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.

Ear pain is the predominant complaint and the only symptom directly related to the severity of acute external otitis. Unlike other forms of ear infections, the pain of acute external otitis is worsened when the outer ear is touched or pulled gently. Pushing the tragus, the tablike portion of the auricle that projects out just in front of the ear canal opening, also typically causes pain in this condition as to be diagnostic of external otitis on physical examination. People may also experience ear discharge and itchiness. When enough swelling and discharge in the ear canal is present to block the opening, external otitis may cause temporary conductive hearing loss.

Because the symptoms of external otitis lead many people to attempt to clean out the ear canal (or scratch it) with slim implements, self-cleaning attempts generally lead to additional traumas of the injured skin, so rapid worsening of the condition often occurs.

In general one ear will be somewhat worse than the other due to the prevailing wind direction of the area surfed or the side that most often strikes the wave first.

- Decreased hearing or hearing loss, temporary or ongoing

- Increased prevalence of ear infections, causing ear pain

- Difficulty evacuating debris or water from the ear causing a plugging sensation

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.

Otitis externa, also called swimmer's ear, is inflammation of the ear canal. It often presents with ear pain, swelling of the ear canal, and occasionally decreased hearing. Typically there is pain with movement of the outer ear. A high fever is typically not present except in severe cases.

Otitis externa may be acute (lasting less than six weeks) or chronic (lasting more than three months). Acute cases are typically due to bacterial infection, and chronic cases are often due to allergies and autoimmune disorders. Risk factors for acute cases include swimming, minor trauma from cleaning, using hearing aids and ear plugs, and other skin problems, such as psoriasis and dermatitis. People with diabetes are at risk of a severe form of "malignant otitis externa". Diagnosis is based on the signs and symptoms. Culturing the ear canal may be useful in chronic or severe cases.

Acetic acid ear drops may be used as a preventative measure. Treatment of acute cases is typically with antibiotic drops, such as ofloxacin or acetic acid. Steroid drops may be used in addition to antibiotics. Pain medications such as ibuprofen may be used for the pain. Antibiotics by mouth are not recommended unless the person has poor immune function or there is infection of the skin around the ear. Typically, improvement occurs within a day of the start of treatment. Treatment of chronic cases depends on the cause.

Otitis externa affects 1–3% of people a year; more than 95% of cases are acute. About 10% of people are affected at some point in their lives. It occurs most commonly among children between the ages of seven and twelve and among the elderly. It occurs with near equal frequency in males and females. Those who live in warm and wet climates are more often affected.

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.

Otitis is a general term for inflammation or infection of the ear, in both humans and other animals.

It is subdivided into the following:

- "Otitis externa", external otitis, or "swimmer's ear" involves the outer ear and ear canal. In external otitis, the ear hurts when touched or pulled.

- "Otitis media" or middle ear infection involves the middle ear. In otitis media, the ear is infected or clogged with fluid behind the ear drum, in the normally air-filled middle-ear space. This very common childhood infection sometimes requires a surgical procedure called "myringotomy" and tube insertion.

- "Otitis interna" or labyrinthitis involves the inner ear. The inner ear includes sensory organs for balance and hearing. When the inner ear is inflamed, "vertigo" is a common symptom.

Surfer's ear is the common name for an exostosis or abnormal bone growth within the ear canal. Surfer's ear is not the same as swimmer's ear, although infection can result as a side effect.

Irritation from cold wind and water exposure causes the bone surrounding the ear canal to develop lumps of new bony growth which constrict the ear canal. Where the ear canal is actually blocked by this condition, water and wax can become trapped and give rise to infection. The condition is so named due to its prevalence among cold water surfers. Warm water surfers are also at risk for exostosis due to the evaporative cooling caused by wind and the presence of water in the ear canal.

Most avid surfers have at least some mild bone growths (exostoses), causing little to no problems. The condition is progressive, making it important to take preventative measures early, preferably whenever surfing.

The condition is not limited to surfing and can occur in any activity with cold, wet, windy conditions such as windsurfing, kayaking, sailing, jet skiing, kitesurfing, and diving.

Prominent ear, otapostasis or bat ear is an abnormally protruding human ear. It may be unilateral or bilateral. The concha is large with poorly developed antihelix and scapha. It is the result of malformation of cartilage during primitive ear development in intrauterine life. The deformity can be corrected anytime after 6 years. The surgery is preferably done at the earliest in order to avoid psychological distress. Correction by otoplasty involves changing the shape of the ear cartilage so that the ear is brought closer to the side of the head. The skin is not removed, but the shape of the cartilage is altered. The surgery does not affect hearing. It is done for cosmetic purposes only. The complications of the surgery, though rare, are keloid formation, hematoma formation, infection and asymmetry between the ears.

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.

Occasionally a preauricular sinus or cyst can become infected.

Most preauricular sinuses are asymptomatic, and remain untreated unless they become infected too often. Preauricular sinuses can be excised with surgery which, because of their close proximity to the facial nerve, is performed by an appropriately trained, experienced surgeon (e.g. a specialist General Surgeon, a Plastic Surgeon, an otolaryngologist (Ear, Nose, Throat surgeon) or an Oral and Maxillofacial Surgeon).

Preauricular sinuses and cysts result from developmental defects of the first and second pharyngeal arches. This and other congenital ear malformations are sometimes associated with renal anomalies. They may be present in Beckwith–Wiedemann syndrome, and in rare cases, they may be associated with branchio-oto-renal syndrome.

Ear pain can be caused by disease in the external or middle ear(because of infection), or inner ear, but the three are indistinguishable in terms of the pain experienced.

External ear pain may be:

- Mechanical: trauma, foreign bodies such as hairs, insects or cotton buds.

- Infective (otitis externa): "Staphylococcus", "Pseudomonas", "Candida", herpes zoster, or viral Myringitis. (See Otitis externa)

Middle ear pain may be:

- Mechanical: barotrauma (often iatrogenic), Eustachian tube obstruction leading to acute otitis media.

- Inflammatory / infective: acute otitis media, mastoiditis.

The symptoms of endolymphatic hydrops include the feeling of pressure or fullness in the ears, hearing loss, tinnitus (ringing in the ears) and balance problems. Individuals who have Meniere’s disease have a degree of endolymphatic hydrops that is strong enough to trigger the symptoms of this disease, but individuals with endolymphatic hydrops do not always progress to Meniere’s disease.

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.

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).

Endolymphatic hydrops is a disorder of the inner ear. It consists of an excessive build-up of the endolymph fluid, which fills the hearing and balance structures of the inner ear. Endolymph fluid, which is partly regulated by the endolymph sac, flows through the inner ear and is critical to the function of all sensory cells in the inner ear. In addition to water, endolymph fluid contains salts such as sodium, potassium, chloride and other electrolytes. If the inner ear is damaged by disease or injury, the volume and composition of the endolymph fluid can change, causing the symptoms of endolymphatic hydrops.

Ear pain, also known as otalgia , is pain in the ear. Primary ear pain is pain that originates inside the ear. Referred ear pain is pain that originates from outside the ear.

Ear pain is not always associated with ear disease. It may be caused by several other conditions, such as impacted teeth, sinus disease, inflamed tonsils, infections in the nose and pharynx, throat cancer, and occasionally as a sensory aura that precedes a migraine.

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.