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EDHI
Malpensa ‑ 9:40 am CST Breakout Session
March 5, 2019

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Development of an Audiological Evaluation and Monitoring Protocol for Infants and Children with Congenital CMV
with Kristen Cortese and Lisa Weber 

   >> LISA WEBER: Good morning. We are here, my colleague and I, Kristen Cortese, to present on behalf of the Ann and Robert H Lurie audiology department. We developed testing protocol for patients with CMV. 
    Just to give you a little information about our department of audiology. We have six locations across the Chicagoland area. Our main hospital is in downtown Chicago. We're a specialty care center and a level 3 NICU, and we are not a birthing facility. 
    This is a picture of our hospital. This is a picture of our audiology team, 23 audiologists, 8 instruments, two hearing instrument dispensers as well as social workers and support staff that help keep our clinic running. I know, it's pretty cool. 
    Our presentation objectives today are to review congenital cytomegalovirus including how it is diagnosed, describe the difference between symptomatic and asymptomatic CMV infection, and explain developing the protocol for monitoring of patients with CMV and we will present some case studies at the end. Congenital cytomegalovirus virus, or CMV, is in the Herpes virus. 
    It infects over half of adults by age 40, and infection during pregnancy can infect unborn children. Congenital CMV occurs when the virus is passed to the fetus through the placenta in utero. It affects up to 40,000 infants born each year in the United States and over 1 million newborns globally. It is a major contributor to permanent deficits in childhood. 
    Congenital cytomegalovirus virus is diagnosed by certain tests, and the only way to differentiate a congenital infection from post natal is to complete testing within the first three weeks or 21 days of life. This can be done through amniocentesis or through urine, saliva, or dried blood spots. Our hospital uses urine or saliva testing. Dried blood is not available in the state of Illinois. 
    NICU physicians, PCPs, infectious disease and many others including early intervention providers. To tell you the difference between symptomatic and asymptomatic CMV, we will start with symptomatic. It affects approximately 10% and physical abnormalities are present at birth. 40 to 60% of infants will experience long term issues including motor, cognitive, hearing or vision loss. 
    Asymptomatic CMV affects approximately 90% of infants, and they have no clinical abnormalities present at birth. They can have some related symptoms, but most infants do not have permanent symptoms. Children who are asymptomatic may go undetected and unidentified. Hearing loss can sometimes be the only symptom of congenital CMV. And an infant with hearing loss and no additional symptoms is still considered asymptomatic. 
    CMV or congenital CMV is second only to genetic causes second only inherited cause. ‑‑ as the only finding, we were lucky that the Illinois state passed the following legislation on January 1, 2016 stating that if an infant fails the two initial hearing screenings in the hospital, then the hospital performing that screening shall provide to the parents of newborn infant information about birth defects from CMV and testing opportunities, including the opportunity to test before leaving the hospital and early intervention services. 
    Currently there's no state or universal guidelines for medical or audiological management of this patient population. Now I will hand it over to Kristen. 
   >> KRISTEN CORTESE: All right. Up until what we will go over during the second half of the presentation, our audiology team had been following these infants, but there were definitely some questions that were coming up amongst staff about should we do things differently for patients that are symptomatic versus asymptomatic, what is the standard. So, the rest of the presentation will go over the push for our department to create what we know there is no best practice guidelines out there for exactly a protocol to follow universally. So, what our department was trying to do to standardize our approach. 
    So we needed to determine what should our protocol be for audiological monitoring, testing, and follow‑up of patients diagnosed with congenital VMV. And should the follow‑up be the same or different for symptomatic versus asymptomatic infants. These are the steps we followed, so I hope you feel like you were in the room where it happened when going over the rest of this. So, we completed a review of the current literature. A discussion with the audiology clinical quality team, a team of audiologists at Lurie. They are on our diagnostic and cochlear implant team as well as those in leadership positions in our department and our director that helped promote evidence‑based practices. A lot of our new processes and procedures go through this team to get a special EDP look on it. And we completed a meeting with our infectious diseases department at Lurie, which was insightful on what their team was doing to manage the infants and how we could work together to best follow them. And finally we had insight from our Lurie children's Dr. Nancy young, who is our audiology director. 
    So these are some of the results of the lit review. So, we looked at the difference between hearing loss between the asymptomatic and symptomatic population of children born with congenital CMV. We know that children who are symptomatic comprise about 10% of the infants with CMV. Hearing loss in this group occurs at a higher rate. It's generally earlier onset and generally greater degree of severity and more bilateral losses. The asymptomatic group is about 90%. The hearing loss does occur at a lower rate, so there's less incidents. But the group is still large, so there are a lot of infants that were diagnosed with say symptomatic but do end up with hearing loss. The hearing loss is generally later onset and generally a lesser degree of severity and more unilateral losses in this group. It's more variable than this slide here, but this is what the research is showing is a trend when they're getting large groups of these infants together. 
    Hearing loss due to congenital CMV is progressive and late onset. Approximately 50% of hearing loss is due to congenital CMV are progressive, and the rate of progression seems similar in the symptomatic and asymptomatic group, but they are seeing that the symptomatic group progresses generally a little earlier, and they found the median age of progression in the asymptomatic group tends to be around 44 months. 
    Approximately 33 to 50% of the hearing loss is late onset. So, maybe it wasn't ‑‑ so it went present at birth and happens later. And the median age also occurs later in the asymptomatic group of infants. Usually occurs before ‑‑ the hearing loss usually occurs before 6 years of age, mainly within the first year of life. But hearing loss at older ages has been reported. So, it was seeing that there was one article that showed that only 88% of hearing losses were diagnosed by 3 years of age.  So there's definitely still potential for progression later.  So we really wanted to look at when the CMV ‑‑ when hearing loss due to CMV progresses, because that will directly impact our decision on protocol. So, the current protocol and procedures that we have in place right now that we decided on from working through this process, for infants and children with a diagnosis of CMV, we complete an initial natural sleep diagnostic AVR, because if there's anything minimal or mild going on at birth, that maybe wouldn't be caught by an AVR screening and we want to be sure to catch it quickly. We do follow‑up every six months until the age of three and yearly until the age of 5. And we do this for infants who are both symptomatic and asymptomatic. We are seeing that there is the potential for the late onset progressive hearing loss. We want to make sure that we're not missing any infants. 
    For those who fail the newborn hearing screening or may not have been tested for CMV, we have to think back to our Illinois mandate. And for any inpatient baby, we refer back to the inpatient medical team, and for outpatient, we want the patient to discuss this with their primary care physician. Lurie is a specialty care hospital, so a lot of our referrals for seeing different physicians in our hospital do go through their primary care physician, so that is why we take that approach. 
    With the meeting with the infectious disease department, we found that we are ‑‑ we have a lot in place in terms of caring for these infants, which is wonderful. So, they have a referral process, multiinstitutional referral module for prompt specialty care, so they want to see these infans very quickly, especially knowing that we can only confirm a congenital infection within the first three weeks or 21 days, we need to act fast. So, they get any referred infants scheduled within 24 to 48 hours, and the following evaluations are completed. They're not only seen by our infectious disease department but also seen approximate for an ophthalmology and MRI as well as natural sleep AVR testing. 
    And there may be additional recommendations for additional neural imaging such as MRI, a neurological evaluation may be recommended. And then if the baby has been diagnosed with hearing loss or has failed multiple hearing screenings, we'll coordinate an appointment with our ENT physicians on the same day. We do our best to coordinate care to get the baby in and see what is going on with the baby. 
    All right. Moving on to treatment and management. So, if the infant is symptomatic or asymptomatic with a diagnosis of isolated hearing loss, anti‑viral therapy is discussed with the family by the ID department. So, studies have shown that among infants with symptomatic congenital CMV, six month treatment with antibiotics had a favorable effect on neurodevelopmental outcomes and has not been associated with an excess risk of adverse side effects. 
    So we've gone over how to identify CMV, how to potentially treat a baby who has CMV, but how do we prevent CMV? So the reduce of CMV transmission can be reduced by the education of pregnant mothers. Unfortunately, and I was at the increasing CMV awareness presentation, which is excellent on Sunday here. And there was a lot of discussion that women of childbearing age often have little information, and if any information, it's often inaccurate. And unfortunately, a lot of health care providers are not pushing this. It can be spread through urine and/or saliva, and prevention can be done by hand sanitizing, not sharing toothbrush, not sharing utensils.  So we need to make this information more available to expectant mothers so that they're able to attempt to protect themselves while they're pregnant. 
    Okay. So, we're going to move on to some case studies which will hopefully explain everything in a more interesting way. The first baby passed the newborn hearing screening at birth in both ears. A different work‑up was done and eventually an ED doctor got involved, and by three months of age, a head ‑‑ and a positive urine test completed. While this patient was not identified in the three‑month period or the ‑‑ one‑month 21 day period. Sorry. They were still considered to have congenital CMV, because they were aware of all of this at birth. So, the patient is considered symptomatic. At three months of age, we saw the patient for a natural sleep ABR. We diagnosed normal hearing in the right ear and borderline to mild in the left ear. On the date, the DPOAEs were present in both ears. We didn't see this patient back until 18 months of age either for a follow‑up or the recommendations were not in place as much as they are now. At that time, behavioral testing was attempted and was unsuccessful likely due to the patient's developmental delay, not being able to provide sharp responses for us. So, at this point, we're now flagging something that is asymmetric. So, now from three months of age, we created a sedated ABR when this patient was 2 year of age. This gap was due to the family not coming in for the appointments. And so, I have a print out of a quick response to the ABR here. And for that left ear, which is borderline normal, this is a click response of the output limits of 90, which is no response. We are seeing a significant drop in hearing here. The right progresses from normal to mild, and the left progresses from borderline to possible mild to moderate to profound. At that point, hearing aids were dispensed. We're confirming something that is not just asymmetric but late onset and progressive. From three years to four years, we are seeing additional shifts. It progresses in left and right. And these are just the first ear specific pictures of the patient's hearing, so you can definitely see the asymmetry in the loss. At this point, the family was interested. The patient had been wearing hearing aids and was interested in pursuing cochlear implant evaluation, so that's the next phase that was moved on to. You can see now the patient is now bilaterally implanted, starting with the left ear, the more significant hearing loss ear. And then the right ear came later. The patient is doing very well. I didn't go into the rest of the story, but we wanted to show you the unaided story in terms of hearing loss. The last slide here is just a picture of the right ear on two different days. Definitely showing some fluctuation in the loss. So, this case is really an example of that late onset progressive flux waiting and asymmetric hearing loss that we do see with congenital CMV. 
   >> [ Inaudible Question ]

   >> KATHLEEN COLELLA: This patient was not because of being confirmed with congenital CMV so late. So, it wasn't recommended. 
   >> LISA WEBER: Case study number two. This little one failed their newborn hearing screening and tested positive for congenital CMV at birth. At 9 days of age, they were evaluated by infectious disease, ophthalmology and received an ultrasound of the head all on the same day. The patient was found to be asymptomatic with isolated neural hearing loss. This is an example of our state legislation law at work, because this kiddo may not have been identified had it not been for that legislation that had been passed. 
    At three weeks of age, they were diagnosed with a profound sensorineural hearing loss in both ears, and this is the results from our ASSR, our auditory steady state response testing. It's another version of the ABR, for those of you who may not be audiologists. They were seen at five weeks and fit with binaural hearing aids at six weeks of age. This is just an example of how quickly we can intervene when we have the right information. 
    This is just an example of the audiogram when they were able to be tested behaviorally. The one on the left is consistent with profound hearing loss on both ears. And the child did receive benefit from hearing aids.  So the squares are their hearing aid responses with both hearing aids. The R and the L is for the right and left hearing aid. So, not only looking at how they hear with both hearing aids together, but each one individually. 
    This family did choose to pursue a cochlear implant evaluation, and we use a lot of information when we recommend a cochlear implant evaluation, including a little ears. 2 out of 35. This indicates that the auditory behavior is not age‑appropriate. About this age it should be around 20 out of 35. And we also got great feedback from a local DTH provider that we work with who was letting us know that this child had no response to their own name or voices, no consistent responses to music, and that they're very visual in watching faces and mouths. And so at this time, this family is getting ready to get a cochlear implant. 
   >> KRISTEN CORTESE: This is our last case study. No risk factors for hearing loss. We evaluated them initially at 3 weeks of age for a follow‑up failed hearing screening and natural sleep ABR testing was incomplete. At five weeks of age, we confirmed through ABR and ASSR a bilateral profound hearing loss in both ears. At this point the infant was referred back to the primary care physician. That physician recommended and discussed CMV testing with the family, and the CMV test came back positive. So, this is just an example of that hearing targeted screening. So, had we not made those recommendations, this is a baby that potentially could have been missed in terms of confirming a diagnosis of congenital CMV. You can see it was done a little past that 21 days. Our department will test a little bit past that time, because they want to do their best to try and give parents some insight if they can. 
    And then at six weeks of age, they came in for the comprehensive evaluation. They were evaluated by our IED department, ophthalmology, and they were found asymptomatic. At two months of age, recorded again. So, they were evaluated by ENT and dispensed with binaural hearing aids. 
    So in conclusion, the connection of hearing loss and congenital CMV is well known. Audiology has a significant role in regards to monitoring, treatment, and management of this patient population. EHDI may lead to a diagnose that may have gone undetected. So, the fact that we're doing universal newborn hearing screenings, it is catching some infants that we may not have known that they had congenital CMV at birth, especially in the asymptomatic population. We hope that our protocol continues to evolve with additional research in the area of anti‑viral treatment, and the benefits to children in regards to stabilization or improvement of hearing and as well as universal newborn hearing screening for congenital CMV. It's coming back that it's great. It does identify babies and catch babies. But there are still babies that will be missed because of the fact that the hearing loss can be so late onset and so progressive in nature that it may not be present at birth.  So we don't have that catch‑all there. And finally what I think we're all hoping for is a vaccination. 
    Do we have any questions? 
   >> [ Inaudible Question ]

   >> For the younger range of infants when you're testing them before you can give them ear specific, do you do ABRs, sedated or unsedated? What's your protocol for the actual tests you do? 
   >> Good question. For that patient population between the newborn testing, and we start to try to do behavioral testing at 6 months of age. We'll do OAE testing if they're between sleep and trying to give us behavioral responses. And then if they're at the age where they can give a sound field audiogram, we are reliant on that. So, we're hoping in the first case we would flag something asymmetric if the OAEs were different. But you're right. We do want ear‑specific information to know for sure if something's going on. So, if we're seeing something abnormal and the baby is not able to give us a full ear‑specific audio, maybe we, because developmentally, we would talk about a sedated testing with the family if we weren't comfortable waiting long enough. Of course we want to intervene as soon as possible, but we don't want to sedate babies. That's a case‑by‑case basis and a discussion with the parents. 
   >> I think we have time for one more question, and then I will ask if you have questions, you can talk to our presenters out in the hallway. 
   >> What have you found the consensus about the ear ID department is doing in symptomatic or asymptomatic? 
   >> So, as we presented in the first presentation, our department does do the treatment, but they will only do treatment for kind of the two out of the three groups.  So that's a group of symptomatic infants and a group of asymptomatic infants. So, if there is a symptom, they'll proceed with therapy. If the patient is asymptomatic, we have not diagnosed a hearing loss. At this point, that recommendation is not being made with the parents. 
   >> Is it like 80% of those... [ off mic ]... 
   >> Our department is, they all do the same thing. But at other hospitals, I can't speak to that. I think it's variable. Some hospitals will only treat patients who are symptomatic. Some maybe are not doing it. I can't speak for the ID departments at every facility. I know our doctors probably have more insight than I do. Definitely a good question. It's a very interesting topic, especially to us. 
   >> Thank you all for coming. I'm sorry I have to wrap it up. It's a great presentation. If you need an evaluation form, please see me. I do have some extra. 
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