New frontiers in tinnitus, hearing loss and hyperacusis

Although considerable progresses have been made in the recent years, there are still wide-open pathways for research in tinnitus, hearing loss and hyperacusis. Among them, I’d like to put particular focus on basic and clinical research on the predisposing elements to ac- quired hearing loss to favour understanding in prevention and treatment, on the exploration of the pathophysiological basis of tinnitus necessary for future possible treatments, and on the correlation between tinnitus and hyperacusis, an interesting direction that could rise questions and give answers to identify target mechanisms able to help in therapeutic strategies

Quality of life measurements for patients with chronic suppurative otitis media: Italian adaptation of "Chronic Ear Survey". La misura della qualità della vita in pazienti con otite media suppurativa cronica: adattamento in italiano del “Chronic Ear Survey”

Il Chronic Ear Survey (CES) è una misura specifica della Qualità della Vita (QoL) nei pazienti affetti da Otite Media Suppurativa Cronica (CSOM). È un questionario composto da 13 domande che indagano frequenza, durata e severità dei sintomi associati a questa malattia. Il CES genera tre sottoscale con rispettivo punteggio che riguardano limitazioni nelle attività fisiche e sociali, sintomi e trattamento medico. Attraverso le risposte ottenute dai pazienti è possibile ricavare un punteggio che va da 0 a 100; il punteggio più alto indica una QoL migliore, mentre quello più basso indica una QoL peggiore. Il questionario è stato creato in lingua inglese. Lo scopo del lavoro è di validare in lingua italiana il CES. La traduzione è stata condotta seguendo le linee guida internazionali. La versione italiana del CES (CES-I) è stata proposta a 54 pazienti con CSOM. Nello stesso tempo, è stato somministrato a tutti i pazienti anche il questionario SF-36. Un modello trasversale è stato usato per esaminare la consistenza interna (Cronbach alpha) e la validità esterna (coefficiente di Pearson). Per confermare la validità esterna del CES-I è stato poi analizzato il test di correlazione di Pearson considerando il punteggio totale, le singole sottoscale del CES e le 8 scale dello Short Form Health Survey (SF-36). Il coefficiente di Cronbach è stato pari a 0.737. Il coefficiente di correlazione interno ha dato un risultato pari a 0.737 (95% CI: 0.600-0.835, p < 0.001) di media e 0.412 (95% CI: 0.237-0.559, p < 0.001) per le singole misure. Sulla base dei nostri risultati il questionario CES-I è risultato essere concorde con l’originale in lingua inglese e può essere considerato uno strumento adeguato per valutare la Qualità della Vita nei pazienti con CSOM di lingua italianaThe chronic ear survey (CES) is a sensitive and disease specific quality of life (QoL) measurement tool in patients with chronic suppurative otitis media (CSOM). It is a 13-item survey that evaluates the frequency, duration and severity of problems associated with this disease. It is composed of three subscales that describe activity restrictions, symptoms and medical resource utilisation. Based on patient's answers, it is possible to obtain a score resulting in a scale ranging from 0 to 100; the highest indicates the best health, while the lowest denotes poor health. The questionnaire was originally created in English. The aim of this study is to validate the CES questionnaire in Italian (CES-I). Translation was made following international guidelines. The application follows the stages of translation from English to Italian and linguistic adaptation, and grammatical and idiomatic equivalence review. The CES-I and the Short Form Health Survey 36 (SF-36) questionnaires were administered to 54 patients with CSOM. A cross-sectional design was used to examine the internal consistency (Cronbach's alpha) and concurrent validity (Pearson's product moment correlation). To confirm the external validity of CES-I, Pearson correlation coefficient, considering the total score and single subscales of CES and the 8 scales of the SF-36, was calculated. Cronbach's alpha coefficient for internal consistency was 0.737. The intraclass correlation coefficient, measured through mixed effects, was 0.737 (95% CI: 0.600-0.835, p < 0.001) for average measures and 0.412 (95% CI: 0.273-0.559, p < 0.001) for individual measures. According to our results, CES-I is a reliable tool for evaluation of QoL in patients with CSOM among the Italian-speaking population

Unilateral enlarged vestibular aqueduct syndrome and bilateral endolymphatic hydrops

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH

Day-case management of chronic suppurative otitis media with cholesteatoma with canal wall down technique surgery: long-term follow-up

The overall number of day-case otologic surgery cases is increasing; however, there is limited experience about performing canal wall down tympanoplasty in patients with chronic suppurative otitis media with cholesteatoma in this setting. The objective of this study was to assess the success of this technique as day-case surgery in terms of results and complications over an 8-year follow up period. We included in this study 42 patients undergoing canal wall down technique tympanoplasty surgery for chronic suppurative otitis media with cholesteatoma performed as day cases during a 2-year period. 30 cases (71.4%) were discharged on the day of surgery, whereas 12 cases (28.6%) were hospitalized and discharged the day after. The principal reasons for failure of discharge on the day of surgery were asthenia (6 cases), vertigo and asthenia (4 cases), undetermined (2 cases). Based on our experience, with a proper preoperative selection, assessment and screening of the patients, mastoidectomy with timpanoplasty for chronic suppurative otitis media with cholesteatoma can be carried out in a day surgery setting with no significant effects on effectiveness of surgery, post-operative symptoms and relapse of disease even in the long term

Systemic lupus erythematosus and hearing disorders: literature review and meta-analysis of clinical and temporal bone findings

Objective: This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design: Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods: PubMed and Google Scholar searches were performed using the following keywords: “auto-immune disease,” “systemic lupus erythematosus (SLE),” “hearing loss,” “temporal bone study,” “vertigo,” “dizziness,” “tinnitus,” “ear symptoms,” “treatment,” “diagnosis,” “symptoms,” “etiopathogenesis,” “Wegener granulomatosis,” “Sjogren,” “polyarteritis nodosa,” “Cogan syndrome,” and “granulomatosis.” Also included were reviews in which the following terms were present: “SLE,” “temporal bone,” and “hearing symptoms.” Review and conclusion: This literature review and meta-analysis focused on the pathological mechanisms through which SLE can damage inner ear structures and determinate hearing and vestibular symptoms. The main mechanisms involved in inner ear damage include the autoimmune response, deposition of immune complexes in the vessels and, to a lesser extent, cytotoxic damage

Hearing disorders in Turner’s syndrome

Introduction: Turner’s Syndrome (TS) is associated with hearing disorders in about 20 to 50% of affected individuals. The most common hearing disorders include congenital auricular malformations, recurrent otitis media and sensorineural hearing loss, although altered vestibular function and tinnitus have also been reported. Objectives: The aim of this paper is to provide an up-to-date overview of the principal ndings and research perspectives about the association between TS and hearing disorders. Review: Middle ear disorders, found in a range between 21 and 91% of subjects, are a consequence of morphological cranio-facial alterations resulting in middle ear ventilation dysfunc- tion. Sensorineural hearing loss follows 2 main audiological pro les: a bilateral symmetrical mid-frequency dip and a high frequency down-sloping curve. Although the pathophysiologic basis of sensorineural hearing loss in TS patients are still unclear, several hypothesis have been made so far and are reviewed in this paper. Conclusion: Literature confirms that hearing disorders, although not the most relevant clinical problem in these patients, have a high incidence in patients with TS and should therefore undergo early evaluation and monitoring over time

Noise induced hearing loss: the role of oxidative stress

Introduction: Noise-induced hearing loss (NIHL) is a relevant source of hearing disability affecting the general population, and accounts for about 16% of all the reported cases of disabling hearing loss in the adult population worldwide. NIHL can follow workplace-related and recreational noise exposure, and can be influenced by individual factors such as age, sex, genetic predisposition and socio-economic factors. Objectives: The aim of this paper is to provide a quick overview of the principal ndings in noise induced hearing loss, focusing on the role of oxidative stress and antioxidant intervention. Review: Oxidative stress plays a central role in leading to a condition of NIHL. Reactive oxygen species (ROS) and reactive nitrogen species (RNS) largely participate in cellular mechanisms that underlie mainly the outer hair cell death after noise exposure and lead to sensorineural hearing loss. The beneficial effects of antioxidant supplementation have been demonstrated by several experimental studies in animals, while the observed results in humans are mixed. Conclusion: NIHL still represents a widespread condition among the general population; with a higher prevalence in developing countries among workers, and in developed countries among young adults exposed to leisure noise. Extensive literature confirms that increasing antioxidant levels in the organ of Corti may be an appropriate approach towards understanding NIHL in humans by increasing the endogenous antioxidant response or by administering antioxidant molecules systemically or locally

Ocular vestibular evoked myogenic potentials and intravestibular intralabyrinthine schwannomas.

Intravestibular intralabyrinthine schwannomas (ILSs) are uncommon benign tumors that arise from the sac-cular, utricular, and lateral and superior ampullary nerves. According to the literature, there is an average delay of 8 years between the onset of symptoms and diagnosis. Te diagnosis is based on an audiovestibular examination and magnetic resonance imaging (MRI). We describe a case of intravestibular ILS in which we included the ocular vestibular evoked myogenic potentials (oVEMPs) test in the diagnostic workup. The oVE-MPs test is a relatively new neurophysiologic diagnostic modality that evaluates the superior vestibular pathway and the ascending contralateral pathway through the vestibulo-ocular reflex. In our case, a 65-year-old man presented with progressive right-sided sensorineu-ral hearing loss, dizziness, and tinnitus and fullness in his right ear. Audiovestibular examination and MRI detected an intravestibular ILS on the right. We found that oVEMPs were absent on the contralateral side, which contributed to the diagnostic process. Te detection of oVEMPs can provide detailed information on the functionality of the macula of the utricle and the lateral and superior ampullary nerves, with a precise identification of the affected area. Based on our findings, we discuss the role of oVEMPs in the diagnosis of an intravestibular ILS

Hearing loss in Takayasu's arteritis: a role for hyperbaric oxygen therapy?

In the scientific community, there is growing interest regarding associated symptoms in Takayasu’s arteritis (TA), an autoimmune condition that mainly affects the medium and large arteries. Hearing loss (HL) is a rare complication of TA that is often overlooked and has severe consequences on the quality of life. HL mainly presents as sudden sensorineural hearing loss (SSNHL) and responds to corticosteroid therapy. In the authors’ opinion, the reasons for misdiagnosing HL in patients with TA are lack of evidence re- garding this rare complication and because TA involves large caliber arteries, instead of small vessels that are typical of the inner ear. We recently used HBOT for a 36-year-old woman with TA, who had two SSNHL episodes in different ears in an 11-month period and observed a significant improvement in the patient's hearing. Although this was a single case finding and HBOT was administered together with steroid therapy, the significant recovery of HL in both ears following this therapeutic approach may be worth sharing with the scientific community. In conclusion, we recommend that awareness for inner ear involvement in TA should increase and that HL should be considered a possible complication of TA and should be treated with corticosteroid therapy and, after collecting further evidence, HBOT

Metastatic renal cell carcinoma presenting as a paranasal sinus mass: the importance of differential diagnosis

Metastases in the paranasal sinuses are rare; renal cell carcinoma is the most common cancer that metastasizes to this region. We present the case of a patient with a 4-month history of a rapidly growing mass of the nasal pyramid following a nasal trauma, associated with spontaneous epistaxis and multiple episodes of hematuria. Cranial CT scan and MRI showed an ethmoid mass extending to the choanal region, the right orbit, and the right frontal sinus with an initial intracranial extension. Patient underwent surgery with a trans-sinusal frontal approach using a bicoronal incision combined with an anterior midfacial degloving; histological exam was compatible with a metastasis of clear cell renal cell carcinoma. Following histological findings, a total body CT scan showed a solitary 6 cm mass in the upper posterior pole of the left kidney identified as the primary tumor. Although rare, metastatic renal cell carcinoma should always be suspected in patients with nasal or paranasal masses, especially if associated with symptoms suggestive of a systemic involvement such as hematuria. A correct early-stage diagnosis of metastatic RCC can considerably improve survival rate in these patients; preoperative differential diagnosis with contrast-enhanced imaging is fundamental for the correct treatment and follow-up strategy