The Role of Endoscopic Assistance in Surgery for Pediatric Cholesteatoma in Reducing Residual and Recurrent Disease.

The primary aim of this study was to evaluate long-term recurrent and residual disease after surgery for acquired cholesteatoma in children according to surgical approach. A total of 71 interventions performed on 67 pediatric patients were included in the study. Canal wall-up tympanomastoidectomy (CWUT) was performed in 31 ears (13 with endoscopic assistance), a transcanal esclusive endoscopic approach (TEEA) was used in 22, and canal wall-down tympanomastoidectomy (CWDT) was performed in 18. Overall, the cholesteatoma relapse rate estimated by the Kaplan-Meier method was 47 ± 6% at 12 years; the recurrent cholesteatoma rate was 28 ± 6% and the residual cholesteatoma rate was 26 ± 5%. The relapse rate according to surgical approach was 33 ± 11% for CWDT, 60 ± 9% for CWUT, and 40 ± 11% for TEEA (p = 0.04). The difference for recurrent disease was no recurrent disease for CWDT, 42 ± 9% for CWUT, and 32 ± 11% for TEEA (p = 0.01). The residual disease rate was significantly reduced with endoscopy: 42 ± 8% without endoscopy vs. 9 ± 5% with (p = 0.003). CWDT can still be considered in primary surgery in case of extensive cholesteatomas and small mastoid with poor pneumatization. TEEA can be recommended for small cholesteatoma not extending to the mastoid to reduce morbidity. Endoscopic assistance seems useful to reduce residual disease in CWUT, whereas it does not have a significant impact on preventing recurrent disease.

Osteoneogenesis at the Round Window: A Possible Cause of Cochlear Implant Failure?

Surgery for cochlear implant is a traumatic procedure, with inflammatory responses leading to immediate and delayed intracochlear changes, resulting in newly formed fibrous and bony tissue. This newly formed tissue is thought to affect speech perception with cochlear implants and can also play a role in causing device malfunctioning and soft failures. We present a case of left cochlear implant explantation and reimplantation in a 15-year-old girl, who experienced deterioration of speech perception and device failure associated with osteoneogenesis of the round window, which could represent a cause of cochlear implant failure. To avoid surgical trauma of the cochlear lateral wall, enlarged round window insertion rather than a cochleostomy, soft surgical techniques, and the application of steroids are all important issues to prevent new tissue formation, although special attention should also be given to the trauma of round window borders.

Presentation, Management, and Hearing Outcomes of Labyrinthine Fistula Secondary to Cholesteatoma: A Systematic Review and Meta-analysis.

OBJECTIVE: The current study systematically reviewed the literature to compare auditory outcomes of patients treated for labyrinthine fistula (LF) based on characteristics of disease and surgical management. DATABASES REVIEWED: PubMed, Scopus, Web of Science. METHODS: Original series (at least five cases) published from 2000 reporting management and hearing results of LF secondary to cholesteatoma were included. Proportion and odds-ratio (OR) meta-analyses were conducted through inverse variance random-effects models based on logit transformation. RESULTS: The prevalence of LF is estimated to be 7% (95% confidence interval [CI], 5-9%). Fistulae involving the lateral semicircular canal (90%; 95% CI, 87-93%) and larger than 2 mm (53%; 95% CI, 43-64%) were common, whereas membranous involvement was less frequent (20%; 95% CI, 12-30%). Complete removal of the cholesteatoma matrix overlying the LF was mostly applied. Bone conduction (BC) preservation was frequently achieved (81%; 95% CI, 76-85%); new-onset postoperative anacusis was rarely reported (5%; 95% CI, 4-8%). A higher chance of BC preservation was associated with sparing the perilymphatic space (OR, 4.67; 95% CI, 1.26-17.37) or membranous labyrinth (OR, 4.56; 95% CI, 2.33-8.93), exclusive lateral semicircular canal involvement (OR, 3.52; 95% CI, 1.32-9.38), smaller size (<2 mm; OR, 3.03; 95% CI, 1.24-7.40), and intravenous steroid infusion (OR, 7.87; 95% CI, 2.34-26.42). CONCLUSION: LF occurs in a significant proportion of patients with cholesteatoma. In the past two decades, complete removal of the cholesteatoma matrix followed by immediate sealing has been favored, supported by the high proportion of BC preservation. Hearing preservation depends primarily on characteristics of the LF, and specific surgical strategies should be pursued. Intraoperative and postoperative intravenous steroid infusion is recommended.

Balance Disorders in Children and Adolescents.

The prevalence of balance disorders in children and adolescents is extremely variable [...].

Endoscopic transcanal tympanoplasty type I in children: Evolving experience in tragus perichondrium vs. acellular porcine small intestinal sub-mucosa grafts.

OBJECTIVES: Endoscopic trans-canal tympanoplasty type I (ETT) is gradually diffusing worldwide. It mainly allows less invasive surgery in children with respect to a microscope approach by avoiding post-auricular access. The aim of this study is to illustrate our experience in endoscopic reconstruction of tympanic membrane, using autologous tragus perichondrium (TP) and non-autologous acellular porcine small intestinal sub-mucosa (SIS) as grafts. METHODS: Between January 2011 and December 2020, the results of a prospective non-randomized series of consecutive ETT were analyzed. The primary outcome was closure rate at 6 months and secondary outcomes are closure rates associated with age, size of perforation, type of perforation and middle ear status, presence of myringosclerosis, type of graft, status of contralateral ear, adenoidectomy and pre-postoperative ABG change. Statistical analysis was performed using the SPSS statistical package. RESULTS: One hundred and sixteen consecutive procedures, mean age 9.4 years (range 4-17 years), were evaluated. TP and SIS grafts were used in 65 (56%) and 51 (44%) procedures, respectively. Mean duration of surgical procedure was 53 ± 21 min for SIS and 77 ± 18 min for TP (P = 0.001) Total graft intake was 82.8%; TP and SIS intake were 86.2% and 78.4% (P = 0.3), respectively. Graft intake w.r.t. in age stratified age groups was not statistically significant. Average preoperative and postoperative air-bone gap was 12.1 ± 7.6 dB and 5.5 ± 3.8 dB, respectively (P = 0.001). The difference in closure rates was not significant. Neither intra- nor postoperative complications were observed. CONCLUSIONS: In children, ETT is an applicable and less invasive technique compared to the microscope and offers less morbidity. The use of SIS contributes additional less invasiveness to endoscopic surgery by avoiding tragus harvesting with a comparable success rate and granting significantly less surgical duration.

Salvage surgery in recurrent sinonasal cancers: Proposal for a prognostic model based on clinicopathologic and treatment-related parameters.

BACKGROUND: Evidence on survival and major prognosticators after salvage surgery in recurrent sinonasal cancers (SNC) is limited. METHODS: A retrospective, single-center study of recurrent SNC treated with salvage surgery between 1997 and 2019 was conducted. Univariate and multivariable analyses were performed to define a prognostic score for overall survival (OS). RESULTS: One hundred and eighteen patients were included. Recurrent SNC originated mostly in the naso-ethmoidal box (67.8%) and were mainly epithelial (76.2%), high-grade (49.2%), and locally advanced (rpT4, 60.1%) malignancies. Negative margins were achieved in 56.6% of cases. Two- and 5-year OS were 71.7% and 56%, respectively. The prognostic model included treatment modality for primary tumor, histology, rpT class, margin status, perineural invasion, and adjuvant radiotherapy and stratified patients into three prognostic groups (5-year OS: 84.4%, 44.9%, and 0%, respectively). CONCLUSIONS: Treatment of recurrent SNC can result in good long-term survival estimates with limited morbidity. Our score can provide excellent prognostic stratification.

Long-Term Surgical Results of Cortical Mastoid Bone Osteomas.

BACKGROUND: Though osteomas can commonly arise in the cranial bones, an extra canalicular mastoid bone location is a rare entity with less than 200 cases described to date. We present three cases of cortical mastoid bone osteomas and compare them with cases presented in the literature. METHODS: In this study, we used a retrospective chart analysis. RESULTS: All three patients presented after years of progressively increasing postauricular swelling without symptoms. Temporal bone non-contrast CT allowed accurate preoperative diagnosis. Surgical treatment was performed for cosmetic issues with minimal postoperative morbidity. Complete excision was achieved in all cases, and to date, there is no evidence of recurrence. CONCLUSIONS: Mastoid osteomas are rare benign slow-growing tumors. They usually present as a painless cosmetic disfigurement and are otherwise asymptomatic. Surgical excision is the treatment of choice when they cause esthetic discomfort or are symptomatic. Recurrences are infrequently reported.

When a Nontuberculous Mycobacterial Infection Reveals an Error of Immunity: A Single Center's Experience.

We present an algorithm that may be applied in case of a diagnosis of pediatric nontuberculous mycobacterial disease to identify the patients who may require an immunologic assessment to discover a possible underlying immune system defect predisposing to their nontuberculous mycobacterial infections.

Parotid pleomorphic and non-pleomorphic adenomas: a mono-institutional series of 512 patients.

PURPOSE: Pleomorphic adenoma (PA) is the most common benign parotid tumor, with a well-known propensity to recur. Many factors have been advocated as prognostic, but there is no consensus on how they affect local control. We studied how PA recurrence-free survival (RFS) may be affected by the most relevant risk factors in a time-to-event analysis, comparing them with those observed in a population of non-PA (NPA). METHODS: Patients undergoing parotidectomy for benign lesions between 2002 and 2018 in a single academic tertiary referral center were included. A description of patients, tumors, and treatment characteristics was performed, highlighting differences between PA and NPA. Analysis of PA RFS and relative risk factors was also conducted. RESULTS: Eight hundred fifty patients underwent parotidectomy for benign lesions, 455 (53.5%) for PA and 57 (6.7%) for NPA. Significant differences between PA and NPA were age at surgery, surgical procedure, and resection margins. Recurrence occurred in 3.1% of PA, with a median disease-free interval of 54 months. 2-, 5-, and 10-year RFS were 99.2, 98.5, and 93.9%, respectively. Age < 18 years (HR = 31.31, p < 0.001), intraoperative tumor spillage (HR = 6.57, p = 0.041), extensive pseudo-capsule interruption (HR = 5.85, p = 0.023), and resection margins < 1 mm (HR = 3.16, p = 0.085) were associated with RFS. CONCLUSION: Patients affected by NPA were significantly older and treated with more conservative surgical procedures compared to those with PA. In PA, younger age, major pseudo-capsule defects, and surgical margins were the most relevant factors affecting local control. These results confirm the importance of an appropriate surgical management and long-term follow-up in PA.

Exploratory tympanotomy in conductive hearing loss with normal pre-operative investigations.

Objective: To investigate whether patients with conductive hearing loss (CHL) and normal preoperative investigations may benefit from exploratory tympanotomy (ET) and tailored treatment performed according to intraoperative findings. Methods: Patients treated with ET for CHL with normal pre-operative otoscopy, tympanometry and CT scan from 2011 to 2019 were reviewed. Data regarding demographics, audiometry, intraoperative findings and surgery were collected and analysed to assess if they can predict post-operative air bone gap (ABG) closure and patient satisfaction. Results: Forty-eight cases were included. Mean ABG significantly reduced (p < 0.001) from preoperative (38.4 dB) to postoperative (14.8 dB). Post-operative ABG closure within 10 dB was observed in 20 cases (41.7%). Overall satisfaction was reported in 60% of cases. Stapes fixation was the most common diagnosis (47.9%) and significantly associated with lower post-operative ABG and higher satisfaction. Conclusions: In CHL with normal pre-operative investigations, ET represents the mainstay of treatment, even if audiological outcomes may widely vary. Intraoperative finding of stapes fixation (thus stapedotomy) ensures the best audiological and satisfaction outcomes.

Prevalence of Pediatric and Adolescent Balance Disorders: Analysis of a Mono-Institutional Series of 472 Patients.

(1) Background: To assess the prevalence and frequency distribution of balance disorders in children and adolescents to delineate the planning of a targeted clinical and instrumental diagnostic work-up; (2) Methods: Retrospective analysis of the clinical documentation of patients under 18 years suffering from balance disorders from 2010 to 2019. Detailed collection of clinical history, accurate clinical examination, including both nystagmus and vestibulospinal signs examinations, and specific instrumental testing were the basis of the diagnostic process. (3) Results: A total of 472 participants were included in the study. Vestibular loss (26.1%) was the most frequent cause of vertigo in children, followed by vestibular migraine (21.2%) and benign paroxysmal positional vertigo (10.2%). In 1.1% of patients, the cause of vertigo remained undefined; (4) Conclusions: The diagnostic process applied was effective in understanding the cause of balance disorders in most cases and prevents more complex and expensive investigations reserved for only a few selected cases.

Long-Term Lateral Semicircular Canal Function in Children with Cochlear Implants: Results of Video Head Impulse Test.

In children with profound deafness, bilateral cochlear implant (CI) is an effective, established procedure. However, its safety on vestibular function has recently been debated. The goal of this study is to evaluate the long-term lateral semicircular canal high-frequency vestibulo-oculomotor reflex (LSC HF VOR) in children with CI by video head impulse testing (vHIT). This is a cross-sectional study assessing a cohort of children who received either a unilateral (12) or a bilateral (12) cochlear implant (CI), compared with a control group of 12 normal-hearing children. No significant LSC HF VOR gain difference was found between CI users and controls. In the unilaterally implanted group, the LSC HF VOR gain measured in the "CI-ON" condition was significantly higher than in the "CI-OFF" condition, both in the implanted and in the non-implanted ear. In the bilaterally implanted group, the difference between the two conditions was not significant. Our results do not show any impairment of LSC HF VOR function in children with CI compared to normal-hearing children in the long-term period. This suggests that both unilateral and simultaneous/sequential bilateral CI are procedures that do not impair HF LSC long-term function when analyzed by vHIT.

Paranasal mucoceles in children with cystic fibrosis: Management of a not so rare clinical condition.

PURPOSE: Paranasal mucocele (PM) is reported as a complication in children with cystic fibrosis (CF) in up to 4% of patients. The objective of this study was to identify PMs in the personal large series of children with CF and to assess their diagnosis and treatment. MATERIAL AND METHODS: Medical records of children with CF and PM who were admitted and treated by means of endoscopic nasal surgery between 2004 and 2020 were retrospectively reviewed. RESULTS: Thirty-four patients were included in the study (mean age 7.7 years). CT scan of sinuses showed a total of 53 PMs. Nasal endoscopic findings suggestive for PM were present in almost 80% of patients. PMs were located in the maxillary, ethmoid, and sphenoid sinuses in 29/34 (85.3%, bilateral in 17 cases), 4/34 (11.8%) and 1/34 (2.9%) patients, respectively. Marsupialization of PMs was performed in all patients using an endoscopic transnasal approach. No complications were observed. Resolution of symptoms and normalization of the endoscopic nasal picture was evident in all patients. After a mean follow-up of 85 months, no recurrences were observed. CONCLUSIONS: To the best of our knowledge, this is the largest series of CF patients with PMs. Even if not frequently reported in the literature, PMs should not be considered an uncommon finding in patients affected by CF. Routine nasal endoscopy is mandatory to favor early diagnosis. Endoscopic transnasal marsupialization represents the gold standard of care for patients with CF and PM(s).

Benign Positional Paroxysmal Vertigo in Children.

The aim of this study was to assess the prevalence and analyze clinical parameters of benign positional paroxysmal vertigo (BPPV) in a pediatric age. A cohort of 423 children under the age of 15 (median age 11. interquartile range 9-13) was submitted to vestibular assessment for balance disorders. Dix-Hallpike and Roll-Supine tests were performed to look for positioning nystagmus using video-infrared goggles. BPPV was found in 43 of 423 children evaluated for balance disorders (10.2%). There were 28 females (65.1%) and 15 (34.9%) males. The posterior canal was involved in 79% of cases and the horizontal canal in 21% of cases. No apogeotropic bilateral or anterior canal form were seen. Thus, BPPV is not an infrequent type of vertigo in children and must be evaluated as soon as possible in order to plan the most appropriate maneuver and restore daily activities as soon as possible, avoiding anxiety and fear.

Olfactory and Gustatory Outcomes in COVID-19: A Prospective Evaluation in Nonhospitalized Subjects.

OBJECTIVE: To prospectively assess the rate and timing of recovery of olfactory (OD) and gustatory (GD) dysfunction in patients affected by COVID-19. STUDY DESIGN: Cohort study. SETTING: Population-based evaluation in a COVID-19 high-prevalence region. SUBJECTS AND METHODS: We analyzed the clinical course of OD and GD in a cohort of home-quarantined SARS-CoV-2-positive patients from Northern Italy. Physicians administered a survey-based questionnaire at recruitment (T0). During follow-up, patients responded to online dedicated surveys modulated according to symptoms at T0. RESULTS: A total of 151 patients completed the follow-up survey. OD and/or GD were observed in 83% and 89% of subjects, respectively. Resolution rates of OD and GD at 30 days from onset were 87% and 82%, respectively. Risk factors for late resolution were grade of dysfunction at onset (total vs partial), gender, and presence of nasal congestion. Three (2%) patients previously reporting complete resolution of symptoms complained of subsequent recurrence of OD and/or GD after a mean of 19 days from resolution of the previous episode. CONCLUSION: COVID-19-related OD and GD had high rate of resolution in the first month from onset of symptoms. However, in 10% to 15% of patients, these symptoms showed only partial improvement after this period.

Smell and taste alterations in COVID-19: a cross-sectional analysis of different cohorts.

BACKGROUND: Olfactory (OD) and gustatory (GD) dysfunction have been proven to be a typical symptom of severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. However, their prevalence in different patient populations still needs to be clarified. METHODS: A cross-sectional study was performed from March 27 to April 1, 2020, in Northern Italy. Physicians administered a survey-based questionnaire to SARS-CoV-2-positive patients with the aim of assessing symptoms, focusing on OD and GD. Two groups were studied: group A, patients hospitalized at Azianda Socio Sanitaria Territoriale (ASST) Spedali Civili University Hospital of Brescia; and group B, home-quarantined subjects. RESULTS: A total of 508 patients were enrolled: 295 in group A and 213 in group B. Mean age ± standard deviation (SD) was 55 ± 15 years; 56% were men. Overall, OD and GD were present in 56% (95% confidence interval [CI], 51% to 60%) and 63% (95% CI, 59% to 67%) of cases, respectively. In group A, the prevalence of OD and GD was 44% (95% CI, 38% to 50%) and 52% (95% CI, 46% to 58%), respectively. In group B, the prevalence of OD and GD was 72% (95% CI, 65% to 79%) and 79% (95% CI, 73% to 84%), respectively. In the entire cohort, total loss of olfaction and taste was reported in 64% and 60% of cases, respectively. OD and GD occurred as the first symptom in 10% and 11% of cases, respectively; in the remaining cases, they occurred after a mean of 4 ± 3 days following the first symptom. At the time of the questionnaire, complete resolution of OD and GD was reported in 52% and 55% of cases, respectively (mean duration, 9 ± 5 days in both). CONCLUSION: OD and GD are more prevalent in home-quarantined subjects, and they are independently associated with younger age and female gender.

Endoscopic approach to the round window through posterior tympanotomy for cochlear implantation in children: A study on feasibility.

OBJECTIVE: To demonstrate the feasibility of rigid endoscopy through posterior tympanotomy, which provides both a view of the round window and direction of the scala tympani in children. METHODS: After a standard mini-invasive surgical approach with postauricular access and transmastoid posterior tympanotomy of 2 mm, a 0°, 1.9 mm diameter and 11 cm long endoscope is positioned in proximity of the upper part of the posterior tympanotomy to obtain a panoramic view of the inferior part of the medial wall of the tympanic cavity. Surgical complications and changes in hearing threshold were analyzed. RESULTS: Eight children were submitted to cochlear implantation with endoscopic assistance through posterior tympanotomy. Complete visualization of the round window niche was possible in every ear. No complications related to the procedure were observed. Preoperative threshold was preserved in 9 of 10 ears. CONCLUSIONS: Direct endoscopic view through the posterior tympanotomy allows visualization of the entire round window niche as well as the angle of introduction of the multi-electrode array along the direction of the scala tympani.

Intratympanic steroid delivery by an indwelling catheter in refractory severe sudden sensorineural hearing loss.

OBJECTIVE: Many studies over the last decade showed favorable outcomes with intratympanic (IT) steroid treatment, alone as salvage treatment or in combination with conventional systemic therapy (ST). However, in severe to profound sensorineural hearing loss resistant to ST, the optimal infusion mode, the type and concentration of the solution, the preferable drug, its total amount, and the duration and fractionation of the treatment are still debated. Aim of the study was to investigate the feasibility and the outcomes of a direct and constant IT delivery of dexamethasone (DEX) by means of a new indwelling catheter. METHODS: A prospective case-control study in a tertiary referral university hospital. Ninety-nine subjects treated with ST only and 28 with additional IT DEX have been included in the study. A 4 Fr catheter inserted in a sub-annular fashion with a minimal postero-inferior tympanotomy through and endocanalar approach under local anesthesia. DEX 4mg/ml delivered daily, up to 7 days. Daily bone and air-conducted pure tone and speech audiometry were performed with a follow-up at 1, 3, 6 months after treatment. RESULTS: Twenty-one out of 28 patients (75%) refractory to ST gained on average 24.0dB±20.5dB HL after IT-DEX, compared to 35.4% (average 6.7dB±16.6dB HL) of those receiving only medical ST (p<0.001). No significant side effects were noted. CONCLUSION: In severe to profound sudden deafness refractory to conventional ST, the daily perfusion of 4mg/ml DEX through an intratympanic catheter is an easy, well accepted procedure that enables patients to receive a drug in the middle ear in a repeatable or sustained form, with minimal discomfort and a partial rescue (67.86%) and a speech recognition gain of 39%.

A Multicenter Clinical Evaluation of Data Logging in Cochlear Implant Recipients Using Automated Scene Classification Technologies.

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