Why Pediatric Hearing Clinic

Transcription

1 Why Pediatric Hearing Clinic RICHARD KANG, M.D., ASHLEY KAUFMAN, AU.D. AND MALLORY AUCH, RN Our Staff: Mallory Auch, RN Nurse Coordinator Kristen Janky, PhD, AuD, CCC-A Vestibular Audiologist Richard Kang, MD Otolaryngologist Ashley Kaufman, AuD, CCC-A Audiologist Sarah Maaske, MSW, MPA Social Worker Kristal Platt, MS Genetic Counselor/Vision Program Coordinator Susan Pryor Clinic Coordinator Kayla Samuelson, MA, CCC-SLP Speech Pathologist Sebastian Troia, MD - Ophthalmologist What do we know about children with hearing loss? Outcomes for Children with Hearing Loss (OCHL) longitudinal, multi-center study of audiological outcomes, speech and hearing outcomes and early intervention University of Iowa, University of North Carolina and Boys Town National Research Hospital 317 children who are hard of hearing (CHH) and 117 children with normal hearing Primarily bilateral, permanent mild to severe hearing loss Looked at hearing thresholds, if the child used hearing aids, how often they used hearing aids, how well the hearing aids were fit, linguistic experience and early intervention services 1

2 Some of the OCHL study conclusions 1) Children with mild-to-severe HL are at risk for depressed language development & the risk increases with severity of unaided HL 2) Provision of well-fit HAs reduces the risk of language delay & greater aided audibility is associated with better language outcomes in preschool 3) Early HA provision results in better early language outcomes, but later-fit children demonstrated accelerated growth patters once aided 4) Consistent HA use provides some protection against language delay & supports auditory development 5) Early intervention, quality of parent linguistic input & quantity of parent linguistic input positively impacts speech and language development Additional effects of hearing loss Children with unilateral and/or mild bilateral hearing loss are at greater risk for repeating a grade Children with hearing loss have a higher prevalence of vestibular loss than those with normal hearing Vestibular loss can result in poor gait, delayed gross motor development and poorer dynamic visual acuity which can affect reading skills (Janky & Givens, 2015) Evaluation of Pediatric Hearing Loss Often times Parents and families hope for a cure or that one day their child will no longer need the hearing aids, intervention, support The parents are provided with limited information and have not been given a review of the big picture Child is only followed by their pediatrician or primary care physician and their audiologist However, we know now that permanent pediatric hearing loss: Impacts communication (speech and language), cognitive and social development and therefore requires multiple specialists to be involved Is not curable presently, life-long management needed 2

3 Patient and Family Centered Evaluation of Pediatric Hearing Loss Pediatric Otolaryngologist Audiologist Social Worker Care Coordinator Patient & Family Geneticist/ Genetic Counselor Speech Language Pathologist Vestibular Audiologist Pediatric Ophthalmologi st Other Specialists: Pediatrician Primary Care Physician Neurology Purpose of the Pediatric Hearing Clinic Work as a multi-disciplinary team with the family to provide the information and support needed for the family to manage the life-long care of their child s hearing loss. Audiology Type Conductive Mixed Sensorineural Severity Unilateral vs. bilateral Mild through profound Progressive Intervention Amplification Early intervention services 3

4 Type of Hearing Loss Type of Hearing Loss Conductive and Mixed Hearing Loss Treacher-Collins, CHARGE Syndrome, Stickler Syndrome, Branchio-Oto-Renal (BOR) Syndrome Sensorineural Hearing Loss Waardenburg, Usher Syndrome, Pendred, Jervell & Lange-Nielson, Neurofibromatosis Type II, Alport Syndrome Severity 4

5 Audiology Test Battery Physiological Tests ABR DPOAEs 0-6 Months 6-24 Months Visual Reinforcement Audiometry (VRA) Conditioned Play Audiometry (CPA) Months Objective Audiology Tests Immittance Measures Tympanometry Measure of tympanic membrane compliance Otoacoustic Emissions (OAEs) Screening measure to determine the presence or absence of hearing loss Acoustic Reflexes Measure of stapedial reflex pathway and auditory nerve function Objective Audiology Tests Auditory Brainstem Response Sticker electrodes are placed on the forehead and behind the ears. Soft inserts are placed in the ears to play tones at different frequencies. The volume of the sound is adjusted to evaluate the softest level where a response from the auditory nerve can be recorded. Determines hearing sensitivity of both ears. 5

6 Behavioral Audiology Tests Visual Reinforcement Audiometry (VRA) Conditioned Play Audiometry (CPA) Conventional Audiometry Audiological Intervention Amplification What amplification devices are used? Are they in good working condition? Do the earmolds fit? How often are they used? Data-logging Does the device provide appropriate audibility? Real-ear verification Early Intervention What services is the child receiving and how often? Parental confidence in hearing aid controls and use Are the parents connected with other parents or support groups if needed? Guide by Your Side program Hearing Aid Verification Fit to manufacturer First Fit (Mueller, 2014) 6

7 Vestibular Testing Rotary Chair Primary test for children 0-3 years old Caloric Testing ~ 7 years old and above Video Head Impulse Test > 3 years old Vestibular Evoked Myogenic Potentials > 3 years old Rotary Chair Testing Vestibular assessment for children < 5 years Children sit in a parent s lap and rotate in the dark. Infrared camera is used to monitor alertness while electrodes measure nystagmus. Vestibular Evoked Myogenic Potential (VEMP) Children of all ages can complete VEMP testing There are two types of VEMPs: Ocular and Cervical. During VEMP testing, sticker electrodes are placed on the neck and under the eyes, and children either turn their heads or look up while listening to loud clicking sounds. Ocular VEMP Cervical VEMP 7

8 Caloric & Video Head Impulse Test (vhit) Caloric Testing Warm or cool water is gently run in the child s ears while goggles are used to measure eye movements. Caloric testing is only completed in older children. vhit The child wears a pair of tight fitting goggles while their head is moved quickly back and forth. Vestibular Loss Associated with Hearing Loss All can result in unilateral or bilateral vestibular loss: Cytomegalovirus (CMV) infection (30 60%) Auditory Neuropathy (show decline in function over time) Waardenburg Syndrome (up to 70/80%) Rubella (30 40%) Pendred Syndrome (Mondini and LVAS) Inner Ear Malformations GJB2 (Connexin 26 mutations) - the most common cause of non-syndromic deafness Usher Syndrome Meningitis Ototoxicity Prevalence of Vestibular Loss with Hearing Loss Vestibular loss estimated between 30 91% (Shinjo et al, 2007; Tribukait et al., 2004; Zhou et al., 2009) Prevalence higher: 1) as severity of HL increases, 2) in post-natally acquired cases, 3) with some syndromic deafness and 4) in labyrinth dysplasia (Angeli et al. 2003) Prevalence of vestibular loss in children who are cochlear implant candidates [Jacot et al. (2009), Jin et al. (2006), Cushing et al (2013), Janky et al. (2015)] 50% have some degree of vestibular loss 20 30% have bilateral loss Vestibular Loss Normal Vestibular Vestibular Loss Bilateral Vestibular Loss 8

9 Predictive Factors for Vestibular Loss in Children with Hearing Loss Kristen L. Janky, Megan L.A. Thomas, Robin R. High, Kendra K. Schmid & Oluwaseye Ayoola Ogun Purpose of this study: to determine if there are factors that predict whether a child with hearing loss also has vestibular loss. 186 charts of children with hearing loss were retrospectively reviewed Neuro-Sensory Genetics: 69 children with hearing loss [35 males, mean age 55 months (range 2 mos to 14 yrs)]. CI candidacy: 117 children with hearing loss [54 males, mean age 23 months (range 4 mos to 11 yrs)] Each child s medical chart was reviewed to obtain the following data: Vestibular Loss Severity Degree of Hearing Loss Imaging abnormalities Parental Concerns for gross motor skills Co-morbidities Developmental Profile- 3 (DP-3) Janky et al. Continued Findings: Both age to sit and age to walk were significantly increased with vestibular loss severity Degree of hearing loss significantly increased with the severity of hearing loss Significantly higher proportion of parents reporting concern for gross motor skills as severity of vestibular loss increased Speech Language Pathology Focus with infants and young babies: Developmental screeners and talking with the family about early intervention services Help the family process their emotions and what is happening with the new diagnosis of hearing loss Focus with toddlers and preschool children: Looking more at language assessments Speech perception tests to break out any areas of weakness Relating assessment results to any speech services the patient is receiving 9

10 Speech Language Assessments Infants and Toddlers Ages and Stages Questionnaire (ASQ) Communication and Symbolic Behavior Scales Development Profile (CSBS-DP) Language Development Scales Toddlers and Preschoolers Fluharty Preschool Speech and Language Screening-2 Preschool Language Scales-5 Spanish School Age Clinical Evaluation of Language Fundamentals -5 Assessment of Children s Language Comprehension Speech Language Pathology If further questions about the role of speech pathology in our Pediatric Hearing Clinic contact: Kayla Samuelson, M.A., CCC-SLP kayla.samuelson@boystown.org Pediatric Otologist Identify the type and degree of hearing loss and review it s implications Sensorineural, conductive, mixed Auditory neuropathy-dyssynchrony spectrum disorder Mild, moderate, severe, profound, unilateral or bilateral Determine cause of hearing loss if possible Interventions recommended for best outcome Who and where are the resources 10

11 Congenital SNHL Etiology Genetic-nonsyndromic 34% Acquired-delayed 37% Genetic-syndromic 16% Acquired-neonatal 13% Genetics ~70% non syndromic At this point, no specific therapy directed genetic mutations that cause hearing loss Multiple gene panel test ideal in most of the cases Congenital CMV Could be the most common single cause of hearing loss (up to 21%) Must be tested and diagnosed within first 3-4 weeks after birth Hearing loss of all types, most common sequelae Oral antiviral therapy (valganciclovir) for 6 month found to be effective in decreasing hearing loss and neurocognitive development Best to be preventative, awareness by pregnant moms 11

12 Pediatric Ophthalmology High refractory correction association with hearing loss (over 50%) Examples of syndromes with both hearing loss and vision loss: Usher s syndrome CHARGE syndrome Care Coordinator Help families navigate their care through medical and school system Pediatrician/PCP ideal but have difficulty keeping up with the latest information This is the case even for many of otolaryngologist offices. Who should be referred to the clinic? Infants and children with permanent unilateral or bilateral conductive, mixed or sensorineural hearing loss. If the child has auditory neuropathy spectrum disorder (ANSD) it is helpful to have behavioral audiometric data if possible Children and families who are interested in trying to determine the cause of the hearing loss Families from communities with limited resources that may need help connecting with physicians, specialists and long-term support When in doubt refer! 12

13 How do you make a referral? To make a referral to the clinic: Call or contact Sue Pryor at susan.pryor@boystown.org. Location: 555 North 30th Street, Omaha, NE, Times: 1 st and 3 rd Friday of every month from 8:00AM to 12:00PM Resources Janky et. al. (2018). Predictive Factors for Vestibular Loss in Children with Hearing Loss. American Journal of Audiology, 27(1), Janky, Kristen & Givens, Diane. (2015). Vestibular, Visual Acuity and Balance Outcomes in Children with Cochlear Implants: A Preliminary Report. Ear and Hearing, 36(6), Outcomes of Children with Hearing Loss; Ear and Hearing, Volume 36, Supplement 1,