Autosomal recessive nonsyndromic deafness 18b is a genetic condition that causes hearing loss without other medical features. Hearing changes are usually present from birth or early childhood and tend to be stable, but severity can vary. People with Autosomal recessive nonsyndromic deafness 18b may notice difficulty hearing soft sounds and speech, especially in noise. It is lifelong, does not affect life expectancy, and is managed with hearing aids, cochlear implants, and communication support. Early hearing screening, family counseling, and speech and language therapy can help daily communication and development.
Overview
Autosomal recessive nonsyndromic deafness 18bSymptoms
Autosomal recessive nonsyndromic deafness 18b causes hearing loss without other health problems. Features usually appear at birth or early infancy as severe sensorineural loss—missing startle to loud sounds, delayed babbling, or not turning to voices. Hearing may be stable or worsen.
Outlook and prognosis
Most people with Autosomal recessive nonsyndromic deafness 18b have stable, lifelong hearing loss that mainly affects everyday communication. Early testing, hearing technology, and language support help children develop speech and social skills. With consistent care, education, and community resources, long-term outlook is strong.
Causes and risk factors
Autosomal recessive nonsyndromic deafness 18b usually results from inheriting two nonworking copies of a hearing-related gene. Risk rises with parental carrier status, family history, or consanguinity. Noise exposure, ototoxic medicines, and ear infections may worsen hearing but aren’t primary causes.
Genetic influences
Genetics is central in Autosomal recessive nonsyndromic deafness 18b, which results from inherited changes in a specific hearing-related gene. Both parents typically carry one silent variant; a child inheriting two variants is affected. Variations can influence severity, age at detection, and test results.
Diagnosis
Diagnosis starts with newborn hearing screening or audiology showing isolated sensorineural loss and a normal exam. Genetic diagnosis of Autosomal recessive nonsyndromic deafness 18b uses multigene panels or exome sequencing; imaging may evaluate inner-ear structure and exclude other causes.
Treatment and drugs
Treatment for Autosomal recessive nonsyndromic deafness 18b focuses on improving hearing and communication. Options often include hearing aids or cochlear implants, plus speech and language therapy, family communication support, and school or workplace accommodations. Genetic counseling can guide testing and future planning.
Symptoms
Many families first notice that a baby doesn’t startle to loud sounds or turn toward a familiar voice. People sometimes look for early features of Autosomal recessive nonsyndromic deafness 18b, like missing softer sounds even in a quiet room. This genetic, non-syndromic hearing loss usually affects both ears and is present from birth or early childhood. Features vary from person to person and can change over time.
Many babies show little or no startle to loud sounds. They may not turn toward a parent’s voice by around 4 to 6 months.
Hearing loss typically involves both ears. This can make it harder to tell where a sound came from and to follow group conversations.
In Autosomal recessive nonsyndromic deafness 18b, hearing can range from moderate to profound. Some can detect loud noises but miss speech, especially soft or high-pitched voices.
Without early hearing support, first words may come late and speech can sound unclear. Hearing devices and speech therapy often help close gaps.
How people usually first notice Autosomal recessive nonsyndromic deafness 18b?
Parents and clinicians often first notice autosomal recessive nonsyndromic deafness 18b in infancy when a baby does not startle to loud sounds or doesn’t turn toward a parent’s voice, and this is often confirmed by a newborn hearing screen that the baby does not pass. As months go by, many families observe delayed babbling and fewer vocal responses, prompting a repeat hearing test that shows permanent hearing loss without other health or physical differences. These first signs of autosomal recessive nonsyndromic deafness 18b are usually isolated hearing concerns rather than part of a broader syndrome, which is why early hearing screening and follow-up are key.
Types of Autosomal recessive nonsyndromic deafness 18b
Autosomal recessive nonsyndromic deafness 18b is a genetic/congenital condition with recognized clinical variants tied to changes in the same gene, which can lead to different ages of onset and degrees of hearing loss. People may notice different sets of symptoms depending on their situation. In practice, clinicians talk about types of Autosomal recessive nonsyndromic deafness 18b based on when hearing loss starts and whether it is stable or progresses over time. Not everyone will experience every type.
Congenital severe-profound
Hearing loss is present from birth and is typically severe to profound. Many children need early amplification or cochlear implants to support speech and language.
Prelingual moderate-severe
Hearing loss appears in infancy and is moderate to severe. Early symptoms of Autosomal recessive nonsyndromic deafness 18b may include not startling to loud sounds or delayed babbling.
Progressive childhood-onset
Hearing begins near typical at birth but worsens during early childhood. Families may notice turning up the TV or missing classroom instructions as hearing thresholds decline.
Stable mild-moderate
Hearing loss is mild to moderate and remains fairly steady over time. People often do well with hearing aids and communication strategies without rapid change.
What genetics can tell you about symptoms
Some people with autosomal recessive nonsyndromic deafness 18b have mutations that disrupt inner‑ear hair cell function, leading to congenital, usually severe-to-profound, sensorineural hearing loss in both ears. Different variants can change loudness sensitivity and pitch clarity, but generally spare balance and other body systems.
Causes and risk factors
Autosomal recessive nonsyndromic deafness 18b comes from a gene change that is passed down from both parents. Risk factors for autosomal recessive nonsyndromic deafness 18b include having two carrier parents and having an affected brother or sister. Risk can also be higher when parents are related by blood or in communities with shared ancestry. Some risks are modifiable (things you can change), others are non-modifiable (things you can’t). Loud noise and certain medicines that are toxic to the inner ear do not cause the condition, but they may worsen hearing over time.
Environmental and biological risk factors
People often ask what environmental or biological risk factors for Autosomal recessive nonsyndromic deafness 18b might exist. Doctors often group risks into internal (biological) and external (environmental). For this condition, risk largely comes from body-based factors present before birth, and outside exposures are not known to trigger it. Here’s what current evidence shows.
Current studies do not show a higher chance of Autosomal recessive nonsyndromic deafness 18b with older maternal or paternal age. Most babies with the condition are born to parents across a wide range of ages.
Common pregnancy conditions like diabetes or high blood pressure have not been linked to causing this condition. Keeping these conditions well managed still supports overall fetal growth and ear development.
Infections during pregnancy (such as rubella or cytomegalovirus) can cause congenital hearing loss, but they are not known to cause Autosomal recessive nonsyndromic deafness 18b. Prenatal care includes screening and prevention where possible.
Prematurity, low birth weight, or brief oxygen problems around delivery may affect hearing in other ways, yet they do not create this specific condition. Babies with these complications are often offered hearing checks.
Genetic risk factors
Risk for Autosomal recessive nonsyndromic deafness 18b comes from changes in a single ear-related gene called OTOG. The condition develops when a child inherits two nonworking copies—one from each parent. Carriers usually hear normally but can pass the change on. Some risk factors are inherited through our genes.
OTOG gene: Variants in the OTOG gene are the main known cause. People develop Autosomal recessive nonsyndromic deafness 18b when they inherit two harmful changes. When early symptoms of Autosomal recessive nonsyndromic deafness 18b appear, testing this gene can help confirm the cause.
Recessive pattern: A child is affected only if both copies of the gene are changed. Each parent is usually an unaffected carrier. This pattern can make the condition seem to skip generations.
Carrier parents: Two carriers have a 25% chance in each pregnancy to have a child with Autosomal recessive nonsyndromic deafness 18b. There is a 50% chance the child will be a carrier and a 25% chance of inheriting neither change. These percentages repeat with every pregnancy.
Sibling recurrence: Brothers and sisters of an affected child have higher chances of also being affected if they inherit the same two changes. Testing can show which siblings carry one or two changes. Early identification can guide hearing support.
Parental relatedness: When parents are related by blood, they are more likely to share the same OTOG variant. This increases the chance of a child inheriting two copies and developing Autosomal recessive nonsyndromic deafness 18b. This is sometimes called consanguinity.
Founder variants: In some communities, a specific OTOG change is more common because of a shared ancestor. This can cluster cases within certain families or regions. People with the same risk factor can have very different experiences.
Lifestyle risk factors
Autosomal recessive nonsyndromic deafness 18b is not caused by lifestyle choices, but daily habits can influence communication outcomes, residual hearing, and complications like ear infections or device problems. Understanding how lifestyle affects Autosomal recessive nonsyndromic deafness 18b can help protect any remaining hearing and optimize assistive technologies. The points below focus on lifestyle risk factors for Autosomal recessive nonsyndromic deafness 18b and practical ways they relate to hearing and communication.
Regular exposure to loud concerts, power tools, or noisy workplaces can further damage residual cochlear function. Using hearing protection reduces the risk of additional hearing loss and tinnitus.
Listening at high volumes, especially with in‑ear earbuds, can accelerate loss of any remaining hearing. Following safe-listening limits and using noise-cancelling can keep volumes lower.
Tobacco and nicotine can reduce blood flow to the inner ear and raise ear infection risk, worsening hearing difficulties. Quitting lowers otitis media and sinus inflammation that interfere with hearing devices.
Heavy drinking impairs balance and spatial hearing cues, making navigation and lip-reading harder. It can also disrupt consistent device use and communication routines.
Risk prevention
Autosomal recessive nonsyndromic deafness 18b is an inherited hearing loss, so you can’t fully prevent the condition itself after conception. Prevention focuses on planning before pregnancy and reducing complications or added hearing damage across life. Early detection and quick support help protect language, learning, and social development. Knowing your risks can guide which preventive steps matter most.
A genetics professional can explain inheritance, carrier chances, and options for planning. They can also discuss testing for partners and at-risk relatives.
If hearing loss runs in your family, ask about carrier screening before pregnancy. Finding out carrier status early opens more choices.
Couples who carry the same variant may consider IVF with embryo testing or prenatal testing. These medical steps aim to reduce the chance of an affected child in future pregnancies.
Make sure your baby completes hospital and follow-up hearing screens on time. Quick referral to audiology speeds diagnosis and early support.
Transmission
Autosomal recessive nonsyndromic deafness 18b is not contagious—you can’t catch it or pass it through everyday contact. It’s passed down in an autosomal recessive way: when two carriers, who usually have normal hearing, have a child, there’s a 25% (1 in 4) chance the child will have Autosomal recessive nonsyndromic deafness 18b, a 50% (1 in 2) chance the child will be a carrier, and a 25% chance the child will inherit neither change. This chance is the same in every pregnancy and for any sex, and it can be higher in families where parents are related. Occasionally, one changed copy may be inherited and the other can occur as a new change in the child, but this is uncommon. A genetics professional can explain how Autosomal recessive nonsyndromic deafness 18b is inherited in your family.
When to test your genes
Autosomal recessive nonsyndromic deafness 18b is a genetic condition, so gene testing is most useful when there’s early hearing loss in childhood, a family history of similar deafness, or parents planning a pregnancy who may be carriers. Testing can confirm diagnosis, guide hearing support, and inform reproductive choices. Discuss newborn screening follow-up promptly.
Diagnosis
You might notice small changes in daily routines, like a baby not startling at loud sounds or a child turning up the TV very high. For many families, the first clues come from newborn hearing checks or early childhood hearing tests. The genetic diagnosis of Autosomal recessive nonsyndromic deafness 18b often involves combining hearing tests with genetic testing to confirm the cause. Genetic testing may be offered to clarify risk or guide treatment.
Hospitals check hearing within the first days of life to spot early signs of hearing loss. If a baby does not pass, a repeat screen and full hearing evaluation are arranged.
Doctors review pregnancy, birth, infections, medicines, and noise exposures, and examine the ears. This helps separate inherited hearing loss from acquired causes.
Age-appropriate hearing tests measure how softly sounds can be heard across pitches. Results help map the degree and pattern of hearing loss.
A tiny ear probe measures echoes from the inner ear hair cells. Absent or reduced emissions point toward sensorineural hearing loss.
Stages of Autosomal recessive nonsyndromic deafness 18b
Autosomal recessive nonsyndromic deafness 18b does not have defined progression stages. Hearing changes can appear at birth or later in childhood and may stay steady or slowly worsen, so clinicians describe the degree of hearing loss rather than stages; early symptoms of autosomal recessive nonsyndromic deafness 18b can include not responding to everyday sounds or delayed speech. Different tests may be suggested to help confirm what’s going on, starting with hearing evaluations and sometimes genetic testing to look for the underlying cause. Ongoing care typically involves regular hearing checks to track any changes over time.
Outlook and prognosis
Living with Autosomal recessive nonsyndromic deafness 18b often means navigating hearing differences from birth or early childhood, which can shape communication at home, school, and work. Many people ask, “What does this mean for my future?”, and the short answer is that life expectancy is typically normal, and overall health is otherwise unaffected. With early identification, access to hearing technology (like hearing aids or cochlear implants when appropriate), and strong language support—spoken, signed, or both—children can develop communication skills, thrive in school, and build independence. Doctors call this the prognosis—a medical word for likely outcomes.
The long-term picture is usually stable, since this condition affects hearing without progressive damage to other organs. Some people experience stable moderate to severe hearing loss, while others notice profound loss from the start, so supports are tailored to the individual. Early care can make a real difference, particularly for language development and social connection. When thinking about the future, it helps to plan for communication needs during transitions, like starting school or a new job, and to revisit technology options as devices and candidacy criteria evolve.
Serious medical complications and mortality linked directly to Autosomal recessive nonsyndromic deafness 18b are not expected. That said, early symptoms of Autosomal recessive nonsyndromic deafness 18b—such as not startling to loud sounds or delayed babbling—should prompt timely hearing evaluation to prevent delays in language access. Support from friends and family can boost confidence and ease daily communication, whether that’s learning some sign language together or fine-tuning home listening environments. Talk with your doctor about what your personal outlook might look like, including which hearing technologies, therapies, and school or workplace accommodations could best fit your goals over time.
Long-term effects
Autosomal recessive nonsyndromic deafness 18b usually leads to lifelong hearing differences without other body systems being involved. Early symptoms of autosomal recessive nonsyndromic deafness 18b are often noticed in infancy, and the long-term course tends to reflect the same hearing profile over time. Long-term effects vary widely, but many people have stable hearing from childhood into adulthood. Vision, balance, growth, and overall health are typically the same as in the general population.
Hearing loss is typically present from birth and often remains stable over the years. In some families, changes can be gradual rather than sudden. Most do not develop other ear-related diseases.
Without early access to communication, delays in first words or signing can occur. Over time, language usually develops well in the chosen communication approach. Reading and learning skills can be strong with consistent access.
Many complete school and higher education at rates similar to peers when information is accessible. Some may face gaps when classroom sound or captions are limited. Cognitive abilities are not affected by the condition itself.
Communication barriers can increase the risk of loneliness or fatigue from concentrating on listening. Many people develop strong coping and advocacy skills over time. Mood or anxiety concerns may appear during transitions like school or job changes.
How is it to live with Autosomal recessive nonsyndromic deafness 18b?
Life with autosomal recessive nonsyndromic deafness 18b often centers on communication, not illness. Many grow up healthy in every other way and build rich language through sign languages, hearing aids or cochlear implants if appropriate, and visual-first strategies like captioning and apps; early access to language—signed, spoken, or both—matters most for learning and confidence. Day to day, school and work can go smoothly with consistent accommodations such as interpreters, captioned meetings, visual alerts, and clear communication norms; social life thrives when friends and family learn basic signs, face the person when speaking, and reduce background noise. For loved ones, the biggest impact is adapting how they communicate, and many find that these changes—more eye contact, turn-taking, and clarity—strengthen connection for everyone.
Treatment and drugs
Autosomal recessive nonsyndromic deafness 18b is managed by improving access to sound, supporting communication, and addressing day-to-day hearing needs rather than changing the underlying genes. Most people are first fitted with hearing aids to amplify sound; if hearing loss is severe to profound or hearing aids don’t help enough, cochlear implants can provide access to speech and environmental sounds. Early symptoms of Autosomal recessive nonsyndromic deafness 18b can be addressed with timely therapy—speech and language therapy, auditory training, and family sign language learning—to support communication and development at home, in school, and at work. Treatment plans often combine several approaches, and regular follow-ups help fine-tune device settings and provide hearing protection, vaccinations that reduce ear infection risk, and counseling for you and your family. Research is ongoing into gene-directed therapies, but for now the focus is on personalized hearing care tailored to your level of hearing and your communication goals.
Non-drug treatment
Many living with Autosomal recessive nonsyndromic deafness 18b benefit most from tools and therapies that improve access to sound and communication from an early age. Non-drug treatments often lay the foundation for daily communication, learning, and social connection. Some families notice early symptoms of Autosomal recessive nonsyndromic deafness 18b in infancy, like not startling to loud sounds or delayed babbling, and early support can make a real difference. Plans are tailored to age, hearing level, language goals, and family preferences.
Coordinated services in the first months and years help language and learning stay on track. This may include family coaching, hearing technology fitting, and regular progress checks.
Small devices amplify sounds to make speech and environmental noises clearer. They are adjusted by specialists to match the child’s or adult’s specific hearing pattern.
For severe or profound loss, surgically placed devices can bypass damaged inner-ear cells to stimulate the hearing nerve. Intensive therapy after activation helps the brain learn to interpret sound.
Structured listening exercises help the brain make sense of amplified or implanted sound. Sessions often include practicing speech sounds, words, and everyday listening tasks.
Your genes may have an impact on how drugs work for you
Genes can change how well certain hearing-supportive treatments or devices work, and how people respond to medications used around procedures, like antibiotics or anesthesia. In DFNB18B, genetic results help clinicians avoid ototoxic drugs and tailor care to protect remaining hearing.
Pharmacological treatments
Day to day, medicines have a limited role for people with autosomal recessive nonsyndromic deafness 18b, because no drug has been proven to restore this type of genetic hearing loss. Care usually centers on hearing technology and communication support, while medicines are used for related ear issues or to protect remaining hearing. Beyond standard options, clinical research is bringing new drug therapies closer to the ear, but these remain experimental. Medicines do not change early symptoms of autosomal recessive nonsyndromic deafness 18b, yet they can help prevent problems that might further reduce hearing.
There are currently no medicines that reverse or restore hearing in autosomal recessive nonsyndromic deafness 18b. Treatment focuses on hearing devices and therapy rather than pills or injections. Doctors may still use medicines for other ear conditions when needed.
If a bacterial middle-ear infection occurs, antibiotics such as amoxicillin or amoxicillin–clavulanate are commonly used. Prompt treatment can help protect any remaining hearing. The specific choice depends on age, allergies, and local guidelines.
Certain drugs can harm hearing, including aminoglycoside antibiotics like gentamicin and tobramycin, chemotherapy such as cisplatin, and high-dose loop diuretics like furosemide. Your care team may choose alternatives or monitor levels closely if these are medically necessary. This is especially important for people with autosomal recessive nonsyndromic deafness 18b.
If wax buildup worsens hearing, softening drops such as carbamide peroxide may help. Avoid cotton swabs or harsh solvents that can irritate the ear canal. Ask a clinician about safe removal if hearing does not improve.
Genetic influences
In Autosomal recessive nonsyndromic deafness 18b, hearing changes happen when a child inherits two copies of the same gene change—one from each parent. Parents usually have typical hearing because they carry only one copy, and this subtype is “nonsyndromic,” meaning the gene mainly affects hearing rather than other parts of the body. A “carrier” means you hold the gene change but may not show symptoms. When both parents are carriers, each pregnancy has about a 25% chance of a child having Autosomal recessive nonsyndromic deafness 18b, a 50% chance the child will be a carrier, and a 25% chance of inheriting neither change. Genetic testing for Autosomal recessive nonsyndromic deafness 18b can confirm the gene involved, help estimate the chance for future children to be affected, and guide family planning. Genetic counseling can also explain how results apply to relatives, since the exact gene change and its impact on hearing can vary between families.
Is Autosomal recessive nonsyndromic deafness 18b genetic?
Genetics is central in Autosomal recessive nonsyndromic deafness 18b, which results from inherited changes in a specific hearing-related gene. Both parents typically carry one silent variant; a child inheriting two variants is affected. Variations can influence severity, age at detection, and test results.
Pharmacogenetics—how genetics influence drug effects
For people with Autosomal recessive nonsyndromic deafness 18b, care centers on hearing support—like hearing aids or cochlear implants—rather than medications to “treat” the hearing loss itself. The gene changes that cause this condition don’t typically alter how your body processes most drugs, but genetics still matters for medication safety. A well-known example is a mitochondrial DNA change that can trigger sudden, permanent hearing loss after even a single dose of aminoglycoside antibiotics; if this risk is known, doctors try to use other antibiotics when possible. Genetic testing can sometimes identify how your body may react to these antibiotics, helping your care team steer clear of avoidable, drug-induced hearing loss. Other ototoxic medicines—such as cisplatin chemotherapy or high-dose loop diuretics—may be necessary at times; clinicians often adjust dosing, add hearing checks, and counsel carefully, while pharmacogenetic guidance for these drugs is still evolving. If you’re scheduled for surgery or hospital treatment, share your hearing history and any past medication reactions so teams can plan safer alternatives when possible. Research on gene-directed therapies for DFNB18B is ongoing, but for now the most practical genetic influence on treatment is preventing additional hearing damage and tailoring supportive care.
Interactions with other conditions
Day to day, other health issues can add to the hearing challenges you’re already managing. In Autosomal recessive nonsyndromic deafness 18b, the ears are the primary concern, but problems like chronic ear infections, fluid behind the eardrum, or heavy earwax can temporarily worsen hearing or make testing less clear. Doctors call it a “comorbidity” when two conditions occur together. Early symptoms of Autosomal recessive nonsyndromic deafness 18b—such as not responding to softer sounds—can also be harder to spot if frequent colds or ear infections are in the mix, since those can cause short-term hearing changes on their own. Over a lifetime, unrelated factors like loud noise exposure, certain medicines that affect the inner ear, or age-related hearing loss can further reduce hearing, so protection and regular checks matter. Mood and communication strain may also overlap, and some living with genetic hearing loss report anxiety or low mood, which deserves the same attention and care as any physical health issue.
How genes can cause diseases
Humans have more than 20,000 genes, each carrying out one or a few specific functions in the body. One gene instructs the body to digest lactose from milk, another tells the body how to build strong bones and another prevents the body's cells from beginning to multiply uncontrollably and developing into cancer. As all of these genes combined are the building instructions for our body, a defect in one of these genes can have severe health consequences.
Through decades of genetic research, we know the genetic code of any healthy/functional human gene. We have also identified that in certain positions on a gene, some individuals may have a different genetic letter from the one you have. We call these hotspots "Genetic Variations" or "Variants" in short. In many cases, studies have been able to show that having the genetic letter "G" in the position makes you healthy, but having the letter "A" in the same position disrupts the gene function and causes a disease. Genopedia allows you to view these variants in genes and summarizes all that we know from scientific research, which genetic letters (Genotype) have good or bad consequences on your health or on your traits.
History
Throughout history, people have described children born with normal growth and learning who did not respond to sound, even when families had no other health concerns. In many households, grandparents recalled an uncle who signed naturally, or siblings who both used lip‑reading while everyone else heard typically; families and communities once noticed patterns without knowing why. These memories hinted that Autosomal recessive nonsyndromic deafness 18b tends to appear when each parent silently carries a single copy of a gene change.
First described in the medical literature as a form of inherited hearing loss without other medical features, this condition was initially grouped with many similar patterns of “nonsyndromic” deafness. Early reports focused on age at onset, how severe the hearing loss was, and whether it affected all tones or mostly high or low pitches. Doctors documented that children often had stable, congenital hearing loss, while some relatives had normal hearing, suggesting a hidden inheritance pattern within the same family.
As medical science evolved, researchers began sorting hereditary hearing loss into categories by how it was passed down and by subtle differences in hearing tests. The label “autosomal recessive” signaled that parents typically had normal hearing but carried one altered gene each, and that a child who inherited both altered copies could have hearing loss. Over time, descriptions became clearer that Autosomal recessive nonsyndromic deafness 18b affects hearing without consistent effects on balance, vision, heart, or other organs.
Advances in genetics helped separate look‑alike conditions that earlier doctors could not tell apart. Gene mapping studies linked certain family patterns to specific regions of DNA, and later pinpointed exact genes tied to this subtype. This shift from symptom‑based labels to gene‑based names allowed scientists to confirm diagnosis more precisely, offer targeted carrier testing to relatives, and design research on how the involved protein works in the inner ear’s delicate sound‑sensing cells.
In recent decades, knowledge has built on a long tradition of observation. Large family studies across different countries showed that Autosomal recessive nonsyndromic deafness 18b occurs in diverse populations, sometimes more often where parents are related or in smaller communities where the same rare variant is shared. Historical differences highlight why older records may have grouped several conditions together; what looked the same at the bedside now separates into distinct genetic types with their own testing and counseling implications.
Looking back helps explain today’s approach: detailed hearing tests establish the pattern, family history points to recessive inheritance, and modern genetic testing can confirm the specific subtype. This arc—from family stories to precise molecular diagnosis—continues to shape early identification, timely communication support, and informed choices for people living with Autosomal recessive nonsyndromic deafness 18b and their families.