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.

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.

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.

Acute mastoiditis in cochlear implanted children: A single-centre experience.

BACKGROUND: Acute mastoiditis (AM) is the most common complication of acute otitis media and primarily affects children under the age of two; current data on its prevalence in paediatric patients with cochlear implant (CI) are still scant. Proper management of AM in CI children is crucial in order to avoid the implications (financial and emotional) of an explant. Aim of this paper is to describe the cases of AM occurred among young patients with CI in follow up at our department, also in order to evaluate its prevalence, potential predisposing factors, clinical course and therapeutic strategies. PATIENTS AND METHODS: Retrospective study. Medical records of all paediatric patients with CI, who had at least one year of follow-up, were searched aiming to identify those who developed AM, from January 1st 2002 to January 31st 2022. The following data were collected and analysed: demographic features, implant type and side, interval between CI surgery and AM, treatment, laboratory tests, clinical course, vaccination history, associated diseases. RESULTS: AM was developed by six (1.3%) of the 439 children with CI (541 implanted ears). In total, 9 episodes (2.05 %) were recorded, as three patients reported two consecutive infections. Average time interval between CI surgery, to the first or only AM diagnosis, was 13.8 months (range 3-30 months). Furthermore, 3/6 of patients had a history of recurrent acute otitis media; 2/6 an autism spectrum disorder, associated to a combined immune deficiency in one case. All patients were hospitalized and promptly treated by intravenous antibiotic therapy; 4/6 also underwent a mastoidectomy. CI was not explanted in any cases of this series. CONCLUSIONS: Over a 20-year period, AM rate in CI children was 1.3%, which is consistent with the current literature rates of 1-4.7%. All cases were successfully treated, preserving the integrity of the device. In our experience, the early parenteral antibiotic therapy and, when necessary, surgical treatment were adequate to eradicate the infection.

Hyperacusis: Focus on Gender Differences: A Systematic Review.

BACKGROUND: While gender differences of several diseases have been already described in the literature, studies in the area of hyperacusis are still scant. Despite the fact that hyperacusis is a condition that severely affects the patient's quality of life, it is not well investigated; a comprehensive understanding of its features, eventually including gender differences, could be a valuable asset in developing clinical intervention strategies. AIM: To evaluate gender differences among subjects affected by hyperacusis. METHODS: A literature search was conducted focused on adult patients presenting hyperacusis, using the MedLine bibliographic database. Relevant peer-reviewed studies, published in the last 20 years, were sought. A total of 259 papers have been identified, but only 4 met the inclusion criteria. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. RESULTS: The four selected papers included data from 604 patients; of these, 282 subjects resulted as affected by hyperacusis (125 females and 157 males). Questionnaires for analyzing factors affecting the attentional, social and emotional variance of hyperacusis (such as VAS, THI, TSCH, MASH) were administered to all included subjects. The data suggest that there are no hyperacusis gender-specific differences in the assessed population samples. CONCLUSIONS: The literature data suggest that males and females exhibit a similar level of hyperacusis. However, in light of the subjective nature of this condition, the eventual set up of further tests to assess hyperacusis features could be very helpful in the near future.

Auditory Neuropathy Spectrum Disorder in the White Sutton Syndrome.

White Sutton Syndrome is a rare autosomal dominant disorder resulting from a de novo mutation of Pogo Transposable Element Derived with Zinc Finger domain gene. The phenotype is characterized by a wide spectrum of cognitive dysfunction and developmental delays. Hearing loss is frequently mentioned as one of the symptoms of this rare disease, but details are usually scant. We report a case of a male child affected by White Sutton Syndrome and sensorineural hearing loss, with audiological findings of an auditory neuropathy spectrum disorder, a dysfunction of the auditory pathway with preserved cochlear outer hair cell function. Up to date, the present case is the first description of hearing loss due to an auditory neuropathy spectrum disorder in White Sutton Syndrome. A comprehensive audiological assessment is therefore mandatory in all White Sutton Syndrome patients in order to recognize a possible auditory neuropathy disorder and then avoid misdiagnosis, or erroneous clinical management.

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.

Cochlear implantation in single-sided deafness: a single-center experience of 138 cases.

OBJECTIVES: Assess the outcomes in patients who underwent cochlear implant (CI) for single-sided deafness (SSD). METHODS: All patients affected by SSD who underwent CI at Gruppo Otologico, Piacenza, from October 2012 to May 2022 with at least 6 months of follow-up were selected in the study group. The analysis included subjective and objective measures performed pre-operative and up to 24 months after surgery. Hearing threshold on both sides was evaluated with a weighted four-frequency average (PTA [0.5 kHz + 1 kHz + 2 kHz + 4 kHz]/4) on pure tone audiometry and speech audiometry (Speech Discrimination Score, SDS). The Speech Spatial and Qualities of Hearing scale (SSQ Questionnaire) for binaural hearing benefits and sound localization, the Tinnitus Handicap Inventory Questionnaire (THI) and Dizziness Handicap Inventory Questionnaire (DHI) were used for subjective assessment. RESULTS: Data from 138 patients, 69 males and 69 females, (mean age 49 years, range 17-77 years) underwent CI for SSD were examined. Single-sided hearing deprivation average before undergoing CI surgery was 2.5 years (range 3 months-35 years). There was a significant reduction of THI and DHI scores compared to pre-operative scores alongside a referred improvement in social, physical, and emotional well-being through the SSQ questionnaire. CONCLUSIONS: To the best of our knowledge, this paper descried the largest cohort of SSD who underwent CI in a single institution. According to our findings CI in patients affected by SSD represents a valuable tool for an overall improvement of tinnitus and dizziness but also quality of life, after at least 6 months of follow-up. Further studies are desirable to improve rehabilitation pathways and possibly set new standards of care of this condition.

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.

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.

Management of Juvenile Otosclerosis: A Systematic Review.

Background. Otosclerosis can occur during childhood, resulting in the early onset of conductive hearing loss. The approach to a child with otosclerosis can present some difficulties in terms of diagnosis and treatment, and the literature on juvenile otosclerosis (JO) is still relatively limited. Aim. To explore the current approaches to JO, in order to clear the management of this condition and evaluate the outcomes and the possible complications of surgical treatment. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline and Embase from January 2002 through to 30 September 2022. A total of 759 papers were identified but based on specified criteria, nine were included in this study. Results. There were 94 children affected by JO and treated by stapes surgery. According to the available data, Male: Female ratio was 1:3−4, whilst the mean ages ranged from 10 to 16.3 years at the time of stapes surgery. After stapes surgery, the target of ABG < 10 dB was achieved in most of the patients. Overall, the 4 complications were reported (4/94= 4%): stenosis of the external ear canal, deterioration of hearing, anacusis with vertigo, tinnitus. Conclusions. The heterogeneity of the available studies does not allow us to draw straight conclusions on this topic, currently. More data about the natural history of the disease in children could help in approaching the treatment correctly, and possibly in drawing guidelines. Studies with a prolonged follow-up could be helpful for assisting clinicians and families in taking the most favorable decision about treatment.

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.

Vertigo and Dizziness in Children: An Update.

BACKGROUND: Vertigo and dizziness are relatively infrequent in paediatric patients, but specific data on the prevalence of these disorders are limited and influenced by various factors, including the age of the examined population. These conditions often have a significant impact on patients' and parents' quality of life. The aim of this paper is to investigate the prevalence of different aetiologies of vertigo in the paediatric population through a systematic review. METHODS: According to PRISMA guidelines, a systematic review of the literature was performed. Medline and Embase were searched from January 2011 through to 10 September 2021. The search yielded 1094 manuscripts, which were reduced to 7 upon the application of inclusion criteria. RESULTS: A total of 2470 paediatric patients were evaluated by the selected papers. Vestibular Migraine was the most frequently diagnosed condition, occurring alone or in association with other diseases. Overall, audio-vestibular disorders represented the second cause of vertigo, and the prevalence appears to increase according to age growth. Over the years, even though we assisted in the amelioration of diagnostic rates, partially related to an improvement in diagnostic tools, the aetiology of vertigo remains still unclear in a variable percentage of patients. CONCLUSION: Vertigo in children, despite being an uncommon symptom, requires a multidisciplinary approach, often involving Paediatricians, Neurologists and Otorhinolaryngologists. A comprehensive evaluation of children suffering from vertigo is crucial for establishing a successful therapy and reducing parental worries.

Laryngeal Taping as a Supportive Tool to Relieve Phonasthenia in Singers: A Preliminary Report.

AIM: This report aims to perform a preliminary evaluation of the role of taping in the improvement of phonasthenia among professional voice users. The larynx is a vital organ but also a work instrument for many people. Prevention of erroneous use of the vocal mechanism is essential for an optimal voice performance. METHODS: Nineteen singers complaining voice fatigue underwent taping application in peri-laryngeal areas of the anterior neck for 10 days. Taping effects were estimated by the evaluation of voice analysis parameters (jitter, shimmer, noise to harmonic ratio and singing power ratio) performed in M1 and M2 mechanisms with PRAAT software before and after treatment. In addition, a subjective assessment of phonasthenia was performed using a self-administrated questionnaire. RESULTS: Statistically significant reduction of Jitter% in M1 (P = 0.021) was reported at the end of treatment; also, an improvement of NHR in M2 was found immediately after the application (P = 0.012) and after 10 days (P = 0.002). These outcomes suggest possible beneficial effects of laryngeal taping on relieving voice fatigue, and therefore improving voice quality. Self-assessment results are consistent with this finding. CONCLUSION: Since the application of muscular taping in phoniatrics is still in its early stage, further studies and in particular a standardized protocol of application, could facilitate the spread of this technique, also offering help in comparing results.

SARS-CoV-2 (COVID-19) and audio-vestibular disorders.

To describe the audio-vestibular disorders related to the newly SARS-CoV-2 infection, including the possible ototoxicity side-effects related to the use of drugs included in the SARS-CoV-2 treatment protocols. A systematic review was performed according to the PRISMA protocol. The Medline and Embase databases were searched from March 1, 2020 to April 9, 2021. Initially the search yielded 400 manuscripts, which were reduced to 15, upon the application of inclusion criteria. Sensorineural hearing loss (SNHL) is the most frequent audio-vestibular symptom described, occurring alone or in association with tinnitus and vertigo. The etiopathogenesis of the inner ear disorders related to COVID-19 infection is still poorly understood. The number of reports of COVID-19 infections associated to audio-vestibular disorders is increasing; even if the quality of the studies available is often insufficient, audio-vestibular disorders should be considered as possible manifestations to be included among the symptoms of this infection.

Role of reinnervation in the management of recurrent laryngeal nerve injury: current state and advances.

PURPOSE OF REVIEW: To present the current state of knowledge concerning different laryngeal reinnervation procedures for unilateral and bilateral vocal palsy. RECENT FINDINGS: Recent reports show positive outcomes on both unilateral and bilateral reinnervations. The phrenic nerve is the most commonly used donor for bilateral vocal palsy, but use of the superior laryngeal nerve has also been suggested. SUMMARY: Reinnervation of the larynx is a complex undertaking that can be performed by ENT surgeons with skills in microsurgery. Advances in this this field represent a paradigm shift in laryngeal rehabilitation and a prerequisite for laryngeal transplantation. Advances in basic understanding of nerve regeneration and in particular the need to surgically manage competitive reinnervation make the results of laryngeal reinnervation more predictable.

HPV Type 6 and 18 Coinfection in a Case of Adult-Onset Laryngeal Papillomatosis: Immunization with Gardasil.

Laryngeal papillomatosis (LP) is a rare human papillomavirus (HPV) related disease that often requires multiple surgical interventions and residual impairment of voice is almost inevitable. We report the case of a patient with adult onset recurrent LP, showing moderate dysplasia and coinfection with HPV types 6 and 18. The tetravalent HPV vaccine Gardasil was prescribed off label, with the aim of triggering an immunogenic response and consequently reducing the probability of further recurrences. The patient was followed for 9 months with no sign of relapse of his LP. The postexposure use of the anti-HPV vaccine could represent a promising therapeutic agent in established LP. Unfortunately, the potential efficacy of this new therapeutic option in this situation has been suggested only by isolated case reports. Further controlled studies, with a longer follow-up and a larger sample size, are needed to assess efficacy of Gardasil in LP.