Search Results (10)

The incomplete partition type III is a severe cochlear malformation present in X-linked deafness. It is a rare, non-syndromic cause of severe to profound mixed hearing loss, often progressive. The complete absence of bony modiolus and the wide communication between the cochlea and the internal auditory canal make cochlear implantation challenging, with still no consensus on the management of these patients. To the best of our knowledge, no results have ever been published in the literature on the treatment of these patients with hybrid stimulation (bone and air). We present three cases in which this hybrid stimulation gave better audiological results then air stimulation alone. A literature review on audiological results of the current treatment options in children affected by IPIII malformation was conducted independently by two researchers. Ethical considerations on the treatment of these patients were conducted by the Bioethics department of the University of Insubria. In two of the patients, the bone–air stimulation, associated with prosthetic–cognitive rehabilitation, meant that surgery was avoided, obtaining similar communication performances of those present in the literature. We believe that, when the bone threshold appears partially preserved, a stimulation through the bone or hybrid modality, such as the Varese B.A.S. stimulation, should be attempted. Full article

The main aim of this study was to assess the clinical effect of steroids (dexamethasone and prednisone) on hearing preservation in patients who underwent cochlear implantation with different cochlear implant systems (Oticon^®, Advanced Bionics^®, Med-El^®). 147 adult patients met the inclusion criteria and were enrolled to the study and divided into three groups depending on the brand of cochlear implant they received and participated in all follow-up visits regularly. They were also randomly divided into three subgroups depending on the steroid administration regime: (1) intravenous dexamethasone (0.1 mg/kg body weight twice a day for three days); (2) combined intravenous and oral steroids (dexamethasone 0.1 mg/kg body weight twice a day plus prednisone 1 mg/kg weight once a day); and (3) no steroids (control group). The results were measured by pure tone audiometry (PTA) at three time points: (i) before implantation, (ii) at processor activation, and (iii) 12 months after activation. A hearing preservation (HP) figure was also calculated by comparing the preoperative results and the results after 12 months. Further measures collected were electrode impedance and hearing threshold in the non-operated ear. The highest HP measures (partial and complete) were obtained in the subgroups who were given steroids. Of the 102 patients given steroids, HP was partial or complete in 63 of them (62%). In comparison, partial or complete HP was achieved in only 15 patients out of 45 (33%) who were not given steroids. There were differences between the three cochlear implant groups, with the Med-El and Advanced Bionics groups performing better than the Oticon group (45% and 43% of the former two groups achieved partial or complete HP compared to 20% in the latter). Hearing thresholds in the non-operated ear were stable over 12 months. Generally, impedance was slightly lower in the 12 month follow-up in comparison with the activation period, with the exception of the Oticon group. (4) Conclusions: Pharmacological treatment with steroids in patients undergoing cochlear implantation helps to preserve residual hearing. Full article

This study aimed to discover expert opinion on the surgical techniques and materials most likely to achieve maximum postoperative residual hearing preservation in cochlear implant (CI) surgery and to determine how these opinions have changed since 2010. A previously published questionnaire used in a study published in 2010 was adapted and expanded. The questionnaire was distributed to an international group of experienced CI surgeons. Present results were compared, via descriptive statistics, to those from the 2010 survey. Eighteen surgeons completed the questionnaire. Respondents clearly favored the following: round window insertion, slow array insertion, and the peri- and postoperative use of systematic antibiotics. Insertion depth was regarded as important, and electrode arrays less likely to induce trauma were preferred. The usefulness of dedicated soft-surgery training was also recognized. A lack of agreement was found on whether the middle ear cavity should be flushed with a non-aminoglycoside antibiotic solution or whether a sheath or insertion tube should be used to avoid contaminating the array with blood or bone dust. In conclusion, this paper demonstrates how beliefs about CI soft surgery have changed since 2010 and shows areas of current consensus and disagreement. Full article

The pathophysiological mechanisms of noise-induced hearing loss remain unknown. Identifying biomarkers of noise-induced hearing loss may increase the understanding of pathophysiological mechanisms of deafness, allow for a more precise diagnosis, and inform personalized treatment. Emerging techniques such as metabolomics can help to identify these biomarkers. The objective of the present study was to investigate immediate-early changes in the perilymph metabolome following acoustic trauma. Metabolomic analysis was performed using liquid chromatography coupled to mass spectrophotometry to analyze metabolic changes in perilymph associated with noise-induced hearing loss. Sheep (n = 6) were exposed to a noise designed to induce substantial hearing loss. Perilymph was collected before and after acoustic trauma. Data were analyzed using univariate analysis and a supervised multivariate analysis based on partial least squares discriminant analysis. A metabolomic analysis showed an abundance of 213 metabolites. Four metabolites were significantly changed following acoustic trauma (Urocanate (p = 0.004, FC = 0.48), S-(5’-Adenosyl)-L-Homocysteine (p = 0.06, FC = 2.32), Trigonelline (p = 0.06, FC = 0.46) and N-Acetyl-L-Leucine (p = 0.09, FC = 2.02)). The approach allowed for the identification of new metabolites and metabolic pathways involved with acoustic trauma that were associated with auditory impairment (nerve damage, mechanical destruction, and oxidative stress). The results suggest that metabolomics provides a powerful approach to characterize inner ear metabolites which may lead to identification of new therapies and therapeutic targets. Full article

Hearing is an important human sense for communicating and connecting with others. Partial deafness (PD) is a common hearing problem, in which there is a down-sloping audiogram. In this study, we apply a practical system for classifying PD patients, used for treatment purposes, to distinguish two groups of patients: one with almost normal hearing thresholds at low frequencies (PDT-EC, n = 20), and a second group with poorer thresholds at those same low frequencies (PDT-EAS, n = 20). After performing comprehensive genetic testing with a panel of 237 genes, we found that genetic factors can explain a significant proportion of both PDT-EC and PDT-EAS hearing losses, accounting, respectively, for approx. one-fifth and one-half of all the cases in our cohort. Most of the causative variants were located in dominant and recessive genes previously linked to PD, but more than half of the variants were novel. Among the contributors to PDT-EC we identified OSBPL2 and SYNE4, two relatively new hereditary hearing loss genes with a low publication profile. Our study revealed that, for all PD patients, a postlingual hearing loss more severe in the low-frequency range is associated with a higher detection rate of causative variants. Isolating a genetic cause of PD is important in terms of prognosis, therapeutic effectiveness, and risk of recurrence. Full article

(1) Background: Current indications for cochlear implants (CIs) have expanded to include patients with appreciable low-frequency hearing. However, longitudinal results indicate that only one-third of these recipients retain full hearing preservation. In another words, the remaining two-thirds lose this facility either partially or fully. This points to the need to better understand the impact of cochlear implantation on cochlear integrity. Intracochlear electrocochleography (ECochG) involves the recording of electrical potentials generated in the inner ear in response to acoustic stimuli, and previous studies have shown that these potentials give an indication of residual inner ear function. Aim of the research: The aim is to monitor intracochlear ECochG during CI surgery and gain a better understanding of how the implant impacted inner ear function. A newly developed SPL Chirp was used for stimulation. (2) Methods: Intracochlear ECochG signals were measured in a subject with residual preoperative low-frequency hearing, while an electrode array was introduced into the cochlea and was continued until the round window was sealed. Afterwards, surgical events were reviewed with the surgeon; preoperative and postoperative radiological data and hearing thresholds were also evaluated. (3) Conclusions: Real-time intraoperative monitoring, with multifrequency evaluation and video recording, has the potential to allow surgeons and audiologists to continuously assess cochlear function. ECochG monitoring may be a useful tool during cochlear implantation to gain frequency-specific information on the status of the patient’s hearing, assisting surgeons to lower hearing trauma during the operation. Full article

(1) Background: The main aim of this study was to assess the clinical effectiveness of two different schemes of administration of steroids ((1) dexamethasone administered intravenously in comparison with (2) combination of steroid treatments: orally administered prednisone and intravenously administered dexamethasone) in comparison with a control group (no steroid administration) on hearing preservation (HP) in patients who underwent an Advanced Bionics cochlear implantation. (2) Methods: Thirty-five adult patients met the inclusion criteria. All patients were randomly divided into three subgroups depending on the scheme of steroid administration: (1) the first subgroup with only intravenously administered dexamethasone (0.1 mg per kg body weight twice a day for three days), (2) the second subgroup with a combination of methods of administration of steroids (intravenous and oral steroid therapy (dexamethasone, 0.1 mg/kg body weight twice a day plus prednisone, 1 mg/kg weight once a day for three days before surgery and after administration of dexamethasone (4th, 5th, 6th day) and after this time the dose of prednisone was reduced)) and (3) the third subgroup without steroid therapy (control group). The results were measured by pure tone audiometry (PTA) in three periods: (1) before implantation, (2) during activation of the processor (one month after implantation), and (3) 12 months after activation. Patients’ hearing thresholds before implantation were on average 82 dB HL, 77 dB HL, and 88 dB HL, respectively. (3) Results: The majority of the patients from the first subgroup had hearing preserved partially (77.8%). A similar result was observed in the second study group (oral + i.v.) (partial hearing preservation was found in 61.5% of the participants). The opposite was true in the control group; a plurality of control patients (38.5%) had no measurable hearing 12 months after the activation of the processor. (4) Conclusions: Pharmacological treatment consisting of the administration of steroids in patients who had undergone cochlear implantation with the Advanced Bionics HiRes Ultra 3D cochlear implant system may be beneficial for preserving residual hearing in patients. Full article

(1) Background: The novel coronavirus COVID-19 has been recognized by the World Health Organization as a public health emergency of international concern and has caused cancellation of elective cochlear implantation in many countries. This article sets out our experience with resuming cochlear implant (CI) surgery under COVID-19 conditions over a period of 3 months. In addition, early results of hearing preservation (HP) after CI surgery are presented; (2) Methods: We adopted epidemic management policies and procedures according to the National Consultant for Infectious Diseases recommendations. During preoperative visits, all patients were tested for COVID-19 with a RT-PCR test. One month postoperatively, HP values in the Partial Deafness Treatment (PDT) group of patients was established using the HEARRING group formula; (3) Results: Between January and March 2021, we performed 312 CI procedures in adult and pediatric patients. Of these, none were subsequently re-admitted to hospital and found to be COVID-19 positive. Postoperative audiometric results showed that complete or partial HP was achieved in more than half the PDT patients; (4) Conclusion: Cochlear implantation during the coronavirus disease pandemic is essential and, with careful planning, is perfectly feasible. Full article

Background: A prospective clinical study was conducted to investigate whether two different pharmacotherapy strategies of steroid administration impact hearing preservation in adult patients who underwent cochlear implantation with the Oticon Medical Neuro cochlear implant system. Methods: Twenty nine adult participants were included. Pure tone audiometry was performed before implantation, during processor activation and 12 months after activation. There were three treatment groups: (1) intravenous steroid therapy (standard steroid therapy with dexamethasone administrated intravenously at the dose 0.1 mg/kg body mass twice a day); (2) combined oral and intravenous steroid therapy (extended steroid therapy with dexamethasone administrated intravenously at the dose 0.1 mg/kg b.m. twice a day and prednisone (orally) at the dose 1 mg/kg body mass/24 h), and (3) no steroid therapy (a control group). Patients’ hearing thresholds before implantation were on average 103 dB HL, 89 dB HL, and 93 dB HL, respectively. Results: Deterioration of hearing thresholds was observed in all three patients’ groups. Twelve months after surgery the patients with and without steroid therapy had similar hearing thresholds. Conclusions: The steroid regimen used in this study did not play a significant role in patients with non-functional residual hearing, who underwent cochlear implantation with the Oticon Medical Neuro cochlear implant system. Full article

Both brainstem auditory evoked potentials (BAEP) and audiometry play a crucial role in neuro-oncological treatment decisions in Neurofibromatosis Type 2 associated (NF2) vestibular schwannoma (VS) as hearing preservation is the major goal. In this study, we investigated the risk of immediate postoperative hearing deterioration (>15 dB and/or 15% loss in pure-tone average [PTA]/ speech discrimination score [SDS] in a cohort of 100 operated VS (ears) in 72 NF2 patients by retrospective analysis of pre- and postoperative hearing data (PTA, SDS, American Association of Otolaryngology–Head and Neck Surgery [AAO-HNS], and brainstem auditory evoked potential [BAEP] class) taking into account relevant influencing factors, particularly preoperative audiometry and BAEP status and the extent of resection. Immediately after surgery, the hearing was preserved in 73% of ears and approximately ~60% of ears kept their hearing classes. Preoperative BAEP (p = 0.015) and resection amount (p = 0.048) significantly influenced postoperative hearing outcome. The prediction model for postoperative hearing deterioration/loss between preoperative BAEP and AAO-HNS class showed increased risk by increasing BAEP class. Twenty-one tumors/ears were identified with large BAEP and AAO-HNS class discrepancies (≥2 points) and were associated with a high (48–100%) risk of deafness after surgery in ears with preoperative available hearing. Overall, the results were heterogeneous but the better both BAEP and audiometry class before surgery, the higher the chance of hearing maintenance afterwards. Large resection amounts (e.g., 100% risk in near-total resections) exhibit a significant (p < 0.05) higher risk compared to smaller amounts (e.g., 10/20% in laser-coagulated/partially resected tumors). Our results emphasized the indispensable role of both hearing monitoring in form of audiometry and neurophysiology (BAEP) in the pre-and perioperative monitoring of NF2-associated VS. Both BAEP and audiometry are good prognostic markers for the postoperative hearing outcome. The extent of resection should be strictly guided by and adjusted to the intraoperative neurophysiological monitoring. Full article