Ears Flashcards
Cranial Nerves
I Olfactory
V Facial sensation
VII Facial movement, tears, taste, acoustic reflex
VIII Hearing and Equilibrium
IX Taste
X Taste
XI Taste, swallowing
XII Tongue movements
Conductive hearing loss
Refers to impairment of the outer and/or middle ear conductive mechanism only.
Sensory hearing loss
Refers to damage to the cochlea (outer hair cells or outer and inner hair cells).
Mixed hearing loss
Refers to the presence of both conductive and sensory impairment.
Neural hearing loss
Refers to damage to the auditory neurons (spiral ganglia) and/or the auditory branch of the eighth nerve.
Auditory Neuropathy and dysynchrony are examples of neural hearing loss.
Central hearing loss
Refers to damage to auditory structures in the brainstem, thalamo-cortex and/or cortex.
How Common is Hearing Loss
- Profound
- 1-2 hearing loss in 1000 infants
- Significant hearing loss
- 1-2 have hearing loss that will impact on their well being and education
- 50% have no known risk
- 12% of NICU graduates develop some form of sensorineural hearing loss
- Newborn screening
- Otoacoustic emission (like a tympanogram listens for action potential of the cochlea (ear talk) and that is what it picks up—picks up cochlea function
- BAER more accurate (assesses wiring of auditory pathway—misses low and high frequency loss)
- Brain stem evoked response is the diagnostic test
- 33 babies are born every day with a hearing loss
- Hearing is about 30 among congenital defects
- 50% are contributed to genetic factors, likely to increase
Newborn Hearing Tests (2)
Otoacoustic Emission
Auditory Brainstem Response Training
Types of Hearing Loss
Conducive
Sensori-neural
Auditory Processing (Central)
Early Hearing Loss
- First trimester structure of ear highly metabolic and therefore exposure to metabolic and highly susceptible to illness and exposure to medication in the mother
- Maternal rubella
- Cytomegalovirus
- Genetic disorders leading to malformation of the cochlea
- Sepsis with prolonged acidosis
- Oxygen deprivation and hyperbilirubinemia
Universal hearing screening in the late 90’s
Mandated but not covered
Now linked to funding
Anatomic Early Hearing Loss
- Genetic Studies
- Meningitis
- Congenital hypothyroidism
- Medications—Lasix, aminoglycosides, vancomycin
Large Cochlear Aqueduct syndrome
- Onset of hearing loss 6 to 7 months with progressive worsening
- Balance is off, progressive loss
- Tends to stabilize at 50 decibels
- They need to not play football
Reasons for Hearing Loss
- Wax
- Foreign objects
- Deafness due to hyperbilirubinemia, CMV
- Cranial facial, tags
- Medications
- Deafness associated with aminoglycosides
Reasons for Hearing Loss
Genetic Syndromes
- Genetic Syndromes
- Deafness associated with white forelock and eye abnormalities
- Waardenburg syndrome
- Deafness associated with long QT syndrome
- Jervell Syndrome
- Lange Nielsen syndrome
- Ion channelopathies
- Progressive hearing loss
- 3% carrier rate (same of CF). Mild to moderate hearing loss
- Rest are autosomal dominant
- 80% are recessive
- Deafness associated with white forelock and eye abnormalities
History of Hearing Loss
- History of hearing loss is critical
- Looking at hearing loss early on
- Wear a hearing aide if they are younger than 60 year
- History of renal abnormalities (8 weeks in utero develop at the same time)
- Alport’s syndrome
- Genetic mediation for hearing loss in old age
Hearing Loss Progression
- Can progress throughout childhood
Gene Mutations associated with hearing loss
Both high- and low-frequency progressive hearing loss represent a wide diversity of gene mutations that are observed in a large number of syndromic and non-syndromic diseases
Human KCNQ4 mutations known as DFNA2 cause non-syndromic, autosomaldominant, progressive high-frequency hearing loss in which the cellular and molecular basis is unclear
Newborn screening
Repeated on follow up center
Goal: Early intervention by age 6 months
Genetic Screening
Syndromic vs. Non-syndromic
- Syndromic—1/3
- Non syndromic 2/3—mitochondrial defect
- Hereditary hearing loss may not be congenital
- Is it stable or progressive?
Non Genetic
CMV infections, high bili etc, trauma, meningitis, noise induce hearing loss
Conductive Hearing Loss
4 examples
- Eustachian tube dysfunction
- Ear fluid
- Hole in eardrum
- Fixed middle ear bone
Sensorineural Hearing Loss
4 examples
- Noise induced hearing loss
- Presbycusis
- Ménière’s Disease
- Tumors of the auditory nerve