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

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.

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.

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.

According to current research, in approximately 2.5% of the general population the bones of the head develop to only 60–70% of their normal thickness in the months following birth. This genetic predisposition may explain why the section of temporal bone separating the superior semicircular canal from the cranial cavity, normally 0.8 mm thick, shows a thickness of only 0.5 mm, making it more fragile and susceptible to damage through physical head trauma or from slow erosion. An explanation for this erosion of the bone has not yet been found.

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

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.

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.

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.

"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.

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.

Anotia ("no ear") describes a rare congenital deformity that involves the complete absence of the pinna, the outer projected portion of the ear, and narrowing or absence of the ear canal. This contrasts with microtia, in which a small part of the pinna is present. Anotia and microtia may occur unilaterally (only one ear affected) or bilaterally (both ears affected). This deformity results in conductive hearing loss, deafness.

There are four grades of microtia:

- Grade I: A less than complete development of the external ear with identifiable structures and a small but present external ear canal

- Grade II: A partially developed ear (usually the top portion is underdeveloped) with a closed [stenotic] external ear canal producing a conductive hearing loss.

- Grade III: Absence of the external ear with a small peanut-like vestige structure and an absence of the external ear canal and ear drum. Grade III microtia is the most common form of microtia.

- Grade IV: Absence of the total ear or anotia.

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.

In aviation and underwater diving, alternobaric vertigo is dizziness resulting from unequal pressures being exerted between the ears due to one Eustachian tube being less patent than the other.

Mondini dysplasia, also known as Mondini malformation and Mondini defect, is an abnormality of the inner ear that is associated with sensorineural hearing loss.

This deformity was first described in 1791 by Mondini after examining the inner ear of a deaf boy. The Mondini dysplasia describes a cochlea with incomplete partitioning and a reduced number of turns, an enlarged vestibular aqueduct and a dilated vestibule. A normal cochlea has two and a half turns, a cochlea with Mondini dysplasia has one and a half turns; the basal turns being normally formed with a dilated or cystic apical turn to the cochlear. The hearing loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth.

Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early adult life. Some children may pass newborn hearing screen to lose hearing in infancy but others present with a hearing loss at birth. Hearing loss is often progressive and because of the associated widened vestibular aqueduct may progress in a step-wise fashion associated with minor head trauma. Vestibular function is also often affected. While the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. The Mondini dysplasia can occur in cases of Pendred Syndrome and Branchio-oto-renal syndrome and in other syndromes, but can occur in non-syndromic deafness.

Many patients will report a history of vertigo as a result of fast head movements. Many patients are also capable of describing the exact head movements that provoke their vertigo. Purely horizontal nystagmus and symptoms of vertigo lasting more than one minute can also indicate BPPV occurring in the horizontal semicircular canal.

Patients do not experience other neurological deficits such as numbness or weakness, and if these symptoms are present, a more serious etiology, such as posterior circulation stroke or ischemia, must be considered.

The spinning sensation experienced from BPPV is usually triggered by movement of the head, will have a sudden onset, and can last anywhere from a few seconds to several minutes. The most common movements patients report triggering a spinning sensation are tilting their heads upwards in order to look at something, and rolling over in bed.

The goal of medical intervention is to provide the best form and function to the underdeveloped ear.

Although large vestibular aqueducts are a congenital condition, hearing loss may not be present from birth. Age of diagnosis ranges from infancy to adulthood, and symptoms include fluctuating and sometimes progressive sensorineural hearing loss and disequilibrium.

The vestibular aqueduct acts as a canal between the inner ear and the cranial cavity. Running through it is a tube called the endolymphatic duct, which normally carries a fluid called endolymph from the inner ear to the endolymphatic sac in the cranial cavity. When the endolymphatic duct and sac are larger than normal, as is the case in large vestibular aqueduct syndrome, endolymph is allowed to travel back from the endolymphatic sac into the inner ear. This often results fluctuations in hearing levels. Enlarged vestibular aqueducts often occur with other inner ear development problems, such as cochlear deformities. Enlarged vestibular aqueducts are part of the classic Mondini deformity. Enlarged vestibular aqueducts with enlarged endolymphatic sacs occur in Pendred syndrome which is caused by a defect on chromosome 7q31.. Enlarged vestibular aqueducts can also occur in Branchio-oto-renal syndrome, CHARGE syndrome and Renal Tubular Acidosis.

Enlarged vestibular aqueducts can be bilateral or unilateral.

Hearing loss caused by large vestibular aqueduct syndrome is not inevitable, although people with the syndrome are at a much higher risk of developing hearing loss than the general population. Hearing loss is very likely.

Reported symptoms include:

- Sensorineural hearing loss

- Vestibular areflexia

- Hearing impairment

- Vertigo

- Nausea and vomiting

- Head movement-dependent oscillopsia

Tumors within the nerve canaliculi initially present with unilateral sensorineural hearing loss, unilateral tinnitus, or disequilibrium (vertigo is rare, on account of the slow growth of neuromas). Speech discrimination out of proportion to hearing loss, difficulty talking on the telephone are frequent accompaniments. Tumors extending into the CPA will likely present with disequilibrium or ataxia depending on the amount of extension on the brainstem. With brainstem extension, midfacial and corneal hypesthesia, hydrocephalus, and other cranial neuropathies become more prevalent.

For example, involvement of CN V from a cerebellopontine mass lesion often results in loss of the ipsilateral (same side of the body) corneal reflex (involuntary blink).

Patients with larger tumours can develop Bruns nystagmus ('dancing eyes') due to compression of the flocculi.

"20% to 40% of children with microtia/anotia will have additional defects that could suggest a syndrome."

Treacher-Collins Syndrome: (TCS) A congenital disorder caused by a defective protein known as treacle, and is characterized by craniofacial deformities; malformed or absent ears are also seen in this syndrome. The effects may be mild, undiagnosed to severe, leading to death. Because the ear defects are much different in this disorder and not only affect the outer ear, but the middle ear as well, reconstructive surgery may not help with the child's hearing and in this case a Bone Anchored Hearing Aid would be best. BAHA will only work, however if the inner ear and nerve are intact.

Goldenhar Syndrome: A rare congenital birth defect that causes abnormalities of facial development. also known as Oculoauricular Dysplasia. The facial anomalies include underdeveloped, asymmetric half of the face. The defect is capable of affecting tissue, muscle, and the underlying bone structure of the side of the face with the abnormality.

Ablepharon-macrostomia Syndrome: (AMS) A rare genetic disorder characterized by various physical anomalies which affect the craniofacial area, the skin, the fingers, and the genitals.

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.

Symptoms typically include imbalance and visual problems. Dark or unsure situations generally increase this imbalance.The imbalance is worse in the dark or in situations where footing is uncertain. Spinning vertigo is unusual. Oscillopsia, visual symptoms of Bilateral Vestibulopathy only occur when the head is moving. For instance, when driving, a person with Bilateral Vestibulopathy may see very blurry objects. Oscillopsia is often common during walking. Transient visual blurring occurs with quick movements of the head.