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Introduction: Bone-anchored hearing devices (BAHD) are well-known good solution for single-sided deafness (SSD). Despite power extension of recently introduced BAHD with implanted active transducer, with indications up to 65 dB Hl of bone conduction (BC) threshold on the implanted side, their indications for SSD still remain better than 25 dB on the good ear, with regards to bone conduction thresholds. The aim of this study was to assess the possibility to enlarge BAHD indications for SSD by means of a newly proposed candidacy evaluation protocol, which includes a new software-aided method. Methods: 20 SSD patients (mean age 56 years, 9 females, and 11 males) were divided into two groups: group A (10 patients, BC Results: With BAHD tester turned off, no recognition was detected. Speech audiometry with BAHD tester turned on revealed mean values of 92% for group A and 89% for group B, with a difference of 3.0% (χ2 = 0.285 and p = 0.5935). As for IFastSRT50 with BAHD tester turned on, mean signal-to-noise ratio value to obtain 50% of recognition was −6.89 for group A and −6, with a difference of 0.89 (t = 1,201 and p = 0.2453). Conclusion: BAHD are confirmed to be a good solution for SSD cases. The absence of statistically significant differences in our two tested groups suggests that newer implanted active transducer device indications should be extended up to 35 dB Hl on the hearing ear. The IFastSRT50 is a reliable and quick method to enhance preoperative candidacy evaluation.

Vestibular schwannoma (VS) is a benign tumor which develops in the internal auditory canal and the cerebellopontine angle, potentially diminishing hearing or balance. Most VS tumors arise from one of two vestibular branches: the superior or inferior vestibular nerve. Determining the specific nerve of origin could improve patient management in terms of preoperative counseling, treatment selection, and surgical decision-making and planning. The aim of this study was to introduce a preoperative testing protocol with high accuracy to determine the nerve branch of origin. The nerve of origin was predicted on the basis of preoperative vestibular evoked myogenic potentials (VEMPs), caloric stimulation test, and pure tone audiometry on 26 recipients. The acquired data were entered into a statistic scoring system developed to allocate the tumor origin. Finally, the nerve of origin was definitively determined intraoperatively. Receiver operating characteristic (ROC) curves analysis of preoperative testing data showed the possibility of predicting the branch of origin. In particular, ROC curve of combined VEMPs absence, nystagmus detectable at caloric stimulation, and PTA 75 dB HL allowed to obtain high accuracy for inferior vestibular nerve implant of the tumor (area under the curve-AUC = 0.8788, p = 0.012). In 24 of 26 cases, the preoperatively predicted tumor origin was the same as the origin determined during surgery. Preoperative audiological and vestibular evaluation can predict the vestibular tumor branch of origin with high accuracy. Despite the necessity of larger prospective cohort studies, these findings may change preoperative approach, possible functional aspects, and counseling with the patients.

Objective: One of the most debated topics in tinnitus is its standard and practical classification. The most popular classification distinguishes subjective from objective tinnitus. Other classifications are based on different features. On the whole, they seem incomplete, and the diagnostic and therapeutic algorithms are often difficult for practical purposes. The aim of this work is to develop a new diagnostic and therapeutic algorithm. Methods: Our model is based on 10 years of experience. In particular, the starting point is the data retrieved from 212 consecutive patients in our Tinnitus Unit between May and December 2013: We found a clear auditory disorder in 74.5% of the population, muscolo-skeletal disorders and/or trigeminal disease in 57.1%, and psychiatric comorbidities in 43.8%. Different features coexisted in 59.9% of the population. Results: Following such data we propose the Tinnitus Holistic Simplified Classification, which takes into account the different tinnitogenic mechanisms and the interactions between them. It differentiates tinnitus that arises from: (1) auditory alterations (Auditory Tinnitus), (2) complex auditory-somatosensory interactions (Somatosensory Tinnitus), (3) psychopathological-auditory interactions (Psychopathology-related Tinnitus), and (4) 2 or all of the previous mechanisms (Combined Tinnitus). Conclusions: In our opinion this classification provides an accurate and easy tailored path to manage tinnitus patients.

Objectives to investigate in Parkinson's Disease affected patients a correlation between hyposmia and gastrointestinal dysfunction and their possible identical etiopathogenesis. Design retrospective cohort study. Setting ENT and Neurology departments (Gemelli Hospital, Rome, Italy). Participants 78 patients with diagnosis of PD according to the UK Brain Bank criteria. Inclusion criteria: informed consent; olfactory testing executed; exclusion criteria: signs of dementia according to the DSM-IV criteria; Mini Mental State Examination score ≤ 26; head trauma; central neurological disorders, nasal or systemic diseases potentially affecting olfactory function. Motor condition was assessed by means of Hoehn and Yahr staging and by section III of the Unified PD Rating Scale, performed off and on medications. Main outcome measures the patients underwent olfactory evaluation (TDI score), after rhinomanometry with nasal decongestion. 25 non-motor symptoms were evaluated trough an interview. Results olfactory dysfunction was objectively found in 91.0% of patients, a percentage higher than the subjective hyposmia reported (55.1%) p=0.0001. 7 patients (9.0%) were normosmic, 49 (62.8%) hyposmic, 22 (28.2%) anosmic. Subjective hyposmia, constipation, bloating and dyspepsia differed across groups, being higher in anosmic and hyposmic ones than in the normosmic group. p value was ≤ 0.05 for each symptom. Despite the original results, this study has the limitation of being based on subjective ratings by a relatively limited group of patients. Conclusions Hyposmia and gastrointestinal symptoms are correlated and this would support a possible common origin; the CNS could be reached through two different pathways, both starting in the peripheral nervous system. This article is protected by copyright. All rights reserved.

In a subpopulation of patients, tinnitus can be modulated by movements of the jaw or head and neck due to complex somatosensory-auditory interactions. In some of these subjects, tinnitus could be related to an underlying temporomandibular (TMJ) or craniocervical (NECK) dysfunction that, if correctly identified, could streamline treatment and increase chances of tinnitus improvement. However, it is still unclear whether somatic modulation of tinnitus could be used as a screening tool for identifying such patients. In this study, we included 310 tinnitus patients with normal hearing, no psychiatric comorbidities, and a positive history of TMJ and/or NECK dysfunction and/or a positive modulation of tinnitus to evaluate the characteristics of somatic modulation, investigate the relationship between positive history and positive modulation, and identify factors most strongly associated with somatic modulation. Tinnitus modulation was present in 79.67% of the patients. We found a significant association within the same subjects between a positive history and a positive tinnitus modulation for the same region, mainly for TMJ in unilateral tinnitus patients and for TMJ + NECK in bilateral tinnitus patients. A strong correlation between history and modulation in the same somatic region within the same subgroups of subjects was also identified. Most TMJ maneuvers resulted in an increased loudness, while NECK maneuvers showed an increase in tinnitus loudness in about 59% of cases. High-pitched tinnitus and male gender were associated with a higher prevalence of modulation; no differences were found for tinnitus onset, Tinnitus Handicap Inventory score, and age. In this paper, we report a strong association between history and modulation for the same regions within the same patients; such an association should always be investigated to improve chances of a correct diagnosis of somatosensory tinnitus.

Recent research has shown that music perception remains very difficult for most cochlear implant (CI) users, who generally report being unsatisfied with the quality of music listening through the CI. In particular, the appreciation of the spectral features of music, which include pitch, melody and timbre, is widely known to be poorer than its rhythmic patterns. One of the possible causes for this may be the misalignment between the conventional frequency band allocation to the electrodes of the array (frequency-place function) and the distribution of pitch percepts generated by electrode stimulation along the array (electrode-pitch function). In this paper the authors investigate whether mismatch correction through individualized fitting of the processor's frequency maps can improve music understanding in ten Nucleus™ cochlear implant users, by comparing scores obtained with experimental and old map; both maps were tested before, immediately after and 1 month after the fitting through a music test battery designed for the purpose of the study.

Objective:To assess the electrode pitch function in a series of adults with postlingually implanted cochlear implants and with contralateral residual hearing, in order to investigate the correlation between the degree of frequency map mismatch and the subjects' speech understanding in quiet and noisy conditions.Design:Case series.Subjects:Seven postlingually deafened adults with cochlear implants, all with detectable contralateral residual hearing. Subjects' electrode pitch function was assessed by means of a pitch-matching test, in which they were asked to match an acoustic pitch (pure tones delivered to the non-implanted ear by an audiometer) to a perceived ‘pitch’ elicited by stimulation of the cochlear implant electrodes. A mismatch score was calculated for each subject. Speech recognition was tested using lists of sentences presented in quiet conditions and at +10, 0 and 5 dB HL signal-to-noise ratio levels (i.e. noise 10 dB HL lower than signal, noise as loud as signal and noise 5 dB HL higher than signal, respectively). Correlations were assessed using a linear regression model, with significance set at p Results:All patients presented some degree of mismatch between the acoustic frequencies assigned to their implant electrodes and the pitch elicited by stimulation of the same electrode, with high between-individual variability. A significant correlation (p r2 = 0.91 and 0.89, respectively).Conclusion:The mismatch between frequencies allocated to electrodes and the pitch perceived on stimulation of the same electrodes could partially account for our subjects' difficulties with speech understanding in noisy conditions. We suggest that these subjects could benefit from mismatch correction, through a procedure allowing individualised reallocation of frequency bands to electrodes.

Objectives: We analyzed the relationships between sudden sensorineural hearing loss (SSNHL) and serum levels of fatty acids, total cholesterol, low-density lipoproteins (LDLs), and the antioxidant coenzyme Q10. Methods: Forty-three patients with SSNHL and 43 healthy subjects were enrolled in the study. The main outcome measures were serum levels of fatty acids, coenzyme Q10, total cholesterol, and LDLs. Results: On univariate logistic regression analysis, high levels of total cholesterol (p < 0.001), LDLs (p = 0.024), behenic acid (p < 0.001), docosahexaenoic acid (p < 0.001), linolenic acid (p = 0.017), and oleic acid (p < 0.001) and low levels of coenzyme Q10 (p < 0.001) and nervonic acid (p < 0.001) were associated with an elevated risk of SSNHL. On multivariate analysis, only hypercholesterolemia (p = 0.15) and low levels of coenzyme Q10 (p = 0.02) and nervonic acid (p = 0.005) were significantly associated with SSNHL. Conclusions: This is the first report of low serum levels of nervonic acid as an independent risk factor for SSNHL. Considering that hypercholesterolemia, high serum levels of LDL, and low serum levels of the antioxidant coenzyme Q10 were associated with SSNHL as well, we hypothesize that saturated fatty acids may play a role in determining the dysmetabolic state in a subset of SSNHL patients. Together, these findings suggest that not only total cholesterol and LDL levels, but also fatty acid determination, may help identify SSNHL patients with cardiovascular risk factors.

Objective: To analyze and compare the preoperative factors that potentially influence the outcome of stapedotomy in our study group. Materials and Methods: 161 cases were enrolled. Clinical variables considered to influence functional results – air conduction (AC) and bone conduction (BC) pure-tone average (PTA), air-bone gaps (ABG), sensorineural hearing loss (SNHL), ABG gain and ΔSNHL – were gender, age, case type (unilateral vs. bilateral), ear side (right vs. left), pregnancy, vascular disease and family history of otosclerosis. The audiometric variables were preoperative AC- and BC-PTA, SNHL and ABG. Results: Univariate logistic regression analysis showed that the probability of obtaining a ≥10 dB gain is significantly affected by the following factors: age 0.05). Nevertheless, multivariate logistic regression analysis maintained a statistically significant correlation only between gain ≥10 dB and both preoperative ABG ≥30 dB and age Conclusions: The accurate knowledge of predictive factors is a valuable tool that permits the surgeon to plan surgery with a better case selection as well as assisting in counseling the patient with regard to the likelihood of success of the procedure.

Tinnitus can be defined as a phantom sensation in the absence of an external sound. In our study, we evaluated the effect of cochlear implant on tinnitus evolution. Among adult, postlingually deaf patients who underwent cochlear implantation at our clinic, we selected 20 subjects with pre-implantation tinnitus (group A) and 10 subjects without pre-implantation tinnitus (group B). Pre- and post-surgery tinnitus was assessed through two questionnaires: the first one dealing with tinnitus characteristics and psychosocial impact, and the second one represented by THI, an internationally validated score of evaluation of the effects of tinnitus on patient's emotions and activities of daily living. None of the patients belonging to group B developed tinnitus after surgery. As for group A, 40% of patients declared suppression of tinnitus, 30% attenuation of tinnitus after surgery, 25% reported tinnitus was unchanged and 5% reported worsening of tinnitus. In the nine patients with bilateral tinnitus (45%), after implantation tinnitus disappeared from both sides in four patients and attenuated bilaterally in four patients. A comparison between pre- and post-implantation THI scores showed decreased score in 65% of cases, unchanged score in 30% and increased score in 5%. The beneficial effect of cochlear implant on tinnitus, reported by a majority of patients, could be due to acoustic masking, to direct electrical stimulation of the acoustic nerve, and above all to a possible cochlear implantation dependent reorganization of the central auditory pathways and associative cerebral areas. In the light of these results, the authors propose (1) to include tinnitus in the selection criteria of which ear to implant; (2) to consider implantation eligibility for patients with bilateral severe hearing loss associated with severe tinnitus; and (3) to inform patients about the small risk of post-operative tinnitus worsening.

The aim of our study was to evaluate the functional results of stapes surgery and to compare the effectiveness of small fenestra stapedotomy with that of total stapedectomy in improving hearing in patients affected by otosclerosis.Three hundred and fifty-seven consecutive ears, in 265 patients affected by otosclerosis, underwent surgery. All cases underwent either primary small fenestra stapedotomy (group A, 196/357, 54.91 per cent) or stapedectomy (group B, 161/357, 45.09 per cent). After surgery, 256/357 (71.71 per cent) cases showed a 0–20 dB gap. There were no significant differences in hearing results between the two groups at either early or late post-operative assessment. The mean post-operative pure tone average and air–bone gap results were slightly greater for group B than for group A, at both early and late post-operative assessments, but these differences were not statistically significant. Therefore, in group A, the mean pure tone average at 4 kHz significantly improved, from 56.60 to 47.66 dB at early post-operative assessment and to 52.98 dB at late post-operative assessment.Our study suggests that the technique of microtomy of the oval window is able to improve hearing results especially at high frequencies.

To assess and compare the functional results obtained by means of multiple-shot Erbium: yttrium-aluminum-garnet (Er:YAG) laser to those obtained using "one-shot" CO(2) laser stapedotomy in patients affected by otosclerosis. A retrospective case review was performed. Of the total number of 123 patients (114 ears) who underwent primary small-fenestra stapedotomy from January 2006 to September 2008, seven patients who received multiple-shot laser CO(2) stapedotomy were excluded from the study. The remaining 116 patients (104 ears) were sorted, and "one-shot" CO(2) laser stapedotomy (group A) was performed in 35/104 and Er:YAG laser stapedotomy (group B) in 69/104. After surgery, air conduction-pure tone average (AC-PTA) and air-bone gap (ABG) improved significantly in both groups, whereas sensorineural hearing loss (SNHL) and bone conduction (BC)-PTA did not change in both the groups. In group A, the postoperative ABG was significantly better (12.63 vs. 14.86 dB). Moreover, after "one-shot" stapedotomy, the AC-PTA significantly improved in all tested frequencies. On the contrary, in group B the AC-PTA improved significantly only in two frequencies (0.5 and 1 kHz). Consistent with previous reports, our findings confirm that laser stapedotomy is a safe and effective surgery, regardless of the technique. Based on our functional results, the "one-shot" CO(2) laser technique seems to be associated with a significantly better postoperative ABG if compared to Er:YAG laser stapedotomy.

Tinnitus is a common symptom which often becomes disabling, affecting the emotional and psychosocial dimensions of life. There are many reports describing tinnitus suppression or attenuation through electrical stimulation of the ear, provided either by cochlear implants or by transtympanic stimulation. Our study project aims to assess the effects of electrical promontory stimulation (EPS) on persistent disabling tinnitus. We enrolled 11 patients affected by postlingual monoaural or binaural profound hearing loss and disabling tinnitus in the worse ear. EPS was performed with direct continuous positive current delivered by an active platinum-iridium needle electrode connected to a promontory stimulator device. The short-term effect on tinnitus was assessed during and immediately after the stimulation. Long-term effects were estimated after one month by comparing pre- and post-EPS Tinnitus Handicap Inventory (THI) scores. Immediately after EPS, five patients (45.4%) reported complete suppression and four (36.4%) reported attenuation of tinnitus. Two patients (18.2%) said it was unchanged. After one month, the THI score was reduced in five patients (45.4%) and remained unchanged in the other six patients (54.6%). The beneficial effects of EPS on tinnitus might be explained by interference with tinnitus generating circuits such as the dorsal cochlear nucleus and the inferior colliculus and by modification of cortical activity. EPS is to be considered a worthwhile attempt at tinnitus suppression, and could help select candidates for the positioning of an implantable electrical stimulator that might provide longer-term beneficial effect on tinnitus.

Mutations in GJB2 gene are the most common cause of genetic deafness. More than 100 mutations have been described. The aim of this work is to describe the personal experience in genetic hearing loss, investigating the audiological and genetical characteristics of Cx26 deafness and correlating genotype and phenotype. We performed audiological and genetical evaluation in 154 patients affected by non-syndromic deafness of different degree. All patients showed a bilateral symmetrical sensorineural hearing loss. From the genetical analysis 127 probands resulted as negatives while 27 as positives (51.8% homozygous for 35 delG, 14.8% compound heterozygosis and 33.3% single mutation); 7.5% of patients had a mild deafness, 37% moderate, 33.3% severe and 22.2% profound. The c.35 delG mutation was detected in 66.6% of patients. Three mutations were found in compound heterozygosis with 35 delG, six different single mutations already described, and a new mutation S138G were also found. Correlation between genotype and phenotype confirmed the high variability of hearing loss.

OBJECTIVES We investigated the association of idiopathic sudden sensorineural hearing loss (ISSNHL) with coenzyme Q (CoQ) and cardiovascular risk factors. STUDY DESIGN A prospective study. SETTING Hospital center. PATIENTS Thirty Italian patients with ISSNHL and 60 healthy Italian subjects. INTERVENTION Diagnostic. MAIN OUTCOME MEASURES Evaluation of serum CoQ levels and cardiovascular risk factors (total cholesterol, low-density lipoprotein [LDL], homocysteine [HCY]). The results were compared with variance analysis and Student's t test. Univariate and multivariate analysis were used to evaluate the association between ISSNHL and CoQ, total cholesterol, LDL, and HCY levels. RESULTS In our series, we found a significant association between ISSNHL and high total cholesterol (p < 0.05), high LDL (p = 0.021), and low CoQ (p < 0.05) levels. We did not find a significant association between ISSNHL and HCY levels. In the univariate analysis, low levels of CoQ, high levels of total cholesterol, and LDL were found to be significantly associated with ISSNHL. In the multivariate analysis, only high levels of total cholesterol and low levels of CoQ remained significantly associated with a high risk of sudden sensorineural hearing loss. CONCLUSION The studies regarding the role of cardiovascular risk factors in ISSNHL are not conclusive. This is the first report regarding the association of ISSNHL and low serum levels of the antioxidant CoQ. Further studies are needed to investigate the role of antioxidants, including CoQ, in ISSNHL.

Preservation of residual hearing should be a desirable outcome of implant surgery. Prevention of neural degeneration due to loss of residual hair cells, together with the continuous progress in cochlear implant technology should be able to preserve cochlear integrity as well as possible. The degree of hearing preservation may vary depending on surgical approach, maximum insertion depth and other factors not uniformly considered to date. The aim of this retrospective case controlled study is to evaluate residual hearing after cochlear implant surgery. In particular, we analyzed data obtained with use of two different kinds of electrode arrays, with and without rigid introductor (stylet). We report the results on 37 patients with measurable preoperative hearing thresholds, mean age of 28 years (5-70 years), having the following implants: seven Advanced Bionics, four Med-El, 24 Cochlear, two MXM; 19 of them were performed using the stylet and the other 18 without it. A minimally invasive surgical approach was performed with a short retroauricular incision and a 1.2 mm cochleostomy. A complete electrode array insertion was obtained in all patients. Responses to pure-tone stimuli were measured for each ear in pre-implantation conditions and 3-12 months after surgery. After implantation 14 patients (38%) showed no hearing threshold variation, 29 (78%) maintained an appreciable hearing threshold level in the implanted ear, 8 (22%) had a total loss of residual hearing. Median increases of threshold levels were, in all 37 studied patients, 5, 10, 10 and 5 dB HL, respectively, for 125, 250, 500 and 1 kHz. For the 18 patients having implants without the stylet median increases of threshold levels were 0, 10, 5 and 7 dB HL; in the stylet group, they were 10, 5, 5 and 10 dB HL. On a comparison between the stylet and the non-stylet group, no significant differences in mean hearing threshold worsening were found. Data seem to suggest that cochlear function is less sensitive to mechanical trauma during implant surgery than was thought. Besides, electrode array stiffness seems not to influence preservation of cochlear residual functional integrity. Finally, the authors hypothesize a direct spiral ganglion activation under strong mechanical stimulation.

Implant and reimplantation surgery should be carried out with preservation of residual hearing. The aim of this study is to evaluate the effects of such a surgery on hearing threshold. We report the results on 40 patients, 20 males and 20 females, aged between 5 and 70 (mean 29) years, 16 pre-verbal and 24 post-verbal, with measurable pre-operative auditory thresholds. We used the following implants: Advanced Bionics, Med-El, Cochlear, and MXM Digisonic. Four of the patients underwent cochlear reimplantation owing to device failure. A complete insertion was obtained in all patients. Responses to pure-tone stimuli were evaluated in each ear in pre-implant conditions and 3 months after cochlear implant or reimplantation. The explantation was performed with minimal cochlear trauma and preservation of the explanted electrode integrity. 35% showed no change of the hearing threshold, 45% showed a slight worsening of the hearing threshold level in the implanted ear, and 20% had a total loss of residual hearing. Median increases of threshold levels were 10, 5, 10 and 3 dB HL respectively for 125, 250, 500 and 1 kHz. In the group of 4 patients who underwent cochlear reimplantation, 2 showed no variation of the hearing threshold, 1 preserved an appreciable hearing threshold, and 1 had a total loss of residual hearing. The data seem to suggest that hearing function is rather resistant to mechanical trauma during implant and reimplant surgery; the authors hypothesize a role for direct spiral ganglion activation under intense mechanical stimulation.

Poor pitch resolution has been shown to have negative implications for speech and music perception in implanted patients. Surprisingly, works on the subject have not focused much on the impact that the non-correspondence between frequencies allocated to electrodes and perceived frequencies could have on speech and music perception. The aim of the present study is to investigate the correlation between pitch mismatch and speech performance with the implant, and to ascertain the effects of mismatch correction through a mapping function making a personalized frequency reallocation possible. We studied ten postlingually deaf adult patients with detectable bilateral residual hearing, implanted in our Clinic with Cochlear® Nucleus devices. In each test session, we asked the patients to find the best match between the pitch elicited by the residual ipsilateral and contralateral pure tones and the pitch elicited by stimulation of electrodes. We also assessed patients’ vowel and consonant recognition performance. Finally, in the only implanted patient in our clinic who had bilateral residual hearing and used a Digisonic DX10/C® device, which makes manual electrode-by-electrode frequency reallocation possible, we modified electrode-assigned frequency ranges on the basis of the pitch matching test results. We found that in none of the studied patients, the electric-to-acoustic pitch matching corresponds to the theoretical assignment pattern. A very strong correlation was detected between the electric-to-acoustic pitch mismatch and patient’s speech performance. In the Digisonic® patient, a remarkable improvement in all phoneme recognition scores was obtained 1 month after frequency reallocation. In the light of our results, we propose to assess, whenever possible, any frequency-to-electrode mismatch in all implanted patients, and correct it through mapping programs allowing manual frequency reallocation for the pitch-matched electrodes, and automated allocation of the non-tested electrodes. Cochlear implantation should therefore be proposed when residuals for all frequencies are still present, at least in one ear, so as to allow optimal alignment between allocated and subjectively perceived frequencies.