Causes of Hearing Loss

Parents who have just received word that their child has a hearing loss can display a wide range of reactions. A common feeling that most parents experience is wondering what caused their child's hearing loss. This section will help parents begin to answer that question. We will walk you through the process that medical professionals use to discover the cause of a hearing loss and determine the appropriate treatment. The emphasis will be on some of the more common causes of hearing loss. Trying to come up with an answer to the question of what caused a hearing loss is somewhat like putting together a jigsaw puzzle. Unfortunately, we do not always have all the pieces to that puzzle and there is not a final picture to guide us. Each physical finding, medical test result, and piece of family medical history provides a piece of the puzzle. Sometimes, one of those pieces can "fill in" a big enough section of the puzzle that the medical professionals feel confident that they know the cause of the hearing loss. It is also possible that after all the testing, after all the questioning, and after all the examinations, the answer remains unclear. You may be told that the cause of the hearing loss is still unknown.

Causes of Hearing Loss

What is the First Step?
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If we know what caused the hearing loss, we can research other cases with the same cause to help parents know what to expect and what to do. The best place to find the answer to that question is with a team of professionals who specialize in the diagnosis of hearing loss in children. These medical professionals routinely see many children with a wide variety of causes for their hearing losses. Your primary care provider should be able to provide you the names of audiologists, Ear Nose and Throat (ENT) doctors and genetic specialists in your area. What can I expect to happen during my first visit with a medical specialist? There are two goals for a first visit. The first goal is to determine the cause of the hearing loss, if possible, or to narrow down the list of possible causes. The second goal is to determine if medical treatment should begin and the nature of that treatment. The way the specialist begins the process of finding the cause of the hearing loss is by asking questions. The doctor or genetic counselor will: 1. Review whatever hearing and medical information is already available, including pre-natal and birth history. 2. Ask additional questions about your family's medical history. Professionals will ask you questions about you, your partner, and all your relatives. From this, they will create your family history tree. They also may ask for medical information about some of your family members. [If you want to know more about how genetic professionals construct a family history, click here to begin the PowerPoint animation] 3. Request that additional testing be done. Depending on the areas of concern, some of those tests may include genetic tests that require blood or urine samples, CT or MRI scans, vestibular (balance) tests or ophthalmology (eye) examinations. You will be asked to give your consent before any additional testing is done.

The information from these three areas will provide additional pieces of the puzzle to help find the cause of the hearing loss. If the information cannot identify the cause, it should provide enough clues to determine both what the cause of the hearing loss IS NOT and what the cause of the hearing loss MIGHT BE. From a geneticist's point of view, the cause of hearing loss will fall into one of three broad categories: (1) Unknown, (2) Non-genetic, or (3) Genetic. This can be an important first step, since it focuses the attention of the investigation into areas that might reveal the cause of the hearing loss AND it saves time, energy, and money by preventing tests that are not likely to reveal the true answer. In Summary: You should expect to be asked questions about your child's and family's medical history. You can expect to have additional medical testing recommended. If additional testing is done, you should expect to have another visit scheduled to discuss the results of that testing. Depending on how much information is obtained during the first visit, a cause for your child's hearing loss may be identified. If medical treatment is recommended, this may also begin.

Causes of Hearing Loss

Non-Genetic Hearing Loss
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In about 25% of cases of hearing loss there is a non-genetic cause that can be identified. Nongenetic hearing loss is most often caused by illness or trauma before birth or during the birth process. Older infants and young children can also develop non-genetic hearing loss due to illness or trauma. Some viral infections are known to be associated with hearing loss. These infections carry the highest risk of causing hearing loss if the mother has the illness during pregnancy or passes the infection to her baby during the birth process. The primary infections are Toxoplasmosis (Catscratch disease), Syphilis, Rubella (German Measles), Cytomegalovirus (also known by the initials CMV) and Herpes. The amount of hearing loss that can result varies widely and some babies show no hearing loss at all, even if they have one of these infections. These infections can affect other systems in the body as well and medical professionals will need extensive birth history and test information to identify these infections as a cause for hearing loss. Low birth weight has also been identified as a risk factor for hearing loss. Newborn specialists identify 1500 grams (approx. 3.3 lbs.) as a cut-off point, with children weighing less than 1500 grams having an increased likelihood of hearing loss. Hyperbilirubinemia (jaundice) that is severe enough to require a blood transfusion can also result in hearing loss. This is related to the potential damage that high levels of bilirubin can cause to the nerves of hearing. Sometimes medications that are known to be ototoxic (damaging to hearing) are prescribed to babies, usually to treat serious infections or birth complications. The most common otoxic medications used at this time include a family of antibiotics called aminoglycosides with names

such as gentamycin, tobramycin, kanamycin. and streptomycin. Hearing loss resulting from the use of these antibiotics may also have a genetic component. They present more of a risk to hearing when they are used multiple times or in combination with other medications, such as diuretics. Although cancer in infants and young children is rare, there are some chemotherapy drugs that are used which are also ototoxic, especially when used to treat tumors that are in the skull. All babies are evaluated at birth on a 10-point scale, called an APGAR score, for A: Activity (muscle tone); P: Pulse; G: Grimace (reflex irritability); A: Appearance (skin color); and R: Respiration. Newborns are given APGAR scores at 1 minute and 5 minutes after birth. The higher the score, the healthier the baby is. When babies have scores of 0-4 at one minute or 0-6 at five minutes, their risk for having hearing loss increases. Also, prolonged mechanical ventilation for a duration of five days or longer due to persistent pulmonary hypertension increases the risk for hearing loss. These conditions of breathing problems and other distress at birth do not mean that a baby will always have a hearing loss, but do indicate the need to monitor hearing closely. One illness that carries a high risk of causing hearing loss and/or balance problems is bacterial or viral meningitis. Because of improvements in immunizations, the cases of bacterial meningitis have declined sharply in recent years, but immunizations are not available for the viral type of this infection. Because meningitis is an infection of the lining of the brain and spinal cord, the sense organs of hearing and balance are especially sensitive to this infection. It is clear that non-genetic hearing loss can result from a wide variety of illnesses and problems at birth. Medical professionals depend on a careful review of birth information to help them identify non-genetic hearing loss.

Causes of Hearing Loss

How do genetic professionals view hearing loss?
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Most genetic professionals working with hearing loss are in general agreement that the cause of hearing loss in about one-quarter of cases is Unknown, onequarter Non-genetic, and about one-half Genetic. For the purposes of this section, we will only focus on the genetic causes. Within the genetic half, about 70% of cases are generally thought to be recessive in nature, about 15% dominant and the remainder include all other inheritance types. The next section will explain these genetic terms in more detail. Genetic 50% Non-genetic Unknown 25% 25%

Understanding genetics and inheritance Many parents are unfamiliar with how hearing loss could be caused by genetic factors. As you work through the process of identifying the cause of your child's hearing loss, it will be helpful to have a basic understanding of genetics and the major categories of inheritance. If you are already familiar with genetic concepts, this list briefly summarizes the important points of genetic inheritance: Genes code for messages. Genes are found on chromosomes. Chromosomes come in pairs called homologs.

The messages on each of the chromosomes in a pair may be different; those differences determine dominant or recessive expression of the gene message. The major forms of inheritance are Dominant, Recessive and Sex-linked. There are other types of inheritance.

For a more detailed tutorial, please click to the following section on basic genetics.

Causes of Hearing Loss

Basic Genetics
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What Are the Most Common Forms of Genetic Hearing Loss? Of the 50% of the genetic forms of hearing loss, an estimated 70% are due to recessive causes, about 15% have a dominant cause; and the remaining 15% include all the other forms of inheritance. GENETIC RECESSIVE 70% NON-GENETIC AND UNKNOWN

DOMINANT 15% OTHER GENETIC 15%

Genetic scientists subdivide genetic hearing loss into two general categories: "Non-Syndromic" (meaning hearing loss and nothing else) and "Syndromic" (meaning hearing loss with other clinical findings). By far, the more common is Non-syndromic hearing loss which includes 2/3 of all genetic hearing losses. SYNDROMIC NONSYNDROMIC

RECESSIVE RECESSIVE DOMINANT DOMINANT OTHER OTHER GENETIC GENETIC

NON-GENETIC AND UNKNOWN

What is the Most Common form of genetic hearing loss? One gene, known as Connexin 26 (abbreviated CX26), is estimated to be responsible for half of all the recessive cases of hearing loss. There are over 400 known genetic causes involving hearing loss. CX26 alone is responsible for about 1/3 of all the cases of genetic hearing loss! SYNDROMIC NONSYNDROMIC CONNEXIN-26 RECESSIVE DOMINANT RECESSIVE DOMINANT

NON-GENETIC AND UNKNOWN

OTHER GENETIC

OTHER GENETIC

What are the Next Most Common Forms of Genetic Hearing Loss? Since CX26 accounts for about 1/3 of all cases of genetic hearing loss, that leaves about 1/3 of all cases as non-syndromic (this includes all types of inheritance) with the remaining 1/3 as syndromic. Among the remaining 1/3 of non-syndromic cases of genetic hearing loss, 13 dominant and 8 other recessive genes have been described. What are the Common Dominant Syndromic Hearing Loss Types? The following descriptions are only a brief review. Medical professionals can guide you for further information. About 5% of all hearing loss is dominant syndromic in nature.http://www.familyvillage.wisc.edu/index.htmlx Waardenburg Syndrome - The hearing loss in Waardenburg Syndrome may be present in one or both ears and is a sensorineural type of loss. The striking features of Waardenburg syndrome may include: premature graying hair, white forelock, fused eyebrows (synophrys), two different-colored eyes (heterochromia irides, usually bright blue and brown), widely-spaced eyes (hypertelorism), high nasal bridge, under-developed nose tip (hypoplastic alae nasia) and partial albinism.http://www.boystownhospital.org/Hearing/info/genetics/syndromes/waardenburg.asp http://www.nidcd.nih.gov/health/hearing/waard.asp Branchio-Oto-Renal (BOR) Syndrome - The hearing loss in BOR Syndrome is conductive, sensorineural or mixed. Cysts (or pits) can be found on the neck (branchial cleft) or in front of the outer ear (prearicular). The outer ear (pinna) may be malformed and stapes fixation, inner ear malformations and/or enlarged vestibular aqueducts may be present. A major medical concern with BOR are the associated renal (kidney) problems, which could be life threatening.http://www.boystownhospital.org/Hearing/info/genetics/syndromes/bor.asp Neurofibromatosis Type II (NFII) - The hearing loss in NFII is progressive sensorineural leading to possible deafness. Caf-au-lait (coffee with cream-colored) spots may appear on the skin, with freckling and cataracts. Acoustic tumors may grow on the VIIth cranial nerve, causing hearing loss. Tumors may also grow on other nerves. http://www.nfinc.org/ www.nf.org http://www.nlm.nih.gov/medlineplus/neurofibromatosis.html#nlmnihresources Stickler Syndrome - The hearing loss in Stickler Syndrome is usually conductive, although some losses may be mixed or sensorineural. Progressive hearing loss has also been reported. There are three syndrome types. All are related to altered expression of a collagen/connective tissue gene. Associated features may include: cleft palate, downward-placed tongue (glossoptosis), small jaw (micrognathia), under-developed midface, progressive severe near-sightedness (myopia), cataracts, retinal detachment/degeneration, bone/joint disorders, early adult-onset arthritis, and middle ear bone (ossicular) malformations. http://www.sticklers.org/sip/ Treacher-Collins Syndrome - The hearing loss in Treacher-Collins is conductive. Striking facial features include cleft palate, down-slanting eye-slit openings (palpebral fissures), unusual pupil openings (coloboma), under-developed cheek bones (malar hypoplasia), absent/malformed outer ears, absent (atresia) or narrow (stenosis) ear canals, skin tags in front of the ear (prearicular), teeth alignment problems (malocclusion) and possible balance (vestibular) problems. What are the Common Recessive Syndromic Hearing Loss Types? Recessive Syndromic hearing loss accounts for about 20% of all types of genetic hearing loss.

Usher Syndrome - There are three (3) types of Usher syndrome with different types of hearing loss. Type I has congenital, profound, sensorineural hearing loss. And vestibular dysfunction. Type II has a downward-sloping, sensorineural hearing loss. Type III has a progressive sensorineural hearing loss and variable vestibular dysfunction. All types have progressive vision loss.http://www.nidcd.nih.gov/health/hearing/usher.asp Alport Syndrome - Hearing loss in Alport syndrome may be sensorineural, conductive or mixed and may be progressive. Alport syndrome may also be Xlinked (linked to sex-chromosome inheritance). Other features include kidney problems (nephritis), near-sightedness (myopia) or cataracts, and palate abnormalities. http://www.cc.utah.edu/~cla6202/ASHP.htm Jervell and Lange-Nielson Syndrome - The hearing loss in JL-N syndrome is sensorineural. The other major finding in JL-N is an abnormal heart rhythm (long Q-T), which could lead to fainting spells and possible sudden death. These might be mistaken for seizures. These abnormal heart rhythms are successfully treated with medication (beta blockers). Pendred Syndrome - The hearing loss in Pendred syndrome may be mixed or sensorineural and may be progressive. Pendred Syndrome may have an associated goiter,and variable inner ear malformations (Mondini malformation with or without enlarged vestibular aqueduct).http://www.medicinenet.com/Script/Main/Art.asp?li=MNI&ArticleKey=9460 What are Some Less-Common Syndromic Hearing Loss Types? CHARGE Syndrome - The letters in CHARGE stand for Coloboma, Heart, Atresia of the choanae, Retardation of growth and development, Genital and urinary abnormalities, and Ear abnormalities. CHARGE is thought to be multifactorial. The hearing loss may be conductive, sensorineural or mixed and range from mild to profound. In addition to the features in the name, there may be partial facial paralysis, cleft palate, cleft lip, kidney problems, and feeding problems due to an opening between the windpipe and the feeding tube.http://www.chargesyndrome.org/ X-Linked Congenital Stapes Fixation with Perilymph Gusher - For boys with this syndrome, the hearing loss is mixed and may be progressive. Females who carry this gene may have mild, mixed or sensorineural hearing loss. Boys with this gene have a further risk of increased hearing loss if middle ear surgery is performed to correct the stapes fixation because the surgery may result in massive and sudden loss of inner ear fluid (perilymph). Mitochondrial Conditions Mitochondria are structures in the cell that produce the energy that cells need to survive. Neither the mitochondria nor the cell can exist without the other. Interestingly, mitochondria have a separate set of genes that are not part of the cell's genes. This is because mitochondria originally came from energy-producing bacteria that merged with the cell. Anything that affects bacteria could also affect the function of the cell's mitochondria, which might eventually affect the cell's energy source. Changes in the mitochondrial genes can also result in syndromes involving hearing loss. Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like Episodes (MELAS) - About 30% have sensorineural hearing loss. The findings are highly variable and may include: intermittent vomiting, limb weakness, stroke-like episodes, partial paralysis, partial blindness, seizures, migraine-like headaches, diabetes, short stature, heart problems and kidney problems.

 Maternally Inherited Diabetes and Deafness (MIDD) - The hearing loss is sensorineural. The only other finding is diabetes. Kearns-Sayre Syndrome (KSS) - The hearing loss is sensorineural. Other findings include: unsteady gait (ataxia), short stature, delayed puberty, progressive paralysis of the eye muscles (ophthalmoplegia) and progressive blindness (retinopathy). Myoclonic Epilepsy and Ragged Red Fibers (MERRF) - The hearing loss is sensorineural. Other findings include unsteady gait (ataxia), epilepsy, and possible blindness (optic atrophy). 1555DELG - The hearing loss is sensorineural and may be progressive. Those who have this condition may have sudden hearing loss when exposed to aminoglycoside antibiotics (e.g., neomycin, gentamycin, streptomycin, kanamycin, tobramycin, or amikacin).

Causes of Hearing Loss

Auditory Neuropathy
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Sensorineural hearing loss is the most common type of permanent hearing loss. A less common type of hearing loss is Auditory Neuropathy or Auditory Dysynchrony. With this type of hearing disorder, the inner ear or cochlea seems to receive sounds normally. However, signals leaving the cochlea may be disorganized or the hearing nerve itself may not process sound normally. Auditory Neuropathy was first identified in the 1980s when advanced testing procedures became available to measure the action of the cells in the cochlea. There are some common features that are usually seen with auditory neuropathy, including: Hearing loss of some degree. The degree of hearing loss can vary widely from mild to profound loss. The degree of hearing loss is not predictable. Difficulty understanding speech, especially in noise. Speech understanding difficulties that are worse than can be predicted from other tests of hearing function. Hearing that appears to fluctuate from day-to-day or even from hour-to-hour. Other neuropathies that may affect coordination for activities like writing, running or talking.

To listen to a simulation of Auditory Neuropathy, use the following link and scroll to #2. The link is provided courtesy of Fan-Gang Zeng, Ph.D. University of California Irvine:http://www.ucihs.uci.edu/hesp/Simulations/simulationsmain.htm. What Causes Auditory Neuropathy? There appear to be multiple causes for auditory neuropathy. It can be inherited genetically or caused by trauma or disease. The most common causes of auditory neuropathy that are known at this time are: Lack of oxygen (anoxia) at birth Hyperbilirubinemia requiring blood transfusion, associated with severe jaundice during the

newborn period Infectious Disease such as mumps Immune Disorders Nonsyndromic Recessive Auditory Neuropathy (see Genetics Section for a further explanation of these terms) Neurological disorders such as Charcot-Marie-Tooth syndrome or Friedreichs Ataxia.

See the following sites for more information about these disorders:

http://www.ninds.nih.gov/disorders/charcot_marie_tooth/charcot_marie_tooth.htm; http://www.ninds.nih.gov/disorders/friedreichs_ataxia/friedreichs_ataxia.htm There are large individual differences among children with this hearing disorder. For some, hearing may improve over time. This is most common when the cause of the disorder is hyperbilirubinemia. How Is Auditory Neuropathy Diagnosed? The diagnosis of auditory neuropathy is based on a certain pattern of results from a number of different hearing tests. Auditory Brainstem Response (ABR) and Otoacoustic Emission (OAE) results are used to find this condition. An ABR test uses small bandaid-type electrodes placed behind each ear and the head. Sounds are presented to the ears using miniature earphones. The electrodes pick up responses from the hearing nerve and estimate how the cochlea and hearing nerve are responding to sound. The OAE test uses a miniature earphone to present a series of tones to the ear. A sensitive microphone measures an echo response from the inner ear. This information estimates how the inner ear responds to sound. The most striking finding with auditory neuropathy is that Otoacoustic Emissions (OAEs) are normal. This means that hair cells in the inner ear are working normally. At the same time, ABR responses are absent or very abnormal. This shows poor responses from the hearing nerve. It appears that only a small percentage of people with hearing loss have auditory neuropathy. Depending upon what method is used for newborn hearing screening (OAEs or ABRs), some children with auditory neuropathy may pass an initial hearing screening conducted in the hospital. This may delay the identification of auditory neuropathy. Parents and physicians may not suspect hearing loss right away if an infants response to sound changes from day to day. As children get older, more hearing information becomes available. Ongoing testing may show poorer than expected speech understanding and great difficulty hearing in noisy backgrounds. A wide variety of hearing loss degrees and hearing loss shapes can be seen. Children with auditory neuropathy may also have hearing responses that fluctuate or worsen over time. What Are The Treatment and Management Options for Auditory Neuropathy? The location of the problem that causes auditory neuropathy is not known at this time. It also may be different from person to person. Therefore, medical treatment of auditory neuropathy is not currently available. Management typically involves either the use of hearing aids or cochlear implants. Audiologists vary in their opinions about using hearing aids for a child with auditory neuropathy. Some children benefit from hearing aids. However, many children get limited hearing aid benefit or

no benefit at all. At this time, there is no reliable way to predict who will and will not benefit. Hearing aids must be set carefully to prevent damage to the parts of the ear that receive sounds normally. Some professionals have recommended the use of Frequency Modulated (FM) systems. FM systems can improve listening in noisy backgrounds. Previously, it was thought that children with auditory neuropathy would not be good cochlear implant candidates. Now we know that some children benefit more from a cochlear implant than from hearing aids. Children with some types of genetically inherited auditory neuropathy may benefit more from cochlear implants than children with other causes of the disorder. We will know more as future genetic and hearing science research becomes available. How Does A Child With Auditory Neuropathy Learn To Talk and Understand Others? It is safe to say that no single teaching approach fits all children with auditory neuropathy. It is helpful to work closely with a team of professionals. A team can help parents observe their child and determine what techniques bring about the best responses. Some children benefit from teaching that focuses only on learning to listen and speak. Sometimes, a child with auditory neuropathy has great difficulty understanding what is heard. These children benefit from visual communication approaches (i.e., sign language or Cued Speech). It is difficult to predict how any one child will do. It is important to be flexible. With guidance, families can try an approach and evaluate how well it is working over time. Approaches can be modified to bring about success. Teachers and other professionals can help families to decide whether or not an approach is working. If it works, the child should make steady progress. Communication and understanding should improve over time. Frustration around communication should lessen. This gives families increasing confidence and success in communicating with their child.

Causes of Hearing Loss

Answers to some frequently asked questions
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Why did this happen to my child? Hearing science professionals estimate that 1 in 300 babies are born with some degree of hearing loss. Human genetic professionals estimate that half of those cases of hearing loss are due to the genes that the baby inherited. About 12 babies per 10,000 have a recessive cause of their hearing loss. Congenital hearing loss (hearing loss present at birth) that is due to one of the many recessive genes is about twice as common as Cystic Fibrosis, another recessive genetic condition. To answer the question, "Why did this happen to my child?" there could be at least one of three answers. 1. Unknown (the cause of the hearing loss cannot be discovered) 2. Non-genetic (illness or trauma before, during or after birth) 3. Genetic (the hearing loss will depend on the type of inheritance present) In 70% of cases of genetic hearing loss, the cause is autosomal recessive; therefore, Both parents are "carriers" of a gene that causes recessive hearing loss. There are many different genes in many different locations that may cause genetic hearing loss. At one of these locations, a parent my carry one of the recessive genes that can

cause deafness and one of the dominant genes that is responsible for normal hearing. Because each parent has only one recessive gene that can cause hearing loss, both parents can hear. If each parent passed on one recessive gene, then the child would receive two recessive genes (one from each parent) and that is the cause of the recessive hearing loss. In 15% of cases of genetic hearing loss the cause is autosomal dominant. In this case only one parent may have the dominant gene for hearing loss. That parent will have some degree of hearing loss. If the autosomal dominant gene also has other physical findings (i.e., it is syndromic), the parent who has the dominant gene also may have some of these other physical findings. The parent with the dominant gene may pass that gene for hearing loss on to the child.

For the final and remaining 15% of genetic hearing loss cases, the answer to the question, "Why did this happen to my child?" is specific to the particular type of genetic inheritance. Why is my child being referred for Genetic Testing? Genetic testing may determine whether your child's hearing loss is due to non-genetic causes or to a gene that causes hearing loss. From this information, professionals can calculate the likelihood that other children in your family may also have a hearing loss due to the same cause. A full genetic evaluation will also determine if there are other health conditions that also need to be addressed. What does this mean for my child? In general, why your child has a hearing loss does not affect how your child will be educated. Management of your child's hearing loss will be based upon the specific characteristics of your child and his or her communication needs. The findings from a genetic evaluation may be relevant for your child's medical care What is the likelihood that this will happen again with any other children in my family? If the cause of your child's hearing loss is an autosomal recessive gene, then the likelihood that this will happen in another child (assuming the same partner) is 1 in 4 (25%). If the cause of your child's hearing loss is an autosomal dominant gene, then the likelihood that this will happen in another child (assuming the same partner) is 1 in 2 (50%). Should I have my other children tested? If it is determined that you and your partner carry the silent (recessive) gene which causes a hearing loss, then each of the children resulting with that partner have a 2 in 3 (66%) chance of also carrying that same gene. Whether or not your hearing children should be tested to determine if they also carry the deafness gene is a matter of choice. Your hearing children may want to know their own gene carrier status so they would know whether to have their partners tested to determine if their children (your grandchildren) have a chance of having hearing loss due to this gene. There is a 2 in 3 chance that hearing siblings (brothers and sisters) of a child with recessive hearing loss carry the hearing loss gene (like you and your partner). If this is the case, then their life partner could be tested to determine if they are also a carrier. If that partner is also a carrier of the same gene, then each of their children would have a 1 in 4 (25%) chance of having the same genetic

hearing loss. If that partner does not carry the same gene, then all of their children would be hearing, although each child would have a 50% chance of carrying the hearing loss gene. There is a 1 in 3 chance that hearing siblings do not carry the recessive hearing loss gene. If this is the case, there is virtually no chance of having a child with a hearing loss due to this gene, as the gene is not passed on. Is this something that I need to be concerned about in my other relatives? If you and your partner carry a recessive gene, then at least one of your parents and one of your partner's parents also carry the gene. This means that each of your brothers and sisters has a 1 in 2 (50%) chance of carrying the same gene. What will all this cost? The monetary costs of a genetic evaluation and medical tests are generally covered by your health insurance. Families should always check with their health insurance carrier to determine if genetic and medical tests are covered under their particular plan. Some people experience the psychological cost of guilt that they were responsible for this condition in their child. Be assured that everyone carries some recessive genes that, if present in a double dose, would cause some sort of genetic condition. Guilt feelings are natural during the stages of grieving and acceptance of your child's hearing loss. However, if those feelings persist or if they affect your ability to function day-to-day, please seek out someone who can help.