Italian Fast Speech Reception Threshold Test: A New Method to Investigate Adult Auditory Impairment in Noise.

INTRODUCTION: Purpose of our study was to compare two competing methods of performing bisyllabic word speech audiometry for the detection of the 50% speech reception threshold in noise (SRT50). METHODS: Classic method is performed submitting multiple word lists at a fixed signal-to-noise ratio. A newer Fast method - Italian Fast Speech Reception Threshold 50 (IFastSRT50) - is performed by means of program software with a single list of bisyllabic words and noise intensity shifting. RESULTS: Means comparison between SRT50 Classic and IFastSRT50 shows a slight significant correlation (r = 0.263; p = 0.044) and a wide significant difference: SRT50 Classic = -2.763 dB (SD = 4.1) and IFastSRT50 = -7.803 dB (SD = 2.1) (p < 0.0001). There is a high difference between the test execution time means (SRT50 Classic = 11 min, IFastSRT50 = 2 min; p < 0.0001). The correlation between test results and execution times was higher for SRT50 Classic than IFastSRT50. CONCLUSION: IFastSRT50 test is a reliable method to quickly investigate signal-to-noise ratio needed to obtain 50% of recognition scores with bisyllabic words; it allows less execution time than SRT50 Classic method and can avoid patient fatigue and other limitations of different speech discrimination tests in noise as sentences based ones.

Pediatric normative data for a novel and fast speech perception test in noise.

OBJECTIVES: Communicating in noisy settings can be difficult due to interference and environmental noise, which can impact intelligibility for those with hearing impairments and those with normal hearing threshold. Speech intelligibility is commonly assessed in audiology through speech audiometry in quiet environments. Nevertheless, this test may not effectively assess hearing challenges in noisy environments, as total silence is rare in daily activities. A recently patented method, known as the SRT50 FAST, has been developed for conducting speech audiometry in noise. This new method enables the acceleration and simplification of free field speech audiometry tests involving competition noise. This study aims to establish normative scores and standardize the SRT50 FAST method as a test for evaluating speech perception in noise in pediatric patients. METHODS: The study included 30 participants with normal hearing, consisting of 11 females and 19 males, ranging in age from 6 to 11 years. A series of speech audiometry tests were conducted to determine the speech reception threshold 50% (SRT50) in competing conditions. This included testing both the fast mode (SRT50 FAST) currently being studied and the traditional method (SRT50 CLASSIC). The SRT50, or Signal to Noise Ratio (SNR) at which 50% of speech recognition occurred, was investigated for both methods. RESULTS: The mean SRT50 FAST test score was -2.69 (SD = 3.15). The dataset exhibited a normal distribution with values ranging from 3.60 to -8.60. Since the scores are expressed in SRT, higher scores indicate poorer performance. We have established a threshold of 3.60 as the upper limit of the normal range, therefore, patients with scores above this threshold are considered to have abnormal results. CONCLUSIONS: This study aimed to establish normative data for the evaluation of free field speech in noise recognition using the SRT50 FAST method in the pediatric population. This method accurately investigates the necessary signal-to-noise ratio for achieving 50% recognition scores with bisyllabic words in a quick manner. The ultimate objective is to employ this test to identify the optimal configuration of hearing rehabilitation devices, particularly for pediatric patients with hearing aids and/or cochlear implants. Additionally, it can be used to assess pediatric patients with unilateral hearing loss.

Implantable hearing devices in clinical practice. Systematic review and consensus statements.

Objective: Implantable hearing devices represent a modern and innovative solution for hearing restoration. Over the years, these high-tech devices have increasingly evolved but their use in clinical practice is not universally agreed in the scientific literature. Congresses, meetings, conferences, and consensus statements to achieve international agreement have been made. This work follows this line and aims to answer unsolved questions regarding examinations, selection criteria and surgery for implantable hearing devices. Materials and methods: A Consensus Working Group was established by the Italian Society of Otorhinolaryngology. A method group performed a systematic review for each single question to identify the current best evidence on the topic and to guide a multidisciplinary panel in developing the statements. Results: Twenty-nine consensus statements were approved by the Italian Society of Otorhinolaryngology. These were associated with 4 key area subtopics regarding pre-operative tests, otological, audiological and surgical indications. Conclusions: This consensus can be considered a further step forward to establish realistic guidelines on the debated topic of implantable hearing devices.

Vitamin D Deficit as Inducer of Adenotonsillar Hypertrophy in Children with Obstructive Sleep Apnea-A Prospective Case-Control Study.

(1) Objective: This prospective case-control study aimed to assess the level of serum vitamin D comparing pediatric non-allergic patients with obstructive sleep apnea (OSA) and healthy controls. (2) Methods: The period of the enrollment was from November 2021 to February 2022. Children with uncomplicated OSA caused by adenotonsillar hypertrophy (ATH) were recruited. Allergy was excluded by skin prick test (SPT), and the determination of serum IgE level using ELISA test. Plasma concentration of 25-hydroxy vitamin D (25-OHD) was quantitatively determined; then, the vitamin D concentration in patients was compared with healthy controls matched for sex, age, ethnicity, and characteristics. (3) Results: Plasma 25-OHD levels were significantly lower in patients than in healthy subjects (mean 17 ng/mL, 6.27 DS, range 6-30.7 ng/mL, vs. mean 22 ng/mL, 9.45 DS, range 7-41.2 ng/ ml; p < 0.0005). The prevalence of children with vitamin D deficiency was significantly higher in the ATH group than controls. The plasma 25-OHD level did not change following the ATH clinical presentation (III or IV grade according to the Brodsky scale), while the different categories of 25-OHD status (insufficiency, deficiency, and adequacy) in the ATH group were statistically significantly different (p < 0.001) from healthy controls. (4) Conclusions: This study identified statistically significant differences between the ATH group and control regarding the plasma concentration of vitamin D; this data, despite not being directly linkable to the lymphoid tissue hypertrophy (p-value not significant), might suggest a negative effect of vitamin D deficit on the immune system.

Comparative Analysis of Intellectual Quotient in Developmental Population with Severe Hearing Loss: Hearing Aids vs. Cochlear Implant Users.

The development of language, memory and intellectual functions is linked to normal hearing and correct sounds interpretation. Hearing loss (HL), especially in its severe form, negatively affects the development of these functions. This prospective study aimed at comparing the Intelligent Quotients (IQ) of children with cochlear implants (CI) with the ones of people wearing hearing aids (HA) after one year of hearing rehabilitation. 21 subjects with severe/profound bilateral hearing loss (deafness) were included in this study. Eleven children with congenital profound HL underwent CI and ten children with moderate to severe HL (congenital and acquired) were rehabilitated by HA. Children's IQs were assessed at enrolment (T0) and 12 months after hearing aids/CI use plus speech therapy. Statistical analyses were performed to analyze the data within and between groups. Comparison of IQs showed no statistically significant differences between CI and HA none at T0 and T1. The subtests showed lower scores in verbal comprehension and process speed index in patients treated with HA when compared to CI. This study showed that auditory rehabilitation can support the normal development of cognitive function in children between six and eight years of age. The use of the correct hearing aids based on the patient's hearing thresholds is important to maximize the rehabilitation outcomes. Due to the small sample size, although stratified for age, our results must be considered preliminary and further analyses on larger samples are needed to confirm our data.

Diagnosis and Treatment of Nodular Fasciitis of Ear Region in Children: A Case Report and Review of Literature.

Nodular fasciitis (NF) is a benign fibroblastic and myofibroblastic proliferation of subcutaneous tissues. Rarely, it has been identified in the ear and more rarely in children. We describe a case in a four-year-old girl and the surgical management of it. The patient was referred to the otolaryngology unit of a tertiary referral center because she was affected by a painless and growing lesion in the left external auditory canal (EAC). The girl was treated by large-spectrum antibiotic therapy for one week without success. For this reason, we requested ultrasonography (US) of the left hemiface, maxillofacial and temporal bone computed tomography (CT) and magnetic resonance imaging (MRI) of the head with and without contrast. The imaging identified an irregular ovoid hypoechoic nodule with distinct margins indissociable from the cartilaginous planes and extending into the parotid loggia with local infiltration of the fascia. The lesion was surgically removed through preauricular access due its extension into the parotid area. The mass was excised in toto and sent to the pathologist for immunohistochemistry. The histopathologist based on the finding diagnosed a nodular fasciitis. In case of suspicion of malignancy, early investigations should be done to evaluate the lesion, then a traditional parotidectomy can be safely and successfully performed even in a very young child. The open technique allows the removal of NF with full control of the surgical area and facial nerve. In this article, we presented the management of a case in a 4-year-old female affected by NF of the external auditory canal (EAC), and we described clinical and surgical management of the case. We also reviewed literature of nodular fasciitis cases of ears in children.

Single-Center-Single-Blinded Clinical Trial to Evaluate the Efficacy of a Nutraceutical Containing Boswellia Serrata, Bromelain, Zinc, Magnesium, Honey, Tyndallized Lactobacillus Acidophilus and Casei to Fight Upper Respiratory Tract Infection and Otitis Media.

Some nutraceuticals have been studied as supportive treatment for fighting upper respiratory tract infection and middle ear disease. Our study aims at evaluating the effect of a specific oral supplementation in the treatment of pediatric otits media. The subjects were randomly assigned by the physician (single-blinded study) to one of three groups: Control Group (CG), Treatment Group 1 (TG1), or Treatment Group 2 (TG2). Both TG were treated with Flogostop Duo (for 20 days-TG1 or 30 days-TG2) in combination with the standard treatment, while CG underwent standard treatment only. The standard treatment was nasal aerosol with Fluticasone and Mucolytic, and nasal washing with hypertonic solution. All patients were analyzed by otoscopy, impedance, fibroscopy, and pure auditory test at the baseline (T0), after 20 days (T1) and 35 days (T2). 120 children were included in the study, 40 in the CG, 40 in the TG1, and 40 in the TG2. Both TG1 and TG2 presented statistically significant differences with respect to controls in otoscopy, impedance, fibroscopy, and PTA at T2. The otoscopy improved at T2 with statistically significant value only in TG2. The impedance and fibroscopy improved at T1 both in TG1 and TG2 compared to CG. A statistically significant improvement was observed in TG2 at T2 in comparison to both CG and TG1. Statistically significant differences were observed in PTA at T2 only compared with controls. This study confirmed the efficacy of nutraceutical as supporting therapy in the upper respiratory tract infection in children. In particular, the supplement containing Boswellia serrata and Bromelain, which are molecules with strong anti-inflammatory and pain-control capacities, could add the benefit without the adverse effects which are related to NSAID use.

OTOPLAN, Cochlear Implant, and Far-Advanced Otosclerosis: Could the Use of Software Improve the Surgical Final Indication?

Cochlear implant surgery in far-advanced otosclerosis can be challenging due to the degenerative process that affects the cochlea. We used OTOPLAN® to plan and define the details of surgery in a patient with such severe alteration of the cochlea that cochlear implant could be contraindicated. A 73-year-old man affected by bilateral far-advanced otosclerosis, previously treated by bilateral stapedotomy, presented 0% of speech discrimination using bilateral hearing aids. A unilateral cochlear implant was planned. The patient underwent radiologic investigation pre-surgery with temporal bone computer tomography, magnetic resonance imaging, and OTOPLAN. Radiology confirmed bilaterally advanced signs of fenestral and cochlear otosclerosis with large osteolytic cavities along the whole cochlea leading to the mixture of endolymph and perilymph. The OTOPLAN identified the alteration of the cochlea in detail. Based on the results of the software, we used a perimodiolar implant on the left ear. No intraoperative or post-operative surgical complications were observed. The patient was checked 6 months after surgery, he did not refer any problems and obtained 75% of speech discrimination at 65 dB. Our case suggests that OTOPLAN is a useful tool in far-advanced otosclerosis because careful planning of the surgery can positively affect the results. Despite the complexity of the anatomy, the software exactly described the real intrasurgical finding. We think that the use of OTOPLAN might improve the surgical indication.

The effect of female hormone in otosclerosis. A comparative study and speculation about their effect on the ossicular chain based on the clinical results.

PURPOSE: This study aimed at identifying gender differences in the hearing thresholds in a sample of patients with otosclerosis before and after surgery to understand the impact of female hormones on auditory thresholds. METHODS: This retrospective study analyzed 184 patients (123 women and 61 men) affected by otosclerosis. All the patients were affected by conductive hearing loss and treated by stapedoplasty. Auditory thresholds at the baseline (T0) and one month after surgery (T30) were collected. Air and bone thresholds and Air Bone Gap (ABG) were compared between females and males using one-way ANOVA. RESULTS: Statistically significant differences were observed comparing the air threshold at T0 vs T30 both in women and men (p < 0.0001). No statistically significant differences were observed in the bone conduction thresholds before and after surgery. The comparison between females and males showed statistically significant differences both at T0 (p < 0.01) and T30 (p < 0.05) for air conduction thresholds and ABG at 4000 Hz. CONCLUSION: Although stapedoplasty reduced the difference between females and males in the air conduction thresholds and ABG, women showed better recovery of their middle ear function with better auditory thresholds and ABG. The female hormones might positively impact the ligaments of the incudostapedial joint improving chain flexibility. This benefit might explain the statistically significant difference observed in women at 4000 Hz before and after surgery.

Bulging of the Oval Window in Common Cavity Deformity: A Possible Predictor of Meningitis.

OBJECTIVE: To investigate the prevalence-rate of oval window bulging in the common cavity and its association with bacterial meningitis. PATIENTS: CT and clinical files of 29 children with preliminary diagnosis of common cavity deformity were collected from 13 Italian centers. INTERVENTION: A retrospective case review study was conducted with a centralized evaluation of the temporal bone CT imaging was performed at Azienda Ospedale - Università Padova, Padova, Italy. MAIN OUTCOME MEASURE: Diagnosis of common cavity was reviewed; in addition, a fluid protrusion into the middle-ear cavity through the oval window at CT imaging was considered as oval window bulging. Its association with the history of bacterial meningitis was investigated. RESULTS: Common cavity deformity was confirmed in 14/29 children (mean-age 11.4 ±â€Š3.8; age-range 5-20; nine females) referred with this diagnosis. In 7/14 patients, the common cavity deformity was bilateral (i.e., 21 common cavities). Oval window bulging was found in 3/19 common cavities (concomitant middle-ear effusive otitis hampered the evaluation in two cases), while the internal acoustic meatus fundus was defective in 10/21 cases. History of bacterial meningitis was found in three children (21%) and two of them had oval window bulging at CT. In the case unrelated to oval window bulging, meningitis occurred late at the age of 12 during acute otitis contralateral to common cavity deformity (ipsilaterally to incomplete partition type 1). CONCLUSION: Patients harboring common cavity deformity have a high risk of meningitis in their first years of life. Oval window bulging seems to be associated with a higher risk of meningitis. This information might be important for appropriate surgical planning.

Hearing loss and dementia: radiologic and biomolecular basis of their shared characteristics. A systematic review.

Dementia and hearing loss share radiologic and biologic findings that might explain their coexistence, especially in the elderly population. Brain atrophy has been observed in both conditions, as well as the presence of areas of gliosis. The brain atrophy is usually focal; it is located in the temporal lobe in patients with hearing loss, while it involves different part of brain in patients with dementia. Radiological studies have shown white matter hyperintensities (WMHs) in both conditions. WMHs have been correlated with the inability to correctly understand words in elderly persons with normal auditory thresholds and, the identification of these lesion in brain magnetic resonance imaging studies has been linked with an increased risk of developing cognitive loss. In addition to WMHs, some anatomopathological studies identified the presence of brain gliosis in the elderly's brain. The cause-effect link between hearing loss and dementia is still unknown, despite they might share some common findings. The aim of this systematic review is to analyze radiologic and biomolecular findings that these two conditions might share, identify a common pathological basis, and discuss the effects of hearing aids on prevention and treatment of cognitive decline in elderly patients with hearing loss.

Use of a 3D reconstruction model in a patient with severe atresia auris for optimal placement of Bonebridge transcutaneous bone conduction implant.

PURPOSE: Patients affected by severe atresia auris (AA) can be a challenge during hearing restoration surgery due to the abnormal position of vascular and nervous structures in the bone. A 3D reconstruction model of malformed temporal bones can be helpful for planning surgery and optimizing intra-, peri-, and post-operative results. METHOD: A 5-year-old girl with severe AA on the right side was implanted with a Bonebridge transcutaneous bone conduction implant (tBCI). 3D printing was used to reproduce the malformed temporal bone, find a good position for the tBCI and plan out the surgical details in advance. Hearing tests were performed before and after surgery and information about intra-, peri-, and post-operative outcomes were collected. RESULTS: The patient did not show any negative outcomes and, thanks to the Bonebridge, completely recovered hearing on the right side. CONCLUSIONS: 3D printing is a useful tool for planning surgery in AA patients and for preventing possible risks related to the unknown malformed anatomy.

A minimally invasive endoscope assisted retrosigmoid approach for removal of arachnoid cysts in the internal auditory canal: a step by step description.

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.

Does Unilateral Hearing Loss Impair Working Memory? An Italian Clinical Study Comparing Patients With and Without Hearing Aids.

Working memory (WM) function can be reduced in patients suffering from unilateral hearing loss (UHL) and can affect their academic performance. We aimed to compare the WM abilities of three categories of children with UHL: patients implanted with hearing aids (HAs), patients receiving a bone-anchored hearing implant (BAHI), and subjects who did not receive hearing devices. A randomized clinical study, in which 45 children (mean age: 9.5 years) were evaluated by pure tone audiometry (to identify the side and the severity of the UHL), was conducted in a tertiary referral center. Patients were simply randomized into three groups: (1) children without HAs (No-HA group), (2) patients with a (digital) HA (HA group), and (3) children with a BAHI (BAHI group). Their working and short-term memories were studied in both noisy and silent conditions at the recruiting time (T0, baseline) and 6 months after (T1) the treatment. Statistical analyses were performed to analyze the variances between T0 and T1 within each group and between the three groups. The No-HA group improved its T1 WM scores in silence (p < 0.01), but not in noise. The HA and BAHI groups showed statistically significant variances of T1 WM in noise (p < 0.01 and p < 0.01, respectively). The HA and BAHI groups did not show statistically significant variances compared to T1. Our results suggest that hearing devices (HA and BAHI) in children with sensorineural UHL (SUHL) can improve WM capacity in noise. We speculate that bilateral hearing capacity might improve the quality of life of this population, especially during everyday activities where noise is present.

A Novel Bone Conduction Hearing System May Improve Memory Function in Children with Single Side Hearing loss: A Case-Control Study.

OBJECTIVES: To evaluate the effects of an adhesive adapter prosthesis (AAP) on memory function in pediatric subjects with single side hearing loss (SSHL). MATERIALS AND METHODS: Case-control study. 19 pediatric subjects with mild to moderate SSHL treated with AAP and 15 subjects with normal hearing (control group) were included in this study. Working and short-term memory functions were tested in all subjects, in silence and noise conditions. In SSHL subjects, tests were performed before the AAP was applied (T0) and at 1-month (T1) follow-up. The control group was tested once. RESULTS: AAP significantly improved working memory function in noise as measured at T1 (p<0.01) compared with T0, but T1 scores in children with SSHL remained significantly different from the ones of the control group (p<0.01). AAP also significantly improved short- term memory function test scores at T1 compared with T0 (p<0.01), but despite being in the normal range for the subjects' age, the scores remained significantly different from those of the control group (p<0.01). CONCLUSION: In pediatric subjects with mild, moderate, and moderate-severe SSHL, restoration of bilateral hearing through AAP improved short-term memory function and working memory function in noise, as measured at 1 month follow-up; however, AAP did not seem to lead to a full restoration of such functions as measured by a comparison with healthy controls. Further studies with longer follow-ups might help elucidate whether AAP can elicit further improvements in memory functions.

Difensil Immuno Reduces Recurrence and Severity of Tonsillitis in Children: A Randomized Controlled Trial.

Oral supplements (OS) support the immune system in fighting upper airways infection. This study aimed to analyze the effect of Difensil Immuno (DI) on the recurrence of tonsillitis and fever in children. A multicentric randomized clinical trial was conducted. One-hundred and twenty children with chronic tonsillitis were randomly assigned to group A, B or control. Patients in group A were treated with 10 mL of DI for 90 consecutive days, patients in group B underwent treatment with 15 mL of DI for 45 consecutive days. The following data were collected at baseline (T0), T1 and T2: tonsillitis and fever episodes, tonsillar volume, blood test results. One-way ANOVA was used to analyze within and between variances. Patients in group A and B statistically improved their clinical parameters (episode of tonsillitis and fever, tonsillar volume) when compared to control group both at T1 and T2. However, T1 variances were more consistent in group A than in group B. All patients in the study groups improved their clinical outcomes. No statistically significant variances were observed in blood parameters both at T1 and T2. Our results suggest that children treated with DI had fewer episodes of tonsillitis and fever and a reduction in their tonsillar volume.

Loop characteristics and audio-vestibular symptoms or hemifacial spasm: is there a correlation? A multiplanar MRI study.

AIM: We investigated if loop characteristics correlate with audio-vestibular symptoms or hemifacial spasm in patients with a vascular loop in the root entry zone (VII and VIII) and in the internal auditory canal. MATERIALS AND METHODS: A retrospective, multicenter study analyzed 2622 consecutive magnetic resonance imaging (MRI) scans of the cerebellopontine angle of patients with asymmetric audio-vestibular symptom or hemifacial spasm; patients' symptoms were confirmed by clinical tests. MRIs displaying vascular loops visible in the axial view were analyzed using multiplanar reconstruction. We evaluated (1) depth of penetration of the loop into the internal auditory canal (IAC); (2) largest diameter of the vessel; (3) nerve(s) involved in the vascular impingement, position of the loop relative to such nerve(s) and number of contacts between vessel and nerve(s); (4) length of such contact. The loop metrics described above were correlated with the patients' audio-vestibular symptoms and hemifacial spasm. RESULTS: Three hundred ninety-nine patients displayed a loop visible in the MRI axial view and out of them only 118 displayed a direct contact between loop and nerve. The cochlear nerve was involved in a contact in 57.7%. Loops in direct nerve contact had a calibre > 0.85 mm, were located in the middle portion of the IAC, and correlated with vertigo (p = 0.002), tinnitus (p = 0.003), and hemifacial spasm (p < 0.001). Asymmetric sensorineural hearing loss (SNHL) correlated with number of contacts (p < 0.001) and length of contact (p < 0.05). The contact was asymptomatic in 41.5% of patients. CONCLUSION: Loop characteristics may help predict whether a vascular impingement is responsible for a symptom and guide the physician to select the best treatment. KEY POINTS: • A vascular loop in the internal auditory canal was observed in 18-20% of the patients in this study; whether a loop can be responsible for a compressive syndrome is still unclear in particular referred to the vestibulocochlear nerve. • Compression by a loop on the facial nerve causes hemifacial spasm; compression by a loop on the cochlear or vestibular nerve may cause audio-vestibular symptoms. • In patients with a loop, the loop calibre, the loop position, and the number of loop-nerve(s) assessed via the multiplanar MRI reconstruction technique may help assess whether the patient will manifest audio-vestibular symptoms or hemifacial spasm.

Can the Jankovic-assessment be used as an alternative to electromyography? A cross-sectional study on facial dystonia patients treated with Botulinum toxin.

PURPOSE: This study aims to quantitatively compare the Jankovic assessment (JA) with electromyography (EMG)-based measures for assessing changes in facial movements in patients with facial dystonia. MATERIALS AND METHODS: Thirteen patients (five males and eight females) affected with different forms of facial dystonia (hemifacial spasm and synkinesis) participated in this study. All patients were treated with Botulinum Toxin (BTX) and evaluated with the JA scale and EMG-based measures, including motor unit potentials (MUP) latency and presence of polyphasic potentials before and after BTX injection. Correlation between the JA scores and the EMG-based measures was calculated. Statistical analysis was performed with the Pearson test. RESULTS: Correlation between the JA scores and the EMG-based measures was found to be statistically significant, both before and after treatment with BTX. CONCLUSION AND RELEVANCE: JA scores significantly correlated with more objective EMG-based measures, suggesting that the JA scale can be used to assess facial movement changes, for example elicited by a treatment such as BTX injection. Thus, in facial dystonia patients, the JA scale may be used for evaluating treatment outcomes as a valid and low-cost alternative to EMG.

E-ABR in Patients with Cochlear Implant: A Comparison between Patients with Malformed Cochlea and Normal Cochlea.

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.

Endoscope-assisted retrosigmoid approach in hemifacial spasm: our experience.

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 24h after surgery and in 12/12 (100%) subjects within 45 days. A significant (p<0.001) correlation between preoperative historical duration of hemifacial spasm and postoperative recovery timing was recorded. Only 1 patient had a complication (meningitis), which resolved after intravenous antibiotics with no sequelae. No cases of cerebrospinal fluid leak, facial palsy or hearing impairment were recorded. Hemifacial spasm recurrence was noticed in the only subject where the neurovascular conflict was due to a vein within the internal auditory canal. CONCLUSIONS: The endoscope assisted retrosigmoid approach technique offers an optimal visualization of the neurovascular conflict thorough a minimally invasive approach, thus allowing an accurate decompression of the facial nerve with low complication rates. Due to the less invasive nature, the procedure should be considered in functional surgery of the cerebellar pontine angle as hemifacial spasm treatment, specially when the procedure is performed by an otolaryngologist.