Medical Perspectives on Children who are Deaf/Hard of Hearing with an Autism Spectrum Disorder

The issue

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It is rare to find professionals with knowledge and expertise in working with children who are deaf/hard of hearing (DHH) with an Autism Spectrum Disorder (ASD).  Therefore it is critical to ensure DHH children with an ASD are linked to both types of expertise.  This is salient in the medical considerations of DHH children with an ASD as well.

What we know

When children are identified as DHH, medical work-ups and related recommendations have the goals of understanding of the possible cause of hearing loss, possible associated conditions (e.g., kidney, thyroid, heart, or developmental problems), the likelihood of other family members have a hearing loss, and in some situations, providing a prediction of the potential for progressive hearing loss.  As recommended by the Joint Committee on Infant Hearing (www.jcih.org), DHH children should see medical providers with knowledge of pediatric hearing loss in the fields of otolaryngology, ophthalmology, and genetics.  Depending on the type (e.g., sensorineural, conductive) and degree (e.g., mild, moderate, profound) of hearing loss, diagnostic evaluation protocols can include imaging of the inner ear and genetic testing (http://hereditaryhearingloss.org/).

When DHH children also have ASD, there are additional medical considerations due to the presence of ASD.  The focus of genetic testing is different than that for hearing loss and often includes testing for conditions with a high likelihood of having ASD symptoms (such as Fragile X testing).  This type of genetic evaluation is intended to gain an understanding of the likelihood a family may have other children with ASD and can help identify ASD-associated conditions which may impact medical needs.  Some conditions and/or syndromes (such as prematurity, congenital CMV infection, CHARGE syndrome) that are associated with hearing loss have also been associated with ASD or the presence of autistic features.

In general, children with ASD have more medical considerations as some conditions occur at higher rates than seen in the general population. Children with ASD have a higher risk of seizures (occurring in about 30% of children with ASD) and intellectual disability.  Many children with ASD have substantial sleep disturbances, gastrointestinal difficulties (diarrhea and/or constipation), and restrictive appetites that can impact nutritional status.  Among children with ASD, approximately 30% experience developmental regression (loss of an acquired function or lack of age-appropriate progress). Developmental regression is particularly important when considering our approach to monitoring the very early childhood developmental progress in children who are DHH.

With changes in the DSM-5, mental health conditions such as ADHD and anxiety disorders are now able to be diagnosed in children with ASD.  Despite DSM-5 definitions, clinicians have recognized co-existing conditions among children with ASD for many years. Behavioral and medical approaches are available that may be helpful in targeting core behavioral challenges. Being able to identify these co-conditions in children with ASD is vital to early and effective interventions.  Early identification of these conditions is equally important for DHH children with ASD. Unfortunately, the challenges for providers lie in the ability to recognize and diagnose ASD in a child who is DHH.

What we don’t know

It can be difficult to provide medical care for DHH children with an ASD due to their challenges in communication, sensory processing needs, and fears associated with medical settings.  Some children have high thresholds for pain and therefore it is not always easy to recognize when a medical problem is occurring (e.g., not showing pain with a significant ear infection).  The Autism Treatment Network Autism Intervention Research Network on Physical Health (ATN/AIR-P) has toolkits that can be helpful for families and medical providers to build strategies for medical care (http://www.airpnetwork.org/site/c.7oJGLPPsFiJYG/b.8296119/k.C6B3/Tools.htm)
However, we still have a lot of gaps in our knowledge of the causes of ASD in general.  While we recognize there are genetic influences, the field of gene-environment interactions (or epigenetics) is still young.

The advances in our understanding of the importance of early identification and ASD-specific interventions for children with ASD have not yet been applied effectively to DHH children who have an ASD.  DHH children have substantial delays in identification making it difficult to access appropriate therapeutic strategies to expand social-emotional and communication skills.

Implications

It is important to consider the medical needs of DHH children diagnosed with an ASD.  Ensuring a broad approach to care is critical in improving the health and functional outcomes of children with the dual diagnosis.

Posted on July 7, 2016 by
Susan Wiley
Cincinnati Children’s Hospital Medical Center
Susan.Wiley {at} cchmc.org

Further reading