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This article discusses a case of a cerebellar flocculus pseudomass, a lesser-known lesion that can occur in the cerebellopontine angle (CPA). The patient, a 73-year-old female, presented with gradual onset of right-sided low frequency hearing loss and was initially diagnosed with a vestibular schwannoma. However, further imaging revealed characteristics inconsistent with a vestibular schwannoma, leading to the diagnosis of a cerebellar flocculus pseudomass. The article emphasizes the importance of comprehensive MRI studies for CPA lesions to avoid misdiagnosis and inappropriate treatment. The presentation of a cerebellar flocculus pseudomass with unilateral hearing loss is considered novel, and more information about this condition is needed in the literature. [Extracted from the article]

This article explores the use of smartphone and wearable activity monitoring as a marker for recovery after skull base surgery. The study included 19 patients who underwent anterior or lateral skull base surgery at NYU Langone Health. The results showed that patients achieved a 75% return to preoperative mobility by the sixth week, although the rates varied depending on the type of surgery. Additionally, the study found that the weekly step count after surgery was significantly correlated with the length of hospital stay. The findings suggest that passive smartphone and wearable activity monitoring could be a reliable metric for assessing outcomes in skull base surgery. [Extracted from the article]

Immune-checkpoint inhibitors (ICI) are being utilized with increasing frequency and may be linked to neurologic and audiovestibular toxicities. This report aimed to describe a case of ICI-induced sensorineural hearing loss ultimately requiring bilateral cochlear implantation. A 42-year-old female with stage IV metastatic melanoma of the perianal skin was treated with ipilimumab (blocker of cytotoxic T-lymphocyte associated protein 4 [CTLA-4]) and nivolumab (anti-programmed cell death protein 1 [PD1]). After 21 weeks of therapy, she developed sudden vertigo and bilateral hearing loss. A full workup including MRI and lumbar puncture ruled out intracranial parenchymal metastases, leptomeningeal metastases, stroke and intracranial infection. ICI-associated aseptic meningoencephalitis was therefore diagnosed. The patient received systemic steroids as well as intratympanic steroids, which temporarily improved hearing, but eventually developed permanent, bilateral profound sensorineural hearing loss. The patient received bilateral cochlear implants and has demonstrated good performance one year after implantation. ICI are being increasingly used to treat a variety of advanced malignancies. This is the first report of bilateral cochlear implants in the context of profound hearing loss after an immunotherapy induced meningoencephalitis. ICI carries the risk of potential ototoxicity, including profound SNHL as depicted in our case. Cochlear implantation proved to be beneficial and may be considered in patients with ICI-related hearing loss. [ABSTRACT FROM AUTHOR]

Objective: To assess a series of patients receiving teprotumumab therapy and objectively quantify the rates of otologic adverse events. Study Design: A prospective cohort study of adult patients receiving teprotumumab between May 2020 and January 2022. Setting: Tertiary referral center. Methods: Prior to treatment initiation, an ototoxicity‐specific audiometric battery was completed, which included conventional audiometry (frequencies 250‐8000 Hz), ultrahigh‐frequency audiometry (9000‐20,000 Hz), tympanometry, speech discrimination scores, and distortion product otoacoustic emissions (DPOAEs). Testing was then repeated after treatment completion. Results: In total, 35 patients were recruited, with a median (range) age of 48.5 years (21‐74), and 8 (22.8%) were male. The most common subjective symptom reported was a hearing decline (25.7%), followed by aural fullness (17.1%) and tinnitus (14.3%). Fourteen patients had both pre‐ and posttreatment audiometric data. Among them, 3 patients (21.4%) were found to have changes in standard frequency audiometry, and 10 (71.4%) had changes in high‐frequency audiometry, with 2 patients having changes in both. Less than half (n = 5) of the 11 patients with changes in standard or high‐frequency pure tone hearing noted subjective hearing decline. Changes in DPOAE were noted in 4 patients out of 13 (30.7%). Two patients discontinued treatment due to hearing decline. Finally, 3 patients (8.6%) were diagnosed with patulous eustachian tube (PET) by an otolaryngologist, and another 3 patients are suspected to have PET based on symptom description during ophthalmologic follow‐up. Conclusion: In our cohort, a high incidence of otologic symptoms was found to be associated with teprotumumab usage. Subjective hearing decline, changes in ultrahigh‐frequency hearing as well as eustachian tube dysfunction may be encountered and suggest the potential ototoxicity of teprotumumab. [ABSTRACT FROM AUTHOR]

Evaluate rates of Advanced Bionics Ultra 3D/Ultra cochlear implant failure in the setting of a worldwide device recall and report surgical and auditory outcomes after revision. Retrospective chart review was performed for adult and pediatric patients implanted with at risk devices at our center from 2016 to 2020. Device failure rates, surgical, and auditory outcomes were recorded and analyzed. Of 113 at-risk devices, 20 devices (17.7%) in 18 patients (two bilaterally implanted) were identified as failures. All devices were with mid-scala electrodes. Eleven patients (61.1%) were children and 7 (38.9%) adults. Twelve patients were found to have failing devices after reporting subjective performance decline; the remainder were prompted by manufacturer notification. All were revised, with the majority (83.3%) choosing the same manufacturer. All had uncomplicated original and revision insertions. Among adults, average word scores on the revised side were stable pre- to post-revision (P = 0.95). Patients with device failure due to this field action performed well after revision implantation. Patients with bilateral at-risk devices but evidence of unilateral failure may elect to undergo simultaneous empiric revision of the contralateral device. Three patients who elected to change device manufacturers on revision have variable results that require further investigation. Patients requiring revision for a device field action overall perform well. At-risk devices continue to require monitoring as a growing number are likely to fail over time. [ABSTRACT FROM AUTHOR]

Objective: To evaluate outcomes following salvage microsurgery (MS) and salvage stereotactic radiosurgery (SRS) after failure of primary treatment for vestibular schwannomas (VS). Study Design: Retrospective chart review. Setting: Tertiary referral center. Methods: Patients with more than 1 intervention for their VS were divided into 4 groups: MS followed by SRS (n = 61), MS followed by MS (n = 9), SRS followed by MS (n = 7), and SRS followed by SRS (n = 7), and outcomes were evaluated. Results: A total of 77 patients were included (84 procedures). In group 1 (MS then SRS), 3% developed a decline in facial function, 3% developed trigeminal sensory loss, and 13% patients had gradual improvement of facial nerve function following SRS. Group 2 (MS then MS) had the highest rates of facial nerve deterioration, although all but 1 patient achieved a House-Brackmann score of II or III. Gross-total resection (GTR) was achieved in 56% of patients. When a different approach was used for salvage resection, GTR occurred more commonly, and facial nerve outcomes were similar. In group 3 (SRS then MS), GTR occurred in 43% of cases, and 2 of 7 patients developed worsened facial function. In group 4 (SRS then SRS), no patient developed facial weakness after reirradiation, and 1 developed a trigeminal nerve deficit. Conclusions: For MS recurrences/residuals, SRS is the mainstay of treatment and does not preclude facial function recovery. If salvage microsurgery is required, an alternate approach should be considered. For SRS failures, when MS is required, less-than GTR may be preferable, and reirradiation is a potential safe alternative. [ABSTRACT FROM AUTHOR]

Objective: Preservation of residual low-frequency hearing has become a priority in cochlear implantation. The purpose of this study was to compare rates of hearing preservation and effects on performance of loss of low-frequency acoustic hearing with two different length electrodes.Study Design: Retrospective chart review.Setting: Tertiary Care Hospital.Patients: Twelve patients were implanted with the CI422 a slim-straight electrode; the second group consisted of 10 patients implanted with the Hybrid-L, a shorter hearing preservation electrode.Main Outcome Measure: Audiometric thresholds and speech perception measures.Results: At 1 year, 3/10 (30%) patients with the Hybrid-L and 7/12 (58%) patients with the CI422 lost residual acoustic hearing resulting in a profound hearing loss in the implanted ear. In comparing these patients in particular, mean CNC words in the implanted ear were 72% in the CI422 electrode group and 15% in the Hybrid-L electrode group at 1 year (P = 0.03). While hearing preservation rates with the Hybrid-L tended to be better, among recipients who lost residual hearing, speech perception was better in those with the longer CI422 electrode.Conclusions: With emphasis on preservation of residual hearing, patients need to be counseled regarding possible outcomes and options should loss of residual hearing occur following implantation. While shorter electrodes may have better rates of hearing preservation, the patients with the longer straight electrode in our study had significantly better speech understanding following the loss of residual hearing. [ABSTRACT FROM AUTHOR]

Debates the efficacy of microsurgery and radiosurgery in the treatment of the disease Vestibular Schwannomas. Details about the prevalence and symptoms of the disease; Three main treatment plans available to patients; Implications of microsurgery and radiosurgery.

Objectives/Hypothesis Surgery has been the most common treatment for acoustic neuromas, but gamma knife radiosurgery has emerged as a safe and efficacious alternative to microsurgery. This meta-analysis compares the outcomes of the two modalities. Study Design A retrospective MEDLINE search was used to find all surgical and gamma knife studies published from 1990 to 1998 and strict inclusion criteria were applied. Results For tumors less than 4 cm in diameter, there is no difference in hearing preservation ( P = .82) or facial nerve outcome ( P = .2). Surgery on all sized tumors has a significantly lower complication rate than radiosurgery performed on tumors smaller than 4 cm ( P = 3.2 × 10−14). Surgery also has a lower major morbidity rate than gamma knife radiosurgery ( P = 2.4 × 10−14). Tumor control was defined as no tumor recurrence or no tumor re-growth. Surgery has superior tumor control when tumors are totally resected ( P = 9.02 × 10−11). Assuming that all partially resected tumors will recur, surgery still retains a significant advantage over radiosurgery for tumor control ( P = .028). Conclusion Data from these studies date back to the late 1960s and do not completely reflect outcomes using current imaging and procedures. A major difficulty encountered in this study is inconsistent data reporting. Future surgical and radiation reports should use standardized outcomes scales to allow valid statistical comparisons. In addition, long-term results from gamma knife radiosurgery using lower dosimetry have not been reported. Surgery should remain the therapy of choice for acoustic neuromas until tumor control rates can be established. [ABSTRACT FROM AUTHOR]

Meningoencephaloceles of the temporal bone are rare. Although most often seen following otologic surgery or trauma, congenital meningoencephaloceles can exist. The clinical presentation, diagnostic evaluation, and surgical management of three patients with congenital meningoencephalocele are presented. Two of the three patients presented to our institution with recurrent episodes of meningitis; one presented with partial complex seizures. Diagnostic evaluation included temporal bone computed tomography with magnetic resonance imaging. In two patients, defects were imaged following high-pressure subarachnoid cisternography with computed tomography. All three patients were found to have congenital defects in the area of Meckel's cave. Early recognition of congenital meningoencephalocele is important to avoid delay of definitive surgical management and neurologic sequelae. [ABSTRACT FROM AUTHOR]

The emergence of magnetic resonance imaging with gadolinium has dramatically enhanced our ability to accurately detect the presence of acoustic tumors as small as 2 mm in diameter. Early diagnosis and improved surgical techniques continue to reduce the morbidity associated with surgical removal of these lesions. There exists, however, a select group of patients in whom no treatment may be the most appropriate management. Since 1979, a total of 51 patients with radiographic evidence of an acoustic neuroma have been prospectively followed for tumor growth and progression of symptoms. Patients were chosen for this conservative approach on the basis of age, medical condition, tumor size, audiometric data, and patient preference. This study reveals that a significant number of patients with acoustic tumors can be safely followed with regular imaging studies and may never require treatment. Discussed are tumor growth rates, epidemiology, and the impact of these factors on patient management. [ABSTRACT FROM AUTHOR]

Objectives: (1) Characterize pitch adaptation patterns in bimodal long‐electrode cochlear implant (CI) users over time, and correlate these with electrode discrimination and speech perception outcomes. (2) Compare to pitch plasticity observed in hybrid CI users. Methods: Observational cohort over a 12‐month period after CI activation or of long‐term CI experience. Severe to profound hearing impairment in 19 newly activated bimodal CI users and 20 long‐term CI users was studied in an audiology clinic. Outcome measures: Electric‐to‐acoustic pitch matches, audiometric thresholds, electrode discrimination performance, and speech perception scores, measured over time. Results: Recently activated (12 months) subjects had these pitch adaptation patterns: pitch‐adapting, pitch‐dropping, and pitch‐unchanging. Long‐term subjects had a parallel set of adaptation patterns: matched‐pitch, low‐pitch, and nonmatched‐pitch. Unlike hybrid CI users who are mostly pitch‐adapting/matched‐pitch, the majority of bimodal CI users demonstrated pitch‐dropping/low‐pitch or pitch‐unchanging/nonmatched‐pitch. Subjects with pitch‐adapting/matched‐pitch patterns trended towards better low‐frequency thresholds. Changes in electrode discrimination were not associated with pitch differences between electrodes. Reductions in speech perception scores over time were associated with dropping‐pitch patterns. Conclusions: Bimodal CI users with more residual hearing seem similar to hybrid CI users in adapting pitch perception to reduce mismatch with the frequencies allocated to the electrodes and acoustic hearing. In contrast, those with less residual hearing exhibit no adaptation or a drop in the pitches of the basal electrodes. Our data suggest that electrode discrimination does not depend on perceived pitch differences between electrodes. Speech perception may depend more on pitch perception and the ability to distinguish pitch between electrodes. [ABSTRACT FROM AUTHOR]