In the 1960s and 1970s, several studies were conducted sponsored by the U.S. Atomic Energy Commission, with the aim of finding a link between genetics and hypodontia.

It is difficult to diagnose clinically. Radiographs taken at different angles can aid detection of concrescence. Histological examination for extracted teeth could confirm diagnosis.

MIH examination should be carried out on clean, wet teeth. The ideal age for examination is when the child is 8 years old - the age where all permanent first molars and most of the incisors are erupted. The permanent first molar will also still be in a comparatively sound condition without excessive post-eruption breakdown. Judgements of each individual teeth should be recorded, aiding the correct diagnosis of the condition.

There is currently a lack of standardisation in the scoring system and severity indices used to record the diagnosis of MIH. Various systems commonly employed in studies include:

- Modified Defect of Dental Enamel (DDE) Index: This set of criteria allows for enamel defects to be detected, enabling a distinction between demarcated and diffused opacities.

- European Academy of Paediatric Dentistry (EAPD) judgement criteria: This set of criteria was developed in 2003 to standardise classifications for use in epidemiological studies. However, while it allows the categorisation of the enamel condition, it does not address the severity of the enamel condition.

- Molar Hypomineralisation Severity Index (MHSI): This set of criteria has been developed to address deficiencies in indices concerning the severity of hypomineralisation. It is based on both the clinical characteristics of hypomineralised defects and the EAPD judgement criteria.

The oral rehabilitation of hypodontia, especially where a significant number of teeth have not developed, is often a multidisciplinary process, involving a specialist orthodontist, a consultant in restorative dentistry, and a paediatric dentist in the earlier years. The process of treating and managing hypodontia begins in the early years of the patient's dentition where absent teeth are identified and the process of maintaining the remaining teeth begins. This is largely conducted by the paediatric dentist with orthodontic input. Once all the adult teeth have erupted the orthodontist is likely to liaise with the restorative dentist regarding optimal positioning of teeth for subsequent replacement with prosthodontic methods. This may include the utilisation of a resin-retained bridge and implants for spaces or composite resin, veneers or crowns where teeth are diminutive or misshaped.

AI can be classified according to their clinical appearances:

- Type 1 - Hypoplastic

Enamel of abnormal thickness due to malfunction in enamel matrix formation. Enamel is very thin but hard & translucent, and may have random pits & grooves. Condition is of autosomal dominant, autosomal recessive, or x-linked pattern. Enamel differs in appearance from dentine radiographically as normal functional enamel.

- Type 2 - Hypomaturation

Enamel has sound thickness, with a pitted appearance. It is less hard compared to normal enamel, and are prone to rapid wear, although not as intense as Type 3 AI. Condition is of autosomal dominant, autosomal recessive, or x-linked pattern. Enamel appears to be comparable to dentine in its radiodensity on radiograpshs.

- Type 3 - Hypocalcified

Enamel defect due to malfunction of enamel calcification, therefore enamel is of normal thickness but is extremely brittle, with an opaque/chalky presentation. Teeth are prone to staining and rapid wear, exposing dentine. Condition is of autosomal dominant and autosomal recessive pattern. Enamel appears less radioopaque compared to dentine on radiographs.

- Type 4: Hypomature hypoplastic enamel with taurodontism

Enamel has a variation in appearance, with mixed features from Type 1 and Type 2 AI. All Type 4 AI has taurodontism in common. Condition is of autosomal dominant pattern.

Other common features may include an anterior open bite, taurodontism, sensitivity of teeth.

Differential diagnosis would include dental fluorosis, molar-incisor hypomineralization, chronological disorders of tooth development.

The Kennedy classification quantifies partial edentulism. An outline is covered at the removable partial denture article.

If the condition is not affecting patient, no treatment is needed. Concrescence teeth could be reshaped and replaced with full crowns. However, if the teeth are having recurrent problems, non-restorable or pain, extraction should be considered.

No intervention is usually recommended unless they are causing difficulty to the infant or mother.

However some recommend that they be removed as the tooth can cut or amputate the tip of the tongue.

They should be left in the mouth as long as possible to decrease the likelihood of removing permanent tooth buds with the natal tooth. They should also not be removed if the infant has hypoprothrombinemia. In case of complications when the natal teeth need to be removed, dental radiographs should be obtained whenever possible, and evaluated and followed up with pediatric dentists.

Because this genetic anomaly is genetically linked, genetic counseling may be the only way to decrease occurrences of Cherubism. The lack of severe symptoms in the parents may be the cause of failure in recognizing the disorder. The optimal time to be tested for mutations is prior to having children. The disorder results from a genetic mutation, and this gene has been found to spontaneously mutate. Therefore, there may be no prevention techniques available.

Diagnosis of otodental syndrome was established using clinical, histopathological and audiometric methodologies. In normal individuals, by the age of 2-3, radiograph images should depict any signs of premolar development. A formal diagnosis of no premolar growth can be done by age 6 in order to check for signs of otodental syndrome. Sensorineural hearing loss can be another measure for proper diagnosis as well as checking for ocular coloboma. The latter is usually noticed at an around birth.

Molecular genetic testing can aid in the diagnosis of the affected individual, which would determine if there are any abnormalities in the FGF3 gene (11q13) or the FADD gene (11q13.3). Additional tests that can help diagnose otodental syndrome are ear infection tests, hearing tests, oral examination, and eye examinations to check for the specific phenotypic associations. Due to the rarity of otodental syndrome, most symptoms are looked at on an individual basis unless multiple symptoms are all apparent at once.

There is potential for differential diagnosis due to similarities in symptoms. Other diseases that share common symptoms are chondroectodermal dysplasia, achondrodysplasia, and osteopetrosis

In 2006, retinoids and antibiotics have been used with a successful dental maintenance for one year. In the past, only Extraction of all teeth and construction of a complete denture were made.

An alternative to rehabilitation with conventional dental prothesis after total loss of the natural teeth was proposed by Drs Ahmad Alzahaili and his teacher Jean-François Tulasne (developer of the partial bone graft technique used). This approach entails transplanting bone extracted from the cortical external surface of the parietal bone to the patient’s mouth, affording the patient the opportunity to lead a normal life.

Notwithstanding this treatment do not scope the disease itself. Actually it is the repositioning of bone from calvaria to the maxillary bones, and placement of dental implants in a completely edentulous maxilae, when the patient has already lost all teeth. An already developed method to reconstruct maxillae in edentulous elderly people by other dental professionals.

There's still no real treatment to help those who suffer from this disease to keep all their natural teeth, though their exfoliation and loss can be delayed.

The maintenance of teeth is done by dental professionals with a procedure called scaling and root planing with the use of systemic antibiotics. The syndrome should be diagnosed as earlier as possible, so the teeth can be kept longer in the mouth, helping the development of the maxillary bones.

Management of teeth with PFE can include extractions of affected teeth, followed by orthodontic space closure or placement of a prosthetic implant with a bone graft. This option can only be applied to a single tooth that is affected. If multiple teeth are affected then, a segmental osteotomy may be performed to bring the entire segment into occlusion. However, minimal success has been shown following this procedure. These teeth usually are "non-responsive" to the orthodontic force and studies have shown that ankylosis of these teeth can occur if force applied.

Preventive and restorative dental care is very important as well as considerations for esthetic issues since the crown are yellow from exposure of dentin due to enamel loss. The main objectives of treatment is pain relief, preserving patient's remaining dentition, and to treat and preserve the patient's occlusal vertical height.

Many factors are to be considered to decide on treatment options such as the classification and severity of AI, the patient's social history, clinical findings etc. There are many classifications of AI but the general management of this condition is similar.

Full-coverage crowns are sometimes being used to compensate for the abraded enamel in adults, tackling the sensitivity the patient experiences. Usually stainless steel crowns are used in children which may be replaced by porcelain once they reach adulthood. These aid with maintaining occlusal vertical dimension.

Aesthetics may be addressed via placement of composite or porcelain veneers, depending on patient factors eg age. If the patient has primary or mixed dentition, lab-made composite veneers may be provided temporarily, to be replaced by permanent porcelain veneers once the patient has stabilized permanent dentition. The patient's oral hygiene and diet should be controlled as well as they play a factor in the success of retaining future restorations.

In the worst-case scenario, the teeth may have to be extracted and implants or dentures are required. Loss of nerves in the affected teeth may occur.

Before root canal treatment or extraction are carried out, the clinician should have thorough knowledge about the root canal morphology to avoid complications.

Future studies will look further into the relationship of talon cusp and Rubinstein-Taybi syndrome and other oral-facial-digital syndromes. A former study showed a direct correlation in which 45 affected patients with Rubinstein-Taybi syndrome, 92% of these patients had talon cusp. Other researchers are attempting to trace talon cusp to ancestors and comparing dentition to modern humans. Another study done in 2007 examined the dentition of 301 Native American Indian skeletons for the presence or absence of talon cusp. The results showed five skeletons (2 percent) in the population had the trait.

In 2011, only 21 cases of talon cusp have been reported and are in literature. It appears that as of 2014 and 2015, additional research continues in hopes of finding the cause and mechanism of talon cusp. With the majority of cases of talon cusp being unreported, it remains difficult to conduct tests, come up with conclusions, conduct surgery and perform research with small numbers.

Females are affected more than males, and the condition occurs in permanent (adult) teeth more than deciduous (baby teeth or milk teeth).

Unerupted microdonts may require surgical removal to prevent the formation of cysts. Erupted microdonts, peg laterals especially, may cause cosmetic concern. Such teeth may be restored to resemble normal sized teeth, typically with composite build ups or crowns. Orthodontics may be required in severe cases to close gaps between the teeth.

The best method of maintaining the health of teeth is to practice exemplary oral hygiene. More tooth loss is likely to occur if intervention takes place. However, factors such as present complaint, patient age, severity of the problem, can affect the treatment plan or options.

Dental implant is one of the treatment options that can be considered when growth is fully attained. For patients who experience maxillo-mandibular alveolar atrophy due to early loss of teeth, alveolar ridge augmentation procedure is recommended prior to the implant placement. Both onlay bone grafting and sinus lift techniques can be carried out together to accomplish implant placement.

Treatment is only required if the occlusion or bite of the person is compromised and causing other dental problems. Multiple long-term clinical problems can arise such as occlusal interferences, aesthetic disturbances, loss of pulp vitality, irritation of tongue during mastication and speech, caries and displacement of the affected tooth. Most people with talon cusp will live their normal lives unless the case is severe and causes a cascade of other dental issues that lead to additional health problems. This dental anomaly would not be considered fatal. Generally talon cusps on lower teeth require no treatment, but talon cusps on upper teeth may interfere with the bite mechanics and may need to be removed or reduced.

Small talon cusps that produce no symptoms or complication for a person can remain untreated. However large talon cusps should not.

Some common treatments include:

- Fissure sealing

- Composite resin restoration

- Reduction of cusp

- Pulpotomy

- Root canal (endodontic treatment)

- Extraction

The condition is usually benign, but it can cause mild irritation to soft tissues around the teeth and the tongue, and if large enough, may pose an aesthetic problem. Talon cusps that are too large are filed down with a motorized file, and then endodontic therapy is administered.

In order to prevent any future dental complications, when talon cusp is present due to an early diagnosis it would be best to see a dentist regularly every six months for routine dental checkups, remain under observation, brush and floss properly and undergo regular topical applications of fluoride gel to prevent caries and to promote enamel strength.

Although these teeth are usually asymptomatic and pose no threat to the individual, they are often extracted for aesthetic reasons. This is done particularly if the mesiodens is positioned in the maxillary central incisor region. The traditional method of removal is done by using bone chisels, although a more advanced technique has been found to be more beneficial, especially if surgery is required . Through the use of Piezoelectricity, Piezoelectric ultrasonic bone surgery may be more time consuming than the traditional method but it seems to reduce the post-operative bleeding and associated complications quite significantly.

The chemical imbalance is usually diagnosed when dental abnormalities are found. These abnormalities include premature deciduous teeth and abnormal growth of permanent teeth due to displacement by cysts and lesions. The only definite way to correctly diagnose the condition is by sequence analysis of the SH3BP2 gene. The gene has been found to have missense mutation in exon 9. Initial study of the patient is usually conducted using x-ray and CT scans. Neurofibromatosis may resemble Cherubism and may accompany the condition. Genetic testing is the final diagnosis tool.

Cast restorations may include full coverage crowns for MIH-affected permanent teeth. Generally cast restorations requiring tooth preparation are not recommended in young children due to large pulp size, short crown height and potential difficulties in obtaining a good impression for subgingival crown margins.

Teeth affected by macrodontia are either contoured, aligned or extracted. Contouring involves shaving the tooth down to change shape and size. However, the result is minimal change as this could be dangerous for the dentin and dental pulp. Aligning involves the use of braces to straighten, align, and make space for larger teeth to grow. When extracted, they are replaced with an implant or bridge. This is done in cases in which the patient suffers from pain that cannot be treated by other methods.

Treatment and prognosis are usually based upon keeping these teeth and preserving the alveolus. For erupted teeth, endodontics is an option if the tooth is devitalized and restorable. For unerupted teeth, function can be restored with a removable partial denture until all major growth has been completed and a final restoration can be placed.