and a facial paralysis can result. The inner ear, composed of a bony labyrinth, can also be partially destroyed. This can lead to sensor neural hearing loss and dizziness. The infection can also spread into the veins carrying blood from the brain to the heart. The sigmoid sinus can get thrombosis. The infection can also spread to the covering of the brain and cause meningitis. In rare circumstances, a brain abscess can occur.

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Facial Nerve Paralysis with Ear Disease

Facial nerve paralysis can be seen in patients who suffer from acute and chronic ear infections (otitis media). Facial paralysis that occurs suddenly during an acute ear infection implies inflammation of the nerve. This tends to occur in infants and young children, because the bony canal surrounding the nerve is not as insulated from the middle ear space as it is in adults. Ordinarily, prompt treatment of the infection, that sometimes includes putting a hole in the ear drum (myringotomy) to remove the infected contents, leads to recovery of the nerve.

Sudden facial paralysis in the setting of chronic ear disease suggests damage caused by expanding cholesteatoma. When sudden facial paralysis occurs in the face of known chronic ear disease, prompt surgical exploration with evacuation of disease and nerve decompression usually results in good recovery of function.

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Diagnosis

Otoscopy and Microscopic examination

Audiometry (hearing tests) may show deafness or hearing loss and are usually performed in a hospital clinic.

Swabs of the ear discharge may also be taken. The discharge often contains a bacterium Pseudomonas which is responsible for the smell.

CT scans might be needed to see the extent of the damage caused by the cholesteatoma, and to plan further treatment.

Treatment-Surgery

Surgery removes all of the diseased areas, including the cholesteatoma itself. Virtually all cholesteatomas should be removed by surgery.

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Mastoidectomy

In the canal wall–down (open) procedure, the posterior canal wall is removed. A large meatoplasty is created to allow adequate air circulation into the cavity that arises from the operation. Canal wall–down operations have the highest probability of permanently ridding patients of cholesteatomas.

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Snoring and Sleep Apnea in Children (Ts Ads)-Any Treatment?

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Snoring and Sleep apnea is a condition of interrupted breathing cause by a narrowing in the throat or upper airway. These breathing pauses typically last between 10 to 60 seconds and can occur up to hundreds of times a night.

This narrowing can be due to enlarged tonsils and adenoids, obesity, or other medical problems. This condition occurs in both adults and children, but is most prevalent in obese patients.

This chronic sleep deprivation in kids results in daytime sleepiness, slow reflexes, poor concentration, and poor performance in classroom.

Causes of Snoring in Children

and Adenotonsillar hypertrophy – the commonest cause

and Obesity – increases the risk of OSA nearly five times.

and Neuromuscular diseases-presence of craniofacial abnormalities.

Symptoms

and Snoring – usually parents seek attention; many will just get better as they grow older.

and Mouth breathing and dental anomalies-Adenoid Face

and Choking and gasping for breath at night

and Daytime somnolence and tiredness are prominent

and Not doing well at school due to poor concentration.

and Failure to thrive.

and Behavioral problems.

and Enuresis – thought to be due to lack of awareness overnight.[2]

Types of sleep apnea

and Obstructive sleep apnea is the most common type of sleep apnea. It occurs when the soft tissue in the back of your throat relaxes during sleep and blocks the airway, often causing you to snore loudly.

and Central sleep apnea is a much less common type of sleep apnea that involves the central nervous system, occurring when the brain fails to signal the muscles that control breathing. People with central sleep apnea seldom snore.

and Mixed sleep apnea is a combination of obstructive sleep apnea and central sleep apnea.

Other signs and symptoms of sleep apnea

and Morning headaches

and Memory or learning problems and not being able to concentrate

and Feeling irritable, depressed, or having mood swings or personality changes

and Dry mouth or sore throat when you wake up

Investigations

The gold standard is probably polysomnography, commonly called sleep studies.

Other investigations that may be needed

and Lateral neck radiography, e.g. CT/MRI scan.

and Blood Tests to rule out Hypothyroidism Diabetes etc

and X-ray Chest and ECG

Treatment

Medical

and Continuous positive airways pressure (CPAP) is rarely used for children. It may be considered if adenotonsillectomy is not possible or has failed.

and Weight loss programme in obese children.

and There is no role for antibiotics (unless tonsillitis is present) or for steroids.

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Surgical

Adenotonsillectomy for adenotonsillar hypertrophy. This is curative in the majority of patients. The surgeon may remove tonsils, adenoids, or excess tissue at the back of the throat or inside the nose. Or the surgeon may reconstruct the jaw to enlarge the upper airway. Children who had these surgeries have experienced improvements in behavior, quality of life, and other symptoms.

Tracheostomy – very rarely indicated and only as an exceptional last resort.

CPAP for sleep apnea?

Continuous Positive Airflow Pressure (CPAP) is the most common treatment for moderate to severe obstructive sleep apnea in adults but not usually recommended unless other treatment fails. A CPAP machine prevents sleep apnea by blowing air into a mask that covers the nose and mouth. The stream of air keeps the airways open.

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