Shingles in and around the ear and face is awful.  This post series has belabored the pain and agony problem enough.  The other big problem, the subject of this final post on the topic, is that it’s often very hard to diagnose and differentiate from other disease processes, especially in older patients.  Here’s where the last post left off:

… do complete audiometric workups on patients who present initially, or return, with complaints of sudden-onset unilateral ear pain, hearing loss, dizziness, facial paresis or rash.  As soon as testing is completed, send the patient–audiogram in hand–for ASAP workup by their physician or an ENT, with the goal of arriving at a diagnosis and treatment as quickly as possible.  In many cases, early treatment can mitigate symptoms and reduce long-term effects. What diagnoses are made or ruled out when patients are worked up medically?  We’ll look at some case histories and competing diagnoses when we next write about shingles in this series.

 

What to Look For and What to Make of It

 

Misdiagnosis of shingles hinders treatment and predictive outcomes.  Audiologists do not make the diagnoses or prescribe the treatments but they should know what to look for and how/when to refer when working with patients who present with a variety of symptoms or complaints. They should also know the complications of shingles, which can affect hearing and auditory function.

Table 1 lists symptoms manifest in shingles which are easily confused with other problems and diseases.

Shingles Presenting Symptom

Differential Diagnosis

Pain/swelling in ear canal External otitis
Unilateral facial weakness Bell’s palsy
Vertigo

Viral labyrinthitis

Stroke,  posterior inferior cerebellar artery (PICA) region

Paroxysmal pain precipitated by cold wind, face washing, etc. Trigeminal neuralgia
Facial pain and/or ear pain

TMJ

Dental abscess

Nasopharyngeal carcinoma

Otitis media

 

Shingles Complications

 

We’re not going to list everything — you can imagine where varicella can take you… and leave you, but here are the two ends of the spectrum:

  • The most common complication is post-herpetic neuralgia — pain and itching remain after shingles rash/blisters resolve.  There is no cure treatment for this condition.
  • A very rare complication is encephalitis. Thankfully, this life-threatening inflammation around the brain takes a mild form in the case of herpes zoster and reportedly resolves quickly, except in patients with impaired immune systems, when it can be deadly.

 

A Shingles Case History

 

Finally, for those audiologists or others who have some degree of comfort looking at brain scans, click on the link for an interesting one showing a case history of Ramsay Hunt at work in the inner ear, cranial nerves, with neural degeneration extending into the brainstem.  This was a 58 year old man who presented with extreme ear canal pain with vesicles, complete unilateral facial palsy,severe hearing loss, and vertigo for 28 hours.  Ramsay-Hunt was confirmed by blood serum and contrast MRI was performed on the 3rd day post-onset.

He was treated with IVAcyclovir and corticosteroids, together with pain medication.  At hospital discharge 8 days later, he had “partial recovery” of hearing loss and facial paralysis.  Considering what he’d been through, it is perhaps not surprising that he refused to return for repeat MRI or other tests, so the extent of permanent hearing loss or neuronal degeneration in the auditory system is unknown.

 

Treatment

 

(Note:  Information in this section is from a single source:   patient.co.uk)

Antiviral drugs approved for shingles include:  acyclovir (Zovirax), famciclovir (Famvir), and valacyclovir (Valtrex).  These drugs are supposed to be prescribed no later than the first week after shingles onset, which is a challenge if the condition is misdiagnosed.  

 If treatment goes into effect in the first three days, about 75% of people achieve full recovery from shingles, although the effects of treatment for Ramsay-Hunt variety are not as clear cut.  Hearing loss reportedly resolves but facial paresis may persist in those who do not fully recover.  In general, aging, diabetes, and high blood pressure are additional risks for full recovery.

In addition to anti-viral treatment, prednisone in combination with acyclovir has been shown to reduce facial paralysis but prednisone has no effect on recovery of hearing loss.

Diazepam is sometimes used for vertigo.

 

Vaccination

 

Zostovax is available for those 50 and over who have already had chicken pox.  The vaccine is a weakened form of the varicella-zoster virus.  It reduces the risk of shingles by about 50%, may reduce the likelihood of recurrence if you’ve already had it, and shortens the period of post-herpetic neuralgia.  It’s not for everyone — people with histories of HIV, leukemia, cancer treatment shouldn’t take it; pregnant women shouldn’t.  These and other contra-indications are checked before you can get the vaccination.

A small group of people never develop immunity to the varicella virus, either because they didn’t get chicken pox or they didn’t get the chicken pox vaccine. Tthose people are not at risk for shingles, but they are at risk for severe infections if they are eventually exposed to the virus.  In people with compromised automimmune systems, such exposure can prove fatal.  A new drug has just been approved to provide some relief to those people if they develop chicken pox.  Looked at from this perspective, perhaps shingles is not so bad after all…..  just ask Barbara Walters, a victim of chicken pox at 83 which was delayed in diagnosis after she suffered a fall and developed persistent fever.

Enough about shingles!  We’re on to other, less painful topics in future posts. Thanks for reading this series and we hope it has been helpful to our readers.

This is the next chapter in a multi-part Shingles series originally prompted by an obscure link to Red Ear Syndrome.  That link lead to posts on shingles in general, shingles in the ear, and the specifically-named Ramsay-Hunt Syndrome Type 2 (RHS/2) .

RHS/2 is a horribly painful condition in which the herpes zoster in the geniculate ganglion reactivates and manifests in any number of ways, including deafness, vertigo, pain, and facial weakness/paralysis. RHS/2 is difficult to diagnose and distinguish from other problems. For example, it is almost always characterized by a severe rash but it can also occur with facial paralysis without a rash, similar to Bell’s palsy and that’s what today’s post is about.  

 

What’s an Erythematous Vesicular Rash? What Does it Mean?

 

This rash is like Supreme Court Justice Potter Stewart’s famous definition of obscenity–you know it when you see it.  In this case, you definitely know it when you feel it because of the extreme pain.  Erythematous vesicular rash affects skin as well as mucous membranes, such as the lining of the mouth. It is characterized by

large, symmetrical red blotches [which] appear all over the skin in a circular pattern. On mucous membranes, it begins as blisters and progresses to ulcers.”

The problem with this rash is that it can be caused by a variety of things (e.g., medications) that have nothing to do with Ramsay Hunt or infection of any kind. For example, it can be misdiagnosed as a kissing bug bite (which raises the spectre of Chagas disease), based on the presence of vesicular rash by the lip.  Even more of a problem is that RHS/2 can occur without any rash anywhere, a situation referred to as “sine herpete” in the medical literature.

The presence or absence of  facial erythematous vesicular rash is not diagnostic for RHS/2, though its presence surely should gain the attention of the physician and its absence in the presence of facial paralysis should not rule out RHS/2.

 

All in the (Herpes) Family

 

We’ve tried to make this series clear and simple, but the anatomy and physiology have defeated our efforts.  Just talking about the geniculate ganglion takes us places we never went in hearing and speech anatomy classes.  The varicella zoster virus (VZV) and its cousin herpes simplex virus (HSV) are better versed in the anatomical pathways than we are. Once unleashed, they seem to ride those pathways like highways, taking little-known, interconnected neural shortcuts as they please.  Pulling it all together, as best we can, here’s a brief summary of what the zosters can get up to:

  • HZ oticus Just the ear.  Caused by VZV in the geniculate ganglion.  Herpes zoster oticus affects any/all parts of the peripheral ear:  external (pinna, ear canal, eardrum), middle, and inner (cochlea).  It causes severe ear pain associated with vesicular rash.  Hearing loss, if present, varies in severity and permanence.
  • RHS/2.  The ear and the face.  Caused by VZV in the geniulate ganglion. When HZ oticus is accompanied by facial paralysis, it becomes  Ramsay Hunt syndrome.   Just to confuse things, we noted in our last post on this subject that strictly speaking, you can have RHS/2 without any signs in the ear, in which case it’s RHS/2 without herpes zoster oticus.  Confusing.  But most of the time the ear’s involved so herpes zoster oticus is frequently referred to simply as Ramsay-Hunt.
  • RHS/2 sine herpete.  The ear and the face, but no rash.  This is confusing.  There is no rash (sine herpete) but there is facial paralysis and other symptoms, some involving the ear.  Caused by VZV in the geniculate ganglion.
  • Bell’s palsy (also called Bell palsy).  Facial paralysis/weakness, no rash.  Unlike HZ oticus and Ramsay-Hunt, Bell’s palsy is thought to be caused by herpes simplex virus (HSV) type I infection in the geniculate ganglion, NOT by VZV.  Bell’s palsy is staged according to degree of facial weakness from Stage I (normal facial function) to Stage VI (total single-sided facial paralysis).Facial paralysis in RHS/2 may be more severe and lasting than that of Bell’s palsy, though that probably depends on the Stage of Bell’s palsy.  You can imagine, though, that RHS/2 sine herpete and Bell’s palsy are easily confused — both show facial paralysis/weakness, neither shows a rash. And just to confuse things even more, Bell’s palsy can paralyze the muscles of the middle ear, effecting the same result as some forms of HZ oticus.  Blood tests can distinguish which virus (VZV or HSV) is present.

The bottom line for audiologists is that a complete audiometric workup is necessary for patients who present initially or upon return, with complaints of sudden-onset unilateral ear pain, hearing loss, dizziness, facial paresis or rash.  As soon as testing is completed,  send the patient–audiogram in hand–for ASAP workup by their physician or an ENT, with the goal of arriving at a diagnosis and treatment as quickly as possible.  In many cases, early treatment can mitigate symptoms and reduce long-term effects.

What diagnoses are made or ruled out when patients are worked up medically?  We’ll look at some case histories and competing diagnoses when we next write about Shingles in this series.

feature image from Mayo Clinic