Basic Medical for Audiologists
Terms
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- Most genetic disorders are the result of?
- an individual gene or gene pair
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Gestational age starts from ?
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The first day of the last menstrual period
- embryonic week 4-8 known as
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organogenetic period.
-major internal and external structures form
- embryo is most susceptible to congenital abnormalities due to teratogen exposure during this period
- drugs, viruses & chemicals - When are congnital anomalies of the brain & spinal cord mosst likey to occur and what is the most common cause?
- Week 4 - this is when the neural tube is closing to form the top portion for the brain. If development is disrupted, can cause spina bifida
- The Fetal period extends from what week?
- from week 9 to birth
- Gestation day 22- what is happening developmentally to the ear
- The otic placode invaginates to form the Otic vessicle. It divides into ventral & dorsal components.
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The ventral component of the otic vessicle gives rise to ?
The dorsal component gives rise to: -
cochlear duct & saccule
the utricle ,semicircular canals &the endolymphactic sac - Reabsorption of mesodermal cartiage between the otic & membranous labyrinth forms?
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- perillymphatic space
-Reissners membrane
-the basilar membrane
-the stria vascularis - The osseous capsule develops from ?
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the cephalic mesoderm
- precartilagenous precursor (week 7)
- adult size by week 22 - pharyngeal grooves develop into
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- 4 of them on each side of head and neck
- 1st pair become external auditory meatus
-the others become cervical sinus & disappear with neck development - The trigeminal verve (V) is the primary nerve of?
- the head & neck- innervates the face, teeth, mucous membranes of the nasal cavity , the palate, mouth & tongue, the motor nerve for mastication muscles
- Some causes of tinnitus are thought to be?
- intracranial hypertension, depression, insects or foreign bodies in the ear canal
- Some warning signs for tinnitus are?
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vascular tumor
turbulent vascular flow
benign intracranial hypertension
myoclonus in palatal muscles - Acute Hearing loss, problems to consider?
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-Intercranial tumors
-Otitis media
secondary labyrintitis, labrinth fistula (round window
-autoimmune inner ear disease
-vascular occlusion
-Menieres disease
-trauma - perilymph fistula
temporal bone fracture, inner ear barotrauma, syphils
acute dizziness - Signs of ear pathology?
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-Hearing loss
-Tinnitus
-Otalgia
-Otorrhea
-facial palsy - ABR and EcochG provide info about?
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The integrity & synchrony of firing of INCs & VIII nerve
-synchrony of primary neurons - What is Aplasia?
- Failure to reach full development
- What is Michels aplasia?
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total absence of inner ear & VIII nerve
- can occur with norma outer & middle ear
-no hearing - What is Mondini's dysplasia?
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Anomalies of bony & membranous ducts of inner ear.
- only 1 coil of cochlea present - What is Scheibes aplasia-
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Dysplasia of membranous ducts of cochlea & saccule
- atrophy of the stria vascularis - Why refer is otalgia present?
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Could be due to: Otitis media complications
-meningitis
-superiosteal obscess
-epidermal empyema
-gradenigos syndrome
-brain abscess
-hydrocephalus
-malignant external otitis
-CPA tumors - Possible causes of facial Palsey?
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Bells - due to herpes simplex virus, herpes zoster oticus or Ramsay Hunt syndrome
-otitis media
-temporal bone fracture
- barotrauma - What is a syndrome?
- A pattern of abnormalities resulting from a single cause
- What is an association?
- A group of abnormalities occurring together more often than occurs by chance
- What is a sequence?
- Pattern of abnormalities resulting from a primary anomaly
- Syndromic hearing loss
- HL associated with malformation of external ear, other organs or other medical conditions
- Non syndromic HL
- HL not associated with other problems (other than possible ME or IE abnormalities)
- Non genetic HL caused by?
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-teratogens
-meningitis
-hyperbilirubinemia
-ototoxicity - Some autosomal dominant syndromic disorders
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BOR-
Crouzon
Neurofibromatosis 2
Osteogenesis Imperfecta
Stickler
Treacher Collins
Waardenberg - BOR (Branchio-otorenal syndrome) also know as Melnick-Fraser syndrome
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Major systems affected- auditory,craniofacial, renal, neck, and pulmonary
- ear pits on the auricle just above the tragus
-HL most common manifestation,either conductive,mixed or SN - Crouzon also known as craniofacial dysostosis
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major system affected-craniofacial
-bulging eyes, abnormal head shape, large forehead
-conductive HL, usually mild
-stenotic ear canals,articulation disorders,eustachian tube dysfunction - Neurofibromatosis 2 (NF2)Acoustic neuroma associated with NF2 but not NF1
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NF2 gene is a type of tumor suppressor gene, hence formation of various types of auditory tumors
- speech onset often delayed
-hearing loss present 50%,mostly unilateral,vestibular function impaired
-cafe au lait macules on skin - Osteogenesis Imperfecta- (Brittle bone)
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Bones that fracture easily,
Blue sclera, short stature, HL,scoliosis, weak muscles,tend to bruise easily, high pitched voice - Stickler Syndrome
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one of the most common connective dysplasia,often resulting in Robin syndrome
-cute,round face,depressed nasal root
child who is wearing thick glasses for myopia, has HFHL may have cleft palate may have this - Treacher Collins Syndrome
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multiple anomaly disorder,major system affected is craniofacial
- microtia,absence of eyelashes on inner 1/3 bottom lid associated with conductive hearing loss. Cond loss associated with fixation of the footplate of the stapes,atresia of ME and ossicles common - Waardenberg
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primarily known as syndrome of HL. 5 types.Type 1 most common.
-heterochromia, white forelock,white eyelashes,SNHL,vaginal hypoplasia may be uni or bilat, mild to profound - Some autosomal Recessive syndromic disorders
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Goldenhar
Jervell & Lange-Nielson
Pendred
Usher
X linked
Alport - Goldenhar syndrome (hemifacial microsomia)
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on of the most common craniofacial anomalies in man
-unilateral microtia, facial asymmetry, ear tags or pits, ocular dermoid cysts
-conductive HLusually unilat
-cleft palet,lip,microtia, anotia,clefts of eyelid,strabismus,small mouth, club foot - Jervell & Lange-Neilson syndrome
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Heart defects associated with SIDS
-deaf at birth
-heart problems
-long QT abnormality
-risks of faintin - Pendred Syndrome
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SN Hearing loss and Goiter
-HL bilateral may be progressive from mod to profound,usually onset early childhood but may be congenital may involve vestibular system. Mondini type anomaly of cochlea
-may have hoarsness (from goiter) - Alport syndrome
- effects men more, renal problems, occular cataracts, HFSNHL, deafness and renal failure by age 50
- Breakdown of Genetic HL
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syndromic vs non-syndromic
autosomal dominant
autosomal recessive
X linked -dom & rec
mitochondrial
new mutations - Purpose of Eustachian tube?
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maintains pressure & drains middle ear
normally closed opens when one yawns, swallows
can be blocked by swelling or fluid from upper resp infection, inflamed or enlarged adenoids, allergies, growths in nasopharynx, structural problems with tensor palatini muscle - Course of middle ear disease
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-ET dysfunction prevents ventilation of ME
-Air becomes absorbed by lining of ME cavity
-Air pressure becomes lower in ME vs outside
-negative pressure causes TM to be retracted
-ET not workingbcannot open well to relieve pressure
-negative pressure causes fluid to be drawn from lining of ME cavity - possible complications of Otitis Media
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-typmanosclerosis
-cholesteatoma
-mastoiditis
-ossicle disarticulation
-ruptured tympanic membrane
-meningits
-labyrinthitis - Complications of OM
- mastoiditis - infection spreads to mastoid air cells can lead to meningitis
- Complication of OM
- Labyynthinitis- perilymph fistula infection spreads to inner ear
- Immittance progression seen in OM
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Type A
Type C
Type B
- if canal volume normal, fluid present
- if volume large>2.5cc)perf or patent PE tube
Type c
Type A - Cholesteotoma - aka epidermoid inclusion cyst, keratoma
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long standing neg pressure causing TM retraction forms around pars flaccida.
Epithelial tissue travels through TM perf, forms cysts, can grow in the attic or ME, can affect ossicles, grow into mastoid, labyrinth. Can be life threatening. Must be removed surgically
can be congenital looks like small pearl in otherwise healthy looking ME - Audiometric results associated with OM
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- Conductive HL
- initially low freq
-Tent shaped curve best at 2000Hz (stiffness mass curve)
-progresses to flat HL - American Academy of Pediatrics guideline for management of Acute Otitis Media
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for children 2mths-12 years
- if no sign of systemic illness 2 options:
-observe 48-72 hours
-administer Amoxicillin
-if no improvement after 48-72
give antibiotic, if on antibiotic, change to different one. - T.O.R.C.H. stands for ?
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acronym for maternal infections
T=toxoplasmosis
O=other, syphilis,hepB HIV,Varicella zoster
R=Rubella
C=cytomegalovirus -blueberry
H=Herpes simplex - Characteristics of SNHL
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Affects inner ear &8th nerve
-no air/bone gaps
-not medically or surgically correctable
-usually permanant
-usually high frequency
-can be profound HL
-loss of audibility & clarity
-loudness recruitment - Glomus Tumor
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"Rising Sun" TM appearance
invades ME space, temporal bone and can extend intracranially
-occur mostly in women in their 30s-50s
-unilateral conductive HL
-pulsing tinnitus that is synchronized with pulse - Otosclerosis/otospongiosis
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Temporal bone disease wherby normal bone is reabsorbed by body and replaced with spongy bone that becomes sclerotic
-most common in caucasian women
-autosomal dominant disorder
-disease progresses during hormonal changes between 20s &40s, pregnancy or menopause
-interferes with normal vibration of stapes footplate in oval window
-results in ankylosis (bone fixation) - Audiologic results of otosclerosis
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-slowly progressive conductive HL that eventually invades the cochlea and becomes a mixed loss.
-mild to severe
-elevated bone conduction thresh at 2000Hz (carhart's notch)
usually bilateral
tinnitis
TM can have a pinkish color(schwartz's sign)
Type A or As tymps, absent acoustic reflexes - TX for otosclerosis
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Sodium Fluoride arrests progression but little improvement in hearing
- amplification
-surgery- good candidates have large air/bone gaps, normal bone conduction thresholds, good word recognition scores - Ossicular discontinuity
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Caused by trauma(head injury or barometric changes), congenital malformation or ME disease.
-dislocation is usually in the incudo-stapedial joint
-tympanometry =Type Ad, static compliance high, absent reflexes
-disarticulation with subluxations-fibrous reconnection
-may have clos to normal hearing, high freq ABG - tympanosclerosis
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-changes in tissue of TM due to repeated infections
-chalky white calcium plaques and scar tissue on pars tensa
-does not affect hearing if confined to TM
-related to calcification on ossicle that affect their movement & cause HL - What is Battle's sign?
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seen with fracture of base of skull
-blue or purple discoloration behind ear from posterior auricular artery
-becomes noticeable a few days after injury - What is Tullio's phenomenon
- sensation of vertigo brought on by loud sounds
- What is henneberts sign?
- nystagmus with pressure applied to the ear canal(aka fistula test)
- 5 main classes of ototoxic drugs.
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aminoglycocide antibiotics
salicylates
quinines
loop diuretics
anti neoplasts - chronic use of acetaminophen can cause?
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reversible ototoxic results
-mild to mod HL
-tinnitus
-liver damage
-large doses can inhibit nociception - Some Antibiotics
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penicillins, cephalosporins
aminoglycosides (gentamicin)
fluroquinolones(ciproflaxin)
macroglides(erythromycin
Tetracyclines (doxycycline)
vancomycin
Antivirals
acycloviris
neucleoside
protease inhibiters
anti protazoal (parasitic agents)
anti fungals - Prozac
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Antidepressant -fluoxetine, long term - oto- toxic effects
may have vertigo, tinnitus, ear ache - Antihypertensives
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lisinopril - ACE inhibitor, causes dizziness and vertigo
don't take during pregnancy - Anti convulsants
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used to treat seizures, lamictal
can cause far sightedness, double vision, HL ,itchy external auditory meatus, dizziness and nystagmus - Anti neoplasts
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chemotherapeutic drugs
Cisplatin
carbolplatin
oxaliplatin
ototoxic- cause loos of OHCs - Loop Diuretics
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Powerful & fast acting, help body get rid of fluids. Inhibit kidneys ability to reabsorb sodium so loss of Na in unine
-treatment of edema (furosemide,lasix)
can increase the ototoxic potential of aminoglycoside antibiotics - symptom
- subjective evidence as perceived by the patient eg. headache
- Sign
- objective evidence of an illness or disorder eg. rash
- Varicella =
- chicken pox
- syncope =
- temporary loss of consciousness
- septicemia =
- blood poisoning
- scoliosis
- curvature of the spine
- Rubella =
- german measles
- Phlebitis
- inflammation of a vein
- Pertussis =
- whooping cough
- myopia =
- near sightedness
- Ischemia
- inadequate blood flow in an artery
- infarction
- formation of dead tissue due to a lack of oxygen to that area
- Herpes Zoster =
- shingle, painful rash confined to one side of body
- Glaucoma =
- defective vision caused by increased ocular pressure
- CVA
- Cerebrovascular accident
- Carcinoma
- a malignant growth arising from epithelium or lining of body organs
- athersclerosis
- progressive narrowing and hardening of arteries over time
- Asphysia
- condition caused by inadequate intake of oxygen
- Arrhythmia
- any variation from the normal rhythm of the hearbeat
- aneurysm
- dilation of the wall of a blood vessel
- Contrecoup
- fracture of the skull at a point opposite where the blow was received
- decubitus ulcer
- bed sore
- diaphoresis
- profuse perspiration
- epistaxis
- nosebleed
- embolism
- sudden obstruction of an artery by a clot or foreign material (air bubble)
- Emesis
- vomiting
- enuresis
- bed wetting
- hirsutism
- abnormal hairiness
- strabismus
- deviation of the eye that cannot be controlled
- Diagnosis
- identification of a disease or condition by scientific evaluaton or ;hysical signs symptoms, history, tests and procedures
- Contrast imaging
- using a medium injected into the body or swallowed so there is greater visibility of internal organs or cavities
- CT, or CAT scan
- Computed tomography - cuts body into detailed cross sections of tissue or bone
- fluoroscopy
- method of viewing a direct x-ray image in real time so that motion can be seen
- Nuclear medicine
- putting radioactive materials into body organs to make them visable on computerized scanners known as gamma cameras
- MRI
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magnetic resonance imaging
- creates images of internal structures based on magnetic properties of chemical elements in the body
- good for tissue resolution - Sonography
- imaging of body structures by sending and receiving high frequency sound waves that are reflected back as echos from the surface of the tissues
- cyanosis
- bluish discolorization of the skin or mucous membranes
- thrombosis
- blood clot formed within a blood vessel that remains attached to its place of origin
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Parents pass traits to their young through gene transmission.
Genes are located on ___ and consist of ____ -
Chromosomes
DNA
they contain specific instructions for protein synthesis - the first gene to be linked to language production and the key to development of brain regions associated with speech is ?
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FOXP2
this discovery suggests that the development of language may be tied to a genetic mutation and could have occurred some 50,000 years ago - On a clinical basis molecular genetic testing is available for the diagnosis of ?
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BOR
Mohr-Tranebjaerg syndrome
Pendred syndrome
Usher syndrome
DFNB1 & DFNA3, DFNA4, DFNA6 -
Testing for deafness causing mutations in the GJB2 gene encodes the protein _____
GJB6 encodes the protein ______ -
Connexin 26
Connexin 30 - Hereditary HL is managed by a team including :
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-otolaryngologist
audiologist
clinical geneticist
pediatrician
sometimes TOD (teacher of deaf)
neurologist
pediatric ophthalmologist -
Laboratory tests (pregnant women) include tests to confirm or rule out possible infectious or inflammatory diseases that are sometimes associated with HL
what are some things tested for? -
- Syphillis
- Rubella
- CMV
- Autoimmune diseases -
All 4 types of immune reactions (Gell&Coombs) have been hypothesized to contribute to inner ear disease
type one? -
type I
- mediated by IgE activation of sensitized mast cells, release histamine & other vasoactive substances. Inhalents & anaphylaxis.This type thought to cause disruption of ionic transport within the inner ear -Endolymphatic Hydrops - Type II immune reactions?
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Type II
-occur when antibodies directed against a specific antigen within tissues elicit activation of complement either from:
- exogenous (virus or drug) source or
- endogenous as in the case of anti DNA antibodies of SLE, Good pastures syndrome and some evidence of Menieres involvement - Type III immune reactions ?
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Type III
-are the result of deposition of intermediate sized immune complexes in the microcirculation. Causes vascular injury to labyrinthine structure.Menieres show 95% elevated levels of circulating complexes, SLE, Wegeners granulomatosis - Type IV immune reactions?
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Type IV
-characterized by T-cell mediated delayed hyper sensitivity. Exemplified by rejection of transplanted organs. Sensitized T-lymphocytes are activated by antigen on the target cell to either directly lyse the cell or produce lymphokines that amplify the response by attracting other inflammatory cells. The lymphocyte transformation Test (LTT)used.
Seen in Menieres, & Cogan syndrome - Patient with progressive SNHL believed to be due to AIED should be considered for ______therapy
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immunosuppressive therapy
- steroids are the foundation of therapy, relatively high dosage needed
-prednisone 1mg/k/day, 60mg daily dose for adulat for 3-4 weeks
-those that respond can be slowly tapered off
-maintence dose - 20mg every other day for 3-6 months - Patients given immunosuppressive therapy should be advised of
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potential side effects
-potential complication of hemorrhagic cystitis, leukopenia, sterility, malignancies of urinary tract, not for use on women of child bearing age - AIEP has also been treated with ?
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Plasmapheresis - limited evidence of efficacy
shortcomings?
-expense and time consumption
-pt required to spend several hours for multiple days - Methotrexate has also been considered as a possible therapeutic agent for ?
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AIED
-has mild side effects & complications -
The immune function within the inner ear is similar to that of the?
The labyrinth is separated from the circulation by the ? -
Brain
Blood-labyrinthine barrier
-this serves to maintain the ionic characteristics of the cochlear fluids - Similar to CSF, perilymph contains?
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immunoglobulins at a concentration of 1/1000th of that in serum
-IgG -immunoglobulin is the predominant antibody while IgM&IgA exist in lesser concentrations - The ____ ____ plays a significant role in inner ear immune response.
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endolymphatic sack
- it contains a population of lymphocytes, but role is not completely clear.
-the lymphocytes responding to antigenic stimulation in the inner ear enter through the systemic circulation via spiral modiolar vein
-the inner ear immune response serves to protect the cochlear structures from insults such as viral labyrinthitis & other infections - AIED =
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Auto immune inner ear disease
- a syndrome of progressive HL & or dizziness which is caused by antibodies or immune cells which attack the inner ear - Otosclerosis causes what type of HL?
-
It can cause either conductive or SN or both
- its abnormal bony growth most often affects the ME causing conductive HL but can also extend into the vestibule & labyrinth and cause SNHL
-will be a mixed loss if affect both ME and IE - All of these can cause chronic serous otitis media
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-mucus in ME
-lymph in ME
-enlargement of adenoids - Otitis Externa is ?
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an inflammation of the ear canal between the ear drum & outside of the ear
-the ear canal can be easily infected because it is dark, warm & bacteria or fungus may grow there
-can be successfully treated topically but often treated systemically by MDs - Perforations - precautions
-
-during the healing process must protect from water
-usually the larger the perf the more the HL
-before surgical correction do a hearing test
-benefits of closing a perf? prevent water from entering ME, diminished tinnitus, improved hearing, prevention of cholesteatoma -
Among children with clefts of the lip and palate, which of the following types of hearing loss is most common?
a)Sensorineural
b)congenital
c)mixed
d)central auditory
e)conductive -
E) conductive
A cleft lip and palate are often associated with abnormalities of the muscles that open the eustachian tube. Eustachian tube dysfunction results in negative middle ear pressure leading to OM which in turn can cause conductive hearing loss