Since the inner ear is not directly accessible to instruments, identification is by patient report and audiometric testing. Of those who present to their doctor with sensorineural hearing loss, 90% report having diminished hearing, 57% report having plugged feeling in ear, and 49% report having ringing in ear (tinnitus). About half report vestibular (vertigo) problems.

For a detailed exposition of symptoms useful for screening, a self-assessment questionnaire was developed by the American Academy of Otolaryngology, called the Hearing Handicap Inventory for Adults (HHIA). It is a 25-question survey of subjective symptoms.

Sensorineural hearing loss (SNHL) is a type of hearing loss, or deafness, in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the vestibulocochlear nerve (cranial nerve VIII) or neural part. SNHL accounts for about 90% of hearing loss reported. SNHL is generally permanent and can be mild, moderate, severe, profound, or total. Various other descriptors can be used such as high frequency, low frequency, U-shaped, notched, peaked or flat depending on the shape of the audiogram, the measure of hearing.

"Sensory" hearing loss often occurs as a consequence of damaged or deficient cochlear hair cells. Hair cells may be abnormal at birth, or damaged during the lifetime of an individual. There are both external causes of damage, including noise trauma, infection and ototoxic drugs, as well as intrinsic causes, including genetic mutations. A common cause or exacerbating factor in sensory hearing loss is prolonged exposure to environmental noise, for example, being in a loud workplace without wearing protection, or having headphones set to high volumes for a long period. Exposure to a very loud noise such as a bomb blast can cause noise-induced hearing loss.

"Neural", or 'retrocochlear', hearing loss occurs because of damage to the cochlear nerve (CVIII). This damage may affect the initiation of the nerve impulse in the cochlear nerve or the transmission of the nerve impulse along the nerve into the brainstem.

Most cases of SNHL present with a gradual deterioration of hearing thresholds occurring over years to decades. In some the loss may eventually affect large portions of the frequency range. It may be accompanied by other symptoms such as ringing in the ears (tinnitus), dizziness or lightheadedness (vertigo). SNHL can be genetically inherited or acquired as a result from external causes like noise or disease. It may be congenital (present at birth) or develop later in life. The most common kind of sensorineural hearing loss is age-related (presbycusis), followed by noise-induced hearing loss (NIHL).

Frequent symptoms of SNHL are loss of acuity in distinguishing foreground voices against noisy backgrounds, difficulty understanding on the telephone, some kinds of sounds seeming excessively loud or shrill (recruitment), difficulty understanding some parts of speech (fricatives and sibilants), loss of directionality of sound, esp. high frequency sounds, perception that people mumble when speaking, and difficulty understanding speech. Similar symptoms are also associated with other kinds of hearing loss; audiometry or other diagnostic tests are necessary to distinguish sensorineural hearing loss.

Identification of sensorineural hearing loss is usually made by performing a pure tone audiometry (an audiogram) in which bone conduction thresholds are measured. Tympanometry and speech audiometry may be helpful. Testing is performed by an audiologist.

There is no proven or recommended treatment or cure for SNHL; management of hearing loss is usually by hearing strategies and hearing aid. In cases of profound or total deafness, a cochlear implant is a specialised hearing aid which may restore a functional level of hearing. SNHL is at least partially preventable by avoiding environmental noise, ototoxic chemicals and drugs, and head trauma, and treating or inoculating against certain triggering diseases and conditions like meningitis.

Conductive hearing loss makes all sounds seem faint or muffled. The hearing loss is worse in low frequencies.

Congenital conductive hearing loss is usually identified through newborn hearing screening or may be identified because the baby has microtia or other facial abnormalities. Conductive hearing loss developing during childhood is usually due to otitis media with effusion and may present with speech and language delay or difficulty hearing. Later onset of conductive hearing loss may have an obvious cause such as an ear infection, trauma or upper respiratory tract infection or may have an insidious onset related to chronic middle ear disease, otosclerosis or a tumour of the naso-pharynx. Earwax is a very common cause of a conductive hearing loss which may present suddenly when water gets behind the wax and this blocks the ear canal.

Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Genetic changes are related to the following types of nonsyndromic deafness.

- DFNA: nonsyndromic deafness, autosomal dominant

- DFNB: nonsyndromic deafness, autosomal recessive

- DFNX: nonsyndromic deafness, X-linked

- nonsyndromic deafness, mitochondrial

Each type is numbered in the order in which it was described. For example, DFNA1 was the first described autosomal dominant type of nonsyndromic deafness. Mitochondrial nonsyndromic deafness involves changes to the small amount of DNA found in mitochondria, the energy-producing centers within cells.

Most forms of nonsyndromic deafness are associated with permanent hearing loss caused by damage to structures in the inner ear. The inner ear consists of three parts: a snail-shaped structure called the cochlea that helps process sound, nerves that send information from the cochlea to the brain, and structures involved with balance. Loss of hearing caused by changes in the inner ear is called sensorineural deafness. Hearing loss that results from changes in the middle ear is called conductive hearing loss. The middle ear contains three tiny bones that help transfer sound from the eardrum to the inner ear. Some forms of nonsyndromic deafness involve changes in both the inner ear and the middle ear; this combination is called mixed hearing loss.

The severity of hearing loss varies and can change over time. It can affect one ear (unilateral) or both ears (bilateral). Degrees of hearing loss range from mild (difficulty understanding soft speech) to profound (inability to hear even very loud noises). The loss may be stable, or it may progress as a person gets older. Particular types of nonsyndromic deafness often show distinctive patterns of hearing loss. For example, the loss may be more pronounced at high, middle, or low tones.

Nonsyndromic deafness can occur at any age. Hearing loss that is present before a child learns to speak is classified as prelingual or congenital. Hearing loss that occurs after the development of speech is classified as postlingual.

There are four main types of hearing loss, conductive hearing loss, sensorineural hearing loss, central deafness and combinations of conductive and sensorineural hearing losses which is called mixed hearing loss. An additional problem which is increasingly recognised is auditory processing disorder which is not a hearing loss as such but a difficulty perceiving sound.

- Conductive hearing loss

Conductive hearing loss is present when the sound is not reaching the inner ear, the cochlea. This can be due to external ear canal malformation, dysfunction of the eardrum or malfunction of the bones of the middle ear. The ear drum may show defects from small to total resulting in hearing loss of different degree. Scar tissue after ear infections may also make the ear drum dysfunction as well as when it is retracted and adherent to the medial part of the middle ear.

Dysfunction of the three small bones of the middle ear – malleus, incus, and stapes – may cause conductive hearing loss. The mobility of the ossicles may be impaired for different reasons and disruption of the ossicular chain due to trauma, infection or ankylosis may also cause hearing loss.

- Sensorineural hearing loss

Sensorineural hearing loss is one caused by dysfunction of the inner ear, the cochlea or the nerve that transmits the impulses from the cochlea to the hearing centre in the brain. The most common reason for sensorineural hearing loss is damage to the hair cells in the cochlea. Depending on the definition it could be estimated that more than 50% of the population over the age of 70 has impaired hearing.

- Central deafness

Damage to the brain can lead to a central deafness. The peripheral ear and the auditory nerve may function well but the central connections are damaged by tumour, trauma or other disease and the patient is unable to hear.

- Mixed hearing loss

Mixed hearing loss is a combination of conductive and sensorineural hearing loss. Chronic ear infection (a fairly common diagnosis) can cause a defective ear drum or middle-ear ossicle damages, or both. In addition to the conductive loss, a sensory component may be present.

- Central auditory processing disorder

This is not an actual hearing loss but gives rise to significant difficulties in hearing. One kind of auditory processing disorder is King-Kopetzky syndrome, which is characterized by an inability to process out background noise in noisy environments despite normal performance on traditional hearing tests.

Congenital hearing loss is a hearing loss present at birth. It can include hereditary hearing loss or hearing loss due to other factors present either in-utero (prenatal) or at the time of birth.

Hearing loss is sensory, but may have accompanying symptoms:

- pain or pressure in the ears

- a blocked feeling

There may also be accompanying secondary symptoms:

- hyperacusis, heightened sensitivity to certain volumes and frequencies of sound, sometimes resulting from "recruitment"

- tinnitus, ringing, buzzing, hissing or other sounds in the ear when no external sound is present

- vertigo and disequilibrium

- tympanophonia, abnormal hearing of one's own voice and respiratory sounds, usually as a result of a patulous eustachian tube or dehiscent superior semicircular canals

- disturbances of facial movement (indicating possible tumour or stroke)

Patients with unilateral hearing loss have difficulty in

- hearing conversation on their impaired side

- localizing sound

- understanding speech in the presence of background noise.

- interpersonal and social relations

- difficulty concentrating in large, open environments

In quiet conditions, speech discrimination is no worse than normal hearing in those with partial deafness; however, in noisy environments speech discrimination is almost always severe.

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.

Individuals with hemifacial microsomia, also called oculoauriculo-vertebral spectrum, often have ear malformations. These malformations can be in the form of preauricular ear pits, complete absence of the auricle, stenosis or atresia of the external auditory canal, ossicular malformations, middle ear deformities, and incomplete pneumatization of the temporal bone. Rahbar "et al." (2001) found that 95% of individuals with this syndrome have an ear malformation of some type. In addition to ear malformations, a conductive hearing loss can be present, typically ranging from mild to severe. There are also reported cases of cochlear involvement and sensorineural hearing loss. Rahbar "et al." (2001) found that 86% of patients with Hemifacial Microsomia have a conductive hearing loss and 10% have a sensorineural hearing loss. There is no correlation between the severity of dysmorphic features and the degree of hearing loss, meaning individuals with mild malformations can have severely impaired hearing.

Other causes of congenital hearing loss that are not hereditary in nature include prenatal infections, illnesses, toxins consumed by the mother during pregnancy or other conditions occurring at the time of birth or shortly thereafter. These conditions typically cause sensorineural hearing loss ranging from mild to profound in degree.

Persons with Pierre Robin sequence (PRS) are at greater risk for hearing impairment than persons with cleft lip and/or palate without PRS. One study showed an average of 83% hearing loss in PRS, compared to 60% in cleft individuals without PRS (Handzic "et al.", 1995). Similarly, PRS individuals typically exhibit conductive, bilateral hearing losses that are greater in degree than in cleft individuals without PRS. Middle ear effusion is generally apparent, with no middle ear or inner ear malformations. Accordingly, management by ear tubes (myringotomy tubes) is often effective and may restore normal levels of hearing (Handzic "et al.", 1995).

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.

Hearing loss is classified as mild, moderate, severe or profound. Pure-tone audiometry for air conduction thresholds at 500, 1000 and 2000 Hz is traditionally used to classify the degree of hearing loss in each ear. Normal hearing thresholds are considered to be 25 dB sensitivity, though it has been proposed that this threshold is too high, and that 15 dB (about half as loud) is more typical. Mild hearing loss is thresholds of 25–45 dB; moderate hearing loss is thresholds of 45–65 dB; severe hearing loss is thresholds of 65–85 dB; and profound hearing loss thresholds are greater than 85 dB.

Tinnitus occurring in only one ear should prompt the clinician to initiate further evaluation for other etiologies. In addition, the presence of a pulse-synchronous rushing sound may require additional imaging to exclude vascular disorders.

TTS imperceptibly gives way to PTS.

In addition to hearing loss, other external symptoms of an acoustic trauma can be:

- Tinnitus

- Some pain in the ear

- Hyperacusis

- Dizziness or vertigo; in the case of vestibular damages, in the inner-ear

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.

Primary symptoms:

- sounds or speech becoming dull, muffled or attenuated

- need for increased volume on television, radio, music and other audio sources

- difficulty using the telephone

- loss of directionality of sound

- difficulty understanding speech, especially women and children

- difficulty in speech discrimination against background noise (cocktail party effect)

Secondary symptoms:

- hyperacusis, heightened sensitivity to certain volumes and frequencies of sound, resulting from "recruitment"

- tinnitus, ringing, buzzing, hissing or other sounds in the ear when no external sound is present

- vertigo and disequilibrium

Usually occurs after age 50, but deterioration in hearing has been found to start very early, from about the age of 18 years. The ISO standard 7029 shows expected threshold changes due purely to age for carefully screened populations (i.e. excluding those with ear disease, noise exposure etc.), based on a meta-analysis of published data. Age affects high frequencies more than low, and men more than women. One early consequence is that even young adults may lose the ability to hear very high frequency tones above 15 or 16 kHz. Despite this, age-related hearing loss may only become noticeable later in life. The effects of age can be exacerbated by exposure to environmental noise, whether at work or in leisure time (shooting, music, etc.). This is noise-induced hearing loss (NIHL) and is distinct from presbycusis. A second exacerbating factor is exposure to ototoxic drugs and chemicals.

Over time, the detection of high-pitched sounds becomes more difficult, and speech perception is affected, particularly of sibilants and fricatives. Patients typically express a decreased ability to understand speech. Once the loss has progressed to the 2-4kHz range, there is increased difficulty understanding consonants. Both ears tend to be affected. The impact of presbycusis on communication depends on both the severity of the condition and the communication partner.

The first symptom of noise-induced hearing loss is usually difficulty hearing a conversation against a noisy background. The effect of hearing loss on speech perception has two components. The first component is the loss of audibility, which is something like a decrease in overall volume. Modern hearing aids compensate this loss with amplification. However, difficulty in understanding speech represents selective frequency loss for which hearing aids and amplification do not help. This is known by different names such as “distortion,” “clarity loss,” and “Signal-to-Noise-Ratio (SNR)-loss.” Consonants, due to their higher frequency, seem to be lost first. For example, the letters “s” and “t” are the common letters that are difficult to hear for those with hearing loss due to them being our highest frequency sound in our language. Hearing loss can affect either one or both ears. When one ear is affected it causes problems with directional hearing. Directional hearing provides the ability to determine from which direction a sound came. Lacking this ability can cause confusion within individuals who have hearing loss in one ear.

Unilateral hearing loss (UHL) or single-sided deafness (SSD) is a type of hearing impairment where there is normal hearing in one ear and impaired hearing in the other ear.

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.

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.

The hearing loss of Pendred syndrome is often, although not always, present from birth, and language acquisition may be a significant problem if deafness is severe in childhood. The hearing loss typically worsens over the years, and progression can be step-wise and related to minor head trauma. In some cases, language development worsens after head injury, demonstrating that the inner ear is sensitive to trauma in Pendred syndrome; this is as a consequence of the widened vestibular aqueducts usual in this syndrome. Vestibular function varies in Pendred syndrome and vertigo can be a feature of minor head trauma. A goitre is present in 75% of all cases.

Imaging is usually not pursued in those with uncomplicated conductive hearing loss and characteristic clinical findings. Those with only conductive hearing loss are often treated medically or with surgery without imaging. The diagnosis may be unclear clinically in cases of sensorineural or mixed hearing loss and may become apparent only on imaging. Therefore, imaging is often performed when the hearing loss is sensorineural or mixed.

A high-resolution CT shows very subtle bone findings. However, CT is usually not needed prior to surgery.

Otosclerosis on CT can be graded using the grading system suggested by Symons and Fanning.

- Grade 1, solely fenestral;

- Grade 2, patchy localized cochlear disease (with or without fenestral involvement) to either the basal cochlear turn (grade 2A), or the middle/apical turns (grade 2B), or both the basal turn and the middle/apical turns (grade 2C); and

- Grade 3, diffuse confluent cochlear involvement (with or without fenestral involvement).

Reported symptoms include:

- Sensorineural hearing loss

- Vestibular areflexia

- Hearing impairment

- Vertigo

- Nausea and vomiting

- Head movement-dependent oscillopsia

Audiometry (measuring ability to hear sounds of a particular pitch) is usually abnormal, but the findings are not particularly specific and an audiogram is not sufficient to diagnose Pendred syndrome. A thyroid goitre may be present in the first decade and is usual towards the end of the second decade. MRI scanning of the inner ear usually shows widened or large vestibular aqueducts with enlarged endolymphatic sacs and may show abnormalities of the cochleae that is known as Mondini dysplasia. Genetic testing to identify the pendrin gene usually establishes the diagnosis. If the condition is suspected, a "perchlorate discharge test" is sometimes performed. This test is highly sensitive, but may also be abnormal in other thyroid conditions. If a goitre is present, thyroid function tests are performed to identify mild cases of thyroid dysfunction even if they are not yet causing symptoms.