Rachel St. John, MD, NCC, NIC-A

1. It’s Not Always About the Hearing: Case Studies in a Holistic Approach to Deaf and Hard of Hearing Patient Care Rachel St. John, MD, NCC, NIC-A Director: Family Focused Center for Deaf and Hard of Hearing Children Dallas Children’s Medical Center/UTSW Dept. of Otolaryngology

2. DISCLOSURE • Neither I nor any member of my immediate family has a financial relationship or interest (currently or within the past 12 months) with any entity producing, marketing, re-selling, or distributing health care goods or services consumed by, or used on, patients. • I do not intend to discuss an unapproved/investigative use of a commercial product/device.

5. Objectives • Examine the complexity of hearing changes occurring with other conditions (behavioral, neurologic, genetic, etc) through clinical case study example • Appreciate common themes in how these conditions may present as either excessively or inadequately focused on hearing status • Discuss a model of low volume/high complexity care coordination as a means for improved identification

6. Clinical Case #1: SN • 7 year old female who presented to FFC for chief complaint of “school behavior problems and concerns about deaf/hoh accommodations in class”

7. SN • Medical history significant for being hard of hearing • Moderate level, bilateral, sensorineural • Used BTE hearing aids (working well) • In mainstreamed education classroom with IEP (FM, pref seating)

8. SN • School behavior details: • Constantly in trouble with teacher: • Putting head down frequently – not paying attention • Rigid behavior • Often explosive bursts of anger

9. SN • Putting head down • Mother and GM would often witness at home • Usually happened when NS was overwhelmed

10. SN • Explosive Behavior: • Often occurred at times of transition between activities, or when teacher was giving strict instructions about activity • Happened at home as well if activity was interrupted or not as expected

11. SN • Rigidity • Rituals extremely important to NS • Activities would take excessively long time due to ritual behavior

12. THOUGHTS?

13. THOUGHTS? OBSESSIVE COMPULSIVE DISORDER Obsessions and/or Compulsions Cause marked distress, time consuming, interrupt daily functioning

14. SN • Referred for mental health services • Diagnosis confirmed • 3 mofollowup receiving services with improving functioning and lower stress • Improved support and understanding at school

15. Clinical Case #2: LH • 2 ½ year old male with mild hearing decrease only in high frequency range and significant spoken language delay • Presented with concerns for “language delay due to hearing loss or possible Autism Spectrum Disorder”

16. LH • Medical history: generally healthy with some ENT history • middle ear fluid necessitating PE tubes • enlarged tonsils – 2-3+, no snoring or recurrent infection

17. LH • Presented to FFC consult appointment • Zero meaningful expressive spoken language– occasional indecipherable babbling. Could point and grunt to indicate wants (inconsistent with audiogram • Could follow simple spoken language command if accompanied by a gesture

18. LH • Tantrums with head banging common when needs not understood • Mother reported wanting to use sign language, but was discouraged by primary ENT who told her if she signed with him “he would never learn to talk”

19. LH • LH was very socially engaging • Waving hello • Hugging • Smiling on receiving a sticker • Good eye contact • No repetitive or rigid behavior noted on exam except when he wanted something from his mother’s purse and could not express what it was – became upset and cried

20. LH • Initial plan included starting sign exposure in addition to spoken language • Planned for developmental evaluation to confirm ruling out ASD • Follow up planned for 3 mo – ended up being 5 due to family needing to reschedule

21. LH • 5 mo followup • Autism evaluation – not c/w ASD • Mother had begun actively signing with him • 25 meaningful consistent signs • Following 2 step commands in spoken + sign language (“go in the other room and give this to Daddy”) • Starting to meaningfully combine signs (“open please”) and approximate verbally • Denver II: ~9-12 mo development to 18-24 mo level for expressive language

22. THOUGHTS?

23. THOUGHTS? CHILDHOOD APRAXIA OF SPEECH Motor speech disorder (brain cannot coordinate motor movements of mouth and tongue needed for speech) Awareness of thought language

24. LH • Referred for developmental apraxia eval – dx confirmed • Continuing with spoken language, sign, and communication board • Significant decrease in stress and behavioral issues

25. Clinical Case #3: DA • 8 year old female • CODA • Presented to FFC clinic with referral from audiologist • Mother kept insisting daughter had issues with hearing • inconsistent subjective/objective testing - ? of trying to “throw” audiogram?

26. DA • Medical history: • Healthy • Strong history of multigenerational deaf family

27. DA • Behavioral history • Mother kept stating she “acted deaf” – hard to get her attention, visually attending many different things • Often in trouble at school for “not paying attention” or “not listening”

28. DA • During history taking, AD had 10-15 second period: • Cessation of talking • Breaking of eye gaze downward to floor • Not responsive to verbal/visual attempts to get attention • When resumed attention, appeared normal but was not be aware of what had just happened • When asked how often this happens, Mother reported “all day long”

29. THOUGHTS?

30. THOUGHTS? • ABSENCE SEIZURES • Abnormal brain electrical activity • Abrupt impairment of consciousness • Interruption of current activity • “Blank stare” • No post-ictal period

31. DA • Referred to neurology for EEG and evaluation • Outcome pending

32. A FEW MORE…

33. 5 yr old Deaf + undiagnosed ASD (zero spoken or sign language development despite trilingual exposure – teacher was concerned “exposed to too many languages”) • 13 yr old Deaf + undiagnosed CHARGE syndrome (chief complaint was “refusing to wear hearing aids”) • 8 yr old CODA + lack of cultural awareness (PMD concern was ADHD/ODD/CD, trouble in school for “hitting other children” and “being excessively loud”)

34. Take Home Points

35. Take Home Points • There can be a tendency to “blame the hearing” for the majority of issues, and miss possibility of a co-existing condition • Conversely, the impact of hearing status may be minimized because it’s assumed is not enough to not cause problems (e.g. mild, unilateral)

36. Take Home Points • High Complexity/Low Volume Model can be very effective • Requires TIME • Required providers familiar with children who are D/deaf or hard of hearing and resources • Chart review prior to consult is extremely helpful • Impossible without effective interdisciplinary COLLABORATION

37. THANK YOU! Family-Focused Center for Deaf and Hard of Hearing Children Rachel.StJohn@utsouthwestern.edu