Delayed facial palsy after cochlear implantation caused by reactivation of Herpesvirus: A case report and review of the literature

Cochlear implantation has many complications; however, delayed facial palsy is relatively rare. In this report, we present the case of a 60-year-old woman with delayed facial palsy after cochlear implantation. Eleven days after the operation, the patient complained of right facial palsy (Yanagihara score 6, House–Brackmann [HB] grade VI). Herpes virus reactivation and local bacterial infection were considered as possible causes of the facial paralysis. A blood test revealed an increase in viral antibody titer, and the patient was diagnosed as having herpes virus reactivation. She was treated with antiviral therapy for the facial palsy until resolution. Slight mouth weakness continued even at 1.5 months (Yanagihara score 34, HB II) but eventually resolved at 3 months (Yanagihara score 40, HB I). Delayed facial palsy is a rare but serious complication that can occur with viral reactivation regardless of the competence of the surgical procedure. Therefore, it is important to inform patients preoperatively regarding the risk of not only immediate but also delayed postoperative facial paralysis.     

Herpes virus reactivation and serum tumor necrosis factor-α levels in patients with acute peripheral facial palsy

Recent studies have shown that tumor necrosis factor-α (TNF-α) plays an important regulatory role in several inflammatory and infectious diseases. In the present study, we evaluated serum TNF-α levels of patients with acute peripheral facial palsy using an ELISA method. We examined sera from each group (n=25 per group) of patients with herpes simplex virus type 1 reactivation (HSV-1), varicella-zoster virus (VZV) reactivation, and with no HSV or VZV reactivation. We also tested the sera of 25 normal controls. No significant difference was found between the serum TNF-α levels in facial palsy and controls. No correlation was found between serum TNF-α levels in cases with HSV-1 or VZV reactivation and with no HSV-1 or VZV reactivation. These results indicate that serum TNF-α levels are not affected by HSV-1 or VZV reactivation in patients with facial palsy.     

Cochlear implant and delayed facial palsy

Abstract

Delayed facial nerve palsy following cochlear implant surgery is less documented though it poses diagnostic and therapeutic challenges. Apart from the functional, aesthetic and emotional concerns, it can raise important medico legal issues. The objectives of this study were: to report a case of delayed facial palsy following cochlear implant surgery in a patient who had positive viral antibody markers pre operatively; and to review the literature on delayed onset facial paralysis following viral reactivation and its relation to cochlear implant surgery. An extensive literature review was done using internet and medical search engines and library facilities. Important articles on the topic were identified and summarised. Data on delayed facial palsy following cochlear implant surgery were collected, constructed in a coherent way and details discussed. Postulated mechanisms of delayed facial palsy include neural oedema, vasospasm and viral reactivation. Of these, reactivation of previous herpes simplex virus infection has special significance, as many of these patients are positive for viral antibody markers. Manipulation of sensory branches of the facial nerve and chorda tympani can be a mechanism in such cases. Correlation of clinical presentation and pre operative positive viral antibody markers with positive polymerase chain reaction can be strongly suggestive of viral reactivation. It is concluded that patients with positive viral antibody markers are more susceptible to facial palsy from viral reactivation. Corticosteroids, antiviral agents and physiotherapy can be useful in producing a quicker and complete recovery. An experienced cochlear implant surgery team and pre operative radiological evaluations are mandatory to decrease the chances of direct facial nerve trauma. Proper irrigation lowers the risk of neural oedema. Copyright © 2009 John Wiley & Sons, Ltd.     

Herpes simplex virus type 1 reactivation and antiviral therapy in patients with acute peripheral facial palsy

Objective: Recent studies provide compelling data for the hypothesis that herpes simplex virus type 1 (HSV-1) is implicated in the pathogenesis of idiopathic peripheral facial palsy (Bell's palsy). The present study analyzed the severity of facial palsy in patients with HSV-1 reactivation and sought to determine the efficacy of acyclovir–prednisone therapy for these patients. Materials and methods: In total, 176 patients, clinically diagnosed with Bell's palsy, were divided into three groups by polymerase chain reaction (PCR) and serological tests — 31 patients with HSV-1 reactivation, 45 patients with VZV reactivation (zoster sine herpete) and 100 patients without HSV-1 or VZV reactivation (Bell's palsy). Results: The difference in the worst grade of facial palsy between patients with zoster sine herpete and Bell's palsy was significant (P=0.01 10, Mann–Whitney U-test). In contrast, no difference in the severity of palsy was observed between patients with HSV-1 reactivation and Bell's palsy. Twelve patients received acyclovir–prednisone treatment within 7 days of onset based on positive PCR results and ten of the 12 (83%) recovered completely. In contrast, 14 patients with HSV-1 reactivation received prednisone treatment because their PCR tests were performed at a later date; ten of these 14 (71%) recovered completely. The difference in the cure rate between the two treatment groups was not significant (P>0.05, Fisher exact test). Conclusions: The results indicate that the severity of palsy in patients with HSV-1 reactivation is similar to that in patients with Bell's palsy and suggest that early diagnosis of HSV-1 reactivation by PCR and subsequent acyclovir–prednisone therapy do not improve recovery from facial palsy.     

Nocturnal onset and development of Bell's palsy

OBJECTIVE/HYPOTHESIS: To examine the mechanism and pathophysiology of idiopathic peripheral facial palsy (Bell's palsy), the mode of onset of facial palsy was investigated. STUDY DESIGN: Retrospective case review. METHODS: We identified the point at which patients with facial palsy first noticed their illness using our medical charts for 648 patients and information from 3,580 facial palsy cases who visited an Internet site; we found that the time of a patient's first awareness of his or her illness was mentioned in 258 (204 Bell's palsy) and 53 cases, respectively. These cases were divided into three periods: morning, afternoon, and night. RESULTS: The ratio of morning:afternoon:night in the two groups was 141:30:33 and 50:0:3, respectively. These findings indicate that the majority of patients first noticed their palsy in the morning. CONCLUSION: Because several hours are required for facial palsy to develop before becoming apparent, this suggests that the onset and development of facial palsy occurred during sleep, when circulatory dynamics are reduced. In humans, ischemia is more likely to occur and produce facial palsy than virus reactivation.     

Delayed peripheral facial palsy in the stapes surgery: can it be prevented?

PURPOSE: The aim of this study was to evaluate poststapedectomy-delayed facial palsy etiopathogenesis, risk factors, evolution, and prevention. MATERIALS AND METHODS: Seven hundred six stapedectomies performed in 580 patients were reviewed. In all patients who developed delayed facial palsy, the dates of onset and subside of facial palsy, the anatomic and pathologic predisposing factors, and a possible history for recurrent labial herpetic lesions were considered. The House-Brackmann (H-B) grading system was used to evaluate the facial function. Virus-specific immunoglobulin (Ig) G and IgM antibodies against herpes simplex virus type 1 (HSV-1) were determined by enzyme-linked immunosorbent assay (ELISA) 3 weeks after the onset of the paralysis. The results were compared with a control group without a history of recurrent herpes labialis. RESULTS: Poststapedectomy facial palsy developed in 7 out of 706 procedures. All 7 patients referred a history of recurrent labial herpetic lesions. One patient showed a facial palsy H-B grade II, 2 a grade III, and 3 a grade IV. After acyclovir therapy, 6 subjects recovered completely, whereas 1 maintained an H-B grade II. An increased IgG antibody titer was found in 6 of the patients with delayed facial palsy and in 1 out of 7 controls. Mean IgG titer was 1:14,050 in the subjects with delayed facial palsy and 1:2,300 in controls (P <.001). CONCLUSIONS: Poststapedectomy-delayed facial palsy is likely caused by a reactivation of HSV-1, latent within the geniculate ganglion. The activation of the latent virus is more frequent in patients with a history of herpes labialis and can be prevented by an adequate acyclovir therapy.     

Recurrent facial palsy: Characteristics of ipsilateral and alternative palsies of 104 cases

ObjectiveRecurrent facial palsy is relatively rare and its clinical details of recurrent facial palsy are not well known. We analyzed recurrent facial palsy cases and clarified its characteristics, especially the difference between ipsilateral and alternative palsies. The analysis aimed to obtain information about recurrent facial palsy that would be useful for delivering explanations to patients and help improve recurrent facial palsy treatments based on the etiology.MethodsWe picked up data from the chart and analyzed the clinical characteristics of patients with recurrent facial palsy from 1243 facial palsy patients (Bell's palsy, VZV-related palsy (Ramsay Hunt syndrome and zoster sine herpete [ZSH])) between 2006 and 2020.ResultsRecurrent facial palsy was observed in 104 of 1243 patients (8.4%). There were 35 cases (34%) of ipsilateral palsy and 69 cases (66%) of alternative palsy. The mean age at the onset of the first palsy was 38.9 years old in the ipsilateral group and 48.4 years old in the alternative group, and a significant difference was observed between them. The number of recurrences ranged from 1 to 4. Among the ipsilateral group, 6 patients experienced more than second recurrence. In two cases, the condition failed to resolve after the second recurrence. A serological examination confirmed that 4 cases had recurrent VZV-related palsy (both the first and second palsies were VZV-related) and all of them initially had ZSH: no cases had Hunt syndrome as the first palsy.ConclusionsThe VZV-specific immunity obtained with ZSH might be insufficient to suppress VZV reactivation, and VZV vaccination should be recommended for ZSH patients to prevent further recurrence of VZV-related facial palsy. More than 2 ipsilateral recurrent episodes may be a risk factor for incomplete recovery.     

Delayed facial palsy after vestibular schwannoma resection: clinical data and prognosis

PURPOSE: The object of the present study was to review a series of surgically removed vestibular schwannoma tumours to establish the incidence of delayed facial palsy and to evaluate the course of recovery according to the possible etiology (surgical postoperative edema or viral reactivation) with reference to the time of onset. MATERIALS AND METHODS: The study group was composed of 98 patients with vestibular schwannoma. Sex, age, location, and extent of tumour and postoperative complications were all taken into consideration in the final evaluation. The course of each patient's postoperative facial function was graded according to House and Brackmann's six-grade scale. The incidence and the time of onset of the delayed facial palsy were also evaluated. RESULTS: The deterioration in the facial function was found to be delayed in 25 of the 98 patients (26%); of these, it occurred in the first 5 days after surgery in 11 cases, between 6 and 13 days in 10 cases, and after 15 days in 14 patients. The incidence rate of the delayed facial dysfunction was not influenced by age, sex, or the size of the tumour. The prognosis of the facial dysfunction was favourable in the majority of cases, and, in fact, there were only five grade III to IV cases 1 year later. Facial dysfunction was over grade III in the majority of the latter five cases, and the period of recovery was long. CONCLUSIONS: Eighty percent of our patients with delayed facial palsy following vestibular schwannoma resection were classified as having excellent or good function. In the remaining patients who had a less favourable recovery, the palsy was more severe, and the onset occurred after some time. This seems to agree with those who are of the opinion that the complication is due to viral reactivation. In these patients, it is advisable to start aggressive medical therapy with antiviral agents such as acyclovir as soon as possible.     

Varicella zoster virus in bell's palsy: a prospective study

Although Bell's palsy is the major cause of acute peripheral facial palsy, its pathogenesis remains unknown. Reactivation of the varicella zoster virus has been implicated as one of the main causes of Bell's palsy, however, studies which investigate the varicella zoster virus reactivation in Bell's palsy patients are mostly Japanese and, therefore, personal and geographic characteristics are quite different from our population.     

小鼠单纯疱疹病毒性面瘫的预防和复发模型的建立

目的建立1型单纯疱疹病毒（herpes　simplex　virus　type1，HSV-1）潜伏感染再激活导致小鼠面瘫的模型，观察免疫球蛋白（IgG）和干扰素对单纯疱疹病毒性小鼠面瘫的预防效果。方法64只4周龄雌性Balb／c小鼠，用26G针头搔刮小鼠耳廓背面近耳根部皮肤，左耳接种HSV．125山，总剂量1．7×10^7空斑形成单位（plaque　forming　unit，PFU）／ml，右耳接种25山磷酸盐缓冲液作为对照，制作小鼠面瘫潜伏模型。按数字表法将小鼠随机分为3组：第一组20只，每天腹腔注射IgG1ml／kg，连续注射3d，待出现面瘫后，面瘫小鼠继续注射3d；第二组20只，腹腔注射干扰素1．5×10’IU／kg，连续3d，待出现面瘫后，面瘫小鼠继续注射3d；第三组24只，腹腔注射相应量的生理盐水作为对照。观察小鼠面瘫情况，8周后取面瘫恢复的小鼠，腹腔注射环孢素50mg／kg，建立面瘫复发模型。处死小鼠，分离双侧面神经和三叉神经节，多聚酶链反应（polymerase chain reaction，PCR）检测HSV-1 DNA的表达。结果第一组小鼠面瘫率50％，面瘫持续时间（7．2±2．2）d（x±s，下同）；第二组小鼠面瘫率30％，面瘫持续时间（4．5±1．8）d；第三组小鼠面瘫率67％，面瘫持续时间（8．9±2．6）d。经统计学分析，IgG不能有效降低面瘫发生率及缩短面瘫持续时间（P值均〉0．05），而干扰素可以有效降低面瘫发生率并缩短面瘫持续时间（P值均〈0．05）。注射环孢素后3只面瘫恢复小鼠（3／28）再次出现面瘫，复发率11％。所有面瘫复发小鼠均检测到HSV-1DNA，而未复发小鼠均未检测出HSV-1DNA。结论HSV-1潜伏感染再激活可能是单纯疱疹病毒性小鼠面瘫复发的原因之一，潜伏病毒的再激活与免疫力低下有关。干扰素可以有效降低单纯疱疹病毒性面瘫发生率和缩短面瘫持续时间；免疫球蛋白（IgG）不能有效降低面瘫发生率和缩短面瘫持续时间。     

Die idiopathische Fazialisparese

Idiopathic facial palsy (IFP), or Bell’s palsy, is an acute peripheral unilateral paresis of the facial nerve with an abrupt onset of unknown origin. Primary infection or reactivation of the Herpes simplex virus is suggested as a possible mechanism in some but not all patients. Since IFP is a diagnosis of exclusion, all other causes, especially other neurological diseases or Herpes zoster reactivation need to be excluded, as does Lyme disease in children and endemic areas. If recovery or defective healing has not taken place within 6–12 months, it is mandatory to exclude malignant disease. Severity of the paresis and electromyography are to date the best prognostic markers for defective healing. Steroid application is the only evidence-based therapy to date with recovery rates >90%. The spontaneous recovery rate is about 80%. There is a lack of well defined diagnostic procedures to detect those patients who will recover spontaneously. On the other hand, patients with severe complete paresis might profit from additional antiviral drugs. There is an urgent need for further clinical trials in patients with severe IFP.     

Delayed facial palsy in a patient with a bifid facial nerve lending support for viral theory of facial palsy.

OBJECTIVE: To describe the level of neurologic impairment in a case of delayed facial palsy occurring after cochlear implantation surgery. PATIENT: A 58-year-old man undergoing cochlear implantation who was found intraoperatively to have congenital bifurcation of the facial nerve just distal to the second genu. INTERVENTION: Cochlear implantation was performed through a facial recess approach. RESULTS: The lateral branch of the nerve impinged on the posterior tympanotomy slot and was uncovered during the procedure, rendering it vulnerable to direct thermal or mechanical injury or to the effects of local tissue injury products. The patient developed facial palsy 9 days later, affecting all facial muscle groups equally. CONCLUSION: Theories regarding the cause of delayed facial palsy after cochlear implantation include direct thermal or mechanical injury to the nerve, local effects of blood breakdown products or other mediators causing vasospasm, and reactivation of latent herpes virus, leading to neural inflammation and neuropathy of the geniculate and labyrinthine segments of the nerve. The fact that the patient developed weakness that affected all facial muscle groups equally suggests that the level of neurologic impairment was proximal to the nerve bifurcation, so distant to the actual site of surgery. This finding lends support for the viral hypothesis of delayed nerve palsy.     

Virology studies and Bell's palsy.

A retrospective analysis of 104 cases of facial nerve paralysis is presented, attention being focused on possible clues to link Bell's palsy with a virus infection. The findings are compared to those of another larger prospective series of cases. Some recent developments which suggest that Bell's palsy represents a reactivation of herpes simplex virus, rather than a primary infection, are discussed.     

Bell's palsy and Herpes simplex virus: fact or mystery?

HYPOTHESIS AND BACKGROUND: In recent years, progress has been made in the understanding of Bell's palsy, the most common form of acute facial weakness. Herpes simplex virus (HSV) reactivation within the geniculate ganglion with subsequent inflammation and entrapment of the nerve at the meatal foramen has been proposed to be the pathogenetic mechanism. We challenged its accuracy by analyzing our own data on the presence of viral genomic DNA of HSV-1 and 2, human herpes virus (HHV)-6A/B, as well as varizella zoster virus (VZV) in patients with Bell's palsy and in control patients without the disease. METHODS: Polymerase chain reaction was performed with primer sets specific for viral genomic DNA of HSV-1, HSV-2, and VZV in facial muscle biopsy specimens from patients with Bell's palsy. As control specimens, the Scarpa's ganglion of patients with Meniere's disease and the geniculate ganglion harvested at autopsy from patients without history of facial palsy. In a second study, we used polymerase chain reaction with primers specific for HSV-1, -2, and HHV-6A, -6B to analyze for the presence of these viruses in tear fluid samples from control patients and patients with acute Bell's palsy. RESULTS: HSV-1 and VZV genomic DNA were detected in 86 and 43%, respectively, of geniculate ganglion preparations from control specimen. We were not able to detect the presence of HSV-1, HSV-2, or VZV genomic DNA in ganglion scarpae or muscle biopsy results in control and Bell's palsy patients. HHV-6A could be detected in tear fluid samples in 40% of control patients and 30% of Bell's palsy patients. CONCLUSIONS: The sole presence of HSV genomic DNA within the sensory ganglion along the facial nerve does not explain the direct association with Bell's palsy. The missing link would be the identification of an active replicating virus, an investigation that has not yet been carried out.     

Postoperative Ramsay-Hunt syndrome after acoustic neuroma resection. Viral reactivation

The aim of this paper is to present a patient suffering from acoustic neuroma and operated on with immediate postoperative hearing and facial function preservation who developed delayed Ramsay-Hunt syndrome. To our knowledge, this is the first case in whom a postoperative delayed facial palsy and hearing loss occurred. The patient gave an history of previously diagnosed herpes zoster reactivation limited to chest one-year before. This is undoubtdetly a predisposing factor for development of delayed facial palsy. It must not be underestimated and it obliges to consider a prophylaxis. Theoretically, the prophylactic antiviral therapy might prevent the evolution towards the herpes zoster oticus or reduce the severity of the symptoms allowing the preservation of the hearing function. It would be pointed out that the delayed facial plasy has favourable prognosis, while the hearing impairment may recover with a greater difficulty even after an antiviral treatment as in our case.     

The inflammatory pseudotumor presenting periodic acid-Schiff-positive inclusions with acute unilateral facial nerve palsy

Although most acute peripheral facial palsies can be attributed to Bell’s palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor.     

Idiopathic facial palsy. Viral, hereditary, or both?

Two cases of acute peripheral facial paralysis occurring in two siblings in a family of six members, who all developed influenza-like symptoms, are reported. Both patients with facial paralysis, as well as two other members of the family had elevated titers against herpes simplex virus, even though it was not possible to demonstrate any raise in titers between the acute and convalescent phase. The "epidemic" occurrence is from a statistical point of view more than just a coincidence, and it is concluded that these findings support the hypothesis that Bell's palsy may be caused by reactivation of herpes simplex virus, probably facilitated by some hereditary component. The authors suggest that a combination of infectious (most likely viral) and hereditary factors may be etiologically important for the occurrence of acute peripheral facial paralysis.     

Central nervous system involvement in patients with facial palsy due to borrelial infection

Patients with a peripheral facial palsy due to an underlying Ixodes-borne borreliosis often have cerebrospinal fluid findings indicating meningeal involvement. The aim of the present study was to identify signs of CNS involvement by means of brain-stem evoked response audiometry (BSER) in patients with a facial palsy due to borreliosis, as well as in patients with a facial palsy of unknown aetiology. Bell's palsy. Abnormalities in BSER were found to be significantly more common along the borrelial patients. Furthermore, a reversal of these abnormalities following treatment with benzyl-penicillin was found in the majority of patients with borreliosis. The results of the present study emphasize the importance of diagnosing and treating an underlying borreliosis in patients with a peripheral facial palsy.     

The true nature of Bell's palsy: analysis of 1,000 consecutive patients

In a series of 1502 patients seen in our Facial Paralysis Research Clinic 1048 were diagnosed as having Bell's palsy. Review of clinical, epidemiologic, and laboratory data, plus review of the literature, has led to the conclusion that Bell's palsy is an acute benign cranial polyneuritis probably caused by reactivation of the herpes-simplex virus, and the dysfunction of the motor cranial nerves (V, VII, X) may represent inflammation and demyelinization rather than ischemic compression. Spinal fluid analysis suggests that the disease is a phenomenon of the central nervous system with secondary peripheral neural manifestations. With our presently available information, treatment of a viral disease with an anti-inflammatory agent is rational. Prednisone treatment started within the first week of the disease can restore better function to the paralyzed face than is achieved without such therapy, and facial nerve decompression has been unnecessary.