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Multidisciplinary Hearing Assessment Clinic in Utah: Rationale and Preliminary Outcomes

This article discusses the development of a multidisciplinary hearing assessment clinic in Utah, focusing on the evaluation and management of pediatric hearing loss. It covers the role of various professionals, such as geneticists, audiologists, and otolaryngologists, and includes case presentations and parental views on the evaluation process. The article also highlights the challenges in pediatric audiology and the importance of early diagnosis and streamlined processes.

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Multidisciplinary Hearing Assessment Clinic in Utah: Rationale and Preliminary Outcomes

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  1. Development of a Multidisciplinary Hearing Assessment Clinic in Utah: The Rationale and Preliminary Outcomes Albert H. Park, MD; Steve Bleyl, MD; John Carey, MD; Cache Pitt, MS, CCC-A

  2. Evaluation and Management of Pediatric Hearing Loss • Emphasis on Screening of Newborns • What to do when a child does not pass screening? • Parental perspective of the evaluation • Rationale for Multidisciplinary clinic • Role of Geneticist • Role or Audiologist • Role of Otolaryngologist • Case Presentations

  3. A Survey of Parental Views Regarding Their Child’s Hearing Loss Albert H. Park, MD; Jonathon Warner, BS; Nanette Sturgill, MS, CCC-A; Stephen Alder, PhD Otolaryngology-HNS journal 2006

  4. Materials and Methods: • All pediatric SNHL pts (2000-2004) • Audiology database @ PCMC • Only 30 level peds center Utah • Survey query experiences with testing and rx

  5. Methods and Materials: • N=389 surveys mailed SNHL families • 113 families responded • 1 family requested not to be included • 4 patients later found normal hearing • 108 respondents basis study

  6. Methods and Materials: • Patient population: • Some identified via newborn screening • Some identified later childhood • Statistical analysis when indicated • Approval obtained UU IRB

  7. Results: • Distribution of patients based screening • 61% not pass screening • 23% passed screening • 13% did not know results screening

  8. Results:

  9. Results: • Information for No Pass Group • Audiology and Labor/delivery (L+D) most helpful • >15% no one provided information

  10. Results: • Primary Care Physician (PCP) involvement mixed • 22.4% PCP not aware

  11. Results: • Reasons for delay diagnosis • Difficulty audio. apt • Multiple tests (test) • Difficulty getting referral from PCP (refer) • Poor medical advice (advice)

  12. Results: • Significant % underwent 4 or more tests

  13. Results: • 62% families using hearing aids – difficulty obtaining the aids • Thirty-one patients underwent cochlear implantation • 58% families using cochlear implants – difficulty obtaining devices

  14. Results: • Steps to dx child’s hearing loss difficult? • 60% families – process difficult • Desire more information • Importance not be intimidated • Importance early rx • Need for streamlined process • Desire for parental network

  15. Discussion: • Utah Newborn Screening Program-successful • > 98% 28,037 newborns born this yr tested • Initial state pass rate 93% • Utah Dept Health- responsible data collection and management

  16. Discussion: • Significant degree parental frustration and obstacles • 15% comments no information @ newborn screening • 22% PCPs not aware hearing loss • Delays in diagnosis- apt, testing, referral problems • Multiple tests

  17. Discussion: • Hearing Assessment Clinic (HAC): • Provide resources for evaluation and rx • Multidisciplinary • Genetics, pediatric audiology and otolaryngology • Close communication with primary care and outside audiologists

  18. The Role of the Pediatric Audiologist • Resource for parent • Review test results • Review implications of hearing loss • Referral source • Rehabilitation recommendations • Resource for audiologist • Repeat testing as necessary • Facilitate communication with the physicians • Resource for referring physicians

  19. Challenges in Pediatric Audiology

  20. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  21. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  22. The Role of the Pediatric Audiologist • Impact of Diagnosis on the family • Diagnosis is new information for the families • Families identified through newborn hearing screening have usually not had indications from the child that they are not hearing

  23. The Role of the Pediatric Audiologist • At the time of diagnosis the family often stops hearing after they learn that their child has a hearing loss • The family may not hear information • The family may misunderstand details provided because of the newness and unfamiliarity of hearing loss • The amount of information regarding hearing loss and rehabilitation is overwhelming, but must be found first.

  24. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  25. The Role of the Pediatric Audiologist • Impact of hearing loss on the child • Hearing loss can potentially affect communication, education, socialization, and employment • Goal is to aid children as quickly as possible to support the acquisition of spoken language

  26. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  27. The Role of the Pediatric Audiologist • Testing • Diagnosis of hearing loss in children is accomplished using a battery of tests and repeatable test results • Even with a cooperative child, diagnosis of hearing loss is not accomplished off of only one test or one set of test results • Non-participatory children will require multiple visits

  28. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  29. The Role of the Pediatric Audiologist • Communication with other professionals • Sometimes it is very difficult to catch-up with the ENT and other physicians to communicate concerns/results

  30. The Role of the Pediatric Audiologist • Challenges in pediatric audiology • Impact of Diagnosis on family • Impact of hearing loss on the child • Testing • Communication with other professionals • Geography

  31. The Role of the Pediatric Audiologist • Geography • Patients often have to travel distances • To have access to many professionals at once eases the burden and cost of care on the family • Some patients may not have access to a managing audiologist, the HAC audiologist may be their only resource

  32. The Role of the Pediatric Audiologist • Resource for parent • Review test results • Review implications of hearing loss • Referral source • Rehabilitation recommendations/discussion • Resource for audiologist • Repeat testing as necessary • Facilitate communication with the physicians • Resource for referring physician

  33. The Role of the Otolaryngologist in HAC: • Evolving • Detection and treatment of middle ear pathology • Seeing patients younger age • Involved diagnostic testing • Involved coordination or imaging and treatment

  34. The Role of Imaging for Idiopathic Sensorineural Hearing Loss (SNHL) • n=46 pediatric patients with inner ear anomalies and SNHL • 21% patients seen in a multidiscipinary hearing assessment clinic (1987-1996) • Most common cochlear abnormalities: cochlear hypoplasia and incomplete partition • Enlarged vestibular aqueduct most common radiolographic abnormality Park et al. Laryngoscope 2000

  35. The Role of Imaging for Idiopathic Sensorineural Hearing Loss (SNHL) • Identification insight other genetic conditions (e.g. Pendred’s syndrome) • Certain inner ear anomalies associated with progressive sensorineural hearing loss or meningitis (e.g. EVA, cochlear hypoplasia) • Early counseling regarding contact sports, genetic testing

  36. Role of MRI vs CT scan: • Controversial • CT scan – faster to obtain, fewer problems with insurance, bony detail • MRI- assess cochlear nerve (cochlear implantation) • MRI – more sensitive detection of EVA?

  37. Role of MRI vs CT scan: From Greinwald (2006)

  38. The Role of the Geneticist in HAC: • What is the role of the geneticist? • What are the key aspects of a genetics evaluation and testing? • What is the value of a making a genetic diagnosis?

  39. The parents have questions… • What caused this? • Will it happen again? • Will our child develop other medical problems? Can those problems be treated? • Will my child's hearing loss stay the same or get worse? • What can we do about it?

  40. Congenital Deafness

  41. Infant with hearing loss ENT, audiology, eye exam consider EKG, CT / MRI Known environmental cause, e.g. CMV No known environmental cause Physical anomalies/ signs  syndrome No other signs If positive: diagnosis Do Cx26/Cx30 Family history Positive Negative Cryptogenic Hearing loss AD, AR X-linked Do confirmatory testing Other testing Modified from Rudolph’s Pediatrics

  42. Step 1: Is there evidence for acquired hearing loss? • Prematurity (2-5% of NICU graduates) • Birth wt <1500g • Jaundice • Gentamycin • Intracranial bleeding • ECMO • Low APGAR scores • In utero infections : CMV, Rubella, toxoplasmosis

  43. Step 1: Is there evidence for acquired hearing loss? • Basic screening Labs: • Urine/saliva -- culture • Blood -- serology • DNA -- PCR • Further investigation: • CT: periventricular calcifications • Eye exam: CMV chorioretinitis Univ Toronto, Dept of Ophthalmology

  44. Step 2: Syndromic or Nonsyndromic? • Syndrome: A pattern of multiple primary malformations (or dysfunctions) resulting from a single underlying cause. • Is this an isolated problem? Or is this an element of a more widespread condition?

  45. Case • Growth retardation • Squared-shaped auricles • Coloboma • Cochlear abnormalities • CHARGE syndrome • CHD7 mutations in 60%

  46. Case Histories:

  47. Case • SGA, microcephaly • Atretic ear canals • Wide nasal root • Broad forehead • Down turned corners of the mouth • Deletion 18q • ~30% of syndromic cases were chromosomal

  48. Step 3: Consider a genetic cause of nonsyndromic hearing loss • 15% of all bilateral prelingual hearing loss is caused by mutations in the GJB2 (Cx26) gene • ~150 other single gene causes of nonsyndromic hearing loss No signs of syndrome If positive: diagnosis Do Cx26/Cx30 Family history Positive Negative Cryptogenic Hearing loss AD, AR X-linked Other testing

  49. What is gained by a positive genetic diagnosis? • Direct (or avoid) further diagnostic testing • No CT or MRI if Cx26 mutation found • Define recurrence risk • Autosomal recessive? Sporadic? • Predict the clinical course • Progressive? • Associated deficits (blindness) or malformations?

  50. Pendred’s syndrome: • Autosomal recessive condition associated with SNHL, goiter • Gene associated with transport chloride and iodide within the ear and thyroid gland • 1/3 patients with ELV have mutation for Pendred syndrome (SLC26A4 gene) • Risk for progressive SNHL, vestibular dysfunction and goiter

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