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CSI – Birmingham
Challenging Clinical Case Studies
The Medical Model
You Be the Detective!
Important Studies,
Work-up, and Protocols

Alan Freint, MD
Paul Pessis, AuD
The Basic Premise
Where to Start?
Identifying the etiology of a health problem is essential
Pretend you're Sherlock Holmes
for proper treatment of any medical condition
Begin with the patient's account of the
Consider the fact that a hearing loss may be a
problem (the history)
manifestation of a systemic illness
Listen carefully to what is, and is not, being
Therefore, identifying the etiology of the hearing loss
is analogous to identifying the etiology of any
Complete a physical exam and diagnostic
other medical condition
Applying the concept of the "medical model" to the
Compile all the clues and consider the
work-up of a hearing or balance disorder is good
possibilities of what could account for the
medicine for the patient and for you
patient's problem (differential diagnosis)
What to Do Next?
What Comprises the Work-Up?
 What's missing from the puzzle?  Advanced audiologic studies  What further information do you need to  Radiologic studies identify the exact diagnosis?  Laboratory testing  What tests do you need to obtain to "fill in  Other diagnostic testing  Consultations with other healthcare  Would it be helpful to call in "Dr. Watson" for a consultation?  CT (Computerized
MRI (Magnetic Resonance
 Preferentially "looks"  Preferentially at soft tissues such as brain, spinal cord,  Ideal for examining nerves, muscle, etc.
 Better contrast mastoid cavity, and between different surrounding bones Carotid MRA
MRA (Magnetic Resonance Angiogram)
 Specialized MRI to evaluate blood vessels  Stenosis or occlusion  Dissection of an arterial wall  Arteriovenous Malformations (AVMs)  Utilizes gadolinium (not iodine contrast) The Scanners
Contraindications to MRI
 "Anything" metallic, if it's magnetic  Cardiac pacemakers and stimulators  Surgical clips in the head (particularly  Some artificial heart valves  Inner ear implants  Metal fragments in the eyes  Implanted pumps Lab Studies
Lab Studies (cont'd)
Complete Blood Count (CBC) with
Anti-nuclear Antibodies (ANA)
Red Blood Count (RBC)
 Antibodies (proteins) directed against one's own tissues (auto-antibodies)  Abnormal red blood cells  Propensity for the body to work against  White Blood Count (WBC)
itself is called autoimmunity  The ANA evaluates for the possible  Bacterial vs. viral etiology presence of autoimmunity, and  Erythrocyte Sedimentation Rate (Sed rate)
therefore, the presence of an  A basic measure of the degree of inflammation autoimmune disease Lab Studies (cont'd)
Lab Studies (cont'd)
Rheumatoid Factor (RF)
Autoimmune Diseases (Connective
 An antibody suggestive of rheumatoid  Sjögren's syndrome  Another test for autoimmune disease  Systemic lupus erythematosis (SLE)  May be present along with findings of other autoimmune disorders  Positive RF results may be seen in healthy  Raynaud's disease patients and in those with endocarditis, TB, syphilis, kidney, liver, or lung  Rheumatoid arthritis disease, and many other conditions  Autoimmune thyroid disorders Lab Studies (cont'd)
Lab Studies (cont'd)
Fluorescent Treponemal Antibody--
Free Thyroxine (T )
Absorbed Test (FTA-Abs)
Thyroid Stimulating Hormone (TSH)
 Measures the function of the thyroid gland  A screening test for syphilis for hypo or hyperthyroidism  A negative result is consistent with a  Evaluates the body's metabolic rate person not having syphilis, but it is most  Antithyroid Antibodies (Follow-up tests)
reliable in the middle stages of the disease  Antithyroglobulin and antimicrosomal antibodies are examples  A negative result may be seen in early  May be present in Hashimoto's thyroiditis, (primary) or late (tertiary) syphilis SLE, Sjögren's, RA, thyroid ca, and autoimmune hemolytic anemia Lab Studies (cont'd)
Lab Studies (cont'd)
Fasting Glucose
Blood Lipids— Fats that circulate in the
 Abnormal glucose levels may affect the blood stream; the two main lipids are: metabolic function of the body  Cholesterol—Essential to the structure
 Long-term disease (Diabetes) causes and function of the body's cells as well narrowing or occlusion of the small blood vessels by decreasing blood flow and as a building-block of certain hormones oxygenation which, in the ears, may lead  Triglycerides—important in the transfer of
energy derived from food into cells  Hemoglobin A1c (Follow-up test)
 High levels may accumulate and clog the  An index of mean blood glucose over the past blood vessels (atherosclerosis), causing 2-3 months, weighted to recent levels narrowing and decreased blood flow Lab Studies (cont'd)
Lab Studies (cont'd)
Lyme Antibody Screen
Kidney Function Tests—Measure how well toxins
 A test that looks for are excreted from the body antibodies to Borrelia
Blood Urea Nitrogen (BUN))—A breakdown
Burgerdorferi to indicate
product of protein, produced in the liver infection by the deer tick and excreted in the urine  Infection may affect the  Creatinine—A breakdown product of muscle,
cochlea and inner ear excreted in the urine structures, possibly  BUN/Creatinine Ratio—Helps to identify the
causing a unilateral SNHL type of problem affecting the kidney Jervell and Lange-Nielsen
Other Diagnostic Testing
Delayed recharging of the heart between beats--'d QT interval
Electrocardiogram (ECG or EKG)
May cause fainting and/or sudden death  Measures the electrical activity of the heart Associated with varying degrees of hearing loss, usually b/l  Includes the rate and regularity of the beats 16 Year-Old Asian Male
 Complained of a constant headache for  Pain radiated from the right temple to the  The throat was slightly sore; no cough  The hearing seemed "okay"; no tinnitus or  Some drainage was noted from the right Past History
 6 weeks earlier, the patient was seen the  Was taking Levaquin tablets, Floxin Otic another ENT and diagnosed with AOM Solution, Darvocet, and Fiorinal (for the in the right ear and treated with an headache) prescribed by another  3 weeks later, the fluid remained, so the ENT inserted a T-tube  Also self-medicated with Motrin, Tylenol,  Told to avoid water in the right ear  Advised to use steam every other day  Patient in obvious pain from headache (Mom  CT of the head with and without contrast wanted a second opinion)  CT of nose and sinuses  Pain radiated from right temple to back of head  MRI of brain with gadolinium  T-tube mostly extruded  FFL with topical anesthetics, if able  Suctioned some middle ear fluid through posterior  Consult pediatric neurologist pending  No apparent signs of being allergic results of the radiographic studies  Could not complete examination of the nasopharynx  Advil for pain; discontinue narcotics due to strong gag reflex  Audiogram and Immittance testing:  CT Sinuses: Nasopharyngeal mass, eccentric to
Audiometry: Normal puretone and
the right, with changes in the sphenoid speech audiometry findings  Tympanometry: Left ear - normal
MRI Brain W & W/O Contrast: Abnormal mass
Right ear - flat with slightly increased
with the epicenter in the right nasopharynx. volume, consistent with reduced The mass extends up to the skull base, patency of the T-tube abutting the right internal carotid artery at the  Ipsi Reflexes: Right - CNT
foramen lacerum region and crossing to the Left - Present
left of midline.
Radiographic Findings (cont'd)
Consider the Facts
 The lesion is associated with eustachian  16 year old male patient tube obstruction and right middle  Asian descent  Hx of unilateral persistent middle ear effusion  Proteinaceous cyst of the pituitary gland  Persistent headache for two months  5-6 mm diameter  No relief from pain medications and other  Marginal thickening of the right maxillary medical and non-medical treatments  Nasopharyngeal mass And the Answer Is…
 Chronic Otitis Media Nasopharyngeal Carcinoma  Nasopharyngeal Carcinoma  Scarring Secondary to Syphilis  Juvenile Nasopharyngeal Angiofibroma (JNA)  Nasopharyngeal carcinoma (NPC) accounts
for 85-95% of malignancies of the • North American and European Whites
nasopharynx (remainder are mostly • African, Eskimo, Polynesians
2 - 4:100,000
 80 – 90% of patients with NPC have increased
Southern Chinese (Guangdong Province,
antibody titers to Epstein-Barr Virus (EBV) Hong Kong, and Taiwan)
 Two thirds of NPC occur in males with the average incidence between ages 40-45 • First generation descendents of above
Appears to be a combination of two factors:
 The nasopharynx is located directly behind the nose at the top of the pharynx • Several genes are linked to NPC • Familial predisposition • Base of the Skull • Lateral walls of the nasopharynx (primarily • Studies show a two to threefold increase risk occupied by the eustachian tube) associated with dry salted fish and other salt preserved fish Anatomic Considerations (cont'd)
 NPCs possess an inherent propensity for  Majority of NPC originates in the lateral wall of the nasopharynx • Adjacent structures include:
 Internal carotid artery  Cavernous sinus  Mandibular nerve (V )  Cranial nerves: 9 – 12  NPC is a cancer of squamous cell origin  Appearance is similar to squamous cell carcinoma (SCC) of other areas of the  SCC typically arises from the surface epithelium as an outgrowth  SCC may spread to lymph nodes  Invades adjacent structures  Lymphatic spread occurs early, often before the primary lesion is discovered  40% spread to lymph nodes of both sides
 25% invade the base of the skull  Distant metastases occur more commonly in NPC than any other head/neck Ca • Lungs, Bones, Liver, Intra-Cranial extension Prognosis and Treatment
 1-year survival is approximately 85-90%  5-year survival is 30% even with early  Recurrent disease developed in half of the patients at 1½ years  Primary treatment is radiation therapy • From base of the skull to the clavicle  Chemotherapy may help metastases, but not the primary tumor. Cisplatin is the drug of choice
• Doesn't increase survival rate  Surgery adds very little benefit 50 Year-old Female
 The patient was riding her bicycle, hit a stone in the roadway, and was thrown over the handlebars  She was wearing a helmet, but  She sustained a short period of Past Medical History
 The paramedics brought the patient to  She had no prior history of head trauma, the ER where she complained of muffled hearing in the left ear, a loss of consciousness, vertigo, or "wet" feeling in the left ear, and vertigo (especially when bending backwards or lying down)  She was on no medications  There was no tinnitus, otorrhea, or  Her health was excellent otalgia; the right ear was fine Exam of the Ear
 The patient had bruises over the left  The TM was intact; temporal region and left mastoid dark fluid in the left middle ear with air  There were no lacerations  There was a visible  There were small amounts of fresh abnormality along blood in the posterior bony ear canal superior canal wall  Right 10
 There was no nystagmus  The Romberg test was negative  100% bilaterally
Right Normal
 The remainder of the neurologic test suggested only a concussion  Right Normal
 Facial nerve function was normal  Right
Failed (2k & >)
Consider the Facts
 Rotary nystagmus was recorded in the  Head trauma
head-hanging right position of the  Blood in the ear canal
Dix-Hallpike maneuver  Blood in the middle ear
 Horizontal pursuit and optokinetic nystagmus were normal and  Vertigo
No nystagmus
 Calorics were balanced  SNHL of the left ear
 No other abnormalities were seen  CT scan
Concussion with Vertigo  Non-displaced longitudinal fracture of the
left temporal bone
Ossicular Dislocation  Extended from the mastoid through the
posterior superior external auditory canal
Idiopathic SNHL, Left Ear to the TMJ
MRI scan
Temporal Bone Fracture • Frontal lobe contusions b/l
Longitudinal Temporal
And the Answer Is…
Non-displaced Longitudinal Fracture
of the Temporal Bone
Labyrinthine Concussion with Vertigo
Temporal Bone Fractures
 Constitute 80%-90% of temporal bone fractures Two basic types:
 Often pass through the superior canal wall to the anterior canal wall and TMJ • Follows the long axis of the petrous bone  Usually associated with:
• Generally results from a blow to the temporoparietal region (side of the head)  Hemotympanum (blood in the ME)  Perforation / tear of the TM  Ossicular dislocation • Crosses the long axis of the petrous bone  Both a CHL and a high frequency SNHL (due to • Usually secondary to a blow to the frontal or labyrinthine concussion) occipital regions  May transect the:
 Fracture extending  Bony labyrinth
from the outer skull through the superior  Cochlea
wall of the EAC and  Internal auditory canal
through the facial nerve  May result in permanent hearing loss and /or  The malleus is dislocated from the  Associated with facial nerve paralysis in up to 50% of the cases  Common denominator is  Delayed endolymphatic hydrops may  Dislodging of otoconia has been  Bottom:
postulated as a cause for positional vertigo of the post-traumatic type if there is no fracture of the labyrinth  Based on findings of exam and CT scan  Serial audiograms to measure changes or  High-dose steroids to reduce improvement in hearing inflammation and swelling of the  If a CHL remains, surgery of the middle ear cochlea, cochlear, vestibular, and for ossicular reconstruction and TM facial nerves, etc.) repair (if needed)  If facial nerve paralysis is present,  If a significant SNHL remains, HAE exploration of the nerve pathway to  Physical Therapy (PT) or canalith remove bone fragments and re- repositioning may be helpful attach the "cut" ends of the nerve  Seen emergently with complaint of frontal headache associated with a sinus infection lasting two weeks  Complains of imbalance, nausea, and tinnitus, and reduced hearing  Feels his foot isn't "planting" itself appropriately; has fallen a few times within the past few months Further Past History
 Taking antidepressants. Was hospitalized recently with head trauma and LOC for one  Dry cerumen was removed minute due to a fall  Epithelial inclusion cyst – left concha • Had MRI/CT/EEG/MRA/ECHO Stress Test
 Crusty yellow mucus from the nose • Tests were all normal; discharge diagnosis was reaction to his antidepressants consistent with acute rhinosinusitis • Current meds replaced with new family of  Drags left foot when walking  Diabetic – glucose levels are stable  Results consistent with cochlear hearing  High frequency asymmetric SNHL right loss: Absolute and interpeak latencies ear worse than left  WRS: Right: 76% Left: 92%
 Nice waveform morphology even with  Normal tympanograms, normal acoustic significant high frequency hearing loss reflexes, and no reflex decay noted  Increased click rate studies show expected shift in wave V, bilaterally Things to Ponder
 Patient could not discontinue medications: • Clonazepam, and Lexapro  Patient is very anxious explaining some  Results:
symptoms but not all • Abnormal saccades for both rightward and  Description of stumbling and "foot not leftward movement planting well" may support etiology • Ocular flutter noted without fixation other than the "ears" • Difficultly keepings eyes open during caloric  Recent head trauma testing; Left cold caloric weakness Could not tolerate any further testing due to  Just started stronger sinus medications uncontrollable headache  VNG with meds not conclusive Impression: Central pathology or effects of
medications causing central findings  Although radiographic studies are only 3  Drug and/or alcohol overdose months old, recommend repeat due to change in central symptoms • Recommend repeat CT studies  Poorly fitting orthotics  Neurological consult ASAP  Subdural hematoma And the Answer Is:
Subdural hematoma  Bilateral subdural hematoma, right larger • Heterogeneous density within the right subdural hematoma raises the question of a chronic problem with an acute worsening, while the left subdural hematoma appears chronic • There is a resulting underlying mass effect on the right cerebral hemisphere and midline shift towards the left Radiographic Studies (cont'd)
 Drainage of right frontoparietal subdural  One month post-operative Brain CT • Right-sided catheter was in place • Right frontal and parietal burr holes • Decrease in left-sided subdural collection • Placed right subdural drainage catheter from pre-operative CT Results: Patient felt relief. Intracranial air was
• DECREASE in the amount of post-operative present. There was resolution of ventricular compression and midline shift. Low density fluid still remained in the posterior frontal- • Subdural collection in the left frontal high convexity region was slightly increased  An ACUTE subdural hematoma (SDH) is a
Subacute phase begins 3-7 days after
rapidly clotting blood collection below the layer of the dura but external to the brain and  Chronic phase begins about 2-3 weeks
arachnoid membrane after acute injury  Two further stages, SUBACUTE and
CHRONIC may develop with untreated
 Often associated with blunt head trauma • SDH is the most common type of intracranial Each type has distinctly different clinical,
mass lesion, occurring in 1/3 of those with pathological, and imaging characteristics
severe head injuries  Acute SDH is associated with high mortality and  Interhemispheric SDHs are often associated with child and elder abuse  Simple SDH is has a 20% mortality rate  Suspect acute SDH whenever there is severe blunt head trauma  SDH is more common in people older than 60 years (elderly have less resilient bridging  Patients lose consciousness, but this is  Chronic SDH is more difficult to anticipate  SDH is also common in infants because and ½ offer no history of head trauma. adhesions existing in the subdural space are C/O's are unexplained headache, absent at birth but develop with aging personality changes, increased ICP Possible Post-Operative
Imaging Studies For SDH:
• Noncontrast CT is the primary means of  New or recurrent bleeding/hematoma making a diagnosis • Midline shift is not uncommon • Uncommon in the posterior fossa since the cerebellum undergoes little movement • Recurrent hematoma (50%) • Imaging should occur 48-72 hours after head • Seizures (up to 10%)  Follow-up with neurosurgeon for serial CT  Typically can't be determined at the time of the emergency evaluation  Ultimate prognosis is related to the  Physical therapy amount of associated direct brain  Reevaluation of "polypharmacy" to address damage and the damage resulting from vestibular side effects the mass effect of the SDH  Hearing aid evaluation 6 Year-old Female
 H/O alternating infections in the ears  Passed screening audio in nursery school  Five episodes of AOM during the past year, even in warm weather  She exhibited decreased hearing  Articulation was poor  PE: Bilateral glue ears with retracted tympanic
Right ear: exhibited a moderate high-frequency
 The child never returned for a post-operative visit, post-operative audiogram, or follow-up  She reappeared 18 months later with  Left ear: showed a mild CHL (30dB bone
worsening speech clarity. Mom described conduction thresholds at 2000hz and 4000hz) the speech as "sloppy"  WRSs were excellent bilaterally  Both tubes had extruded and were lying in the wax in the ear canals  Tympanograms were flat  The TMs were thickened and retracted  Acoustic reflexes were absent The Current Audiogram
 Documented a bilateral mid-to-high frequency mild-degree sensorineural  A CT scan of the middle and inner ears hearing loss with a mild bilateral conductive loss at 500Hz showed normal inner ear structures  The right tympanogram showed a shallow and broad peak at –225 and chronic mastoiditis bilaterally daPa; the left tracing had a sharp peak at –150 daPa Consider the Facts:
You Be the Detective!
Laboratory testing:
 Speech disorder • Normal results:
 Mixed (mostly sensorineural) hearing loss  CBC with differential  Normal ossicles and inner ear  Fasting blood glucose  ANA, RF, FTA-abs structures on CT scan • Abnormal results:
 Elevated sed rate  Sedimentation (sed) rate  Elevated C-reactive protein  C-reactive protein And the Diagnosis Is:
Congenital SNHL with underlying COM Noise-induced hearing loss Auto-immune Inner Ear Disease
Auto-immune inner ear disease (AIED) Chronic Otitis Media
Chronic otitis media Auto-immune Inner Ear
Auto-immune Inner Ear
Disease (AIED) (cont'd)
 AIED is a connective-tissue disease that  Typically marked by a rapidly is mediated by the immune system progressive bilateral SNHL, but it can  About 30% of patients with elevated labs will exhibit systemic immunologic disease like rheumatoid arthritis,  Most common in middle-aged females, Sjøgren's syndrome, lupus, and but may occur in both sexes at any age other similar diseases Auto-immune Inner Ear
Course of the AIED
Disease (AIED) (cont'd)
 Progressive SNHL  Usually bilateral • Immunosuppressant drugs • Steroids (prednisone, etc.)  More common in females  The earlier the medical intervention,
Associated complaints:
the better the chance for recovery
• Ménière's-like vertigo  AIED is recognized as one of the few
• Ataxia or unsteadiness reversible causes of SNHL
Otitis Media
 Myringotomy with placement of a new set of  Depends on the activity of the underlying ventilating tubes will remedy the eustachian tube dysfunction and correct the  In patients who rapidly re-develop hearing loss,very small doses of steroids given in pulsed, or every-other-day,  The child will need to be monitored over time for regimens may maintain the hearing further recurrence of middle ear effusions level with relatively mild side-effects Natural Course of Otitis Media
 Tubes remain in place for an average of one year, but may extrude in as little as 3 months, or stay in as long as 2-3 years  Approximately 50% of children will need
multiple sets of tubes
 The other 50% may out-grow the problem and develop normal eustachian tube function Otoscopy Made Easy
 It is impossible to predict when the child will no longer have otitis media  Select an otoscope with a good light  Optimally, visualization occurs when the acoustic meatus is in line with the canal  For adults, pull the auricle upward and  Use largest speculum that can be tolerated  In children, the auricle should be pulled downward and backward  External auditory canal can be sensitive, so  Hold the otoscope like a pen/pencil and use "explore" with a gentle touch the little finger area as a fulcrum. This prevents injury should the patient turn  Evaluation under a microscope is optimal  Auditory Canal: hair, often with yellow to
brown cerumen (ear "haircut"??)  Tympanic Membrane:
• Pinkish gray in color, translucent and not A normal tympanic membrane • Malleus lies in oblique position behind the (TM) as seen through the upper part of tympanic membrane otoscope. The TM is in normal position, gray, and translucent A: Pars flaccidaB: Short process of malleusC: Pars tensa (AS)D: Manubrium of malleusE: UmboF: Light reflexG: Pars tensa (AI)H: Promontory of cochleaI: Grains of sandK: Round window nicheL: Pars tensa (PI)M: Incus: lenticular processN: Chorda tympaniO: Incudostapedial jointP: Incus: long processQ: Pars tensa (PS) Note the light reflex. The absence of a light reflex doesn't necessarily indicate middle ear effusion Normal Eardrums - Notice the
External Otitis (Swimmer's Ear) This is an
different shades of color: The
infection of the ear canal itself. Notice the
eardrum still remains an opaque
translucent appearance in all the

swelling of the ear canal
Fungal infection of the ear canal
Stages of Otitis Media
In this patient with OME, the TM is seen as retracted, faintly amber and white, and semi-opaque. A small air-fluid interface is seen anterosuperiorly Serous Otitis - fluid build up is seen behind
the eardrum. Common in children
with chronic allergies and/or
inflammation of the Eustachian tube

In this patient with AOM, the TM is seen as bulging, white,and opaque Acute Otitis with bulging of the tympanic membrane due to
pressure from purulence (pus) behind it. The last picture reveals an ear tube that has gotten prematurely blocked and the ear is once again infected And the Diagnosis is?
Ear Drum Perforations
Serous Otitis Media Diagnosis? What Kind? Acute Otitis Media Attic Cholesteatoma Spider in the ear canal 5 year-old male
 Normal shape and size of auricles  Referred after failing a screening audiogram in one ear  Normal tympanic membranes  No history of chronic ear infections  Healthy middle ears  No family history of hearing loss  The remainder of the physical exam was  Speech acquisition was age-appropriate  No tinnitus or imbalance  General health is good  In view of the asymmetric SNHL, the following tests/labs were ordered:  Right ear: normal hearing at all
• CT scan of the inner ear  Left ear: mild-to-borderline moderate low
frequency SNHL rising to normal at  CBC with diff  Thyroid function tests  SRTs: Right 5 dB
Left 15 dB
 Triglycerides  WRS: Excellent bilaterally
 Lyme antibody screen  Tymps: Normal bilaterally
 Fasting blood glucose  Pediatric ophthalmologic evaluation  Labs: all normal  EKG, Urinalysis, Retinal Exam: normal  For examination of the retina  CT scan: Abnormal appearance of the  Electrocardiogram cochlea with partial absence of the  To r/o a prolongation of the QT interval bony partition between the apical and middle coil, consistent with a variant of  To r/o protein in the urine Mondini's Aplasia (anomaly)  Deformed cochlea in which only the basal coil can be identified clearly Mondini's Aplasia (anomaly)  Not necessarily bilateral  65% bilateral  35% unilateral Mondini's Aplasia (anomaly)  Hearing loss is more apt to be progressive if there is an absence of Mondini's Aplasia (anomaly) the bony divisions between coils of the cochlea More to Know
May also include (but may occur on its own):  Serial hearing diagnostics to document ENLARGED VESTIBULAR AQUEDUCTS
potential progressive hearing loss • There is a chance for progressive hearing loss  Consideration for hearing aid(s) and/or • Usually is bilateral, but not necessarily • Hearing loss is not always present • Head trauma, such as with contact sports, should be  Educational audiologist referral avoided since hearing loss may be precipitated 46 year-old school psychologist
 She had a URI followed by 10 days of  5 days before her OV, she suddenly developed a blocked right ear, right otalgia,and right-sided tinnitus  She denied vertigo or otorrhea Past Medical History
 The patient's father had an acoustic  The malleus and attic of the right ear appeared reddened (consistent with  The patient was fearful that she, too, was developing an acoustic tumor  The tympanic membrane and middle  She had no café-au-lait spots  Her PCP treated her with two different  The left ear was normal antibiotics without relief (she actually  The remainder of the ENT exam was  To treat the AOM, the patient was  There was a mild conductive hearing placed on a sulfa-based antibiotic, loss in the low frequencies in the right ear, with a mild sloping-to-moderate SNHL component in the  To treat the sudden SNHL (SSNHL), she higher frequencies was given prednisone (a steroid), beginning at a high dose, and tapering  Hearing for the left ear was normal down over a three-week period  The word recognition score was reduced slightly on the right Three Weeks Later
 The patient stated that she felt better  Normal
and the hearing was "back to normal" • CBC with differential --Fasting Glucose  The AOM resolved; the TM was healthy --Thyroid testing  The repeat audiogram documented symmetric hearing now. The word • Lyme Antibody recognition score improved to 100%  Abnormal
 The ABR was normal and symmetric >640 dilutions Consider the Facts
 Normal sed rate Auto-immune inner ear disease (AIED)  Improvement after a course of antibiotics and steroids SSNHL secondary to AOM Sudden Sensorineural
And the Diagnosis Is:
Hearing Loss (SSNHL)
 Defined as a loss of 20dB or more over at least three contiguous frequencies SSNHL secondary to AOM
occurring within three days or less  Etiology not identified in 85-95% of all  Increased incidence with age  No gender predominance Sudden Sensorineural
Possible Causes of SSNHL
Hearing Loss (SSNHL) (cont'd)
 For this patient, it appears that the etiology is infectious
 The prognosis is best if recovery begins  The prognosis is worse the longer the  15% of patients have progressive Acute Otitis Media (AOM)
Treatment of AOM
 May be bacterial or viral  Antibiotic therapy for at least 10 days  Bacterial is the most common form of  Occasionally, a myringotomy is middle ear infection performed to drain the pus and relieve pain and pressure  Typically, AOM is confined to the middle ear, but in unusual circumstances,  If there is pus in the middle ear and facial paralysis is present, a "wide" can penetrate the barrier between myringotomy is indicated the middle and inner ear at the round  Steroids when SSNHL is present with or without tinnitus and/or vertigo  Due to early intervention and full recovery of the hearing loss, it is expected that this problem is resolved  There should be no concern for 44-Year Old Male
 Presented with a complaint of a plugged left ear  Normal ENT exam  5'10'' tall, weight 254 pounds  Tinnitus began two months prior to his office visit with the onset of vertigo and  Hearing Evaluation: nausea during the past month • Flat 50dB SNHL with 96% word recognition  Family history of elevated cholesterol score for the left ear  Vertiginous with movement for 2 months • Slight high frequency SNHL with 88% word recognition score for the right ear Objective Findings (cont'd)
Objective Findings (cont'd)
 Tympanograms and acoustic reflexes were normal for the right ear and absent (contra and ipsi for the left) • Positive for "mild" diabetes  ENG showed a 30% caloric weakness • Elevated Triglycerides  ABR was consistent with cochlear • Elevated Cholesterol  MRI studies were normal And the diagnosis is:
Viral Labyrinthitis Ménière's Disease Hyperlipoproteinemia (elevated blood Multiple Sclerosis  After several additional months on the diet  Vasodilators (such as nitroglycerin) for one month yielded slight with exercise, weight loss improvement of hearing loss, but the tinnitus and vertigo remained occurred, tinnitus and vertigo  He was placed on a low-carbohydrate, improved, and the hearing almost high-protein diet  Consultation with dietician returned to normal  Exercise program  It is postulated that lipid (fatty) deposits in the capillaries cause obstruction of  Studies indicate that up to 5% of the stria vascularis which leads to patients with inner ear disease have oxygen deprivation and injury to the  Another study found that 12% of  With proper diet and vasodilators, the patients presenting with SNHL of hearing loss may be reversible over unknown etiology, had elevated  The majority of patients with lipid disorders present with obesity  Low-carbohydrate, low-fat, and high-  Symptoms include hearing loss, tinnitus, and vertigo; most common are tinnitus and hearing loss  Serial audiograms and blood tests 20 Year-old Female
 Known to our practice since a young girl when treated for chronic otitis media  Had moved away for 11 years  Last audiogram in our office yielded normal hearing bilaterally  Currently, complains of reduced hearing bilaterally and is wearing binaural hearing aids dispensed from another facility 20 Year-old Female (cont'd)
 Her mother accompanied her daughter and  Normal otologic exam reported that she is disappointed that the dispensing facility did not identify the  Audiogram showed bilateral profound reason for the hearing loss sensorineural hearing loss  The patient reports satisfaction with her  Acoustic reflexes were present current hearing aids, but felt her hearing loss may be worsening  ABR yielded normal tracings  Had worn glasses for many years without  OAE responses were normal change in prescription  MRI of brain, posterior fossa, and the Functional (non-organic) hearing loss internal auditory canals was normal Auditory neuropathy  Extensive blood work-up –normal  Speech – normal, but late onset of the hearing loss was noted And the Diagnosis Is:
 Person truly believes deep within the mind that he/she can't hear  Related to, but different from, malingering Functional hearing loss; a type of Conversion Reaction  Malingering: person feigns the hearing
loss. He/she is consciously motivated and wants to gain benefits from being hearing impaired Higher prevalence in females of lower  Must be confident with the diagnosis socio-economic status before confronting the patient Persons with hearing loss conversion reactions typically have a prior  Once confident, proceed with kindness, history of chronic otitis media but have a plan of action Commonly, patients are of average or  This patient had a deaf boyfriend at below average intelligence Gallaudet University and was The degree of the functional hearing identifying with the "deaf world" loss is associated with the severity of the psychological problem  Psychotherapy, often including hypnosis  Prompt resolution desired in order to prevent chronicity and secondary gain  Serial diagnostic hearing evaluation 14 Year Old Female
 Hearing loss since birth; unknown etiology  Complains that left ear is distorted and  Subarachnoid bleed at delivery; child doesn't feel hearing aids are helping went home at three days  Currently complains of feeling vertiginous especially in the morning  Did not require oxygen  Headache and facial pain  Had a "work-up" (Mom not sure what  Wears BTEs and is followed by educational was ordered) by an ENT; reported as  Refuses to wear FM system  Siblings have normal hearing  Bilateral asymmetric sensorineural Progressive hearing loss related to puberty hearing loss worse in the lows (left (hormonally induced) worse than right)  Word recognition score: right 96%
Undiagnosed intra-cerebral hemorrhage  Serial audiograms show a sensorineural SNHL of unknown etiology worsening of the better ear And the Diagnosis is…
 Complete lab testing and radiographic studies were negative, except for:  Lyme antibody screen
Western Blot: a specific test for
confirmation of reaction to
Borrelia Burgdorferi, the
spirochete that causes Lyme

 Transmitted by a deer-borne tick (Ixodes
 Severe headache  Neck stiffness  Facial paralysis  More specific to the Northern climates  Fever and chills  Patient was at camp in the "Woods"
 Profound fatigue  Initial sign is a rash at the site of the bite  Multiple neuropathies in 20% of patients  Cases where facial paralysis is noted, Lyme disease is present 50% of the  Cervical adenopathy  Oral antibiotics for three-to-four weeks  Good chance for recovery to prior  Word recognition score may improve, as  Ceftin
 Once treated, there should be no  Serial audiograms to monitor for hearing recurrence unless there is new Diagnosis?
Ear of corn

Advice: When you hear the
sounds of hooves,
think horses…not zebras!


Patient Information VIRACEPT® (VI-ra-cept) (nelfinavir mesylate) TABLETS VIRACEPT® (VI-ra-cept) (nelfinavir mesylate) ORAL POWDER VIRACEPT can interact with other medicines and cause serious side effects. It is important to know the medicines that should not be taken with VIRACEPT. See the section "Who should not take VIRACEPT?"

Rhetoric and hype: wheres the ethics in pharmacogenomics?

Am J Pharmacogenomics 2003; 3 (6): 375-383  Adis Data Information BV 2003. All rights reserved. Rhetoric and HypeWhere's the ‘Ethics' in Pharmacogenomics? Bryn Williams-Jones1,2 and Oonagh P. Corrigan1,3 1 Centre for Family Research, Faculty of Social and Political Sciences, University of Cambridge, Cambridge, UK2 Homerton College, University of Cambridge, Cambridge, UK3 Cambridge Genetics Knowledge Park, University of Cambridge, Cambridge, UK