As I explained to Cool, using the interbet to find legit, peer-reviewed journal articles for a school paper–is like trying to write a 10 page research paper using only the outside cover of all the books in our personal library. You look for certain, specific information, but can only see the back general description of the book’s contents. That’s what I’m doing now. But it’s still better then putting on clothes and trying my luck at a physical library. I’m not sure if my school’s library is open, or what the hours of operation might be. And I’m fairly certain the city library doesn’t have anything as specific as I require. So here’s my list (in progress) of the facts I need and their journal sources.
Introduction: One broad paragraph summarizing your topic (no more than 1/3 of the page) the style is similar to a journal abstract.
-J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt’s analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome (4).
-Ramsay Hunt syndrome (RHS) type 2 also known as herpes zoster oticus (20).
-Herpes zoster oticus (HZO) is a viral infection of the ear and when associated with acute facial paralysis is known as Ramsay Hunt syndrome (?).
-The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash (14)
Population: Discuss the population affected by the pathology such as: men, women, race
-children are not usually affected (9)
-The overall annual incidence of zoster was 4.97 cases per 1000 people, with women having a significantly higher incidence than men (5.20 per 1000 vs. 4.72 per 1000, p < 0.001). The incidence increased stepwise with age, with 5.18 cases per 1000 in people 40–50 years old, 8.36 in those 50–60, 11.09 in those 60–70, and 11.77 in those above 70 years old. The estimated lifetime risk of developing herpes zoster was 32.2%. Zoster-related hospitalizations and medical cost per patient increased with age. In conclusion, about two-thirds of Taiwan’s zoster cases occur in adults older than 40 years old and about one-third of the population would develop zoster within their lifetime (2)
-VZV is found in a worldwide geographic distribution but is more prevalent in temperate climates (7).
-Herpes zoster has been described in all age groups, and lifetime risk is estimated to be 10%-20%. The incidence of herpes zoster is about 150-300 cases per 100 000, with the incidence dramatically increased in patients older than 60 years [Ragozzino MW, Melton LJ III, Kurland LT, et al. Population-based study of herpes zoster and its sequelae. Medicine (Baltimore) 1982;61:310-6.].6 Ramsay Hunt syndrome is much less common, approximately 5 cases per 100 000 population; nevertheless, it is the second most common cause of atraumatic facial paralysis [Adour KK. Otological complications of herpes zoster. Ann Neurol 1994;35(suppl):S62-4.].7 The incidence of herpes zoster in patients with peripheral facial palsy is 4.5%-8.9%. Compared with Bell palsy, Ramsay Hunt syndrome generally has more severe paralysis at onset, and patients are less likely to recover completely (18).
-occurred more often in patients older than 50 years. Of the patients with partial facial nerve function at disease onset, 66% recovered completely, whereas only 10% of those who presented with complete loss of function recovered complete (18)
Time of onset: Discuss whether the pathology is congenital or acquired or delayed in onset
-In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell’s palsy (4).
-His mother had been infected with chickenpox during the second trimester of pregnancy (9).
-Compared with Bell’s palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely (14).
Etiology: Discuss whether the etiology is genetic or environmental or idiopathic
-Varicella-zoster virus (VZV) is a ubiquitous human alphaherpesvirus that causes varicella (chicken pox) and herpes zoster (shingles). Varicella is a common childhood illness, characterized by fever, viremia, and scattered vesicular lesions of the skin. As is characteristic of the alphaherpesviruses, VZV establishes latency in cells of the dorsal root ganglia. Herpes zoster, caused by VZV reactivation, is a localized, painful, vesicular rash involving one or adjacent dermatomes. The incidence of herpes zoster increases with age or immunosuppression. The VZV virion consists of a nucleocapsid surrounding a core that contains the linear, double-stranded DNA genome; a protein tegument separates the capsid from the lipid envelope, which incorporates the major viral glycoproteins (7).
-Varicella, usually a benign manifestation of primary infection, and zoster, a result of reactivation of latent virus, can cause considerable morbidity in patients with immune impairment (6).
-A new case of Ramsay Hunt syndrome will occur every 52 minutes, compared to every 10 minutes for a new case of Bell’s palsy (8).
-Varicella zoster virus (VZV) is an exclusively human neurotropic alphaherpesvirus. Primary infection causes varicella (chickenpox), after which virus becomes latent in cranial nerve ganglia, dorsal root ganglia, and autonomic ganglia along the entire neuraxis. Years later, in association with a decline in cell-mediated immunity in elderly and immunocompromised individuals, VZV reactivates and causes a wide range of neurologic disease (12).
-He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt’s analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome (14).
-Varicella zoster virus (VZV) is an exclusively human neurotropic alphaherpesvirus. Primary infection causes varicella (chickenpox), after which virus becomes latent in cranial nerve ganglia, dorsal root ganglia, and autonomic ganglia along the entire neuraxis. Years later, in association with a decline in cell-mediated immunity in elderly and immunocompromised individuals, VZV reactivates and causes a wide range of neurologic disease (15).
Symptoms: Describe the symptoms of the pathology
-Otological complications of varicella-zoster virus (Ramsay Hunt syndrome) include facial paralysis, tinnitus, hearing loss, hyperacusis (dysacousis), vertigo, dysgeusia, and decreased tearing. Cranial nerves V, IX, and X are often affected. Gadolinium-enhanced magnetic resonance imaging demonstrates enhancement of the geniculate ganglion and facial nerve. These manifestations are identical to Bell’s palsy but are more severe and carry a graver prognosis (8).
-frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus (14)
-Ramsay Hunt syndrome is a disorder characterized by herpetic eruptions on the auricle, facial paralysis, and vestibulocochlear dysfunction, and is attributed to varicella zoster virus infection in the geniculate ganglion. Although it is a common cause of acute peripheral facial paralysis (9)
-Ramsay Hunt syndrome is defined as herpes zoster oticus associated with an acute peripheral facial nerve paresis and quite often with other cranial nerve lesions. The combination of motor, sensory and autonomic involvement leads to a variety of neurological damage patterns, i. e. facial muscle paresis, hearing and balance disorders, sensory problems and disturbances of taste as well as lacrimal and nasal secretion. Additional variability of the clinical picture of Ramsay Hunt syndrome is produced by varying patterns of skin involvement explained by individual anastomoses between cranial and cervical nerves (10).
-severe burning pain referred to the ear and adjacant skin areas characteristically preceeds facial palsy that differentiates it from Bel’s Palsy (19).
-HZO patients with vertigo had facial palsy on the lesioned side and spontaneous nystagmus beating toward the healthy side (11) he nerve trunks within the internal auditory canal are widely affected in HZO patients with vertigo. Both superior division and inferior division of the vestibular nerve attribute to the vertiginous attack (11).
-14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell’s palsy (14).
-Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with “Bell’s palsy” have Ramsay Hunt syndrome zoster sine herpete (14).
-Three major symptoms, auricular vesicles, facial paralysis and vestibulo-cochlear dysfunction, were found in 57.6% of the patients although these symptoms did not always appear simultaneously. Auricular vesicles appeared before (19.3%), during (46.5%), or after (34.2%) the onset of facial paralysis (16).
-causing otalgia, auricular vesicles and peripheral facial paralysis. Vesicles occurring anywhere along the sensory distribution of the facial nerve, including the anterior two-thirds of the tongue, the pinna or the external auditory canal. Otalgia. [Takasu T, Furuta Y, Sato-Matsumura KC, et al. Detection of varicella-zoster virus DNA in human geniculate ganglia by polymerase chain reaction. J Infect Dis 1992;166:1157-9.] (18)
-3-day history of gradual increasing right-sided head pain overlying her right ear, exacerbated by traction on the pinna. head pain was different from her past migraines, especially in that it radiated to the right mastoid. (18)
-5 days post: including right-sided pulsatile tinnitus, right-sided aural fullness, vertigo, reduced ability to close the right eye and decreased taste sensation. She had no hearing loss. mild right-sided facial weakness with sluggish right eye closure (18).
-syndrome characterized by a painful, unilateral vesicular eruption in a restricted dermatomal distribution.4 Dermatomal pain may precede lesions by 48-72 hours and total disease duration is 7-10 days. Immunocompromised and elderly patients may have a more prolonged and severe course.5 Ramsay Hunt syndrome can be precipitated by reactivation of VZV in the geniculate ganglion, resulting in peripheral facial paralysis, otalgia and auricular vesicles (18).
- In approximately 10% of cases, there is no vesicular rash with the facial paralysis, but there is either a 4-fold rise in antibody to VZV or the detection of VZV DNA in skin, blood mononuclear cells or middle ear fluid. This condition is known as Ramsay Hunt syndrome sine herpete (18)
-Facial palsy is not associated with otitis externa.14 Trigeminal neuralgia is manifest by sudden, usually unilateral, severe, brief, stabbing, recurrent episodes of pain in the distribution of one or more branches of the trigeminal nerve. Most importantly, trigeminal neuralgia does not cause neurologic deficit, and the pain cannot be attributed to another disorder (18).15
Hearing Loss: Describe the resultant type (nature) and degree of hearing loss
-variable hearing loss if present from mild to profound, with prognosis worse in more severe (19; 21).
-Diagnosis is based on the sudden onset of unilateral peripheral facial paralysis, usually over hours, but sometimes more gradually. Additional symptoms such as decreased tearing, hyperacusis, loss of taste sensation over the anterior two-thirds of the tongue and ear pain are variable. Bell palsy does not involve the presence of vesicles in the external meatus [Adour KK, Byl FM, Hilsinger RL Jr, et al. The true nature of Bell's palsy: analysis of 1000 consecutive patients. Laryngoscope 1978;88:787-801.] (18)
-Otoscopy revealed herpetic eruptions in the right ear canal. Otoacoustic emissions were absent in the right ear and auditory brainstem responses confirmed moderate sensorineural hearing loss (9).
-swollen right external auditory canal with a normal tympanic membrane (18).
-right auricular swelling and redness with 3 small vesicles on the concha. The right tympanic membrane was not visualized owing to meatal swelling, but the left tympanic membrane was normal (18).
-The neurotropic herpes virus family may have a special relationship to ISHL, in addition to the Ramsay-Hunt syndrome; Nakajima et al (1976) have detected the herpes virus in the cerebrospinal fluid (CSF) of two of three patients studied with sudden hearing loss. Elevations of antibody titers to the herpes virus usually occur in association with two or more other viral titer elevations (Wilson, 1986), suggesting a possible reactivation of this virus (13).
-Hearing loss was observed subjectively in only 20% but objectively in 48.2% of the patients. Hearing loss appeared before (34.3%), during (34.3%), or after (31.3%) the onset of facial paralysis (16).
-Complete recovery of hearing was also achieved in 45.4% of the patients, and the recovery was better in patients having light hearing loss, less than 35dB (16).
-on the basis of BAEP findings, to suggest that in Ramsay Hunt syndrome both cochlear and retrocochlear involvement may occur (17).
-sudden, unilateral HL, usually sloping (21).
-Otitis externa, inflammation of the external auditory canal or auricle, commonly presents with otalgia, pruritus, discharge and hearing loss. Examination usually reveals pain with tragal pressure and a red edematous ear canal [Agius AM, Pickles JM, Burch KL. A prospective study of otitis externa. Clin Otolaryngol 1992;17:150-4](18).
Treatment options: Discuss if the condition is permanent or treatable, if treatable, discuss treatment options such as surgical or medical. (No need to describe surgical procedure itself.)
- Prognostic indicators of poor hearing recovery include advanced age, retrocochlear hearing loss, male gender, vertigo, and speech frequency hearing loss (1).
-Antiviral agents are the standard first-line treatment for herpes zoster infections at other body sites and are thought to reduce or minimise nerve damage, thereby improving outcomes. It has been suggested that these agents improve the chance of facial weakness improving or resolving completely in patients with Ramsay Hunt syndrome (3).
- In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell’s palsy with a 7–10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3–5 days) (4).
-Although the antiviral agent acyclovir is currently used for the treatment of Ramsay Hunt syndrome, its effects on facial nerve and hearing recovery remain controversial. We retrospectively analyzed the effects of acyclovir-prednisone treatment in 80 Ramsay Hunt patients. Of 28 patients for whom treatment was begun within 3 days of the onset of facial paralysis, the recovery from paralysis was complete in 21 (75%). By comparison, of 23 patients for whom treatment was begun more than 7 days after onset, recovery from facial paralysis was complete in only 7 (30%). A significant difference in facial nerve recovery was found between these groups. Early administration of acyclovir-prednisone was proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery also tended to be better in patients with early treatment. There was no significant difference in facial nerve outcome between intravenous and oral acyclovir treatment (5).
-An experimental live vaccine also prevents varicella, but problems regarding its virulence for immunosuppressed patients and the durability of the protective response are still being addressed (6).
-We retrospectively analyzed the effects of acyclovir-prednisone treatment in 80 Ramsay Hunt patients. Of 28 patients for whom treatment was begun within 3 days of the onset of facial paralysis, the recovery from paralysis was complete in 21 (75%). By comparison, of 23 patients for whom treatment was begun more than 7 days after onset, recovery from facial paralysis was complete in only 7 (30%). A significant difference in facial nerve recovery was found between these groups. Early administration of acyclovir-prednisone was proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery also tended to be better in patients with early treatment. There was no significant difference in facial nerve outcome between intravenous and oral acyclovir treatment (5)
-Appropriate treatment resulted in slight improvement after the first week and complete recovery within 4 months (9)
-he patients younger than 16 years old showed better recovery from both facial paralysis and hearing loss than the patients older than 60 years (16).
-another retrospective study of 26 patients treated with acyclovir and steroids, age greater than 60 years, diabetes mellitus, essential hypertension and associated vertigo were identified as prognostic factors for worse outcome and decreased chance of recovery (18)
-recovery of facial, cochlear, and vestibular funct is unpredictable (19).
4; 14) http://jnnp.bmj.com/content/71/2/149.short
12; 15) http://www.sciencedirect.com/science/article/pii/S0733861908000406
20) Hunt JR (1907). “On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications”. J Nerv Ment Dis 34 (2): 73–96. doi:10.1097/00005053-190702000-00001
-Don’t copy this or steal my sources, mmkay? This seriously sucked to compile and I was going to publish it on Monday (after it’s due) but my cut & paste is doing that awful white highlight crap that makes me crazy, so I have to get it on the blog in order to put it on my word document. Karma will come back to you if you cheat.