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

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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. 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 Evaluation Functional 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

51. Making Decisions as a Team: The primary goal of auditory intervention is to give the child hearing sufficient for him/her to develop receptive and spoken language. It is for this reason that speech recognition ability is considered a primary outcome measure for assessing the benefits of amplification. Humphries et al, 2013

52. Auditory Based Care for children with ANSD: Monitoring Progress Auditory functioning and speech and language performance is monitored and documented as part of every treatment session. A tracking form is used to document the child’s progress across sessions. Parents provide ongoing information about how the child is functioning in different environments and are encouraged to keep a log of auditory functioning

53. ADDAadd add 9/6/201553 Add SLP Tracking Form here

54. Auditory Based Care for children with ANSD: Progress Testing Documentation is completed, minimally, every three months. This includes re-administration of formal tests (usually the PLS and GFTA).

55. Auditory Based Care for children with ANSD: Progress Testing Progress testing should include info on: –Level and consistency of child’s comprehension of spoken language –Level and consistency of child’s expressive spoken language –Articulation abilities and intelligibility A comprehensive summary of child’s Auditory Functioning including consistency and nature of child’s responses, dependency on visual information *

56. Auditory Based Care for children with ANSD: Progress Testing The progress report includes documentation of observations in therapy as well as the observations of parents and others (teachers, day care providers, grandparents etc.). Information is gathered from formal testing, tracking chart, parent report/journal, and other resources such as SIFTER, preschool SIFTER or FISHER.

57. Auditory Based Care for children with ANSD: Progress Testing This information is then shared with the family and other team members following the child’s progress in order for the team to recommend future directions for intervention.

58. Making Decisions as a Team: SLP Role To discuss parent’s goals for their child and provide guidance on how to reach those goals, including education regarding different habilitative options and available community services To guide family in accessing resources for education and support To support the unique strengths and abilities of the child and family

59. Hearing Aids and FM Systems Fitting challenges –Functional test limitations –ANSD patients have increased difficulties listening in noise –Fluctuating hearing levels

60. Hearing Aids and FM Systems Fitting challenges –ANSD identified in children with limited language creates challenges in determining speech perception abilities with hearing aids

61. Hearing Aids and FM Systems: Audiologist Role To utilize all measures (behavioral and objective) to assist in choosing and fitting hearing aids

62. Hearing Aids and FM Systems: Audiologist Role To monitor and report progress regarding hearing aid use to the team

63. Hearing Aids and FM Systems: Audiologist Role To closely monitor the patient for any necessary adjustments or modifications as needed –Query SLP regarding child’s responses and use of sound. –Discuss with parents what sounds child is responding to at home, with and without the hearing aids on

64. Hearing Aids and FM Systems: Audiologist Role To discuss with the family their goals for the child and provide guidance on how to reach those goals. –Collaborate with team SLP regarding habilitation options, community resources and educational choices that support the goals of the family and needs of the child

65. Hearing Aids and FM Systems: Audiologist Role To discuss with families that ANSD presents a challenge in making a prognosis regarding potential for success with traditional hearing aids –Pure tone audiogram is not a reliable measure –Limited clinical tools available relative to making predictions for success

66. Hearing Aids and FM Systems: Audiologist Role To advise the family that consistency of hearing aid use and participation in language therapy will be key to determining success of the hearing aid fitting

67. Hearing Aids and FM Systems: Audiologist Role Provide the family with recommendations and information regarding considering cochlear implantation

68. Cochlear Implantation: When to Consider? Is child capable of using auditory information as provided by hearing aids (or without sensory devices) for language learning purposes??

69. Cochlear Implantation: When to Consider? Has the child made a month of progress in language development for every month of therapy? Discuss with SLP the established auditory based goals for language development and whether the goals are being attained Discuss parental expectations and desired outcome in language learning

70. Cochlear Implantation To best evaluate effectiveness of hearing aids, the child should be enrolled in language therapy that emphasizes auditory based goals.

71. Cochlear Implantation Prior to approving cochlear implantation, 3 rd party payers may require a hearing aid trial for a minimum of 3 months

72. Cochlear Implantation It is important to establish that the cochlear implant is of benefit to children with ANSD. This further supports bypassing of traditional audiological candidacy guidelines. Humphries et al, 2013

73. The All Children’s Hospital ANSD Experience 23 children identified with ANSD 8 children implanted at ACH 6 implanted at other facilities

74. The All Children’s Hospital ANSD Experience 11 currently enrolled in language therapy at ACH –4 using cochlear implants –7 using hearing aids –2 in CI candidacy process

75. The All Children’s Hospital ANSD Experience Breakdown of patients enrolled at ACH in therapy: 0-3 year olds= 0 4-5 year olds= 7 6-8 year olds= 4 9-11 year olds= 0

76. The All Children’s Hospital ANSD Experience – Multidisciplinary Approach Audiological evaluation and diagnosis of ANSD Medical evaluation with otolaryngologist Hearing aid trial Enrollment in Auditory Based Care (ABC) Therapy Consultation with educators Recommendations regarding cochlear implant candidacy

77. The All Children’s Hospital ANSD Experience It Takes a Village!