Management of Petrous Bone Cholesteatoma: The Gruppo Otologico Experience.

BACKGROUND: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. OBJECTIVE: To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. STUDY DESIGN: Retrospective medical record review. MATERIAL AND METHODS: Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. RESULTS: A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. CONCLUSION: Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.

Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

The Role of Primary Mitochondrial Disorders in Hearing Impairment: An Overview.

Background. Defects of mitochondrial DNA (mtDNA) involved in the function of the mitochondrial electron transport chain can result in primary mitochondrial diseases (PMDs). Various features can influence the phenotypes of different PMDs, with relevant consequences on clinical presentation, including the presence of hearing impairment. This paper aims to describe the hearing loss related to different PMDs, and when possible, their phenotype. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline until December 2022. A total of 485 papers were identified, and based on specified criteria, 7 were included in this study. Results. A total of 759 patients affected by PMDs and hearing loss were included. The age of patients ranged from 2 days to 78 years old, and the male-to-female ratio was 1.3:1. The percentage of subjects affected by hearing loss was 40.8%, (310/759), and in most cases, hearing impairment was described as sensorineural, bilateral, symmetrical, and progressive, with different presentations depending on age and syndrome severity. Conclusions. PMDs are challenging conditions with different clinical phenotypes. Hearing loss, especially when bilateral and progressive, may represent a red flag; its association with other systemic disorders (particularly neuromuscular, ocular, and endocrine) should alert clinicians, and confirmation via genetic testing is mandatory nowadays.

The prognostic role of IL-10 in non-severe COVID-19 with chemosensory dysfunction.

OBJECTIVES: Olfactory and gustatory dysfunction (OD/GD) are now recognized as typical symptoms of COVID-19 infection. However, their pathogenesis remains unclear and no clear prognostic factors have been identified. We have analyzed a cohort of mild/moderate hospitalized patients to identify possible clinical or immunological predictors of recovery from OD/GD. METHODS: Clinical and biological parameters were reviewed along with associated comorbidities. Chemosensory Complaint Score was administered on admission and 30 days after the first negative swab. Unpaired Wilcoxon and chi-squared tests were used to compare the variables in the patients who recovered versus those who did not. RESULTS: From a cohort of 119 hospitalized patients, 43 (36%) reported OD/GD on admission. 60.6% had a full recovery from OD and 69.2% from GD. Only the concentration of IL-10 on admission emerged as significantly associated with recovery of taste (p = 0.041) while allergic respiratory disease was more prevalent in the group who did not recover from OD (p = 0.049) and GD (p = 0.007). CONCLUSION: These findings suggest that COVID-19 associated OD/GD is an inflammatory-mediated condition and that clinical and immunological parameters could predict the evolution of these symptoms.

The impact of the COVID-19 pandemic on the quality of life of head and neck cancer survivors.

BACKGROUND: Head and neck cancer (HNC) survivors are particularly vulnerable to the deleterious consequences of lockdown and social distancing. The psychosocial effects of the COVID-19 pandemic on this group are still unknown, and we want to explore how their quality of life (QoL) has changed in this unique situation. MATERIALS AND METHODS: An online survey, composed of pandemic-specific items, plus the EORTC QLQ-C30, was administered to a cohort of HNC survivors. Using previously published reference values as a control group, we have evaluated the impact of the pandemic on their QoL. We have also explored the differences between those who had received a total laryngectomy (LP, laryngectomized population) vs other HNC patients, in order to assess the role of tracheostomy in this regard. RESULTS: One hundred and twenty-one HNC patients completed the survey. The scores of the physical (80.5 vs 85, p = 0.028), role (78 vs 84, p = 0.030), and emotional functioning (76 vs 81, p = 0.041) were significantly different in the two groups, with worse functioning in our patients. Comparing LP with the other HNC patients, social (76.6 vs 88.9, p = 0.008) and physical functioning (75.5 vs 86.1, p = 0.006) were significantly worse in the former group. LP also reported a greater perception that others are afraid to be close to them (1.67 vs 1.32, p = 0.020). No differences were found between LP with and without voice prosthesis. CONCLUSIONS: Our results show how HNC patients are at high risk for a worsening in QoL because of the ongoing COVID-19 global pandemic.

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review.

Children with single-sided deafness (SSD) may experience delays in language and speech development. Reduced speech discrimination and poor sound localization abilities in young SSD patients may result in greater cognitive efforts required to focus and process auditory information, as well as increased listening-related fatigue. Consequently, these children can have a higher risk of academic failure and are often in need of extra help at school. Recently, cochlear implants (CIs) have been introduced as a rehabilitative option for these children, but their effectiveness is still a topic of debate. A literature review was performed according to PRISMA guidelines, searching the Medline database from inception to October 2023. The research identified nine papers that met the inclusion criteria. Data extracted from the selected studies included 311 children affected by SSD and cochlear implants. The reported audiological outcomes were further analyzed. Overall, a high level of satisfaction was described by parents of children with SSD and CI, and those who received a CI under the age of 3 presented better results. However, a proportion of patients did not use the device daily. Our review highlights the possible, and still controversial, role of CI for the hearing rehabilitation of children with unilateral deafness, underlining the need for further research in this field. To date, careful and comprehensive counseling with the child and the family is necessary before considering this option.

Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas.

OBJECTIVE: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. STUDY DESIGN: Retrospective study. METHOD: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. RESULT: Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. CONCLUSIONS: Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.

Customized Vestibular Rehabilitation for Vestibular Schwannoma Excision via Translabyrinthine Approach: A Single-Center Experience.

Objectives: To evaluate the effectiveness of intensive customized vestibular rehabilitation after vestibular schwannoma (VS) excision. Methods: 52 patients who underwent VS removal via a translabyrinthine approach from 2020 to 2022 were involved in this study. Bedside examination, video head impulse test (vHIT), functional head impulse test (fHIT), and the dizziness handicap inventory (DHI) were performed before and after the rehabilitation, which consisted of 10 sessions of specifically designed vestibular, visual, and physical integrated training. Results: After rehabilitation, the vHIT showed overall unchanged values on the affected and healthy side. In contrast, the scores of fHIT, which explores the higher connection of the vestibular system with visual and cerebellar pathways, improved on both the pathological and healthy sides after training (p-value 0.004 and 0.000, respectively). The effectiveness of the rehabilitation was reinforced by the DHI scores, which were considerably lower after training. Conclusions: To our knowledge, this is the first study to explore fHIT outcomes after removal of VS, estimating the impact of rehabilitation on the overall compensation process. The outcomes support the role of extensive postsurgical rehabilitation in the compensatory process, even just a few days after surgery.

Auditory Screening in Newborns after Maternal SARS-CoV-2 Infection: An Overview.

BACKGROUND AND AIM: Several viruses have previously been reported to be responsible for congenital hearing loss; therefore, since the beginning of the SARS-CoV-2 infection pandemic, various reports have investigated a possible link. The aim of this review is to assess the possible link between maternal COVID-19 infection and congenital hearing loss. METHODS: This systematic review was performed using PRISMA criteria, searching Medline and Embase databases from March 2020 to February 2023. A total of 924 candidate papers were identified; however, considering the specific selection criteria, only nine were selected for additional analysis. RESULTS: The overall number of children born from mothers infected with COVID-19 during pregnancy identified through this review was 1687. The confirmed cases of hearing loss were 0.7% (12/1688); a description of its nature (sensorineural vs. conductive) is missing in the selected studies, and the follow-up period is variable across the analyzed papers. Surprisingly, a large proportion of false positives were recorded at the first stage of screening, which resulted normal at the re-test. CONCLUSIONS: Currently, a correlation between congenital hearing loss and SARS-CoV-2 infection cannot be definitively established. Further studies are desirable to provide additional evidence on this topic.

Vertigo in the Elderly: A Systematic Literature Review.

Background: Dizziness and vertigo are among the most prevalent complaints in the elderly and have a major negative influence on (i) the perception of the quality of life; and (ii) the risk of falling. Due to population aging, particularly in wealthy nations, vertigo represents a growing issue and a serious public health concern. In order to approach the patient correctly and to offer the best treatment options, it is mandatory to identify vertigo's underlying causes. The aim of this paper was to identify the different etiologies of vertigo and possibly their frequency in the elderly population, by reviewing the scientific literature of the last decade (2012-2022). Methods: A systematic review was performed according to PRISMA guidelines, searching the Medline database from January 2012 through to December 2022. The search identified 1025 candidate papers, but after the application of specific selection criteria, only five were considered for further analysis. Results: A total of 2148 elderly patients (60-90 y old) presenting with vertigo were reported in the selected papers. A total of 3404 conditions were identified as the cause of vertiginous symptoms, (some patients presented multiple etiologies). All major diagnoses were categorized into different subgroups: the most common origin of vertigo was represented by audio-vestibular disorders (28.4%), followed by cardiovascular (20.4%) and neurological diseases (15.1%). Furthermore, 9.1% of patients were diagnosed with psychiatric conditions, whilst ophthalmologic and musculoskeletal disorders accounted for 7.5% and 6.3% of the cases respectively. Medication adverse effects and metabolic-related diseases were also considered among the causes. For 3.4% of cases the etiology remains unclear. Conclusions: Audio-vestibular disorders represent the most frequent cause of vertigo in the elderly. The etiologies affecting the vertigo patient must be defined in order to identify potential life-threatening conditions, such as cardiovascular and neurological disorders, which according to the data of this review constitute the second and third common causes of vertigo. A multidisciplinary strategy, involving different specialists (such as ENTs, Neurologists, Cardiologists, Geriatricians) is recommended for the correct assessment of these disorders.

Sensorineural Hearing Loss Post-COVID-19 Infection: An Update.

The course of COVID-19 infection may be complicated by a variety of neurological manifestations. Since the inner ear is vulnerable to viruses, sensorineural hearing loss (SNHL) has been reported to occur following the SARS-CoV-2 infection, often resulting in long-term morbidity and worsening the quality of life. The interest in how the virus affects the inner ear has gradually increased since the pandemic's spread, but little is still known about the SNHL potentially caused by SARS-CoV-2. The aim of this paper is to evaluate the possible association between SNHL and COVID-19 infection, through a systematic literature review. Currently available data suggest that SARS-CoV-2 may hamper cochlear function; however, available reports are still limited. Large cohort and prospective studies are necessary to evaluate the long-term effects of this viral infection in the inner ear.

The clinical consequences of the COVID-19 lockdown: A report from an Italian referral ENT department.

Objectives: To evaluate the clinical effects of health care reorganization because of COVID-19, in a non-red zone Italian referral department of Otorhinolaryngology. Materials and Methods: We retrospectively reviewed the statistics of admissions to the Emergency Department (ED) of the Careggi University Hospital, Florence, from January 1, 2020, to May 17, 2020. Data regarding elective and urgent head and neck disorders were reviewed and the most challenging cases were discussed. Results: During the lockdown period, outpatient otolaryngological consultations and elective procedures decreased by 78% and 75% respectively, while emergency/urgency surgery increased by 128%. The five most emblematic clinical cases were thoroughly analyzed. Conclusion: Our analysis shows how the profound rearrangement of clinical and surgical activities, along with the citizens' fear to seek medical care has probably exacerbated the management of many routine head and neck conditions. Level of Evidence: 4.