The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis.

BACKGROUND: A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment. Although MRI-compatible cochlear implants are now available, concerns about the feasibility of recoverable hearing with cochlear implantation in these patients as well as the practical difficulty of disease monitoring due to implant artifact must be considered. RESULTS: We present 3 recent cases from different institutions. The first is a 39-year-old man with a history of progressively worsening hearing loss, followed by visual loss, delusions, agitation, ataxia, and musical auditory hallucinations, diffuse leptomeningeal enhancement on MRI with a normal serum angiotensin-converting enzyme (ACE) level but elevated cerebrospinal fluid (CSF) ACE levels, suggesting neurosarcoidosis, was treated with corticosteroids, and underwent successful cochlear implantation. The second is a 36-year-old woman with rapid-onset horizontal diplopia, left mixed severe sensorineural hearing loss (SNHL) and tinnitus, diffuse leptomeningeal enhancement on MRI, and progressive palsy of the left CNs IV, VI, VII, IX, X and XI, with altered mental status requiring admission following high-dose intravenous corticosteroids. The third is a 15-year-old boy who presented with sudden, bilateral, profound SNHL, recurrent headaches, and left facial weakness refractory to antivirals, ultimately diagnosed with neurosarcoidosis following an aborted cochlear implantation where diffuse inflammation was found, and histopathology revealed Schaumann bodies; he was treated with methotrexate and later underwent successful cochlear implantation. CONCLUSIONS: Neurosarcoidosis is an elusive diagnosis and can cause hearing loss and psychiatric symptoms. Cochlear implantation for patients with severe hearing loss should be considered once the diagnosis is confirmed, as it is possible to achieve a successful level of hearing. Psychiatric symptoms can manifest with the onset of neurosarcoidosis, result from CN deficits, or develop as a side effect from long-term, high-dose corticosteroids, and should be monitored carefully in patients with neurosarcoidosis.

Cryptococcal meningitis with isolated otologic symptoms.

Sensorineural hearing loss (SNHL) is a known complication of cryptococcal meningitis; however, it is unusual for a patient to present with isolated otologic symptoms. We review the case of a patient who is not immunocompromised and who presented with progressive gait instability and sudden onset of left-sided SNHL followed by progression to bilateral SNHL within a 3-week period. Cryptococcal meningitis was confirmed by lumbar puncture with positive cryptococcus antigen in the cerebrospinal fluid. The patient was treated with systemic antifungals, and the hearing loss persisted. The presented report outlines this patient's unusual presentation and his treatment course and reviews the literature on the otologic manifestations of cryptococcal meningitis.

Phylogeny of the stapes prosthesis.

OBJECTIVE: To create the first ever stapes prosthesis phylogenetic tree for the evolution of the stapes prosthesis. STUDY DESIGN: Retrospective literature review, personal interviews. SETTING: University Medical Center. PATIENT: Not applicable. INTERVENTION: Not applicable. MAIN OUTCOME MEASURES: Construction of a stapes prosthesis phylogenetic tree with branches capable of including all stapes prostheses. RESULTS: One hundred five different stapes prostheses were reviewed, starting with the first-ever prosthesis used in the first stapedectomy and continuing up to the present time. The stapes prosthesis family tree contains 4 main branches that allow for categorization of all the commercial prostheses currently used. Many examples of atavistic prostheses, single surgeon use, and dead-end characteristics exist. CONCLUSION: An overview of the complicated phylogenetic tree for stapes prostheses gives great perspective to the history of stapedectomy and insights into many characteristics that are useful for designing new stapes prostheses.

Patient, disease, and outcome characteristics of benign paroxysmal positional vertigo with and without Meniere's disease.

BACKGROUND: Meniere's disease (MD)-associated benign paroxysmal positional vertigo (BPPV) is complex and difficult to diagnose, and reports of its prevalence, pathologic features and outcomes are sparse and conflicting. OBJECTIVE: Report disease characteristics and outcomes associated with the presence of MD in patients with BPPV. MATERIALS/METHODS: A retrospective study of patients with BPPV between 2007 and 2017 at a single, high-volume institution. RESULTS: Of 1581 patients with BPPV identified, 7.1% had MD and 71.9% of those patients had BPPV in the same ear(s) as MD. Patients with MD were more likely to have lateral semicircular canalithiasis (11.6% vs. 5.5%, p = .009) and multiple canalithiasis (7.1% vs. 2.5%, p = .005). MD was associated with an increased rate of resolution of BPPV (p = .008) but also increased time to resolution (p = .007). There was no association between MD and recurrence of BPPV. CONCLUSIONS: MD is associated with lateral canalithiasis. Contrary to prior reports, BPPV in MD can affect either ear and was not associated with poorer outcomes than idiopathic BPPV. SIGNIFICANCE: The largest series to date investigating disease and outcome characteristics for BPPV in MD is presented. These data inform diagnosis and expectations in the management of these complex patients.

Magnetic properties of middle ear and stapes implants in a 9.4-T magnetic resonance field.

HYPOTHESIS: A 9.4-T magnetic resonance (MR) field may cause motion displacement of the middle ear and stapes implants not previously observed with 1.5- and 3.0-T magnets. BACKGROUND: Publications have described the safety limitations of some otologic implants in 4.7-T field and resulted in several companywide patient safety-related recalls. To date, no studies have been reported for otologic implants in a 9.4-T MR field nor have comparisons been made with 4.7-T field strengths. METHODS: Twenty-three commonly used middle ear and stapes prostheses were selected and exposed to 9.4-T MR fields in vitro within petri dishes, and eight of the 23 implants were further studied ex corpus in human temporal bones (TBs) in a 9.4-T MR field. This study has been approved by the institutional review board. RESULTS: Eight prostheses in petri dishes grossly displaced at 9.4 T, three of which had not previously moved in either the 1.5- or 3.0-T magnets. The eight TB preparations showed no avulsions or motion indicators after exposure at 9.4 T. CONCLUSION: Middle ear and stapes implants can move dramatically in petri dishes at 9.4-T MR field, more so than at 1.5 and 3.0 T. The absence of avulsions in the TB group strongly suggests that the surgical means used to fixate the middle ear implants to the middle ear structures successfully overcomes the magnetic moment produced at MR field strengths up to 9.4 T. The use of MR imaging is not contraindicated by this study's findings.

Acoustic Reflex Screening of Conductive Hearing Loss for Third Window Disorders.

OBJECTIVE: This study examines the effectiveness of acoustic reflexes in screening for third window disorders (eg, superior semicircular canal dehiscence) prior to middle ear exploration for conductive hearing loss. STUDY DESIGN: Case series with chart review. SETTING: Outpatient tertiary otology center. SUBJECTS AND METHODS: A review was performed of 212 ears with acoustic reflexes, performed as part of the evaluation of conductive hearing loss in patients without evidence of chronic otitis media. The etiology of hearing loss was determined from intraoperative findings and computed tomography imaging. The relationship between acoustic reflexes and conductive hearing loss etiology was assessed. RESULTS: Eighty-eight percent of ears (166 of 189) demonstrating absence of all acoustic reflexes had an ossicular etiology of conductive hearing loss. Fifty-two percent of ears (12 of 23) with at least 1 detectable acoustic reflex had a nonossicular etiology. The positive and negative predictive values for an ossicular etiology were 89% and 57% when acoustic reflexes were used alone for screening, 89% and 39% when third window symptoms were used alone, and 94% and 71% when reflexes and symptoms were used together, respectively. CONCLUSION: Acoustic reflex testing is an effective means of screening for third window disorders in patients with a conductive hearing loss. Questioning for third window symptoms should complement screening. The detection of even 1 acoustic reflex or third window symptom (regardless of reflex status) should prompt further workup prior to middle ear exploration.

Otosclerosis: incidence of positive findings on high-resolution computed tomography and their correlation to audiological test data.

OBJECTIVES: Computed tomographic (CT) scanning with slices of 1 mm or more has not been sufficient to demonstrate otosclerotic foci in most cases to date. METHODS: We investigated the validity of CT scans with a 0.5-mm cubical scan technique, with and without planar reconstruction, and correlated these findings with audiological data. Forty-four temporal bone CT scans from 30 patients with conductive or mixed hearing loss were evaluated. RESULTS: Otosclerotic foci were visualized in 74% of the cases. With reconstruction at the workstation, the sensitivity increased to 85%. Whereas in fenestral otosclerosis a correlation was found between the size of the focus and the air-bone gap, no correlation was seen between the size of the focus and bone conduction thresholds with cochlear involvement. Otosclerotic foci in patients treated with sodium fluoride were smaller than those in patients without treatment. This finding may indicate a beneficial effect of sodium fluoride on otosclerotic growth. CONCLUSIONS: High-resolution CT scans are a valid tool that can be used to confirm, localize, and determine the size of clinically suspected otosclerotic foci.

Hearing preservation in patients with vestibular schwannoma treated with Gamma Knife surgery.

OBJECT: Hearing loss after Gamma Knife surgery (GKS) in patients with vestibular schwannoma has been associated with radiation dose to the cochlea. The purpose of this study was to evaluate serviceable hearing preservation in patients with VS who were treated with GKS and to determine if serviceable hearing loss can be correlated with the dose to the cochlea. METHODS: Forty patients with vestibular schwannoma with serviceable hearing were treated using GKS with a median marginal dose of 12.5 Gy (range 12.5-13 Gy) to the 50% isodose volume. Audiometry was performed prospectively before and after GKS at 1, 3, and 6 months, and then every 6 months thereafter. Hearing preservation was based on pure tone average (PTA) and speech discrimination (SD). Serviceable hearing was defined as PTA less than 50 dB and SD greater than 50%. RESULTS: The median cochlear maximum and mean doses were 6.9 Gy (range 1.6-16 Gy) and 2.7 Gy (range 0.7-5.0 Gy), respectively. With a median audiological follow-up of 35 months (range 6-58 months), the 1-, 2-, and 3-year actuarial rates of maintaining serviceable hearing were 93%, 77%, and 74%, respectively. No patient who received a mean cochlear dose less than 2 Gy experienced serviceable hearing loss (p = 0.035). Patients who received a mean cochlear dose less than 3 Gy had a 2-year hearing preservation rate of 91% compared with 59% in those who received a mean cochlear dose of 3 Gy or greater (p = 0.029). Those who had more than 25% of their cochlea receiving 3 Gy or greater had a higher rate of hearing loss (p = 0.030). There was no statistically significant correlation between serviceable hearing loss and age, tumor size, pre-GKS PTA, pre-GKS SD, pre-GKS Gardner-Robertson class, maximum cochlear dose, or the percentage of cochlear volume receiving 5 Gy. On multivariate analysis there was a trend toward significance for serviceable hearing loss with a mean cochlear dose of 3 Gy or greater (p = 0.074). Local control was 100% at 24 months. No patient developed facial or trigeminal nerve dysfunction. CONCLUSIONS: With a median mean cochlear dose of 2.7 Gy, the majority of patients with serviceable hearing retained serviceable hearing 3 years after GKS. A mean cochlear dose less than 3 Gy was associated with higher serviceable hearing preservation.

Large asymptomatic pneumocephalus developing years after middle cranial fossa surgery--a case report.

Whereas acute pneumocephalus has been well characterized in the literature: typically seen postoperatively after neurosurgical and neurotological procedures, a chronic pneumocele developing several years after surgery is highly unusual. Most otogenic pneumocephali develop in an acute or subacute fashion, presenting with focal neurological symptoms, headache or signs of meningitis secondary to translocation of bacteria into the cavity. We describe a patient with a supra-auricular soft-tissue swelling as the only presenting symptom of a large chronic epidural pneumocele with extension into the extracranial subcutaneous tissues. It presented several years after surgical resection of a meningioma that involved the temporal bone.

BAHA devices and magnetic resonance imaging scanners.

HYPOTHESIS: That an osteointegrated Bone-anchored Hearing Aid (BAHA System) will show movement or displacement when exposed to a 9.4-T magnetic resonance imaging (MRI) magnet and that any movement may have implications for patient safety and image quality. BACKGROUND: BAHA System implanted patients may require MRI scans for various pathologies. The possibility of BAHA fixture and abutment movement can be explored to help clarify patients' and physicians' decisions regarding safety issues. SETTING: University Medical Center (tertiary care center). METHODS: BAHA System assembled fixture with abutment of 3 and 4 mm were each placed inside of a Petri dish and mounted into human cadaver temporal bones, then exposed to a 9.4-T MRI magnet. The BAHA System external processor was entered into the MRI suite. Head MRI images and charts of 2 patients with BAHA System devices who had undergone MRI scanning at 1.5 and 3.0 T, respectively, were reviewed. RESULTS: The BAHA System fixture with abutment moved inside of the Petri dish. The temporal bone studies revealed no sign of motion or displacement. The external BAHA processor showed strong movement toward the magnet. MRI scannings of patients were without any adverse effects. Image MRI quality was affected only around the BAHA post. CONCLUSION: The bone-mounted fixture with abutment of 3 and 4 mm resisted any magnetic forces trying to displace them up to 9.4 T. Two patients underwent scanning at 1.5 and 3.0 T with no adverse effects. MRI image quality was excellent starting at 1 cm from the fixture with abutment. The study findings lend strong evidence that patients can be safely scanned up to 9.4 T without adverse effects.