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Introduction CyberKnife (CK) radiosurgery is a treatment strategy for vestibular schwannoma (VS). Objectives To evaluate hearing preservation (HP) after CK for VS. Data Synthesis The study was conducted following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) statement, and it was registered at the International Prospective Register of Systematic Reviews (PROSPERO, under number CRD42021250300). The inclusion criteria were based on the population, intervention, comparison, outcome, timing and study design (PICOTS) strategy: population - patients with VS; intervention - CK; Comparison - none; Outcome - serviceable HP defined by Gardner and Robertson as grades I or II, or by the American Academy of Otolaryngology and Head and Neck Surgery as classes A or B; timing - mean follow-up longer than 1 year; and study design - retrospective or prospective studies. The exclusion criteria were: studies not published in English; studies published before January 2000 and after October 2021; and studies only including patients with neurofibromatosis type 2 or submitted to a previous treatment. The PubMed/MEDLINE, EMBASE, Web of Science, Cochrane Library, LILACS, and IBECS databases were used and last searched on October 27th, 2021. Statistical heterogeneity was assessed using I 2 statistics. The appraisal checklist was used to assess the risk of bias in the included studies. A total of 222 studies were analyzed, and 13 were included in the synthesis, which represents 493 participants with serviceable hearing before intervention. The mean HP rate after CK using a random effects model was of 68% (95% confidence interval [95%CI]: 59-76%) at a mean follow-up of 42.96 months. Conclusion The longer follow-up period was associated with a lower HP rate after CK radiosurgery for VS in the qualitative synthesis.

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BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach. CASE REPORT We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid. CONCLUSIONS This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.

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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.

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(1) Background: Performance after Cochlear Implantation (CI) can vary depending on numerous factors. This study aims to investigate how meningitis or otosclerosis can influence CI performance. (2) Methods: Retrospective analysis of CI performance in patients with etiological diagnosis of meningitis or otosclerosis, comparing the etiologies and analyzing the image findings, along with electrode array insertion status and technique. (3) Results: Speech recognition in CI patients with otosclerosis improves faster than in patients with meningitis. Other features such as radiological findings, degree of cochlear ossification, surgical technique used and total or partial insertion of electrodes do not seem to be directly related to speech recognition test performance. (4) Conclusions: Patients should be warned that their postoperative results have a strong correlation with the disease that caused their hearing loss and that, in cases of meningitis, a longer duration of speech-language training may be necessary to reach satisfactory results.

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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.

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(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.

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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.

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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.

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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.

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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.

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