Cochleovestibular Nerve Research Articles

Evaluation of cochleo-vestibular functions in patients with auditory neuropathy

BackgroundAuditory neuropathy (AN) is a specific hearing disorder with abnormal auditory neural responses in the presence of normal cochlear function. Affection of vestibular portion of cochleo-vestibular (VIII) nerve was only reported in few studies. ObjectivesTo evaluate the cochleo-vestibular function in these patients and to detect any relationship between both cochlear and vestibular functions. MethodThe study was conducted on forty (AN) patients. All subjects were submitted to: full audiological test battery; history taking, clinical otological examination, basic audiological evaluation, Auditory Brainstem Response (ABR), Transient Evoked Otoacoustic Emissions (TEOAEs) and threshold equalizing test (TEN). Vestibular test battery; (vestibular evoked myogenic potential (VEMP) and VNG test battery). ResultsAudiological test battery confirmed the audiological criteria for auditory neuropathy reported in the literature. Results of this work showed that, there was preservation of TEOAE in all patients. Also, there was an increase in amplitude of CM in AN. Moreover, all AN patients showed presence of dead regions that started first in low frequencies and with increase number of dead regions the mid and high frequencies also affected. In addition to that the vestibular function tests showed that VEMP was preserved bilaterally in 15 patients, unilateral in 10 patients and absent in 15 patients. On the other side, VNG showed normal central vestibular system with unilateral weakness in 10 ears only. ConclusionPatients with auditory neuropathy could also have vestibular neuropathy. Vestibular neuropathy could be classified into three groups: superior vestibular neuropathy, inferior vestibular neuropathy and superior/inferior vestibular neuropathy.

Radiological assessment of the Indian children with congenital sensorineural hearing loss.

Introduction. Congenital sensorineural hearing loss is one of the most common birth defects with incidence of approximately 1 : 1000 live births. Imaging of cases of congenital sensorineural hearing loss is frequently performed in an attempt to determine the underlying pathology. There is a paucity of literature from India and for this reason we decided to conduct this study in Indian context to evaluate the various cochleovestibular bony and nerve anomalies by HRCT scan of temporal bone and MRI with 3D scan of inner ear in a tertiary care centre. Material and Methods. A total of 280 children with congenital deafness (158 males and 122 females), between January 2002 to June 2013 were included in the study and they were assessed radiologically by HRCT scan of temporal bone and MRI with 3D scan of inner ear. Results. In the present study we found various congenital anomalies of bony labyrinth and vestibulocochlear nerve. Out of 560 inner ears we found 78 anomalous inner ears. Out of these 78 inner ears 57 (73%) had cochlear anomaly, 68 (87.1%) had anomalous vestibule, 44 (56.4%) had abnormal vestibular aqueduct, 24 (30.7%) had anomalous IAC, and 23 (29.4%) had abnormal cochleovestibular nerves. Conclusion. In present study, we found lower incidences of congenital anomalies comparative to existing literature.

Cochleovestibular nerve development is integrated with migratory neural crest cells

The cochleovestibular (CV) nerve, which connects the inner ear to the brain, is the nerve that enables the senses of hearing and balance. The aim of this study was to document the morphological development of the mouse CV nerve with respect to the two embryonic cells types that produce it, specifically, the otic vesicle-derived progenitors that give rise to neurons, and the neural crest cell (NCC) progenitors that give rise to glia. Otic tissues of mouse embryos carrying NCC lineage reporter transgenes were whole mount immunostained to identify neurons and NCC. Serial optical sections were collected by confocal microscopy and were compiled to render the three dimensional (3D) structure of the developing CV nerve. Spatial organization of the NCC and developing neurons suggest that neuronal and glial populations of the CV nerve develop in tandem from early stages of nerve formation. NCC form a sheath surrounding the CV ganglia and central axons. NCC are also closely associated with neurites projecting peripherally during formation of the vestibular and cochlear nerves. Physical ablation of NCC in chick embryos demonstrates that survival or regeneration of even a few individual NCC from ectopic positions in the hindbrain results in central projection of axons precisely following ectopic pathways made by regenerating NCC.

Bilateral deafness secondary to diffusion weighted imaging-proven cochleo-vestibular nerve and brainstem infarctions

stv.2013.0788 Auteur(s) : Joseph Ben Zakoun1, Guillaume Turc1 g.turc@ch-sainte-anne.fr, Myriam Edjlali-Goujon2, Catherine Oppenheim2, Jean-Louis Mas1 1 Centre hospitalier Sainte Anne, pole Raymond Garcin, service de neurologie, 1 rue Cabanis, 75014 Paris, France ; Universite Paris Descartes Sorbonne Paris Cite, unite INSERM UMR S894, Paris, France 2 Centre hospitalier Sainte Anne, service d’imagerie morphologie et fonctionnelle, 1 rue Cabanis, 75014 Paris, France Tires a part : G. Turc La [...]

Audiovestibular Characteristics of Small Cochleovestibular Schwannomas in Neurofibromatosis Type 2

Objectives: Describe the relationship between cochleovestibular schwannoma (CVS) volume, audiovestibular changes, and magnetic resonance imaging (MRI) findings in patients with neurofibromatosis Type 2 (NF-2). Methods: Subgroup analysis of NF-2 prospective natural history study from June 2008 to June 2011. Subjects: NF-2 patients with small treatment- naïve CVS (volume < 1cm3) by ear. N = 49 ears, 32 patients. Setting: Quaternary medical research institute. Intervention: None. Outcome Measurements: Tumor size, auditory brainstem response (ABR), audiogram (4-frequency PTA - 0.5, 1, 2, 4KHz), vestibular evoked myogenic potential (VEMP), caloric videonystagmography (VNG), rotary chair testing (240°/sec velocity step test), and magnetic resonance imaging (MRI) findings. Results: Larger tumor volume (mean 0.489cm3; 95% confidence interval [CI] 0.313-0.664) correlated with abnormal ABR findings, compared to smaller tumors with normal ABR (mean 0.151cm3; 95%CI 0.89-0.213). Larger tumors also correlated with weak caloric response (mean 0.520cm3; 95%CI 0.300-0.741 vs. mean 0.199cm3; 95%CI 0.102-0.296). Tumor volume was not significantly correlated with PTA, VEMP, or rotary chair testing. Elevated intralabyrinthine protein on MRI fluid-attenuated inversion recovery sequences was correlated with larger tumor size (mean volume 0.331cm3; 95%CI 0.246-0.419 vs. 0.055cm3; 95%CI 0.008-0.103) and abnormal ABR and PTA ( P < 0.05), but it did not correlate with VEMP or caloric responses. Conclusions: In our sample, ABR, caloric response, and intralabyrinthine protein correlated with tumor volume, but PTA, VEMP, and high velocity step testing did not. Abnormal ABR and PTA were correlated with elevated protein. These findings may provide insight on the effect of small CVS on the inner ear and cochleovestibular nerves, which may aid in their management.

The lower cranial nerves: IX, X, XI, XII

The lower cranial nerves innervate the pharynx and larynx by the glossopharyngeal (CN IX) and vagus (CN X) (mixed) nerves, and provide motor innervation of the muscles of the neck by the accessory nerve (CN XI) and the tongue by the hypoglossal nerve (CN XII). The symptomatology provoked by an anomaly is often discrete and rarely in the forefront. As with all cranial nerves, the context and clinical examinations, in case of suspicion of impairment of the lower cranial nerves, are determinant in guiding the imaging. In fact, the impairment may be located in the brain stem, in the peribulbar cisterns, in the foramens or even in the deep spaces of the face. The clinical localization of the probable seat of the lesion helps in choosing the adapted protocol in MRI and eventually completes it with a CT-scan. In the bulb, the intra-axial pathology is dominated by brain ischemia (in particular, with Wallenberg syndrome) and multiple sclerosis. Cisternal pathology is tumoral with two tumors, schwannoma and meningioma. The occurrence is much lower than in the cochleovestibular nerves as well as the leptomeningeal nerves (infectious, inflammatory or tumoral). Finally, foramen pathology is tumoral with, outside of the usual schwannomas and meningiomas, paragangliomas. For radiologists, fairly hesitant to explore these lower cranial pairs, it is necessary to be familiar with (or relearn) the anatomy, master the exploratory technique and be aware of the diagnostic possibilities.

Editorial

FigureAs part of the American Board of Internal Medicine Foundation's Choosing Wisely campaign, the American Academy of Otolaryngology–Head and Neck Surgery Foundation (AAO-HNSF) recently published its list of “Five Things Physicians and Patients Should Question.” The goals of the campaign were to promote awareness of the overuse of tests and treatments and to improve the overall quality of American healthcare. The AAO-HNSF Patient Safety and Quality Improvement Committee was charged with leading an unbiased and equitable process to arrive at five conditions. The committee reviewed 20 initial items for consideration and then refined the list based on the amount of supporting clinical evidence and the frequency of use of the test, procedure, or treatment. Three of the final five items address diseases of the ear. 1. Don't order a computed tomography (CT) scan of the head/brain for sudden hearing loss. The support for this item comes from a recent AAO-HNSF Clinical Practice Guideline (Otolaryngol Head Neck Surg 2012;146[suppl 3]:S1-35).Figure: Michael E. McCormick, MDThe role of imaging in a patient with sudden sensorineural hearing loss (SSNHL) is to identify pathology that may be causing acute symptoms. SSNHL typically results from an abnormality of the cochlea, cochleovestibular nerve (CN VIII), or brainstem. While we are unable to accurately image the inside of the cochlea with current modalities, more central etiologies, such as the CN VIII and brainstem, are best evaluated by magnetic resonance imaging.Figure: Rahul K. Shah, MDTherefore, a routine CT scan of the head/brain adds no diagnostic yield to the workup of these patients in most clinical situations. Furthermore, CT scans expose the patient to potentially harmful radiation and intravenous contrast (Expert Opin Drug Metab Toxicol 2009;5[4]:403-416; Arch Intern Med 2009;169[22]:2078-2086). 2. Don't prescribe oral antibiotics for uncomplicated acute tympanostomy tube otorrhea. Ototopical antibiotic drops are typically 100 to 1,000 times more concentrated than oral preparations and do not need to undergo systemic metabolism and biodistribution (Drugs 1999;58[3]:509-531). As a consequence, topical preparations often can treat bacteria that oral antibiotics cannot. Using antibiotic eardrops instead of oral agents can also help prevent the development and spread of resistant bacteria (Pediatr Infect Dis J 2001;20[1]:102-103) and avoids the systemic side effects associated with oral therapy, such as vomiting, diarrhea, and rash.Figure. David: W. Roberson, MD3. Don't prescribe oral antibiotics for uncomplicated acute external otitis. Similar to the first recommendation, this item is supported by evidence from an AAO-HNSF Clinical Practice Guideline (Otolaryngol Head Neck Surg 2006;134[suppl 4]:S4-S23). The item applies primarily to healthy patients without complications or extension beyond the ear canal. In addition to the benefits of curbing overuse of oral antibiotics, there are multiple nonantibiotic preparations that are efficacious. Awareness of and general adherence to these simple recommendations by our health professional colleagues could potentially save an immeasurable amount of resources while improving the quality and perhaps outcomes of care in the United States.

Cochleovestibular nerve involvement in multifocal fibrosclerosis

To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites. A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine. This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.

AN ENBLARGED DISMANTABLE ANATOMICAL MODEL OF EAR IN PAPIER MACHE BY AUZOUX, OBSERVATION AND PHOTOGRAPHICAL TRI‐DIMENSIONAL RECONSTRUCTION

The aim of the study was to determine the accuracy of a 19th century anatomical model of ear by Auzoux (a french manufacturer). It measured 63cm in length; 43cm in width; 37cm in height. The disassembled parts were studied on the morphological aspect in its whole and after removing of its components: external ear (43×23×15cm), tympanic drum and middle ear ossicles; cochleo‐vestibular apparatus. The main dimensions of each part were recorded. The arterial vascularisation of the three components of the ear and its sensitive and sensorial nervous system were established on the model which represented the external carotid artery with its terminal bifurcation, several collateral branches specially the posterior auricular artery at the two extremities of the carotid canal. The cochleo‐vestibular and facial nerve as well as other elements of small caliber were exactly figured and labelled by a number. The middle ear components were disassembled. The ossicles of the middle ear and a dried, peritoneal tympanic membrane were dismountable as a whole. This kind of rare model can be dated at the earliest 1835.

Cogan Syndrome

Patients with Cogan syndrome typically present with nonsyphilitic interstitial keratitis and acute onset of sensorineural hearing loss. Neurological manifestations have been reported, but various frequencies and mechanisms have been proposed. We critically reviewed the English literature of Cogan syndrome to determine the nature, frequency, and most likely mechanisms of its neurological manifestations. On the basis of our review, we believe that Cogan syndrome can be associated with neurological manifestations. Our conclusion is based on reported tissue evidence of vasculitis involving the dura, brain, optic nerve, cochleovestibular nerve, and muscle, in patients with referable symptoms. However, we believe that the frequency of neurological manifestations may have been over reported due to lack of confirmatory testing in many of these cases.

Microvascular decompression of cochleovestibular nerve in patients with tinnitus and vertigo

The aim of this study was to investigate the patient selection, indications for operation, surgical methods and effects of microvascular decompression (MVD) for treatment of intractable and incapacitating tinnitus and vertigo. A total of 35 patients were recruited from January 2009 to June 2010 for the MVD surgery. Arterial compression of cochleovestibular nerve was found in all the 35 patients. The immediate effect of MVD was observed in 22 patients with tinnitus and 10 patients with vertigo. The follow-up data for the effect of MVD showed complete resolution of tinnitus in 14 patients and vertigo in 6 patients. Vascular compression of cochleovestibular nerve in cerebellopontine angle may be one of the causes of intractable and incapacitating tinnitus and/or vertigo. MVD may be a safe and effective surgical procedure for treatment of severe tinnitus and/or vertigo in patients.

Imaging of sensorineural hearing loss: a pattern-based approach to diseases of the inner ear and cerebellopontine angle

An overview is presented of the common and uncommon diseases of the inner ear and of the cochleovestibular nerve within the internal acoustic meatus and cerebellopontine angle cistern causing sensorineural deafness.An imaging-pattern-based approach is used to help detect disease and narrow the differential diagnosis. Main Messages • The most common soft tissue mass lesions in the cerebellopontine angle are schwannoma and meningioma. • Contrast-enhanced MRI may reveal clinically unsuspected inflammatory, auto-immune or tumoural disease. • Hearing loss may be caused by infection, inflammation or, rarely, perineural tumour spread along the cochleovestibular nerve. • Labyrinthitis may lead to rapidly progressive ossification of the labyrinth.

Non-accidental caustic ear injury: Two cases of profound cochleo-vestibular loss and facial nerve injury

Non-accidental caustic injury is a rare form of child abuse usually secondary to forced ingestion. Caustic injury to the ear most commonly arises from battery lodgement in the external canal. This case series represents the first report of non-accidental caustic injury to previously normal ears resulting in profound sensorineural hearing loss, vertigo with horizontal canal hypofunction and in one patient a severe facial nerve paralysis. Both patients required blind-end sac closure of the injured ear and one required sural nerve interposition combined with transfer of the masseteric branch of the trigeminal nerve to the ipsilateral facial nerve.

CHD7 Mutational Analysis and Clinical Considerations for Auditory Rehabilitation in Deaf Patients with CHARGE Syndrome

BackgroundOtologic manifestations are one of the most consistent findings of CHARGE syndrome found in more than 90%. Since genetic analysis of the CHD7 gene has rarely been performed in previous reports dealing with ear abnormalities, the genotypic spectrum of CHD7 mutations was analyzed in deaf patients with CHARGE syndrome, and the clinical considerations concerning auditory rehabilitation were investigated.MethodsNine Korean patients with CHARGE syndrome showing profound hearing loss and semicircular canal aplasia were included. All 38 exons of CHD7 were analyzed by direct sequencing. For splice site variations, in silico and exon-trapping analyses were performed to verify the pathogenicity of nucleotide variations. Clinical features and the outcome of auditory rehabilitation were also analyzed.ResultsEight of 9 patients revealed alterations of the CHD7 gene including 3 frameshift, 2 nonsense, 2 splice site, and 1 missense mutations. Five of 9 patients were clinically diagnosed as atypical CHARGE syndrome but demonstrated various mutations of the CHD7 gene. One familial case showed intra-familial variability. Radiologic findings suggesting cochleovestibular nerve deficiency were identified in most of the patients. Of the 8 patients who underwent cochlear implantation, 5 patients demonstrated favorable outcome. Larger diameter of the cochleovestibular nerve on imaging and absence of severe mental retardation were factors related to better outcome after cochlear implantation rather than the type of CHD7 mutations. Auditory brainstem implantation was performed in two patients who did not benefit from cochlear implantation.ConclusionsGenetic analysis of the CHD7 gene should be performed in cases with semicircular canal aplasia even when other typical features of CHARGE syndrome are absent. For auditory rehabilitation in CHARGE syndrome, cochlear implantation should be strongly recommended in selected cases with favorable prognostic factors. Auditory brainstem implantation may be a viable option in patients with CHARGE syndrome who have failed to benefit from cochlear implantation.

Cochlear Implant Outcomes in Children with Aplasia/Hypoplasia of the Cochleovestibular Nerve

Objective: Cochlear implant (CI) candidates undergo a preoperative workup including radiologic, audiologic, and neurologic testing to determine if they are candidates for implantation. A percentage of these patients are found to have aplasia/hypoplasia of the cochleovestibular nerve (CVN). Past literature has debated if this population of children benefit from CI. Method: Retrospective review evaluating 17 children with radiographically diagnosed aplasia/hypoplasia of CVN. Eight of these children underwent cochlear implantation during the period from 2002 to 2009. The remaining 9 children were treated nonsurgically with hearing aids. Medical records, audiologic testing, imaging studies, and speech and language evaluations were reviewed. Results: Meaningful Auditory Integration Scale (MAIS) scores were available for 6 out of 8 CI patients. The average MAIS score pre-CI was 6.16. The average scores post-CI were 46.6, 72.8, 70.25, and 75. Auditory Preschool Language Scale (PLS-4) pre-CI was 4.75, and post-CI score was 23. The pre- and post-CI Expressive PLS-4 scores improved from 11.25 to 23.5. Phonetic inventory scores were 3.7 for vowel sounds and 3.17 for consonant sounds. Scores post-CI were 10.8 for vowel sounds and 12.5 for consonants. Linear regression analysis revealed a significantly faster acquisition of consonant sounds with the CI group. Conclusion: Children with CVN aplasia/hypoplasia who received a CI had improved audiologic and speech and language assessments post-CI. The CI group averaged higher scores in vowel and consonant sound production, which was statistically significant. Long term follow-up is needed to further evaluate outcomes of CI with CVN aplasia/hypoplasia.

Superficial Siderosis of the Central Nervous System

Superficial siderosis of the central nervous system (SSCN) results after chronic subarachnoid hemorrhage. Consequent demyelination, particularly of the cochleovestibular nerve and cerebellum, causes auditory-vestibular dysfunction. Predominant symptoms include progressive sensorineural hearing loss, imbalance, and ataxia. Despite characteristic auditory-vestibular involvement, SSCN is not well known among the hearing health community. Clinical records of 49 patients diagnosed with SSCN were reviewed. Analysis included review of demographic, audiometric, and vestibular data of the largest sample to date and comparison to 31 audiovestibular case reports in the literature. Hearing loss and disordered balance were reported by 92% and 67% of patients, respectively. Results suggest variable but substantial auditory-vestibular involvement related to SSCN. Hearing loss is typically progressive, sloping, and asymmetric and exceeds hearing loss expected based on age or sex. Decreased word recognition is possible and traditional amplification may fail to provide benefit. SSCN is a destructive disorder affecting the auditory-vestibular system. Although not a common diagnosis, SSCN may be more prevalent than clinicians realize. Site of lesion may be anywhere within the auditory-vestibular system from the inner ear to the cortex, although the cochleovestibular nerve and cerebellum are particularly vulnerable. The progressive retrocochlear nature of the disorder makes differential diagnosis difficult and development of effective treatment options challenging. It is essential that audiologists and otologists recognize this uncommon cause of sensorineural hearing loss and balance disorder and the implications for evaluation, treatment, and counseling.

The cochleovestibular nerve identified during auditory brainstem implantation in patients with narrow internal auditory canals: Can preoperative evaluation predict cochleovestibular nerve deficiency?

To analyze the value of preoperative diagnostic tools in predicting the status of the cochleovestibular nerve (CVN) in patients with narrow internal auditory canals (IAC). Retrospective case series at a tertiary hospital. Eight profoundly deaf patients with narrow IACs who received auditory brainstem implantation were included in this study. The results of preoperative imaging, electrophysiologic, and auditory tests were correlated with the CVN status identified during auditory brainstem implantation. Temporal bone computed tomography (CT) findings, including the patency of the bony cochlear nerve canal and the diameter of the IAC, were limited in accurately reflecting the status of the CVN. Magnetic resonance imaging (MRI) and preoperative auditory responses to either pure tone or environmental sounds were more accurate markers for detecting the presence of a CVN than CT; however, there were limitations in cases with a very thin CVN or combined severe mental retardation. Absence of promontory or intracochlear electrically evoked auditory brainstem responses were not always indicative of an absent CVN. Visualization on MRI and detection of auditory responses suggested the presence of a CVN in patients with narrow IACs; however, the possibility of the presence of a CVN should be considered even when there is no clear evidence of a CVN on preoperative evaluations. Therefore, physicians should be prudent when determining candidacy for cochlear implantation or auditory brainstem implantation in patients with narrow IACs.

Pontine Tegmental Cap Dysplasia

Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors' experience with bilateral cochlear implantation in 1 patient are described. Retrospective review. Two tertiary medical centers. Three patients were evaluated by an otolaryngologist and underwent magnetic resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed. All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response. The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.

Options for hearing rehabilitation in children with cochleovestibular nerve dysplasia

Introduction Nowadays, children with limited abilities to develop open-speech understanding are considered as cochlear implant candidates. Children with anatomical abnormalities of the inner ear and particularly with deficiency of the cochleovestibular nerve (CVN) represent such great challenges. The achievement of speech development in these patients may be impossible (Nikolopoulos and Kiprouli, 2004). In 1997, Casselman et al. described for the first time the malformations of CVN, with the help of magnetic resonance imaging (MRI). A classification of abnormalities of three types, based on embryologic knowledge, was proposed. Type one includes aplasia of the CVN which is associated with stenosis of the internal auditory canal. Type two describes common CVN with aplasia or hypoplasia of its cochlear branch and finally type three refers to the presence of a common CVN with aplasia or hypoplasia of the vestibular branch (Casselman et al., 1997). Due to the wide use of MRI, CVN dysplasias were better identified and examined (Adunka et al., 2006). In this way, different methods of diagnostic assessment and therapeutic intervention were suggested in case series, presenting each of them one sole solution to the problem, either cochlear or auditory brainstem implantation (Govaerts et al., 2003; Colletti and Zoccante, 2008; Song et al., 2010). Systematic approach and universally established algorithms are still missing, though. The aim of this study was to present a variety of diagnostic and treatment options when dealing with CVN dysplasia.

Prognosis of ramsay hunt syndrome presenting as cranial polyneuropathy

Ramsay Hunt syndrome is known to be accompanied with cranial polyneuropathy very occasionally. We reviewed our experience to analyze the clinical manifestations and prognosis of these cases. Retrospective review. Eleven patients with Ramsay Hunt syndrome presenting as cranial polyneuropathy between 1999 and 2009 were included in this study retrospectively. All patients were treated with systemic steroids and antiviral agents. Assessment of recovery from facial nerve palsy (FNP) using the House-Brackmann (HB) grading system was performed, and the follow-up period was 4 to 28 months. The patient numbers for the initial degree of FNP were five for HB grade II, one for grade III, two for grade IV, and three for grade V. The cochleovestibular nerve was involved in 10 cases, and hearing loss was observed in nine patients. Other involved cranial nerves were IX in seven cases, X in five cases, V in three cases, and III/XII in one case each. Antiviral agents and steroids were administered within 3 days after the onset of symptoms for about 1 to 2 weeks in most cases. After the treatment the degree of FNP improved to HB grade I or II in nine of 11 patients (81.8%). Symptoms of other cranial nerves also improved in most patients, whereas recovery for hearing loss showed poor results. Ramsay Hunt syndrome associated with cranial polyneuropathy has a good prognosis for FNP and other cranial nerve palsies with a combination therapy of antiviral agents and steroids, but not for recovery of hearing loss.