Zion AM report: Rethinking tinnitus and eustachian tube dysfunction!

Thanks Molly for presenting the case of a young woman in primary care for aural fullness and episodic L>R “dripping” sound in her ears – found to have eustachian tube dysfunction from middle ear myoclonus! This was a great opportunity to review our history taking for otologic complaints, redefining tinnitus, referral decisions, and management of eustachian tube dysfunction!

**Shameless reference to a previous Zion ambulatory report blog on approach to tinnitus!

Consider the following exam maneuvers when evaluating patients with hearing/otologic complaints:

  • External ear – evaluate the pinna for pain/rash, TMJ for clicking/sticking, mastoid process for pain
  • Middle/inner ear – otoscope exam! Evaluate for presence of cerumen (and have removed by MAs if can’t see TMs!!), appearance of TM (should be shiny & translucent), presence of TM retraction/effusion/perforation
  • Nose, sinus, oropharynx exam – check for signs of allergic rhinitis, sinusitis
  • Check for bruits – neck, mastoid, and preauricular areas if c/o pulsatile tinnitus
  • Neuro exam – especially if complaints of headaches, balance/gait dysfunction, visual complaints, vertigo!
  • Gross hearing exam – can use whisper test or finger rubs
  • Tuning fork exam if complaints of hearing loss:

Image result for rinne and weber tuning fork

Remember tinnitus is ANY perceived sound in the absence of an external source – two broad ways to differentiate etiology:

**Steph shared a key tip – ask patient’s to “mimic the sound they hear”, can be easier than asking patients to describe in words!

  1. Vascular – key features = pulsatile (heartbeat!), positional, associated with bruits
  2. Non-vascular – most common cause = presybacusis but see below for a wide variety of causes!

*Make sure to rule out barotrauma and head injury as possible etiology of tinnitus!


Key features of middle ear spasm/myoclonus:

  • Unilateral clicking, buzzing, or “dripping” tinnitus
  • Sometimes examiner can hear the sounds
  • Caused by repetitive and synchronized contractions of the tensor tympani and stapedius muscles
  • Very rare!
  • Treatment is usually conservative – manage expectations, limit factors that exacerbate tinnitus (stress reduction, sleep hygiene, limit caffeine intake, stay hydrated), for severe cases can consider botox or surgery!

The limited treatments for Eustachian tube dysfunction:

  • Most important – treat the underlying cause!!
    • Allergic rhinitis – intranasal steroids, antihistamines
    • Rhinosinusitis – intranasal saline (Era swears by NeilMed Nasal Rinse!), intranasal steroids, +/- antibiotics
    • Laryngopharyngeal reflux – dietary modifications
  • In the absence of one of the above causes, very limited data to support our commonly used medications – benefit lacks for systemic decongestants like pseudophed, antihistamines, nasal steroids!



Wu V, Cooke B, et al. Approach to tinnitus. . 2018 Jul; 64(7): 491–495.



Week of Pearls: Behcets, Lithium Tox, Cavitary Lung Lesions & Hyperammonemia

From the last week of amazing cases… Thanks to all of those who presented! Selected pearls below.


Behcet’s Disease

Epi: Highest prevalence along the “Silk road” with Turkey having the highest prevalence. Age range 20-50’s

Clinical Presentation:

  • Uveitis (can be Anterior OR posterior)
  • Ulcers (urogenital ulcers occur in 75% of pt’s w/ Behcets)
  • Vasculitis (Any size, arterial or vascular)
    • Beware pulmonary artery vasculitis
  • Cutaneous lesions (various rashes from aceniform lesions to erythema nodosum to EM and PG-like lesions)

Dx: There are no pathognomonic lab tests for Behcet syndrome. Diagnose using ISG criteria


Tx: Syndrome is relapsing and remitting. Goal is to suppress exacerbations to prevent organ damage. Treatment depends on the organ(s) involved.


Related pearl: Note the difference between diagnostic criteria and classification criteria. “Disease classification criteria are developed to categorize patients for research studies and are not intended to diagnose individual patients. The classification criteria favor increased specificity at the expense of sensitivity to avoid misclassifying patients as having a disease, which would compromise the results of research studies.”
To see how a master diagnostician would think through a similar case, check out this clinical care conundrum featuring our own Gurpreet Dhaliwal and Brad Monash https://www.journalofhospitalmedicine.com/jhospmed/article/148442/hospital-medicine/thinking-outside-checkbox


Jimmy Feeney’s Approach to Hyperammonemia!

The key is that although hepatic causes (namely, the many causes of decompensated liver disease) are the most common, there are also many nonhepatic causes. They can be grouped into increased production of ammonia and decreased metabolism. See the diagram below.


Non hepatic causes of hyperammonemia

-Impaired elimination of ammonia

  • Inherited Urea Cycle Disorders
  • Portosystemic shunts
  • Urinary diversion – ureterosigmoidostomy, ileal conduit
  • Medications
    • Valproic acid
    • Chemotherapy agents – 5FU, asparaginase

-Excess (production) of ammonia

  • Urease producing UTI (i.e proteus)
  • Multiple myeloma, CML, AML
  • Large GI bleed
  • Significant endogenous dietary protein

-Other (rare)

  • Reye syndrome (Peds)
  • Primary carnitine deficiency


The (different) Differential for Cavitary Lung Lesions vs. Cystic Lung Disease

Cavitary Lung Lesions

  • a. Infectious: Reactivation Tb (primary Tb = nodule w/ LAN, military =  small diffuse nodules), bacterial (anaerobes 2/2 aspiration, staph/strep 2/2 septic emboli from endocarditis vs. primary pneumonia, klebsiella, actinomyces, rhodoccocus), fungal (histoplasmosis, blasto, cocci, crypto), molds (aspergillus)
  • b. Malignancy
  • c. Vasculitis: GPA
  • d. Aspiration of foreign body

Cystic Lung Disease (thinner walled cavities, much more rare diagnoses)

  • LAM (lymphangioleiomyomatosis)
    • Think females in 30’s-40’s with pneumothorax, pleural effusion
  • LIP (lymphoid interstitial pneumonia)
    • Confined to the lungs, but is associated w/ other autoimmune diseases including Sjogren syndrome
  • PLCH (Pulmonary Langerhans)
    • Think young adults, 20% have extrapulmonary manifestations (cystic bone lesions, DI)
  • BHD (Birt-Hogg Dube Syndrome)
    • Think young adults with lung, kindey and skin involvement

Lithium Toxicity

Risk factors 

  • Medications: especially Loop diuretic use!!! A 2004 case-control study in Ontario in pts > 66 yo showed: (thanks to Greg Ow!)
    • Increased rates of admission for lithium toxicity for the 28 days after pts on chronic lithium were started on a loop diuretic (2.9% vs 0.4%, RR 5.5)
    • Lithium toxicity admission rate was also increased for ACEi initiation (3.4% vs 0.3%, RR 7.5)
    • Classically NSAIDs also increase risk, though data has been mixed
  • Worsened renal function
  • Volume depletion

Clinical features

  • Sluggishness, confusion, ataxia
  • Neuromuscular excitability including tremors, fasciculations, myoclonic jerks


  • Fluids
  • Stop lithium
  • Dialysis if levels are very high


Compare and contrast this with NMS and SS, both of which usually include autonomic signs like HTN and hyperthermia that should be absent in lithium toxicity.

Pearl: Opioids have serotonergic properties, so beware high doses in patients on an SSRI!

Serotonin Syndrome vs NMS (thanks to a prior post!)

nms SS


Mt. Zion report: ALS!

Thanks Lev for presenting the case of an elderly man with weight loss, fatigue, and progressive dyspnea on exertion ultimately diagnosed with ALS! This case was such a good reminder to think about the broad differential diagnosis for subacute fatigue and weight loss, including neurologic causes! See below for pearls from the case.

Quick review of differential diagnosis for fatigue:

Heme: Anemia
Endocrine/Metabolic: Adrenal insufficiency, hypothyroidism, uncontrolled DM, electrolyte derangement, hypercalcemia, hypoglycemia, hypoaldosteronism
Cardiovascular: CHF, CAD, pulmonary hypertension
Neurological: steroid myopathy, myasthenia gravis, polymyalgia rheumatica, ALS, polymyositis, Lambert Eaton
Renal: CKD
Infection: HIV, fungal, syphilis, TB, scabies, subacute endocarditis, hepatitis, occult abscess
Pulmonary: COPD, ILD
Infiltrative disease: sarcoidosis
Malignancy: multiple myeloma, hematologic malignancies, age-appropriate solid tumors
  • Classically presents with upper and lower motor neuron findings:

    • UMN – hyperreflexia, spasticity, pathologic reflexes (jaw jerk, babinski)

    • LMN – weakness, muscle atrophy, fasciculations, hyporreflexia

  • Consider ALS diagnosis in patients with:
    • Weight loss and weakness – especially if both are progressive
    • Dyspnea, quiet voice (signifies diaphragmatic weakness)
    • Bulbar weakness – dysphagia (even coughing or choking with meals can represent poor ability to clear secretions), dysarthria
    • Asymmetric limb weakness
    • Cognitive changes – impaired executive function, apathy, overlap with FTD and Parkinsonism
    • Muscle cramps


  • ALS mimickers:
    • Cervical radiculomyelopathy (LMN signs at the level of lesion with UMN signs below it)
    • Inflammatory myopathy – polymyositis, dermatomyositis
    • Thyrotoxicosis (UMN signs with pyramidal tract dysfunction and LMN signs from peripheral neuropathy)
    • Malignancy with intraspinal lesions or paraneoplastic symptoms
    • Metabolic disorders – severe B12 deficiency, copper deficiency
    • HIV
    • Neuromuscular junction disease – myasthenia gravis, Lambert Eaton


Special neurologic exam maneuvers:

  • Fatigability: ask patient to gaze upward, concerning for fatigability if patient develops ptosis
  • Fasciculations: be sure to check the tongue, thigh, and hand muscles!
  • Single breath count test: ability to count ≥25 in single breath suggests normal respiratory muscle function [can also have patients sing ABCs in single breath]. Correlates with forced vital capacity (FVC).
  • Weak neck flexion also correlates with diaphragmatic dysfunction.  Test by asking patient to lift head off bed and tuck chin.
  • Negative inspiratory force (NIF) – Being able to generate negative inspiratory force of greater than -60 is normal. Less than -60 suggests diaphragmatic weakness.


Evernote: https://www.evernote.com/l/AN2LydI6NItIc7OKT9NQUWAJgHnuUH30VYc/ 

Moffitt Morning Report 4/5/19: Systemic Sclerosis!

Hi All,

Thanks to JD for presenting the case of a young woman with systemic sclerosis. This is a rare and complicated diagnosis, so I’m including some key pearls and a great review article below.

TLC (Tim and Laura, your Chiefs)


What is systemic sclerosis? (aka scleroderma)

An immune-mediated rheumatic disease of the skin, small vasculature and internal organs. The pathophysiology is not well understood, but involves early diffuse microvascular inflammation and damage, and later progressive replacement of the inflammatory changes around the vessels with fibrosis.


What are the different types?

The two major subtypes are 1) Limited cutaneous systemic sclerosis, in which skin findings only occur distal to the knee’s/elbows and on the neck/face, and 2) Diffuse cutaneous systemic sclerosis, in which skin findings also include the proximal limbs or trunk.

These distinctions are important because they have different patterns of organ involvement. For example, the limited subtype commonly involves pAH, while the diffuse subtype has an increased risk of renal crisis and severe lung fibrosis.

CREST syndrome describes a subset of the patients with limited cutaneous systemic sclerosis who have prominent vascular involvement. With that said, the vascular involvement is often asymptomatic early on and patient’s manifestations are often much more heterogeneous than the acronym suggests, so the term has largely fallen out of favor.

There are other subtypes that are more rare, including Sine Scleroderma, in which there are systemic findings (positive serologies, organ manifestations) and Raynaud’s, but no skin findings, and overlap syndromes, in which patients have one of the above subtypes plus another autoimmune disease like SLE, RA, etc.


How do you diagnosis systemic sclerosis?

Not something to memorize, but there are criteria from the ACR (Panel 1 in the attached paper)

Associated serologies are anti-centromere, anti-topoisomerase I, and anti-RNA polymerase III.


What are the organ manifestations, and how do you treat them?

Think about 4 major organs: Esophagus (and other parts of GI tract), heart, lungs and kidneys. See the two figures below from Denton & Khanna re: monitoring for and treating the complications of systemic sclerosis.

scleroderma monitoring

scleroderma tx

Great review article: https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(17)30933-9.pdf

Evernote: https://www.evernote.com/shard/s582/sh/929b9dbf-9564-42c0-bac0-6804c3dfdb44/2f3d07466a47f28dd3b8143e0e9bbc12






Mt. Zion report – HypoK, HTN, and hyperaldo!

Thanks to Cam for presenting the case of a middle-age male with persistent muscle cramps found to have hypokalemia secondary to hyperaldosteronism!

Approach to hypokalemia:


  • Urine electrolytes (K) and osmolality can help differentiate etiologies of hypokalemia
    • Urine K > 40: renal loss
    • Urine K < 20: extra-renal loss
    • Urine K 20-40: calculate transtubular potassium gradient (TTKG):
      • [Urine K/serum K] / [urine osm/serum osm] > 7 indicates renal potassium loss
  • Testing for hyperaldosteronism recommended in the following patients:
    • Hypertension + hypokalemia
    • Resistant hypertension
    • Adrenal incidentaloma and hypertension


How do you test (correctly!) for hyperaldosteronism?

See UptoDate’s great algorithm below – remember the test is most valid in the morning (after patient has been awake for 2 hours) and seated for 15 minutes before sample is drawn.

What about interfering meds?

  • Harder to interpret testing if patients on spironolactone or eplerenone
  • Consider stopping NSAIDs 2 weeks before testing
  • ACEi, ARBs do not necessarily have to be stopped (given risk of poorly controlled hypertension) but caution in excluding diagnosis with elevated plasma renin activities.


If laboratory evidence suggestive of hyperaldosteronism, proceed with adrenal CT to determine unilateral versus bilateral nature.

  • Unilateral lesion –> adrenalectomy
  • Bilateral nodularity OR normal adrenals –> consider adrenal vein sampling (technically difficult procedure, only done at select centers with advanced IR)
    • Bilateral adrenal hyperplasia confirmed? Medical therapy

Get Endocrine involved early for abnormal plasma aldosterone and renin ratios – consider e-consult especially if the aldosterone is “normal” as Cam taught us that this can be inappropriately normal and must be interpreted in the context of renin activity as well!


Evernote: https://www.evernote.com/l/AN3Rbikqvh5J4pfDxitINEaJjOe8FJYRYiQ/


Moffitt Morning Report: HCV Cryoglobulinemia!

Big thanks to Fangdi for presenting a case of a woman who presented with a subacute complaint of bilateral foot pain, also with wrist drop and facial droop on exam, ultimately found to have HCV and AKI with possible cryoglobulinemia!  Pearls below.

TLC (Tim and Laura, your Chiefs)


Mononeuritis Multiplex (aka multiple mononeuropathy)

  • Definition: peripheral neuropathy in two or more noncontiguous nerves
  • Progression: LE nerves often affected first, particularly in vasculitis
  • Harry’s Top 3 Differential: Vasculitis, DM, HIV


Cryoglobulinemia and HCV (adapted from prior blog posts)

What are cryoglobulins? immunoglobulins and complement that precipitate at temperatures less than 37C. There are 3 main types of cryos (don’t memorize, but table below). Type 1 is monoclonal and more common w/ lymphoproliferative disorders and II/III are polyclonal more common w/ chronic infections/rheum conditions (type II also has RF activity and +RF can be seen in these patients).

What is cryoglobulinemia? Technically, this is the presence of cryoglobulins in the serum, but this term is often used to refer to the vasculitic syndrome caused by cryos. This is a small-medium vessel vasculitis. This is an important distinction because cryos are VERY common in patients with chronic infections/inflammatory disorders, but cryo vasculitis is much more rare. Cryos may be present in 15-20% of HIV patients, 15-25% of patients w/ connective tissue disease, and ~50% of patients w/ HCV, but only a fraction of these actually develop symptoms.

How do these patients present? The different types have slightly different clinical presentations (see table), but the cutaneous manifestations are the most common. Peripheral neuropathy is the most common neuro complication. Renal complications include an immune complex GN and MPGN.

How is cryoglobulinemia diagnosed? Diagnosis is confirmed when you have 1. A consistent history, 2. Typical clinical manifestations like purpura and hypocomplimentemia and 3. Presence of cryoglobulins. Since cryoglobulin testing may be negative in as many as 30-40% of cases on presentation, you can also use +RF and low C4 in combination with other specific findings like membranoproliferative GN to diagnose.

How is cryoglobulinemia treated? It depends on the etiology and type (see table). In HCV-related cryoglobulinemia, treatment consists of a combination of immunosuppressive therapy (usually rituximab or steroids) and treatment for HCV.


Zion AM report: Erosive OA??! Immunotherapy side effects?? Are we are Moffitt!?

Thanks to Arielle for presenting an interesting case of an elderly man with metastatic melanoma on immunotherapy presenting with new bilateral hand swelling, with physical exam concerning for symmetric inflammatory arthritis! This was a great case to review the entity called Erosive Osteoarthritis as well as side effects of immunotherapy and checkpoint inhibitors! See below for pearls 🙂

Remember key descriptors that can narrow your illness script for arthritis!

  1. Acute versus chronic
  2. Inflammatory versus non-inflammatory
    • Inflammatory – morning stiffness, warmth, tenderness, swelling
    • Noninflammatory – no/little morning stiffness, no warmth, usually affecting weight bearing joints
  3. Number of joints involved
    • 1 joint = monoarticular (ALMOST ALWAYS need arthrocentesis to rule out infection!) – think septic joint versus crystal induced
    • 1-5 joints = oligoarticular
    • > 5 joints = polyarticular (think viral causes, RA, SLE, psoriatic arthritis)
  4. Symmetric versus asymmetric 

How do I arrange an arthrocentesis in the outpatient setting?

  • Most PCPs feel comfortable with knee joint arthrocentesis; however, smaller joint arthrocentesis or level of comfort with procedure warrants help from our specialists!
  • If urgent/concerned for septic joint –> send patient to ED
  • If suspected gout/pseudogout but joint has never been aspirated –> refer to Rheumatology or Resident Injection Clinic (if knees)
  • If involving small joints of hand/wrist –> refer to Rheumatology or Orthopedics Hand


  • Usually described as chronic non-inflammatory asymmetric oligoarthritis!
  • But what about erosive osteoarthritis??
    • Illness script: UNCOMMON but AGGRESSIVE subset of HAND OA
    • Can be associated with pain, stiffness, soft tissue swelling
    • Usually affects DIPs/PIPs, can be misdiagnosed as psoriatic arthritis or rheumatoid arthritis
    • Can see subchondral erosions on xray

    • Interestingly,  some association with poorly controlled hyperlipidemia!


Extra pearl: Immunotherapy Side Effects

Checkpoint inhibitors have changed the way we treat and prognosticate metastatic malignancy! Now that they are being utilized more frequently, here’s a chance to brush up on some of the side effects to look out for:

  • Opportunistic infections
  • Fatigue (most common side effect)
  • Infusion reactions
  • Dermatologic manifestations (rash, dry mouth, pruritis, vitiligo)
  • Colitis (both infectious and non-infectious causes)
  • Hepatotoxicity
  • Pneumonitis
  • Endocrinopathies (thyroid dysfunction, hypopituitarism, adrenal insufficiency)
  • Musculoskeletal/rheumatologic (inflammatory arthritis, myositis)


Evernote: https://www.evernote.com/l/AN1-5hcGiblINZtwQipA8ZbuH5RxOmMISWU/