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.

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.

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.

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.

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.

Hearing preservation surgery: cochleostomy or round window approach? A systematic review.

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.

Combined posterior tympanotomy/endomeatal access in cochlear implantation.

Alternative techniques for cochlear implant surgery have been described, such as endomeatal- and suprameatal-alone approaches, without traditional posterior tympanotomy. A combined posterior tympanotomy/endomeatal approach is proposed as a way to enhance surgical safety and effectiveness. 64 patients, 34 men and 30 women, mean age 28 (range 1-81 years), 26 pre-verbal and 38 post-verbal, were submitted to cochlear implantation, 32 by means of the described combined posterior tympanotomy/endomeatal approach and 32 with traditional posterior tympanotomy-alone approach. Good anatomic and functional results were observed, with intraoperative improvements in visibility and accessibility of cochleostomy site in difficult cases without any complication, such as tympanic membrane perforation, external canal skin lesions, or extrusion at 12 months. No significant differences were found with traditional technique complication rates excepting for partial insertion data, with statistically significant better results with combined access. The combined posterior tympanotomy/endomeatal approach facilitates the array insertion in conditions of bad exposition/accessibility of promontory and round window. Moreover, this double-way access hinders an incomplete or incorrect positioning. It should be always considered as an alternative to the traditional posterior tympanotomy of one-way access.

Stability of osseointegrated bone conduction systems in children: a pilot study.

OBJECTIVES: To obtain objective data of bone conduction implant stability and osseointegration in children; to compare in pediatric subjects the stability and osseointegration of the novel TiOblast-coated implant system (BI300) to the previous generation, as-machined model. STUDY DESIGN: Multicenter, controlled, nonrandomized, longitudinal, prospective study. SETTING: Tertiary referral center. LEVEL OF EVIDENCE: IIIb PATIENTS: Ten subjects were enrolled, aged younger than 16 years and without comorbidities that negatively affect osseointegration. All patients were implanted "single stage": 5 received the previous generation, pure titanium fixture (control group), and 5 were implanted the BI300, TiOblast-coated fixture (test group). INTERVENTIONS: Measurement of implant stability and osseointegration. MAIN OUTCOME MEASURE(S): Implant stability and osseointegration as measured by means of resonance frequency analysis intraoperatively, at 1 week, 2 weeks, 1 month, and every month till processor loading 6 months after surgery. RESULTS: BI300 is, on average, more stable than the control fixture, both intraoperatively and over time till processor loading. Over the 6 months' follow-up, a stability increase was observed with both models, although it resulted statistically not significant. CONCLUSION: The BI300 implant system has a greater primary stability as compared with the previous generation model, but its faster osseointegration could not be definitely verified. It is reasonable to assume that the BI300 fixture will enable surgeons to perform single-stage surgery more safely and with a lower failure rate. The possibility of an earlier processor loading remains to be confirmed in a greater sample.