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This uncommon tumor accounts for less than 2% of all ear tumors. While patients present with symptoms related to the middle ear cavity location of the tumor, the tumor may expand into the adjacent structures (external auditory canal, mastoid bone, and eustachian tube). Patients come to clinical attention with unilateral (one sided) hearing loss, usually associated with decreased auditory acuity, and particularly conductive hearing loss if the ossicular bone chain (middle ear bones) is involved. Tinnitus (ringing), otitis media, pressure or occasionally ear discharge are seen. At the time of otoscopic exam, the tympanic membrane is usually intact, with a fluid level or mass noted behind the ear drum. Even though this is a "neuroendocrine" type tumor, there is almost never evidence of neuroendocrine function clinically or by laboratory examination.
Pain is the most common symptom, followed by either sensorineural or conductive hearing loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow growing.
In general, an axial and coronal bone computed tomography study without contrast will yield the most information for this tumor. The tumor appears as a soft tissue mass usually within a well-aerated mastoid bone. The features of chronic otitis media are not usually seen. Bone invasion and destruction are usually not seen in this tumor which expands within the mesotympanum (middle ear cavity). Encasement of the ossicles is usually present. There may be an irregular margin at the periphery, especially if the tumor has been present for a long time, with associated bone remodeling.
Imaging studies are used to define the extent of the tumor and to exclude direct extension from the parotid gland or nasopharynx. The imaging findings are usually non-specific, and cannot give a specific diagnosis.
Ceruminous adenoma are rare tumors, accounting for less than 1% of all external ear tumors. The patients will present with a mass, perhaps associated pain, and may have changes in hearing (usually a sensorineural or a conductive hearing loss). Some patients have tinnitus. Nerve paralysis is very uncommon.
The majority (98%) of patients with cholesteatoma have ear discharge or hearing loss or both in the affected ear.
Other more common conditions, such as otitis externa, may also present with these symptoms, but cholesteatoma is much more serious and should not be overlooked. If a patient presents to a doctor with ear discharge and hearing loss, the doctor should consider the patient to have cholesteatoma until the disease is definitely excluded.
Other less common symptoms (all less than 15%) of cholesteatoma may include: pain, balance disruption, tinnitus, ear ache, headaches and bleeding from the ear. There can also be facial nerve weakness.
Balance symptoms in the presence of a cholesteatoma raises the possibility that the cholesteatoma is eroding the balance organs, which form part of the inner ear.
On initial inspection, an ear canal full of discharge may be all that is visible. Until the doctor has cleaned the ear and inspected the entire tympanic membrane, cholesteatoma cannot be either confirmed or excluded.
Once the debris is cleared, cholesteatoma can give rise to a number of appearances. If there is significant inflammation, the tympanic membrane may be partially obscured by an aural polyp. If there is less inflammation, the cholesteatoma may present the appearance of 'semolina' discharging from a defect in the tympanic membrane. The posterior and superior parts of the tympanic membrane are most commonly affected. If the cholesteatoma has been dry, the cholesteatoma may present the appearance of 'wax over the attic'. The attic is just above the ear drum.
The patient may commonly also have clinical signs of conductive hearing loss. Less frequently, there may be signs of imbalance or facial weakness.
If untreated, a cholesteatoma can eat into the three small bones located in the middle ear (the malleus, incus and stapes, collectively called ossicles), which can result in nerve deterioration, deafness, imbalance and vertigo. It can also affect and erode, through the enzymes it produces, the thin bone structure that isolates the top of the ear from the brain, as well as lay the covering of the brain open to infection with serious complications (rarely even death due to brain abscess and septicemia).
Both the acquired as well as the congenital types of the disease can affect the facial nerve that extends from the brain to the face and passes through the inner and middle ear and leaves at the anterior tip of the mastoid bone, and then rises to the front of the ear and extends into the upper and lower face.
Trotter's syndrome is a cluster of symptoms associated with certain types of advanced nasopharyngeal carcinoma. The cause of pain is the mandibular nerve of the foramen ovale, through which the tumor enters the calvarium. Symptoms include the following:
1. Unilateral conductive deafness due to middle ear effusion
2. Trigeminal neuralgia due to perineural spread
3. Soft palate immobility
4. Difficulty opening mouth
Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. Although cholesteatomas are not classified as either tumors or cancers, they can still cause significant problems because of their erosive and expansile properties resulting in the destruction of the bones of the middle ear (ossicles), as well as their possible spread through the base of the skull into the brain. They are also often infected and can result in chronically draining ears.
This is an inherited disease. The primary form of hearing loss in otosclerosis is conductive hearing loss (CHL) whereby sounds reach the ear drum but are incompletely transferred via the ossicular chain in the middle ear, and thus partly fail to reach the inner ear (cochlea). This usually will begin in one ear but will eventually affect both ears with a variable course. On audiometry, the hearing loss is characteristically low-frequency, with higher frequencies being affected later.
Sensorineural hearing loss (SNHL) has also been noted in patients with otosclerosis; this is usually a high-frequency loss, and usually manifests late in the disease. The causal link between otosclerosis and SNHL remains controversial. Over the past century, leading otologists and neurotologic researchers have argued whether the finding of SNHL late in the course of otosclerosis is due to otosclerosis or simply to typical presbycusis.
Most patients with otosclerosis notice tinnitus (head noise) to some degree. The amount of tinnitus is not necessarily related to the degree or type of hearing impairment. Tinnitus develops due to irritation of the delicate nerve endings in the inner ear. Since the nerve carries sound, this irritation is manifested as ringing, roaring or buzzing. It is usually worse when the patient is fatigued, nervous or in a quiet environment.
Adenoid hypertrophy (or enlarged adenoids) is the unusual growth ("hypertrophy") of the adenoid tonsil first described by the Danish physician Wilhelm Meyer (1824-1895) in Copenhagen in 1868. He described that a long term adenoid hypertrophy will cause an obstruction of the nasal airways. These will lead to a dentofacial growth anomaly that was defined as "adenoid facies" (see long face syndrome).
There is very little lymphoid tissue in the nasopharynx of young babies; humans are born without substantial adenoids. The mat of lymphoid tissue called adenoids starts to get sizable during the first year of life. Just how big the adenoids become is quite variable between individual children.
Vestibular schwannoma patients sometimes complain of a feeling that their ear is plugged or "full".
Otosclerosis or otospongiosis is an abnormal growth of bone near the middle ear. It can result in hearing loss. The term otosclerosis is something of a misnomer. Much of the clinical course is characterised by lucent rather than sclerotic bony changes, hence it is also known as otospongiosis.
Superior canal (SCD) can affect both hearing and balance to different extents in different people.
Symptoms of SCDS include:
- Autophony – person's own speech or other self-generated noises (e.g. heartbeat, eye movements, creaking joints, chewing) are heard unusually loudly in the affected ear
- Dizziness/ vertigo/ chronic disequilibrium caused by the dysfunction of the superior semicircular canal
- Tullio phenomenon – sound-induced vertigo, disequilibrium or dizziness, nystagmus and oscillopsia
- Pulse-synchronous oscillopsia
- Hyperacusis – the over-sensitivity to sound
- Low-frequency conductive hearing loss
- A feeling of fullness in the affected ear
- Pulsatile tinnitus
- Brain fog
- Fatigue
- Headache/migraine
- Tinnitus – high pitched ringing in the ear
Recurring headaches are an uncommon symptom, also tending to occur only in cases of larger tumors.
The majority of tympanic membrane retractions do not cause any symptoms. Some cause hearing loss by restricting sound-induced vibrations of the eardrum. Permanent conductive hearing loss can be caused by erosion of the ossicles (hearing bones). Discharge from the ear often indicates that the retraction pocket has developed into a cholesteatoma.
Enlarged adenoids can become nearly the size of a ping pong ball and completely block airflow through the nasal passages. Even if enlarged adenoids are not substantial enough to physically block the back of the nose, they can obstruct airflow enough so that breathing through the nose requires an uncomfortable amount of work, and inhalation occurs instead through an open mouth. Adenoids can also obstruct the nasal airway enough to affect the voice without actually stopping nasal airflow altogether.
Nasal blockage is determined by at least two factors: 1) the size of the adenoids, and 2) the size of the nasal pharynx passageway.
The adenoid usually reaches its greatest size by about age 5 years or so, and then fades away ("atrophies") by late childhood - generally by the age of 7 years. The lymphoid tissue remains under the mucosa of the nasopharynx, and could be seen under a microscope if the area was biopsied, but the mass is so reduced in size that the roof of the nasopharynx becomes flat rather than mounded. Just as the size of the adenoids is variable between individuals, so is the age at which adenoids atrophy.
The symptoms caused by enlarged adenoids are mostly due to "where" this lymphoid tissue is located. The adenoids are in the midline of the nasopharynx, and the Eustachian tubes open from either ear to the right and left of them. In children with excessive middle ear infections and chronic middle ear fluid, there is a high bacterial count in the adenoids as compared to children without problematic otitis media, even if the size of the adenoids is small. The adenoids in these cases provides a reservoir of pathogenic bacteria that cause ear infections and subsequent middle ear effusions (fluid).
The nasopharynx lies right above the throat. Splashes of excessive "drip" from infected adenoids may land directly on the vocal cords. Although the larynx and vocal cords do not ordinarily become infected from adenoiditis, their mucosa does become irritated. The vocal cords are extremely sensitive to touch, and any fluid drops falling on them cause an irresistible urge to cough. Adenoiditis therefore is one of the causes of cough.
Myringosclerosis rarely causes any symptoms. Tympanosclerosis, on the other hand, can cause significant hearing loss or chalky, white patches on the middle ear or tympanic membrane.
A ceruminous adenoma (also known as adenoma of the ceruminous gland and ceruminoma) is a benign glandular neoplasm which arises from the ceruminous glands located within the external auditory canal. These glands are found within the outer one third to one half of the external auditory canal, more common along the posterior surface; therefore, the tumor develops within a very specific location.
Patients usually present with otorrhea, conductive hearing loss, and otalgia, while bleeding and a sensation of a mass are much less common.
Tympanic membrane retraction describes a condition in which a part of the eardrum lies deeper within the ear than its normal position.
The eardrum comprises two parts, the pars tensa, which is the main part of the eardrum, and the pars flaccida, which is a smaller part of the eardrum located above the pars tensa. Either or both of these parts may become retracted. The retracted segment of eardrum is often known as a retraction pocket. The terms "atelectasis" or sometimes "adhesive otitis media" can be used to describe retraction of a large area of the pars tensa.
Tympanic membrane retraction is fairly common and has been observed in one quarter of a population of British school children. Retraction of both eardrums is less common than having a retraction in just one ear. It is more common in children with cleft palate. Tympanic membrane retraction also occurs in adults.
Attempts have been made to categorise the extent of tympanic membrane retraction though the validity of these classifications is limited.
"Myringosclerosis" refers to a calcification only within the tympanic membrane and is usually less extensive than "intratympanic tympanosclerosis", which refers to any other location within the middle ear such as the ossicular chain, middle ear mucosa or, less frequently, the mastoid cavity.
Although imaging is not required to yield a diagnosis, it may be obtained to exclude other disorders, such as a concurrent cholesteatoma.
Most patients present clinically with progressive, one sided hearing loss, much more often of the sensorineural rather than conductive type. Patients may also experience tinnitus, vertigo, and loss of vestibular function (ataxia). Symptoms are usually present for a long time, which supports the slow growth of these tumors. Patients may also present with other symptoms related to von Hippel-Lindau syndrome in other anatomic sites, which will result in imaging evaluation of the head.
The most common location by far is the gingival margin and other areas of the masticatory oral mucosa, these occur more frequently in the fifth decade of life, and have good prognosis, the treatment of choice for oral VXs is surgical excision, and recurrence is rare.
The condition can affect other organs of body, such as the penis, vulva, and can occur in anal region, nose, the ear, lower extremity, scrotum.
Differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma