SLHS 570 ME Pathologies

TM perforation

• hole in the TM

• related to trauma (blasts, penetrating injuries, head trauma) or infections such as otitis media

• diagnosed by: otoscopy, large vol flat tymp

• CHL: large perf up to 50 dB of HL, largest at low freq., effect of perf location is small

• tx: heal spontaneously or can be patched, tissue applied for structure for layers to grow back

posterosuperior quadrant

top of x

anteroinferior quadrant

bottom of x

anterosuperior quandrant

side of x touching malleus and cone of light

posteroinferior quadrant

side of x not touching malleus

Monomeres

• healed perf with incomplete fibrous layer - results in transparency

• middle ear space cannot be seen clearly

• pneumo-otoscopy helps differentiate from perf

• can result in hypermobile TM or normal

• no symptoms unless severe sudden pressure changes

• perf more easily

tympanosclerosis

• scarring of TM after perfs or repeated infections and PE tubes

• thickened plaque that can impair vibrations, rarely measurable HL

• early - reddish or yellowish

• mature - chalky white

• worst case

  • can spread to ME and stiffen ossicles and TM

  • CHL and slightly reduced compliance in tymp

  • tx - removal and myringoplastry

otosclerosis

• progressive bone-remodeling disease affecting temporal bone

• most common cause of acquired conductive HL

  • Caucasians > Asians > African Americans - 10% of Caucasians, only 1% symptomatic

  • women more affected than men

  • pregnancy accelerates presentation of disease

  • presents between 2nd and 4th decade of life, could be any age, mean age at diagnosis has risen

Etiology:

• unknown

• hereditary - autosomal dominant with incomplete penetrance, (OTSC1-OTSC10)

  • 24-40 % penetrance

• viral (measles) hypothesis

  • RNA detected in majority of footplates

• sporadic mutation

  • no clinical or histologic difference between inherited and sporadic

Site:

• endochondral layer of otic capsule

• fissula ante fenestram, anterior to oval window

Progression:

• otospongiosis - preliminary, bone resorption and regrowth (bone tissue increase vascularity, become porous)

• otosclerotic lesions - new bone grows, vascular space become dense cellular

stapedial otosclerosis

• conductive HL

• ‘pure’ cochlear otosclerosis

  • SNHL debated and rare

(stapedial plus) Cochlear otosclerosis

otosclerotic focus is seen over to round window and promontory

• new bone extends into cochlea

• mixed HL

Otosclerosis natural history

• pt. with unilateral CHL (size of ABG proportional to dree of fixation)

• hearing loss advances and initially detected during pregnancy

• progresses: in severity, 80% eventually become bilateral

• symptom: tinnitus, dizziness or vertigo

otosclerosis audiology findings

otoscopy:

• normal TM

• schwartz sign: pinkish color over promontory (otospongiosis)

immittance:

• tymps are almost always normal, could be shallow (normal ME pressure - decreased compliance)

• width may reduce

acoustic reflexes elevated or absent with progression

• early sign: on-off (diphasic) reflex pattern before reflexes disappear

progressive CHL

• early stiffness pattern: ABG largest at low frequencies

• carhart’s notch - BC thresholds poorer, maximal at 2000 hz (about 15dB)

• over time ABG widen and BC scores drop

• 
  

unilateral loss

• can become bilateral

• 2 ears can show asymmetric progression

otosclerosis: schwartz sign

• reddish blush between malleus and incus

• result of hyperemic (increased blood flow) ME mucosa over active otosclerosis

otosclerosis: carharts notch

• BC threshold elevated, maximal at 2000 Hz

• not true sensorineural loss - reverses after stape surgery

• reflects change in BC vibration mode

• theories:

  • stapes fixation disrupts normal ossicular resonance at 2000 Hz

  • normal compression mode of BC disturbed bc of relative perilymph immobility

CI users with otosclerotic bone

• demineralized otic capsule conducts current beyond cochlea - dense capsule normally confines current inside; otosclerosis breaks that

• consequence: facial nerve stimulation - most otosclerosis specific CI risk

• audiologic outcomes not worse than other CI users

otosclerosis: differential diagnosis

• otitis media with effusion: type B tympanogram

• tympanosclerosis

• ossicular discontinuity: type Ad tympanogram

• superior canal dehiscence: low freq. ABG with a normal tymp and present acoustic reflexes BC may be better than 0 dB; low threshold VEMPs

• temporal bone trauma / ossicular disarticulation: history of head injury

otosclerosis management

1. observation: audios 6m, consider stable if no change over 18 mo.

2. hearing aid - always discuss, best used for motivated patient with bilat. loos, young pts with unilateral loss typically prefer surgery, consider possibility of progression

3. medical treatment

• sodium fluoride

• rationale: promotes recalcification of active otospongiotic foci and inhibits proteolytic enzymes - focus on inactive sclerotic phase is the goal

• evidence is weak and conflicting: systematic review found 2 placebo-controlled studies with opposite results

• concerns: GI upset, arthralgia, skeletal fluorosis, not standard of care in US

4. surgery

• goal: restore ossicular mobility

• remove fixed stapes and replace with prosthesis

• ear selection

  • poor ear first

  • 2nd surgery 6-10 mo later

surgical indications

• clinical suspicion of fixed stapes

  • CHL >25 dB and significant ABG of 15 dB or greater

  • negative Rinne (BC > AC)

• fully informed patient

  • SNHL is possible

  • tinnitus not improved by surgery

  • must comply with post-op activity limitations

• fully prepared surgeon

  • occasional stapedectomy is not good

otosclerosis: surgical contraindication

• stapedectomy on only hearing ear

• TM perforations

• active middle or external ear infection

• recent stapes surgery on other ear (<6 mo)

• menieres disease

• inadequate cochlear reserve

• continuing noise exposure

• expected noncompliance

• 
  

otosclerosis surgical options

stapedectomy:

• stapes is removed and replaced with a prosthetic stapes

• prosthesis will not be fixated by additional bone growth

stapedotomy:

• small (.6-.8mm) opening made in footplate with laser or micro-drill

• a piston prosthesis is crimped to long process of incus

• replaced stapendectomy:

  • less inner-ear exposer = lower risk of SNHL and post-op vertigo

  • more predictable air bone gap closure

• AR:

  • pre-op - document ABG, bone conduction (cochlear reserve) and speech scores

  • post-op audiogram at 4-6 weeks - expect ABG closure and reversal of carhart notch

Cholesteatoma

• abnormal collection of keratin-producing squamous epithelial cells in ME or mastoid - unilateral

  • often starts when a layer of TM becomes pinched off/forms pouch

  • skin/epithelial debris collects inside the cyst to form a mass

• pathophysiology

  • not a tumor, bone erosion driven by inflammatory mediators in perimatrix that activate osteoclasts, plus enzymatic breakdown and pressure from expanding sac

  • over time, cholesteatoma can get bigger and destroy surrounding ME bones leading to HL (surgery can improve)

• causes

  • acquired: poor eustachian tube function and infection in ME

    • more common

    • primary - starts from retraction pocket (pars flaccida/attic)

    • secondary - squamous epithelium migrates through existing perf

    • mean age 10 yo, peak in early teens

    • 
      

  • congenital: occur in ME and in nearby skull bones

    • can be at birth, usually dx’d at 5 yo, more males

cholesteatoma symptoms

• intermittent aural drainage with foul odor

• white mass medial to TM

• feeling of fullness or pressure and HL when cholesteatoma enlarges

• ache behind or in ear - at night

• retraction of pars flaccida or atrophic pocket

• perforation possible

• bone of ear canal eroded

• epithelial debris in EAC

• progressive unilateral HL

• dizziness or facial weakness (AR: vertigo or nystagmus by pressure changes = positive fistula sign)

• if untreated - deafness, brain abcess, meningitis, death (rarely)

cholesteatoma: audiology findings

• abnormal otoscopy

• tuning fork test consistent with unil. CHL

• thresholds vary by size and location (ABG as large as 40-50 dB)

• erosion into cochlea possible - mixed HL

• tymps vary

  • may be consistent with perf

  • dependent on degree of ossicular chain involvement

  • may show reduced mobility due to mass in ME space

• reflexes typically absent in conductive ear

cholesteatoma tx

• cleaning, antibiotics, drops (goal - to stop drainage in ear by controlling infection)

• surgical tx for complicated cases

  • goal - safe dry ear w/ preservation of normal anatomy, improve hearing

  • CT scans used to determine extent of bone erosion before surgery

  • one surgery to remove cholesteatoma and debride middle ear

  • canal wall up vs canal wall down: CWD leaves open mastoid cavity - affects EM fit, water precautions and HA selection

  • second surgery 6-12 months later; exploration and ossiculoplasty

• will recur if portion of mass is left in ME

Otitis Media

• infectious, inflammatory condition of the middle ear associated with an effusion behind intact TM

• associated with URI and eustachian tube dysfunction

• purulent effusion

  • thick and infected

  • acute otitis media

• serous effusion

  • thin/watery (air bubbles)

  • HL, blocked or stuffy feeling, fullness or pressure, clicking or popping on swallowing

  • retraction: indicate negative ME pressure - can affect atrophic portions of drum or suck in whole drum

• mucoid effusion

  • chronic otitis media

AOM

• bacterial or viral infection (80% bacterial)

  • strep

  • follows URI

• recurrent >= 3 bouts w/in 6 mo or >= 4 episodes in 1 year

• symptoms:

  • rapid onset of otalgia, irritability

  • hearing loss

  • fever - 50%

  • decreased appetite

  • otorrhea - if TM perf

  • ear tugging no reliable indicator

AOM incidence

• 2nd most common health problem in childhood

• highest incidence between 6-18 months

• incidence decreases after 7 years

• rare in adults: red flag - unilateral OME requires nasopharyngeal examination to rule out nasopharyngeal carcinoma

• AR: unilateral type B tymp w/ unilateral CHL → refer

• correlates:

  • Eustachian tube dysfunction

  • hereditary

  • daycare/large fam

  • bottle feeding - poorer immunity

  • smoking in home

  • low SES

AOM otoscopy and progression

• bulging, erythematous TM loss of landmarks

• effusion visible behind TM

progression

• TM after 12 h of otalgia

  • slight TM bulge

  • loss of landmarks

  • some effusion at base of TM

  • increased vascularization

• several days later

  • irritation, redness is improved

  • fluid line is clear

  • fluid appears to be suppurative (pus forming)

  • otalgia is resolved

AOM diagnostic criteria

• moderate to severe bulging of TM or new onset otorrhea not from otits externa; mild bulging plus otalgia of <48 hours or intense TM erythema

• do not diagnose:

  • without ME effusion - pneumatic otoscopy or tympanometry

COME (chronic OM with effusion)

• accumulation of liquid in ME cleft w/o inflammation or infection

• usually follows episode of AOM could be w/o

• chronic OME: effusion persisting >= 3 months

• can be persistent and relatively inactive

• persistent inflammation and chronic otorrhea through per is chronic suppurative OM (CSOM) associated with cholesteatoma

• etiology? infection of ME causes mucosal cells to become secretory; acute inflammation resolve sbut changes cellular structure of ME

COME aud findings

• otoscopy

  • TM is bulging or retracted and opaque

  • pneumatic otoscopy shows decreased mobility

  • serous or mucoid fluid

• CHL

• can cause mastoiditis or cholesteatoma

OME

• symptoms:

  • fever, malaise, pain, anorexia, inadequate sleep

  • poor attention span, social isolation, irritability

  • mild to moderate, fluctuating CHL

  • may impair speech/language development

  • may make learning more difficult

  • impacts family dynamics

  • one of the most expensive childhood conditions in US

• management

  • observation - most resolve spontaneously

  • watchful waiting with documented follow-up (anitbiotics, steroids, antihistamines, decongestants not recommended) - re-evlauate every 3-6 mo until effusion gone, HL found, or TM changes

  • surgery - PE tubes

  • autoinflation - balloon device, low cost and risk, need cooperative child

AOM management

• AOM gets antibiotics - first line high dose

• observation for non-severe AOM may be deferred 48-72 h w/ follow up

• rationale: ABX lowers rise of meningitis and mastoiditis; shortens duration of symptoms; lessens risk of perf

• follow up in 10 days

• recheck in 3 months if fluid still present

• course:

  • 1 mo - 40% still fluid

  • 2 mo - 20% still fluid

  • 3 mo - 10% still fluid (becomes chronic)

• PE tubes

  • chronic OME >= 3 months and hearing difficulty, balance, school performance, ear discomfort

  • get hearing eval before surgery

  • recurrent AOM: if effusion present

  • at risk children (developmental, sensory, syndromic): lower threshold applies

  • AR: 3 month hearing eval is AuD

  • good for comfort, fewer trips to physician, less antibiotic usage, outpatient surgery, improve hearing

  • water precautions not recommended, earplugs for dirty water, diving, pain with exposure

Glomus tumors (paraganglioma)

• benign, highly vascularized

• uncontrolled growth of paraganglia cells

• originate or invade ME space

• 1 in 1 mil people

• caucasian women 30-70 yo

Glomus tympanicum

• originates in ME

• reddish/bluish mass behind intact TM

• brown sign: mass blanches under pneumatic- otoscopy pressure

• symptoms:

  • pulsatile tinnitus (80%) - synchronized with heart beat, audible with stethoscope placed on ear; pulses observed during immittance test

  • CHL - if interfering with ossicular vibration (60%)

  • non-auditory symptoms: late stages - facial nerve weakness; hoarseness, swallowing difficult, shoulder drop;

Glomus Jugulare

• originates in jugular bulb

• grows unchecked to fill jugular bulb - impedes blood flow

• can extend into ME - often how tumor is found

• symptoms

  • same as glomus tympanicum - pulsing tinnitus, CHL

  • hoarseness and/or swallowing difficulties - CN close to jugular bulb

  • advanced cases - facial weakness

  • 1-3%: tumors secrete catecholamines into bloodstream - hypertension, tachycardia, sweating, diarrhea

glomus tumor vs Hemotypanums

• hemotympanums: presence of blood in tympanic cavity of ME - result of basilar skull fracture

• differential diagnosis

  • glomus: red, pulsatile, blanches with pressure, pulsations on tymp

  • hemotympanum: dark blue/purple, non-pulsatile, recent trauma or barotrauam, clears over weeks

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