Multiple cranial nerve injury in Ramsay Hunt Syndrome: a case report.

Herpes zoster oticus (Ramsay Hunt Syndrome) is characterized by facial nerve paralysis, ear pain and auricular skin rash. It occurs as a result of reactivation oflatent varicella zoster virus infection in the geniculate ganglion of the facial nerve. Major clinical symptoms include 7th nerve paralysis or cranial nerve paralysis and vesicles along the nerve with cocomitant ear pain. Other cranial nerve involvement although uncommon, can be found in some cases. In this study, a 74-year-old female patient had ipsilateral 8th, 9th and 10th cranial nerves injury. Cranial nerve paralysis accompanied with injury has been repor ted in R amsay Hunt Syndrome.

Recurrent facial palsy: The prognostic value of electrophysiological tests according to recurrence interval.

OBJECTIVE: The prognostic value of nerve excitability tests (NET) and electroneurography (ENoG) for recurrent peripheral facial palsy is poorly understood. This study aimed to evaluate the association between NET/ENoG results for the current palsy and recovery. METHODS: We extracted data on patients who were referred to our hospital (2005-2017). Adult patients with recurrent peripheral facial palsy who underwent NET and ENoG within 3 weeks of onset were retrospectively analyzed (n = 26). Favorable recovery was defined as the achievement of either House-Brackmann grade I/II or the same level of facial movement as before the current palsy. We evaluated the predictive NET/ENoG results by making comparison between the favorable recovery group and the unfavorable recovery group, which were subdivided based on the length of time after the previous palsy. RESULTS: In terms of patients with a >4-year recurrent interval, 8 out of 12 patients achieved favorable recovery. Compared to the favorable recovery group, the unfavorable recovery group had significantly higher NET results (9.03 mA vs. -1.08 mA, p = 0.017). Also, the unfavorable recovery group had significantly higher NET results in patients with a >2-year recurrent interval (9.03 mA vs. 1.06 mA, p = 0.036). However, other test results (NET in ≤4-year recurrent interval/all 26 patients, and ENoG in >4-year recurrent interval/≤4-year recurrent interval/all 26 patients) did not differ significantly between patients with favorable and unfavorable recovery. CONCLUSION: NET might be a useful prediction method in patients with at least a few years interval between the previous and the current palsy.

Prognostic value of the blink reflex test in Bell's palsy and Ramsay-Hunt syndrome.

OBJECTIVE: This study was designed to evaluate the prognostic value of the blink reflex (BR) test in patients with Bell's palsy (BP) or Ramsay Hunt syndrome (RHS). METHODS: The House-Brackmann (HB) grade of patients diagnosed with BP and RHS was determined at first visit and 3 months later. Final HB grade III-VI was defined as an incomplete recovery. Factors evaluated as prognostic of poor recovery included electroneurography (ENoG) degeneration rate (DR)>90%, and absence of BR. Rates of complete and incomplete recovery were calculated and the associations between prognostic factors and recovery were determined. RESULTS: Of the 129 included patients, 98 (76%) had BP and 31 (24%) had RHS. Absence of BR and low mean ENoG value were significantly associated with incomplete recovery in both the BP and RHS groups (p<0.05 each). Initial HB grade V-VI was significantly associated with rate of incomplete recovery in patients with RHS (p<0.05 each). Severe residual palsy (final HB grade V-VI) in the absence of BR was significantly more frequent in patients with RHS than with BP (p<0.05). CONCLUSION: BR test results were a good prognostic indicator in patients with BP and RHS, as were ENoG value. Absence of BR was more frequently associated with severe residual palsy in RHS than in BP.

Factors affecting the effect of physical rehabilitation therapy for synkinesis as a sequela to facial nerve palsy.

OBJECTIVE: To investigate factors affecting the effect of physical rehabilitation therapy for synkinesis as a sequela to facial nerve palsy. METHODS: A total of 37 patients with peripheral facial nerve palsy in Teine-Keijinkai Hospital were enrolled in this study. All patients showed synkinesis at 6 months after the onset of facial nerve palsy and were instructed in physical rehabilitation by expert staff from their first visit. The degree of synkinesis was evaluated at 6, 9 and 12 months after the onset of facial nerve palsy based on Sunnybrook facial grading system score and asymmetry in eye opening width. The patients were divided into two groups by age, gender, cause of palsy, electroneurography (ENoG) value, onset of synkinesis, initial treatment and timing of the start of physical rehabilitation. RESULTS: Female patients and younger patients did not show any deterioration in synkinesis. Patients in the lower ENoG group and the later onset of synkinesis group showed significant deterioration in synkinesis after the 6th month from onset of facial palsy. CONCLUSION: Physical rehabilitation was shown to prevent significant deterioration in synkinesis in female and younger patients with facial nerve palsy. Careful follow-up with regard to synkinesis is required in cases in which the facial nerve damage is thought to be severe.

Electroneurography in the acute stage of facial palsy as a predictive factor for the development of facial synkinesis sequela.

OBJECTIVE: We investigated whether the value of ENoG is a predictive factor for the development of facial synkinesis in patients with facial palsy. METHODS: The degree of oral-ocular synkinesis was evaluated quantitatively by an asymmetry of the interpalpebral space width during the mouth movement (% eye opening). Twenty healthy volunteers without a history of facial palsy (12 men and 8 women; 25-65 years old; mean age: 42.3±9.7years) were included in the study to examine the normal range of % eye opening. Fifty-one patients with facial palsy including 38 with Bell palsy and 15 with herpes zoster oticus (28 men and 25 women; 11-86 years old; mean age: 54±19years) were enrolled to examine the relationship between the ENoG value 10-14days after the onset of facial palsy, and the % eye opening 12 months later. Receiver operating characteristic (ROC) curve for the ENoG value was then used to decide the optimum cut-off value as a predictor of the development of oral-ocular synkinesis. RESULTS: We defined a % eye opening inferior to 85% as an index of the development of oral-ocular synkinesis. There was a significant correlation between the values of ENoG 10-14days after the onset of facial palsy and those of % eye opening 12 months later (ρ=0.81, p<0.001). The area under the ROC curve for the ENoG value was the predictor for the development of oral-ocular synkinesis at 0.913 (95%CI: 0.831-0.996, p<0.001). The optimum cut-off value of ENoG 10-14days after the onset of facial palsy was 46.5% to predict the development of oral-ocular synkinesis 12 months after the onset of facial palsy (sensitivity 97.1% and specificity 77.5%). CONCLUSION: The value of ENoG 10-14days after the onset of facial palsy is a predictive factor for the development of facial synkinesis 12 months later. Since facial palsy patients with a ENoG value inferior to 46.5% have a high risk of developing synkinesis, they should receive the facial biofeedback rehabilitation with a mirror as a preventive therapy.

Vestibular Restoration and Adaptation in Vestibular Neuritis and Ramsay Hunt Syndrome With Vertigo.

OBJECTIVE: To evaluate vestibular restoration and the evolution of the compensatory saccades in acute severe inflammatory vestibular nerve paralysis, including vestibular neuritis and Ramsay Hunt syndrome with vertigo. STUDY DESIGN: Prospective. SETTING: Tertiary referral center. PATIENTS: Vestibular neuritis (n = 18) and Ramsay Hunt syndrome patients with vertigo (n = 13) were enrolled. INTERVENTION: After treatment with oral corticosteroids, patients were followed up for 6 months. MAIN OUTCOME MEASURES: Functional recovery of the facial nerve was scored according to the House-Brackman grading system. Caloric and video head impulse tests were performed in every patient at the time of enrolment. Subsequently, successive video head impulse test (vHIT) exploration was performed at the 1, 3, and 6-month follow-up. RESULTS: Eighteen patients with vestibular neuritis and 13 with Ramsay Hunt syndrome and associated vertigo were included. Vestibular function was significantly worse in patients with Ramsay Hunt syndrome than in those with vestibular neuritis. Similar compensatory saccades velocity and latency values were observed in both groups, in both the caloric and initial vHIT tests. Successive vHIT results showed a significantly higher vestibulo-ocular reflex gain recovery in vestibular neuritis patients than in Ramsay Hunt syndrome patients. A significantly faster reduction in the latency, velocity, and organization of the compensatory saccades was observed in neuritis than in Ramsay Hunt syndrome patients. CONCLUSIONS: In addition to the recovery of the vestibulo-ocular reflex, the reduction of latency, velocity and the organization of compensatory saccades play a role in vestibular compensation.

Effects of a sensory branch to the posterior external ear canal: coughing, pain, Ramsay Hunt's syndrome and Hitselberger's sign.

BACKGROUND: A distinct nerve innervating the external auditory canal can often be identified in close relation to the facial nerve when gradually thinning the posterior canal wall. This nerve has been attributed to coughing during cerumen removal, neuralgic pain, Hitselberger's sign and vesicular eruptions described in Ramsay Hunt's syndrome. This study aimed to demonstrate the origin and clinical impact of this nerve. METHODS AND RESULTS: In patients with intractable otalgia or severe coughing whilst inserting a hearing aid, who responded temporarily to local anaesthesia, the symptoms could be resolved by sectioning a sensory branch to the posterior canal. In a temporal bone specimen, it was revealed that this nerve is predominantly a continuation of Arnold's nerve, also receiving fibres from the glossopharyngeal nerve and facial nerve. Histologically, the communicating branch from the facial nerve was confirmed. CONCLUSION: Surgeons should be aware of the posterior auricular sensory branch and its clinical implications.

Characteristics of hearing loss in patients with herpes zoster oticus.

Patients with herpes zoster oticus (HZO) may commonly show symptoms associated with 7th and 8th cranial nerve (CN VII and CN VIII) dysfunction. The aim of this study is to investigate the characteristics of hearing loss in patients with HZO and discuss possible mechanisms.Ninety-five HZO patients who showed at least one of the symptoms of CN VII and CN VIII dysfunction between January 2007 and October 2014 were included in this study. Hearing loss was defined when the mean thresholds of pure tone audiometry (PTA) in speech frequency (0.5 kHz + 1 kHz + 2 kHz/3) or isolated high frequency (4 kHz + 8 kHz/2) were greater than 10 dB in the affected ear compared with the healthy ear, and a total of 72 patients were classified as the hearing loss group.The difference of mean PTA thresholds between affected and healthy ears was significantly greater in the high frequency range than in low range (20.0 ±â€Š11.5 dB vs. 12.9 ±â€Š15.7 dB, P = 0.0026) in patients with hearing loss (n = 72). The difference between affected and healthy ear was significantly greater in patients with vertigo (n = 34) than those without vertigo (n = 38) in both the high (P = 0.033) and low (P = 0.024) frequency ranges. In contrast, the differences between affected and healthy ears were not significantly different between patients with facial palsy (n = 50) and those without facial palsy (n = 22) in both the high (P = 0.921) and low (P = 0.382) frequency ranges.In patients with HZO, hearing loss is more severe in the high frequency range than in the low frequency range. Hearing impairment is more severe in patients with vertigo than in those without vertigo in both the high and low frequency ranges, even though the degree of hearing impairment is not significantly different between patients with and without facial palsy. These findings indicate that the mechanisms of viral spread from CN VII to CN VIII may differ between vestibular and audiologic deficits.

Síndrome de Ramsay Hunt. A propósito de un caso / Ramsay Hunt syndrome. A case report

Presentamos el caso de una paciente con parálisis facial periférica coincidente con lesiones vesiculosas herpéticas óticas. El síndrome de Ramsay Hunt es la segunda causa más frecuente de parálisis facial periférica atraumática, con una presentación clínica muy variada. La parálisis facial de este síndrome ocurre en el 60-90 % de los casos, es periférica y puede preceder o seguir a las lesiones cutáneas con un peor pronóstico que la parálisis idiopática de Bell (AU)

We present the case of a female patient with peripheral facial paralysis coinciding with vesicular herpetic otic lesions. Ramsay Hunt syndrome is the second most common cause of atraumatic peripheral facial paralysis, with a very varied clinical presentation. The facial paralysis in this syndrome occurs in 60-90 % of cases, is peripherical and can precede or follow the cutaneous lesions with a worse prognosis than idiopathic Bell paralysis (AU)

Clinical manifestations in patients with herpes zoster oticus.

Patients with herpes zoster oticus (HZO) may exhibit diverse symptoms regarding cochleovestibular dysfunction. This study investigated the clinical manifestations of HZO by comparing symptoms associated with dysfunctions of the 7th and 8th cranial nerves (CN VII and VIII, respectively). This study is a retrospective case series. Eighty-one patients with HZO who had dysfunction of CN VII or VIII were included in this study. Electroneuronography (ENoG) values were compared among patient groups with facial weakness. Patients with ipsilateral facial weakness (62 of 81) were more common than those without. Among 81 patients, those with facial weakness, hearing loss, and vertigo were most common, and only 1 patient had vertigo without hearing loss or facial weakness. Most patients with vertigo also had hearing loss (28 of 30), and patients without hearing loss did not have vertigo (19 of 21). While patients with vertigo had worse ENoG values than those without vertigo, ENoG values were not significantly different between patients with and without hearing loss. In conclusion, various clinical manifestations of CN VII and VIII dysfunction are possible in patients with HZO. Patients with vertigo had worse ENoG values than those without, which may indicate that vertigo reflects more severe facial nerve degeneration in HZO patients with facial weakness.

Quantitative analysis of facial palsy using a three-dimensional facial motion measurement system.

OBJECTIVE: The prognosis for facial nerve palsy (FNP) depends on its severity. Currently, many clinicians use the Yanagihara, House-Brackmann, and/or Sunnybrook grading systems to assess FNP. Although these assessments are performed by experts, inter- and intra-observer disagreements have been demonstrated. The quantitative and objective analyses of the degree of FNP would be preferred to monitor functional changes and to plan and evaluate therapeutic interventions in patients with FNP. Numerous two-dimensional (2-D) assessments have been proposed, however, the limitations of 2-D assessment have been reported. The purpose of this study was to introduce a three-dimensional (3-D) image generation system for the analysis of facial nerve palsy (FNP) and to show the correlation between the severity of FNP assessed by this method and two conventional systems. METHODS: Five independent facial motions, resting, eyebrow raise, gentle eye closure, full smile with lips open and whistling were recorded with our system and the images were then analyzed using our software. The regional and gross facial symmetries were analyzed. The predicted scores were calculated and compared to the Yanagihara and H-B grading scores. We analyzed 15 normal volunteers and 42 patients with FNP. RESULTS: The results showed that 3-D analysis could measure mouth movement in the anteroposterior direction, whereas two-dimensional analysis could not. The system results showed good correlation with the clinical results from the Yanagihara (r(2)=0.86) and House-Brackmann (r(2)=0.81) grading scales. CONCLUSION: This objective method can produce consistent results that align with two conventional systems. Therefore, this method is ideally suited for use in a routine clinical setting.

Comparison of facial synkinesis at 6 and 12 months after the onset of peripheral facial nerve palsy.

OBJECTIVE: To investigate the time course of synkinesis as a sequela of facial nerve palsy so that we are able to determine an appropriate time for deciding the outcome of recovery in patients with facial nerve palsy. METHODS: Nineteen consecutive patients with peripheral facial nerve palsy who developed synkinesis were enrolled. We compared the degree of synkinesis at 6 and 12 months after the onset of palsy. Our investigation consisted of (1) scoring using the Sunnybrook facial grading system and (2) computing the asymmetry (%) in eye opening width. We also judged whether all 19 cases were cured or not based on the treatment outcome criteria of the Japan Society of Facial Nerve Research at 6 and 12 months. RESULTS: The synkinesis score based on Sunnybrook facial grading system and the degree of asymmetry in eye opening width during mouth movement deteriorated significantly between the 6th and 12th month after the onset of palsy. One of the cases regarded as "cured" at the 6th month was later judged to be "non-cured" due to deterioration in the synkinesis score at the 12th month. CONCLUSION: From our results, synkinesis deteriorated after the 6th month from the onset of palsy. Therefore, we should follow up the degree of synkinesis until at least the 12th month, and the outcome of recovery in patients with facial synkinesis should be evaluated at least 12 months after the onset.

Test battery of cranial nerves VII and VIII for assessing herpes zoster oticus.

OBJECTIVE: This study adopted a test battery of cranial nerves (CNs) VII and VIII comprising a facial nerve function test, audiometry, a caloric test, and ocular and cervical vestibular-evoked myogenic potential (oVEMP and cVEMP, respectively) tests to assess the function of CNs VII and VIII comprehensively so as to predict facial nerve recovery in patients with herpes zoster oticus (HZO). STUDY DESIGN: Case series with chart review. SETTING: University hospital. METHODS: A total of 20 patients with HZO underwent a test battery of CNs VII and VIII. Registering the unaffected nerve bundles in the internal auditory canal was based on the number of normal results in the test battery of CNs VII and VIII. Fair facial nerve recovery is defined as the improvement of facial paresis to facial nerve grades I to II/VI. RESULTS: In 20 patients with HZO, 6, 7, 3, and 4 patients had 0, 1, 2, and 3 unaffected nerve bundles, respectively. A significantly positive correlation was identified between the number of unaffected nerve bundles and fair facial nerve recovery. Similarly, a statistically significant predictor of fair facial nerve recovery was noted for unaffected nerve bundles (odds ratio, 15.42) but not for grading of the facial nerve (odds ratio, 0.49). CONCLUSION: Grading of the facial nerve alone fails to predict the outcome of facial paresis in patients with HZO mainly because it overlooks the involvement of CN VIII. Alternatively, a combined test battery of CNs VII and VIII may serve as a strong predictor for facial nerve recovery.

[Herpes zoster-induced neuralgia (neuropathy)].

Neuralgia (neuropathy) is the most common manifestation of herpes zoster (HZ). In spinal and cranial neuralgia, there are 3 types of pain: 1) spontaneous, persistent, burning pain; 2) intermittent sharp pain; 3) pain occurring with nonpainful stimulation. The skin exhibits areas of hypesthesia, anesthesia, and dysesthesia. Ophthalmic neuralgia (of the first branch of the trigeminal nerve) is encountered in 20% of HZ cases. HZ of the auricle and external auditory meatus concurrent with facial and vestibulocochlear neuropathy is diagnosed as Ramsay Hunt syndrome. Postherpetic neuralgia (neuropathy) is characterized by pain present for 3 months or more after the appearance of herpetic eruptions. Combined therapy involving the earlier use of antiviral agents, tricyclic antidepressants, analgesics, and neuromidine is the most effective option for HZ-induced neuralgia (neuropathy).

Prognostic factors of synkinesis after Bell's palsy and Ramsay Hunt syndrome.

OBJECTIVE: This study evaluated the prognostic factors of synkinesis following Bell's palsy and Ramsay Hunt syndrome. METHODS: A total of 345 patients consisting of 309 cases of Bell's palsy and 36 cases of Ramsay Hunt syndrome were enrolled in our study. The following 13 factors were considered as candidate prognostic factors for the presence of synkinesis at 6 months from onset: age, sex, diagnosis, diabetes mellitus, initial onset or recurrence, electroneurography (ENoG), number of days from onset to first visit to our hospital, the lowest Yanagihara grading system score, the change in Yanagihara score after 1 month, otalgia, hearing loss, vertigo and taste disturbances. These factors were analyzed by logistic regression. RESULTS: Logistic regression analysis clarified the lowest Yanagihara score, the change in Yanagihara score after 1 month, and the ENoG value for a prognosis of synkinesis. The most predictive prognostic factor was the lowest Yanagihara score, and the adjusted odds ratio in the multivariate model was 11.415. As for other prognostic factors, the adjusted odds ratios ranged from 7.017 (ENoG value) to 8.310 (the change in Yanagihara score after 1 month). These findings were therefore considered as high risk factors for synkinesis. CONCLUSION: It is possible to predict synkinesis following Bell's palsy and Ramsay Hunt syndrome on the basis of clinical symptoms. The lowest Yanagihara score, and the change in Yanagihara score after 1 month, together with the ENoG value at the onset, were found to be especially important factors for predicting synkinesis following Bell's palsy and Ramsay Hunt syndrome.