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Shelly Ash, MS, CCC A Anne Oliver, MA, MEd, CCC SLP

A Multidisciplinary Approach to the Management of Children with ANSD: The Importance of Collaboration. Shelly Ash, MS, CCC A Anne Oliver, MA, MEd, CCC SLP. Main Campus in St. Petersburg 259 Beds More than 43 pediatric medical and surgical specialties 10 Outpatient Care Centers in 7 counties.

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Shelly Ash, MS, CCC A Anne Oliver, MA, MEd, CCC SLP

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  1. A Multidisciplinary Approach to the Management of Children with ANSD:The Importance of Collaboration Shelly Ash, MS, CCC A Anne Oliver, MA, MEd, CCC SLP

  2. Main Campus in St. Petersburg259 BedsMore than 43 pediatric medical and surgical specialties10 Outpatient Care Centers in 7 counties All Children’s Hospital

  3. Developmental and Rehabilitative Services:Acute Care RehabilitationApplied Behavior AnalysisAudiologyAutism CenterOccupational TherapyPediatric Rehabilitative MedicinePhysical TherapySpeech, Language and FeedingSports MedicineFind out more at: allkids.org All Children’s Hospital

  4. A Multidisciplinary Approach to the Management of Children with ANSD:The Importance of Collaboration Shelly Ash, MS, CCC A Anne Oliver, MA, MEd, CCC SLP

  5. Learner Objectives The attendee will become familiar with the medical testing for management for children with ANSD. The attendee will become familiar with the audiological testing, management and associated challenges for children with ANSD, and the speech and language testing, management and associated challenges for children with ANSD. The attendee will become familiar with a team approach to managing the hearing, speech and language related needs of children with ANSD and when these children may be considered for cochlear implantation.

  6. What is ANSD?

  7. What is ANSD? Auditory Neuropathy Spectrum Disorder A unique type of hearing loss where sounds aren’t clearly transmitted to the brain from the ear.

  8. What is ANSD? Hearing loss of some degree (can vary widely from mild to profound loss) Difficulty understanding speech, especially in noise.

  9. What is ANSD? Speech understanding difficulties that are worse than can be predicted from other tests of hearing function. Hearing that appears to fluctuate from day-to-day and even from hour to hour.

  10. What does ANSD sound like? Car radio analogy (credit Elaine Blackford)

  11. ANSD Prevalence Accounts for 8-15% of new pediatric hearing losses Teagle et al, 2010, Hang et al 2012

  12. ANSD Presents a unique challenge to patients, families and professionals!

  13. The Multidisciplinary Team • Family • Audiologist • Speech Pathologist • Otolaryngologist • Educators • Other treating professionals • Neurology, Occupational Therapist, Physical Therapist

  14. Challenges in Diagnosing ANSD Diagnosis Prognosis What treatments will be of benefit? What treatments will suit the desires and anticipated outcomes of the family? ??

  15. Evaluative Measures for Diagnosis Medical Audiological Speech and language

  16. Medical Evaluation Otologic Radiological studies (MRI/CT) Lab work Genetics Neurology Opthalmologic

  17. Otologic Evaluation Medical history Ear exam Radiologic Studies

  18. Otologic Evaluation • Medical History • Evaluation for other health problems • Developmental • Vision • ??

  19. Otologic Evaluation Ear exam

  20. Otologic Evaluation • Radiological studies (MRI/CT) • Evaluate for cochlear integrity, malformations • Evaluate cochlear nerve integrity

  21. Role of the Otolaryngologist in the Management of ANSD • To provide a thorough medical examination and recommend testing as needed to diagnose etiology or associated conditions

  22. Role of the Otolaryngologist in the Management of ANSD To provide medical clearance for the use of hearing aids

  23. Role of the Otolaryngologist in the Management of ANSD To make recommendations to the family and/or team regarding additional specialists that should be consulted relative to etiology or associated conditions

  24. Audiological Evaluation • Auditory Brainstem Response testing • Acoustic Immittance testing to include Tympanometry and Acoustic Reflex testing • Otoacoustic Emissions testing • Behavioral Audiometry

  25. Audiological Evaluation • Speech Perception Measures • Infant Toddler Meaningful Auditory Integration Scale or Meaningful Auditory Integration Scale • Early Speech Perception Test • Multilexical and Lexical Neighborhood Word tests • HINT sentences

  26. Audiological Evaluation:Challenges Pure tone thresholds may not reflect the true degree of auditory deficit Especially critical when making recommendations regarding consideration for cochlear implantation

  27. Audiological Evaluation:Challenges Poor behavioral test/retest reliability may not reflect an uncooperative patient

  28. Audiological Evaluation:Challenges For ANSD, behavioral thresholds are NOT a primary consideration for cochlear implant candidacy. Breneman, Gifford and DeJong, 2012

  29. Audiological Evaluation:Challenges Behavioral test methods must be administered in consideration of the child’s developmental age and skills

  30. Audiological Evaluation:Necessities Be thorough in collecting objective measures

  31. Audiological Evaluation:Necessities Give strong consideration to the observations of parents, teachers and/or other treating professionals

  32. Audiological Evaluation:Necessities • It is imperative to obtain a thorough evaluation of expressive and receptive language skills

  33. Speech and Language Evaluation • Collaboration with Audiologist • Review audiological testing results • Discuss observations • Prepare and plan • Objective and Subjective Measures • Standardized tests • Parent observations • Therapist observations

  34. Speech and Language EvaluationFunctional Auditory Skills • Required tests: • Ling 6 Sound • LittlEars • (Parent Report!)

  35. Speech and Language Evaluation:Functional Auditory Skills Probe and observe: Responses to noisemakers Responses to environmental sounds Responses to Learning to Listen Toys Responses to speech (calling name, simple phrases)

  36. Speech and Language Evaluation:Functional Auditory Skills Optional: Auditory Learning Guide SPICE Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) Listening Skills Scale for Kids with Cochlear Implants Auditory Skills Hierarchy (Erber) St. Gabriel’s Curriculum (Audition) ELF CHILD Auditory Processing Abilities Test (APAT) APT-HI

  37. Speech and Language Evaluation:Language Skills Required tests: Rossetti Infant-Toddler Language Scale (Language Comprehension/Expression) Preschool Language Scale Clinical Evaluation of Language Fundamentals (CELF)

  38. Speech and Language Evaluation:Language Skills Optional: St. Gabriel’s Curriculum (Language) SKI-HI Language Development Scale (if school system hasn’t already administered it) Test of Oral Language Development (TOLD) Expressive One Word Picture Vocabulary Test (EOWPVT) Receptive One Word Picture Vocabulary Test (ROWPVT)

  39. Speech and Language Evaluation:Language Skills Observe and Document: Intentionality Communicative Functions (and how they are met) Parent’s primary mode of communication (speech/sign/natural gestures)

  40. Speech and Language Evaluation:Speech Skills Vocal and Phonemic Repertoire form St. Gabriel’s Curriculum (See Speech) Goldman Fristoe Test of Articulation

  41. Speech and Language Evaluation:Speech Skills Observe and Document: Phonemic repertoire Spontaneous vocalizations Imitative vocalizations (pitch/duration/intensity) Frequency of use of voice Ease of vocalizations, spontaneous and imitative Ease of imitation of vowels and consonants Stimulability of vowels and consonants Intelligibility of word approximations Intelligibility of connected speech Resonance (if concerns, give Bzock)

  42. Speech and Language Evaluation:Other Areas of Development Oral Mechanism Exam Voice/Fluency Rossetti Infant-Toddler Language Scale (Interaction/Attachment, Pragmatics, Gesture, Play) St. Gabriel’s Curriculum (Cognition, starting at 6 months)

  43. Challenges in Managing ANSD Patients Auditory responses are variable. Parents describe “good hearing days” and “bad hearing days”. This variability makes it difficult to judge child’s listening skills. Significant health issues and challenges often accompany ANSD. (Many infants with this diagnosis have had a difficult and complicated neonatal course). Due to the variable presentations of ANSD, and the variety of outcomes, parents often express feelings of confusion regarding the diagnosis and anxiety about their child’s future.

  44. Challenges in Managing ANSD Patients Decisions regarding cochlear implantation are complicated by fluctuating auditory functioning and fluctuating speech and language performance. Cochlear implant candidacy considerations are different for children with ANSD diagnosis. (If insufficient speech and language progress, they are considered CI candidates regardless of audiometric thresholds). Auditory functioning may change over time and future outcomes with CI cannot be predicted.

  45. Challenges in Managing ANSD Patients Professionals and interventionists may be unfamiliar with the diagnosis of ANSD and its implications. This may make finding the appropriate interventionist difficult. Evidence-based data comparing outcomes of visually based intervention and auditory based intervention with children who have ANSD is lacking.

  46. Challenges in Managing ANSD Patients The SLP can expect to observe auditory behaviors that are different from both hearing children and children with hearing loss.

  47. Making Decisions as a Team:Considerations Language acquisition and therapeutic intervention Trial period with hearing aids and/or FM system Cochlear implantation

  48. Making Decisions as a Team:Considerations The benchmark for children with ANSD is language acquisition and development. The goal is for the child to make a month of progress in language skills for every month enrolled in appropriate intervention

  49. Making Decisions as a Team:SLP Role in Language Acquisition To develop the child’s language and listening skills To coach the family in how to incorporate language and listening into daily routines To help the team (Family, School, Audiology,) determine if the child is benefiting from hearing aids

  50. Making Decisions as a Team:SLP Role • To monitor (through systematic observations and analysis) and report progress/lack of progress in speech, language and listening to the team • To collaborate with the treating audiologist and/or the cochlear implant team on a regular basis • To provide ongoing counseling and education to the family regarding ANSD and its impact on speech and language learning

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