Advanced Management of Complex Cases: Children with Multiple Medical Conditions

Editor’s Note: This text course is an edited transcript of a live webinar. Download supplemental course materials.

Learning Objectives

After today’s course, you will be able to identify expansions in cochlear implant candidacy guidelines for children, identify medically challenging conditions which are associated with hearing loss, and identify treatment options and prognoses for pediatric cochlear implant candidates and recipients with multiple medical diagnoses. 

Introduction

Cochlear implantation was introduced in the United States as a viable treatment for hearing loss in the 1980s.  Originally, the anticipated benefits were awareness of sound and enhancement of speech reading.  Therefore, the early candidacy guidelines were very strict.  There were concerns that the benefit from a cochlear implant would be limited or potentially be very difficult to measure, so the industry was conservative about who it considered for an implant. 

Original FDA Guidelines

At the time of the original Food and Drug Administration (FDA) guidelines for the pediatric clinical trials, children 24 months of age and older were considered.  They had to demonstrate a profound hearing loss in both ears and no benefit from traditional amplification.  These children would also demonstrate no co-existing medical conditions, normal vision and normal intelligence.  The children would have no radiological contraindications.  They would demonstrate evidence of strong family support and appropriate expectations and motivation of family members and caregivers. 

Current FDA Guidelines

The current FDA guidelines for pediatric cochlear implant candidacy include infants 12 months of age and older with 90 dB or greater hearing loss in both ears for infants 12-24 months or 70 dB or greater hearing loss in both ears at 24 months of age and older who receive marginal benefit from hearing aids.  There can be no medical or radiological contraindications, and there must be strong family support and appropriate expectations and motivation from the family and caregivers. 

Then and Now: Comparing FDA Guidelines

Age

Over the course of time, the implant age range was lowered from 24 to 18 months, and then finally to 12 months when the safety and efficacy data revealed that a cochlear implant procedure was not dangerous.  The primary goal of cochlear implants in children has shifted over time from basic awareness of sound to our expectation that children will use their hearing to acquire language.  With that in mind, earlier implantation is equated with closing the gap in language delay more quickly.  At present, infants under two years of age may be considered with a pure-tone average of 90 dB or greater in both ears, and children over the age of two years may be considered with a pure-tone average of 70 dB or greater in both ears. 

Benefit

I would like to discuss the difference between “no benefit” and “marginal benefit” from traditional amplification.  Originally, aided benefit was defined as a patient having audibility of sound within the speech banana.  That could be one frequency at 500 Hz or that could be all four frequencies.  The concern would be that if a patient had some audibility of sound, we would anticipate that we would be destroying the residual hearing by placing a cochlear implant electrode.  We did not want to risk that in someone who already had some audibility, however slight it might have been. 

The current guidelines allow for pediatric candidates to have audibility in the speech banana, and that has progressed on to the point where those patients who are able to participate can demonstrate as great as 30% open-set understanding on word tests. 

Going back to the idea that a cochlear implant was originally thought to be an enhancement for lip-reading abilities, it was important to have normal vision in order to benefit from the implant.  The current guidelines do allow for visually impaired individuals to be considered.  Obviously the performance data demonstrates that a patient can receive benefit far beyond the simple speech reading tasks.  We would want anyone who had the dual impairment of hearing and visual loss to be able to access sound for communication purposes. 

Originally, we felt that it was important to have normal cognitive function, because how would we fetter out the benefit that a candidate received from a cochlear implant?  The concern was that it would be almost impossible to demonstrate if benefit were affected by the cognitive delay itself.  At present, it is widely accepted that individuals with less than normal intelligence can, in fact, receive benefit from a cochlear implant. 

Contraindications

The medical conditions and contraindications have changed over time.  Persons with additional medical conditions were originally not implanted for a variety of reasons.  The main concern at first was that the risk of surgery would outweigh the anticipated benefit, keeping in mind the goal was audibility of sound and improved speech reading.  The effects of chronic electrical stimulation on the brain were not known.  Do we risk someone who already has other conditions and then add electrical stimulation not knowing whether it was going to also have a negative effect on the person? 

There were concerns regarding any kind of post-operative infection or other complication that could put the person at increased risk, and we had anticipated challenges in measuring benefit due to the impact of any of those co-existing conditions.  However, as we all know, cochlear implantation is currently recognized as a very low-risk procedure with many places doing a same day discharge following the implantation for the majority of individuals. 

Radiologic Studies

Those candidates whose radiological studies were atypical were not considered, because of concerns about a limited insertion of the electrode array and a limitation of the derived benefit from the implant device.  At present, we can consider candidates with ossification in the cochlea or fibrous tissue, cochlear malformation, or enlarged vestibular aqueduct.  The only exclusions on cochlear implantation would be if the patient had a lack of internal auditory canal and aplastic auditory nerve or an absent cochlea. 

Family Support

I cannot overstate or overemphasize the importance of family support and appropriate expectations and motivation.  This is the only unchanged guideline over time, and especially in children, whether they are typically developing children or children who have multiple conditions, this is key to their success and outcome with a cochlear implant device.  This is something that is very important and will likely never change over time. 

Factors Affecting Outcomes

There are some factors that can affect outcomes as well as broadening the candidacy guidelines that Cosetti and Waltzman (2012) reported.  First are the technology advancements themselves.  Things have changed since the beginning of cochlear implants.  We are seeing patients with excellent and improved speech understanding. 

Something that we did not anticipate originally, but are seeing with more current technology, is the improvement in music appreciation for patients.  With advancements in surgical techniques, preservation of the cochlear structures and preservation of residual hearing, these are important and have certainly allowed us to consider a wider range of candidates over time.  I think for children in particular, the availability of electrophysiological measures and objective measures for programming of cochlear implants devices for very young children, children with limited communication, or limited ability to give responses, is key in programming.  A great standard of internal device reliability makes us feel more confident in offering the cochlear implant to a wider range of patients. 

When looking at children with multiple medical diagnoses, there is the question of why this population of candidates has grown.  One answer I could find in the literature was that the survival rate of prematurity has increased greatly in the latter half of the 20^th century.  Aspects that contribute to hearing loss and/or other medical diagnoses would include low birth weight, oxygen dependency, and micro preemies being delivered at fewer than 26 weeks gestational age; these are all traits that can carry greater risk for multiple diagnoses. 

Multiple Medical Diagnoses

Wakil et al. (2014) reported 30% to 40% of children with sensorineural hearing loss will also have additional developmental disabilities.  Hang et al. (2012) also reported that 30% of sensorineural hearing loss is associated with a genetic syndrome.  As an aside, now that we are identifying hearing loss so early and also implanting children earlier, another likelihood is that an increasing number of children are being implanted prior to certain disabilities or conditions even being identified. 

Other statistics from Corrales and Oghalai in 2013 indicate that 25% of cases of congenital hearing loss can be attributed to prenatal or postnatal disease or trauma, 18% to undiagnosed genetic factors, 15% to autosomal dominant genetic mutations, 40% to autosomal recessive genetic mutations, and 2% to sex-linked genetic mutations.

When we are evaluating patients for cochlear implant candidacy, we do the following types of measures.  First, we need to verify the hearing loss using both behavioral and objective tests measures, parent questionnaires such as the Infant/Toddler Meaningful Auditory Integration Scale (IT MAIS), and therapist and teacher observations.  In a typically developing child, it is very important to involve the family.  In a child with multiple medical conditions, it is even more important because we are depending so greatly on family as informants, acting as the bridge between professionals, and lastly as decision makers in this process.

Parents and Caregivers as Informants

It is important for the family to provide thorough and accurate information regarding the child’s conditions; likewise, they try to give full disclosure regarding the child’s conditions and therapies.  In my population of patients, these children are often very young with a lot going on, and it can be hard for the parents to keep track of every doctor that sees their child and every therapy that they are receiving, but we need to be able to integrate all of that into the process, and that full disclosure is critical and key in their care.

Parents and Caregivers as the Bridge Between Professionals

It is important for the family members to discuss their desire for consideration of a cochlear implant with the other treating professionals involved in the child’s care.  Likewise, because they are sometimes acting as the bridge between professionals, providing information about the cochlear implant to other doctors and therapists who may not be familiar with this technology is important.  Also, they are needed to request and encourage the treating professionals to communicate with one another. 

I work for a medium-sized children’s hospital.  The majority of my patients receive all of their care under our umbrella, which makes it easy for me to access their medical records and the other treatment professionals involved with the child.  However, in some cases, that is not always the case, especially if children travel from a distance or are working with independent professionals.  Asking and providing information so that the treating professionals can all be in touch with one another and can co-treat is critical for these families.

Parents and Caregivers as Decision Makers

As decision makers, parents and caregivers need to be empowered to give their truthful and unabashed opinion on what they would like to see for their child, how they are feeling about participating in the process, and whether they want to proceed with an implant.  They should be able to say at any point in time that they are not comfortable or they need to take a break from the process because the child has other things going on that are superseding the cochlear implant process.  This would include things like vaccinations.  I had a child recently who came to see who was diagnosed with a severe immunodeficiency, and she cannot have certain vaccinations.  In working with other professionals and the parents themselves, we can decide whether or not we can pursue an implant if the child cannot have the meningitis vaccine. 

Children who are at greater risk when placed under anesthesia need to have special consideration as to whether or not it is the best idea to proceed with the cochlear implant surgery or if the anesthesia risk is too great.  If there is a long-term need for MRI or studies that might be contraindicatory to the cochlear implant, parents might need to think about specific considerations. 

For those children who have sensory issues or might be intolerant of the stimulation from the implant or wearing equipment, we need to decide if implant makes the most sense at that time or if we should work on compensatory strategies with the child before proceeding with the implant. 

Evaluations

When verifying lack of benefit from the hearing aids, the evaluation measures need to be selected not only on the child’s chronological age, but tailored specifically to the child’s cognitive status and language age.  We want to make sure that we choose tests that do no create an unfair bias or make the child look “worse” or less viable a candidate.  It is important to look at the child’s developmental abilities, not just their chronological age.

For the medical evaluation, the surgeon needs to consider whether the child is healthy enough to undergo anesthesia for the surgical process and can also participate in the recovery process with a minimal risk of additional complications. 

Radiologic Studies

Radiological studies will indicate verification of the cochlear status and presence of the intact auditory cochlear nerve.  The radiological studies will also identify any findings that could result in prolonging or complicating the procedure.  While we are stating that a patient who has ossification in the cochlea or an atypically formed cochlea still can be considered for candidacy, that might result in the surgeon needing to choose a specific electrode array or to advise the parent that the patient will be under anesthesia longer because they will have to work harder to get the implant in just the right way.  Also, radiological studies may help the surgeon determine if one ear is more viable for receiving a cochlear implant that the other.  Inner ear malformations are present in approximately 20% of children with congenital hearing loss.  It is important to have solid radiological studies in place when we are making this decision.

Cognitive and Language Skills

Cognitive and language skills can be challenging to assess in children who have more severe developmental delays or in very young children.  It may turn out to be very subjective in nature.  Something important to consider in this process would be whether the child demonstrates communicative intent.  That is how the child is currently expressing his or her wants and needs.  Is the child even interested in expressing his or her wants and needs? 

Something else to consider is a pseudo handicap effect, where you have coexisting disabilities that increase the overall disability.  We need to look at whether or not these things that are cohabitating. Are they making the child look more delayed, disabled or cognitively impaired than they really are?

Quality of Life

Another aspect for our children with multiple medical diagnoses is the impact of a cochlear implant on quality of life.  The World Health Organization’s definition (1995) of quality of life is “the individual’s perception of their position in life, in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns.”  I feel this definition is wordy and a bit complex.  In my mind, the quality of life consideration for a child when we are looking at a cochlear implant is if the child is experiences a greater awareness of his or her surroundings and improves his or her ability to communicate his or her desires or needs, then the cochlear implant has a positive impact on quality of life. 

Cochlear implants are utilized with the intent of improving hearing for learning auditory-based language.  For some of these children, we are discussing that they may still achieve that, but with the additional disabilities, we may see poorer or slower progress on traditional measures of speech perception or language acquisition.  We need to step back and look at whether or not the child’s actual quality of life is being improved as a function of positive outcome with that implant. 

When we are making consideration for impact on quality of life, it is key to cultivate family support, appropriate expectations and motivation.  This is my pitch for the importance of enrolling the child and family in auditory-based therapy or with a good language therapist prior to a cochlear implant.  Families need parent coaching in order to understand what they are getting themselves into over the long-term, and a good language therapist is the right person for that job.  They need to be able to talk with the family about what we are doing today before the implant and also what we are going to be doing after the implant to create that appropriate expectations for the long-term habilitation and to cultivate the support to the child so that at the time of implant. 

Then, the therapy component is already well-established and in place and the child does not have to undergo another transition after the implant; this would be a single transition from a hearing aid to a cochlear implant device.   Second to that would be monthly hearing aid checks with a cochlear implant team audiologist, which will promote the opportunity for discussion with the family to cultivate support, discuss their expectations, address any concerns, and be there for the family. 

Medical Diagnoses Associated with Hearing Loss

We are going to talk about medical diagnoses which are associated with hearing loss.  There are so many conditions.  I tried to choose some of the most common and those with which we are familiar, as well as some that are not so common.  I will review some case studies as well. 

Congenital Cytomegalovirus

Congenital cytomegalovirus (CMV) infection is the most frequent infectious cause of hearing loss.  Ninety percent of affected patients do not show the typical clinical symptoms (asymptomatic).  There are many associated disorders with CMV infection which can include autism, learning disabilities or cognitive delays.  Symptoms of CMV infection in infants would include jaundice, enlarged liver, enlarged spleen, petechiae, and pneumonia.  Some external characteristics that we observe in CMV patients are small head size, brain abnormalities, eye problems, hearing loss, and central nervous system damage. 

Usher Syndrome

Usher syndrome is the most autosomal recessive cause of hearing loss.  With Usher syndrome, you may also see associated vestibular dysfunction and progressive loss of vision.  Because of the progressive loss of vision, we recommend early implantation prior to visual impairment so they can maximize vision and hearing at the same time. 

CHARGE

CHARGE syndrome is a very rare congenital disorder, and more than 90% of children with CHARGE syndrome also demonstrate hearing loss.  CHARGE stands for coloboma, heart defects, atresia of the choanae, retardation of growth and development, genital/urinary abnormalities, ear abnormalities and/or hearing loss.  Nearly all children with CHARGE syndrome will demonstrate semicircular canal aplasia, cochlear nerve deficiency and vestibular dysplasia.  A good percentage of them will have incompletely developed cochleae, which leads to very important surgical considerations including atypical anatomical landmarks, aberrant facial nerve course, and abnormal cochlear anatomy.  This goes back to the idea that we would need to discuss potentially a longer time in surgery or changes in electrode array or a limitation in insertion. 

Cochlear implants should be considered for these patients due to the additional potential for visual impairment.  Quality of life improvements tend to be more related to awareness of sound, because speech understanding is not often achieved in this population, which I would presume routes back to the fact that there is cochlear nerve deficiency and the majority of these patients have atypically developed cochleae. 

Down Syndrome

In patients with Down syndrome, we see low muscle tone, flat facial features with small nose and upward slanting eyes, small, low-set ears, and hyperflexibility.  Fifty percent of patients with Down syndrome will demonstrate cardiac defects, 10% will have thyroid disease, over 50% will have vision issues, and 10-12% will have gastrointestinal disorders requiring surgical intervention.  This is a population that does struggle with recurrent otitis media.  Otitis media management is critical due to the increased risk of post-operative meningitis.  Pressure equalization (PE) tube placement may be necessary.  Sometimes hypoplastic cochleae occur.

These patients oftentimes show cognitive delays ranging from mild to severe.  Due to the range of cognitive delays, the rehabilitation process may be longer with this population. 

Cerebral Palsy

Another condition associated with hearing loss is cerebral palsy, which may range from very mild to very severe.  These patients may present with low muscle tone or be floppy.  They may also demonstrate muscle spasms.  There may be poor muscle control, reflexes and posture.  Oftentimes there are delays in physical development and feeding difficulties.  Patients with cerebral palsy also may show difficulties with swallowing, breathing, head or neck control, bladder or bowel control, feeding, dental issues, and digestive issues. 

For these patients, communication may be impacted by oral-motor weakness.  Of these patients, 10% will also demonstrate severe vision impairment.  Fifty percent will demonstrate some level of cognitive delay, and one in 50 will have profound hearing loss.  Hearing loss can sometimes be diagnosed later due to the severity of other medical diagnoses. 

Something important with this population is that 35% of affected individuals are non-ambulatory.  We have to give special consideration for these patients in terms of placement of sound processors, specifically the microphone and retention of the headpiece. 

Cleft Lip and Palate

Congenital cleft lip/palate affects up to 7,000 babies in the United States annually.  Cleft lip refers to a split or opening in the lip, which may be large enough to connect the upper lip and nose.  Cleft palate refers to an incomplete closure of the roof of the mouth.  The hard palate is the bony front portion and the soft palate is the soft tissue in the back of the mouth.  Patients with cleft lip and palate often experience frequent episodes of otitis media.  They also demonstrate speech and articulation issues, feeding difficulties, dental problems, and socio-emotional issues due to differences in appearance. 

Surgical repair of a cleft lip and palate is recommended within the first year of life.  For these patients, 89% will experience conductive hearing loss, 8% experience a mixed hearing loss, and 3% experience sensorineural hearing loss.  Medical management of the chronic conductive component is critical in this population. 

Waardenburg Syndrome

Waardenburg syndrome is an autosomal dominant syndrome.  Sensorineural hearing loss is a primary component.  Another characteristic of this syndrome is an iris pigment abnormality; specifically, there are two different colored eyes.  Sometimes there is a white forelock of hair.  You may also see also wide-set eyes, broad nasal root, and a low hairline.  These patients experience intestinal defects as well as spinal defects.  Type I and II are the most common forms, and type III and IV are rare.  This syndrome accounts for 2% of congenital profound hearing losses. 

Autism Spectrum Disorder

Autism spectrum disorder is characterized by extreme unresponsiveness to others, communication deficits, poor turn-taking skills, difficulty managing emotions, and rigid and repetitive behaviors.  We have all seen the commercials which say that 1 in 68 children have been diagnosed on the spectrum, with a greater incidence occurring in boys.  Approximately 3 to 4% of children with autism spectrum disorder will also demonstrate profound hearing loss.  They typically also have other developmental milestone delays. Other characteristics might include poor eye contact, resistance to touch, cuddling and hugging, and atypical play by not engaging with toys. 

Patients will autism spectrum disorder or those patients who demonstrate sensory integration issues in general do require some special considerations.  One is tolerance and consistent use of hearing devices.  A well-completed hearing aid trial would be important with this population in particular.  There are often challenges with making earmolds or keeping hearing aids in place.  Some of these patients have a dislike of light pressure, which could include the placement of a hearing aid or a sound processor over the ear.  Patients with autism spectrum disorder sometimes demonstrate challenges with loud sounds or specific sounds.  They may have challenges with the overall ability to transition and difficulty with change.  This is a population where we need to look at the desire to communicate or communicative intent. 

Congenital Rubella

Congenital rubella is another medical diagnosis associated with hearing loss.  Fifty-eight percent of infants with congenital rubella will demonstrate profound sensorineural hearing loss.  Along with that, patients will have vision and cardiac problems.  They may also display developmental delay, autism, schizophrenia, growth retardation, learning disabilities, diabetes, and glaucoma. 

Donnai Barrow Syndrome

Donnai Barrow syndrome is a rare autosomal recessive inherited disorder. There are only a handful of cases reported.  I do have a case study, which we will discuss.  This manifests with unusual facial features.  They have wide set eyes, short nose and flat nasal bridge.  They also have posteriorly rotated ears and a widow’s peak hairline.  They have sensorineural hearing loss, extreme nearsightedness, underdeveloped or absent corpus callosum, diaphragmatic hernia and omphalocele.  They display mild to moderate cognitive delays. 

Case Studies

Case #1: HT

The first case study is HT, who was born 10/26/2010.  Her mother contracted rubella in the first four weeks of pregnancy.  Upon evaluation, HT presented with profound bilateral sensorineural hearing loss identified at two years of age while still living in Vietnam.  She has cataracts and a significant visual impairment, and is followed by ENT and ophthalmology. 

Her communicative skills were assessed, indicating a severe expressive and receptive language delay.  I first evaluated her when she was almost four years of age.  She was using only simple gestures, open-mouthed vocalizations and crying.  There was no babbling or expressive jargon.  She was reported to understand pointing and the signs for the words “no”, “want” and “all done.”  She had never worn hearing aids until she came to the United States.   She was nearly four when she was fit with amplification. 

She was enrolled in therapy for eight months, in part because we needed a trial period and in part because there were some insurance issues that needed to be remedied before we could get someone to pay for the implant itself.  She was able to achieve full-time hearing aid use of 10 hours a day. 

Some of the challenges with this patient were the lack of communication system and severe language delay, the duration of her deafness, and visual impairment which was lending itself to challenges in learning sign language.  She had no viable communication abilities, and she was an indulged child.  She was prone to tantrums and was allowed to run the show. 

The strengths were the parental involvement.  These parents were very receptive to parent coaching and bought into full-time hearing aid use, participation in therapy, and carry over of the therapy goals to the home.  Nonetheless, because of her age, duration of deafness, and the lack of language system, our prognosis for her was guarded. 

Her CT scan and MRI were both within normal limits, and she received an Advanced Bionics Mid Scala device in February of 2015.  All of the channels were activated.  She was able to maintain a sustained conditioned response to sound for programming, and we were also able to obtain neural response imaging across the electrode array. 

Her progress with a hearing age of six months was pretty impressive.  She is responding to her name at home and in therapy.  She is imitating the Ling 6 sounds in therapy.  She will indicate when she hears environmental sounds.  She really likes music.  She is emerging on imitating the number of syllables in a word.  She will associate Learning to Listen sounds with the corresponding toy, such as /a/ for an airplane or /b/ for the boat.  However, as we have had her longer and longer in therapy, we are also seeing a moderate pragmatic delay, which is characterized by poor communicative intent, poor turn taking, and difficulty expressing and managing her emotions.  She has not been diagnosed on the autism spectrum as yet, but that is a definite concern for us with this patient. 

Case #2: IM

The second patient is IM, who was born 04/25/2012.  He has Donnai Barrow syndrome.  He has a diaphragmatic hernia, which necessitated ECMO (extracorporeal membrane oxygenation) treatment while in the NICU.  His intestines, liver and other organs were all outside the outer abdominal wall.  He had ventriculomegaly and bilateral coloboma, which are congenital defects of the eye.  He has a feeding tube and feeding issues.  He spent a couple of months in the NICU, during which time he was diagnosed with bilateral profound sensorineural hearing loss.

He shows many global physical developmental delays.  He is enrolled in occupational, physical, and speech therapies.  It may be easier to list the specialists he does not see, as he sees most all specialties for his care.  There were many people to incorporate into making this decision.  Upon evaluation, he demonstrated a severe expressive and receptive language delay using only crying and some vocalizing.  He was participating in auditory verbal therapy at home via Skype, and his hearing aid use was more than 8 hours a day. 

This child has significant health-related issues.  One setback was that he was hospitalized for 39 days after his omphalocele was repaired due to renal failure.  The risk versus benefit for the surgery caused us to be worried that he would not be healthy enough to withstand the cochlear implant procedure.  He also has global developmental delays which could confound our assessment of post-implant progress. 

He has a strong advocate in his mother.  She flies from across the state to obtain care for him at our hospital.  She lives in an area where there is not a children’s hospital and very few specialists.  Our prognosis for him is fair to guarded. 

We were finally able to get a CT scan and MRI after he became healthy enough to be sedated for the tests.  Those studies were within normal limits.  He was implanted in June and July of 2014.  This child does not make any kind of conditioned response to sound.  We programmed him using behavioral observation and neural response imaging.  His progress with a hearing age of 12 months is that he is searching for a sound source.  He knows his name and family members’ names and some basic commands.  He will imitate singing and speech sounds, and enjoys music.  He can say “uh-oh” and “kitty.”  He has an emerging conditioned response to sound and a functional language age recently assessed at 7 to 8 months. 

Case #3: AT

Our next case is AT, who was born on 12/06/2012 and experienced birth trauma.  She was anoxic at birth and had an intraventricular hemorrhage.   She has a G-tube and feeding issues.  She still has an overnight nurse for 12-hour feeds.  She has suspected vision problems, low muscle tone, and recurrent otitis media.  At the time of her evaluation, she was receiving occupational, physical, speech and vision therapies.  She is followed by ENT, Gastroenterology, Neurology, Genetics and Ophthalmology.  Her hearing loss was identified at two months of age.  She has a severe expressive and receptive language delay, but she is a very vocal child. 

From the minute I met her at 12 months, she was babbling and using expressive jargon.  She likes to interact and engage people.  She has oral dysphasia and a feeding aversion.  This is something they are still working on.  She was able to be enrolled in auditory-based-care therapy with 80% attendance, and we achieved full time hearing aid use with some difficulty because the child was always pulling out the hearing aids and sticking them in her mouth. 

Some of the challenges are her co-existing feeding issues and global developmental delays.  She had to have PE tube placement prior to cochlear implantation.  Mom basically functions as a single parent; Dad is in the military and is gone almost more than he is home.  She is very involved and active in getting her to therapy and carrying over the goals into the home. This is a child with very strong communicative intent.  We felt her prognosis was very good. 

She was implanted a little over a year ago in the right and left ears.  All channels are active. She has an emerging conditioned response for programming, and we were able to obtain neural response imaging.  With a hearing age of 12 months, she now demonstrates a mild-to-moderate receptive language delay and borderline-to-low average expressive language delay.  She is using single words, single signs, and labeling objects.  She is able to follow directions, identify pictures and respond to yes and no questions, auditory only.  She was discharged from Physical Therapy because her goals were met.  She is doing very well, and her prognosis was right on for good results. 

Case #4: EC

The last case, EC, was born prematurely on 08/24/2008. The conditions included placenta previa with delivery at 30 weeks gestational age.  He had an extended NICU stay of two months and repaired cleft lip and palate.  He was also anemic and had a patent foramen ovale, both resolved.  He experienced jaundice and required phototherapy.  He was followed for leukocytosis and still sees hematology about once a year for monitoring.  He was diagnosed with auditory neuropathy spectrum disorder (ANSD).  He also experienced chronic otitis media, oral dysphagia, and failure to thrive. 

He is followed by ENT, Facial Plastics, Hematology, Cardiology, and Gastroenterology.  His hearing loss was identified while in the NICU.  After his discharge, his parents felt he was more responsive to sound than anticipated based on the ABR.  A repeat ABR at 24 months reconfirmed ANSD, and the parents decided they would try fitting of hearing aids at two years of age. 

When we first evaluated him, he was demonstrating a moderate expressive and receptive language delay.  When he initially started participating in therapy, the hearing aid use was very inconsistent.  He also has a severe articulation disorder and moderate oral-motor delay.  He had slow and steady progress over the next year to year-and-a-half in therapy, but remained moderately delayed in language. 

The parents continue to observe that the child heard well with or without the hearing aids.  They were reluctant to pursue the cochlear implant because they felt that he was smaller than everyone else and has had so many other complications, that as long as he continued to make acceptable progress, even if he is a year behind age-mates, they were okay with that. 

We did a cochlear implant evaluation at five years of age.  His behavioral responses were in the range of a mild to moderate hearing loss.  He was making progress, but at that point, we were trying to point out to the parents that when he was just using his hearing, he did not understand anything; that was not where he was getting his language from.  His parents committed to full-time hearing aid use and intense therapy over a three-month period of time.  There was no improvement noted in language acquisition; auditory only.  He was able to use three-word phrases with inconsistent labeling of items.  He had a significant reliance on lip-reading and visual cues, and no open-set speech understanding, which was the clincher for the Dad, who was the reluctant parent throughout this process.

The challenges in this case were oral-motor delay, which impacted speech production.  There was a history of inconsistent hearing aid use, and our concern was that it would influence consistent use of the sound processors.  He did have chronic otitis media requiring PE tube placement.  The parents’ support improved over time with recognition of auditory limitations.  With the moderate language delay, we knew he had auditory pathways that could be stimulated, and there was less of a language gap to close.  His prognosis was fair to good.  The parents were concerned about intelligibility of speech, which, with the lip repair and oral-motor issues, may not improve as desired. 

EC is a bilateral recipient.  He has a great sustained conditioned response.  We were also able to obtain neural response imaging with this patient.  His hearing age range is now 7 months on the right, 3 months on the left.  He still demonstrates a moderate to severe expressive and receptive language delay. 

He has turned six years old, and the items are getting harder on the test materials; I think that is why we he is moderate to severe in terms of his language delay now.  He shows a mild to moderate oral-motor delay.  He can imitate the Ling 6 sounds and identifies target items from pictures.  He is able to give answers to questions after a short story without visual cues.  He has difficulty with multi-elemental instructions and is using 3 to 5 word phrases inconsistently. 

Conclusion

In conclusion, children with multiple medical diagnoses are not precluded from cochlear implantation.  Families should be actively involved in the candidacy process.  Consideration should be given to improvements in quality of life versus looking at the traditional anticipated benefits of auditory-based language learning.  With the case studies, we have demonstrated that family support and expectations/motivation are key to success with a cochlear implant for children who have multiple medical diagnoses.   

References

Corrales, C., & Oghali, J. (2013). Cochlear implant considerations in children with additional disabilities.  Current Otorhinolaryngology Reports, 1(2), 61-68.

Cosetti, M., & Waltzman, S. (2012). Outcomes in cochlear implantation: variables affecting performance in adults and children. Otolaryngologic Clinics of North America, 45(1), 155-171.

Hang, A., Kim, G., & Zdanski, C. (2012). Cochlear implantation in unique pediatric populations.  Current Opinion Otolaryngology Head Neck Surgery, 20(6), 507-517.

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