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.

Epidemiologic, Imaging, and Clinical Issues in Bezold's Abscess: A Systematic Review.

Bezold's abscess is a deep neck abscess related to otomastoiditis. Due to the insidious clinical presentation, diagnosis can be extremely challenging, leading to delays in treatment and possible life-threatening complications. The literature currently provides a fragmented picture, presenting only single or small number of cases. The present study aims at examining our experience and the literature findings (based on PRISMA criteria) of 97 patients with Bezold's abscess, summarizing their epidemiology, pathogenesis, clinical presentation, imaging findings, and treatments. Bezold's abscess is found at any age, with overt male prevalence among adults. The clinical presentation, as well as the causative pathogens, are strikingly heterogeneous. Otomastoiditis and cholesteatoma are major risk factors. A clinical history of otitis is commonly reported (43%). CT and MRI are the main diagnostic tools, proving the erosion of the mastoid tip in 53% of patients and the presence of a concomitant cholesteatoma in 40%. Intracranial vascular (24%) or infectious (9%) complications have also been reported. Diagnosis might be easily achieved when imaging (CT) is properly applied. MRI has a limited diagnostic role, but it might be crucial whenever intracranial complications or the coexistence of cholesteatoma are suspected, helping to develop proper treatment (prompt antibiotic therapy and surgery).

Cochlear implant in vestibular schwannomas: long-term outcomes and critical analysis of indications.

PURPOSE: To describe our institutional experience in cochlear implantation after vestibular schwannoma (VS) resection, and compare the audiological outcomes between sporadic and neurofibromatosis type 2 (NF2) VS sub-cohorts of patients, and in relation to preoperative contralateral hearing. METHODS: Seventeen patients (8 sporadic and 9 NF2-associated VSs) who had undergone VS resection and cochlear implant (CI) were analyzed retrospectively. Audiological outcomes at 24 months were correlated with preoperative clinical variables. The results according to VS type (sporadic vs. NF2-associated) and contralateral hearing (impaired vs. normal) were compared. RESULTS: Fourteen CIs were actively used by the patients (77.8%). Twenty-four months after CI activation, the median postoperative PTA (pure tone average) was 45.6 dB nHL and a measurable WRS (Word Recognition Score) was achieved by 44.4% of patients (median WRS = 40%). The median postoperative PTA in the implanted ear resulted better in the group with an impaired contralateral hearing (36.3 dB nHL vs. 78.8 dB nHL, p = 0.019). Good preoperative contralateral hearing status (A-B classes of AAO-HNS) was a negative prognostic factor for CI performance on open-set discrimination (OR = 28.0, 95% CI 2.07-379.25, p = 0.012). CONCLUSIONS: CI is a viable rehabilitative option for patients with sporadic or NF2-associated VS. A good contralateral hearing adversely affects CI outcome and should be taken into consideration for patients' selection and rehabilitation programs.

Pediatric Hearing Loss Management in the COVID-19 Era: Possible Consequences and Resources for the Next Future.

Pediatric hearing loss early diagnosis and treatment have been limited by the current restrictions due to the coronavirus disease 2019 pandemic. The difficulty in accessing the multiple facilities required for the rehabilitative process is influencing the timing of each step of the process. Auditory hearing screening programs, etiological characterization, surgical timing, and speech therapies have all been limited in the past year. The current conditions have forced us to adopt different strategies to overcome the necessary social distancing prescriptions. Although their efficacy should be proved over time, some of these resources will be probably useful even in a nonpandemic future.

Cochlear implantation in adults with auditory deprivation: What do we know about it?

INTRODUCTION: In the ENT community, auditory deprivation is frequently considered as a negative prognostic factor for a good hearing outcome of cochlear implantation (CI), even if a growing literature suggests that this is not completely true. The purpose of this study is to evaluate the results of CI in patients with hearing deprivation, to compare them to results from non-deprived patients and then estimate how time of deprivation impacts on CI outcome and how a bilateral deprivation can affect the outcome compared to a unilateral deprivation. METHODS: Seventy-eight adults with severe to profound post-verbal hearing loss, with and without auditory deprivation history, received CI; audiological results obtained at 3-6-12-24 months follow up post CI were analyzed. RESULTS: No differences were founded between patients with unilateral deprivation and patients with no deprivation. Patients with bilateral deprivation seem to have a worse hearing outcome compared to that of those patients with unilateral deprivation or no deprivation at all. Long time deprivation (>15 years) seems to have a negative influence on the hearing outcome but results with CI remain excellent. CONCLUSIONS: Auditory deprivation should not be considered a contraindication to CI. The duration of auditory deprivation in the implanted ear seems to be a negative prognostic factor only for ears deprived from more of 15 years.

Cochlear implant outcomes in the elderly: a uni- and multivariate analyses of prognostic factors.

PURPOSE: To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. METHODS: All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. RESULTS: No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale). CONCLUSIONS: A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.

Epidemiologic, Imaging, Audiologic, Clinical, Surgical, and Prognostic Issues in Common Cavity Deformity: A Narrative Review.

IMPORTANCE: Common cavity deformity is a rare congenital inner ear malformation associated with profound hearing loss and attributed to an early developmental arrest of the cochlear-vestibular structures. This narrative review highlights the need to identify reliable indicators of hearing rehabilitation outcome. OBSERVATIONS: Despite its relatively simple definition, common cavity deformity varies widely in morphologic features, presence of cochlear nerve fibers and remnants of Corti organ, and outcome after cochlear or brainstem implant. CONCLUSIONS AND RELEVANCE: Cochlear implant has been shown to be a valid option for common cavity deformity, but its outcome remains variable and poor. Identification of specific neuroradiologic, audiologic, and neurophysiologic prognostic features; tailoring of the surgical approach; and standardization of outcome measures are needed to optimize the management of common cavity deformity and hearing rehabilitation after implant.

Surgical timing for bilateral simultaneous cochlear implants: When is best?

INTRODUCTION: Hearing loss is considered the most common congenital disease and the prevalence of neonatal deafness can be estimated between 1 and 2 cases per 1000 live births. Infant deafness must be diagnosed as early as possible and an effective therapeutic intervention needs to be carried out in order to avoid the serious consequences of hearing deprivation during the evolutionary period: alterations in the development of central auditory pathways and lack of language acquisition. The cochlear implant (CI) has proved to be the best instrument to solve the problem of auditory deprivation. In particular, the bilateral CI gives the patient access to binaural hearing which results in benefits in terms of sound localisation and discrimination. The optimal age of application of the CI is a widely discussed topic in the scientific community and the current guidelines indicate a period between 12 and 24 months of age, even though the supporters of the application before 12 months of age are nowadays increasing. MATERIALS AND METHODS: The study is observational, retrospective, monocentric. 49 paediatric patients (<18 years) with simultaneous bilateral CIs were included. The audiometric threshold and speech tests were carried out during the follow-up 3, 6 and 12 months after the CIs activation and when the patient reached 2 years of age. RESULTS: The statistical analysis showed that undergoing bilateral implantation surgery before 2 years of age allows a satisfactory audiometric performance, while there are no particular benefits in performing the surgery before 1 year of age. As far as the speech outcome is concerned, the statistical analysis didn't show significant correlation between the earlier age of implantation and better speech performance if the operation is carried out before 2.5 years of age. CONCLUSIONS: The results of the study indicate that the optimal age to perform the simultaneous bilateral CIs surgery is between 12 and 24 months, without demonstrating any particular benefit in carrying out the procedure before 1 year of age. This may be clinically relevant in terms of avoiding the risks of diagnostic mistakes and reducing the related surgical risk in children under 1 year of age.

Salivary glands abnormalities in oculo-auriculo-vertebral spectrum.

BACKGROUND: Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (OAVS) patients. Salivary gland abnormalities have been reported in OAVS patients but their rate, features, and relationship with phenotype severity have yet to be defined. MATERIAL AND METHODS: Parotid and submandibular salivary gland hypo/aplasia was evaluated on head MRI of 25 OAVS patients (16 with severe phenotype, Goldenhar syndrome) and 11 controls. RESULTS: All controls disclosed normal salivary glands. Abnormal parotid glands were found exclusively ipsilateral to facial microsomia in 21/25 OAVS patients (84%, aplasia in six patients) and showed no association with phenotype severity (14/16 patients with Goldenhar phenotype vs 7/9 patients with milder phenotype, p = 0.6). Submandibular salivary gland hypoplasia was detected in six OAVS patients, all with concomitant ipsilateral severe involvement of the parotid gland (p < 0.001). Submandibular salivary gland hypoplasia was associated to Goldenhar phenotype (p < 0.05). Parotid gland abnormalities were associated with ipsilateral fifth (p < 0.001) and seventh cranial nerve (p = 0.001) abnormalities. No association was found between parotid gland anomaly and ipsilateral internal carotid artery, inner ear, brain, eye, or spine abnormalities (p > 0.6). CONCLUSIONS: Salivary gland abnormalities are strikingly common in OAVS. Their detection might help the management of OAVS-associated swallowing and feeding impairment.

Intracranial venous sinus thrombosis as a complication of otitis media in children: Critical review of diagnosis and management.

OBJECTIVES: Otogenic lateral sinus thrombosis (LST) is a rare intracranial complication of acute otitis media (AOM), which can lead to severe neurological sequelae and death. The aim of this study was to analyze the clinical presentation, management and outcome of LST in children, investigating a possible correlation between clinical aspects, radiological findings and anatomical variations. METHODS: At a tertiary Italian hospital, a retrospective review was conducted on the medical records of eight patients diagnosed with otogenic LST over a 3-year period. Four children were males and mean age was 4.7 years. RESULTS: All patients had a history of otitis media at diagnosis and 4/8 presented also with more than one neurological sign or symptom. Mastoiditis signs were detected in 5/8 patients. Thrombosis was diagnosed by computed tomography, enhanced magnetic resonance and magnetic resonance venography. Treatment was medical, alone or combined with surgery. Medical treatment consisted in anticoagulants eventually combined with anti-edema medication on clinical basis. Mastoidectomy and/or myringotomy±trans-tympanic drainage placement were performed in 7/8 patients. Complete vessel recanalization was obtained in 6/8 children after a median follow-up time of 4.8 months. No complications, neither clinical sequelae occurred. In our series, neurological signs and symptoms were significantly associated with the presence of hypoplasia of the contralateral venous sinus (p=0.029). CONCLUSION: LST is a severe condition occurring even in absence of otological signs, and despite adequate antibiotic therapy for AOM, which should be ruled out and promptly treated. A dominant neurological presentation is associated in our series with anatomical variations of cerebral sinus venous drainage patterns. This should be carefully evaluated and considered in diagnosis, treatment planning and prognosis.

Trastornos autoinmunitarios del oído interno / Autoimmune inner ear disorders

La incidencia de enfermedad autoinmunitaria del oído interno (EAOI) es difícil de determinar: probablemente,se trata de una entidad poco frecuente, responsable de menos del 1% del total de casos de trastornos auditivos o mareos. Sin embargo, el diagnóstico de EAOI puede pasarse por alto debido a la falta de pruebas diagnósticas específicas. La principal característica de esta afección clínicamente diagnosticada es la presencia de una pérdida de audición neurosensitiva (PANS) bilateral, rápidamente progresiva y, con frecuencia, fluctuante, que se produce durante un período de semanas a meses. La progresión de la pérdida auditiva es demasiado rápida como para ser diagnosticada como presbiacusia y demasiado lenta como para llevar al diagnóstico de PANS súbita. Los síntomas vestibulares, talescomo inestabilidad generalizada, ataxia, vértigo posicional y vértigo episódico, pueden estar presentes en casi el 50% de los pacientes. En ocasiones, sólo un oído se encuentra afectado inicialmente, pero la pérdida auditiva bilateral sucede en la mayoría de los pacientes, con umbrales audiométricos simétricoso asimétricos. Casi el 25% al 50% de los pacientes también presentan acúfenos y sensación de plenitud auditiva, los cuales pueden ser fluctuantes. Las enfermedades autoinmunitarias sistémicas coexisten enel 15% al 30% de los pacientes

ncidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for less than 1% of all cases of hearing impairment or dizziness. Nevertheless, the diag-nosis of AIED might be overlooked because of the lack of specific diagnostic tests. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25%-50% of patients have also tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15%-30% of patients.

EYA1-related disorders: two clinical cases and a literature review.

OBJECTIVES: To delineate the diagnostic and rehabilitative aspects of syndromes that have overlapping features, we present the cases of two unrelated Caucasian males affected by hearing impairment, preauricular pits and cervical fistulae. Specific findings that are helpful in the diagnosis and management of EYA1-related disorders are highlighted. METHODS: Genetic, otologic, imaging, eye and renal evaluations were conducted to achieve a detailed and comprehensive assessment, leading to the most accurate diagnosis and appropriate treatment. A literature review was also carried out. RESULTS: Diagnostic criteria indicated that the two patients were affected by BOS1 (Branchio-Otic Syndrome 1). We also identified a novel sporadic missense mutation in the EYA1 gene: p.G533R (c.1597G>A, NM_000503.4), a highly conserved, heterozygotic amino acid substitution. In the other case, we identified the p.X593QextX6 (c.1777T>A, NM_000503.4) substitution. Both variants lead to isoform 1 (EYA1B and EYA1C) which is composed of 592 amino acids. Clinical and in silico evidence suggests a pathogenic role for the new mutations. Imaging evaluation revealed a complex pathology, characterized by external, inner and middle ear malformations, without renal anomalies. CONCLUSIONS: Our results demonstrate the importance of considering the imaging evaluation and the complete DNA sequencing of the EYA1 gene for the differential diagnosis of deafness and related branchio-oto-renal disorders.

Endocochlear inflammation in cochlear implant users: case report and literature review.

OBJECTIVES: Cochlear implantation is a relatively safe procedure with a low complication rate. The overall rate of complications among cochlear implant patients ranges from 6% to 20%. Major complications are those that are life-threatening or require surgery, whereas minor complications are those that can be medically treated. Nonetheless, certain complications, even if highly rare, may require specific investigations and treatments. Among these rare complications are those with endocochlear involvement, such as cochleitis or labyrinthitis, with fibrosis or ossification that could lead to explantation. The aims of the present study were to report a particular case of post-operative cochleitis and to review the rate of complications after cochlear implantation, emphasising those conditions with proven endocochlear involvement. METHODS: We refer to the case of an eight-year-old Italian boy affected by the sudden onset of headache, ipsilateral otalgia and facial paresis, who presented to our clinic for inexplicable worsening of the performance of his implant and his residual hearing, six years after surgery. A complete investigation including (clinical history, routine, autoimmune and serological blood tests, electrophysiological measurements from the cochlear implant and neuroimaging) was performed and is herein described. Additionally, a comprehensive review of the literature was conducted using internet search engines; 274 papers were selected, 88 of which were best suited to our purposes. RESULTS: In our case, the progression of the symptoms and the performance decrement required explantation, followed by a complete recovery. Reviewing the literature revealed only three reports concerning cases of proven endocochlear phlogosis that required revision surgery. Wound swelling/infection and vertigo remain the two most common complications of cochlear implantation. Failure of the device is the third most frequent complication (10.06% of all complications and 1.53% of cochlear implantations). Other rare conditions (such as granulating labyrinthitis with cochlear fibrosis, ossification and erosion, silicone allergy and the formation of a biofilm around the internal device) are possible and unpredictable. Although rare (approximately 1%), such cases may require explantation. CONCLUSIONS: Despite efforts by both surgeons and manufacturers, device-related and surgical complications still occur. These and other rare conditions demand specific management, and their frequency may be underestimated. Further studies are needed to assess more realistic rates of complications and devise more efficient strategies for early diagnosis and treatment.

Tinnitus and cochlear implants.

OBJECTIVE: Several published studies seem to confirm the clinical observation that cochlear implants (CI) have a suppressive effect on tinnitus in most CI users. The aim of this paper is to present our personal experiences on tinnitus improvement after cochlear implantation. METHODS: Before surgery, 51 post-lingually profoundly deaf adults, who underwent cochlear implantation between 2005 and 2007, filled out a questionnaire reporting tinnitus characteristics and the Italian version of the Tinnitus Handicap Inventory (THI-I). Moreover, they were asked to scale tinnitus loudness and annoyance. Six months after implant activation, patients repeated the same evaluations. Scores were statistically analysed by means of a Wilcoxon signed ranks test on the hypothesis that a CI could change the tinnitus magnitude and/or its annoyance. RESULTS: In our series of 36 patients, 36.10% reported total loudness suppression and another 41.6% reported a reduction. Annoyance scores reduced in 75% of patients. THI reduced in 72.2% of patients. CONCLUSION: Tinnitus reduction after CI may manifest as several mechanisms, such as habituation, acoustic masking, direct electrical nerve stimulation and cortical reorganization. Nevertheless, it is our opinion that data on CI benefits on tinnitus should be interpreted with caution and it seems reasonable to admit that further data is still necessary before considering CI as a treatment of tinnitus especially in unilateral deafness.

Vertigo and autoimmunity.

Autoimmune inner ear disease probably accounts for less than 1% of all cases of balance disorders, but its incidence is often overlooked due to the absence of a specific diagnostic test. Furthermore, in several systemic autoimmune diseases the vestibulo-cochlear system may be affected. Clinical features comprise generalized imbalance, ataxia, motion intolerance, episodic vertigo and positional vertigo. An autoimmune mechanism seems to be responsible for 6% of unilateral and 16% of bilateral forms of Ménière's disease. Oscillopsia and disequilibrium secondary to a bilateral vestibular paresis are probably caused by an autoimmune response in 5% of cases. Balance disorders of central origin may be due to other immuno-mediated disorders such as multiple sclerosis, brainstem encephalitis and vasculitidis. Aim of this paper is to assess the clinical features of autoimmune vertigo disorders through a systematic literature review.

Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection.

The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech-language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 +/- 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.

The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls.

The purpose of this paper is to review the current diagnostic work-up for patients with suspected Autoimmune Inner Ear Disease (AIED). AIED is a rare disease accounting for less than 1% of all cases of hearing impairment or dizziness, characterized by a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. While specific tests for autoimmunity to the inner ear would be valuable, at the time of writing, there are none that are both commercially available and proven to be useful. Thus far, most of the identified antigens lack a clear association with localized inner ear pathology and the diagnosis of AIED is based either on clinical criteria and/or on a positive response to steroids. For clinical practice, we recommend an antigen-non-specific test battery including blood test for autoimmune disorders and for conditions that resemble autoimmune disorders. Nevertheless, if financial resources are limited, a very restricted work-up study may have a similar efficiency.

Merkel cell tumour of the external ear. Report of a case.

Merkel cells carcinoma (MCC) is an uncommon skin lesion, considered a malignancy of the neuroendocrine system, which is found mainly in elderly people. Its incidence is highly correlated with sun exposure or immunodeficiency syndromes. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastasis. To our best knowledge 20 cases originated from the auricle have been described, 2 of them arising from external ear canal. The authors report a case of the ear canal characterized by two others synchronous tumours and the occurrence of a malignant high grade lymphoma, in which contribute of the pathologist was essential for a critical review. MCC diagnosis is not always easy for its pathological and clinical features and it should always be considered in presence of lymphoma. A multidisciplinary approach is basic.