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.

Cholesteatomas occur in two basic classifications: Acquired cholesteatomas, which are more common, are usually caused by pathological alteration of the ear drum leading to accumulation of keratin within the middle ear. Congenital cholesteatomas are usually middle ear epidermal cysts that are identified deep within an intact ear drum.

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.

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.

Some common symptoms and signs of mastoiditis include pain, tenderness, and swelling in the mastoid region. There may be ear pain (otalgia), and the ear or mastoid region may be red (erythematous). Fever or headaches may also be present. Infants usually show nonspecific symptoms, including anorexia, diarrhea, or irritability. Drainage from the ear occurs in more serious cases, often manifest as brown discharge on the pillowcase upon waking.

PLF is a cause of dizziness, imbalance, and hearing loss—any or all of these symptoms can exist. Vertigo (an illusion of motion) is not common in this disorder. The most common cause of this fistula is head or ear trauma. Rapid increases of intracranial pressure can also result in a PLF. Rarely, these fistulas can be congenital, leading to progressive hearing loss and vertigo in childhood. It has also been a complication of a stapedectomy.

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.

"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.

A labyrinthine fistula is an abnormal opening in the bony capsule of the inner ear, resulting in leakage of the perilymph from the Cochlea into the middle ear. This includes specifically a perilymph fistula (PLF), an abnormal connection between the fluid of the inner ear and the air-filled middle ear. This connection is caused by a rupture of the round window that separate the inner and middle ear. Another type of l.f. is a semicircular canal dehiscence, which allows the inner ear to be influenced by the intracranial pressure directly.

Mastoiditis is the result of an infection that extends to the air cells of the skull behind the ear. Specifically, it is an inflammation of the mucosal lining of the mastoid antrum and mastoid air cell system inside the mastoid process. The mastoid process is the portion of the temporal bone of the skull that is behind the ear which contains open, air-containing spaces. Mastoiditis is usually caused by untreated acute otitis media (middle ear infection) and used to be a leading cause of child mortality. With the development of antibiotics, however, mastoiditis has become quite rare in developed countries where surgical treatment is now much less frequent and more conservative, unlike former times. Additionally, there is no evidence that the drop in antibiotic prescribing for otitis media has increased the incidence of mastoiditis, raising the possibility that the drop in reported cases is due to a confounding factor such as childhood immunizations against Haemophilus and Streptococci. Untreated, the infection can spread to surrounding structures, including the brain, causing serious complications.

Fluid accumulation is the most common cause of conductive hearing loss in the middle ear, especially in children. Major causes are ear infections or conditions that block the eustachian tube, such as allergies or tumors. Blocking of the eustachian tube leads to decreased pressure in the middle ear relative to the external ear, and this causes decreased motion of both the ossicles and the tympanic membrane.

- acute or serous otitis media

- otitis media with effusion or 'glue ear'

- Perforated eardrum

- Chronic suppurative otitis media (CSOM)

- Cholesteatoma

- Otosclerosis, abnormal growth of bone in or near the middle ear

- middle ear tumour

- ossicular discontinuity as a consequence of infection or temporal bone trauma

- Congenital malformation of the ossicles. This can be an isolated phenomenon or can occur as part of a syndrome where development of the 1st and 2nd branchial arches is seen such as in Goldenhar syndrome, Treacher Collins syndrome, branchio-oto-renal syndrome etc.

- Barotrauma unequal air pressures in the external and middle ear. This can temporarily occur, for example, by the environmental pressure changes as when shifting altitude, or inside a train going into a tunnel. It is managed by any of various methods of ear clearing manoeuvres to equalize the pressures, like swallowing, yawning, or the Valsalva manoeuvre. More severe barotrauma can lead to middle ear fluid or even permanent sensorineural hearing loss.

Conductive hearing loss occurs when there is a problem conducting sound waves anywhere along the route through the outer ear, tympanic membrane (eardrum), or middle ear (ossicles).

This type of hearing loss may occur in conjunction with sensorineural hearing loss (mixed hearing loss) or alone.

An integral symptom of acute otitis media is ear pain; other possible symptoms include fever, and irritability (in infants). Since an episode of otitis media is usually precipitated by an upper respiratory tract infection (URTI), there are often accompanying symptoms like a cough and nasal discharge.

Discharge from the ear can be caused by acute otitis media with perforation of the ear drum, chronic suppurative otitis media, tympanostomy tube otorrhea, or acute otitis externa. Trauma, such as a basilar skull fracture, can also lead to discharge from the ear due to cerebral spinal drainage from the brain and its covering (meninges).

Otitis media is a group of inflammatory diseases of the middle ear. The two main types are acute otitis media (AOM) and otitis media with effusion (OME). AOM is an infection of abrupt onset that usually presents with ear pain. In young children this may result in pulling at the ear, increased crying, and poor sleep. Decreased eating and a fever may also be present. OME is typically not associated with symptoms. Occasionally a feeling of fullness is described. It is defined as the presence of non-infectious fluid in the middle ear for more than three months. Chronic suppurative otitis media (CSOM) is middle ear inflammation of greater than two weeks that results in episodes of discharge from the ear. It may be a complication of acute otitis media. Pain is rarely present. All three may be associated with hearing loss. The hearing loss in OME, due to its chronic nature, may affect a child's ability to learn.

The cause of AOM is related to childhood anatomy and immune function. Either bacteria or viruses may be involved. Risk factors include exposure to smoke, use of pacifiers, and attending daycare. It occurs more commonly among Indigenous peoples and those who have Down syndrome. OME frequently occurs following AOM and may be related to viral upper respiratory infections, irritants such as smoke, or allergies. Looking at the eardrum is important for making the correct diagnosis. Signs of AOM include bulging or a lack of movement of the tympanic membrane from a puff of air. New discharge not related to otitis externa also indicates the diagnosis.

A number of measures decrease the risk of otitis media including pneumococcal and influenza vaccination, exclusive breastfeeding for the first six months of life, and avoiding tobacco smoke. The use of pain medications for AOM is important. This may include paracetamol (acetaminophen), ibuprofen, benzocaine ear drops, or opioids. In AOM, antibiotics may speed recovery but may result in side effects. Antibiotics are often recommended in those with severe disease or under two years old. In those with less severe disease they may only be recommended in those who do not improve after two or three days. The initial antibiotic of choice is typically amoxicillin. In those with frequent infections tympanostomy tubes may decrease recurrence. In children with otitis media with effusion antibiotics may increase resolution of symptoms, but may cause diarrhoea, vomiting and skin rash.

Worldwide AOM affect about 11% of people a year (about 325 to 710 million cases). Half the cases involve children less than five years of age and it is more common among males. Of those affected about 4.8% or 31 million develop chronic suppurative otitis media. Before the age of ten OME affects about 80% of children at some point. Otitis media resulted in 3,200 deaths in 2015 – down from 4,900 deaths in 1990.

Patients usually present with otorrhea, conductive hearing loss, and otalgia, while bleeding and a sensation of a mass are much less common.

Although imaging is not required to yield a diagnosis, it may be obtained to exclude other disorders, such as a concurrent cholesteatoma.

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.

Neuroendocrine adenoma of the middle ear has gone by several different names, including middle ear adenoma, carcinoid tumor, amphicrine adenoma, adenocarcinoid, and adenomatoid tumor of middle ear. The various names have created some confusion about this uncommon middle ear tumor. Regardless of the name applied, the "middle ear" anatomic site must be known or confirmed.

A tumor compressing the facial nerve anywhere along its complex pathway can result in facial paralysis. Common culprits are facial neuromas, congenital cholesteatomas, hemangiomas, acoustic neuromas, parotid gland neoplasms, or metastases of other tumours.

Often, since facial neoplasms have such an intimate relationship with the facial nerve, removing tumors in this region becomes perplexing as the physician is unsure how to manage the tumor without causing even more palsy. Typically, benign tumors should be removed in a fashion that preserves the facial nerve, while malignant tumors should always be resected along with large areas of tissue around them, including the facial nerve. While this will inevitably lead to heightened paralysis, safe removal of a malignant neoplasm is worth the often treatable palsy that follows. In the best case scenario, paralysis can be corrected with techniques including hypoglossal-facial nerve anastomosis, end-to-end nerve repair, cross facial nerve grafting, or muscle transfer/transposition techniques, such as the gracilis free muscle transfer.

Patients with facial nerve paralysis resulting from tumours usually present with a progressive, twitching paralysis, other neurological signs, or a recurrent Bell's palsy-type presentation.

The latter should always be suspicious, as Bell's palsy should not recur. A chronically discharging ear must be treated as a cholesteatoma until proven otherwise; hence, there must be immediate surgical exploration. Computed tomography (CT) or magnetic resonance (MR) imaging should be used to identify the location of the tumour, and it should be managed accordingly.

Other neoplastic causes include leptomeningeal carcinomatosis.

Physical trauma, especially fractures of the temporal bone, may also cause acute facial nerve paralysis. Understandably, the likelihood of facial paralysis after trauma depends on the location of the trauma. Most commonly, facial paralysis follows temporal bone fractures, though the likelihood depends on the type of fracture.

"Transverse fractures" in the horizontal plane present the highest likelihood of facial paralysis (40-50%). Patients may also present with blood behind the tympanic membrane, sensory deafness, and vertigo; the latter two symptoms due to damage to vestibulocochlear nerve and the inner ear. "Longitudinal fracture" in the vertical plane present a lower likelihood of paralysis (20%). Patients may present with blood coming out of the external auditory meatus), tympanic membrane tear, fracture of external auditory canal, and conductive hearing loss. In patients with mild injuries, management is the same as with Bell's palsy – protect the eyes and wait. In patients with severe injury, progress is followed with nerve conduction studies. If nerve conduction studies show a large (>90%) change in nerve conduction, the nerve should be decompressed. The facial paralysis can follow immediately the trauma due to direct damage to the facial nerve, in such cases a surgical treatment may be attempted. In other cases the facial paralysis can occur a long time after the trauma due to oedema and inflammation. In those cases steroids can be a good help.

The first sign of hemifacial spasm is typically muscle movement in the patient's eyelid and around the eye. It can vary in intensity. The intermittent twitching of the eyelid, which can result in forced closure of the eye which gradually spreads to the muscles of the lower part of the face (Typical form- See Image). In atypical form the spasms start in the cheekbone area and spreads to the eyelid. Ultimately, all the muscles on that side are affected, nearly all the time. This sometimes causes the mouth to be pulled to the side. Experts have linked hemifacial spasm to facial nerve injury, Bell's palsy and tumors. Although the most frequent cause is a blood vessel pressing on the facial nerve at the spot where it leaves the patient's brain stem, sometimes there is no known cause. When the affected individual is younger than 40, doctors suspect an underlying cause such as multiple sclerosis.

Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular, involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial). The facial muscles are controlled by the facial nerve (seventh cranial nerve), which originates at the brainstem and exits the skull below the ear where it separates into five main branches.

This disease takes two forms: typical and atypical. In typical form, the twitching usually starts in the lower eyelid in orbicularis oculi muscle. As time progresses, it spreads to the whole lid, then to the orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area. The reverse process of twitching occurs in atypical hemifacial spasm; twitching starts in orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area in the lower face, then progresses up to the orbicularis oculi muscle in the eyelid as time progresses. The most common form is the typical form, and atypical form is only seen in about 2–3% of patients with hemifacial spasm. The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.

This disorder occurs in both men and women, although it affects middle-aged or elderly women more frequently. Hemifacial spasm is much more common in some Asian populations. It may be caused by a facial nerve injury, a tumor, or it may have no apparent cause. Individuals with spasm on both sides of the face are very rare.

Characteristics of paroxysmal sympathetic hyperactivity include:

- fever

- tachycardia

- hypertension

- tachypnea

- hyperhidrosis or diaphoresis

- dystonic posturing

- pupillary dilation

- flushing

In cases where PSH episodes develop post-injury, specifically traumatic brain injury, symptoms typically develop quickly, usually within a week. Symptom onset has been seen to average 5.9 days post-injury. Episodes vary in duration and occurrence. Episodes can last as little as a few minutes or as long as ten hours, and they can occur multiple times a day. Episode duration has been seen to average 30.8 minutes and occur five to six times a day. Episodes can occur naturally or arise from external triggers. Common triggers include pain or stimulation, body turning or movements, and bladder distention. Bladder distention has been observed in patients being treated in intensive care units with the concurrent use of catheters. Symptoms of PSH can last from weeks to years following initial onset. As episodes persist over time, they have been found to become less frequent in occurrence but last for prolonged periods.

Paroxysmal sympathetic hyperactivity (PSH) is a syndrome that causes episodes of increased activity of the sympathetic nervous system. Hyperactivity of the sympathetic nervous system can manifest as increased heart rate, increased respiration, increased blood pressure, diaphoresis, and hyperthermia.

Previously, this syndrome has been identified as general dysautonomia but now is considered a specific form of it. It has also been referred to as paroxysmal sympathetic instability with dystonia, or PAID, and sympathetic storm. Recently, however, studies have adopted the name paroxysmal sympathetic hyperactivity to ensure specificity. PSH is observed more in younger patients than older ones. It is also seen more commonly in men than women. There is no known reason why this is the case, although it is suspected pathophysiological links may exist. In patients surviving traumatic brain injury, the occurrence of these episodes is one in every three. PSH can also be associated with severe anoxia, subarachnoid and intracerebral hemorrhage, and hydrocephalus.