In cases where the causes are environmental, the treatment is to eliminate or reduce these causes first of all, and then to fit patients with a hearing aid, especially if they are elderly. When the loss is due to heredity, total deafness is often the end result. On the one hand, persons who experience gradual deterioration of their hearing are fortunate in that they have learned to speak. Ultimately the affected person may bridge communication problems by becoming skilled in sign language, speech-reading, using a hearing aid, or accepting elective surgery to use a prosthetic devices such as a cochlear implant.

Spatial hearing loss, refers to a form of deafness that is an inability to use spatial cues about where a sound originates from in space. This in turn affects the ability to understand speech in the presence of background noise.

As of 2012 there has only been one small-scale study comparing CROS systems.

One study of the BAHA system showed a benefit depending on the patient's transcranial attenuation. Another study showed that sound localisation was not improved, but the effect of the head shadow was reduced.

Cortical deafness is a rare form of sensorineural hearing loss caused by damage to the primary auditory cortex. Cortical deafness is an auditory disorder where the patient is unable to hear sounds but has no apparent damage to the anatomy of the ear (see auditory system), which can be thought of as the combination of auditory verbal agnosia and auditory agnosia. Patients with cortical deafness cannot hear any sounds, that is, they are not aware of sounds including non-speech, voices, and speech sounds. Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.

Cortical deafness is caused by bilateral cortical lesions in the primary auditory cortex located in the temporal lobes of the brain. The ascending auditory pathways are damaged, causing a loss of perception of sound. Inner ear functions, however, remains intact. Cortical deafness is most often cause by stroke, but can also result from brain injury or birth defects. More specifically, a common cause is bilateral embolic stroke to the area of Heschl's gyri. Cortical deafness is extremely rare, with only twelve reported cases. Each case has a distinct context and different rates of recovery.

It is thought that cortical deafness could be a part of a spectrum of an overall cortical hearing disorder. In some cases, patients with cortical deafness have had recovery of some hearing function, resulting in partial auditory deficits such as auditory verbal agnosia. This syndrome might be difficult to distinguish from a bilateral temporal lesion such as described above.

Currently, no forms of treatment have proven effective in treating amusia. One study has shown tone differentiation techniques to have some success, however future research on treatment of this disorder will be necessary to verify this technique as an appropriate treatment.

Universal Newborn Hearing Screenings (UNHS) is mandated in a majority of the United States. Auditory neuropathy is sometimes difficult to catch right away, even with these precautions in place. Parental suspicion of a hearing loss is a trustworthy screening tool for hearing loss, too; if it is suspected, that is sufficient reason to seek a hearing evaluation from an audiologist.

In most parts of Australia, hearing screening via AABR testing is mandated, meaning that essentially all congenital (i.e., not those related to later onset degenerative disorders) auditory neuropathy cases should be diagnosed at birth.

Auditory perception can improve with time.There seems to be a level of neuroplasticity that allows patients to recover the ability to perceive environmental and certain musical sounds. Patients presenting with cortical hearing loss and no other associated symptoms recover to a variable degree, depending on the size and type of the cerebral lesion. Patients whose symptoms include both motor deficits and aphasias often have larger lesions with an associated poorer prognosis in regard to functional status and recovery.

Cochlear or auditory brainstem implantation could also be treatment options. Electrical stimulation of the peripheral auditory system may result in improved sound perception or cortical remapping in patients with cortical deafness. However, hearing aids are an inappropriate answer for cases like these. Any auditory signal, regardless if has been amplified to normal or high intensities, is useless to a system unable to complete its processing. Ideally, patients should be directed toward resources to aid them in lip-reading, learning American Sign Language, as well as speech and occupational therapy. Patients should follow-up regularly to evaluate for any long-term recovery.

At present, presbycusis, being primarily sensorineural in nature, cannot be prevented, ameliorated or cured. Treatment options fall into three categories: pharmacological, surgical and management.

- There are no approved or recommended pharmaceutical treatments for presbycusis.

Prelingual hearing loss can be either acquired, meaning it occurred after birth due to illness or injury, or it can be congenital, meaning it was present at birth. Congenital hearing loss can be caused by genetic or nongenetic factors. The nongenetic factors account for about one fourth of the congenital hearing losses in infants. These factors could include: Maternal infections, such as rubella, cytomegalovirus, or herpes simplex virus, lack of oxygen, maternal diabetes, toxemia during pregnancy, low birth weight, prematurity, birth injuries, toxins including drugs and alcohol consumed by the mother during pregnancy, and complications associated with the Rh factor in the blood/jaundice. Genetic factors account for over half of the infants with congenital hearing loss. Most of these are caused by an autosomal recessive hearing loss or an autosomal dominant hearing loss. Autosomal recessive hearing loss is when both parents carry the recessive gene, and pass it on to their child. The autosomal dominant hearing loss is when an abnormal gene from one parent is able to cause hearing loss even though the matching gene from the other parent is normal.

In some cases, the loss is extremely sudden and can be traced to specific diseases, such as meningitis, or to ototoxic medications, such as Gentamicin. In both cases, the final degree of loss varies. Some experience only partial loss, while others become profoundly deaf. Hearing aids and cochlear implants may be used to regain a sense of hearing, with different people experiencing differing degrees of success. It is possible that the affected person may need to rely on speech-reading and/or sign language for communication.

In most cases the loss is a long term degradation in hearing loss. Discrediting earlier notions of presbycusis, Rosen demonstrated that long term hearing loss is usually the product of chronic exposure to environmental noise in industrialized countries (Rosen, 1965). The U.S. Environmental Protection Agency has asserted the same sentiment and testified before the U.S. Congress that approximately 34 million Americans are exposed to noise pollution levels (mostly from roadway and aircraft noise) that expose humans to noise health effects including the risk of hearing loss (EPA, 1972).

Certain genetic conditions can also lead to post-lingual deafness. In contrast to genetic causes of pre-lingual deafness, which are frequently autosomal recessive, genetic causes of post-lingual deafness tend to be autosomal dominant.

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.

Each year in the United States, approximately 12,000 babies are born with hearing loss. Profound hearing loss occurs in somewhere between 4 to 11 per every 10,000 children.

About half of people with SSNHL will recover some or all of their hearing spontaneously, usually within one to two weeks from onset. Eighty-five percent of those who receive treatment from an otolaryngologist (sometimes called an ENT) will recover some of their hearing.

- vitamins and antioxidants

- vasodilators

- betahistine (Betaserc), an anti-vertigo drug

- hyperbaric oxygen

- anti-inflammatory agents, primarily oral corticosteroids such as prednisone, methylprednisone

- Intratympanic administration - Gel formulations are under investigation to provide more consistent drug delivery to the inner ear. Local drug delivery can be accomplished through intratympanic administration, a minimally invasive procedure where the ear drum is anesthetized and a drug is administered into the middle ear. From the middle ear, a drug can diffuse across the round window membrane into the inner ear. Intratympanic administration of steroids may be effective for sudden sensorineural hearing loss for some patients, but high quality clinical data has not been generated. Intratympanic administration of an anti-apoptotic peptide (JNK inhibitor) is currently being evaluated in late-stage clinical development.

Research has shown that PC based spatial hearing training software can help some of the children identified as failing to develop their spatial hearing skills (perhaps because of frequent bouts of otitis media with effusion). Further research is needed to discover if a similar approach would help those over 60 to recover the loss of their spatial hearing. One such study showed that dichotic test scores for the left ear improved with daily training. Related research into the plasticity of white-matter (see Lövdén et al. for example) suggests some recovery may be possible.

Music training leads to superior understanding of speech in noise across age groups and musical experience protects against age-related degradation in neural timing. Unlike speech (fast temporal information), music (pitch information) is primarily processed by areas of the brain in the right hemisphere. Given that it seems likely that the right ear advantage (REA) for speech is present from birth, it would follow that a left ear advantage for music is also present from birth and that MOC efferent inhibition (of the right ear) plays a similar role in creating this advantage. Does greater exposure to music increase conscious control of cochlear gain and inhibition? Further research is needed to explore the apparent ability of music to promote an enhanced capability of speech in noise recognition.

Bilateral digital hearing aids do not preserve localization cues (see, for example, Van den Bogaert et al., 2006) This means that audiologists when fitting hearing aids to patients (with a mild to moderate age related loss) risk negatively impacting their spatial hearing capability. With those patients who feel that their lack of understanding of speech in background noise is their primary hearing difficulty then hearing aids may simply make their problem even worse - their spatial hearing gain will be reduced by in the region of 10 dB. Although further research is needed, there is a growing number of studies which have shown that open-fit hearing aids are better able to preserve localisation cues (see, for example, Alworth 2011)

Treatment options that offer “cures” for NIHL are under research and development. Currently there are no commonly used cures, but rather assistive devices and therapies to try and manage the symptoms of NIHL.

NIHL can be prevented through the use of simple, widely available, and economical tools. This includes but is not limited to personal noise reduction through the use of ear protection (i.e. earplugs and earmuffs), education, and hearing conservation programs. For the average person, there are three basic things that can be kept in mind to reduce NIHL, “walk away, turn it down, protect your ears.”

Non-occupational noise exposure is not regulated or governed in the same manner as occupational noise exposure; therefore prevention efforts rely heavily on education awareness campaigns and public policy. The WHO cites that nearly half of those affected by hearing loss could have been prevented through primary prevention efforts such as: “reducing exposure (both occupational and recreational) to loud sounds by raising awareness about the risks; developing and enforcing relevant legislation; and encouraging individuals to use personal protective devices such as earplugs and noise-cancelling earphones and headphones.”

A 2005 study achieved successful regrowth of cochlea cells in guinea pigs. However, the regrowth of cochlear hair cells does not imply the restoration of hearing sensitivity, as the sensory cells may or may not make connections with neurons that carry the signals from hair cells to the brain. A 2008 study has shown that gene therapy targeting Atoh1 can cause hair cell growth and attract neuronal processes in embryonic mice. Some hope that a similar treatment will one day ameliorate hearing loss in humans.

Recent research, reported in 2012 achieved growth of cochlear nerve cells resulting in hearing improvements in gerbils, using stem cells. Also reported in 2013 was regrowth of hair cells in deaf adult mice using a drug intervention resulting in hearing improvement. The Hearing Health Foundation in the US has embarked on a project called the Hearing Restoration Project. Also Action on Hearing Loss in the UK is also aiming to restore hearing.

Researchers reported in 2015 that genetically deaf mice which were treated with TMC1 gene therapy recovered some of their hearing. In 2017, additional studies were performed to treat Usher syndrome and here, a recombinant adeno-associated virus seemed to outperform the older vectors.

Presbycucis is the leading cause of SNHL and is progressive and nonpreventable, and at this time, we do not have either somatic or gene therapy to counter heredity-related SNHL. But other causes of acquired SNHL are largely preventable, especially nosocusis type causes. This would involve avoiding environmental noise, and traumatic noise such as rock concerts and nightclubs with loud music. Use of noise attenuation measures like acoustic ear plugs is an alternative.

Auditory neuropathy (AN) is a variety of hearing loss in which the outer hair cells within the cochlea are present and functional, but sound information is not faithfully transmitted to the auditory nerve and brain properly. Also known as auditory neuropathy/auditory dys-synchrony (AN/AD) or auditory neuropathy spectrum disorder (ANSD).

A neuropathy usually refers to a disease of the peripheral nerve or nerves, but the auditory nerve itself is not always affected in auditory neuropathy spectrum disorders.

Pharmacological treatment options are limited, and remain clinically unproven. Among these are the water-soluble coenzyme Q10 formulation, the prescription drug Tanakan, and combination antioxidant therapy.

- In a study performed in 2010, it was found that the water-soluble formulation of coenzyme Q10 (CoQ10) caused a significant improvement in liminar tonal audiometry of the air and bone thresholds at 1000 Hz, 2000 Hz, 4000 Hz, and 8000 Hz.

- Antioxidant therapy - age-related hearing loss was reduced in animal models with a combination agent comprising six antioxidant agents that target four sites within the oxidative pathway: L-cysteine-glutathione mixed disulfide, ribose-cysteine, NW-nitro-L-arginine methyl ester, vitamin B12, folate, and ascorbic acid. It is thought that these supplements attenuate the decline of cochlear structure due to prolonged oxidative stress. However, more recent studies have had conflicting results. In 2012, a study was done with CBA/J female mice. They were placed on an antioxidant-rich diet for 24 months consisting of vitamins A, C, E, L-carnitine, and α-lipoic acid. While this increased the inner ear’s antioxidant capacity, the actual hearing loss was unaffected. Therefore, in this study, antioxidants were shown not to improve presbycusis mechanisms.

- The effects of the pharmaceutical drug Tanakan were observed when treating tympanophonia in elderly women. Tanakan was found to decrease the intensity of tympanitis and improve speech and hearing in aged patients, giving rise to the idea of recommending treatment with it to elderly patients with presbycusis or normal tonal hearing.

- AM-111, an otoprotective peptide, was shown in a chinchilla study to rescue and protect against hearing loss following impulse noise trauma. AM-111 acts as a cell-permeable inhibitor of JNK-mediated apoptosis. IP injections or local injections into membrane of the round window were given, and permanent threshold shifts (PTS) were measured three weeks after impulse noise exposure. AM-111 animals had significantly lower PTS, implicating AM-111 as a possible protective agent against JNK-mediated cochlear cell death and against permeant hearing deficits after noise trauma.

- The anti-inflammatory, anti-oxidant substance Ebselen was observed to reduce hearing loss in a study done in 2007. . It has been previously shown that noise trauma correlates with decreases in glutathione peroxidase (GPx) activity, which has been linked to loss of the outer hair cells. GPx1, an isoform of GPx, is predominantly expressed in stria vascularis, cochlea, spiral ligament, organ of Corti, and spiral ganglion cells. The stria vascularis displayed significant decreases in GPx1 immunoreactivity and increased swelling following noise exposure in rats. There was also significant outer hair cell loss in the cochlea within five hours of noise exposure. Administration of Ebselen before and after the noise stimulus reduced stria vascularis swelling as well as cochlear outer hair cell loss. This implicates Ebselen as a supplement for GPx1 in the outer hair cell degradation mechanism of hearing loss. This treatment is currently in active clinical trials.

- A γ-secretase inhibitor of Notch signaling was shown to induce new hair cells and partially recover hearing loss . Auditory hair cell loss is permanent damage due to the inability of these cells to regenerate. Therefore, deafness due to this pathology is viewed as irreversible. Hair cell development is mediated by Notch signaling, which exerts lateral inhibition onto hair cells. Notch signaling in supporting hair cells leads to prevention of differentiation in surrounding hair cells. After identifying a potent γ-secretase inhibitor selective for stimulating differentiation in inner ear stem cells, it was administered in acoustically injured mice. The animals who received the injury and treatment displayed an increased hair cell number and stimulated hearing recovery. This suggests that γ-secretase inhibition of Notch signaling can be a potential pharmacological therapy in approaching what was previously viewed as permeant deafness.

Globally, hearing loss affects about 10% of the population to some degree. It caused moderate to severe disability in 124.2 million people as of 2004 (107.9 million of whom are in low and middle income countries). Of these 65 million acquired the condition during childhood. At birth ~3 per 1000 in developed countries and more than 6 per 1000 in developing countries have hearing problems.

Hearing loss increases with age. In those between 20 and 35 rates of hearing loss are 3% while in those 44 to 55 it is 11% and in those 65 to 85 it is 43%.

A 2017 report by the World Health Organization estimated the costs of unaddressed hearing loss and the cost-effectiveness of interventions, for the health-care sector, for the education sector and as broad societal costs. Globally, the annual cost of unaddressed hearing loss was estimated to be in the range of $750–790 billion international dollars.

Over the past decade, much has been discovered about amusia. However, there remains a great deal more to learn. While a method of treatment for people with amusia has not been defined, tone differentiation techniques have been used on amusic patients with some success. It was found with this research that children reacted positively to these tone differentiation techniques, while adults found the training annoying. However, further research in this direction would aid in determining if this would be a viable treatment option for people with amusia. Additional research can also serve to indicate which processing component in the brain is essential for normal music development. Also, it would be extremely beneficial to investigate musical learning in relation to amusia since this could provide valuable insights into other forms of learning disabilities such as dysphasia and dyslexia.

The hierarchy of controls provides a visual guide to the effectiveness of the various workplace controls set in place to eliminate or reduce exposure to occupational hazards, including noise or ototoxic chemicals. The hierarchy includes the following from most effective to least effective:

- Elimination: complete removal of the hazard

- Substitution: replacement the offers a smaller risk

- Engineering controls: physical changes to reduce exposure

- Administrative controls: changes in work procedures or training

- Personal protective equipment (PPE): individual equipment to reduce exposure, e.g. earplugs

Administrative control, behind engineering control, is the next best form of prevention of noise exposure. They can either reduce the exposure to noise, or reduce the decibel level of the noise itself. Limiting the amount of time a worker is allowed to be around an unsafe level of noise exposure, and creating procedures for operation of equipment that could produce harmful levels of noise are both examples of administrative controls.

A child with a congenital hearing loss should begin receiving treatment before 6 months of age. Studies suggest that children treated this early are usually able to develop communication skills (using spoken or sign language) that are as good as those of hearing peers.

In the United States of America, because of a Federal law (the Individuals with Disabilities Education Act), children with a hearing loss between birth and 3 years of age have the right to receive interdisciplinary assessment and early intervention services at little or no cost. After age 3, early intervention and special education programs are provided through the public school system.

There are a number of treatment options available, and parents will need to decide which are most appropriate for their child. They will need to consider the child’s age, developmental level and personality, the severity of the hearing loss, as well as their own preferences. Ideally a team of experts including the child’s primary care provider, an otolaryngologist, a speech-language pathologist, audiologist and an educator will work closely with the parents to create an Individualized Family Service Plan. Treatment plans can be changed as the child gets older.

Children as young as 4 weeks of age can benefit from a hearing aid. These devices amplify sound, making it possible for many children to hear spoken words and develop language. However, some children with severe to profound hearing loss may not be able to hear enough sound, even with a hearing aid, to make speech audible. A behind-the-ear hearing aid is often recommended for young children because it is safer and more easily fitted and adjusted as the child grows as compared to one that fits within the ear.

Parents also will need to decide how their family and child are going to communicate. If the child is going to communicate orally (speech), s/he may need assistance learning listening skills and lip reading skills to help her/him understand what others are saying. Many children with hearing loss also need speech or language therapy.

A child also can learn to communicate using a form of sign language. In the United States of America, the type preferred by most deaf adults is American Sign Language (ASL), which has rules and grammar that is distinct from English. There are also several variations of sign language that can be used along with spoken English which are standard in English-speaking countries outside the United States.

There is also a visual model of spoken language called cued speech. Learning to lip read is very difficult because many sounds look the same on the lips. Cued speech enables young children with hearing loss to clearly see what is being said, and learn spoken languages with normal grammar and vocabulary. It clarifies lip reading using 8 hand shapes in 4 positions and usually takes less than 20 hours to learn the entire system.

Surgery may be recommended if a child has a permanent conductive hearing loss caused by malformations of the outer or middle ear, or by repeated ear infections. Although fluid in the middle ear usually results in only temporary hearing loss, chronic ear infection can cause a child to fall behind in language skills. In some cases, a doctor may suggest inserting a tube through the eardrum to allow the middle ear to drain. This procedure generally does not require an overnight hospital stay.

Surgery also may be an option for some children with severe to profound sensorineural hearing loss. A device called a cochlear implant can be surgically inserted in the inner ear of children as young as 12 months of age to stimulate hearing. The surgery requires a hospital stay of one to several days. With additional language and speech therapy, children with cochlear implants may learn to understand speech and speak reasonably well, but the amount of improvement is variable.

Once a child is diagnosed, the immediate and anticipated reaction of the parents and immediate family is one of the denial. Doctors or the audiologists need to counsel the family, help them cope with the situation and encourage them to look forward to solutions to overcome the problem. Often when the family is told about the excellent options available for a hearing impaired child, the chances of acceptance are much better. Once the family accepts the handicap, half the battle is over and rehabilitation can begin.

The type of intervention required depends on several factors. Chief among these is the degree of impairment. When a child has a fair degree of residual hearing, the correct intervention would be fitting "optimised" hearing aids. "Optimisation" means fitting the child with a hearing aid appropriate to its degree of deafness.

Today a variety of good quality hearing aids are available – analog or digital body worn (for small children) or ear level for older children. When fitting a hearing aid, a competent audiologist has to assess the child's residual hearing, look at the hearing aid's performance and fit the child with an appropriate instrument. Equally important is the ear mould, which has to be custom made to suit the shape of the child's ear.

If a child has profound or total deafness, the benefits of hearing aids are limited. Depending upon the level and type of hearing loss, cochlear implants may be used instead of hearing aids.