From per ENT to HAE: Pulling the trigger on conductive hearing loss Team Katie, Au.D. MAC 2015 Pediatric Grand Rounds

T: 9/28/11 13-years-old Passed newborn hearing screening Normal speech and language development No developmental delays Using FM and strategic seating in school Past medical history – Chronic otitis media – “several” sets of tubes placed starting at 1-year-old – Bilateral cholesteatomas removed in 5 th & 6 th grade

General note All audiograms in presentation used a conventional method of testing with good reliability Audiology visits always preceded ENT visits

T: 9/28/11 Audiology plan BEFORE ENT – Retest per ENT – HAE to discuss management options Medical management in office – Cerumen removed AFTER audio Medical management plan – CT scan and f/u in October Audiology plan AFTER ENT – Retest hearing in October coordinated with ENT visit

T: 10/26/11 Audiology plan – HAE discussed if next round of medical management did not resolve air conduction thresholds ENT plan – CT scan visualized bilateral cholesteatomas – Left tympanomastoidectomy, left tube removal, and right tube removal scheduled 12/23/11 – Discussed T would need a second surgery for his right ear

T: 2/08/12 Audiology plan – Discussed continuing with classroom accommodations and monitoring auditory fatigue – Retest post next surgery ENT plan – Left ear reported healing well post-operatively – Scheduled right tympanomastoidectomy for 6/29/12

T: 5/09/12 Audiology only Plan – Per ENT – Retest after procedure on 6/29/12

T: ENT Encounters 6/29/12 Right tympanomastoidectomy Right tragal cartilage harvest Left granulation tissue posterior to the tube and debris in the ear canal that was removed 7/09/12 Healing well post- operatively Plan – Follow-up in 4-6 months with audiogram

T: 8/22/12 Audiology plan – Discussed the importance of reporting difficulties hearing in classroom setting ENT plan – Right ear tympanomastoidectomy planned for 3/04/13

T: 4/17/13 Audiology plan – Discussed scheduling a HAE ENT – Referred him to another UofM ENT for consultation

T: 4/23/13 Hearing aid evaluation Selected Oticon Safari 600 BTEs Skeleton earmolds in his high school colors Hearing aid fitting scheduled on 5/23/13

T: Today Reports he benefits from hearing aids, especially in school Wears full time, even under football helmet Has continued to have fluctuating conductive hearing loss Periods where he would only wear one hearing aid – ear would be draining – s/p surgery Going to college next year out of state

Review Almost 20 months between first audiogram at UofM and hearing aid fitting 5 audiologists Audiology recommendations not taken into consideration by ENT

E: 09/12/11 1 month old Referred to ENT by plastic surgery for debridement of EAC’s Failed AABR outside facility Deformed pinnae and stenosis of both EAC’s ABR at U of M showed – R) moderately severe – L) mild moderate – Bone conduction: (click) 20 dB Flat tympanograms with small volume (1000 Hz)

General Note Point of Entry: Plastic Surgery ENT Parents primary concern was the shape of the ear All Audiology visits preceded ENT visits (with the exception of the first visit)

ABR Impressions and Recommendations It is noteworthy that the size and shape of Emma's right ear canal may have had an impact on the ABR results. Implications of Emma's hearing loss were discussed and her mother was actively engaged in the conversation. We discussed the importance of monitoring speech and language development. Follow-up with Pediatric Otolaryngology and Audiology as planned. Appropriate management will be initiated. Emma's mother expressed concern regarding middle ear pathology. She finished the Cipro drops treatment last week but desires to see Pediatric Otolaryngology to follow-up. She scheduled an add-on follow-up appointment for this afternoon.

E: 10/19/11 Audiology Visit 2 nd ABR – R) moderate to severe – L) moderate – Prolonged wave V latencies with normal interpeak latencies. Impressions: “I suspect that Emma's stenotic ear canals and otorrhea have influenced the validity of her current and previous ABR studies. At this time, it is a challenge to reliably ascertain the degree of conductive involvement, as air conduction thresholds may be spuriously altered by inadequate transducer insertion depth.”

10/19/11 Otolaryngology Visit “Today's external auditory canals were full of debris bilaterally, prohibiting examination or visualization of the tympanic membrane. Mother was given directions for hydrogen peroxide and water irrigations to help debride the canals”

Hmmmm??? Multiple factors contributing to hearing loss – Debris in canal – Size of external auditory canals – Unknown middle ear status re: middle ear effusions and/or ossicular abnormalities – Low set ears???? – Preauricular tag???

E: 2/15/12

E: 4/12/12

E: 5/16/12

E: 6/19/12 “Otoscopy was performed before testing commenced and showed TM's clear to inspection bilaterally. The ABR was conducted while Emma while was in a natural sleep state. “ – Right: severe – Left : Moderately severe – Masked Bone Conduction: Right * 20 dB (* patient startled whenever stimulus was turned on) Resumed use of ponto on soft band Attempted use of a traditional BTE device

E: 10/18/12 BAHA fitting Own device BP 100

E: 11/16/12

E: 3/14/13

E: 12/03/13

E: 1/20/14 Hearing aid fitting Oticon Sensei Pro BTE for left ear

E: 6/12/14 BAHA softband on right side Traditional Oticon Sensei Pro BTE on left side Early Intervention Auditory Verbal Evaluation Pre-school Parents are still considering genetic testing and imaging

E: 3/12/15

Discussion When should children with middle ear pathologies transition from “ENT patients” to “managed patients”? How do educate ENT’s on “interim” amplification options?