In one study, the number of new cases of cholesteatoma in Iowa was estimated in 1975–6 to be just under one new case per 10,000 citizens per year. Cholesteatoma affects all age groups, from infants through to the elderly. The peak incidence occurs in the second decade.

More commonly, keratin accumulates in a pouch of tympanic membrane which extends into the middle ear space. This abnormal folding or 'retraction' of the tympanic membrane arises in one of the following ways:

- "Wittmaack's theory" : Invagination of tympanic membrane from the attic or part of pars tensa in the form of retraction pockets lead to the formation of cholesteatoma.

- "Ruedi's theory" : The basal cells of germinal layer of skin proliferate under the influence of infection and lay down keratinising squamous epithelium.

- "Habermann's theory": The epithelium from the meatus or outer drum surface grows into the middle ear through a pre-existing perforation and form cholesteatoma.

Cholesteatoma may also arise as a result of metaplasia of the middle ear mucosa or implantation following trauma.

Myringosclerosis seems to be more common than tympanosclerosis. Most research has not been conducted upon the general, healthy population, but rather those with otitis media or patients who have had tympanostomy tubes in prior procedures. Of the children studied who had 'glue ear', and who were treated with tympanostomy tubing, 23-40% of cases had tympanosclerosis. One study suggested that people with atherosclerosis were more likely to have tympanosclerosis than otherwise healthy individuals.

Various strategies may be used to manage tympanic membrane retraction, with the aims of preventing or relieving hearing loss and cholesteatoma formation.

As retraction pockets may remain stable or resolve spontaneously, it may be appropriate to observe them for a period of time before considering any active treatment.

Long-term antibiotics, while they decrease rates of infection during treatment, have an unknown effect on long-term outcomes such as hearing loss. This method of prevention has been associated with emergence of antibiotic-resistant otitic bacteria. They are thus not recommended.

Pneumococcal conjugate vaccines (PCV) in early infancy, decreases the risk of acute otitis media in healthy infants. PCV is recommended for all children, and, if implemented broadly, PCV would have a significant public health benefit. Influenza vaccine is recommended annually for all children. PCV does not appear to decrease the risk of otitis media when given to high-risk infants or for older children who have previously experienced otitis media.

Risk factors such as season, allergy predisposition and presence of older siblings are known to be determinants of recurrent otitis media and persistent middle-ear effusions (MEE). History of recurrence, environmental exposure to tobacco smoke, use of daycare, and lack of breastfeeding have all been associated with increased risk of development, recurrence, and persistent MEE. Thus, cessation of smoking in the home should be encouraged, daycare attendance should be avoided or daycare facilities with the fewest attendees should be recommended, and breastfeeding should be promoted.

There is some evidence that breastfeeding for the first year of life is associated with a reduction in the number and duration of OM infections. Pacifier use, on the other hand, has been associated with more frequent episodes of AOM.

Evidence does not support zinc supplementation as an effort to reduce otitis rates except maybe in those with severe malnutrition such as marasmus.

Adhesive otitis media occurs when a thin retracted ear drum becomes sucked into the middle-ear space and stuck (i.e., adherent) to the ossicles and other bones of the middle ear.

In most cases, tympanosclerosis does not cause any recognisable hearing loss up to ten years after the initial disease onset. Sclerotic changes seem to stabilise, but not resolve or dissolve, after 3 years.

These can be both congenital or develop over time with the thinning of the otic capsule by the persistent pulsations of the intracranial pressures against the bones of the skull. Finally, disease conditions—for example cholesteatoma—can result in a labyrinthine fistula. Traumatic events, with excessive pressure changes to the inner ear such as in scuba diving, head trauma, or an extremely loud noise can lead to rupture and leakage.

When diagnosing, PLF should be differentiated from Ménière's disease. Tympanostomy has been reported to be a way to diagnose and cure PLF.

In the United States and other developed countries, the incidence of mastoiditis is quite low, around 0.004%, although it is higher in developing countries. The condition most commonly affects children aged from two to thirteen months, when ear infections most commonly occur. Males and females are equally affected.

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.

With prompt treatment, it is possible to cure mastoiditis. Seeking medical care early is important. However, it is difficult for antibiotics to penetrate to the interior of the mastoid process and so it may not be easy to cure the infection; it also may recur. Mastoiditis has many possible complications, all connected to the infection spreading to surrounding structures. Hearing loss is likely, or inflammation of the labyrinth of the inner ear (labyrinthitis) may occur, producing vertigo and an ear ringing may develop along with the hearing loss, making it more difficult to communicate. The infection may also spread to the facial nerve (cranial nerve VII), causing facial-nerve palsy, producing weakness or paralysis of some muscles of facial expression, on the same side of the face. Other complications include Bezold's abscess, an abscess (a collection of pus surrounded by inflamed tissue) behind the sternocleidomastoid muscle in the neck, or a subperiosteal abscess, between the periosteum and mastoid bone (resulting in the typical appearance of a protruding ear). Serious complications result if the infection spreads to the brain. These include meningitis (inflammation of the protective membranes surrounding the brain), epidural abscess (abscess between the skull and outer membrane of the brain), dural venous thrombophlebitis (inflammation of the venous structures of the brain), or brain abscess.

Third window effect caused by:

- Superior canal dehiscence – which may require surgical correction.

- Widened vestibular aqueducts

This is an uncommon lesion, usually affecting young patients (mean age, 30 years), with a male to female ratio of 2:1. The middle ear is involved, although it may extend to the external auditory canal if there is tympanic membrane perforation.

Most individuals come to clinical attention during the 5th decade, although the age range is broad (20 to 80 years). There is an equal gender distribution.

Since this lesion is usually a complication of long standing otitis media, it is important to use an appropriate antibiotic therapy regimen. If the patient fails first line antibiotics, then second-line therapies should be employed, especially after appropriate culture and sensitivity testing. Surgery may be required if there is extension into the mastoid bone, or if a concurrent cholesteatoma is identified during surgery or biopsy. In general, patients have an excellent outcome after appropriate therapy.

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.

Reactivation of herpes zoster virus, as well as being associated with Bell's palsy, may also be a direct cause of facial nerve palsy. Reactivation of latent virus within the dorsal root ganglion of the facial nerve is associated with vesicles affecting the ear canal, and termed Ramsay Hunt syndrome type II. In addition to facial paralysis, symptoms may include ear pain and vesicles, sensorineural hearing loss, and vertigo. Management includes Antiviral drugs and oral steroids.

Otitis media is an infection in the middle ear, which can spread to the facial nerve and inflame it, causing compression of the nerve in its canal. Antibiotics are used to control the otitis media, and other options include a wide myringotomy (an incision in the tympanic membrane) or decompression if the patient does not improve.

Chronic otitis media usually presents in an ear with chronic discharge (otorrhea), or hearing loss, with or without ear pain (otalgia). Once suspected, there should be immediate surgical exploration to determine if a cholesteatoma has formed as this must be removed if present. Inflammation from the middle ear can spread to the canalis facialis of the temporal bone - through this canal travels the facial nerve together with the steatoacoustisus nerve. In the case of inflammation the nerve is exposed to edema and subsequent high pressure, resulting in a periferic type palsy.

Lyme disease, caused by chronic "Borrelia burgdorferi" infection, is a common cause of facial nerve paralysis in areas.

Other causes may include:

- Diabetes mellitus

- Facial nerve paralysis, sometimes bilateral, is a common manifestation of sarcoidosis of the nervous system, neurosarcoidosis.

- Bilateral facial nerve paralysis may occur in Guillain–Barré syndrome, an autoimmune condition of the peripheral nervous system.

- Moebius syndrome is a bilateral facial paralysis resulting from the underdevelopment of the VII cranial nerve (facial nerve), which is present at birth. The VI cranial nerve, which controls lateral eye movement, is also affected, so people with Moebius syndrome cannot form facial expression or move their eyes from side to side. Moebius syndrome is extremely rare, and its cause or causes are not known.

The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons. Hemifacial spasm is more prevalent among females over 40 years of age. The estimated prevalence for women is 14.5 per 100,000 and 7.4 per 100,000 in men. Prevalence for hemifacial spasm increase with age, reaching 39.7 per 100,000 for those aged 70 years and older. One study divided 214 hemifacial patients based on the cause of the disease. The patients who had a compression in the facial nerve at the end of the brain stem as the primary hemifacial spasm and patients who had peripheral facial palsy or nerve lesion due to tumors, demyelination, trauma, or infection as secondary hemifacial spasm. The study found that 77% of hemifacial spasm is due to primary hemifacial spasm and 23% is due to secondary hemifacial spasm. The study also found both sets of patients to share similar age at onset, male to female ratios, and similar affected side. Another study with 2050 patients presented with hemifacial spasm between 1986 and 2009, only 9 cases were caused by a cerebellopontine angle syndrome, an incidence of 0.44%.

Microvascular decompression appears to be the most popular surgical treatment at present. Microvascular decompression relieves pressure on the facial nerve, which is the cause of most hemifacial spasm cases. Excellent to good results are reported in 80% or more cases with a 10% recurrence rate. In the present series approximately 10% had previously failed surgery. Serious complications can follow microsurgical decompressive operations, even when performed by experienced surgeons. These include cerebellar haematoma or swelling, brain stem infarction (blood vessel of the brain stem blocked), cerebral infarction (ischemic stroke resulting from a disturbance in the blood vessels supplying blood to the brain), subdural haematoma and intracerebral infarction (blockage of blood flow to the brain). Death or permanent disability (hearing loss) can occur in 2% of patients of hemifacial spasm.

The number of events that can lead to the development of PSH symptoms is many. The exact pathways or causes for the development of the syndrome are not known. Traumatic brain injury, hypoxia, stroke, anti-NMDA receptor encephalitis (although further associations are being explored), injury of the spinal cord, and many other forms of brain injury can cause onset of PSH. Even more obscure diseases such as intracranial tuberculoma have been seen to cause onset of paroxysmal sympathetic hyperactivity. It is observed that these injuries lead to the development of PSH or are seen in conjunction with PSH, but the pathophysiology behind these diseases and the syndrome is not well understood.

Patients who develop PSH after traumatic injury have longer hospitalization and longer durations in intensive care in cases where ICU treatment is necessary. Patients often are more vulnerable to infections and spend longer times on ventilators, which can lead to an increased risk of various lung diseases. PSH does not affect mortality rate, but it increases the amount of time it takes a patient to recover from injury, compared to patients with similar injuries who do not develop PSH episodes. It often takes patients who develop PSH longer to reach similar levels of the brain activity seen in patients who do not develop PSH, although PSH patients do eventually reach these same levels.