Exam 1 Flashcards
1
Q
What are variants of the pinna?
A
Size, position, width, length and angle
2
Q
Darwin Tubercle/Notch
A
One normal variant of the external ear
Atavistic remnant; usually located in the posterosuperior helix as a cartilaginous protuberance usually on concave edge but may be convex
Autosomal dominant
Variable expressivity
3
Q
Four mild malformation of the pinna
A
Lop ear, Stahls ear, prominent ear and cup ear
4
Q
Syndromes associated with outer ear malformations
A
Down syndrome, other trisomies, Treacher Collins, BOR, chromosomal deletions, CHARGE, Goldenhar etc.
5
Q
Stenosis
A
Narrowing of ear canal, may or may not have hearing loss
Common in children with down syndrome
Can be accompanied with atresia
Treatment- stint in cartilage portion
6
Q
Anotia
A
No external ear and the most severe pinna malformation
No ear, no pinna, no canal
Unilateral is more common
7
Q
Atresia
A
No ear canal
Birth defect
Usually accompanied by abnormalities of middle ear bones and external ear
Three forms
Can be genetic- may be a family Hx
Microtia (small ear) may also be associated
Can have max. conductive component (60 dB)
CNT tymps
Speech and language will develop normally if other ear is okay
Early amp is important- BAHA
Can have surgery to restore hearing but it is very complicated and can put the facial nerve at risk
Refer (if they have not been already)
8
Q
Grade I atresia
A
Most mild form. Characterized be small external canal with most features being present, canal often looks stenotic, the tympanic bone and membrane are often developed but ossicular abnormalities are common
9
Q
Grade II atresia
A
Moderate and most common form
Characterized by the pinna being present but being formed abnormally, a significant defect in canalization of the tympanic bone, the membranous external canal will be absent or ends in pinpoint fistula opening, a narrow bony canal, deformed ossicles and fused incus and malleus
10
Q
Grade III atresia
A
Most severe form
Characterized by severely deformed or absent pinna otherwise called “peanut ear”, ossicles are typically an amorphous mass, undefined stapes
This form is often associated with a syndrome
Complete atresia
11
Q
Microtia
A
External ear malformations
12
Q
How many grades of microtia are there?
A
Three, with one being the mildest and three being most severe
13
Q
What is more common, unilateral or bilateral atresia?
A
Unilateral
14
Q
When does the six hillocks of his develop?
A
Week 5
15
Q
What are the six hillocks of his?
A
Tragus, crus of helix, ascending helix, upper helix/scapha/antihelix, middle scapha/antihelix/helix, antitragus/lobule
16
Q
Pinna development week 7
A
Moves dorsolaterally
17
Q
Pinna development week 18
A
Separates from head
18
Q
Pinna development week 20-22
A
Approaches mature shape
19
Q
When is the pinna fully developed
A
Age 9
20
Q
Preauricular tags and pits
A
Higher rates in individuals with hearing loss so it is important to look when doing an examination
21
Q
Preauricular sinus
A
Abnormality of the otic hillocks, infected
22
Q
Development of ossicles and middle ear week 4
A
First pharyngeal pouch endoderm extends outward to form the tubotympanic recess and approaches the 1st cleft ectoderm
23
Q
Development of ossicles and middle ear week 5-6
A
Neural crest mesenchyme concentrates (ossicles) between cleft and pouch; cleft widens (future external auditory meatus)
24
Q
Development of ossicles and middle ear week 15
A
Malleus and incus are adult size and shape
25
Development of ossicles and middle ear week 20
Tympanic cavity grows to enclose ossicles but still full of mesenchyme
26
Development of ossicles and middle ear week 26-30
Ossicles ossified
27
Development of ossicles and middle ear week 32
Stapes adult size and ossified
28
Development of ossicles and middle ear week 33
Early pneumatization (development process of the mastoid air cells)
29
Canalization
How to canal opens up, can halt at any time
30
Canalization week 7-8
Tympanic ring forms
31
Canalization end of month 2
External canal is a thick epithelial plug, migrated inward toward ossicles
32
Canalization week 21
Plug starts to recanalize (hollow) via apoptosis, forming the external canal
33
In what direction does canalization move?
Starts medially then moves laterally
34
What happens if canalization halts before it starts?
Total atresia but can have normal middle ear
35
What happens if canalization halts during development?
Patency of ear canal depends on when it is halted
36
Development of tympanic membrane at 5 weeks
Tubotympanic recess contacts ectoderm (which will form the outer layer)
37
Development of tympanic membrane at 10 weeks
Bony ring in place but is incomplete
38
Development of tympanic membrane at age 3
Changes in the face of it
38
Disorders of the external ear and ear canal that are not congenital (6)
Cerumen impaction/foriegn bodies/collapsing canals
External otitis
Tumors of ear canal
Abnormalities/lesions of the pinna
Results of traums
TM abnormalities: myringitis, tympanosclerosis, perfs
38
What are three ossicular abnormalities (one for each)?
Malleus head fixation (common)
Incus- no long process (common)
Stapes fixation (perilymphatic gusher)
38
Development of tympanic membrane at week 11-16
Increases in size
38
Three other middle ear abnormalities from development
Congenital cholesteatoma- epidermoids
High jugular bulb
Congenital PLF
39
Franks Sign
Creased lobule
40
Hypertrichosis Lanuginosa Acquisita
Fine hair
Associated conditions:
Medications- phenytoin, streptomycin, penicillamine, minoxidil
Porphyria (werewolf myth)
Pregnancy
Malnutrition/anorexia
Malignancy
41
Auricular Hematoma
Collection of blood between cartilage of pinna and connective tissue
Possible causes:
Trauma
Scarring or thickening of the cartilage
Treatment:
Surgical
Prevention (drain the blood)
Refer and avoid activities that may cause trauma
42
Cauliflower ear
Causes: repeated trauma, common in high impact head sports like wrestling and rugby
Hearing loss is a possibility if the canal is closed off
Refer
43
Neomycin allergy
Medicine allergy that can cause intense itching and/or pain
Only a small percent of patients will have a reaction
Refer
44
Keloids
Excessive scar tissue formation following trauma or surgery to pinna; benign; more common in racial groups with more skin pigment
HL may be present if it occludes the canal
Treatment: steroid injection, pressure, surgery in some cases
Refer
45
Carcinoma
Malignant lesion that looks like a sore
Refer
Avoid frequent sun exposure
46
Basal cell carcinoma
Very common, from sun exposure, may look pink or flesh-colored and its usually a nodule with rolled borders and telangiectasias
47
Squamous cell carcinoma
Looks like a sore but the presentation varies
48
External otitis
Infection in the skin of the external auditory canal; often called "swimmers ear"
Can be caused by both fungi and bacteria, worse in hot/humid environments and can develop following lacerations of the canal, may be a chronic issue, can be infectious so use gloves
Symptoms: ear pain (particularly if the tragus is palpated), otorrhea, itching, aural fullness, tenderness/pain, fever and cellulitis, mild CHL if there is swelling, localized furuncle, redness
Refer
May be from swimming in a dirty place (counsel)
49
Malignant (necrotizing) external otitis
Severe inflammation caused by bacterial agent that is most common in elderly and diabetics
Symptoms: progressive pain and drainage, severe otalgia especially at night, granulation tissue in the ear canal
Can spread through eardrum to middle ear space and mastoid system
Can cause massive bone destruction in external, middle and inner ear
Occasional facial nerve palsy
Treatment: IV antibiotics, hyperbaric treatment, control of blood glucose, debridement
50
Hyperceruminosis
Abnormal accumulation of cerumen caused by defective production or clearance
Cerumen blockage can also result from obstruction of the canal, overly tortuous canals, stenotic canals
Can become stenotic with age
Treatment: frequent removal
More common in nursing home/people who are institutionalized
May have many colors and appear dry or wet (this is determined by genetics and diet)
May complain of tinnitus, fullness but rarely vertigo, coughing and pain
Hearing may be sudden or fluctuate, flat 30-40dB CHL with complete occlusion, tymps should be normal (may have smaller volume)
Remove or refer
51
Keratosis obturans
Accumulation of corneocytes due to failure of lateral migration
Forms a plug that often adheres to the skin
Will complain of fullness
HL with full occlusion
52
Canal cholesteatoma
Accumulation of keratin, usually related to a lesion. Typically there is bone erosion, dull pain and drainage
Refer- may need a CT to see if eroded to the bone
53
Collapsing canals
Most common in the elderly and very young children
Can be fixed by using inserts rather than headphones
Will usually occur in people with very cartilaginous canals
May be unilateral
ABG in mid-high frequencies (10-50) especially at 6kHz
54
Herpes zoster oticus/Ramsay-Hunt
Reactivation of the varicella-zoster virus at the geniculate ganglion
Viral infection of the OE, ME and IE
Trigeminal in origin
Symptoms: vesicular rash, ear pain, hearing loss, tinnitus, eyelid palsy, facial palsy, vertigo, change/loss of taste, will see it on pinna, canal and sometimes TM
Mild SNHL, affects the facial and auditory nerve so reflexes will look weird
Treatment:
Vaccination
Steroids
Anti-viral
Refer
55
Bell palsy
Symptoms:
Decreased tearing
Hyperacusis
Taste disturbances, metallic
Weakness of the facial muscles
Poor eyelid closure
Posterior auricular pain
Otalgia
Acute onset of unilateral upper and lower facial paralysis
Tingling or numbness of the cheek/mouth
Blurred vision
Increased salivation on affected side
56
Upper motor neuron palsy
Only the lower half of the face is affected
Causes:
Stroke
MS
Tumor
57
Horners syndrome
Problem with sympathetic nerve supply to one side of face
Symptoms:
Miosis- constricted pupils
Ptosis- droopy eyelid
Anhidrosis- failure to sweat
58
Trigeminal neuralgia
Sharp unilateral facial pain, usually the right side
Triggers:
Chewing, drinking, talking, smiling
Causes:
Vascular compression, tumor, inflammation, idiopathic
59
Polyps
Thickened nodules of skin in canal
60
Osteomas
Bony tumors that are benign, smooth nodule of spongy bone covered with skin
Usually at the junction of bony and cartilaginous, unilateral, pedunculated, found in kids more commonly, can occlude the canal/block cerumen
Treatment:
Surgical removement
61
Exotsoses
Benign bony lesions deep in the EAC
Causes:
Exposure to cold water, wind, "surfers ear"
Treatments:
Surgical if the canal is blocked
Ear plugs prevent recurrence
Usually found in adults
62
Development of the inner ear shape and size week 8/9
Cochlea reaches 2.5 turns
Semicircular canals attain general form
63
When does the inner ear reach full size and shape
The canals reach at 17-19 weeks and the cochlea 16-19 weeks
64
Development of the organ of corti week 9/10
Sensory epithelium develops
65
Development of the organ of corti week 10-20
Stria vascularis develops
66
Development of the organ of corti week 11
IHC and OHC present, immature stereocilia develops
67
Development of the organ of corti week 14
Hair cells and stereocilia better developed, supernumerary hair cells, pillar cells, tunnel of corti
68
Development of the organ of corti week 19
Mostly mature appearance
69
Development of the organ of corti week 20-25
Modiolus ossification
70
Development of the spiral ganglion week 4
Statoacoustic ganglion developed
71
Development of the spiral ganglion week 5
Cochlea and vestibular ganglia distinct
72
Development of the spiral ganglion week 7-9
Fibers enter cochlear epithelium
73
Development of the spiral ganglion week 11
Synapses on the hair cells develop
74
Development of the spiral ganglion week 14
Efferents on IHC develop
75
Development of the spiral ganglion week 22
Afferent synapses morpholol are mature
76
Development of the spiral ganglion week 15-24
Myelination occurs
77
When does the saccule and utricle develop
Weeks 6-8
78
What are the four labyrinth anomalies
Michel aplasia, common cavity malformation, cochlear hypoplasia and Mondini IP2
79
Michel aplasia
Aplasia of all inner ear structures (no structures)
Caused by- thalidomide, Slippel-Feil, anencephaly
Will occur during the 4th week
HL- severe to profound, bilateral deafness, absent reflexes
HL can vary
Contraindication for CI!!!!
Refer
80
Common Cavity Malformation
Membranous labyrinth present but poorly differentiated
Severe-profound hearing loss
Will occur during the 4th week
If a CI is used, there will be a lot of leaks
81
Cochlear Hypoplasia
Rounded, small cochlea
Degree of hearing loss varies depending on membranous differentiation
Will occur in the 6th week
Can have a whole variation of hearing depending on membranous labyrinth
Turns could be 2 or fewer
82
Mondini IP2
Incomplete partition, most common bony anomaly, most often bilateral
Often associated with LVAS
Usually occurs around week 7
Cochlea will be about 1.5 turns
Absent interscalar septum middle and apical turns
At risk with perilymphatic fistula, meningitis
Associated with Waardenburg, DiGeorge, CHARGE and Pendred syndromes
Ask about family history and genetics
Usually severe to profound SNHL with some preservation of the high frequencies due to basal turn being still functioning
May be severe rising to mild SNHL, bilateral, congenital
Degree of SNHL depends on degree of malformation
SRT and WRS could be better than PTA due to good HF hearing
OAE will be absent where there is hearing loss
Reflexes will be absent where hearing loss
Refer
Surgery is the only treatment
83
Bing-Siebenmann malformation
Otherwise known as cochleosaccular dysplasia (did not grow the right way)
Bony but intact organ of corti has collapsed Reissners and abnormal stria vascularis
Usually profound SNHL
Associated with Jervell and Lange-Neilsen, Usher
84
Schiebe malformation
Most common membranous malformation
Partial/complete aplasia of ossicular chain, collapse of cochlear duct
Severe to profound SNHL
Associated with Jervell and Lange-Neilsen, Waardenburg, Refsum and trisomy 18
85
Alexander malformation
Dysplastic basal turn, otherwise normal labyrinth
Hereditary high frequency SNHL
86
Large vestibular aqueduct syndrome (EVA, LVA)
Most common anomaly seen in imaging
Associated with incomplete partition, Pendred, BOR, perilymphatic gusher, Mondini
Aqueduct that is 1.5mm or greater at midpoint
Can also have a large endolymphatic sac
3rd window theory- ABG
Similar symptoms to perilymph fistula
Needs to be diagnosed with CT or MRI
More common for kids and usually diagnosed during childhood, progressive (related to 250 Hz) and will fluctuate, cochlear in cause, fake ABG but normal tymps and reflexes, may be unilateral (more common) or bilateral
Usually mixed, primarily SNHL, possibly severe to profound CHL but may have extremely good bone at low frequencies
Characterized by sudden drops and fluctuations
87
Superior semicircular canal dehiscence (SSCD)
Seen in young children but incidence increases with age
Symptoms:
Tullio, Hennebert, gaze evoked tinnitus, hypersensitive to sound, autophony, conductive hyperacusis
May complain of feeling full in the ear, unsteadiness when sneeze or cough, onset of symptoms are not linked to a specific event
Usually unilateral with gradual onset
No evidence of TM or ossicular abnormality
CHL will be similar to otosclerosis but unlike an ME problem will have AR and tymps, DPOAEs if hearing loss allows, VEMP and WAI findings, case history
Will have fake ABG
Surgery to patch the SCC
88
What percent of hearing loss is genetic in prelingual Deaf children?
50%
89
What percent of hearing loss is non-syndromic in prelingual Deaf children?
70%
90
Genotype
The specific genetic constitution of an organsim
91
Phenotype
The observable properties of an organism; the expression of genes in traits or symptoms
92
Heterogeneity
Any of several genes can produce the same phenotype
Diverse in character/content
93
Autosomes
Chromosomes other than sex
94
Variant
Change in a genes DNA
95
Mutagen
Substance that causes a mutation
96
Spontaneous mutation
Change in bases during replication
97
Mendelian inheritance
Autosomal dominant, autosomal recessive and X-linked
98
Syndromic
Group of characteristics that co-occur
99
For a person to have a autosomal recessive disorder, what must they have?
A double dose of the gene
100
Polygenic traits
Determined by more than one gene
101
Multifactorial traits
Determined by one or more genes as well as the environment
102
Usher syndrome
Dual sensory impairment- will have a auditory, vestibular and visual presentation
Several subtypes with some being nonsyndromic during childhood before vision loss
Autosomal recessive
103
Jervelle and Lange-Neilsen Syndrome
Signs and symptoms:
Syncope (fainting) most often elicited by emotion or exercise
Autosomal recessive
Due to cardiac issues, risk of sudden death without treatment is high
Congenital profound bilateral SNHL
Refer
104
Pendred syndrome
Autosomal recessive
Can be congenital or late onset often progressive hearing loss (may be sudden or fluctuate) but is often bilateral
Associated with Mondini and EVA
Possible thyroid enlargement (goiter)
Testing- imaging, genetic testing, vestibular, counseling, issue of progression for treatment
105
Pierre Robin sequence
At 7-10 weeks of development the jaw grows rapidly OR the jaw does not grow so the tongue cannot lie flat, it then rests at the back of the mouth and prevents the palate from closing causing a cleft palate.
Often respiratory and feeding problems
Isolated version of this- jaw of affected child is often able to grow and catch up to normal size but often is associated with other conditions
Mainly CHL due to OM auricular anomalies
Auricular anomalies in 75%
106
Craniosynostosis
Premature fusion of neurocranial sutures
Craniofacial dysmorphologies
Usually nonsyndromic
Will involve hands and feet
107
Muenke syndrome
May have normal cognitive function to mild ID, changes in extremities
SNHL is most common and CHL would be related to OME
Autosomal dominant
108
Penetrance
The percentage of individuals who possess a dominant gene and express it
109
Variable expressivity
A genotype producing a phenotype that varies among individuals
110
Apert syndrome
Up to 80% of these patients will have hearing loss due to stapedial fixation, middle ear structural anomalies, OME
Will typically have cleft palate, flat "dished" face, syndactyly, ID about 50% of the time
Possible heart or kidney problems
Primarily sporadic but is related to paternal age effect
Autosomal dominant
111
Crouzon
75% of these patients will have hearing loss in addition to stenosis, atresia, microtia
CHL is most common yet middle ear structural anomalies are rare, OME, SNHL more often than other syndromes
112
Treacher Collins syndrome
Lower jaw and cheekbones are smaller than normal, lower eyelid coloboma (notch), downward sloping eyes
Outer ear abnormalities (microtia, ear tags, ear pits, atresia), CHL, rarely SNHL
Cleft palate 30% of the time
60% of the time its sporadic (related to the advanced age of the father)
Autosomal dominant
100% penetrance, variable expressivity
Maximum CHL (around 60)
113
Hemifacial microsomia, Goldenhar syndrome, Oculo-Auriculo-Vertebral (OAV) spectrum
Facial asymmetry of varying degrees- vascular or neural crest disruption
Vertebral changes such as cervical spine and cranial base malformation, occasional heart and kidney issues
Ocular findings, may involve brain and other organs, usually no ID
Auricular abnormalities (a spectrum from mild morphologic changes of the pinna to complete anotia)
Microtia if the most common, ossicular malformation common, absence of the IAC (inner auditory canal) or inner ear abnormality infrequent
50% have CHL and/or SNHL
114
Stickler syndrome
Hearing loss most common, specifically high frequency SNHL but CHL may also be common
Midfacial underdevelopment
Cleft palate
Eye problems- myopia, cataract and retinal detachment in childhood
Joint problems- arthritis by 40s
Hypermobile TM 46% of patients
Underidentified
Variable phenotypic expression
5 types
Mostly autosomal dominant
115
Alport
X-linked recessive, autosomal dominant
Kidney function- hematuria in early childhood
Progressive hearing loss, eye involvement (except when autosomal dominant), rare aneursyms in males
Bilateral high frequency SNHL in late childhood
90% of patients are deaf by age 40
Less severe progress and outcome in females
116
Branchio oto renal syndrome
Fistulas and cysts associated with 2nd brachial arch
Pits, tags, pinna abnormalities, ME and inner ear structural abnormalities, may be unilateral
May have small or abnormal kidneys, absent kidney, ultimately end stage renal disease possible
May not be identified
Often confused with Alport
Hearing loss in 95% of the patients
Malformed auricles
Second branchial cleft anomalies
Preauricular sinus
Renal abnormalities
AD
117
Otosclerosis
Not a craniofacial syndrome
No effect on facial structure
Youngest presentation is around age 5
Autosomal dominant
Multifactorial
118
Waardenburg
Four types
Dystopia canthorum, a broad nasal root, confluence of the medial eyebrows, heterochromia irides, a white forelock
Autosomal dominant/recessive
A neural crest defect
Hearing loss is not typically progressive, usually profound but can be mild in high frequencies, unilateral or bilateral SNHL (usually profound bilateral SNHL)
Expressivity is extremely variable
May be not identified
May have temporal bone abnormalities including EVA
Not associated with progressive hearing loss
Refer
119
Otitis media
Inflammatory process of the middle ear
120
When does the ET open?
During active processes so yawning, sneezing, swallowing or when eccessive pressure is applied from the nose
121
When does the ET reach adult size and configuration?
Age 7
122
ET purpose
Ventilation, drainage, protection, pressure equalization
123
Patulous
Always opening (in reference to the ET)
May be due to anatomic structures, radiation, MS, radical head neck surgery
124
Mastoid
Area of the skull that surrounds the ear; it is "honeycombed" with hundreds of air cells, air cells are lined with mucous membrane
125
Role of chorda tympani
Sensory branch of the facial nerve, has a role in taste
126
Acute otitis media (AOM)
History of acute onset, middle-ear effusion, signs and symptoms of middle ear inflammation
Complaints for adults- mild intermittent pain, fullness, popping, balance problems, clumsiness
Complaints for infants- ear rubbing, irritability, fever, headache, cough, rhinitis, listlessness, anorexia, vomiting, diarrhea, sleep interruption
Pacifier use can increase the likelihood of this
Otoscopy- moderate to severe bulging, new onset of otorrhea, ear pain
May have CHL
127
Otitis media with effusion
Presence of fluid in middle ear, no signs or symptoms of acute ear infection, hearing loss may be present
Otoscopy- amber, grey, white, blue
Hearing loss- mild
Will see best air frequency at 2kHz
128
Recurrent acute otitis media
Three or more episodes of AOM within 4 or 6 months in a 12 month period
Affects 10-20%, 40% of these have 6+ episodes
129
Chronic otitis media with effusion
Persistent fluid behind intact tympanic membrane in the absence of acute infection
130
Chronic suppurative otitis media
Persistent inflammation of the middle ear or mastoid cavity
Recurrent or persistent otorrhea through a perforated tympanic membrane
131
At what ages does OM prevalence peak?
6-18 months and 4-5 years of age
132
What is the incidence of OM by age 2?
90%
133
What are typical complaints of OM for adults?
Usually with otalgia (pain), crackling/popping tinnitus, fullness
134
Two subtypes of AOM
Bacterial and viral (cannot be treated with antibiotics if viral)
135
Symptoms of AOM for infants
Ear rubbing, irritability, fever, headache, cough, rhinitis, listlessness, anorexia, vomiting, diarrhea, sleep interruption
136
General symptoms for AOM
Mild intermittent ear pain, fullness, "popping", hearing loss, problems with school performance, balance problems, clumsiness and gross motor issues
137
Color of the TM for a patient with AOM
White usually, also yellow or red
138
Color of the TM for a patient with OME
Amber diagnostic, also grey, white or blue
139
Myringitis
Inflammation of the TM, sudden pain, blebs or vesicles visible
140
Atelectosis
TM sucked in so much, it sits on structures; may look like a perf
141
Purulent
Infected, drainage
142
Chronic otitis media
Usually follows recurrent AOM
Usually results in CHL
Can be serous or mucoid
143
Serous
Fluid
144
Mucoid
Mucous
145
Do you expect acoustic reflexes with otitis media
No- do not expect reflexes with CHL
146
Acute Mastoiditis
May be due to untreated bacterial AOM
High fever, pain/tender behind ear, may not have acute pain (especially with adults)
Children under 2 (immature immune system)
Risks of meningitis, abcess, CN palsy
Less common in developed countries
Testing: otoscopy, tymps (may be flat B or not), pure tones (CHL), possible poor word recognition, CT scan and blood tests
147
Meningitis
Relatively common of the intracranial complication of OM
Symptoms- fever accompanied by neck stiffness
Tests- CT or MRI, lumbar puncture
Treatment- antibiotics
148
Abcess- brain or epidural
One of the most common intracranial complication of COM
Latter- temporal bone through veins or boney erosion in the tegmen tympani or bone by posterior cranial fossa or sigmoid sinus
Former- usually temporal lobe or cerebellum, likely direct spread of infection
Symptoms- headache, vomiting, lethargy, seizures, hemiparesis, cranial nerve palsies and aphasia
Test- CT, MRI
Treatment- antibiotics and/or surgery
149
Cholesteatoma
Cystlike lesions of the temporal bone
May be caused by CSOM
150
Labyrinthitis
Inflammation of any part of the labyrinth (via round window, erosion)
Vestibular and/or auditory symptoms (hearing loss, vertigo)
Temporary- assumption is that the cause was serous and mostly due to inflammation
Permanent- more likely due to suppurative, bacteria in the inner ear
Treatment- intravenous antibiotics
151
Facial Paralysis
CSOM especially with cholesteatoma causes inflammation of the facial nerve which then triggers edema and nerve fiber compression causing this
152
Biofilm
Bacterial slime
153
Treatment for OME
Non-surgical- wait and watch, autoinflation, drugs
Surgical- PE tubes, adenoidectomy, tonsillectomy
154
CT scan
Focuses on bone
White = bone
Black = air
Risk for children
155
MRI
Better for soft tissue and neural structures
No radiation but more time consuming
156
Perilymphatic gusher
Basically a fistula that when ruptured perilymph rushes out and causes severe hearing loss
157
Alexander malformation
Least severe membranous labyrinth malformation
158
Lumen
Opening of ET
159
Third window theory
Air conduction will be impacted because the air will go through the extra space (thresholds will be high), bone will be normal
Energy is lost through the enlarged aqueduct
159
Neuritis
Facial paralysis
159
Common cavity malformation
Membranous labyrinth present but barely functioning
160
Cochlear hypoplasia
Small aplasia, still a little development of the cochlea, degree of hearing loss depends on membranous labyrinth development
161
Precision medicine
Looks at everything including, but not limited to, SES, gender, living area/conditions, genetics etc.
Will give better outcomes
162
Otoferlin
A gene often seen in ANSD patients and it encodes for synaptic vesicle fusion to the membrane
163
Karyotype
Picture of all the chromosomes in the body
164
Epigenetics
Heritable, changes that affect the codes accessibility, happens in response to environmental conditions
Genetic code that is altered due to the environment
165
Incomplete penetrance
People with the gene have the same phenotype
166
Connexin 26
AR and non-syndromic, imaging will not help diagnose
Most common form of congenital deafness
Family history of deafness important here
Normal tymps and otoscopy
Down sloping audiogram with HL in the high frequencies are the worst
Severe to profound deafness
Early onset with minimal progression
Excellent results with CI
Not as frequent symptoms- progressive HL, mild to moderate deafness, asymmetry dominant inheritance and associated with skin disorders
167
Mitochondrial linked disorders
Will impact all kids
Only goes through the mother
Disorders characterized by symptoms in tissues with high energy demands
168
X-linked disorders
Typically only impact the male children
If it is recessive, the mother will never show the affects but will show up in males in the family
If it is dominant, anyone who gets the gene will have HL
169
Otosclerosis
Multifactorially mediated metabolic bone disease
Autosomal dominance penetrance
Variable expressivity
Affects only the human otic capsule and ossicles
Progressive CHL
Tymps are variable
Typically stapes fixation
Can invade the cochlea in advanced cases
Most common in caucasian population and most common in the 30s
More common in women
Uncommon in children
Heredity is a factor
Genetically heterogeneous
Declining due to less measles presence
Bluish cast to whites of eyes (this is seen in other bone diseases)
Schwartze sign/risin sun (reddish blush near the promontory
Tinnitus 50% of the time
Dizziness has been reported but is not common
Paracusis willisii (speech is easier to understand in noise than in quiet)
Difficulty hearing while chewing
Excellent word discrimination
Bone will decrease at 2k
Acoustic reflexes typically absent
Diagnosis/treatment:
CT, surgery confirmation and history
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Cochlear otosclerosis
Affects the round window and invades the cochlea to cause SNHL that progresses overtime
Rare
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Carhart notch
Bone conduction thresholds decreased at 2k, 500 and 1k
Persistent ABG at 500 may be due to malleus/incus fixation
Air conduction thresholds may or may not be normal
Mechanical artifact possibly due to shift in resonant frequency produced by immobile oval window
After surgery there is recovery of about 8 dB
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Candidates for stapedectomy
Air bone gap of 15 or more
Speech discrimination score of 60% or more
Absence of anatomic or medical contraindications, risk of gusher
usually operate one one ear at a time if bilateral
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Outcomes of stapedectomy
Possible:
Permanent facial nerve injury, perf, vertigo, obliterative otosclerosis, CSF leak, floating footplate, subluxation of incus/malleus joint, tinnitus, hyperacusis
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Malleus/incus fixations
Presentation is similar to otosclerosis
Normal otoscopy but with pneumatic otoscopy will not see malleus move
ABG
Diagnosis during exploratory surgery
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Ossicle fixation
Recurrent OME, ossicular discontinuity due to incus necrosis or ossicular chain fixation due to tympanosclerosis
Poorly developed mastoid air cells is a risk factor
Congenital stapes fixation
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Paget Disease
Irregular breakdown and formation of bone, affects spine, skull and other bones
Symptoms:
Headache, pain, deformed bone otherwise asymptomatic
Diagnosis through alkaline phosphatase
Rare
CHL and/or SNHL
Vertigo
Some reports of low frequency CHL with high frequency SNHL
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Osteogenesis imperfecta
Otherwise known as brittle bone syndrome
1/20000
Genetic
Affects the collagen formation
AD and variable expressivity
Most severe form if incompatible with life- fractures in utero
Blue sclera
Four subtypes
Progresses from SNHL in kids to mostly mixed in older ages
Chance of having significant ABG
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Cholesteatoma
Invasive progressively enlarging tumor of the middle ear
Benign
Cyst like lesion that contains keratin and decaying materials
Often starts at tympanic membrane perf when epithelial layer sheds skin and forms a mass
Highly invasive, can erode the bone
May develop anywhere in the temporal bone but typically in the middle ear space
Can be acquired, congenital or iatrogenic
Signs and symptoms:
Painless smelly drainage, maybe dizziness, pain progressive unilateral CHL, retraction of TM, perforation, granulation tissue, unresponsive to antibiotics, possible epithelial debris in the canal, in otoscopy will see white mass medial and retraction pocket and perf, sometimes dizziness
Tymps will consistent with perf, may showed reduced mobility due to mass
Absent reflexes
Diagnose with CT
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Congenital Cholesteatoma
Present at birth
Detected in childhood
More common in males
Occurs in children with no history of perf, otorrhea, otologic procedures
Presents as a white mass medial to an intact TM
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Hemotympanum
Blood behind the TM due to trauma
Can take 4 weeks to resolve
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Barotrauma
Change in pressure
Symptoms:
Pain, hearing loss, tinnitus, vertigo
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Glomus Tumor
Benign highly vascularized tumors
Originate from cells called paraganglia that are found in the middle ear
Two types
May have hoarseness, tongue paralysis, vocal weakness, shoulder drop, aspiration
Pulsatile tinnitus is the characteristic symptom
Tymp- saw tooth pattern
Otoscopy- reddish/blueish mass behind TM
Reflexes- contralateral decay
CHL if the tumor is large enough to interfere with ossicular vibration
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Glomus tympanicum
Glomus bodies on Jacobsons nerve that crosses promontory or Arnolds nerve
Signs:
Reddish/bluish mass behind an intact TM, pulsatile tinnitus, late non-auditory symptoms (hoarseness, aspiration, tongue paralysis, shoulder drop, vocal weakness, facial nerve paralysis)
Sawtooth tymps
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Glomus jugulare tumors
Paraganglia in the jugular bulb under the middle ear
Signs:
Pulsing tinnitus, CHL, swallowing difficulties, possible dizziness
Slow growth
May eventually invade the brain/brainstem
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Wegeners granilomatosis
Systemic disease of connective tissue
Characterized by infalmmatory reactions around blood vessels
OME but effusion does not resolve with antibiotics or PE tubes
Treatment in steroids and immunosuppressant therapy
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TB
Bacterial infection that can cause an infection in the middle ear
TM will be red and look thickened
Effusion can block ossicles
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Syphilis
Can involve the middle ear, mastoid and inner ear
Can cause fibrous tissue in the middle ear
Fluctuating low frequency SNHL
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Immotile cilia syndrome
AR
Flat TM, bilateral CHL
Will not move as effectively
Will affect other parts of body
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What factors would qualify a hearing loss as nongenetic congenital? (4)
Infectious, chemical, physical and maternal
190
Perinatal timeline
About 4 months before birth to 1 month after birth
191
Perinatal hearing loss causes (7)
Hyperbilirubinemia, anoxia, trauma, prematurity, NICU, ECMO, other teratogens
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Hyperbilirubinemia
Neonatal jaundice that often occurs 2-4 days postnatal
Build up of bilirubin
Yellow whites of eyes and skin
If extreme excess- kernicterus which is when bilirubin accumulates in the brain, neurological damage including cerebral palsy, hearing loss, ANSD
Prematurity is a risk factor
Temporary HL and permanent HL, specifically mild to severe SNHL
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Toxoplasmosis
From undercooked meat, unpasteurized goats milk, infected cat litter, unwashed fruits/veggies
A risk for a weakened immune system
Prenatal exposure to active infection
Ocular, neurological, cardiac effects
About 10-15% in US have antibodies, can reoccur with immundeficiency (AIDS)
Congenital 1/10000 in US (higher in Europe, Poland and 9/1000 in Brazil)
If it occurs in the first trimester fetal death will occur but in the 3rd will have disease
Early treatment seems effective
Hearing loss may range from mild unilateral to bilateral profound; congenital and delayed onset hearing loss
Ocular, neurological and cardiac effects
194
Congenital Toxoplasmosis
85% infected infants are asymptomatic at birth
HL may range from mild unilateral to bilateral profound
Congenital and delayed onset HL
Highly variable
Associated with cognitive issues and late appearing learning disabilities (limited data)
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Syphilis
In utero fetal death is 50%
23.1 cases per 100000 births/year, 90% with asymptomatic syphilis
Effects- notched incisors, corneal scarring, flat SNHL, blindness, deafness, facial deformity, nervous system problems including neuropathy
Diagnosed through rapid plasma reagin, VDRL, FTA-abs
Treatment through penicillin
Late onset HL the rapidly progressive, may fluctuate
May have CHL from ossicles
Low word discrimination
Tullio or Henneburt
196
Otosyphilis
HL- flat SNHL, late onset, progressive, may fluctuate, SSNHL, CHL, vertigo, Hennebert, Tullio, low discrimination, may look like Meniere
37% onset in childhood, 51% between 25 and 35 years of age and 12% later in life
More likely profound and bilateral or high frequency rapidly progressing to profound bilateral in young kids
Highly variable, progressive, often asymmetric in adults
197
Rubella
Mostly eliminated in the US
Virus, mild disease, may be asymptomatic
1st trimester infection serious for fetus- 90% growth retardation, ID, HL, cataracts, heart and other defects, miscarriage, dual sensory impairment
2nd semester 40% HL
Usually flat severe bilateral SNHL but may be any mild to profound and can be unilateral and/or asymmetric
Typically congenital, can be late onset
Otoscopy, tymps should be normal, no reflexes
Usually flat severe bilateral SNHL but can be mild or profound
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Cytomegalovirus (CMV)
Asymptomatic or mild-flu like symptoms, may be latent throughout life and may reactivate
Estimate of prevalence of congenital infection in industrialized countries is 0.7%
Person to person transmission or through fluids
Effects when congenital- asymptomatic or have jaundice, splenomegaly, thrombocytopenia, intrauterine growth retardation, microcephaly, retinitis, HL
Symptomatic are at greater risk for HL and more severe HL
HL is mostly late onset, can be progressive and fluctuating
Asymptomatic may have moderate to profound bilateral HL
Symptoms are variable
199
What is symptomatic CMV associated with?
Greater risk of HL, more severe HL, vision loss, microcephaly, ID
200
What is associated with asymptomatic CMV
Moderate to profound bilateral hearing loss by age 6
Vestibular issues (late walking very common, head movement, abnormal calorics)
201
Testing for CMV
Congenital- must test at or immediately after birth
Postnatal- not uncommon and does not have same effects
Blood spot, saliva, urine
202
Herpes simplex virus (HSV)
Cold sores, genital herpes
Infection usually occurs during birth
Very rare cause of HL but can be unilateral or bilateral, severe to profound SNHL
May interact with other factors
203
Postnatal meningitis
Can be viral or bacterial
Symptoms- rapid, high fever, stiff neck, vomiting, headache, rashes, sensitivity to light
Type will vary if there is HL
HL may be mild to profound
HL may resolve when disease resolve
Inflammation of the membranes of the brain and spinal cord
Suppurative labyrinthitis damages the organ of corti
Loss of the spiral ganglion cells
It is important to monitor for fluctuations in the first year
May be ossification of the cochlea
Older adults are mostly affected by this
204
Zika
Microcephaly
Variable
Associated with West Nile, Herpes zoster
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Aminoglycosides
Used for serious bacterial infections
May cause cochlear, vestibular toxicity depending on the meds
Symptoms- high frequency HL, tinnitus, dysequilibrium, oscillopsia, aural fullness
206
Loop diuretics
Removes water from the body which is good for heart failure, hypertension, edema and renal failure
Likely to affect stria vascularis
Usually reversible side effects
207
Cisplain (antineoplastic agent)
Incidence is high and may be delayed and affected by dose
OHC degeneration
Bilaterally symmetric SNHL usually high frequency and irreversible, tinnitus often initial
208
NSAIDS
Often will cause tinnitus more commonly then HL
209
Quinine
Used to treat restless leg syndrome
Symptoms- tinnitus, HL (usually SNHL), vertigo, headache, nausea, and vision loss
210
Sildenafil
Viagra
Sudden hearing loss
211
X-ray
Electromagnetic, attenuated by structures, image on film, quick
Sometimes used for intraoperative/post-operative of CI electrode placement
Better for bone but not usually used in audiology
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CT scans
Sums multiple x-rays, primarily used for assessing bony skull base pathology (dysplasia of bony labyrinth, congenital external/middle ear anomalies, SSCD)
Shows up as bone (white), air space (black), tissue (grey)
213
MRI (magnetic resonance imaging)
Uses electromagnetic properties of hydrogen nuclei to produce cross sectional images of internal body structures, best for soft tissues (excellent contrast), can show if CN VIII is absent or present
Primarily used for membranous labyrinth, IAC and intra-axial structures
Cannot have metal in body for this
214
PET scan (position emission tomography)
Uses radioactive tracer to identify active parts of the brain or tumors (including glomus tumors)
215
Etiology
The cause, set of causes or manner of causation of a disease or condition
216
Pathophysiology
The disordered physiological processes associated with disease or injury
217
Comorbid
Presence of one of more additional disorders co-occurring with a primary disease
218
Variable expressivity
Phenotype expressed to different degree among people with the same genotype
219
Epidemiology
Study and analysis of patterns, causes and affects of health and disease conditions in defined populations
220
Why is it important to recognize pinna variants? (4)
Can be associated with hearing loss and other syndromes
Funny looking ear can have a big effect on kids (social, emotional etc.)
There might be an effect on the transfer function
Might be challenging to give hearing aid (think atresia III)
221
Hairy tragus
Normal variant
Generally found in older men
222
Hairy pinna
Normal variant
Coarse hair
Usually in the lower helix
223
Pressure atrophy
Normal variant
Can get from glasses or hearing aids
224
Is prominent ear associated with HL or other audiological difficulties?
No hearing loss or difficulties are reported
225
Is cup ear associated with HL or other audiological difficulties?
No hearing loss or difficulties are reported
226
Is lop ear associated with HL or other audiological difficulties?
Possibly...is associated with some syndromes so may be associated with hearing loss but may not be the cause
227
Is stahls ear associated with HL or other audiological difficulties?
Otherwise known as Spock ear
Significant delineation of anti-helix
Possibly... is associated with certain syndromes so may be associated but not the cause
228
Tags and pits
From hillock of his (so present at birth)
Occurs in high rates in individuals with HL especially with some syndromes
Refer
229
Frostbite and burns
Refer
230
Aplasia
Nothing grew, nothing happened, no structure, failure of an organ or tissue to develop or function normally
231
Hypoplasia
Congenital condition
Incomplete development or underdevelopment of an organ or tissue
232
What is a possibility for every bony anomaly?
It can affect the facial nerve
233
Cystic cochleovestibular malformation (pseudo mondini)
Cystic cochlea lacking septae and the modiolus
Poorer hearing than classic mondini
234
Vestibular anomalies
Aplasia or dysplasia of lateral canal most common (happens late in development)
SNHL, mixed or CHL
Associated with CHARGE syndrome
May be present with cochlear anomalies
235
PSCD (posterior semicircular canal dehiscence) and LSCD (lateral semicircular canal dehiscence)
Clinically rare
Associated with a high-riding jugular bulb and fibrous dysplasia
Possible mild CHL to significant CHL
236
Prematurity
Low birth weight (under 6 pounds)
Co-existing factors- ototoxic, hyperbilirubinemia, in utero infection, craniofacial anomalies, syndromes, low APGAR score, mechanical ventilation for over 5 days, bacterial meningitis, family history of hereditary childhood SNHL, endocranial hemorrhage, hypoxic ischemic encephalopathy, convulsions, sepsis, GI surgery need, persistent pulmonary hypertension, intraventricular hemorrhage, patent ductus arteriosis ligation, lower SE
237
ECMO HL
Higher risk for progressive/delayed SNHL
238
TORCH
Toxoplasmosis
Other- syphilis, varicella, parvovirus, HIV
Rubella
CMV
Herpes simplex
Teratogens (such as FAS, dilantin, isoretinoin, thalidomide)
239
HIV
Hearing loss can occur
May be unilateral or bilateral, progressive, sudden
Can be CHL, SNHL or mixed
Greater risk for otitis media
240
Varicella
Rare now due to vaccine
Tinnitus is common and vertigo in 1/3 of cases
Unilateral mild-moderate SNHL
May cause sudden SNHL but that is rare
241
Measles
Rare now due to vaccine
Bilateral moderate to profound SNHL
242
Mumps
Rare due to vaccine
May cause profound HL that is often unilateral
243
What is the most common cause of intrauterine infection
CMV
244
What is the most common cause of non-genetic congenital SNHL in developed countries
CMV
245
Autosomal dominant inheritance
50% change of having a baby with HL, only need one copy
246
Autosomal recessive inheritance
Both copies of gene needed to have deafness element
Chance for offspring is 25%
Complete penetrance and expressivity
247
Syndromic auditory syndromes (15)
BOR, Treacher Collins, Apert, Congenital stapes fixation, Alport, Jervell & Lange-Neilson, Pendred, Waardenburg, Crouzon, Goldenhar, Stickler, Hunter, Usher, Down Syndrome, Pierre Robin, Wolfram
248
COMPT
Color
Other conditions (fluid level, perf, otorrhea etc.)
Mobility of TM
Position
Translucency
249
Perf TM
Often trauma related
Will see it in otoscopy, tymps will have big volume
CHL increases as size of perf increases, largest losses at lowest frequencies
Should heal without intervention but in a big enough perf may need a patch
Monitor, antibiotic drops or steroid drops maybe
250
Ossicular discontinuity
Case Hx may be- insertion of foreign body to the external ear canal, trauma, cholesteatoma, chronic OME
Tymp- Ad
Acquired CHL, may have SNHL too
Can have max CHL
Maybe get reflexes
251
Temporal bone fracture
May present with facial nerve with facial paresis or paralysis
Case Hx may include black eye, bruising and blood behind the ear; can be from a car accident or blunt trauma
Usually young males
SNHL if there is a transverse fracture and CHL if longitudinal
Balance disturbances
Tinnitus
Vertigo
CSF leak through fracture lines
