Electrical stimulation of the cochlea for treatment of chronic disabling tinnitus: an open-label trial towards the development of an implantable device.

BACKGROUND: Chronic tinnitus affects millions of people globally and constitutes the most commonly compensated disability among military service members in the United States. Existing treatment options largely surround helping patients cope with their disease as opposed to directly suppressing tinnitus perception. The current study investigated the efficacy of electrical stimulation of the cochlea on chronic disabling tinnitus. METHODS: In this single-arm, open-label clinical trial, 22 adult subjects with severe-range asymmetric or unilateral non-pulsatile tinnitus underwent electrical stimulation of the cochlea through use of an extra-cochlear electrode positioned on the cochlear promontory. Each subject underwent 3 stimulation treatments over 3 weeks at 7-day intervals. Tinnitus severity was determined by Tinnitus Handicap Inventory (THI), Tinnitus Functional Index (TFI), and Tinnitus Visual Analog Scale (VAS). Inclusion criteria required subjects have no worse than moderate sensorineural hearing loss determined by pre-enrollment audiometric testing. The primary outcome was nadir post-treatment THI scores, obtained at seven timepoints following electrical stimulation, with clinically significant improvement defined as a decrease of ≥ 7. RESULTS: All 22 (100%) subjects experienced clinically significant improvement in the THI during the study period with a mean decrease in scores of - 31 (95% CI - 38 to - 25) from a baseline of 48. Twenty (91%) experienced clinically significant improvement detectable on at least two of the three tinnitus survey instruments and 17 (77%) experienced clinically significant improvement detectable on all three survey instruments (i.e., THI, TFI, and VAS). Eight (36%) subjects reported either complete (THI of 0; n = 3) or near-complete (THI 1-4; n = 5) suppression of their tinnitus following a stimulation session. Thirteen (59%) subjects reported a nadir following stimulation at or below the threshold for "no or slight handicap" on the THI (≤ 16). No adverse events were observed. CONCLUSIONS: These findings establish the foundation for the development of an extra-cochlear implantable device that delivers electrical stimulation to the cochlea for the treatment of disabling tinnitus. For patients considering device implantation, trans-tympanic cochlear promontory stimulation can facilitate patient selection. Trial Registration ClinicalTrials.gov Identifier: NCT03759834. URL: https://clinicaltrials.gov/ct2/show/NCT03759834.

Hyperbaric oxygen therapy for wound breakdown after oronasal fistula and cleft palate repair: four cases.

We offer the first report of hyperbaric oxygen (HBO2) therapy to treat early surgical wound breakdown after oronasal fistula and cleft palate repair in the pediatric population. We present four patients' experiences after undergoing HBO2 therapy. HBO2 was initiated as soon as an oronasal fistula was identified. Three of the children underwent 10 HBO2 treatments with the fourth undergoing 11 treatments. There were no adverse effects during treatment; none of the patients required decompressive myringotomy. Follow-up shows portions of the repair have been salvaged by initiating HBO2 at the first sign of fistula formation. In our case series, the fistulas all decreased in size over the course of treatment and demonstrated granulation tissue at the wound edges. Given the positive outcomes from our preliminary results, our results suggest concurrent usage of HBO2 therapy for wound breakdown after cleft palate repair is an effective treatment option.

Radiation-associated atypical vascular lesions: vascular lesions with endothelial cell atypia presenting in the radiation port of breast cancer patients.

Atypical vascular lesions are an uncommon adverse sequela to the radiotherapy of tumors. Many characteristics are shared between atypical vascular lesions caused by radiation port and well-differentiated radiation-induced angiosarcomas. The authors retrospectively reviewed the medical literature using PubMed, searching the terms acquired, atypical, benign, lymphangioma, lymphangioendothelioma, lymphangiomatous, lesion, papules, progressive, and vascular. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. Atypical vascular lesions associated with the radiation port present as clinically innocuous flesh-colored to erythematous papules or plaques. The condition presents within the radiation field, approximately 3 years after initial treatment. While the exact me chanism remains to be elucidated, growing evidence supports an association between radiation-associated atypical vascular lesions and radiation-induced angiosarcomas. Atypical vascular lesions within a radiation port are suggested to be in a state of morphologic continuum, which may progress into the more aggressive, malignant angiosarcoma. The authors recommend consideration for biopsy of new skin lesions within or adjacent to radiation. While it is clear that atypical vascular lesions caused by radiation are not equivalent to angiosarcoma, growing evidence supports that radiation-associated atypical vascular lesions may progress to angiosarcoma in some patients; therefore, the authors recommend excision of the lesion with margins depending on clinical judgment and the lesion encountered.

Imatinib-induced postoperative periorbital purpura: GASP (Gleevec-Associated Surgical Purpura) in a woman with imatinib-treated chronic myelogenous leukemia.

BACKGROUND: Imatinib mesylate is a selective tyrosine kinase inhibitor used in the treatment of chronic myelogenous leukemia. Ocular side effects of imatinib include periorbital edema, which may become so severe as to obstruct the visual field. PURPOSE: The purpose of this case study is to describe the clinical characteristics of imatinib- induced postoperative periorbital purpura. MATERIALS AND METHODS: We retrospectively reviewed the medical literature using PubMed, searching the terms edema, Gleevec, imatinib, periorbital, postoperative and purpura. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. RESULTS: Three patients have undergone surgery to reduce the imatinib-induced periorbital edema; two of these individuals have developed imatinib-induced postoperative periorbital purpura. CONCLUSION: We recommend discontinuing imatinib usage one week prior to periorbital surgery and not resuming therapy until the eighth postoperative day.

Radiation-induced angiosarcoma.

BACKGROUND: Radiation-induced angiosarcomas are uncommon adverse sequelae related to treatment of tumors. Early diagnosis and treatment are key to successful management. PURPOSE: The purpose of this case study is to describe the clinical characteristics of radiation-induced angiosarcomas. MATERIALS AND METHODS: We retrospectively reviewed the medical literature using PubMed, searching the terms angiosarcoma, breast, post, radiation, and treatment. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. RESULTS: Approximately one hundred patients have been diagnosed with radiation-induced angiosarcomas. The condition presents within the radiation field, approximately six years after initial treatment. We describe the dramatic efficacious response of our patient's angiosarcoma to adjuvant chemotherapy both preoperatively (gemcitabine and docetaxel) and postoperatively (gemcitabine and docetaxel followed by ifosfamide and adriamycin). CONCLUSION: We recommend that new skin lesions within or adjacent to radiation ports should be considered for biopsy. Also, for lesions that are larger, ill-defined, or both, several sites should be sampled to ensure an accurate diagnosis and to prevent the possibility of a false negative interpretation.

Assessing Patient Barriers to Cochlear Implantation.

OBJECTIVE: Evaluate barriers that deter adult patients from following through with cochlear implantation. STUDY DESIGN: Retrospective chart review and phone survey. SETTING: Single tertiary referral center. PATIENTS: Between January 2019 and August 2021, 113 patients, without a previous cochlear implant (CI), were determined to be candidates for cochlear implantation. Thirty-eight (33.6%) patients deferred cochlear implantation. Survey response rate was 61.1% (22/36). INTERVENTION: None. MAIN OUTCOME MEASURES: Demographic, socioeconomic, otologic history, and comorbidity factors associated with deferment of cochlear implantation. Patient survey assessment of factors that had the greatest impact, rated on a scale of 1 to 10 (10 being the most impactful), on their decision to defer a CI. RESULTS: Out of the 113 patients who met inclusion criteria, 75 (66.3%) underwent cochlear implantation and 38 (33.6%) patients deferred. Comparing implanted versus deferred groups, there was no statistical difference in age (67.1 y versus 68.5 y; p = 0.690) or male sex (53.3% versus 57.9%; p = 0.692). The deferred group had higher mean neighborhood disadvantage state decile (5.3 versus 4.3; p = 0.064) and national percentile (73.0 versus 66.2; p = 0.106) scores, although neither were statistically significant. A greater proportion of the deferred group were not living independently at time of CI evaluation (13.2 versus 2.7%; p = 0.017). Fear of losing residual hearing was rated the highest among the survey respondents (mean rating of 5.1), followed by general medical health (4.9) and cost and financial concerns (3.6). CONCLUSION: The greatest concern among the patients was the fear of losing residual hearing. Spending greater time educating patients about the success and failure rates of cochlear implantation may reduce patient hesitancy with implantation.

Racial Differences in Disease Presentation and Management of Intracranial Meningioma.

Objective/Hypothesis The aim of the study was to determine the impact of race on disease presentation and treatment of intracranial meningioma in the United States. Study Design This study comprised of the analysis of a national population-based tumor registry. Methods Analysis of the surveillance, epidemiology, and end results (SEER) database was performed, including all patients identified with a diagnosis of intracranial meningioma. Associations between race, disease presentation, treatment strategy, and overall survival were analyzed in a univariate and multivariable model. Results A total of 65,973 patients with intracranial meningiomas were identified. Of these, 45,251 (68.6%) claimed white, 7,796 (12%) black, 7,154 (11%) Hispanic, 4,902 (7%) Asian, and 870 (1%) patients reported "other-unspecified" or "other-unknown." The median annual incidence of disease was lowest among black (3.43 per 100,000 persons) and highest among white (9.52 per 100,000 persons) populations ( p < 0.001). Overall, Hispanic patients were diagnosed at the youngest age and white patients were diagnosed at the oldest age (mean of 59 vs. 66 years, respectively; p < 0.001). Compared with white populations, black, Hispanic, and Asian populations were more likely to present with larger tumors ( p < 0.001). After controlling for tumor size, age, and treatment center in a multivariable model, Hispanic patients were more likely to undergo surgery than white, black, and Asian populations. Black populations had the poorest disease specific and overall survival rates at 5 years following surgery compared with other groups. Conclusion Racial differences among patients with intracranial meningioma exist within the United States. Understanding these differences are of vital importance toward identifying potential differences in the biological basis of disease or alternatively inequalities in healthcare delivery or access Further studies are required to determine which factors drive differences in tumor size, age, annual disease incidence, and overall survival between races.

Increased Accuracy, Confidence, and Efficiency in Anterior Ethmoidal Artery Identification with Segmented Image Guidance.

OBJECTIVE: To determine whether using image guidance technology with 3-dimensional image segmentation increases the endoscopic surgeon's accuracy, efficiency, and confidence in identifying the anterior ethmoidal artery. METHODS: This is a cross-sectional study of attending physicians and residents at an academic medical center. Because identification of the anterior ethmoidal artery during image-guided surgery can be challenging, we studied the effect of anterior ethmoidal artery image segmentation (ie, partitioning and coloring) on surgeon test performance. A computerized test was administered to 16 surgeons who were asked to identify the anterior ethmoidal artery on multiplanar computed tomographic images and to answer multiple-choice questions. Half the questions showed segmented images of the anterior ethmoidal artery, and half showed images without segmentation. Efficiency and accuracy of identification and subjective surgeon confidence were determined for each question. Descriptive statistics were used to compare test performance for identification of the anterior ethmoidal artery on images with or without segmentation. RESULTS: Percentage of correct answers ( P < .001), efficiency ( P < .001), and confidence ( P < .001) in identification of the anterior ethmoidal artery were significantly better with segmented computed tomographic images. DISCUSSION: We demonstrated that use of segmented images improves surgeons' accuracy, confidence, and efficiency for identification of the anterior ethmoidal artery. IMPLICATIONS FOR PRACTICE: We describe how segmentation can allow surgeons to improve the surgical course by increasing their accuracy, confidence, and efficiency in identifying the anterior ethmoidal artery.

For Whom the Bell's Toll: Recurrent Facial Nerve Paralysis, A Retrospective Study and Systematic Review of the Literature.

PURPOSE: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. METHODS: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. RESULTS: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk-75 yr)] were evaluated for recurrent facial nerve paralysis at the authors' institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2-20). The median nadir House-Brackmann score was 4, with a median recovery to House-Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1-420 d). Diagnostic evaluation confirmed Melkersson-Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. CONCLUSION: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

Primary cervical leiomyoma: A rare cause of a posterior neck mass in a pediatric patient.

A 13-year-old male presents for evaluation of a right-sided posterolateral neck mass, first noted four years prior to presentation; incisional biopsy two years ago suggested a benign lymph node. Recent growth and increased pain prompted referral to our tertiary care center. MR imaging revealed a densely calcified mass in the right posterior paraspinous muscles with intense enhancement with gadolinium contrast, approximately 5 cm × 2.8 cm x 4.6 cm. Incisional biopsy showed leiomyoma with extensive dystrophic calcifications. This case describes a rare finding of extraesophageal leiomyoma of the neck; this is only the second such case reported in a pediatric patient.

Cochlear Implantation in Charcot-Marie-Tooth Disease: Case Report and Review of the Literature.

INTRODUCTION: Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. METHODS: Case report and review of the English literature. RESULTS: A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear. Audiometric testing confirmed profound SNHL in the left ear, while the right ear exhibited moderate-to-severe SNHL. Left-sided cochlear implantation was performed using a conventional length lateral wall electrode. Intraoperative device testing found normal impedance levels throughout the array; however, electrically evoked auditory potentials were absent on all electrodes. Upon initial activation 3 weeks after surgery, the patient reported excellent access to sound in the cochlear implant-only condition. He has made good progress at each subsequent visit; speech perception testing after seven months showed improvement from 0% to 32% on AzBio sentence and 53% on CNC phoneme testing in the cochlear implant-only condition. CONCLUSION: We report the third case of cochlear implantation in a patient with CMT. SNHL in CMT is hypothesized to result from disruption of synchronous activity of the cochlear nerve. In patients with CMT, cochlear implantation may reconstitute synchronous neural activity by way of supraphysiological electrical stimulation. Our results corroborate two earlier reports that cochlear implantation is a viable option for rehabilitation of SNHL in this unique subset of patients.

Presentation and Management of Giant Fibrovascular Polyps of the Hypopharynx and Esophagus.

OBJECTIVE: Fibrovascular polyps of the hypopharynx and esophagus are rare, with few case reports in the literature. In this article, we present our institutional experience with a focus on airway and surgical management. STUDY DESIGN: Case series. SETTING: Tertiary academic institution. METHODS: A retrospective review was conducted of 4 patients that presented to a tertiary medical center with fibrovascular polyps between 1990 and 2012. Patient demographics, clinical presentation, diagnostic studies, and surgical approaches were reviewed. A review of the published literature was also performed. RESULTS: The average age at presentation was 72 years (range, 59-85 years). Among the 4 patients, 2 presented with airway compromise requiring tracheotomy. All patients had removal of the polyp shortly after presentation; 2 underwent transcervical approaches with lateral pharyngotomy/esophagotomy, and the other 2 had endoscopic removal. The polyps arose from the hypopharynx in 3 patients and upper esophagus in 1. Three patients had complete resolution of their symptoms and remained disease free. One patient had recurrence of the polyp 2 years later and is currently being observed. CONCLUSION: Fibrovascular polyps are rare tumors of the hypopharynx/esophagus that present in older adults. Although benign, they can cause life-threatening airway compromise that may necessitate tracheotomy. We present 4 cases of fibrovascular polyps and discuss our evolving surgical management, including endoscopic removal.

Generalized eruptive keratoacanthomas of Grzybowski.

BACKGROUND: The initial patient with generalized eruptive keratoacanthomas was described by Grzybowski in 1950. The condition presents as hundreds to thousands of small (1-5 mm), flesh-colored to erythematous papules. OBJECTIVE: To review the clinical characteristics and treatments of generalized eruptive keratoacanthomas of Grzybowski. MATERIALS AND METHODS: We retrospectively reviewed the medical literature using PubMed, searching the terms "eruptive," "generalized," "Grzybowski," "keratoacanthoma," and "multiple." Patient reports and previous reviews of the subject were critically assessed and the salient features presented to the best of our knowledge. RESULTS: Generalized eruptive keratoacanthomas of Grzybowski typically present in the fifth to seventh decades of life and display an equal preponderance for both men and women. The keratoacanthomas are pruritic and predominately appear in sun-exposed regions, such as the face (in which they often demonstrate the sign of Zorro) and the upper trunk; the tumors also show a predilection for intertriginous areas. Topical, intralesional, and systemic treatment modalities have been used; oral retinoids are considered the preferred method of treatment. CONCLUSION: Generalized eruptive keratoacanthomas of Grzybowski is a rare form of keratoacanthoma and is considered a serious condition because the eruptions are diffuse, persistent, and recurrent. Constant pruritus, ectropion, visceral neoplasms, and unsatisfactory response to treatment are ominous prognostic factors. Successful management of this condition has been observed in patients treated with systemic retinoids, such as acitretin.

Nocardia yamanashiensis in an immunocompromised patient presenting as an indurated nodule on the dorsal hand.

BACKGROUND: Nocardia are ubiquitous, aerobic, gram-positive actinomycetes. Nocardiosis typically occurs in immunocompromised patients, although immunocompetent individuals can also be affected. PURPOSE: The purpose of this case study is to review the clinical characteristics and treatments of a unique form of cutaneous nocardiosis. MATERIALS AND METHODS: We retrospectively reviewed the medical literature using PubMed, searching the terms cutaneous, host, immunocompromised, Nocardia, primary, yamanashiensis. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. RESULTS: Cutaneous nocardiosis typically presents as pustular nodules and the lesions may progress to become abscesses, cellulitis, granulomas or keloid-like tumors. N. brasiliensis is the predominant species involved in primary cutaneous nocardiosis; other common Nocardia species involved in human disease are N. farcinica, N. abscessus, N. cyriacigeorgica, and N. nova. Only two individuals (including the patient presented here) with primary cutaneous infection by N. yamanashiensis have been described in the literature; a third clinical isolate was recovered from a lung biopsy. CONCLUSION: Nocardia yamanashiensis is a rare clinical form of primary cutaneous nocardiosis. 16S ribosomal gene sequencing, as well as Gram stain and modified Fite acid-fast stain, play a vital role in identifying this clinical variant.