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Hearing Loss

Hearing Loss. Peter Rigby M.D. Department of Otolaryngology Head and Neck Surgery Louisiana State University Health Sciences Center, New Orleans. Strategies in Patient Management. Review and compare the clinical presentation of hearing loss for children and adults

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Hearing Loss

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  1. Hearing Loss Peter Rigby M.D. Department of Otolaryngology Head and Neck Surgery Louisiana State University Health Sciences Center, New Orleans

  2. Strategies in Patient Management • Review and compare the clinical presentation of hearing loss for children and adults • Review diagnostic workup for new onset hearing loss • Review initial management for new onset hearing loss

  3. Strategies in Patient Management OLD YOUNG

  4. Strategies in Patient Management SENSORINEURAL CONDUCTIVE

  5. NORMAL LEFT EAR

  6. Epidemiology • Congenital: • unilateral or mod 50/1000 • Profound SNHL 4/1000 • Genetic SNHL 1/1000 • Adult SNHL (age 65) 166/1000 • Hereditary SNHL 27/1000 • Mature SNHL (age 80) 500/1000 • CHL 10/1000

  7. Strategies in Patient Management Child with newly discovered hearing loss: Infant screening Language delay Difficulty in school

  8. Genetic Aquired Unknown Congenital Hearing Loss Etiology of Congenital Hearing Loss

  9. Epidemiology • Congenital Infection: • Cytomemalovirus • *found in 1-2% of all live births • Rubella *progressive hearing loss • Syphilis • measles (rubeola), mumps, toxoplasmosis, herpes simplex

  10. Epidemiology • Neonatal Infection: • bacterial meningitis • *7% of all childhood hearing loss • pneumococcus • NeisseriaHaemophilus influenza

  11. Mitochondrial AR AD Sex Linked Congenital Hearing Loss Inheritance of Genetic Hearing Loss

  12. Recessive skip generations HF SNHL birth to one year Dominant every generation variable pattern HL second to third decade Genetic Hearing Loss

  13. Maternal Infection rubella CMV toxoplasmosis medications Birth History hypoxia kernicterus toxemia prematurity medications Pregnancy History

  14. Infection meningitis measles mumps syphilis medications Otologic history ototoxic exposure noise trauma head trauma medications Postnatal History

  15. Two Generations: hearing loss hearing aids balance problems Consanguinity test all siblings Family History

  16. Strategies in Patient Management Adult with hearing loss: Sudden loss Progressive loss Vertigo

  17. Hypercoag lipids BCP’s atherosclerosis arthritis medications Vascular diabetes HTN CAD CVA medications Medical History

  18. Noise trauma guns military machinery Medical History

  19. General stature milestones pigmentation hypogonadism craniofacial Ophthalmologic keratitis - syphilis, Cogans retinitis pigmentosa - Ushers cataracts - NF2 , rubella inclusions - CMV, toxo visual acuity Physical Exam

  20. Otologic trauma malformations cholesteatoma infection Head and Neck orbit - Apert, Crousan mid face - Treacher Collins, Digeorge, Goldenhar mandible - Pierre Robin neck- brachio-oto-renal, Pendred Physical Exam

  21. 0.125k 0.25k 0.5k 1k 2k 4k 8k 0 10 20 30 40 50 60 (VERTEX) 70 LEFT RIGHT 80 AIR 90 BONE- MASKED 100 BONE- UN MASKED Audiogram: left conductive loss

  22. 0.125k 0.25k 0.5k 1k 2k 4k 8k 0 10 20 30 40 50 60 (VERTEX) 70 LEFT RIGHT 80 AIR 90 BONE- MASKED 100 BONE- UN MASKED Audiogram: left sensorineural loss

  23. 0.125k 0.125k 0.25k 0.25k 0.5k 0.5k 1k 1k 2k 2k 4k 4k 8k 8k 0 0 10 10 20 20 30 30 40 40 50 50 60 60 70 70 LEFT LEFT RIGHT RIGHT (VERTEX) 80 80 AIR AIR 90 90 LOUDER! BONE- MASKED BONE- MASKED LOUDER! 100 100 BONE- UN MASKED BONE- UN MASKED Weber test: left hearing loss SENSORINEURAL CONDUCTIVE

  24. 0.125k 0.125k 0.25k 0.25k 0.5k 0.5k 1k 1k 2k 2k 4k 4k 8k 8k 0 0 10 10 20 20 30 30 40 40 50 50 60 60 70 70 LEFT LEFT RIGHT RIGHT (VERTEX) 80 80 AIR AIR 90 90 BONE- MASKED BONE- MASKED 100 100 BONE- UN MASKED BONE- UN MASKED Rinne test: left hearing loss SENSORINEURAL CONDUCTIVE LOUDER! LOUDER!

  25. Audiogram: presbycusis 0.125k 0.25k 0.5k 1k 2k 4k 8k 0 10 20 30 40 50 60 (VERTEX) 70 LEFT RIGHT 80 AIR 90 BONE- MASKED 100 BONE- UN MASKED

  26. Renal Function BUN Creatinine Urinalysis: Hematuria Proteinuria Alport’s, polycystic kidney disease Metabolic TFT’s - Pendred, cretinism glucose - diabetes CBC - infection, discraisas Laboratory Testing

  27. Immune Sed rate ANA, RF Western Blot: Connexin 26 Cogan’s 68 kd Protein Serology RPR/ FTA-ABS TORCH Toxoplasmosis Rubella cytomegalovirus herpes Laboratory Testing

  28. ECG prolonged QT peaked T Jervell and Lange-Neilson CT temporal bones mondini vestibular aqueduct cochlear aqueduct cholesteatoma osteodysplasia osteogenesis imperfecta, Stickler, Laboratory Testing

  29. Laboratory Testing • MRI temporal bones • Acoustic neuroma • Facial schwannoma • Multiple sclerosis • Cholesteatoma • encephalocele • Central axis tumors

  30. Information cause progression family risk family testing resources Treatable causes PLF immune hydrops otosclerosis tumor hypercoag Intervention

  31. Systemic Rx eyes cardiac vascular renal thyroid tumor Hearing Rx seating amplification vision qued speech cochlear implants sign Intervention

  32. Ossicular Fixation • Audiogram • CT- Coronal • ossicles, middle ear size, oval window, facial nerve, otic capsule, IAC • vestibular aquaduct • cochlear aquaduct • Watch out for SNHL, balance symptoms

  33. Stapes Fixation • juvenile otosclerosis • Treacher Collins • Klippel-Feil • Pfeiffer • branchio-oto-renal • ear pits-deafness • cervico-acoustic syndromes • Crouson • X linked mixed deafness with gusher • osteogenesis imperfecta

  34. Atresia • 1:10,000 - 1:20,000 • 1/3 bilateral • usually asymmetric defects

  35. Atresia: Timing • Bilateral Atresia: • Audiometric evaluation: early • Amplify: early • Auditory/Speech assistance in school • CT age 4-5 • Repair age 6 • Unilateral Atresia: • Audiometric evaluation: early • Amplify: usually not accepted • CT age 4-5 (also screen for canal cholest) • balance need/timing of repair with risks

  36. Herpes zoster oticus

  37. Polyp in ear canal

  38. Acute otitis media

  39. tympanosclerosis

  40. cholesteatoma

  41. cholesteatoma

  42. Chronic otitis media Cholesteatoma tympanosclerosis

  43. aspergillosis

  44. Basal cell carcinoma

  45. Squamous Cell carcinoma

  46. Squamous Cell carcinoma

  47. Conclusion • A stepwise approach to hearing loss evaluation aids in identification of etiology without excessive testing • Workup should be tailored to age, history, and early examination of the ear • Workup should be directed at treatable causes

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