Hearing Loss

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Hearing Loss
Peter Rigby M.D.

• Department of Otolaryngology Head and Neck
  Surgery
• Louisiana State University Health Sciences
  Center, New Orleans

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Strategies in Patient Management

1. Review and compare the clinical presentation of
   hearing loss for children and adults
2. Review diagnostic workup for new onset hearing
   loss
3. Review initial management for new onset hearing
   loss

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Strategies in Patient Management
OLD
YOUNG
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Strategies in Patient Management
CONDUCTIVE
SENSORINEURAL
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NORMAL LEFT EAR
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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

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Strategies in Patient Management
Child with newly discovered hearing loss Infant
screening Language delay Difficulty in school
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Congenital Hearing Loss
Etiology of Congenital Hearing Loss
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Epidemiology

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

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Epidemiology

• Neonatal Infection
• bacterial meningitis
• 7 of all childhood hearing loss
• pneumococcus
• Neisseria Haemophilus influenza

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Congenital Hearing Loss
Inheritance of Genetic Hearing Loss
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Genetic Hearing Loss

• Dominant
• every generation
• variable pattern HL
• second to third decade

• Recessive
• skip generations
• HF SNHL
• birth to one year

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Pregnancy History

• Birth History
• hypoxia
• kernicterus
• toxemia
• prematurity
• medications

• Maternal Infection
• rubella
• CMV
• toxoplasmosis
• medications

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Postnatal History

• Otologic history
• ototoxic exposure
• noise trauma
• head trauma
• medications

• Infection
• meningitis
• measles
• mumps
• syphilis
• medications

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Family History

• Consanguinity
• test all siblings

• Two Generations
• hearing loss
• hearing aids
• balance problems

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Strategies in Patient Management
Adult with hearing loss Sudden loss Progressive
loss Vertigo
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Medical History

• Vascular
• diabetes
• HTN
• CAD
• CVA
• medications

• Hypercoag
• lipids
• BCPs
• atherosclerosis
• arthritis
• medications

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Medical History

• Noise trauma
• guns
• military
• machinery

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Physical Exam

• General
• stature
• milestones
• pigmentation
• hypogonadism
• craniofacial

• Ophthalmologic
• keratitis - syphilis, Cogans
• retinitis pigmentosa - Ushers
• cataracts - NF2 , rubella
• inclusions - CMV, toxo
• visual acuity

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Physical Exam

• 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

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Audiogram left conductive loss
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Audiogram left sensorineural loss
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Weber test left hearing loss
SENSORINEURAL
CONDUCTIVE
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Rinne test left hearing loss
SENSORINEURAL
CONDUCTIVE
LOUDER!
LOUDER!
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Laboratory Testing

• Renal Function
• BUN
• Creatinine
• Urinalysis
• Hematuria
• Proteinuria
• Alports, polycystic kidney disease

• Metabolic
• TFTs - Pendred, cretinism
• glucose - diabetes
• CBC - infection, discraisas

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Laboratory Testing

• Immune
• Sed rate
• ANA, RF
• Western Blot
• Connexin 26
• Cogans
• 68 kd Protein

• Serology
• RPR/ FTA-ABS
• TORCH
• Toxoplasmosis
• Rubella
• cytomegalovirus
• herpes

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Laboratory Testing

• CT temporal bones
• mondini
• vestibular aqueduct
• cochlear aqueduct
• cholesteatoma
• osteodysplasia
• osteogenesis imperfecta, Stickler,

• ECG
• prolonged QT
• peaked T
• Jervell and Lange-Neilson

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Laboratory Testing

• MRI temporal bones
• Acoustic neuroma
• Facial schwannoma
• Multiple sclerosis
• Cholesteatoma
• encephalocele
• Central axis tumors

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Intervention

• Treatable causes
• PLF
• immune
• hydrops
• otosclerosis
• tumor
• hypercoag

• Information
• cause
• progression
• family risk
• family testing
• resources

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Intervention

• Systemic Rx
• eyes
• cardiac
• vascular
• renal
• thyroid
• tumor

• Hearing Rx
• seating
• amplification
• vision
• qued speech
• cochlear implants
• sign

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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

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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

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Atresia

• 110,000 - 120,000
• 1/3 bilateral
• usually asymmetric defects

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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

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Herpes zoster oticus
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Polyp in ear canal
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Acute otitis media
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tympanosclerosis
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cholesteatoma
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cholesteatoma
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Chronic otitis media Cholesteatoma tympanosclerosi
s
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aspergillosis
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Basal cell carcinoma
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Squamous Cell carcinoma
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Conclusion

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