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Introduction: The use of surgical decompression of facial hemispasm due to the loop in the internal auditory canal is not always accepted due to the risk related to the surgical procedure. Currently a new surgical technique allows surgeons to work in safer conditions. Objective: To report the results with endoscope-assisted retrosigmoid approach for facial nerve microvascular decompression in hemifacial spasm due to neurovascular conflict. The surgical technique is described. Methods: We carried out a prospective study in a tertiary referral center observing 12 (5 male, 7 female) patients, mean age 57.5 years (range 49–71) affected by hemifacial spasm, that underwent to an endoscope assisted retrosigmoid approach for microvascular decompression. We evaluated intra-operative findings, postoperative HFS resolution and complication rates. Results: Hemifacial spasm resolution was noticed in 9/12 (75%) cases within 24 h after surgery and in 12/12 (100%) subjects within 45 days. A significant (p

OBJECTIVES: The aim of the present study was to investigate current treatment practices and self-reported effectiveness in Meniere’s disease. MATERIALS and METHODS: Members of two Italian Meniere’s disease support (n=170) with ≥6-month history of Meniere’s disease were administered an online survey about recent treatments. Vertigo episode count, work absenteeism, and limitations in family life, social life, work, or travel as included in the Social Life and Work Impact of Dizziness Questionnaire before and after recent treatments were queried. RESULTS: Twenty-four different treatments were reported for Meniere’s disease, with dietary modifications (55%), diuretics (47%), and betahistine (41%) being the most common. The majority (71%) received multiple simultaneous treatments. Prior to the most recent treatments, 78%-89% of respondents indicated limitations in family or social life, work, or traveling. After their most recent treatment, respondents reported improvements in mean vertigo episode counts (5.7±7.6 vs. 2.6±4.6, p

Myringoplasty is a frequently performed procedure in children, with a heterogeneous failure rate. Our study aimed to evaluate the outcome of myringoplasty in a pediatric hospital and to identify which are risk factors for reperforation or poor hearing improvement after surgery.Preoperative and intraoperative variables between pediatric patients who had undergone myringoplasty with an intact tympanic membrane at follow-up and the cases with reperforation were compared. The same factors were investigated as potential predictors of audiological success. Pre and postoperative PTA and ABG were compared in the whole population, in structural success and failure groups and closure of ABG was calculated and used to compare the audiological outcomes between the two groups.Parameters that affected the postoperative integrity of TM were age, the time between diagnosis and surgery, the intraoperative status of the middle ear, and secondhand smoke exposure. Early perforations occurred mostly after surgeries performed by trainees, while late perforations were more frequently in autumn. Myringoplasty, regardless of the structural outcome, can improve the ABG and PTA. No preoperative and intraoperative parameters affected the audiological outcome.Pediatric myringoplasty is a safe and successful procedure that can improve hearing, regardless of the structural outcome. In light of our results, parameters to consider before surgery are age, the time between diagnosis and surgery, the intraoperative status of the middle ear, and secondhand smoke exposure.

Introduction: Arachnoid cyst in the internal auditory canal is a quite rare pathology but due to its compressive action on the nerves in this district should be surgically removed. Several surgical techniques have been proposed but no surgeons have used the minimally assisted endoscope retrosigmoid approach for its removal. Objective: To investigate the feasibility of using a minimally invasive endoscope assisted retro-sigmoid approach for surgical removal of arachnoid cysts in the internal auditory canal. Methods: Minimally invasive endoscope assisted retrosigmoid approach allows to access to the internal auditory canal through a minimally invasive retrosigmoid approach that combines the use of a microscope and an endoscope. It is performed in six steps: soft tissue step, bone step, dura step, cerebellopontine angle step (performed using an endoscope and a microscope), microscope-endoscope assisted arachnoid cysts removal and closure. We tested minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal on two human cadaveric heads (specimens) of subjects affected from audio-vestibular disorders and with arachnoid cysts in the internal auditory canal confirmed by magnetic resonance imaging. Results: The mass was completely and successfully removed from the two specimens with no damage to the nerves and/or vessels in the surgical area. Conclusion: The results of our study are encouraging and support the feasibility of using minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal. While further clinical in-vivo studies are needed to confirm the accuracy and safety of using the minimally invasive endoscope assisted retrosigmoid approach for this specific surgery, our group has successfully used the minimally invasive endoscope assisted retrosigmoid approach in the treatment of microvascular compressive syndrome, schwannoma removal and vestibular nerve resection. Resumo Introdução: O cisto aracnóide no conduto auditivo interno é uma doença bastante rara, mas, devido à sua ação compressiva sobre os nervos deste local, ele deve ser removido cirurgicamente. Várias técnicas cirúrgicas foram propostas, mas ninguém utilizou a abordagem retrosigmoide minimamente invasiva assistida por endoscopia para a sua remoção. Objetivo: Investigar a viabilidade do uso da abordagem retrosigmoide minimamente invasiva assistida por endoscopia para remoção cirúrgica de cistos aracnóides no conduto auditivo interno. Método: A abordagem retrosigmoide minimamente invasiva assistida por endoscopia permite o acesso ao conduto auditivo interno através de uma abordagem retrosigmóide minimamente invasiva que combina o uso de um microscópio e um endoscópio. É realizada em seis etapas: do tecido mole, óssea, dura-máter, do ângulo pontocerebelar (realizado com um endoscópio e um microscópio), remoção e fechamento assistidos por endoscópio-microscópico. Testamos a abordagem retrosigmoide minimamente invasiva assistida por endoscopia para remoção de cistos aracnóides no conduto auditivo interno em duas cabeças de cadáveres humanos (espécimes) de indivíduos afetados por distúrbios auditivos-vestibulares e com cistos aracnóides no conduto auditivo interno confirmado por imagem de ressonância magnética. Resultados: A lesão foi removida completamente e com sucesso nos dois espécimes sem dano aos nervos e/ou vasos na área cirúrgica. Conclusão: Os resultados do nosso estudo são encorajadores e apoiam a viabilidade do uso da abordagem retrosigmoide minimamente invasiva assistida por endoscopia para remoção de cistos aracnóides no conduto auditivo interno. Embora mais estudos clínicos in vivo sejam necessários para confirmar a precisão e a segurança do uso da abordagem retrosigmoide minimamente invasiva assistida por endoscopia para essa cirurgia específica, nosso grupo utilizou com sucesso a abordagem retrosigmoide minimamente invasiva assistida por endoscopia no tratamento da síndrome compressiva microvascular, remoção de schwannoma e ressecção do nervo vestibular.

To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients’ demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Es werden die diagnostischen Kriterien fur M. Meniere beschrieben, die vom Klassifikationskomitee der Barany-Gesellschaft, der Japanischen Gesellschaft fur Gleichgewichtsforschung, der Europaischen Akademie fur Otologie und Neurootologie (EAONO), dem Komitee fur Gleichgewichtsstorungen der Amerikanischen Akademie fur Otolaryngologie, Kopf- und Hals-Chirurgie (AAO-HNS) sowie der Koreanischen Gesellschaft fur Gleichgewichtsstorungen erarbeitet worden sind. Diese Klassifikation enthalt 2 diagnostische Kategorien: M. Meniere und wahrscheinlicher M. Meniere. Die Diagnose eines definitiven M. Meniere basiert auf klinischen Kriterien und erfordert rezidivierende episodische Schwindelsymptome, die mit einer sensorineuralen Horminderung im niedrigen bis mittleren Frequenzbereich assoziiert sein mussen sowie mit fluktuierenden Symptomen (Horminderung, Tinnitus und/oder Vollegefuhl) im betroffenen Ohr. Die Dauer der Attacken liegt zwischen 20 min und 12 h. Ein wahrscheinlicher M. Meniere ist definiert als episodische Schwindelsymptome (Dreh- oder Schwankschwindel), assoziiert mit fluktuierenden Ohrsymptomen und einer Dauer von 20 min bis 24 h.

The European Academy of Otology and Neurotology (EAONO) has previously published a consensus document on the definitions and classification of cholesteatoma. It was based on the Delphi consensus methodology involving the broad EAONO membership. At the same time, the Japanese Otological Society (JOS) had been working independently on the "Classification and Staging of Cholesteatoma." EAONO and JOS then decided to collaborate and produce a joint consensus document. The EAONO/JOS joint consensus on "Definitions, Classification and Staging of Middle Ear Cholesteatoma" was formally presented at the 10th International Conference on Cholesteatoma and Ear Surgery in Edinburgh, June 5-8, 2016. The international otology community who attended the consensus session was given the chance to debate and give their support or disapproval. The statements on the "Definitions of Cholesteatoma" received 89% approval. The "Classification of Cholesteatoma" received almost universal approval (98%). The "EAONO/JOS Staging System on Middle Ear Cholesteatoma" had a majority of approval (75%). Some international otologists wanted to see more prognostic factors being incorporated in the staging system. In response to this, the EAONO/JOS steering group plans to set up an "International Otology Outcome Working Group" to work on a minimum common otology data set that the international otology community can use to evaluate their surgical outcome. This will generate a large database and help identify relevant prognostic factors that can be incorporated into the staging system in future revisions.

Children hospitalized in Neonatal Intensive Care Units (NICU) present an increased risk for Sensorineural Hearing Loss (SNHL) due to prematurity, hypoxia-ischemia, hyperventilation, low birth weight and the use of ototoxic drugs. The aim of this study was to assess the prevalence of SNHL in newborns hospitalized in a NICU using Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Responses (A-ABR) and analyze the associated risk factors. A sample of 153 newborns hospitalized in NICU underwent TEOAE, A-ABR and clinical ABR to evaluate the presence of hearing deficits. Prevalence of SNHL was calculated and odds ratio for specific risk factors was measured. One-hundred fifteen babies (86.7%) presented normal hearing at TEOAE and A-ABR. Fifteen children had a REFER response at TEOAE and a PASS response at A-ABR. Twenty-five children (16.3%) had a REFER A-ABR and were addressed to clinical ABR. A diagnosis of SNHL was made in 12 (7.8%) newborns. An increased risk of SNHL was observed in preterm children

Objectives This study aims to compare the electrical auditory brainstem response (EABR) following cochlear implant (CI) surgery in pediatric subjects with cochlear malformation and a normal cochlea, in order to assess the sensitivity of EABR and to evaluate the surgery outcome. Materials and methods A total of 26 pediatric subjects who were deaf and scheduled for CI surgery were enrolled into this case control study. Group A (n=20) included subjects with a normo-conformed cochlea. Group B (n=6) included subjects with cochlear malformation. Subjects were evaluated with EABR immediately (T0) and 6 months (T1) post-CI surgery. The EABR Waves III and V average amplitude and latency were compared across time, separately for each group, and across groups, separately for each time. Results Auditory brainstem response (ABR) could only be recorded in Group A. We were able to record EABR from all subjects at T0 and T1, and waves III and V were present in all the recorded signals. There were no statistically significant differences between T0 and T1 in EABR Waves III and V in terms of average amplitude and latency in neither group. When comparing Groups A and B, the only statistically significant difference was the average amplitude of wave V, both at T0 and T1. Conclusion EABR is a valid tool to measure the auditory nerve integrity after CI surgery in patients with a normal and malformed cochlea, as shown by its ability to measure waves III and V when ABR is absent. The EABR testing should be performed before and after CI surgery, and EABR should be used as a measure of outcome, especially in patients with a malformed cochlea.

This case describes the strong utility of optical coherence tomography in multidisciplinary management of a complex case of type 2 neurofibromatosis.

In uncooperative patients, electrical compound action potential (ECAP) thresholds are reliable in predicting T-levels, but are not in determining the C-level profile. The present study aims to assess if the C-level profile can be predicted by a new objective procedure (C-NRT) which uses the amplitude growth function (AGF) and is based on the assumption that equal ECAP amplitudes elicit equal loudness percepts. This is a correlational study conducted in five tertiary care referral hospitals with 21 post-lingually deaf adult cochlear implant users. Two maps were created: a behavioral, bitonal balanced (BB) map and an objective map, in which T-levels were the same as in the BB map, and C-levels were obtained with C-NRT. C-NRT consisted of performing the AGF of nine electrodes, and of setting the current level eliciting a 100 μV ECAP amplitude as C-level in the map. AutoNRT was also measured. Main outcome measures were correlation between behavioral C-profile level, objective C-profile level, behavioral T-profile level and objective T-profile (AutoNRT) level; disyllabic word recognition scores in quiet and in noise conditions (SNR = + 10 and 0) with both maps. A strong correlation was found between behavioral and C-NRT-derived C-levels (mean per electrode correlation: R = 0.862, p

Purpose The aim of the present study was to assess if the combined therapy of intratympanic dexamethasone (ITD) and high dosage of betahistine (HDBH) is able to provide increased vertigo control compared to ITD alone in patients suffering from definite unilateral Meniere’s disease (MD). Materials and methods Consecutive MD patients were enrolled and randomly divided in two groups, each comprising 33 cases. Group A received a combination of ITD and identical-appearing placebo pills while Group B received a combination of ITD and HDBH. ITD protocol consisted of three consecutive daily injections. HDBH comprised 144 mg/day (48 mg tid). The main outcome measures were: 1) vertigo class, pure tone average (PTA), speech discrimination score (SDS) and Functional Level Score (FLS) according to the American Academy of Otolaryngology-Head and Neck Surgery criteria; 2) complete and substantial vertigo control according to the Kaplan–Meier survival method. Results Sixty two patients completed the 24-month follow-up. A complete vertigo control was achieved in 14 patients (44%) from Group A and in 22 patients (73.3%) from Group B, statistically significant (p = 0.01). Complete vertigo relief is also significant according to the Kaplan–Meier method: p = 0.027, log rank test. Substantial vertigo control was obtained in 21 patients (65.6%) in Group A and 27 patients (90%) in Group B. The difference is statistically significant, p = 0.02. The difference is significant according to the Kaplan–Meier method: p = 0.035, log rank test. No significant differences between hearing levels and tinnitus scores were demonstrated between the groups. Conclusions Our preliminary results demonstrate that complete and substantial vertigo control is significantly higher in patients treated with a combination of HDBH and ITD.

This paper presents diagnostic criteria for Meniere's disease jointly formulated by the Classification Committee of the Barany Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes 2 categories: definite Meniere's disease and probable Meniere's disease. The diagnosis of definite Meniere's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low-to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 min and 12h. Probable Meniere's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 min to 24h.

Meniere Disease keeps challenges in its diagnosis and treatment since was defined by Prosper Meniere at the beginning of 19th Century. Several classifications and definition were made until now and speculations still exist on its etiology. As the etiology remains speculative the treatment models remain in discussion also. The European Academy of Otology and Neurotology Vertigo Guidelines Study Group intended to work on the diagnosis and treatment of Meniere's disease and created the European Positional Statement Document also by resuming the consensus studies on it. The new techniques on diagnosis are emphasized as well as the treatment models for each stage of the disease are clarified by disregarding the dilemmas on its treatment. The conservative, noninvasive and invasive therapeutic models are highlighted.

OBJECTIVES: Spiral ganglion (SG) counting is used in experimental studies conducted on age-, noise-, and drug-induced sensorineural hearing loss, as well as in the assessment of cochlear implant performances. Different methods of counting have been reported, but no definite standardization of such procedure has been published. The aim of our study is to identify the best method to count human spiral ganglions (SGs). MATERIALS AND METHODS: By identification of nuclei or nucleoli as described by Schucknect, seven researchers with different experience levels counted SGs in 123 human temporal bones (TBs). Data on time of post-mortem bone removal post-mortem, methods of specimen’s fixation, decalcification, and coloration were collected to test their possible influence on human tissue. Percentage, two-tailed t-test, Spearman’s test, and one-way ANOVA were used to analyze the data. RESULTS: Nucleoli were identified in 61% of cases, whereas nuclei were recognized in 100% of cases (p

The importance of a good hearing function to preserve memory and cognitive abilities has been shown in the adult population, but studies on the pediatric population are currently lacking. This study aims at evaluating the effects of a bone-anchored hearing implant (BAHI) on speech perception, speech processing, and memory abilities in children with single side deafness (SSD). We enrolled n = 25 children with SSD and assessed them prior to BAHI implantation, and at 1-month and 3-month follow-ups after BAHI implantation using tests of perception in silence and perception in phonemic confusion, dictation in silence and noise, and working memory and short-term memory function in conditions of silence and noise. We also enrolled and evaluated n = 15 children with normal hearing. We found a statistically significant difference in performance between healthy children and children with SSD before BAHI implantation in the scores of all tests. After 3 months from BAHI implantation, the per­formance of children with SSD was comparable to that of healthy subjects as assessed by tests of speech perception, working memory, and short-term memory function in silence condition, while differences persisted in the scores of the dictation test (both in silence and noise conditions) and of the working memory function test in noise condition. Our data suggest that in children with SSD BAHI improves speech perception and memory. Speech rehabilitation may be necessary to further improve speech processing.

Objective The aim of this study is to evaluate the effects of an intratympanic gentamicin-dexamethasone combination on the inner ear. Materials and methods Twenty-six Wistar albino rats were divided into four groups: Group I (Control), group II (Intratympanic dexamethasone; ITD), group III (Intratympanic gentamicin; ITG), and group IV (Intratympanic gentamicin and dexamethasone; ITGD). On the first day after basal auditory brainstem response (ABR) measurements, the ITG group received 0.03 mL of intratympanic gentamicin (26.7 mg/mL). Intratympanic injection of 0.06 mL of a solution containing 13.35 mg/mL gentamicin and 2 mg/mL dexamethasone was performed in the ITGD group. 0.03 mL of physiological intratympanic serum and dexamethasone (4 mg/mL) was applied in control and ITD groups, respectively. On the 7th day, ABR measurements were repeated and vestibular functions were evaluated. On the 21th day, ABR and vestibular tests were repeated, and the animals were sacrificed for histopathological investigation. Results The ITG group's hearing thresholds deteriorated in all frequencies. The ITGD group's hearing thresholds were significantly better than the ITG group, except at 8 kHz on the 7th day and in all frequencies at the 21th day measurements. The vestibular function scores of the ITG and ITGD groups were higher than the controls. Apoptotic changes were seen in cochlea, spiral ganglion, and vestibule of the ITG group. Cochlear and vestibular structures were well preserved in the ITGD group, similar to the controls. Conclusion The ITGD combination led to a significant hearing preservation. Although in subjective vestibular tests, it seemed that vestibulotoxicity was present in both ITG and ITGD groups the histopathological investigations revealed no signs of vestibulotoxicity in the ITGD group in contrast to the ITG group. Further studies using a combination of different concentrations of gentamicin and dexamethasone are needed.

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed.

Background: The main advantage of using the KTP (potassium-titanyl-phosphate) laser for stapedotomy instead of the conventional micropick instrument is the smaller risk for mechanical damage. However, the KTP laser could theoretically inflict damage to inner ear structures. We hypothesize that KTP laser light [wavelength (λ) = 532 nm] is hardly absorbed in perilymph but well absorbed in solid structures. The aim of this pilot study was to assess if damage occurred after KTP laser cochleostomy in an animal model and, if so, to what extent and at which settings. Materials and Methods: In six guinea pigs, a KTP laser cochleostomy at the basal turn was created. Laser settings of 1, 3 and 5 W and 100 ms pulse time (n = 2 each) were used. Histological preparations were studied for damage to neuroendothelial cells and intrascalar blood. Results: No damage to inner ear neuroendothelial cells was observed, even at the highest power. Blood clots in the scala tympani from vessels in the cochlear wall were seen. The effects were minimal in the lowest, currently clinically used settings. Conclusion: KTP laser cochleostomy gives no damage to inner ear neuroendothelial cells but may cause intrascalar hemorrhages.

Objectives/hypothesis An increasing number of patients with low-frequency residual hearing are fitted with a cochlear implant. The challenge is to optimize cochlear implant device properties and develop atraumatic surgical techniques to preserve residual hearing. In view of the ongoing debate about the optimal procedure for opening the cochlea during cochlear implantation, we reviewed the evidence on the round window and the cochleostomy insertion techniques and compared their effects on postoperative residual hearing. Design Systematic review. Methods Electronic databases were systematically searched for relevant studies published up to January 2012. All studies reporting on residual hearing and hearing preservation surgery were included. Results Sixteen studies, with a total of 170 patients, were included. There were no studies directly comparing both surgical insertion techniques. The methodologic quality of the studies was poor and might be subjected to a high risk of bias. Because there were no studies directly comparing the 2 techniques and controlling for possible influencing factors, differences between studies might also be influenced by intersurgeon variance in many facets regarding cochlear implantation surgery. The available data show a postoperative low-frequency hearing loss ranging from 10 to 30 dB at 125, 250, and 500 Hz, regardless of surgical technique. The number of patients with a postoperative complete hearing preservation ranged from 0% to 40% for the cochleostomy group and from 13% to 59% in the round window group. Conclusion The available data do not show that there is a benefit of one surgical approach over the other regarding the preservation of residual hearing. To provide solid evidence, a double-blind randomized trial is needed, which compares the clinical outcomes, notably the degree of hearing preservation, of both surgical approaches.

Objectives/Hypothesis The need for class I and II studies on the efficacy of liberatory maneuvers in the treatment of lateral canal benign paroxysmal positional vertigo (LC-BPPV) motivated the present double-blind randomized trial on the short-term efficacy of the Gufoni liberatory maneuver (GLM). Study Design Double-blind randomized controlled trial. Methods Seventy-two patients with unilateral LC-BPPV were recruited for a multicentric study. Patients were randomly assigned to treatment by GLM (n = 37) or sham treatment (n = 35). Subjects were followed up twice (at 1 hour and 24 hours) with the supine roll test by blinded examiners. Results At 1- and 24-hour follow-up, 75.7% and 83.8% of patients, respectively, undergoing GLM had recovered from vertigo, compared to around 10% of patients undergoing the sham maneuver (P < 0.0001). Conclusion To the best of our knowledge, this is the first class I study on the efficacy of the GLM in the treatment of LC-BPPV in both geotropic and apogeotropic forms. GLM proved highly effective compared to the sham maneuver (P < 0.0001). The present class I study of the efficacy of the GLM changes the level of recommendation of the method for treating LC-BPPV from level U to level B for the geotropic variant and from level B to level A for the apogeotropic variant of LC-BPPV. Level of Evidence 1b. Laryngoscope, 2013

The usefulness of cortical mastoidectomy in myringoplasty remains an issue of controversy. We aimed to assess the effectiveness of mastoidectomy on outcomes of tympanoplasty performed in patients with persistent or intermittent discharging chronic suppurative otitis media without cholesteatoma.Prospective, randomized, single-blinded study. Level of evidence-1B.Tertiary referral center.Three hundred twenty consecutive adult patients presenting persistent or intermittent otorrhea during the preceding 6 months scheduled for myringoplasty were included.Patients were randomly assigned to undergo either myringoplasty with cortical mastoidectomy (Group A) or myringoplasty only (Group B).Graft success rate and mean postoperative air-bone gap. Auditory outcomes were evaluated at one year postoperatively.At the end of the follow-up period, there were 24 failures within Group A (success rate, 82.8%) and 34 within Group B (success rate, 76%), statistically not significant. In the univariate analysis, 3 factors were found to be significant in predicting success rate: healthy opposite ear, a long dry period preceding the operation and nonsmoker status. The only factor attaining significance in the multivariate analysis was a dry period longer than 3 months.Cortical mastoidectomy offers no additional benefit in myringoplasty performed on patients with persistent or intermittent discharging CSOM and no evidence of cholesteatoma or mucosal blockage within the antrum.

This paper presents diagnostic criteria for Menière's disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes 2 categories: definite Menière's disease and probable Menière's disease. The diagnosis of definite Menière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low-to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 min and 12h. Probable Menière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 min to 24h.

This paper presents diagnostic criteria for Menière's disease jointly formulated by the Classification Committee of the Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO), the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean Balance Society. The classification includes two categories: definite Menière's disease and probable Menière's disease. The diagnosis of definite Menière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 minutes and 12 hours. Probable Menière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms occurring in a period from 20 minutes to 24 hours.

This is a study of Subjective Visual Vertical (SVV) perception during acute attacks of Ménière's disease (MD) with comparative evaluation of concurrent nystagmus. We studied 21 patients with unilateral MD during the acute phase and 7 days later. Of the nine patients with an initial attack of MD, seven had an alteration of SVV perception and of these, three indicated a match with canal functional signs, while four patients showed an opposite trend of SVV perception relative to the spontaneous nystagmus. Nine of the 12 patients with definite MD had a pathological SVV perception always in correspondence with the same type of canal event. At 1-week control, no patient with an initial MD attack had alteration of SVV perception, whereas 5 patients with definite MD presented a pathological SVV perception toward the affected side. In the course of acute attacks of unilateral MD, clinical manifestations may include otolithic involvement and this may have an opposite trend compared to concomitant canal signs, especially during initial attacks. This behavior allows us to distinguish clinical signs of maculo-canal "correspondence" and "dissociation" with a significant prevalence of the second indication in those subjects with an initial MD attack.

To present a series of temporal en plaque meningiomas involving the middle ear or mastoid, whose main symptoms suggested a serous otitis media.Multicentric retrospective study reviewing clinical records originating from eight tertiary referral centers.The clinical records of 10 patients presenting with signs and symptoms suggesting serous otitis media and whose neuroimaging studies revealed a temporal en plaque meningioma involving the middle ear or mastoid are reported.All the patients were women, ranging from 49 to 71 years old. The delay between the onset of symptoms and the diagnosis of meningioma varied from 1 to 10 years. All the patients underwent various procedures usually applied for the treatment of serous otitis media, which failed in all the cases, particularly ventilating tube placement, which was followed by severe episodes of discharge. In all cases, the computed tomographic scans showed three imaging signs: soft tissue mass filling the middle ear or mastoid, hyperostosis of the petrous bone, and hairy aspect of the intracranial margins of the affected bone. This imaging triad must alert the otologist of the possibility of intracranial meningioma. Magnetic resonance imaging was the method of choice to assess the diagnosis of intracranial meningioma involving the middle ear or mastoid. When analyzing management options, it appeared that conventional middle ear procedures were inefficient.Temporal en plaque meningioma involving the middle ear or mastoid can mimic a serous otitis media. A computed tomographic scan is recommended for cases of atypical or prolonged unilateral serous otitis media to investigate indirect signs of a meningioma, which has to be confirmed with magnetic resonance imaging.

Eustachian tube (ET) primary tumors and tumor-like lesions are rare diseases presenting with common ear, nose and throat symptoms. Pathology can range from developmental anomalies to high malignant neoplasms. Hence this review aimed at suggesting a classification and outline relevant aspects of ET primary tumors and tumor-like lesions, describing clinical findings, diagnostic management and therapeutic approaches. MEDLINE, CINAHL, OVIDSP, HIGHWIRE, and GOOGLE databases were searched from inception to July 2011 for relevant studies. Further papers were identified by examining the reference lists of all included. Sixty-five papers met the inclusion criteria, enclosing 78 cases. Case reports are increasing in the past few years. Benign lesions and tumor-like lesions of ET have been reported. Moreover, melanomas, carcinomas, and sarcomas can affect the ET as a primary site.

This study aims to compare the outcomes of patients with Meniere’s disease submitted to either endolymphatic mastoid shunt (ES) or tenotomy of the stapedius and tensor tympani muscles (TSTM). This is a retrospective chart review of patients treated with ES or TSTM between 2000 and 2010 and followed up for at least 12 months. The main outcomes were represented by: (1) vertigo class, hearing stage and functional level according to the American Academy of Otolaryngology-Head and Neck Surgery criteria; (2) adjustment of dizziness handicap inventory (DHI) and (3) complete and substantial vertigo control using the Kaplan–Meier survival method. Sixty-three patients met the inclusion criteria: 34 underwent ES and 29 TSTM. The baseline demographic characteristics, the hearing stage, the functional level, the DHI and hearing levels were not different between the two groups. No significant difference in vertigo class was demonstrated: 66 % of TSTM patients attained class A compared to 44 % in the ES group (p = 0.14). Kaplan–Meier survival curves specific to class A showed significant differences, favoring TSTM (log-rank test, p = 0.022). TSTM patients demonstrated significantly improved functional level (p = 0.0004) and improved DHI scores (p = 0.001). Eight ES patients (25 %) demanded a second surgical attempt compared to none in the TSTM. Aural fullness was significantly improved in TSTM group (p = 0.01), while the difference in tinnitus improvement was non-significant. Hearing preservation was significantly better in TSTM group (p = 0.001). TSTM is a safe surgical procedure, with significant vertigo control rates, and important hearing preservation rates. More patients and longer follow-up are needed to support our preliminary findings.

Purpose The objective of our randomized, double-blind study was to compare the effectiveness of intratympanic (IT) dexamethasone versus high-dosage of betahistine in the treatment of patients with intractable unilateral Meniere disease (MD). Materials and methods Sixty six patients with definite unilateral MD were randomly divided in two groups: Group A received a combination of IT dexamethasone (DX) and identical-appearing placebo pills while Group B received a combination of high-dosage betahistine and IT saline. Intratympanic injections were repeated for three times with an interlude of 3 days. High-dosage of betahistine entailed 144 mg/day. Mean outcome measures consisted of vertigo control, pure tone average (PTA), speech discrimination score, Functional Level Score, Dizziness Handicap Inventory and Tinnitus Handicap Inventory. Results Fifty nine patients completed the study and were available at 12 months for analysis. In Group A complete vertigo control (class A) was attained in 14 patients (46.6%) and substantial control (class B) in 7 patients (20%). In Group B, 12 patients (41%) achieved complete vertigo control (class A), 5 patients (17%) substantial control (class B). There is no statistical difference in vertigo control between the two treatment groups. In Group A hearing was unchanged in 14 patients and improved in 4 patients, while in Group B hearing was unchanged in 16 patients and improved in 2 patients. Conclusions Our preliminary results demonstrate that high-dosage of betahistine achieved similar outcomes as IT dexamethasone in the control of vertigo and hearing preservation.

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with "CHARGE syndrome" submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy-posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid's audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.

The aim of the study was to evaluate the long-term results after surgery for acquired cholesteatoma in children and to contribute to the search for predictors of recurrence. During a 15-year period, 114 children underwent surgery. The patients were re-evaluated with a median observation time of 5.8 years. At the last re-evaluation 85 per cent of the ears were dry with an intact drum. Recurrence of cholesteatoma developed in 27 ears. The cumulated total recurrence rate was 24 per cent using the incidence rate calculation, applying Kaplan-Meier survival analysis the corresponding recurrence was 33 per cent. Recurrent disease occurred significantly more frequently in children younger than eight years, with a negative pre-operative Valsalva, with ossicular resorption and with large cholesteatomas. In conclusion, young children with poor Eustachian tube function and a large cholesteatoma with erosion of the ossicular chain, are at special risk of recurrence and should be observed for several years after surgery.

Objective: To achieve a European consensus about the bestV evidence informedVmanagement of patients with common otologicrelated problems. Design: Consensus process, supported by the European Academy of Otology & Neuro-Otology. Setting: The European Academy of Otology & Neuro-Otology. Methods: A guideline of a specific disease should include evidence-informed recommendations about questions relating to treatment, prognosis, and diagnosis. A standardized method to design guideline questions to search for evidence and to grade the quality of available evidence results in an overview of the best available literature. Experts achieve a consensus on best practice and articulate recommendations on evidence-based actions to be taken in patient care accordingly. The assets of such evidence-based consensus usually are the opinion of experts and arguments on availability, transferability, applicability, and affordability. Discussion: The volume of new information increases at a staggering pace. As a result, there is an increasing demand for consistent systematic management of the available evidence. Systematic filtering of available evidence will help clinicians to find and apply best available and latest evidence efficiently and quickly. Conclusion: Despite the otologic line of approach, the present article provides a step-by-step ‘‘guideline development instruction manual,’’ which could be used within other medical specialties.

A woman in her 60s was evaluated in the neurosurgery intensive care unit for purulent discharge from her right ear. She had undergone an emergency craniotomy for brain decompression the day before. She was receiving intravenous antibiotics and steroids and was sedated and mechanically ventilated. Computed tomography (CT) of the temporal bone detected middle ear, mastoid, and infralabyrinthine involvement (Figure, A). Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain demonstrated meningitis and a large cerebellar lesion with rim enhancement. Moreover, diffusionweighted imaging (DWI) images and apparent diffusion coefficient (ADC) maps were consistent with cerebellar abscess (Figure, B-D). Axial CT of the head A Axial T1-weighted MRI B

The first case of bilateral orbital preseptal cellulitis complicating combined adenotonsillectomy and strabismus surgery is reported. The issues of antimicrobial prophylaxis are discussed. The authors speculate about the possible routes of surgical site infection. Transient bacteraemia secondary to adenotonsillectomy may be theoretically a source of distant surgical site infection to the orbit, raising the issue of distant surgical site contamination during multidisciplinary surgery. Combined adenotonsillectomy and eye surgery might benefit from prophylactic systemic antibiotic administration.

The aim of this study was to assess the natural course of positional down-beating nystagmus (pDBN) and vertigo in patients with no evidence of central nervous system involvement and of presumed peripheral origin. Fifty-three patients with pDBN had a complete otoneurological examination. All subjects, apart from three (excluded from the study), showed no additional neurological signs and normal brain imaging. Patients were randomly assigned to two groups: with or without treatment with exercise. Patients were seen again after 24 h, and then weekly for up to 6 months. Forty-seven patients (94 %) showed pDBN in the straight head-hanging position and in a Dix–Hallpike position. A torsional component was detected in 17 patients (34 %). The mean latency and duration of pDBN was 4.7 ± 5 s and 40.1 ± 22 s, respectively. After 2 weeks, only 12 patients (24 %) still had pDBN and all but one patient had recovered by 1 month. Twenty patients (40 %) were diagnosed with a typical posterior canal benign paroxysmal positional vertigo (PC BPPV) before or after pDBN. This study assessed for the first time the natural course of presumed peripheral pDBN, which was characterized by a spontaneous remission in 24 patients in the first week and in 49 patients within 4 weeks. pDBN is much more common than previously suggested, with about the same frequency as lateral canal BPPV. Furthermore, the clinical characteristics of pDBN have been highlighted, as well as its possible relationship to PC BPPV.

We evaluated the incidence and characteristics of hyperventilation-induced nystagmus (HVN) in 49 patients with gadolinium-enhanced magnetic resonance imaging evidence of vestibular schwannoma and 53 patients with idiopathic unilateral sensorineural hearing loss and normal radiological findings. The sensitivity and specificity of the hyperventilation test were compared with other audio-vestibular diagnostic tests (bedside examination of eye movements, caloric test, auditory brainstem responses) in the two groups of patients. The hyperventilation test scored the highest diagnostic efficiency (sensitivity 65.3 %; specificity 98.1 %) of the four tests in the differential diagnosis of vestibular schwannoma and idiopathic unilateral sensorineural hearing loss. Small tumors with a normal caloric response or caloric paresis were associated with ipsilateral HVN and larger tumors and severe caloric deficits with contralateral HVN. These results confirm that the hyperventilation test is a useful diagnostic test for predicting vestibular schwannoma in patients with unilateral sensorineural hearing loss.

Context: Anecdotal evidence suggests a high incidence in Trentino, Italy, of head and neck paragangliomas (HNPGL), a rare autosomal dominant disease called paraganglioma type 1 syndrome and caused by germ-line mutations of the SDHD gene. Objective: The aim of this study was to investigate the origin, spread, and clinical expression of the disease in this geographic region. Design, Setting, and Participants: Trentino natives with HNPGL were recruited for establishing clinical expression of the disease, presence of a founder effect, and age of common ancestor. A large sample of the local population was recruited for determination of mutation prevalence and spread. Main Outcome Measures: SDHD genetic testing was offered to first-degree relatives, and clinical surveillance was offered to at-risk carriers. The hypothesis of a founder effect was explored by haplotype analysis, and time to the most recent common ancestor was estimated by decay of haplotype sharing over time. Results: A total of 287 of the 540 recruited individuals from 95 kindreds carried the SDHD c.341A>G p.Tyr114Cys mutation. The prevalent phenotype was bilateral or multiple HNPGL, with low prevalence of pheochromocytoma and malignant forms. Penetrance was high. A common ancestor was dated between the 14th and 15th century, with the mutation spreading from the Mocheni Valley, a geographic, cultural and, presumably, a genetic isolate to 1.5% of the region's population. Conclusions: A combination of particular demographic, geographical, and historical conditions has resulted in the oldest and largest SDHD founder effect so far characterized and has transformed a rare disease into an endemic disease with major public health implications.

The aims of this investigation were to review the clinical behavior of deep neck infections (DNIs) treated in our institution in order to identify the predisposing factors of life-threatening complications and propose valuable recommendations for management and treatment. A total of 365 adult patients with DNIs were retrospectively identified. One-hundred and thirty-nine patients (38.1%) underwent surgical drainage. Overall, 226 patients (61.9%) responded effectively to intravenous antimicrobial therapy only. There were 67 patients (18.4%) developing life-threatening complications. Diabetes mellitus (odd ratio 5.43; P < 0.001) and multiple deep neck spaces involvement (odd ratio 4.92; P < 0.001) were the strongest independent predictors of complications. The mortality rate was 0.3%. Airway obstruction and descending mediastinitis are the most troublesome complications of DNIs. In selected patients, a trial of intravenous antibiotic therapy associated with an intensive computed tomography-based wait-and-watch policy may avoid an unnecessary surgical procedure. However, about one-fourth of patients present significant comorbidities, which may negatively affect the course of the infection. In these cases and in patients with large or multiple spaces infections, a more aggressive surgical strategy is mandatory.

The aim of the present investigation is to evaluate the outcome after induction chemotherapy and concurrent multi-drug chemoradiotherapy (IC/CCRT) with or without post-chemoradiation neck dissection in medically fit elderly patients with loco-regionally advanced head and neck squamous cell carcinoma (HNSCC). Retrospective study including 44 elderly patients (median age 71 years; range 66–77 years) with previously untreated, inoperable, histologically proven non-metastatic stage III or IV HNSCC. Following one cycle of IC, two cycles of cis-platinum and 5-fluorouracil CCRT with conventional fractionated radiotherapy up to a dose of 66–70 Gy were administrated. A neck dissection was recommended for patients with node metastasis larger than 3 cm regardless of the response to therapy and for patients who had suspected persistent neck disease 8–12 weeks after completing treatment. Salvage surgery was considered for histologically proven persistent or recurrent tumor in the primary site. Time-to-event data were described using Kaplan–Meier actuarial curves. Overall, 37 patients (84.1%) completed the planned treatment. There were no cases of treatment-related deaths. Twenty-nine patients (65.9%) developed severe toxicities with grade 4 toxicity accounting for 22.7%. The median follow-up time in survivors was 41 months. Three-year overall survival, progression-free survival, and functional progression-free survival estimates were 70.9, 67.0, and 57.3%, respectively. In selected medically fit elderly patients with loco-regionally advanced HNSCC, cis-platinum-based chemoradiotherapy can be successfully applied, with moderate adverse events, in attempt to preserve a functional upper aerodigestive tract.

The aim of this study is to describe an alternative technique to secure the receiver-stimulator of the cochlear implant to the skull with a tailored flap of periosteum. Other techniques are also reviewed and discussed. 179 consecutive patients were implanted by the same surgeon in a tertiary care setting. Age ranged from 11 months to 74 years. Patients were retrospectively evaluated for device migration. No cases of migration were observed during follow up, ranged 1-99 months with a median of 48 months. The alternative technique proposed is safe and reliable. © 2011 Springer-Verlag.

Results One-hundred and twenty-one responses were recorded, coming from 43 nations in the 5 continents (Fig. 1). Sixty-two otologists (53%) have considerable experience, each having performed more than 100 implantation surgeries. This experienced group of surgeons perform most of the bilateral and sequential operations (P< 0.01 and P< 0.05, respectively). Single-stage bilateral CI surgery is preferred by 54% of surgeons. CI in SSD is seldom performed. Implantation of electric-acoustic stimulation devices is performed by 37% of the respondents. Senior surgeons are more often involved in implantation surgery at extreme ages (P< 0.001). Significant changes in the surgical procedure are recommended for very young children (P< 0.05). No changes in the standard implantation procedure are recommended for the elderly. In the routine radiological assessment of the temporal bone, plain X-rays are performed by 2% of respondents preoperatively and 68% postoperatively. Computed tomography is performed by 91% preoperatively and 12% postoperatively. Seventy-four per cent of surgeons routinely obtain magnetic resonance preoperatively. These results did not vary by the surgeon group. Thirty-five per cent of surgeons are of the opinion that the type and shape of the electrode array, and the insertion technique, can significantly influence CI outcomes. Opinions were expressed about electrode array shape (n= 15), length (n= 14), depth of insertion (n= 11), best technique to preserve residual hearing (n= 10), and position of contacts (n= 8) (Fig. 2). Sixty-four per cent of surgeons always use facial nerve monitoring. Fifteen per cent of surgeons utilize facial nerve monitoring only in selected cases. Almost all surgeons use antibiotic prophylaxis perioperatively (96%). Custom-made instruments are present in 39% of CI surgical kits. Half of the respondents pay attention to a cosmetic hair shave. A small skin incision is preferred (55%) to a standard incision (20%). Minimally invasive incisions are also common (24%). Young surgeons rely more often than senior surgeons on a traditional skin incision with a posterior extension over the receiverstimulator area (P< 0.001). Fifty-five per cent of surgeons utilize a bilayer flap while 40% use a full thickness flap. Mastoidectomy and posterior tympanotomy are preferred by 95% of the respondents. The insertion of the array through the round window (65%) is favoured over a promontory cochleostomy (35%) and is generally preferred by more experienced surgeons (P< 0.05). Half of the respondents, and more often senior surgeons (P< 0.001), rely on soft insertion techniques (Fig. 3). The receiver-stimulator is secured by sutures passed through bone (40%) or periosteum (30%), screws (9%), or by other techniques (23%) (Fig. 4). Correspondence to: Enrico Muzzi, MD Audiology and ENT Unit, Department of Pediatrics, Institute for Maternal and Child Health – IRCCS “Burlo Garofolo”, Via dell’Istria 65/1, 34137 Trieste, Italy. Email: enrico.muzzi@burlo.trieste.it, enr.muz@gmail.com

The aim of this investigation was to determine if there is any association between the size of the canal dehiscences and the symptoms and signs of patients presenting with the superior semicircular canal dehiscence syndrome. Prospective multicenter study. Tertiary referral center. Twenty-seven patients, 14 females and 13 males, aged 25 to 83 years, coming from Switzerland, France, Belgium, or Italy, with dehiscence of the superior semicircular canal diagnosed by high-resolution computed tomographic scans of the temporal bone. Audiologic tests, a battery of vestibular tests (Tullio phenomenon, Hennebert sign, Valsalva maneuver), vestibular evoked myogenic potentials (VEMPs), and high-resolution computed tomographic scans of the temporal bone. Association between the symptoms/signs and the size of the superior canal dehiscence. Clinically patients could be divided into three different groups: Superior canal dehiscences (≥2.5 mm) presented predominantly with cochleovestibular symptoms and/or signs (sensitivity, 91.7%; specificity, 70%), whereas smaller one's showed either cochlear or vestibular dysfunction. Patients with larger dehiscences were significantly more associated with vestibulocochlear symptoms/signs, lower VEMP thresholds, and objective vestibular findings (e.g., Tullio phenomenon) than subjects with smaller bony defects. No significant association between the size of the dehiscence and the audiogram pattern or individual findings could be found. The location of the dehiscence seemed to have no influence on the clinical manifestation and findings. Patients with larger superior canal dehiscences show significantly more vestibulocochlear symptoms/signs, lower VEMP thresholds, and objective vestibular findings compared with smaller ones. Smaller dehiscences mainly present with either cochlear or vestibular dysfunction.