Term
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Definition
Two unaffected "carrier" parents:
1:2 unaffected "carrier" child
1:4 unaffected "non-carrier" child
1:4 affected child |
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Term
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Definition
| etiology is most idiopathic, but viral disease, vascular insult, endocrine imbalance, allergies, cochlear damage, atrophy of the stria vascularis, or collapse of cochlear duct may be possibilities. |
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Term
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Definition
| Found to help with malaria and noctural leg cramps, and arthritis. Found in many types of tonic water. Can cause permanent damage to auditory system (has cochleotoxic effects) |
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Term
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Definition
| Albinism, hereditary piebaldism (strips of hyper/hypopigmentation), lentigines (leopard-like spots), and Waardenburg syndrome (heterochromia irdis, flattening of nose bridge, and widely set eyes and maxilla) |
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Term
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Definition
| Recessive; 3-10% of Deaf children; characterized by retinitis pigementosa (slow, progressive, unilateral tapetorential degeneration of the retina and inner ear). May be accompanied by MR, epilepsy, vertigo. |
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Term
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Definition
| short stature; normal looking at birth, but abnormalities with age; CNS/PNS changes w/ MR; death usually occurs ~20-30 years of age. 1/2 of all patients have moderate sensorineural HL; bone/joint abnormalities; middle ear/cochlear ab. |
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Term
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Definition
| more aggressive form of Hunter's. Age of death ~10yo. May have some progressive HL. |
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Term
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Definition
| Extra chromosome at position #21. 1:600-1:700 births. 85% incidence of middle ear disorders; less mobile ossicular chain w/ HL; up to 50 different anomalies; MR, small rounded ears, slanted eyes, and flattened facial expression most common. |
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Term
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Definition
#1 Normal father, carrier mother:
25% carrier F, 25% affected M, 25% normal F, 25% normal M
#2 Affected father, normal mother:
50% normal M
50% carrier F |
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Term
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Definition
| 93% incidence of mother passing rubella to child in 1st trimester; child will have MR/congenital HL; rubella sets up home in child's middle ear; attacks CNS/PNS; can cause cerebral palsy and blindness; passed transplacentally or membranes of birth canal. |
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Term
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Definition
| Spread via bodily fluids (saliva, seminal fluid, blood, transplacental); can cause learning delays, CNS/PNS issues, HL; 80% of adult pop. can carry CMV |
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Term
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Definition
| a condition existing at birth and often before birth, or that develops during the first month of life (neonatal disease), regardless of causation. |
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Term
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Definition
| heterochromia irdis (alteration of eye color), flattening of nose bridge, widely set eyes and maxilla, alteration of hair color |
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Term
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Definition
| conditions acquired after birth, particularly if the cause is obscure, casual, or accidental. |
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Term
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Definition
| One cause of tinnitus might be a homeostatic response of central dorsal cochlear nucleus auditory neurons that makes them hyperactive in compensation to auditory input loss; Temporal lobe cells fire out of boredom when nerve fibers of the 8th CN do not fire off. |
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Term
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Definition
Affected father, Normal mother:
1:2 affected, 1:2 unaffected
If both parents are affected, 3:4 affected |
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Term
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Definition
| condition of the cochlea that makes intense sounds abnormally loud to a patient who cannot hear sounds moderate or lesser intensities; theory? when intensity is sufficient to stimulate hair cells adjacent to damaged area, neural excitation is initiated because healthy hair cells in the are will fire (more and more healthy cells fire than damaged) |
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Term
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Definition
| 2/3 of patients manifest dz. Inflames parotid gland and may enter inner ear via internal auditory meatus. May damage organ of Corti. Can lead to severe/profound unilateral HL. |
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Term
| Incidence of congenital HL in US |
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Definition
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Term
| 2 possible causes of Meniere's Dz |
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Definition
| Allergy, loss of electrolytes |
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Term
| 3 diseases not mentioned so far! |
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Definition
Alport Dz: nephritis, congenital HL;
Pendred Syndrome: goiter, HL @ birth or post-natal; Auditory neuropathy: change in VIII CN to brainstem, psuedo-HL, but cochlea is fine, fibers working wrong |
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Term
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Definition
| cochleotoxic/vestibulotoxic with tinnitus, vertigo, and HL. Used to help people with TB & meningitis |
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Term
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Definition
| general abx for children at birth; less ototoxic than streptomycin; HL in teens and adults |
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Term
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Definition
| Tonic water contains quinine which may cause HL. Quinine used as an antimalarial and for noctural leg cramps |
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Term
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Definition
| Known as aspirin; can cause HL and tinnitus; may have direct effect on outer hair cells of cochlea; hearing may improve 3 days after rx is stopped; If HL does occurs, it rarely exceeds 40dB loss |
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Term
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Definition
| Otocervical syndrome; shortness or absence of neck; limited mobility of head; primarily cochlear/inner ear malformations; autosomal dominant |
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Term
| Incidence of Deafness/HL resulting from rubella in the mother in the first trimester of pregnancy |
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Definition
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Term
| High Risk Register for Early Identification of HL |
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Definition
1. Family history of HL
2. In utero infection (CMV, rubella, herpes, etc.)
3. Craniofacial abnormalities
4. Birth weight <1500g (3.3lbs)
5. Hyperbilirubinemia (liver blood count)
6. Ototoxic meds
7. Bacterial meningitis
8. Apgar of 1-4 at 1 min./0-6 at 5 min.
9. Mechanical ventilation >5 days
10. Stigamata w/ syndrome including a HL |
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Term
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Definition
| No inner ear or cochlea present |
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Term
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Definition
| Sensorineural HL because of non-development of sensory receptors in cochlea |
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Term
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Definition
| Missing patches of receptors hairs in cochlea/ strange speech, HL |
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Term
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Definition
1. Integumentary
2. Skeletal
3. Ocular
4. Other |
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Term
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Definition
| Generally Deaf, may be blind |
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Term
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Definition
| Affects inner ear causing dizziness and unsteadiness; progressive HL; normally resolves self, but have permanent implications |
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Term
| AIDS/HIV/PAIDS in the auditory system |
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Definition
| Suppressed immune system can lead to otitis media or viral dzs |
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Term
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Definition
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Term
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Definition
| In temporal area, 70-80% of fractures result in HL |
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Term
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Definition
| Demylenation/atrophy of cells with possible HL |
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Term
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Definition
| Overproduction of endolympathic fluid from dura mater into cochlear duct towards Reisner's and basiler membranes |
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Term
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Definition
| Slow release abx from fat/ unilateral HL |
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Term
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Definition
| (Neosporin) similar effects as streptomycin if given IV; used for bacterial infection |
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Term
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Definition
| Derivate of streptomycin; causes vestibular issues, but not dizziness |
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Term
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Definition
| Unusual; can anesthetize auditory/vestibular systems |
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Term
| Fetal Alcohol Syndrome (FAS) |
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Definition
| Can cause HL, blindness, MR, physical retradation |
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