49
Hearing Loss Department of Otolaryngology Head and Neck Surgery Louisiana State University Health Sciences Center, New Orleans Peter Rigby M.D.
Hearing Loss
Department of Otolaryngology Head and Neck Surgery
Louisiana State University Health Sciences Center, New Orleans
Peter Rigby M.D.
Strategies in Patient Management1. 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
Strategies in Patient Management
YOUNGOLD
Strategies in Patient Management
CONDUCTIVE SENSORINEURAL
NORMALLEFT EAR
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
Strategies in Patient Management
Child with newlydiscovered hearing loss:
Infant screeningLanguage delayDifficulty in school
Congenital Hearing LossEtiology of Congenital Hearing Loss
GeneticAquiredUnknown
Epidemiology Congenital Infection:
– Cytomemalovirus
– *found in 1-2% of all live births
– Rubella *progressive hearing loss
– Syphilis
– measles (rubeola), mumps, toxoplasmosis, herpes simplex
Epidemiology Neonatal Infection:
– bacterial meningitis– *7% of all childhood hearing loss– pneumococcus– Neisseria Haemophilus influenza
Congenital Hearing LossInheritance of
Genetic Hearing Loss
MitochondrialARADSex Linked
Genetic Hearing Loss Recessive skip generations HF SNHL birth to one year
Dominant every generation variable pattern HL second to third decade
Pregnancy History
Maternal Infection
rubella CMV toxoplasmosis medications
Birth History hypoxia kernicterus toxemia prematurity medications
Postnatal History
Infection meningitis measles mumps syphilis medications
Otologic history ototoxic exposure noise trauma head trauma medications
Family History
Two Generations:
hearing loss hearing aids balance problems
Consanguinity test all siblings
Strategies in Patient Management
Adult with hearing loss:
Sudden lossProgressive lossVertigo
Medical History
Hypercoag lipids BCP’s atherosclerosis arthritis medications
Vascular diabetes HTN CAD CVA medications
Medical History
Noise trauma guns military machinery
Physical Exam
General stature milestones pigmentation hypogonadism craniofacial
Ophthalmologic keratitis - syphilis, Cogans
retinitis pigmentosa - Ushers
cataracts - NF2 , rubella
inclusions - CMV, toxo
visual acuity
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
Audiogram: left conductive loss
100
90
80
70
60
50
40
30
20
10
08k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT (VERTEX)
Audiogram: left sensorineural loss
100
90
80
70
60
50
40
30
20
10
08k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT (VERTEX)
100
90
80
70
60
50
40
30
20
10
0
8k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT
100
90
80
70
60
50
40
30
20
10
0
8k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT(VERTEX)
LOUDER!LOUDER!
CONDUCTIVE SENSORINEURAL
Weber test: left hearing loss
100
90
80
70
60
50
40
30
20
10
0
8k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT
100
90
80
70
60
50
40
30
20
10
0
8k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT(VERTEX)
CONDUCTIVE SENSORINEURAL
Rinne test: left hearing loss
LOUDER!
LOUDER!
Audiogram: presbycusis
100
90
80
70
60
50
40
30
20
10
08k4k2k1k0.5k0.25k0.125k
BONE- UNMASKED
BONE- MASKED
AIR
RIGHTLEFT (VERTEX)
Laboratory Testing Renal Function BUN Creatinine Urinalysis:
– Hematuria
– Proteinuria– Alport’s, polycystic kidney
disease
Metabolic TFT’s - Pendred,
cretinism
glucose - diabetes
CBC - infection, discraisas
Laboratory Testing 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
ECG prolonged QT peaked T
– Jervell and Lange-Neilson
CT temporal bones mondini vestibular aqueduct cochlear aqueduct cholesteatoma osteodysplasia
– osteogenesis imperfecta, Stickler,
Laboratory Testing
MRI temporal bones Acoustic neuroma Facial schwannoma Multiple sclerosis Cholesteatoma encephalocele Central axis tumors
Intervention Information cause progression family risk family testing resources
Treatable causes PLF immune hydrops otosclerosis tumor hypercoag
Intervention Systemic Rx eyes cardiac vascular renal thyroid tumor
Hearing Rx seating amplification vision qued speech cochlear implants sign
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
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
Atresia 1:10,000 - 1:20,000 1/3 bilateral usually asymmetric
defects
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
Herpes zoster oticus
Polyp inear canal
Acute otitis media
tympanosclerosis
cholesteatoma
cholesteatoma
Chronic otitis mediaCholesteatomatympanosclerosis
aspergillosis
Basal cell carcinoma
SquamousCellcarcinoma
SquamousCellcarcinoma
Conclusion1. 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
