Late Bilateral Sequential Cochlear Implant and Quality of Life.

Introduction Hearing impairment is one of the main disorders that can interfere with the development of speech and language. In an individual, it can cause significant communication difficulties, social isolation, negative feelings, and depressive disorders. The hearing aids (HAs) and cochlear implant (CI) are options for profound and severe hearing loss, and the CI can be indicated for individuals who do not obtain benefits from HAs. Objective To evaluate the quality of life of individuals who underwent sequential bilateral CIs with a long surgical interval between procedures. Methods Fifteen patients, aged 8 to 70 years old, who underwent sequential bilateral CI, with an interval ≥ 4 years between surgeries, were evaluated. Quality of life was evaluated using three questionnaires: WHOQOL-BREF, SSQ-12 and HHIA in Portuguese. Results The WHOQOL-BREF questionnaire showed that the study participants had a good quality of life in all domains assessed. According to the SSQ-12, few reported inability to listen in communication situations. Most individuals were classified as having medium disability by the HHIA, but the social and emotional effects did not significantly affect the quality of life. Conclusion The use of questionnaires to assess the quality of life of patients with hearing impairment is a valuable tool to measure adaptation to CI. Patients undergoing bilateral sequential CI, even with a long interval between procedures, presented high indices of quality of life.

New variants of ALMS1 gene and familial Alström syndrome case series.

OBJECTIVES: To report two new variants of ALMS1 gene and to discuss the audiological evolution and clinical phenotype in two pairs of siblings with Alström syndrome. REPORT: This paper is a multi-disciplinary diagnostic evaluation, with genetic and audiological analysis that aims to report two new variants of the ALMS1 gene and to discuss the audiological evolution and clinical phenotype in a case series of patients with familial Alström syndrome. Therefore, we describe 4 cases presenting a complete audiometric profile of two pairs of unrelated siblings, to provide a better understanding of this very rare disease. Additionally, the present study identified two heterozygous mutations in the ALMS1 gene. CONCLUSION: This Clinical Capsule Report highlights the importance of audiological monitoring throughout the development of patients with Alström syndrome. The two variants found were not previously reported in the literature, which expands the spectrum of ALMS1 variants in Alström syndrome.

Middle Ear Cholesteatoma and Vestibular Schwannoma Resection Followed by Cochlear Implant: Surgical Challenges and Audiological Outcomes.

(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such cases. Moreover, the presence of middle and inner ear pathologies combined may represent a surgical challenge. No studies have described a combined surgical approach for these coexisting conditions (VS and cholesteatoma), nor the hearing rehabilitation outcomes of using cochlear implants for these patients. (2) Case Report: This paper is on a female patient who underwent simultaneous surgical treatments for VS and middle ear cholesteatoma in the right ear followed by a cochlear implant, describing the technique and the audiological results. (3) Conclusions: The surgical approach was successful and enabled the resection of lesions with the auditory nerve and cochlea preservation. Cochlear implantation in the right ear showed positive postoperative results, with an improvement in the results with the CI in silent and noisy environments.

Cochlear Implantation in Children with Auditory Neuropathy: Meta-Analysis.

BACKGROUND: Auditory neuropathy (AN) is a nosological entity of unknown etiology, which is associated with fluctuations in rates of speech discrimination. Its diagnosis is based on presence of otoacoustic emissions and lack of, or abnormal, brainstem auditory evoked potential. With respect to treatment, we have variable results in the literature about development of speech perception and skills, in children with AN and cochlear implant (CI) rehabilitation. OBJECTIVES: Comparatively assessing results recorded for the development of auditory and speech skills in children with auditory neuropathy spectrum disorder (ANSD), who were subjected to cochlear implantation, in comparison to results recorded for children with sensorineural hearing loss associated with other causes was the objective of this study. METHOD: A systematic literature review with meta-analysis was performed, with studies published from 1975 to 2023. RESULTS: Nineteen studies were included in the systematic review, and eight were selected for the meta-analysis, which showed there was no evidence allowing the conclusion that the two groups were different from each other about results in speech performance after 1 year of CI placement. CONCLUSION: Therefore, this study shows that CI provides the comparable benefit to children with ANSD in comparison to children with neurosensory hearing loss associated with other causes in their speech development.

Benefits of Bimodal Stimulation to Speech Perception in Noise and Silence.

Introduction Understanding all the benefits of bimodality with self-assessment questionnaires on the effect of hearing on quality of life is still necessary. Objective To present whether bimodality still offers hearing benefits to the population who uses acoustic stimulation associated with electrical stimulation. Methods The present study included 13 participants aged between 16 and 80 years old who were users of cochlear implants from Cochlear Corporation and hearing aids. All patients underwent the Hearing in Noise Test, and their visual analog scale score was obtained. Four-tone means were collected, and the participants answered the Speech, Spatial and Hearing Qualities questionnaire. Results Bimodal users had an average sentence recognition rate of 76.0% in silence and 67.6% in fixed noise, and the signal-to-noise ratio in adaptive noise was +2.89dB. In addition, a lower level of difficulty was observed in the test using the visual analog scale. The domain with the highest average was auditory qualities (6.50), followed by spatial hearing (6.26) and hearing for speech (5.81). Individuals with an average between 50 and 70 dB of hearing level showed better sentence recognition in silence and noise. Conclusion Bimodal stimulation showed benefits for users with different degrees of hearing loss; however, individuals who presented greater hearing residue had better performance in speech recognition with noise and in silence in addition to a good perception of hearing quality.

Novel cochlear implant assessment tool: Comparative analysis of children and adults.

Objectives: To analyse the results of children and adults with cochlear implants (CIs) in pure tone audiometry (PTA) and speech perception tests. Tests were performed in two ways: using loudspeakers in the sound booth (SB) and with direct audio input (DAI) employing the Cochlear Latin America BOX (CLABOX). Methods: Fifty individuals (33 adults and 17 children) participated in the study, including children aged between 8 and 13 years; of these, 15 users had bilateral CIs, 35 had unilateral CIs, and all had severe to profound bilateral sensorineural hearing loss. All participants were evaluated in the SB with loudspeakers and the CLABOX with DAI. The following evaluations were conducted: PTA, speech recognition tests with the hearing in noise test (HINT). Results: The results for PTA and HINT conducted in SB and with CLABOX presented no significant difference between children and adults. Conclusion: The CLABOX tool presents a new possible method to evaluate PTA and speech recognition tests in adults and children, with results comparable to the conventional evaluation in the SB.

IgG4-Related Disease of Temporal Bone Presenting as Unilateral Mastoiditis and Cerebral Thrombosis in a 22-Year-Old Man.

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a rare autoimmune disease that can affect multiple organs and manifest itself as a mass at any region of the body. Due to its several differential diagnoses, investigation and treatment are still challenging. Therefore, imaging, serology, and histopathology are required to confirm the diagnosis. The involvement of the temporal bone is an uncommon presentation, often mistaken for malignancy, with vague symptoms. Therefore, we present a 22-year-old Brazilian man, diagnosed with IgG4-related disease, manifesting with unilateral mastoiditis, sensorineural hearing loss, cerebral venous sinus thrombosis, and a mass in the left temporal bone. CASE REPORT A 22-year-old Brazilian male patient first presented with coughing and precordialgia. Chest scans showed pleural effusion and diffuse areas of ground-glass opacity. A year later, the patient developed severe headache, along with aural fullness, facial pressure, and otorrhea. Imaging detected cerebral thrombosis with failure in the filling of the transverse and left sigmoid sinuses and pachymeningeal thickening in the right cerebral hemisphere, with contrast enhancement. Pure tone audiometry showed thresholds consistent with severe sensorineural hearing loss in the left ear. The patient underwent mastoidectomy with removal of large amounts of inflammatory tissue that were sent to histopathological analysis with compatible signs of IgG4-RD. Corticosteroids and rituximab completed the treatment. CONCLUSIONS Early recognition and appropriate treatment of IgG4-RD are imperative to avoid complications and serious irreversible organ damage. This report has presented an atypical case of IgG4-RD of the left temporal bone that was diagnosed and managed according to current guidelines.

Cochlear implant in tinnitus management.

PURPOSE OF REVIEW: To evaluate whether cochlear implantation can mitigate tinnitus perception and its discomfort among patients with severe-to-profound hearing loss, in order to provide an answer on a possible treatment for a health condition with high prevalence and impact on quality of life. RECENT FINDINGS: Tinnitus can develop after peripheral hearing loss and is associated with altered auditory processing. It does not only involve auditory structures but also aberrant neural activity and interaction with other regions of the Central Nervous System associated with emotion, attention, anguish, memory and motor activity. SUMMARY: Recent studies investigated the changes in tinnitus perception among patients who underwent cochlear implantation and demonstrated a postoperative decrease. Hence, patients with severe-to-profound sensorineural hearing loss and tinnitus are likely to benefit from cochlear implantation.

Central Vertigo Disorder as Evidence of Creutzfeldt-Jakob Disease: A Case Report.

OBJECTIVE: To report a case of Creutzfeldt-Jakob disease (CJD) that presented atypical initial manifestations and highlight the importance of complementary investigation to avoid diagnosis delay. PATIENTS: A 54-year-old female patient who presented with vertigo with positional nystagmus, nausea, and vomiting as initial CJD manifestations. INTERVENTION: Neuro-otological and brain magnetic resonance images, which showed abnormal hyperintense cortical signal in both hemispheres. MAIN OUTCOME MEASURE: We reached a presumptive diagnosis of the prion disease after the first magnetic resonance imaging. Eventually, the patient presented with typical neurological findings and met the criteria for probable CJD. RESULTS: Our case report presents a patient with sporadic CJD who experienced dizziness as an initial manifestation and met the diagnostic criteria for probable CJD a few weeks after symptoms onset. CONCLUSION: We believe that this case may serve to help otolaryngologists pay better attention to cases of dizziness associated with neurological signs and highlight the importance of complementary investigation using magnetic resonance imaging and neuro-otological tests to prevent delayed or incorrect diagnosis.

Surgical Treatment Outcome for Familial Melkersson-Rosenthal Syndrome.

BACKGROUND Recurrent facial nerve palsy, orofacial edema, and fissured tongue are a triad of manifestations that characterize a rare disorder named Melkersson-Rosenthal syndrome. It is important to consider this syndrome when diagnosing atypical, unilateral, or bilateral facial palsies with characteristics of familial prevalence. There is no established outcome prediction for this disease and the syndrome does not have a specific duration or prospective timeline. Recurrent facial paralysis can require surgery and a multidisciplinary approach with regular follow-up. CASE REPORT We describe a 38-year-old woman presenting with a third episode of facial paralysis and discuss her pedigree chart and the treatment course chosen. After conservative treatment with oral corticosteroids, antiviral therapy, and motor physical therapy with no significant improvements, the patient underwent facial nerve decompression surgery with outstanding results. Eight months after surgery and intense postoperative physical therapy, the patient improved from grade VI to grade II palsy on the House-Brackmann Scale. The patient's older brother also presented a fissured tongue and had a history of 2 episodes of facial paralysis. The patient's son, mother, and sister also presented tongue fissuring but did not have any other clinical signs of the syndrome. CONCLUSIONS Despite being rare, Melkersson-Rosenthal syndrome is associated with a family inheritance and its diagnosis has prognostic implications. Therefore, it is of the utmost importance to have suspicion of this disorder in order to improve quality of care and target the treatment accordingly. Surgical treatment in these cases seems to be an excellent choice to treat current facial paralysis and prevent further episodes.

Task force Guideline of Brazilian Society of Otology - hearing loss in children - Part II - Treatment.

OBJECTIVES: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0-18 years. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: (1) treatment of sensorineural hearing loss: individual hearing aids, bilateral cochlear implants, cochlear implants in young children, unilateral hearing loss, and auditory neuropathy spectrum disorder; and (2) treatment of conductive/mixed hearing loss: external/middle ear malformations, ventilation tube insertion, and tympanoplasty in children. CONCLUSIONS: In children with hearing loss, in addition to speech therapy, Hearing AIDS (HAs) or implantable systems may be indicated. Even in children with profound hearing loss, both the use of HAs and behavioral assessments while using the device are important.

Task force Guideline of Brazilian Society of Otology ‒ hearing loss in children - Part I ‒ Evaluation.

OBJECTIVES: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0 to 18 years. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The evaluation and diagnosis of hearing loss: universal newborn hearing screening, laboratory testing, congenital infections (especially cytomegalovirus), genetic testing and main syndromes, radiologic imaging studies, vestibular assessment of children with hearing loss, auditory neuropathy spectrum disorder, autism spectrum disorder, and noise-induced hearing loss. CONCLUSIONS: Every child with suspected hearing loss has the right to diagnosis and appropriate treatment if necessary. This task force considers 5 essential rights: (1) Otolaryngologist consultation; (2) Speech assessment and therapy; (3) Diagnostic tests; (4) Treatment; (5) Ophthalmologist consultation.

A Rare Case of Giant Cavernous Hemangioma of the Maxillary Sinus.

BACKGROUND Hemangiomas are commonly located in the head and neck and rarely in the paranasal sinuses. These are benign vascular lesions, but with an increased risk of bleeding. The surgical approach must have detailed prior planning, given the increased risk of intraoperative bleeding. We herein describe the case of a 32-year-old male patient with recurrent epistaxis, nasal obstruction, and facial deformity due to a giant cavernous hemangioma successfully treated by endoscopic sinus surgery. CASE REPORT A 32-year-old man had nasal obstruction and intermittent epistaxis for 2 months. Physical examination also revealed facial deformity with enlargement of the nasal base and bulging in the maxillary region on the right. A soft and friable lesion occupying the entire right nasal cavity without bone erosion was observed on computed tomography (CT scan). Before surgery, the patient underwent angiographic evaluation, with evidence of main irrigation of the lesion by the right maxillary artery, which was then embolized. The patient underwent endoscopic nasal surgery. He maintained postoperative follow-up for 18 months, without recurrence of the lesion. Anatomopathological examination confirmed a cavernous hemangioma. CONCLUSIONS Cavernous hemangioma is a benign lesion of the paranasal sinuses. Due to non-specific clinical and radiological findings, its preoperative diagnosis is always challenging. The high index of suspicion of the malignancy should only be discarded after complete anatomopathological evaluation. A correct diagnosis is essential to avoid facial anatomical remodeling while excluding the diagnosis of other malignant lesions.

The effect of cochlear implant surgery on vestibular function in adults: A meta-analysis study.

Issue: The findings in literature indicate inconsistency in the complications caused by the implant of electrodes in the cochlea; vestibular alterations and balance disorders are mentioned as the most likely. Purpose: To evaluate, in literature, through the results of multiple vestibular function tests, the effects of cochlear implant surgery on postural stability in adult patients and to analyze. Hypothesis: From the PICO strategy, where the Population focuses on adults, Intervention is cochlear implant surgery, Comparisons are between implanted patients, and Outcomes are the results of the assessment of cochlear function, the research question was formulated: Are there deficits in vestibular function in adults undergoing cochlear implant placement? Method: Systematic review based on cohort, case-control, and cross-sectional observational studies. Information sources: Databases between 1980 and 2021, namely, PubMed, Cinahl, Web Of Science, Cochrane, and Scopus. Search strategy using Mesh terms: "Adult," "Cochlear Implant," "Postural Balance," "Posturography," "Cochlear Implant," "Dizziness," "Vertigo," "Vestibular Functional Tests,"and "Caloric Tests." Populational inclusion criteria: studies with adult patients; intervention: cochlear implant placement surgery; comparison: analysis of a vestibular function with vestibular test results and pre- and postoperative symptoms; outcome: studies with at least one of the vestibular function tests, such as computerized vectoelectronystagmography (VENG), vestibular-evoked myogenic potentials (VEMPs), caloric test, video head impulse test (VHIT), head impulse test (HIT), videonystagmography, (VNG) and static and dynamic posturography. Exclusion criteria: studies without records of pre- and postoperative data collection and studies with populations under 18 years of age. Screening based on the reading of abstracts and titles was performed independently by two reviewers. In the end, with the intermediation of a third reviewer, manuscripts were included. Risk of bias analysis, performed by two other authors, occurred using the JBI "Critical Appraisal Checklist." Results: Of the 757 studies, 38 articles met the inclusion criteria. VEMP was the most commonly used test by the studies (44.7%), followed by the caloric test (36.8%) and vHIT (23.6%). Most studies performed more than one test to assess vestibular function. Conclusion: Among all vestibular tests investigated, the deleterious effects on vestibular function after cochlear implant surgery were detected with statistical significance (P < 0.05) using VEMP and caloric test. Comparing abnormal and normal results after implant surgery, the vestibular apparatus was evaluated as having abnormal results after cochlear implant surgery only in the VEMP test. The other tests analyzed maintained a percentage mostly considered normal results. Systematic review registration: identifier: CRD42020198872.

Analysis of Gabapentin's Efficacy in Tinnitus Treatment: A Systematic Review.

OBJECTIVES: Tinnitus can be a chronic symptom that brings disability and distress. Some studies suggested that gabapentin might be effective on tinnitus relief. The objective of the study is to perform a systematic review in order to evaluate the efficacy of oral gabapentin in patients with tinnitus. METHODS: A literature search was conducted in English and following the recommendations from PRISMA. The terms used were: ("tinnitus" OR "subjective tinnitus") AND ("gabapentin"). The study selection was performed following the eligibility criteria in accordance to the PICOS (population, intervention, comparison, outcome, study design) strategy-patients with tinnitus; oral gabapentin; placebo; reduction of tinnitus severity questionnaires scores; prospective, double-blind, randomized controlled trial, respectively. The selected studies were included in qualitative synthesis. The studies were analyzed according to Joanna Briggs Institute's critical appraisal checklist for randomized controlled trials. RESULTS: One hundred twenty-one studies were found in 9 databases and 8 studies were found in gray literature. After study selection, 6 articles were read in full. Then, 2 studies were excluded and 4 were included in qualitative synthesis. All 4 articles were analyzed according to critical evaluation checklist. CONCLUSIONS: There is insufficient evidence to recommend the use of gabapentin for patients with tinnitus.

Titanium prostheses versus stapes columella type 3 tympanoplasty: a comparative prospective study.

INTRODUCTION: Tympanoplasty is a surgical procedure designed to reconstruct the mechanisms of sound transmission in the middle ear. OBJECTIVE: Analyze, from an audiological point of view, patients with chronic otitis media undergoing type 3 tympanoplasty major columella with total ossicular replacement titanium prosthesis or with cartilage graft stapes columella. METHODS: This is a prospective analytical study, carried out at the otorhinolaryngology outpatient clinic in a tertiary care hospital, through the evaluation of 26 patients with chronic otitis media who underwent tympanoplasty using different materials for auditory rehabilitation such as titanium prostheses or cartilage autografts. RESULTS: There was no statistically significant association between the group factors (cartilage or titanium reconstruction) and preoperative variables. There was no statistically significant association between the postoperative characteristics of the patients and the type of reconstruction. Neither subjective improvement (hearing improvement) nor residual perforation were associated with a type of material. The via factor was the only one that showed a statistically significant difference once air-conduction pathway improved more than bone-conduction pathway, decreasing the air-bone gap. CONCLUSION: There was no statistical difference between the two groups in relation to the audiometric improvement. There was hearing improvement in both groups. More studies must be done with a longer follow-up to better evaluate the outcome.

Is there benefit from the use of cochlear implants and hearing aids in cognition for older adults? A systematic review.

Objectives: The aim of the study was to assess whether hearing aids (HA) and cochlear implants (CI) bring benefits to cognition or mitigate cognitive decline in older adults. Methods: This is a systematic literature review registered on the International Prospective Register of Systematic Reviews (PROSPERO) and based on the criteria recommended by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). The Population, Intervention, Comparison, Outcome, and Study type (PICOS) strategy was used to define eligibility. Studies that met the criteria were included in the qualitative synthesis. We assessed the risk of bias through the Joanna Briggs Institute Critical Appraisal Checklists. Results: A total of 3,239 articles, found in eight databases, addressed the relationship between HA, CI, and cognition. We selected 30 experimental articles reporting measures of cognitive outcomes for older adults to include in the qualitative analysis. Of those, 23 studies reported a significant improvement in outcome and seven reported no significant change. Conclusions: This systematic review indicates that CI and HA can bring benefits to cognition in older adults. Systematic review registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021273690.

The effect of caffeine on tinnitus: Randomized triple-blind placebo-controlled clinical trial.

OBJECTIVE: To test the hypothesis that caffeine can influence tinnitus, we recruited 80 patients with chronic tinnitus and randomly allocated them into two groups (caffeine and placebo) to analyze the self-perception of tinnitus symptoms after caffeine consumption, assuming that this is an adequate sample for generalization. METHODS: The participants were randomized into two groups: one group was administered a 300-mg capsule of caffeine, and the other group was given a placebo capsule (cornstarch). A diet that restricted caffeine consumption for 24 hours was implemented. The participants answered questionnaires (the Tinnitus Handicap Inventory-THI, the Visual Analog Scale-VAS, the profile of mood state-POMS) and underwent examinations (tonal and high frequency audiometry, acufenometry (frequency measure; intensity measure and the minimum level of tinnitus masking), transient otoacoustic emissions-TEOAE and distortion product otoacoustic emissions-DPOAE assessments) at two timepoints: at baseline and after capsule ingestion. RESULTS: There was a significant change in mood (measured by the POMS) after caffeine consumption. The THI and VAS scores were improved at the second timepoint in both groups. The audiometry assessment showed a significant difference in some frequencies between baseline and follow-up measurements in both groups, but these differences were not clinically relevant. Similar findings were observed for the amplitude and signal-to-noise ratio in the TEOAE and DPOAE measurements. CONCLUSIONS: Caffeine (300 mg) did not significantly alter the psychoacoustic measures, electroacoustic measures or the tinnitus-related degree of discomfort.