Deaf children with additional disabilities (AD+): CODEPEH recommendations

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There are other variables to consider such as gender, cognitive abilities, presence of an additional disability, educational or socio-economic level of the parents, age at which the child was diagnosed and treated audiologically, type of hearing device chosen, quality of the fitting and the appropriate use of the device.<a class="elsevierStyleCrossRef" href="#bib0010">2</a> Between 40% and 49% of deaf children have an additional developmental disorder or major medical problem which, in addition to in many cases delaying the age at which hearing loss is diagnosed, requires the intervention of other specialists.<a class="elsevierStyleCrossRefs" href="#bib0015">3–5</a> These children are known in the literature as "deaf or hard of hearing PLUS",<a class="elsevierStyleCrossRef" href="#bib0030">6</a> although it seems more appropriate in Spanish to use the term "hypoacusis or deafness and added or associated disability", which applies both to diagnosed deafness with other associated disorders and to diagnosed disabilities with associated deafness. Children with a hearing impairment with an associated disability are increasing considerably in number and diversity, posing a major challenge for early detection and diagnosis, and early intervention programmes for childhood hearing loss.<a class="elsevierStyleCrossRef" href="#bib0035">7</a> The population of hearing-impaired children is more likely to have associated disabilities because risk factors for hearing loss<a class="elsevierStyleCrossRef" href="#bib0040">8</a> overlap with risk factors for many other disabilities, such as the presence of certain genetic syndromes, prematurity, congenital infections, and meningitis. These factors can affect various aspects of development as well as language acquisition. Early detection is a challenges posed by the association of a disability with hearing impairment, therefore 6-monthly assessment of speech and language development in every child with deafness is extremely important, because identifying a disability in addition to deafness enables early and appropriate intervention. Another challenge is the need for smooth and coordinated transdisciplinary collaboration between all professionals involved.In this article, CODEPEH (Commission for the Early Detection of Childhood Deafness), with the aim of making recommendations in the form of a review paper and expert opinion, analyses childhood deafness with an additional disability, as well as disability with associated deafness, the effect that both entities (AD+) have on language development, and strategies for their early identification and treatment, to bridge the gap between children in these situations and deaf children without an added disability.Incidence of AD+ in different clinical contextsDiagnosed deafness and other associated disabilitiesData published in a 2017 global morbidity study on the prevalence of childhood epilepsy, intellectual disability, and sight and/or hearing loss showed that globally, 11.2% of children and adolescents had one of these four disabilities. Their prevalence increased with age, from 6.1% among one-year-olds to 13.9% among adolescents aged 15−19. Although 94.5% lived in low- and middle-income countries and areas, predominantly South Asia and sub-Saharan Africa, this problem is not unknown in developed countries.<a class="elsevierStyleCrossRef" href="#bib0045">9</a>The most common disability associated with hearing loss is intellectual disability (ID), which occurs in 8.3% of these children.<a class="elsevierStyleCrossRefs" href="#bib0025">5,10</a> Intellectual ability is an important factor in determining better levels of language development in deaf children with hearing aids and cochlear implantation.<a class="elsevierStyleCrossRef" href="#bib0055">11</a>It has also been shown that the global burden of developmental disabilities has not improved significantly since 1990, suggesting inadequate attention to the developmental potential of children.Within the process for assessing congenital hearing loss, we must consider that approximately 30%–40% of children have an additional disability (mainly cognitive impairment), and about 20% of this population has more than 2. For adequate language development in the case of hearing loss, not only early diagnosis and treatment of the hearing impairment is required, but other non-auditory factors influencing progress must be ruled out. In a Spanish study of children with deafness diagnosed at less than 3 years of age, 22% had psychomotor delay from this early age (one in four to five children).<a class="elsevierStyleCrossRef" href="#bib0060">12</a>Communication and language development is likely to be more delayed in children with hearing loss who have one or more associated disabilities, and therefore it is important to identify this population as the additional disability is usually identified later in children with hearing loss than in those with normal hearing.<a class="elsevierStyleCrossRef" href="#bib0065">13</a>Although not an exhaustive list, the most prominent syndromes that may be associated with deafness are detailed below, differentiating between those that do not affect cognitive skills and those that entail intellectual disability:Entailing intellectual disability (to a greater or lesser degree)Down’s syndrome: The prevalence of hearing loss in Down's syndrome varies from 2% to 78%, because these children usually have chronic otitis media. Nevertheless, with appropriate audiological management, less than 2% have permanent hearing loss.<a class="elsevierStyleCrossRef" href="#bib0070">14</a>CHARGE syndrome: This is the second syndrome affecting both hearing and sight. Complex cardiac defects, swallowing and breathing disorders often make it difficult to assess hearing and sight loss, as well as cognitive delay. Most people with CHARGE syndrome have profound sensorineural or mixed hearing loss and a reduced visual field. The incidence of severe to profound hearing loss varies from 34% to 38%.<a class="elsevierStyleCrossRef" href="#bib0075">15</a> Approximately 50% of children with CHARGE syndrome have preserved intellectual ability, it is impaired in 25%, and severely impaired in 25%.No cognitive impairmentTreacher-Collins Syndrome (TCS): Most of those affected have facial malformations, cleft palate, and bilateral auricular atresia often resulting in conductive hearing loss. TCS does not usually result in cognitive deficits, although the impairments may affect speech and language development.Waardenburg Syndrome (WS): This is a group of genetic defects inherited in an autosomal dominant fashion, which can also cause hearing loss and changes in hair, skin, and eye pigmentation.<a class="elsevierStyleCrossRef" href="#bib0080">16</a> There is no evidence of a link between WS and cognitive deficits.Usher Syndrome (US): This is the most common disease affecting both hearing and vision, with 3 clinical subtypes. It occurs in 3%–6% of children with congenital deafness, and in up to 50% of the deaf-blind population.<a class="elsevierStyleCrossRef" href="#bib0085">17</a> Any child with severe-profound bilateral sensorineural hearing loss should have an ophthalmological evaluation to rule out Usher syndrome.<a class="elsevierStyleCrossRef" href="#bib0090">18</a> Children with type 1 US have absent or limited vestibular function, and therefore the mean age that they start walking is 23 months.<a class="elsevierStyleCrossRef" href="#bib0095">19</a> The prevalence of intellectual disability in children with US is unknown, and existing evidence is contradictory. One study shows that 15% of the sample studied had some degree of intellectual deficit,<a class="elsevierStyleCrossRef" href="#bib0100">20</a> whereas another study found similar levels of cognitive development when comparing deaf children with US and cochlear implants with deaf children without the syndrome with hearing aids.<a class="elsevierStyleCrossRef" href="#bib0105">21</a>DeafblindnessAs seen above, among associations with disabilities, deafblindness stands out as the result of the combination of 2 sensory impairments (visual and hearing), and causes unique communication problems and special needs, essentially due to the difficulty to perceive globally, know, be interested, and to function in the environment. Deafblindness should be considered a distinct disability, requiring specialised services.<a class="elsevierStyleCrossRef" href="#bib0110">22</a> The most common causes of deafblindness are usually hereditary syndromes and complications of prematurity. Early detection is critical to provide visual and hearing aids and optimise development.OtherThere are many other syndromes or entities with associated deafblindness. Among others, a significant number of cases with profound hearing loss who also have autism spectrum disorder have been documented. This proportion is not the same the other way round (ASD with suspected hearing loss).Diagnosed disability with suspected hearing lossAs mentioned above, there is much less information on the presence of hearing loss in individuals diagnosed with a disability or autism spectrum disorder (ASD). Children and young people with an intellectual disability are also at high risk for other disabilities. Intellectual disabilities have been found to be more strongly associated with sensory disabilities and/or physical disabilities, but it has also been shown that individuals diagnosed with ASD are independently associated with intellectual disability. The combination of intellectual disability and/or ASD with a hearing impairment alters communication more than expected, which means that these cases are more complex to assess and treat.<a class="elsevierStyleCrossRef" href="#bib0115">23</a> In ASD, the risk of associated hearing loss is five times higher, visual impairment 8 times higher, and intellectual disability almost 50 times higher, and therefore it is important that professionals are made aware of the high degree of comorbidity of this disorder and to consider accurate prevalence data to plan prevention and intervention measures.<a class="elsevierStyleCrossRef" href="#bib0120">24</a>There are also other disorders commonly associated with cognitive delay, such as global developmental delay (GDD) and cerebral palsy (CP), which are more prone to hearing loss, with a prevalence between 4% and 39% in the case of CP, exacerbating delay in the development of speech, language and cognitive function.Unidentified hearing loss in individuals with AD+ due to intellectual disability (ID) can significantly worsen their quality of life, and therefore it is crucial to identify hearing loss early to establish immediate care and treatment. Assessment of hearing in individuals with ID may require more time and better preparation for techniques such as auditory evoked potential testing to be used. The impact of hearing loss in combination with ID can vary, depending on the type and degree of hearing loss, as well as the age at which it presents.<a class="elsevierStyleCrossRef" href="#bib0125">25</a> Many children with cognitive delay have a history of prematurity, ranging from 26% to 83%. Prematurity is a known risk factor for ASD and CP, and is also a risk factor for hearing loss with a frequency between 1% and 19%.<a class="elsevierStyleCrossRef" href="#bib0130">26</a>The association of deafness and intellectual disability may also be a consequence of congenital or acquired infection, especially in the meningoencephalic area, with multiple repercussions on development, especially congenital cytomegalovirus (CMV) infection, which is one of the most frequent (up to one case per 200 newborns).<a class="elsevierStyleCrossRef" href="#bib0135">27</a> Congenital CMV often combines disabilities associated with sensorineural hearing loss. In a recent review, the prevalence of hearing loss at birth was over 33% among symptomatic infected infants and less than 15% in asymptomatic infections. This difference in prevalence was maintained throughout childhood with more than 40% prevalence detected for symptomatic CMV and less than 30% for asymptomatic. Late-onset and progressive hearing loss appears to be characteristic of this congenital infection. In terms of associated disabilities, a high incidence of developmental delays (81%), microcephaly (93%), seizures (33%), neonatal encephalopathy (10%), and ocular abnormalities (14%) has been reported, which may result in a poorer overall developmental prognosis.Risk factors and warning signs in the child with AD+Early detection and early treatment of hearing impairment with the currently available technological advances have improved access to sound for children with hearing impairment and prevented delayed oral language development. There is, therefore, a new trajectory for these children who have benefited from these advances that clinicians need to be aware of so that they can suspect and detect when progress in language acquisition and development is not adequate and intervene appropriately.<a class="elsevierStyleCrossRef" href="#bib0030">6</a> It is important to note that not all children will respond equally to traditional therapeutic strategies for language development (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). When it is wrongly assumed that developmental delay is exclusively due to hearing loss, the opportunity is lost to intervene early and appropriately in associated disabilities and thus improve communication skills, among other developmental milestones, in the long term. Thus, in the field of early care and educational intervention with children with deafness, the next step should be to concentrate efforts to minimise the disparity in the level of language and communication development of this large group of children.<elsevierMultimedia ident="tbl0005"></elsevierMultimedia>Risk factors for associated disabilitiesAmong developmental disabilities, a number of factors can be identified that indicate an increased risk of adding another disability to a hearing impairment. These risk factors can be classified as prenatal, perinatal, and postnatal. Recognising these risk factors helps to alert families and to ensure that these children’s needs are met early to promote the maximum development of their abilities, as shown in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>.<a class="elsevierStyleCrossRef" href="#bib0140">28</a><elsevierMultimedia ident="tbl0010"></elsevierMultimedia>Warning signs of developmental delay in a child with hearing lossAlthough the risk factors listed in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a> can help in identifying children with different learning needs, not all children necessarily have one of these identifiable risk factors. This is important to remember, particularly in cases where there is a known cause of deafness that a priori does not lead to developmental problems. Indeed, having an identified cause, e.g. genetic, does not protect the child from the influence of other genes or other causes on their development. Thus, clinicians, speech therapists, educators, and teachers must be vigilant to detect atypical learning that may indicate that a child with hearing loss is not progressing appropriately:Motor developmentChildren with hearing loss generally have typical motor development.<a class="elsevierStyleCrossRef" href="#bib0145">29</a> If this is not the case, vestibular problems, vision problems, or the presence of cerebral palsy, or myopathies of various kinds should be ruled out. Warning signs to suspect a motor developmental delay or disorder include poor head and trunk posture control, failure to walk by 15 months of age, frequent falls, failure to develop dominant hand preference by 2.5 years of age, or an immature grasp and release pattern.Visual impairmentChildren diagnosed with deafness are at increased risk of visual impairment, as mentioned above, because certain syndromes or diseases that cause hearing loss also have an impact on vision. In children with deafness, a visual disorder must always be ruled out, especially in cases diagnosed with a syndrome (Usher, CHARGE, Waardenburg, etc.), with congenital infections causing retinopathy (CMV, retinopathy of prematurity) or with brain damage such as that caused by infantile cerebral palsy. Warning signs are: lack of gaze fixation, poor eye tracking, oscillating eye movement, wandering eye, cephalic tilt, stumbling over objects, poor night vision, and difficult acclimatisation from dark to light, or vice versa.Learning difficultiesAlthough deafness has been excluded from consideration as a specific learning disability, it is common for children with deafness to have some learning difficulties or a specific disorder. It is difficult for professionals to separate the impact of deafness and the specific learning disability itself on these children's academic performance. Professionals with a good understanding of the differences between the two entities can plan appropriate intervention.Communication disordersIt is also difficult to determine whether there is a specific language disorder, beyond the anticipated impact of deafness on language development. It is clear that specific communication disorders and autistic spectrum disorder can co-exist with deafness in a child. In addition, some children appear to have significant difficulties in language processing that would not be explained by their hearing impairment alone. Receptive language skills are much more delayed in these children than would be expected based on their cognitive potential and ability to learn. This pattern is easier to recognise in cases with mild to moderate hearing loss, and therefore early identification enables the most appropriate educational support and intervention.Intellectual disabilityCognitive skills can have an impact on children's progress in all aspects of their development.<a class="elsevierStyleCrossRef" href="#bib0150">30</a> Although intellectual ability is not the only factor affecting language development, it is highly correlated with language outcomes, and therefore reliable assessment of cognitive abilities (typically determined by non-verbal cognitive measures) can help provide a better structure for identifying whether a child is making the progress that would have been anticipated over time.The algorithm described in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a> for the diagnostic and therapeutic approach to childhood deafness with AD+ can guide professionals, knowing the individual and learning characteristics of the child, to act step by step to determine intervention needs adapted to each case.<elsevierMultimedia ident="fig0005"></elsevierMultimedia>Language development in AD+The development of oral language, receptive and expressive, in children with deafness treated with hearing aids and/or implants is determined, among other factors, by the severity of the hearing loss, the time of onset, the age of the hearing aid fitting, cognitive development, and the communicative model used in the early intervention. There are 2 other variables that behave differently in children fitted with a hearing aid and implanted children. On the one hand, the level of maternal education which, in any case, favours speech and language development, especially in children fitted with a hearing aid. On the other hand, the presence of a disability in addition to deafness negatively affects development, especially in the case of implanted AD+ children.<a class="elsevierStyleCrossRef" href="#bib0010">2</a> Previous studies have described the presence of a disability in addition to hearing loss as one of the most reliable predictors of speech and language development in children. In this regard, in a study by Cupples, most of the children were diagnosed with autism spectrum disorder, cerebral palsy, and global developmental delay, disabilities that have greater impact on speech and language development than others.<a class="elsevierStyleCrossRef" href="#bib0155">31</a> Therefore, the disabilities that are most frequently added to hearing impairment have been classified into 2 groups according to their impact on speech and language development (<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>). The first group identifies the disabilities with the greatest impact (ASD, cerebral palsy, ASD associated with cerebral palsy, global developmental delay associated with another syndrome, and isolated global developmental delay). In the second group are vision disorders, speech and phono-articulatory disorders, other syndromes without developmental delay, and various medical and health problems. Research in 3-year-old children found that children with autism, cerebral palsy, and/or developmental delay score significantly worse in language development than children with other disabilities (such as visual impairments, speech and language disorders, syndromes without developmental delay, and diseases). Children with a disability in addition to a hearing impairment achieve worse levels of language development than children without a hearing impairment; they are typically one to two standard deviations (SD) below normal hearing children of the same age. In contrast, non-verbal cognitive ability is approximately .3 SD below the typical mean.<a class="elsevierStyleCrossRef" href="#bib0160">32</a> The literature highlights variability in language development in implanted children with an associated disability. The different cognitive ability of each child or group of children may be a factor explaining this variability. Many studies indicate that better language development is related to better levels of cognitive development,<a class="elsevierStyleCrossRef" href="#bib0010">2</a> although other factors may also be found to explain this: non-verbal skills, less profound degree of hearing loss, use of oral language in early intervention, higher educational level of the mother, and early fitting of hearing aids or cochlear implants.<elsevierMultimedia ident="tbl0015"></elsevierMultimedia>Adaptation of infant hearing impairment programmes for intervention with children with AD+Widespread neonatal hearing screening and early intervention programmes have significantly increased the detection of children who are deaf and hard of hearing. As demographics have changed, the educational complexity and service needs for this population have also increased. Technological advances have improved access to sound and language development, but at the same time there are gaps in the skills of staff providing services to this diverse group of children.<a class="elsevierStyleCrossRef" href="#bib0035">7</a>Diagnosis of a disability in addition to deafnessThe first challenge for a screening and early treatment programme for childhood hearing loss is to detect or recognise deaf children with an additional disability. Identifying additional disabilities as early as possible is essential to ensure that these children have access to appropriate intervention, and thus achieve the maximum possible development in each case. As screening and early care make language delays in children with a hearing impairment increasingly rare, the diagnosis of other disabilities, such as ASD in a child with deafness, may seem easier than in the past. The recommendations of the Joint Committee on Infant Hearing (JCIH) and CODEPEH state that all children with hearing loss under the age of 3 years should undergo six-monthly assessment of their speech, language, and cognitive skills,<a class="elsevierStyleCrossRef" href="#bib0165">33</a> which enables detection of cases where there are delays or deviations from milestones and expected outcomes.Diagnosing hearing loss in severe developmental disordersAudiometric assessment to rule out a hearing deficit in a child with a disability can be a major challenge. In fact, many children undergo multiple attempts at behavioural audiometry without success. The impossibility of determining auditory thresholds in this context means that sedation is often necessary for objective audiological testing. While it is always desirable to minimise sedation or anaesthesia, repeated unsuccessful attempts at behavioural audiometry can cause diagnostic delays, resulting in increased financial cost and concern for families. Patients with cognitive delay, autism spectrum disorder, global developmental delay, or cerebral palsy are at the highest risk of developing additional hearing loss, even if they have passed neonatal screening tests for hearing loss.<a class="elsevierStyleCrossRef" href="#bib0130">26</a> They should therefore be more closely monitored to rule out an additional hearing loss.<a class="elsevierStyleCrossRef" href="#bib0165">33</a> Given the great benefit to be gained from early intervention for hearing loss in addition to a cognitive or developmental disorder, it is imperative to avoid diagnostic delays, which some studies report can be as long as two years (2–3 failed attempts at behavioural audiometry on average before auditory evoked potential testing).<a class="elsevierStyleCrossRef" href="#bib0130">26</a> This evidence supports the early indication for objective audiological testing to avoid delays in diagnosis, using sedation or general anaesthesia if necessary. It is important to remember that some of the commonly used audiological tests do not confirm normal central auditory function.<a class="elsevierStyleCrossRef" href="#bib0170">34</a> There is some tendency to report that hearing is adequate to ensure speech and language development based on the normality of these tests: however, we must remember that there are central auditory processing problems that prevent adequate development. Children and adults with ASD describe difficulties in listening in noise, in maintaining attention to auditory stimuli, and in using auditory information in sensory stressful situations,<a class="elsevierStyleCrossRef" href="#bib0175">35</a> which may indicate a central auditory processing disorder.Recommended audiological treatment in cases of AD+Once hearing loss is diagnosed in a child with AD+and a hearing aid fitting is indicated, hearing aids should be selected and fitted using a prescriptive approach that considers the acoustics of the auditory canals and thresholds. If behavioural thresholds are unreliable, hearing aid programming should be adjusted based on the electrophysiological results of steady state evoked potential testing, and then the fitting corrected as information on behavioural thresholds is obtained in subsequent assessments.<a class="elsevierStyleCrossRef" href="#bib0180">36</a> The goal of any hearing aid fitting is to provide the child with access to acoustic speech information without the hearing aid exceeding the recommended amplification limits. Children with ASD are no different in terms of this goal, but it should be noted that some children with ASD experience increased loudness perception and may exhibit behaviour consistent with an exaggerated response to auditory stimuli,<a class="elsevierStyleCrossRef" href="#bib0185">37</a> therefore it may be necessary to limit the levels of prosthetic stimulation to help acceptance of the new device. Another characteristic of these children is poor tolerance to the tactile sensation of contact with other people, which may make it difficult to measure real-ear to coupler differences (RECD) or to take impressions for earmoulds for the hearing aid fitting, and therefore it would not be unusual to sedate the child for this purpose.<a class="elsevierStyleCrossRef" href="#bib0190">38</a> As with any child fitting, access to the hearing aid batteries should be blocked and the volume and programming control buttons should be disabled. With regard to cochlear implantation in children with ASD, improvements have been found in social communication, behaviour, attention to surroundings, increased vocalisation, eye contact, and reaction to music.<a class="elsevierStyleCrossRef" href="#bib0195">39</a> Comparing children with ASD and children with various additional disabilities, it has been observed that children with ASD have limited development of auditory perception. However, cochlear implantation is not contraindicated in children with ASD, provided that parents are informed that successful implantation cannot be predicted and it is important to manage expectations. In these cases, an experienced team is required, who are willing to consult with ASD experts.<a class="elsevierStyleCrossRef" href="#bib0200">40</a>Hearing loss is a symptom to be aware of as it is frequently observed in children with ASD. It consists of a pattern of exaggerated behavioural reactions to auditory sensory stimuli. Although its presence is not universal, it is so frequent in these cases that a differential diagnosis of ASD needs to be made. Psychoacoustic tests have been used to measure the perception of acoustic intensity in children with ASD and it has been observed that they present a narrow dynamic range and a reduced tolerance to it, which is consistent with the hearing loss observed in this group.<a class="elsevierStyleCrossRef" href="#bib0205">41</a> The prevalence of this symptom in children with ASD is not universal, but it is so frequent that it is necessary to establish a differential diagnosis of ASD. The prevalence of this symptom varies from 18% to 53% in children with ASD, while it is not found in typically developing children. The presence of hearing loss may be due to certain characteristics of the subject such as mental age, because it diminishes with increasing mental age. It is therefore considered to be a general deficit associated with developmental disabilities and is not specific to children with ASD.<a class="elsevierStyleCrossRef" href="#bib0200">40</a>Cochlear implantation has very diverse results. In particular, a review of several studies on cochlear implants in children with WS indicates that they develop similar abilities to those implanted for typical sensorineural deafness. There are several studies on the improvement in both hearing ability and speech intelligibility after cochlear implantation in children with WS.<a class="elsevierStyleCrossRef" href="#bib0210">42</a> Also results in CHARGE patients showed that the majority continued to have mild hearing loss; however, improvement was seen in the parents’ perception of auditory responsiveness. Most of the children used gestural communication and were in special education classes.Early intervention in children with AD+Another challenge for a child hearing impairment care programme is to plan the services made available to each child with AD+. The approach to these cases must be based on the individual needs of the children, avoiding categorising or labelling their disability. Thus, if each case is considered unique, it will be possible to enhance their strengths and provide responses tailored to their individual needs. This person-based approach allows families to play an important role in designing the care for their children. AD+ children, if not detected early and treated appropriately, risk missing out on the critical learning period and consolidating a developmental gap compared to deaf children without an additional disability.<a class="elsevierStyleCrossRef" href="#bib0215">43</a> Early intervention requires interdisciplinary teams to care for children with AD+, involving multiple service providers incorporating teachers, speech therapists, psychologists, educationalists, and audiologists, and may extend to other professions such as physiotherapists, and requires the use of assistive products and technologies according to the needs of the child. Although early childhood professionals see collaboration with families and other professionals as an important aspect of their work, interdisciplinary collaboration is often a challenge. This may be due to poor preparation for this cooperative work, as collaboration is a subtle and very complex field that requires explicit instructions for professionals. A continuum of collaborative models has been used to provide services to children with special educational needs: multidisciplinary, interdisciplinary, and transdisciplinary. The multidisciplinary model involves professionals working with children separately. The results of this model are fragmented services and even conflicting activities and recommendations.<a class="elsevierStyleCrossRef" href="#bib0220">44</a> If the interdisciplinary model is used, professionals communicate with each other and make decisions by consensus, but assessments and implementation tend to take place separately. This model does not allow professionals to fully coordinate their activities and can result in a child having to work with multiple professionals, which can be difficult for young children. The transdisciplinary model includes not only communication between professionals and families, but also transfers skills from one team member to another.<a class="elsevierStyleCrossRef" href="#bib0225">45</a> Transdisciplinary collaboration is also known as the indirect therapy model and is characterised by a planned release of roles, in which roles and responsibilities are shared and exchanged between team members. This model enables fewer team members providing services directly, while other team members act as consultants. This approach may be less stressful for young children. However, the most effective programming for children with AD+ should be highly flexible and individualised, with early intervention services focused on the child and family. A transdisciplinary approach enables coordinated services based on input from a team of professionals with diverse experience and expertise.Care and support for families of children with AD+The actions that determine the effectiveness of treatment and intervention with a child with a disability, include offering global care and, as has been said, care centred on the child and their family, avoiding fragmented, decontextualised, and uncoordinated responses, giving confidence and security to families, reducing their disorientation having to wander through various specialists and services, and starting them as early as possible.<a class="elsevierStyleCrossRef" href="#bib0230">46</a> Coming to terms with the news and the lack of information are, in turn, two of the main difficulties faced by families in the first moments after learning the diagnosis. In the case of children with AD+, this becomes even more important as, often, the diagnoses overlap or occur over time, which causes families greater emotional stress and interferes in their acceptance of the situation and decision-making. Therefore, families must be able to count on stable teams and structures with specialist, qualified professionals able to deal with the individual case and based on analysis of the specific socio-familial situation. The role of the professionals involved at each level is fundamental, especially in handling a new diagnosis and, in general, in conveying all the information to families, hence the importance of training programmes aimed at capacity building for effective teamwork.<a class="elsevierStyleCrossRef" href="#bib0165">33</a> The support needs of families have been shown to go beyond receiving information on health issues or hearing technology to the emotional. Different studies have collected information about the needs of families, some to do with the way in which information is conveyed to them, the lack of coordination between professionals during the different stages of the screening programme, partial information about treatment, and difficulty in referral to appropriate services, and lack of awareness of available resources.33 Therefore, a transdisciplinary team, co-responsible and working in collaboration with the family, is essential at all levels of case management, especially in the case of AD+. This team must provide quality, complete, understandable, objective, and truthful information to manage expectations about prognosis. It should cover medical diagnosis, treatment, use of hearing aids, early intervention, and speech therapy options, as well as referral to support structures and resources for families. Family support programmes should be integrated as a supportive and cooperative element within the team.<a class="elsevierStyleCrossRefs" href="#bib0090">18,33</a> The message must be coherent and consistent among the different professionals involved. It is also important to sequence and group consultations close together in time, coordinating care and the provision of information between them, respecting current regulations in relation to personal data protection. The family should not be responsible for conveying information between professionals nor be the mediator of its coordination, and it is advisable to control the amount of information offered, the time and setting for communication. Inter-administrative and inter-sectorial coordination is also necessary, with simplified and coordinated procedures for access to all services and benefits for the child and their family. Referral and care pathways must be established for both the child and their family, which are coordinated with each other and ensure the continuity of the process.<a class="elsevierStyleCrossRef" href="#bib0230">46</a> The family should receive information, help and advice; their collaboration and intervention is indispensable and irreplaceable, because the effectiveness and/or the best outcome of treatment largely depends on them.Key points and recommendations from CODEPEH<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005">1More than 40% of children with deafness have an added developmental disorder and/or a major medical problem which, in addition to delaying the age of diagnosis in many cases, may require the intervention of other specialist professionals.</li><li class="elsevierStyleListItem" id="lsti0010">2Early detection of this association is vital and therefore global developmental assessment every 6 months in every child with deafness is extremely important. Similarly, associated deafness should be ruled out in children with a diagnosed disability who do not reach expected developmental milestones.</li><li class="elsevierStyleListItem" id="lsti0015">3The situation of these children is termed in the literature "deaf or hard of hearing PLUS", but the term "deafness with additional disability (AD+)" is preferable.</li><li class="elsevierStyleListItem" id="lsti0020">4Identifying AD+ allows for early and appropriate intervention which will be reflected, among other areas, in improved language and communication skills. The relevant audiological tests need to be conducted as soon as possible, even with sedation if necessary.</li><li class="elsevierStyleListItem" id="lsti0025">5Early care should include transdisciplinary teams to care for children with AD+, using an approach based on the individual needs of the child and in collaboration with the family.</li></ul>Conflict of interestsThe authors have no conflict of interests to declare." "textoCompletoSecciones" => array:1 [ "secciones" => array:12 [ 0 => array:3 [ "identificador" => "xres2023503" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1732283" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres2023504" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1732282" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:3 [ "identificador" => "sec0010" "titulo" => "Incidence of AD+ in different clinical contexts" "secciones" => array:2 [ 0 => array:3 [ "identificador" => "sec0015" "titulo" => "Diagnosed deafness and other associated disabilities" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Entailing intellectual disability (to a greater or lesser degree)" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "No cognitive impairment" ] 2 => array:2 [ "identificador" => "sec0030" "titulo" => "Deafblindness" ] 3 => array:2 [ "identificador" => "sec0035" "titulo" => "Other" ] ] ] 1 => array:2 [ "identificador" => "sec0040" "titulo" => "Diagnosed disability with suspected hearing loss" ] ] ] 6 => array:3 [ "identificador" => "sec0045" "titulo" => "Risk factors and warning signs in the child with AD+" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0050" "titulo" => "Risk factors for associated disabilities" ] 1 => array:3 [ "identificador" => "sec0055" "titulo" => "Warning signs of developmental delay in a child with hearing loss" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0060" "titulo" => "Motor development" ] 1 => array:2 [ "identificador" => "sec0065" "titulo" => "Visual impairment" ] 2 => array:2 [ "identificador" => "sec0070" "titulo" => "Learning difficulties" ] 3 => array:2 [ "identificador" => "sec0075" "titulo" => "Communication disorders" ] 4 => array:2 [ "identificador" => "sec0080" "titulo" => "Intellectual disability" ] ] ] ] ] 7 => array:2 [ "identificador" => "sec0085" "titulo" => "Language development in AD+" ] 8 => array:3 [ "identificador" => "sec0090" "titulo" => "Adaptation of infant hearing impairment programmes for intervention with children with AD+" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0095" "titulo" => "Diagnosis of a disability in addition to deafness" ] 1 => array:2 [ "identificador" => "sec0100" "titulo" => "Diagnosing hearing loss in severe developmental disorders" ] 2 => array:2 [ "identificador" => "sec0105" "titulo" => "Recommended audiological treatment in cases of AD+" ] 3 => array:2 [ "identificador" => "sec0110" "titulo" => "Early intervention in children with AD+" ] 4 => array:2 [ "identificador" => "sec0115" "titulo" => "Care and support for families of children with AD+" ] ] ] 9 => array:2 [ "identificador" => "sec0120" "titulo" => "Key points and recommendations from CODEPEH" ] 10 => array:2 [ "identificador" => "sec0125" "titulo" => "Conflict of interests" ] 11 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-07-27" "fechaAceptado" => "2022-10-10" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1732283" "palabras" => array:7 [ 0 => "Deaf" 1 => "Deaf with additional disabilities" 2 => "Language development" 3 => "Development delay" 4 => "Disabilities" 5 => "Early intervention" 6 => "Trans-disciplinary" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1732282" "palabras" => array:7 [ 0 => "Sordera" 1 => "Discapacidad añadida" 2 => "Desarrollo del lenguaje" 3 => "Trastorno del desarrollo" 4 => "Discapacidades" 5 => "Intervención temprana" 6 => "Transdisciplinariedad" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "Approximately 40% of children with deafness have an additional developmental disorder or major medical problem, which may delay the age of diagnosis of hearing loss and/or require intervention by other professionals. This situation is referred to as “deafness with added disability” (AD+). The reason why the population of hearing-impaired children is more likely to have associated added disabilities is that the risk factors for hearing impairment overlap with those for many other disabilities. These factors can influence various aspects of development, including language acquisition. It is important to check that appropriate care is received, the effectiveness of hearing aids or implants, as well speech therapy intervention strategies, and family adherence to sessions and appointments. The challenge posed by AD+ is early detection, to allow early and appropriate intervention, and the need for fluid transdisciplinary collaboration between all professionals involved, together with the involvement of the family." ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "Aproximadamente el 40% de los niños con sordera tienen añadido un trastorno del desarrollo o un problema médico importante, que puede retrasar la edad de diagnóstico de la hipoacusia y/o precisar de la intervención de otros profesionales. Esta situación se designa como “hipoacusia o sordera con discapacidad añadida” (DA+). El motivo por el que la población de niños con problemas auditivos es más propensa a asociar discapacidades añadidas radica en que los factores de riesgo para la hipoacusia se superponen con los de muchas otras discapacidades. Estos factores pueden influir en diversos aspectos del desarrollo, incluida la adquisición del lenguaje. Es importante comprobar que se recibe la adecuada atención, la efectividad de audífonos o implantes, así como de las estrategias de intervención logopédica, y la adherencia de la familia a sesiones y citas. Los desafíos que plantea la DA+son su detección precoz, para permitir una temprana y adecuada intervención, y la necesidad de una colaboración transdisciplinar fluida entre todos los profesionales que han de intervenir, junto con la implicación de la familia." ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "CODEPEH papers are undertaken within the framework of a collaboration agreement between the Real Patronato sobre Discapacidad (Royal Board on Disability) and FIAPAS." ] ] "multimedia" => array:4 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 4191 "Ancho" => 2925 "Tamanyo" => 1008272 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0170" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "Diagnostic and therapeutic approach to hearing loss with added disability recommended by CODEPEH." ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0175" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Type of disability \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Population with hearing loss \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">General population \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No added disability \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">60% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">86% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Intellectual disability \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9.8% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2.5% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cerebral palsy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4%–39% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">.3% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visual problems \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3.9% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">.03% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Attention deficit/hyperactivity disorder \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6.6% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5%–10% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Autism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1%−4% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Specific learning disability \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5−10% \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Other \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12% \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3359374.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Incidence of disabilities associated with hearing loss." ] ] 2 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0180" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "AD+: Childhood deafness with additional disability." "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Risk factors for AD+ \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Prenatal risk factors \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Exposure to toxic substances such as alcohol and lead \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Obstetric factors such as: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Placental insufficiency \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Twin pregnancy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Gestational hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Gestational diabetes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Infections such as cytomegalovirus \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Genetic factors such as a syndrome or family history of learning difficulties \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Atypical embryonic development such as spina bifida or brain anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Perinatal risk factors \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Acute perinatal hypoxia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Prematurity \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Perinatal infections (group B streptococcal infection) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hyperbilirubinaemia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Postnatal risk factors \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Environmental exposure to tobacco smoke or lead \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Malnutrition \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Infections such as meningitis or encephalitis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Complex medical problems such as heart disease, visual problems, or epilepsy. Traumatic brain injury \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Physical or emotional abuse \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Inappropriate environmental experiences \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3359376.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Risk factors for AD." ] ] 3 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0185" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Type of disability \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Group A: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Autism \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Childhood cerebral palsy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Developmental delay \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Developmental delay+other non-autism, non-cerebral palsy syndrome \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Group B: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visual disorders \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Speech disorders \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Other syndromes not involving developmental delay \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Medical conditions \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3359375.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Disabilities frequently associated with hearing loss." ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:46 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Language ability after early detection of permanent childhood hearing impairment" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C.R. Kennedy" 1 => "D.C. McCann" 2 => "M.J. Campbell" 3 => "C.M. Law" 4 => "M. Mullee" 5 => "S. Petrou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1056/NEJMoa054915" "Revista" => array:6 [ "tituloSerie" => "N Engl J Med" "fecha" => "2006" "volumen" => "354" "paginaInicial" => "2131" "paginaFinal" => "2141" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16707750" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Spoken language and everyday functioning in 5-year-old children using hearing aids or cochlear implants" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Cupples" 1 => "T.Y. Ching" 2 => "L. Button" 3 => "M. Seeto" 4 => "V. Zhang" 5 => "J. 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Deaf children with additional disabilities (AD+): CODEPEH recommendations

Sordera infantil con discapacidad asociada (DA+): recomendaciones CODEPEH

Faustino Núñez-Batallaa,

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fnunezb@telefonica.net

Corresponding author.

, Carmen Jáudenes-Casaubónb, Jose Miguel Sequí-Canetb, Ana Vivanco-Allendeb, Jose Zubicaray-Ugartecheb

a Presidente de la Comisión para la Detección Precoz de la Hipoacusia (CODEPEH)

b Vocales de la CODEPEH

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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Neonatal hearing screening programmes have led to significant advances in the early identification and treatment of children with hearing loss&#44; resulting in improved speech and language development&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Although the audiometric threshold is an important factor influencing the deaf child&#8217;s language development&#44; it is not the only one&#46; There are other variables to consider such as gender&#44; cognitive abilities&#44; presence of an additional disability&#44; educational or socio-economic level of the parents&#44; age at which the child was diagnosed and treated audiologically&#44; type of hearing device chosen&#44; quality of the fitting and the appropriate use of the device&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Between 40&#37; and 49&#37; of deaf children have an additional developmental disorder or major medical problem which&#44; in addition to in many cases delaying the age at which hearing loss is diagnosed&#44; requires the intervention of other specialists&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5</span></a> These children are known in the literature as &#34;deaf or hard of hearing PLUS&#34;&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> although it seems more appropriate in Spanish to use the term &#34;hypoacusis or deafness and added or associated disability&#34;&#44; which applies both to diagnosed deafness with other associated disorders and to diagnosed disabilities with associated deafness&#46; Children with a hearing impairment with an associated disability are increasing considerably in number and diversity&#44; posing a major challenge for early detection and diagnosis&#44; and early intervention programmes for childhood hearing loss&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> The population of hearing-impaired children is more likely to have associated disabilities because risk factors for hearing loss<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> overlap with risk factors for many other disabilities&#44; such as the presence of certain genetic syndromes&#44; prematurity&#44; congenital infections&#44; and meningitis&#46; These factors can affect various aspects of development as well as language acquisition&#46; Early detection is a challenges posed by the association of a disability with hearing impairment&#44; therefore 6-monthly assessment of speech and language development in every child with deafness is extremely important&#44; because identifying a disability in addition to deafness enables early and appropriate intervention&#46; Another challenge is the need for smooth and coordinated transdisciplinary collaboration between all professionals involved&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">In this article&#44; CODEPEH &#40;Commission for the Early Detection of Childhood Deafness&#41;&#44; with the aim of making recommendations in the form of a review paper and expert opinion&#44; analyses childhood deafness with an additional disability&#44; as well as disability with associated deafness&#44; the effect that both entities &#40;AD&#43;&#41; have on language development&#44; and strategies for their early identification and treatment&#44; to bridge the gap between children in these situations and deaf children without an added disability&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Incidence of AD&#43; in different clinical contexts</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Diagnosed deafness and other associated disabilities</span><p id="par0015" class="elsevierStylePara elsevierViewall">Data published in a 2017 global morbidity study on the prevalence of childhood epilepsy&#44; intellectual disability&#44; and sight and&#47;or hearing loss showed that globally&#44; 11&#46;2&#37; of children and adolescents had one of these four disabilities&#46; Their prevalence increased with age&#44; from 6&#46;1&#37; among one-year-olds to 13&#46;9&#37; among adolescents aged 15&#8722;19&#46; Although 94&#46;5&#37; lived in low- and middle-income countries and areas&#44; predominantly South Asia and sub-Saharan Africa&#44; this problem is not unknown in developed countries&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The most common disability associated with hearing loss is intellectual disability &#40;ID&#41;&#44; which occurs in 8&#46;3&#37; of these children&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;10</span></a> Intellectual ability is an important factor in determining better levels of language development in deaf children with hearing aids and cochlear implantation&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">It has also been shown that the global burden of developmental disabilities has not improved significantly since 1990&#44; suggesting inadequate attention to the developmental potential of children&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Within the process for assessing congenital hearing loss&#44; we must consider that approximately 30&#37;&#8211;40&#37; of children have an additional disability &#40;mainly cognitive impairment&#41;&#44; and about 20&#37; of this population has more than 2&#46; For adequate language development in the case of hearing loss&#44; not only early diagnosis and treatment of the hearing impairment is required&#44; but other non-auditory factors influencing progress must be ruled out&#46; In a Spanish study of children with deafness diagnosed at less than 3 years of age&#44; 22&#37; had psychomotor delay from this early age &#40;one in four to five children&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Communication and language development is likely to be more delayed in children with hearing loss who have one or more associated disabilities&#44; and therefore it is important to identify this population as the additional disability is usually identified later in children with hearing loss than in those with normal hearing&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Although not an exhaustive list&#44; the most prominent syndromes that may be associated with deafness are detailed below&#44; differentiating between those that do not affect cognitive skills and those that entail intellectual disability&#58;</p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Entailing intellectual disability &#40;to a greater or lesser degree&#41;</span><p id="par0045" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Down&#8217;s syndrome&#58;</span> The prevalence of hearing loss in Down&#39;s syndrome varies from 2&#37; to 78&#37;&#44; because these children usually have chronic otitis media&#46; Nevertheless&#44; with appropriate audiological management&#44; less than 2&#37; have permanent hearing loss&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">CHARGE syndrome&#58;</span> This is the second syndrome affecting both hearing and sight&#46; Complex cardiac defects&#44; swallowing and breathing disorders often make it difficult to assess hearing and sight loss&#44; as well as cognitive delay&#46; Most people with CHARGE syndrome have profound sensorineural or mixed hearing loss and a reduced visual field&#46; The incidence of severe to profound hearing loss varies from 34&#37; to 38&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> Approximately 50&#37; of children with CHARGE syndrome have preserved intellectual ability&#44; it is impaired in 25&#37;&#44; and severely impaired in 25&#37;&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">No cognitive impairment</span><p id="par0055" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Treacher-Collins Syndrome &#40;TCS&#41;&#58;</span> Most of those affected have facial malformations&#44; cleft palate&#44; and bilateral auricular atresia often resulting in conductive hearing loss&#46; TCS does not usually result in cognitive deficits&#44; although the impairments may affect speech and language development&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Waardenburg Syndrome &#40;WS&#41;&#58;</span> This is a group of genetic defects inherited in an autosomal dominant fashion&#44; which can also cause hearing loss and changes in hair&#44; skin&#44; and eye pigmentation&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> There is no evidence of a link between WS and cognitive deficits&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Usher Syndrome &#40;US&#41;&#58;</span> This is the most common disease affecting both hearing and vision&#44; with 3 clinical subtypes&#46; It occurs in 3&#37;&#8211;6&#37; of children with congenital deafness&#44; and in up to 50&#37; of the deaf-blind population&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> Any child with severe-profound bilateral sensorineural hearing loss should have an ophthalmological evaluation to rule out Usher syndrome&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> Children with type 1 US have absent or limited vestibular function&#44; and therefore the mean age that they start walking is 23 months&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">19</span></a> The prevalence of intellectual disability in children with US is unknown&#44; and existing evidence is contradictory&#46; One study shows that 15&#37; of the sample studied had some degree of intellectual deficit&#44;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">20</span></a> whereas another study found similar levels of cognitive development when comparing deaf children with US and cochlear implants with deaf children without the syndrome with hearing aids&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">21</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Deafblindness</span><p id="par0070" class="elsevierStylePara elsevierViewall">As seen above&#44; among associations with disabilities&#44; deafblindness stands out as the result of the combination of 2 sensory impairments &#40;visual and hearing&#41;&#44; and causes unique communication problems and special needs&#44; essentially due to the difficulty to perceive globally&#44; know&#44; be interested&#44; and to function in the environment&#46; Deafblindness should be considered a distinct disability&#44; requiring specialised services&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">22</span></a> The most common causes of deafblindness are usually hereditary syndromes and complications of prematurity&#46; Early detection is critical to provide visual and hearing aids and optimise development&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Other</span><p id="par0075" class="elsevierStylePara elsevierViewall">There are many other syndromes or entities with associated deafblindness&#46; Among others&#44; a significant number of cases with profound hearing loss who also have autism spectrum disorder have been documented&#46; This proportion is not the same the other way round &#40;ASD with suspected hearing loss&#41;&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Diagnosed disability with suspected hearing loss</span><p id="par0080" class="elsevierStylePara elsevierViewall">As mentioned above&#44; there is much less information on the presence of hearing loss in individuals diagnosed with a disability or autism spectrum disorder &#40;ASD&#41;&#46; Children and young people with an intellectual disability are also at high risk for other disabilities&#46; Intellectual disabilities have been found to be more strongly associated with sensory disabilities and&#47;or physical disabilities&#44; but it has also been shown that individuals diagnosed with ASD are independently associated with intellectual disability&#46; The combination of intellectual disability and&#47;or ASD with a hearing impairment alters communication more than expected&#44; which means that these cases are more complex to assess and treat&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">23</span></a> In ASD&#44; the risk of associated hearing loss is five times higher&#44; visual impairment 8 times higher&#44; and intellectual disability almost 50 times higher&#44; and therefore it is important that professionals are made aware of the high degree of comorbidity of this disorder and to consider accurate prevalence data to plan prevention and intervention measures&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">24</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">There are also other disorders commonly associated with cognitive delay&#44; such as global developmental delay &#40;GDD&#41; and cerebral palsy &#40;CP&#41;&#44; which are more prone to hearing loss&#44; with a prevalence between 4&#37; and 39&#37; in the case of CP&#44; exacerbating delay in the development of speech&#44; language and cognitive function&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Unidentified hearing loss in individuals with AD&#43; due to intellectual disability &#40;ID&#41; can significantly worsen their quality of life&#44; and therefore it is crucial to identify hearing loss early to establish immediate care and treatment&#46; Assessment of hearing in individuals with ID may require more time and better preparation for techniques such as auditory evoked potential testing to be used&#46; The impact of hearing loss in combination with ID can vary&#44; depending on the type and degree of hearing loss&#44; as well as the age at which it presents&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">25</span></a> Many children with cognitive delay have a history of prematurity&#44; ranging from 26&#37; to 83&#37;&#46; Prematurity is a known risk factor for ASD and CP&#44; and is also a risk factor for hearing loss with a frequency between 1&#37; and 19&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">26</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">The association of deafness and intellectual disability may also be a consequence of congenital or acquired infection&#44; especially in the meningoencephalic area&#44; with multiple repercussions on development&#44; especially congenital cytomegalovirus &#40;CMV&#41; infection&#44; which is one of the most frequent &#40;up to one case per 200 newborns&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">27</span></a> Congenital CMV often combines disabilities associated with sensorineural hearing loss&#46; In a recent review&#44; the prevalence of hearing loss at birth was over 33&#37; among symptomatic infected infants and less than 15&#37; in asymptomatic infections&#46; This difference in prevalence was maintained throughout childhood with more than 40&#37; prevalence detected for symptomatic CMV and less than 30&#37; for asymptomatic&#46; Late-onset and progressive hearing loss appears to be characteristic of this congenital infection&#46; In terms of associated disabilities&#44; a high incidence of developmental delays &#40;81&#37;&#41;&#44; microcephaly &#40;93&#37;&#41;&#44; seizures &#40;33&#37;&#41;&#44; neonatal encephalopathy &#40;10&#37;&#41;&#44; and ocular abnormalities &#40;14&#37;&#41; has been reported&#44; which may result in a poorer overall developmental prognosis&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Risk factors and warning signs in the child with AD&#43;</span><p id="par0100" class="elsevierStylePara elsevierViewall">Early detection and early treatment of hearing impairment with the currently available technological advances have improved access to sound for children with hearing impairment and prevented delayed oral language development&#46; There is&#44; therefore&#44; a new trajectory for these children who have benefited from these advances that clinicians need to be aware of so that they can suspect and detect when progress in language acquisition and development is not adequate and intervene appropriately&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It is important to note that not all children will respond equally to traditional therapeutic strategies for language development &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; When it is wrongly assumed that developmental delay is exclusively due to hearing loss&#44; the opportunity is lost to intervene early and appropriately in associated disabilities and thus improve communication skills&#44; among other developmental milestones&#44; in the long term&#46; Thus&#44; in the field of early care and educational intervention with children with deafness&#44; the next step should be to concentrate efforts to minimise the disparity in the level of language and communication development of this large group of children&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Risk factors for associated disabilities</span><p id="par0105" class="elsevierStylePara elsevierViewall">Among developmental disabilities&#44; a number of factors can be identified that indicate an increased risk of adding another disability to a hearing impairment&#46; These risk factors can be classified as prenatal&#44; perinatal&#44; and postnatal&#46; Recognising these risk factors helps to alert families and to ensure that these children&#8217;s needs are met early to promote the maximum development of their abilities&#44; as shown in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">28</span></a></p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Warning signs of developmental delay in a child with hearing loss</span><p id="par0110" class="elsevierStylePara elsevierViewall">Although the risk factors listed in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a> can help in identifying children with different learning needs&#44; not all children necessarily have one of these identifiable risk factors&#46; This is important to remember&#44; particularly in cases where there is a known cause of deafness that a priori does not lead to developmental problems&#46; Indeed&#44; having an identified cause&#44; e&#46;g&#46; genetic&#44; does not protect the child from the influence of other genes or other causes on their development&#46; Thus&#44; clinicians&#44; speech therapists&#44; educators&#44; and teachers must be vigilant to detect atypical learning that may indicate that a child with hearing loss is not progressing appropriately&#58;</p><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Motor development</span><p id="par0115" class="elsevierStylePara elsevierViewall">Children with hearing loss generally have typical motor development&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">29</span></a> If this is not the case&#44; vestibular problems&#44; vision problems&#44; or the presence of cerebral palsy&#44; or myopathies of various kinds should be ruled out&#46; Warning signs to suspect a motor developmental delay or disorder include poor head and trunk posture control&#44; failure to walk by 15 months of age&#44; frequent falls&#44; failure to develop dominant hand preference by 2&#46;5 years of age&#44; or an immature grasp and release pattern&#46;</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Visual impairment</span><p id="par0120" class="elsevierStylePara elsevierViewall">Children diagnosed with deafness are at increased risk of visual impairment&#44; as mentioned above&#44; because certain syndromes or diseases that cause hearing loss also have an impact on vision&#46; In children with deafness&#44; a visual disorder must always be ruled out&#44; especially in cases diagnosed with a syndrome &#40;Usher&#44; CHARGE&#44; Waardenburg&#44; etc&#46;&#41;&#44; with congenital infections causing retinopathy &#40;CMV&#44; retinopathy of prematurity&#41; or with brain damage such as that caused by infantile cerebral palsy&#46; Warning signs are&#58; lack of gaze fixation&#44; poor eye tracking&#44; oscillating eye movement&#44; wandering eye&#44; cephalic tilt&#44; stumbling over objects&#44; poor night vision&#44; and difficult acclimatisation from dark to light&#44; or vice versa&#46;</p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Learning difficulties</span><p id="par0125" class="elsevierStylePara elsevierViewall">Although deafness has been excluded from consideration as a specific learning disability&#44; it is common for children with deafness to have some learning difficulties or a specific disorder&#46; It is difficult for professionals to separate the impact of deafness and the specific learning disability itself on these children&#39;s academic performance&#46; Professionals with a good understanding of the differences between the two entities can plan appropriate intervention&#46;</p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Communication disorders</span><p id="par0130" class="elsevierStylePara elsevierViewall">It is also difficult to determine whether there is a specific language disorder&#44; beyond the anticipated impact of deafness on language development&#46; It is clear that specific communication disorders and autistic spectrum disorder can co-exist with deafness in a child&#46; In addition&#44; some children appear to have significant difficulties in language processing that would not be explained by their hearing impairment alone&#46; Receptive language skills are much more delayed in these children than would be expected based on their cognitive potential and ability to learn&#46; This pattern is easier to recognise in cases with mild to moderate hearing loss&#44; and therefore early identification enables the most appropriate educational support and intervention&#46;</p></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Intellectual disability</span><p id="par0135" class="elsevierStylePara elsevierViewall">Cognitive skills can have an impact on children&#39;s progress in all aspects of their development&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> Although intellectual ability is not the only factor affecting language development&#44; it is highly correlated with language outcomes&#44; and therefore reliable assessment of cognitive abilities &#40;typically determined by non-verbal cognitive measures&#41; can help provide a better structure for identifying whether a child is making the progress that would have been anticipated over time&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">The algorithm described in <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a> for the diagnostic and therapeutic approach to childhood deafness with AD&#43; can guide professionals&#44; knowing the individual and learning characteristics of the child&#44; to act step by step to determine intervention needs adapted to each case&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span></span></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Language development in AD&#43;</span><p id="par0145" class="elsevierStylePara elsevierViewall">The development of oral language&#44; receptive and expressive&#44; in children with deafness treated with hearing aids and&#47;or implants is determined&#44; among other factors&#44; by the severity of the hearing loss&#44; the time of onset&#44; the age of the hearing aid fitting&#44; cognitive development&#44; and the communicative model used in the early intervention&#46; There are 2 other variables that behave differently in children fitted with a hearing aid and implanted children&#46; On the one hand&#44; the level of maternal education which&#44; in any case&#44; favours speech and language development&#44; especially in children fitted with a hearing aid&#46; On the other hand&#44; the presence of a disability in addition to deafness negatively affects development&#44; especially in the case of implanted AD&#43; children&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Previous studies have described the presence of a disability in addition to hearing loss as one of the most reliable predictors of speech and language development in children&#46; In this regard&#44; in a study by Cupples&#44; most of the children were diagnosed with autism spectrum disorder&#44; cerebral palsy&#44; and global developmental delay&#44; disabilities that have greater impact on speech and language development than others&#46;<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">31</span></a> Therefore&#44; the disabilities that are most frequently added to hearing impairment have been classified into 2 groups according to their impact on speech and language development &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;&#46; The first group identifies the disabilities with the greatest impact &#40;ASD&#44; cerebral palsy&#44; ASD associated with cerebral palsy&#44; global developmental delay associated with another syndrome&#44; and isolated global developmental delay&#41;&#46; In the second group are vision disorders&#44; speech and phono-articulatory disorders&#44; other syndromes without developmental delay&#44; and various medical and health problems&#46; Research in 3-year-old children found that children with autism&#44; cerebral palsy&#44; and&#47;or developmental delay score significantly worse in language development than children with other disabilities &#40;such as visual impairments&#44; speech and language disorders&#44; syndromes without developmental delay&#44; and diseases&#41;&#46; Children with a disability in addition to a hearing impairment achieve worse levels of language development than children without a hearing impairment&#59; they are typically one to two standard deviations &#40;SD&#41; below normal hearing children of the same age&#46; In contrast&#44; non-verbal cognitive ability is approximately &#46;3 SD below the typical mean&#46;<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">32</span></a> The literature highlights variability in language development in implanted children with an associated disability&#46; The different cognitive ability of each child or group of children may be a factor explaining this variability&#46; Many studies indicate that better language development is related to better levels of cognitive development&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> although other factors may also be found to explain this&#58; non-verbal skills&#44; less profound degree of hearing loss&#44; use of oral language in early intervention&#44; higher educational level of the mother&#44; and early fitting of hearing aids or cochlear implants&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Adaptation of infant hearing impairment programmes for intervention with children with AD&#43;</span><p id="par0150" class="elsevierStylePara elsevierViewall">Widespread neonatal hearing screening and early intervention programmes have significantly increased the detection of children who are deaf and hard of hearing&#46; As demographics have changed&#44; the educational complexity and service needs for this population have also increased&#46; Technological advances have improved access to sound and language development&#44; but at the same time there are gaps in the skills of staff providing services to this diverse group of children&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Diagnosis of a disability in addition to deafness</span><p id="par0155" class="elsevierStylePara elsevierViewall">The first challenge for a screening and early treatment programme for childhood hearing loss is to detect or recognise deaf children with an additional disability&#46; Identifying additional disabilities as early as possible is essential to ensure that these children have access to appropriate intervention&#44; and thus achieve the maximum possible development in each case&#46; As screening and early care make language delays in children with a hearing impairment increasingly rare&#44; the diagnosis of other disabilities&#44; such as ASD in a child with deafness&#44; may seem easier than in the past&#46; The recommendations of the Joint Committee on Infant Hearing &#40;JCIH&#41; and CODEPEH state that all children with hearing loss under the age of 3 years should undergo six-monthly assessment of their speech&#44; language&#44; and cognitive skills&#44;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">33</span></a> which enables detection of cases where there are delays or deviations from milestones and expected outcomes&#46;</p></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Diagnosing hearing loss in severe developmental disorders</span><p id="par0160" class="elsevierStylePara elsevierViewall">Audiometric assessment to rule out a hearing deficit in a child with a disability can be a major challenge&#46; In fact&#44; many children undergo multiple attempts at behavioural audiometry without success&#46; The impossibility of determining auditory thresholds in this context means that sedation is often necessary for objective audiological testing&#46; While it is always desirable to minimise sedation or anaesthesia&#44; repeated unsuccessful attempts at behavioural audiometry can cause diagnostic delays&#44; resulting in increased financial cost and concern for families&#46; Patients with cognitive delay&#44; autism spectrum disorder&#44; global developmental delay&#44; or cerebral palsy are at the highest risk of developing additional hearing loss&#44; even if they have passed neonatal screening tests for hearing loss&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">26</span></a> They should therefore be more closely monitored to rule out an additional hearing loss&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">33</span></a> Given the great benefit to be gained from early intervention for hearing loss in addition to a cognitive or developmental disorder&#44; it is imperative to avoid diagnostic delays&#44; which some studies report can be as long as two years &#40;2&#8211;3 failed attempts at behavioural audiometry on average before auditory evoked potential testing&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">26</span></a> This evidence supports the early indication for objective audiological testing to avoid delays in diagnosis&#44; using sedation or general anaesthesia if necessary&#46; It is important to remember that some of the commonly used audiological tests do not confirm normal central auditory function&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">34</span></a> There is some tendency to report that hearing is adequate to ensure speech and language development based on the normality of these tests&#58; however&#44; we must remember that there are central auditory processing problems that prevent adequate development&#46; Children and adults with ASD describe difficulties in listening in noise&#44; in maintaining attention to auditory stimuli&#44; and in using auditory information in sensory stressful situations&#44;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">35</span></a> which may indicate a central auditory processing disorder&#46;</p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Recommended audiological treatment in cases of AD&#43;</span><p id="par0165" class="elsevierStylePara elsevierViewall">Once hearing loss is diagnosed in a child with AD<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>and a hearing aid fitting is indicated&#44; hearing aids should be selected and fitted using a prescriptive approach that considers the acoustics of the auditory canals and thresholds&#46; If behavioural thresholds are unreliable&#44; hearing aid programming should be adjusted based on the electrophysiological results of steady state evoked potential testing&#44; and then the fitting corrected as information on behavioural thresholds is obtained in subsequent assessments&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">36</span></a> The goal of any hearing aid fitting is to provide the child with access to acoustic speech information without the hearing aid exceeding the recommended amplification limits&#46; Children with ASD are no different in terms of this goal&#44; but it should be noted that some children with ASD experience increased loudness perception and may exhibit behaviour consistent with an exaggerated response to auditory stimuli&#44;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">37</span></a> therefore it may be necessary to limit the levels of prosthetic stimulation to help acceptance of the new device&#46; Another characteristic of these children is poor tolerance to the tactile sensation of contact with other people&#44; which may make it difficult to measure real-ear to coupler differences &#40;RECD&#41; or to take impressions for earmoulds for the hearing aid fitting&#44; and therefore it would not be unusual to sedate the child for this purpose&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">38</span></a> As with any child fitting&#44; access to the hearing aid batteries should be blocked and the volume and programming control buttons should be disabled&#46; With regard to cochlear implantation in children with ASD&#44; improvements have been found in social communication&#44; behaviour&#44; attention to surroundings&#44; increased vocalisation&#44; eye contact&#44; and reaction to music&#46;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">39</span></a> Comparing children with ASD and children with various additional disabilities&#44; it has been observed that children with ASD have limited development of auditory perception&#46; However&#44; cochlear implantation is not contraindicated in children with ASD&#44; provided that parents are informed that successful implantation cannot be predicted and it is important to manage expectations&#46; In these cases&#44; an experienced team is required&#44; who are willing to consult with ASD experts&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">40</span></a></p><p id="par0170" class="elsevierStylePara elsevierViewall">Hearing loss is a symptom to be aware of as it is frequently observed in children with ASD&#46; It consists of a pattern of exaggerated behavioural reactions to auditory sensory stimuli&#46; Although its presence is not universal&#44; it is so frequent in these cases that a differential diagnosis of ASD needs to be made&#46; Psychoacoustic tests have been used to measure the perception of acoustic intensity in children with ASD and it has been observed that they present a narrow dynamic range and a reduced tolerance to it&#44; which is consistent with the hearing loss observed in this group&#46;<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">41</span></a> The prevalence of this symptom in children with ASD is not universal&#44; but it is so frequent that it is necessary to establish a differential diagnosis of ASD&#46; The prevalence of this symptom varies from 18&#37; to 53&#37; in children with ASD&#44; while it is not found in typically developing children&#46; The presence of hearing loss may be due to certain characteristics of the subject such as mental age&#44; because it diminishes with increasing mental age&#46; It is therefore considered to be a general deficit associated with developmental disabilities and is not specific to children with ASD&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">40</span></a></p><p id="par0175" class="elsevierStylePara elsevierViewall">Cochlear implantation has very diverse results&#46; In particular&#44; a review of several studies on cochlear implants in children with WS indicates that they develop similar abilities to those implanted for typical sensorineural deafness&#46; There are several studies on the improvement in both hearing ability and speech intelligibility after cochlear implantation in children with WS&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">42</span></a> Also results in CHARGE patients showed that the majority continued to have mild hearing loss&#59; however&#44; improvement was seen in the parents&#8217; perception of auditory responsiveness&#46; Most of the children used gestural communication and were in special education classes&#46;</p></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Early intervention in children with AD&#43;</span><p id="par0180" class="elsevierStylePara elsevierViewall">Another challenge for a child hearing impairment care programme is to plan the services made available to each child with AD&#43;&#46; The approach to these cases must be based on the individual needs of the children&#44; avoiding categorising or labelling their disability&#46; Thus&#44; if each case is considered unique&#44; it will be possible to enhance their strengths and provide responses tailored to their individual needs&#46; This person-based approach allows families to play an important role in designing the care for their children&#46; AD&#43; children&#44; if not detected early and treated appropriately&#44; risk missing out on the critical learning period and consolidating a developmental gap compared to deaf children without an additional disability&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">43</span></a> Early intervention requires interdisciplinary teams to care for children with AD&#43;&#44; involving multiple service providers incorporating teachers&#44; speech therapists&#44; psychologists&#44; educationalists&#44; and audiologists&#44; and may extend to other professions such as physiotherapists&#44; and requires the use of assistive products and technologies according to the needs of the child&#46; Although early childhood professionals see collaboration with families and other professionals as an important aspect of their work&#44; interdisciplinary collaboration is often a challenge&#46; This may be due to poor preparation for this cooperative work&#44; as collaboration is a subtle and very complex field that requires explicit instructions for professionals&#46; A continuum of collaborative models has been used to provide services to children with special educational needs&#58; multidisciplinary&#44; interdisciplinary&#44; and transdisciplinary&#46; The multidisciplinary model involves professionals working with children separately&#46; The results of this model are fragmented services and even conflicting activities and recommendations&#46;<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">44</span></a> If the interdisciplinary model is used&#44; professionals communicate with each other and make decisions by consensus&#44; but assessments and implementation tend to take place separately&#46; This model does not allow professionals to fully coordinate their activities and can result in a child having to work with multiple professionals&#44; which can be difficult for young children&#46; The transdisciplinary model includes not only communication between professionals and families&#44; but also transfers skills from one team member to another&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">45</span></a> Transdisciplinary collaboration is also known as the indirect therapy model and is characterised by a planned release of roles&#44; in which roles and responsibilities are shared and exchanged between team members&#46; This model enables fewer team members providing services directly&#44; while other team members act as consultants&#46; This approach may be less stressful for young children&#46; However&#44; the most effective programming for children with AD&#43; should be highly flexible and individualised&#44; with early intervention services focused on the child and family&#46; A transdisciplinary approach enables coordinated services based on input from a team of professionals with diverse experience and expertise&#46;</p></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Care and support for families of children with AD&#43;</span><p id="par0185" class="elsevierStylePara elsevierViewall">The actions that determine the effectiveness of treatment and intervention with a child with a disability&#44; include offering global care and&#44; as has been said&#44; care centred on the child and their family&#44; avoiding fragmented&#44; decontextualised&#44; and uncoordinated responses&#44; giving confidence and security to families&#44; reducing their disorientation having to wander through various specialists and services&#44; and starting them as early as possible&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">46</span></a> Coming to terms with the news and the lack of information are&#44; in turn&#44; two of the main difficulties faced by families in the first moments after learning the diagnosis&#46; In the case of children with AD&#43;&#44; this becomes even more important as&#44; often&#44; the diagnoses overlap or occur over time&#44; which causes families greater emotional stress and interferes in their acceptance of the situation and decision-making&#46; Therefore&#44; families must be able to count on stable teams and structures with specialist&#44; qualified professionals able to deal with the individual case and based on analysis of the specific socio-familial situation&#46; The role of the professionals involved at each level is fundamental&#44; especially in handling a new diagnosis and&#44; in general&#44; in conveying all the information to families&#44; hence the importance of training programmes aimed at capacity building for effective teamwork&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">33</span></a> The support needs of families have been shown to go beyond receiving information on health issues or hearing technology to the emotional&#46; Different studies have collected information about the needs of families&#44; some to do with the way in which information is conveyed to them&#44; the lack of coordination between professionals during the different stages of the screening programme&#44; partial information about treatment&#44; and difficulty in referral to appropriate services&#44; and lack of awareness of available resources&#46;<span class="elsevierStyleSup">33</span> Therefore&#44; a transdisciplinary team&#44; co-responsible and working in collaboration with the family&#44; is essential at all levels of case management&#44; especially in the case of AD&#43;&#46; This team must provide quality&#44; complete&#44; understandable&#44; objective&#44; and truthful information to manage expectations about prognosis&#46; It should cover medical diagnosis&#44; treatment&#44; use of hearing aids&#44; early intervention&#44; and speech therapy options&#44; as well as referral to support structures and resources for families&#46; Family support programmes should be integrated as a supportive and cooperative element within the team&#46;<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">18&#44;33</span></a> The message must be coherent and consistent among the different professionals involved&#46; It is also important to sequence and group consultations close together in time&#44; coordinating care and the provision of information between them&#44; respecting current regulations in relation to personal data protection&#46; The family should not be responsible for conveying information between professionals nor be the mediator of its coordination&#44; and it is advisable to control the amount of information offered&#44; the time and setting for communication&#46; Inter-administrative and inter-sectorial coordination is also necessary&#44; with simplified and coordinated procedures for access to all services and benefits for the child and their family&#46; Referral and care pathways must be established for both the child and their family&#44; which are coordinated with each other and ensure the continuity of the process&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">46</span></a> The family should receive information&#44; help and advice&#59; their collaboration and intervention is indispensable and irreplaceable&#44; because the effectiveness and&#47;or the best outcome of treatment largely depends on them&#46;</p></span></span><span id="sec0120" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Key points and recommendations from CODEPEH</span><p id="par0220" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1</span><p id="par0190" class="elsevierStylePara elsevierViewall">More than 40&#37; of children with deafness have an added developmental disorder and&#47;or a major medical problem which&#44; in addition to delaying the age of diagnosis in many cases&#44; may require the intervention of other specialist professionals&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2</span><p id="par0195" class="elsevierStylePara elsevierViewall">Early detection of this association is vital and therefore global developmental assessment every 6 months in every child with deafness is extremely important&#46; Similarly&#44; associated deafness should be ruled out in children with a diagnosed disability who do not reach expected developmental milestones&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3</span><p id="par0200" class="elsevierStylePara elsevierViewall">The situation of these children is termed in the literature &#34;deaf or hard of hearing PLUS&#34;&#44; but the term &#34;deafness with additional disability &#40;AD&#43;&#41;&#34; is preferable&#46;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">4</span><p id="par0205" class="elsevierStylePara elsevierViewall">Identifying AD&#43; allows for early and appropriate intervention which will be reflected&#44; among other areas&#44; in improved language and communication skills&#46; The relevant audiological tests need to be conducted as soon as possible&#44; even with sedation if necessary&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">5</span><p id="par0210" class="elsevierStylePara elsevierViewall">Early care should include transdisciplinary teams to care for children with AD&#43;&#44; using an approach based on the individual needs of the child and in collaboration with the family&#46;</p></li></ul></p></span><span id="sec0125" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Conflict of interests</span><p id="par0215" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare&#46;</p></span></span>"
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          "titulo" => "Introduction"
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          "identificador" => "sec0010"
          "titulo" => "Incidence of AD&#43; in different clinical contexts"
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              "identificador" => "sec0015"
              "titulo" => "Diagnosed deafness and other associated disabilities"
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                  "titulo" => "Entailing intellectual disability &#40;to a greater or lesser degree&#41;"
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              "titulo" => "Warning signs of developmental delay in a child with hearing loss"
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                0 => array:2 [
                  "identificador" => "sec0060"
                  "titulo" => "Motor development"
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                  "identificador" => "sec0065"
                  "titulo" => "Visual impairment"
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                2 => array:2 [
                  "identificador" => "sec0070"
                  "titulo" => "Learning difficulties"
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                3 => array:2 [
                  "identificador" => "sec0075"
                  "titulo" => "Communication disorders"
                ]
                4 => array:2 [
                  "identificador" => "sec0080"
                  "titulo" => "Intellectual disability"
                ]
              ]
            ]
          ]
        ]
        7 => array:2 [
          "identificador" => "sec0085"
          "titulo" => "Language development in AD&#43;"
        ]
        8 => array:3 [
          "identificador" => "sec0090"
          "titulo" => "Adaptation of infant hearing impairment programmes for intervention with children with AD&#43;"
          "secciones" => array:5 [
            0 => array:2 [
              "identificador" => "sec0095"
              "titulo" => "Diagnosis of a disability in addition to deafness"
            ]
            1 => array:2 [
              "identificador" => "sec0100"
              "titulo" => "Diagnosing hearing loss in severe developmental disorders"
            ]
            2 => array:2 [
              "identificador" => "sec0105"
              "titulo" => "Recommended audiological treatment in cases of AD&#43;"
            ]
            3 => array:2 [
              "identificador" => "sec0110"
              "titulo" => "Early intervention in children with AD&#43;"
            ]
            4 => array:2 [
              "identificador" => "sec0115"
              "titulo" => "Care and support for families of children with AD&#43;"
            ]
          ]
        ]
        9 => array:2 [
          "identificador" => "sec0120"
          "titulo" => "Key points and recommendations from CODEPEH"
        ]
        10 => array:2 [
          "identificador" => "sec0125"
          "titulo" => "Conflict of interests"
        ]
        11 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2022-07-27"
    "fechaAceptado" => "2022-10-10"
    "PalabrasClave" => array:2 [
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec1732283"
          "palabras" => array:7 [
            0 => "Deaf"
            1 => "Deaf with additional disabilities"
            2 => "Language development"
            3 => "Development delay"
            4 => "Disabilities"
            5 => "Early intervention"
            6 => "Trans-disciplinary"
          ]
        ]
      ]
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec1732282"
          "palabras" => array:7 [
            0 => "Sordera"
            1 => "Discapacidad a&#241;adida"
            2 => "Desarrollo del lenguaje"
            3 => "Trastorno del desarrollo"
            4 => "Discapacidades"
            5 => "Intervenci&#243;n temprana"
            6 => "Transdisciplinariedad"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Approximately 40&#37; of children with deafness have an additional developmental disorder or major medical problem&#44; which may delay the age of diagnosis of hearing loss and&#47;or require intervention by other professionals&#46; This situation is referred to as &#8220;deafness with added disability&#8221; &#40;AD&#43;&#41;&#46; The reason why the population of hearing-impaired children is more likely to have associated added disabilities is that the risk factors for hearing impairment overlap with those for many other disabilities&#46; These factors can influence various aspects of development&#44; including language acquisition&#46; It is important to check that appropriate care is received&#44; the effectiveness of hearing aids or implants&#44; as well speech therapy intervention strategies&#44; and family adherence to sessions and appointments&#46; The challenge posed by AD&#43; is early detection&#44; to allow early and appropriate intervention&#44; and the need for fluid transdisciplinary collaboration between all professionals involved&#44; together with the involvement of the family&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Aproximadamente el 40&#37; de los ni&#241;os con sordera tienen a&#241;adido un trastorno del desarrollo o un problema m&#233;dico importante&#44; que puede retrasar la edad de diagn&#243;stico de la hipoacusia y&#47;o precisar de la intervenci&#243;n de otros profesionales&#46; Esta situaci&#243;n se designa como &#8220;hipoacusia o sordera con discapacidad a&#241;adida&#8221; &#40;DA&#43;&#41;&#46; El motivo por el que la poblaci&#243;n de ni&#241;os con problemas auditivos es m&#225;s propensa a asociar discapacidades a&#241;adidas radica en que los factores de riesgo para la hipoacusia se superponen con los de muchas otras discapacidades&#46; Estos factores pueden influir en diversos aspectos del desarrollo&#44; incluida la adquisici&#243;n del lenguaje&#46; Es importante comprobar que se recibe la adecuada atenci&#243;n&#44; la efectividad de aud&#237;fonos o implantes&#44; as&#237; como de las estrategias de intervenci&#243;n logop&#233;dica&#44; y la adherencia de la familia a sesiones y citas&#46; Los desaf&#237;os que plantea la DA<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>son su detecci&#243;n precoz&#44; para permitir una temprana y adecuada intervenci&#243;n&#44; y la necesidad de una colaboraci&#243;n transdisciplinar fluida entre todos los profesionales que han de intervenir&#44; junto con la implicaci&#243;n de la familia&#46;</p></span>"
      ]
    ]
    "NotaPie" => array:1 [
      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">CODEPEH papers are undertaken within the framework of a collaboration agreement between the Real Patronato sobre Discapacidad &#40;Royal Board on Disability&#41; and FIAPAS&#46;</p>"
      ]
    ]
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Diagnostic and therapeutic approach to hearing loss with added disability recommended by CODEPEH&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Type of disability&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Population with hearing loss&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">General population&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t">No added disability&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">60&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">86&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Intellectual disability&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9&#46;8&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">2&#46;5&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Cerebral palsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">4&#37;&#8211;39&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#46;3&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Visual problems&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&#46;9&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#46;03&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Attention deficit&#47;hyperactivity disorder&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6&#46;6&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">5&#37;&#8211;10&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Autism&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">1&#37;&#8722;4&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">Placental insufficiency&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">Gestational hypertension&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">Gestational diabetes&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">Infections such as cytomegalovirus&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Genetic factors such as a syndrome or family history of learning difficulties&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Atypical embryonic development such as spina bifida or brain anomalies&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Acute perinatal hypoxia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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Article information

ISSN: 21735735
Original language: English

DOI:10.1016/j.otoeng.2022.10.008

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