Progressive Sensorineural Hearing Loss Research Articles

Oxytocin as a protective agent in cisplatin-induced ototoxicity.

Cisplatin is a potent chemotherapeutic drug with serious side effects such as ototoxicity which is characterized by irreversible, bilateral, progressive sensorineural hearing loss. Oxytocin, which is a well-known hormone secreting during pregnancy, has antioxidant and antiinflammatory effect. Our study aims to test and compare the effect of intratympanic (IT) and intraperitoneal (IP) oxytocin on cisplatin ototoxicity with DPOAE. A total of 24 Wistar albino rats were randomly divided into four groups: Group 1 received 0.1-0.3ml IT saline+IP saline solutions for 4days (n=6), Group 2 received cumulative dose of 20mg/kg IP cisplatin divided into two equal doses in first and second days of experiment+0.1-0.3ml IT saline for 4days, Group 3 received same dose of cisplatin as Group 2+0.1-0.3ml IT oxytocin for 4days, and Group 4 received same dose of cisplatin as Groups 2 and 3+IP oxytocin with dose of 1mg/kg. DPOAE was performed prior to procedure and at the end of the experiment on day 5. Group 2 showed severe ototoxic effect of cisplatin according to DPOAE result (p<0.05). When compared with Group 2, DPOAE amplitude reductions were smaller in Group 3 (3.2, 3.8, 4.5, 6.3 and 7.6kHz) (p<0.05) and Group 4 which is statistically significant in 5.4, 6.3 and 7.6kHz (p<0.05). When Group 3 and Group 4 were compared, reductions were smaller in 2.7 and 3.2kHz in Group 3 (p<0.05). In this study, we showed the protective effect of IT and IP oxytocin on cisplatin ototoxicity. We suggest oxytocin in cisplatin ototoxicity, especially via IT route even with high-dose cisplatin.

Mutations in EXOSC2 are associated with a novel syndrome characterised by retinitis pigmentosa, progressive hearing loss, premature ageing, short stature, mild intellectual disability and distinctive gestalt

BackgroundRetinitis pigmentosa in combination with hearing loss can be a feature of different Mendelian disorders. We describe a novel syndrome caused by biallelic mutations in the ‘exosome component 2’ (EXOSC2)...

A Case of Leptomeningeal Carcinomatosis with Progressive Sensorineural Hearing Loss and Facial Paralysis on Both Sides

A Case of Leptomeningeal Carcinomatosis with Progressive Sensorineural Hearing Loss and Facial Paralysis on Both Sides

両側の進行性感音難聴および顔面神経麻痺をきたした髄膜癌腫症例

両側の進行性感音難聴および顔面神経麻痺をきたした髄膜癌腫症例

Immune-mediated cochleovestibular disease.

The aim of this study is to prove the involvement of the immune response in the etiopathogenesis of some cochleovestibular disorders by a demonstration of antibodies against inner ear antigens and identify the benefits of immunosuppressive therapy. McCabe in 1979 postulated the hypothesis of autoimmune inner ear disease. Sodium dodecyl sulfate polyacrylamid gel electrophoresis and immunoblotting were used to examine the serum of 74 subjects for the presence of antibodies against inner ear antigens. The subjects were divided into three groups: A--subjects with idiopathic progressive sensorineural hearing loss, B--subjects with Menière´s disease, C--healthy subjects. Individuals with proven antibodies received immunosuppressive therapy. We detected antibodies against inner ear antigens with molecular weight of 30, 50, 60, 80, 100 kDa. In group A they were found in 52% of 25 subjects, in group B in 44% of 25 subjects and they were not detected in group C. An improvement of hearing was recorded in 69% of subjects in group A. An improvement of hearing was observed in 72%, significant relief of vertigo in 81% of subjects in group B. The present study supports the hypothesis of immune-mediated cochleovestibular disease (Tab. 3, Ref. 15).

Two Cases of Traumatic Stapedial Dislocation Caused by a Cotton Tipped Swab

A stapedial dislocation is a rare complication of ossicular trauma that is most commonly caused by direct, penetrating injury to the external ear canal. In this type of ossicular dislocation, patients usually suffer from cochleovestibular symptoms including progressive sensorineural hearing loss, conductive hearing loss, tinnitus, and vertigo. We present rare cases of internal stapedial dislocation after penetrating trauma, which resulted in the preservation of bone conduction hearing thresholds and complete resolution of the vestibular symptoms after an urgent repair. We have also reviewed the literature to determine the optimal management of stapedial dislocations. Korean J Otorhinolaryngol-Head Neck Surg 2016;59(2):155-61 Key WordsZZLabyrinthine fistula ㆍOssicular disruption ㆍStapes.

Hair Cell Loss, Spiral Ganglion Degeneration, and Progressive Sensorineural Hearing Loss in Mice with Targeted Deletion of Slc44a2/Ctl2.

SLC44A2 (solute carrier 44a2), also known as CTL2 (choline transporter-like protein 2), is expressed in many supporting cell types in the cochlea and is implicated in hair cell survival and antibody-induced hearing loss. In mice with the mixed C57BL/6-129 background, homozygous deletion of Slc44a2 exons 3–10 (Slc44a2Δ/Δ) resulted in high-frequency hearing loss and hair cell death. To reduce effects associated with age-related hearing loss (ARHL) in these strains, mice carrying the Slc44a2Δ allele were backcrossed to the ARHL-resistant FVB/NJ strain and evaluated after backcross seven (N7) (99 % FVB). Slc44a2Δ/Δ mice produced abnormally spliced Slc44a2 transcripts that contain a frameshift and premature stop codons. Neither full-length SLC44A2 nor a putative truncated protein could be detected in Slc44a2Δ/Δ mice, suggesting a likely null allele. Auditory brain stem responses (ABRs) of mice carrying the Slc44a2Δ allele on an FVB/NJ genetic background were tested longitudinally between the ages of 2 and 10 months. By 6 months of age, Slc44a2Δ/Δ mice exhibited hearing loss at 32 kHz, but at 12 and 24 kHz had sound thresholds similar to those of wild-type Slc44a2+/+ and heterozygous +/Slc44a2Δ mice. After 6 months of age, Slc44a2Δ/Δ mutants exhibited progressive hearing loss at all frequencies and +/Slc44a2Δ mice exhibited moderate threshold elevations at high frequency. Histologic evaluation of Slc44a2Δ/Δ mice revealed extensive hair cell and spiral ganglion cell loss, especially in the basal turn of the cochlea. We conclude that Slc44a2 function is required for long-term hair cell survival and maintenance of hearing.

Theory-of-mind in individuals with Alström syndrome is related to executive functions, and verbal ability.

Objective: This study focuses on cognitive prerequisites for the development of theory-of-mind (ToM), the ability to impute mental states to self and others in young adults with Alström syndrome (AS). AS is a rare and quite recently described recessively inherited ciliopathic disorder which causes progressive sensorineural hearing loss and juvenile blindness, as well as many other organ dysfunctions. Two cognitive abilities were considered; Phonological working memory (WM) and executive functions (EF), both of importance in speech development.Methods: Ten individuals (18–37 years) diagnosed with AS, and 20 individuals with no known impairment matched for age, gender, and educational level participated. Sensory functions were measured. Information about motor functions and communicative skills was obtained from responses to a questionnaire. ToM was assessed using Happés strange stories, verbal ability by a vocabulary test, phonological WM by means of an auditory presented non-word serial recall task and EF by tests of updating and inhibition.Results: The AS group performed at a significantly lower level than the control group in both the ToM task and the EF tasks. A significant correlation was observed between recall of non-words and EF in the AS group. Updating, but not inhibition, correlated significantly with verbal ability, whereas both updating and inhibition were significantly related to the ability to initiate and sustain communication. Poorer performance in the ToM and EF tasks were related to language perseverance and motor mannerisms.Conclusion: The AS group displayed a delayed ToM as well as reduced phonological WM, EF, and verbal ability. A significant association between ToM and EF, suggests a compensatory role of EF. This association may reflect the importance of EF to perceive and process input from the social environment when the social interaction is challenged by dual sensory loss. We argue that limitations in EF capacity in individuals with AS, to some extent, may be related to early blindness and progressive hearing loss, but maybe also to gene specific abnormalities.

Congenital cytomegalovirus infection in pregnancy: a review of prevalence, clinical features, diagnosis and prevention.

Human cytomegalovirus (CMV) is under-recognised, despite being the leading infectious cause of congenital malformation, affecting ~0.3% of Australian live births. Approximately 11% of infants born with congenital CMV infection are symptomatic, resulting in clinical manifestations, including jaundice, hepatosplenomegaly, petechiae, microcephaly, intrauterine growth restriction and death. Congenital CMV infection may cause severe long-term sequelae, including progressive sensorineural hearing loss and developmental delay in 40-58% of symptomatic neonates, and ~14% of initially asymptomatic infected neonates. Up to 50% of maternal CMV infections have nonspecific clinical manifestations, and most remain undetected unless specific serological testing is undertaken. The combination of serology tests for CMV-specific IgM, IgG and IgG avidity provide improved distinction between primary and secondary maternal infections. In pregnancies with confirmed primary maternal CMV infection, amniocentesis with CMV-PCR performed on amniotic fluid, undertaken after 21-22 weeks gestation, may determine whether maternofetal virus transmission has occurred. Ultrasound and, to a lesser extent, magnetic resonance imaging are valuable tools to assess fetal structural and growth abnormalities, although the absence of fetal abnormalities does not exclude fetal damage. Diagnosis of congenital CMV infection at birth or in the first 3 weeks of an infant's life is crucial, as this should prompt interventions for prevention of delayed-onset hearing loss and neurodevelopmental delay in affected infants. Prevention strategies should also target mothers because increased awareness and hygiene measures may reduce maternal infection. Recognition of the importance of CMV in pregnancy and in neonates is increasingly needed, particularly as therapeutic and preventive interventions expand for this serious problem.

Cochlear Implantation in Patients With Superficial Siderosis: Seven Cases and Systematic Review of the Literature.

To date, there have been less than 30 cases of cochlear implantation (CI) in patients with superficial siderosis (SS) reported in the literature. The primary objective of the current study is to evaluate CI outcomes in six additional patients (seven ears) with SS and sensorineural hearing loss (SNHL) and to perform a systematic review of the literature. Case series and systematic review of the literature. Two tertiary academic CI centers. All patients with SS who underwent CI between 2007 and 2014. Cochlear implantation. Pre- and post-implantation speech perception scores and durability of benefit. A total of seven ears (four males; median age 52 yr) with SS and SNHL met inclusion criteria. All patients developed progressive bilateral SNHL that was no longer amenable to conventional hearing aids. Additional presenting symptoms included vestibulopathy (n = 4), cerebellar ataxia (n = 3), mild dementia (n = 1), and myelopathy (n = 1). All patients underwent uncomplicated CI, and intraoperative device telemetry revealed normal responses in all electrodes. The median postoperative auditory threshold average was 32.5 dB HL (range 16-36 dB) and the median postoperative CNC word score was 51% (range 46-64%). The median duration of follow-up was 15.5 months (range 3-64 mo). All patients demonstrated initial improvement in speech perception testing. Two patients had performance decline and worsening dementia resulting from progressive SS. Cochlear implantation is a viable strategy for auditory rehabilitation in patients with SS and associated SNHL. Most individuals enjoy benefit from CI; however, patients should be counseled regarding the risks of performance decline with progressive SS.

Progressive Hearing Loss and Head Trauma in Enlarged Vestibular Aqueduct: A Systematic Review and Meta-analysis.

Enlarged vestibular aqueduct is the most common radiographically identified cause of congenital sensorineural hearing loss and is frequently progressive. Imaging is often ordered during the workup of children with congenital sensorineural hearing loss in part to identify enlarged vestibular aqueduct given concern for progression with head trauma. However, this association has not been systematically evaluated. We aimed to determine the rate of progression and association with head trauma in individuals with enlarged vestibular aqueduct. Systematic review of primary studies identified through PubMed, Embase, Cochrane, and Web of Science. Meta-analysis was performed on patient-level data describing enlarged vestibular aqueduct, progressive sensorineural hearing loss, and head trauma extracted from articles identified on systematic review according to PRISMA guidelines. Twenty-three studies (1115 ears with enlarged vestibular aqueduct) met inclusion criteria. Progressive sensorineural hearing loss was found in 39.6% of ears, with trauma-associated progression in 12%. Limited case-control data show no difference in the incidence of progression between patients with and without head trauma. Long-term progressive sensorineural hearing loss is common in enlarged vestibular aqueduct, but its association with head trauma is not strongly supported.

Whole Exome Sequencing Identified MCM2 as a Novel Causative Gene for Autosomal Dominant Nonsyndromic Deafness in a Chinese Family.

We report the genetic analysis of autosomal dominant, nonsyndromic, progressive sensorineural hearing loss in a Chinese family. Using whole exome sequencing, we identified a missense variant (c.130C>T, p.R44C) in the MCM2 gene, which has a pro-apoptosis effect and is involved in the initiation of eukaryotic genome replication. This missense variant is very likely to be the disease causing variant. It segregated with hearing loss in this pedigree, and was not found in the dbSNP database or databases of genomes and SNP in the Chinese population, in 76 patients with sporadic hearing loss, or in 145 normal individuals. We performed western blot and immunofluorescence to test the MCM2 protein expression in the cochlea of rats and guinea pigs, demonstrating that MCM2 was widely expressed in the cochlea and was also surprisingly expressed in the cytoplasm of terminally differentiated hair cells. We then transiently expressed the variant MCM2 cDNA in HEK293 cells, and found that these cells displayed a slight increase in apoptosis without any changes in proliferation or cell cycle, supporting the view that this variant is pathogenic. In summary, we have identified MCM2 as a novel gene responsible for nonsyndromic hearing loss of autosomal dominant inheritance in a Chinese family.

Reduced Cochlear Implant Performance After the Use of Growth Hormone With Regain of Function After Cessation of Growth Hormone Therapy

To assess whether recombinant growth factor (hGH) therapy has an effect on cochlear implant (CI) performance. Two pediatric CI recipients (S1, S2) who underwent treatment with hGH for short stature were identified for review. S1 has bilateral labyrinthine dysplasia and received implants at ages 10 months (right) and 4 years 3 months (left). S2 was diagnosed with severe to progressive sensorineural hearing loss bilaterally and received a CI at age 9 years 10 months (left). Case series. Cochlear implant, hGH, and speech perception data were collected. Phonetically Balanced Kindergarten (PBK) and Consonant Nucleus Consonant (CNC) word recognition scores were reviewed to assess auditory perception. Electrode impedances, threshold levels, and comfort levels were also reviewed. After 4 months of hGH, word recognition scores for S1 were observed to decrease from 90 to 72% (right) and were stable at 40% (left). Despite troubleshooting, performance continued to decline bilaterally to 52% (right) and 28% (left), and the decision was made to discontinue hGH. One month after cessation of hGH, word recognition scores began improving to 74% (right) and 68% (left). Word recognition scores for S2 were observed to have decreased from 92% the previous year to 82% after taking hGH for 2 months. Given both our previous experience with S1 and discussions with S2's parents, hGH was discontinued after 10 months of therapy. Two months after cessation of hGH, S2's word recognition had improved to 86% (left). Our case studies illustrate that implanted children undergoing treatment with hGH may experience a decrease in speech perception, which recovers after the cessation of treatment. Since hGH use has become more prevalent in recent years, it is important to inquire whether children undergoing, or who have undergone, implantation are receiving hGH so that they may be appropriately monitored.

Aspects of learning from the perspective of people with Alström syndrome

Purpose: The aim of the study was to explore aspects of learning, from a lifelong perspective, in individuals with Alström syndrome (AS). AS is an autosomal recessive disorder causing early blindness, progressive sensorineural hearing loss, cardiomyopathy, endocrine disorders, metabolic dysfunction, and abbreviated lifespan. Method: Eleven individuals with AS participated. The study had a qualitative explorative design, giving voice to the participants’ perspectives on their situation. Data were collected using semi-structured interviews, which were subjected to conventional (inductive) qualitative content analysis. Results: The analysis revealed in the participants a quest for independence and an image of themselves as capable people willing to learn, but in constant need of support to continue learning throughout their lives to be as independent as possible. Conclusion: Based on the levels of functioning, i.e. personal resources, revealed in the interviews, supervisors, caregivers, and teachers are encouraged to allow people with AS to be their own advocates, as they know best how, what, and with whom they learn, and what type of sensory material – tactile, auditory, visual, or a combination – is most helpful.Implications for RehabilitationIndividuals with AS strive for independence, and to be independent they need to continue to learn throughout their lives.Individuals with AS know best how they learn, and should be asked what modalities are the most effective for them.The tactile modality for learning will continue throughout life and should be emphasized early in the individual’s education and rehabilitation.

Identification of a novel truncation mutation of EYA4 in moderate degree hearing loss by targeted exome sequencing.

The EYA4 gene encodes a 640-amino-acid protein that serves as a transcription factor. This protein contains a highly conserved Eya domain (eya-HR) and a variable domain (eya-VR). Mutations of this gene are known to cause postlingual and progressive sensorineural hearing loss, either as non-syndromic (DFNA10) or syndromic hearing loss, depending on the location of truncation of the mutant protein. Since our previous report, we have recruited 14 families segregating autosomal dominant moderate SNHL. A thorough medical history and physical examination including evaluation of heart problems ruled out any syndromic features in these families. Screening of EYA4 was performed by targeted exome sequencing of 134 known deafness genes (TES-134) from the probands. After basic filtering of the variants, we identified one proband who carried a novel truncation mutation, c.1194delT (p.Met401TrpfsX3) of EYA4, making the frequency of DFNA10 to be 7.14 % (1/14) in Koreans. The variant co-segregated perfectly with a slightly down-sloping, moderate degree of SNHL in the family (SH117), and was not detected in any of the 592 normal control chromosomes. This variant is likely to generate protein products that are truncated just downstream of the eya-VR domain. None of the three affected family members showed any syndromic features, including cardiac problems, which was compatible with a previous genotype-phenotype correlation. The identification of a novel EYA4 truncation mutation associated with DFNA10, rather than syndromic hearing loss, supports a previously reported genotype-phenotype correlation in this gene. Considering its detection rate, EYA4 mutations should be suspected in hereditary moderate hearing loss with a corresponding audiologic configuration, and a cardiac examination should be included in the initial evaluation.

Exome Sequencing Identifies a Mutation in EYA4 as a Novel Cause of Autosomal Dominant Non-Syndromic Hearing Loss.

Autosomal dominant non-syndromic hearing loss is highly heterogeneous, and eyes absent 4 (EYA4) is a disease-causing gene. Most EYA4 mutations founded in the Eya-homologous region, however, no deafness causative missense mutation in variable region of EYA4 have previously been found. In this study, we identified a pathogenic missense mutation located in the variable region of the EYA4 gene for the first time in a four-generation Chinese family with 57 members. Whole-exome sequencing (WES) was performed on samples from one unaffected and two affected individuals to systematically search for deafness susceptibility genes, and the candidate mutations and the co-segregation of the phenotype were verified by polymerase chain reaction amplification and by Sanger sequencing in all of the family members. Then, we identified a novel EYA4 mutation in exon 8, c.511G>C; p.G171R, which segregated with postlingual and progressive autosomal dominant sensorineural hearing loss (SNHL). This report is the first to describe a missense mutation in the variable region domain of the EYA4 gene, which is not highly conserved in many species, indicating that the potential unconserved role of 171G>R in human EYA4 function is extremely important.

Novel PTPRQ mutations identified in three congenital hearing loss patients with various types of hearing loss.

We present 3 patients with congenital sensorineural hearing loss (SNHL) caused by novel PTPRQ mutations, including clinical manifestations and phenotypic features. Two hundred twenty (220) Japanese subjects with SNHL from unrelated and nonconsanguineous families were enrolled in the study. Targeted genomic enrichment with massively parallel DNA sequencing of all known nonsyndromic hearing loss genes was performed to identify the genetic cause of hearing loss. Four novel causative PTPRQ mutations were identified in 3 cases. Case 1 had progressive profound SNHL with a homozygous nonsense mutation. Case 2 had nonprogressive profound SNHL with a compound heterozygous mutation (nonsense and missense mutation). Case 3 had nonprogressive moderate SNHL with a compound heterozygous mutation (missense and splice site mutation). Caloric test and vestibular evoked myogenic potential (VEMP) test showed vestibular dysfunction in Case 1. Hearing loss levels and progression among the present cases were varied, and there seem to be no obvious correlations between genotypes and the phenotypic features of their hearing loss. The PTPRQ mutations appeared to be responsible for vestibular dysfunction.

Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss

Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve–brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention.

CHARGE syndrome and Cochlear implantation: Difficulties and outcomes in the paediatric population

ObjectivesCHARGE syndrome is a complex cluster of congenital abnormalities, these children may have absent or hypoplastic auditory nerves. Our objective was to assess preoperative factors and outcomes for paediatric cochlear implant recipients with CHARGE syndrome, to enable better surgical preparation and family counselling. MethodsThe Sydney Cochlear Implant Centre database was searched for children with CHARGE syndrome who had received a cochlear implant at ages 16 and less. Data were collected regarding clinical history; hearing assessments; MRI and CT scan findings; preoperative transtympanic electrical Auditory Brainstem Response (ABR); intraoperative findings and intraoperative electrical ABR and Neural Response Telemetry; and language outcomes in terms of main language used and Categories of Auditory Performance scores (0–7 ranking). ResultsTen children were identified. All seven prelingual profoundly deaf children with CHARGE syndrome had hypoplastic or absent auditory nerves bilaterally on MRI scans. Middle ear anatomy was often abnormal, affecting surgical landmarks and making identification of the cochlea very difficult in some cases. Three cases required repeated surgery to obtain successful cochlear implant insertion, one under CT scan image guided technique. All seven children used sign language, or simpler gestures, as their main mode of communication. Two children of of these children, who were implanted early, also attained some spoken language. CAP scores ranged from 0 to 6.The three children with CHARGE syndrome and progressive sensorineural hearing loss had a normal auditory nerve in at least one ear on MRI scans. All had preoperative verbal language, with CAP scores of 6, and continued with CAP scores of 6 following receipt of the cochlear implant. ConclusionChildren with CHARGE and congenital profound hearing loss all had hypoplasia or absent auditory nerves, affecting their outcomes with cochlear implants. They often had markedly abnormal middle ear anatomy and CT image guided surgery can be helpful. These children should be offered a bilingual early intervention approach, using sign language and verbal language, to ensure best language outcomes. Children with CHARGE syndrome and progressive profound hearing loss did well with cochlear implants and continue to be able to use verbal language.

Clinical characteristics and outcomes of intratemporal facial nerve neurofibromas

This study aimed to present clinical features and outcomes of 10 patients with intratemporal facial nerve neurofibromas. The ten patients underwent complete tumor removal, and nerve grafting was performed on 3 cases whose nerve integrity was sacrificed. They were followed up after surgery. All patients firstly complained of facial palsy, and progressive sensorineural hearing loss was also present in one case. Insidious onset of facial palsy was found in 6 of 10 cases (60.0%). There was multi-segment involvement in 5 of 10 cases (50.0%). During the follow-up of 4.2±1.5 ys (range, 2-6 ys), 1 of 6 cases who successfully maintained nerve integrity recovered to Grade III, and the others remained or recovered to Grade V or even Grade VI. In contrast, all of the 3 cases with nerve grafting recovered from Grade VI to Grade III or Grade IV. No tumor recurrence was noted. Facial nerve neurofibromas mainly presented with insidious facial palsy, and tended to affect multiple segments of facial nerve. It seemed that complete tumor removal and nerve grafting had better outcomes of facial nerve compared to complete tumor removal with nerve integrity preserved.