Fully Implanted Brain-Computer Interface in a Locked-In Patient with ALS.

Options for people with severe paralysis who have lost the ability to communicate orally are limited. We describe a method for communication in a patient with late-stage amyotrophic lateral sclerosis (ALS), involving a fully implanted brain-computer interface that consists of subdural electrodes placed over the motor cortex and a transmitter placed subcutaneously in the left side of the thorax. By attempting to move the hand on the side opposite the implanted electrodes, the patient accurately and independently controlled a computer typing program 28 weeks after electrode placement, at the equivalent of two letters per minute. The brain-computer interface offered autonomous communication that supplemented and at times supplanted the patient's eye-tracking device. (Funded by the Government of the Netherlands and the European Union; ClinicalTrials.gov number, NCT02224469 .).

A retrospective review of swallow dysfunction in patients with severe traumatic brain injury.

In the acute-care setting, it is difficult for clinicians to determine which patients with severe traumatic brain injury will have long-term oropharyngeal dysphagia (>6 weeks) and which patients will begin oral nutrition quickly. Patients frequently remain in the acute-care setting while physicians determine whether to place a percutaneous endoscopic gastrostomy (PEG) tube. To improve the acute-care clinician's ability to predict long-term oropharyngeal dysphagia and subsequent need for PEG tube placement in patients with severe traumatic brain injury [Glascow Coma Scale (GCS) ≤ 8), a novel prediction model was created utilizing clinical information and acute-care swallowing evaluation findings. Five years of retrospective data were obtained from trauma patients at a Level 1 trauma hospital. Of the 375 patients who survived their hospitalization with a GCS ≤ 8, a total of 269 patients received Ranchos Los Amigos (RLA) scores. Of those patients who were scored for RLA, 219 patients underwent swallowing evaluation. Ninety-six of the 219 patients were discharged from the hospital with a feeding tube, and 123 patients were discharged without one. Logistic regression models examined the association between clinical and patient characteristics and whether a patient with severe traumatic brain injury exhibited long-term oropharyngeal dysphagia. Multivariable logistic regression analysis revealed that increased age, low RLA score, tracheostomy tube placement, and aphonia observed on the initial swallowing evaluation significantly increased the odds of being discharged from the acute-care hospital with a feeding tube. The resultant model could be used clinically to guide decision making and to counsel patients and families.

Management of aphonic patients following total laryngectomy and trachea esophageal puncture.

BACKGROUND: Trachea esophageal puncture (TEP) is performed following total laryngectomy to allow speech and communication. The most common reason for long-term speech failure in this population is hypertonicity of the constrictor muscle. OBJECTIVES: To present our experience with the treatment of aphonic patients after total laryngectomy and TEP and suggest a protocol for treatment. METHODS: Of 50 patients who underwent total laryngectomy and TEP, 6 suffered from aphonia after surgery. All patients underwent radiotherapy with or without chemotherapy. Delay in speech continued for more than 6 months after surgery. The patients received percutaneous lidocaine injection to the neopharynx in different locations around the stoma in order to map the hypertonic segments in the neopharynx. RESULTS: Lidocaine injection immediately enabled free speech in five patients. One patient (patient 6) suffered from aphonia and from severe dysphagia and required a feeding tube. This patient succeeded to pronounce abbreviations after lidocaine injection. Another (patient 4) gained permanent ability to speak following a single lidocaine injection; this patient was not injected with botolinium toxin (BTX). For the other five, lidocaine had a transient effect on speech. These patients received BTX percutaneous injections. After BTX injections four regained free speech within 14 days. The fifth patient (patient 6) gained a conversational voice and his swallowing improved only after additional intensive speech therapy. CONCLUSIONS: Percutaneous lidocaine and BTX injections represent first-line treatment in this population, with good success and minimal complications.

Ewing's sarcoma of the larynx. Effective treatment with organ preservation.

Extraskeletal Ewing's sarcoma arising in the head and neck region is an extremely rare malignant neoplasm. We describe the unusual case of a tumor originating in the larynx of a 68-year-old male with hoarseness and occasional aphonia. We report successful treatment with sequential chemo- and radiotherapy. Complete regression and larynx preservation with voice function recovery was achieved. To our knowledge, this is the first report of this type of tumor in the larynx with cartilage invasion that documents the effectiveness of radiotherapy as an alternative to surgical management. At present, after 30 months of follow-up, the patient is free of tumor relapse and in very good condition.

Aphonia Following Bilateral Cervical Plexus Blocks for an Awake Hemithyroidectomy: A Case Report.

A 35-year-old female patient was scheduled for a left hemithyroidectomy. We performed bilateral cervical plexus blocks with ultrasound guidance for an awake thyroidectomy. Soon after the blocks, she developed a weak voice, which gradually progressed to complete aphonia. Due to her apprehension, general anesthesia was administered. After an uneventful surgery, the patient spontaneously regained her normal voice in the postoperative period. The case report describes a previously unreported complication of aphonia presumably due to bilateral recurrent laryngeal nerve blocks, which might have occurred from the infiltrated local anesthetic extravasating to the deeper planes through the cervical fascia.

Giant pseudoaneurysm of right subclavian artery presenting with severe respiratory distress.

Subclavian artery pseudoaneurysms are rare; and cases involving subclavian artery pseudoaneurysms presenting with compression on trachea leading to breathing difficulty are exceptional. We present a case report of huge subclavian artery pseudoaneurysm presenting with hoarseness of voice, respiratory distress, and aphonia. Patient was operated through median sternotomy, with the incision extended over the swelling. Aortic looping was done before opening the aneurysm. Rent in the right subclavian artery was identified and repaired. Because of the absence of a distal pulsation beyond the repaired site, we placed a reversed saphenous vein graft from the ascending aorta (end-to-side) to the right subclavian artery distal to repaired site (end-to-side). Good pulsation was achieved in the right brachial, right radial, and right ulnar arteries. Patient was extubated after 6 hours in stable condition.

Cytoskeleton of cells in vocal fold macula flava unphonated for a long period.

Cells in the maculae flavae (MFe) are inferred to be involved in the metabolism of extracellular matrices of the human vocal fold mucosa. The latest research has supported the hypothesis that the tension caused by phonation (vocal fold vibration) regulates the behavior of these cells in the MFe of the human vocal fold. Tensile and compressive strains have direct effects on cell morphology and structure including changes in cytoskeletal structure and organization. Cytoskeletons are one of the structures which play a role as mechanoreceptors for the cells. The microstructure of the intermediate filaments and the expression of their proteins were investigated regarding the cells in the MFe of the human vocal fold unphonated over a decade. The adult vocal fold mucosa of a 64-year-old male with cerebral hemorrhage unphonated for 11 years was investigated by immunohistochemistry and electron microscopy. The intermediate filaments in the cytoplasm of the cells had become fewer in number. And the expression of their characteristic proteins (vimentin, desmin, GFAP) was also reduced. The results of this study are consistent with the hypothesis that mechanotransduction caused by vocal fold vibration could possibly be a factor in regulating the function and fate of the cells in the MFe.

Rehabilitation Therapy for Vocal Fold Paralysis Caused by Lung Cancer: A Case Report.

OBJECTIVE: Unilateral vocal fold paralysis (UVFP) can be caused by iatrogenic injury or tumor-induced damage to the recurrent laryngeal nerve. Studies of comprehensive rehabilitation therapies for patients suffering from severe UVFP are limited. The purpose of this case report is to describe an improvement in complete aphonia after comprehensive rehabilitation therapies in a patient with severe UVFP due to a lung tumor. METHODS: An 81-year-old woman with a history of bronchial adenoma had complete aphonia due to compression of the left recurrent laryngeal nerve by the tumor. Dynamic fibrolaryngoscope revealed paralysis of the left vocal fold. The patient was treated with interferential current therapy, vocal training, and kinesiology taping. Indicators of voice recovery were scored according to the grade, roughness, breathiness, asthenia, strain scale, and the voice handicap index. RESULTS: After 10 days of comprehensive rehabilitation treatment, the patient recovered from complete aphonia to normal communication. The hoarseness and breathiness of patient were significantly improved. In addition, the grade, roughness, breathiness, asthenia, strain, and the voice handicap index scores changed from severe to mild or absent. CONCLUSION: This case provided a novel comprehensive treatment for a patient with UVFP, which was safe, cost-effective, and easy to implement in clinic.

Laryngeal Cryptococcosis: An Evolving Rare Clinical Entity.

OBJECTIVES: Describe the demographics and clinical manifestations of laryngeal cryptococcosis. Develop a simple approach to the diagnostic workup and treatment of localized laryngeal cryptococcal infection. METHODS: A new case of laryngeal cryptococcosis encountered at our institution is presented and placed in context of the literature surrounding prior reported cases. PubMed, Google Scholar, SCOPUS, and Web of Science were queried from inception to August 2018 with the terms Larynx or Laryngeal and Cryptococcosis or Cryptococcus by two independent reviewers for English-language cases of cryptococcal infection of the larynx. RESULTS: Twenty-nine unique cases of laryngeal cryptococcosis were identified. Median age at presentation was 65 years old. All patients presented with persistent or progressive hoarseness. Lesions were predominantly on the true vocal cords (79%), 38% associated with an adherent white exudate or leukoplakia. A minority (28%) was immunocompromised, and of the remaining immunocompetent hosts, 67% were found to be using nebulized or inhaled corticosteroids (ICS) prior to infection. Diagnosis should be suspected in patients with chronic laryngitis or mass lesions with the aforementioned risk factors. Diagnosis was made by histopathology with cryptococcal yeasts identified on methenamine silver (55%) and/or mucicarmine stains (48%). Serum cryptococcal antigen testing was unreliable (sensitivity = 39%). The mainstay of effective treatment was prolonged oral Fluconazole therapy, with two cases of laser therapy ablation of residual lesions. Improvement in voice and vocal lesions varied from weeks to months. CONCLUSIONS: Laryngeal cryptococcosis is a rare cause of persistent hoarseness, which appears to be clinically evolving and more frequently affecting immunocompetent hosts chronically using nebulized or inhaled corticosteroids. Laryngeal cryptococcal infection is readily treatable with prolonged oral antifungals once biopsy and histopathological stains confirm the diagnosis.

Aphonogelia After Recovery From Severe Traumatic Brain Injury: A Case Report.

During rehabilitation from a severe traumatic brain injury, a 16-year-old girl became aware that she had lost the ability to laugh out loud. This rare phenomenon previously has been described as "aphonogelia." A discussion of therapeutic avenues that were explored with this patient is presented in the first case, to our knowledge, of aphonogelia after a traumatic brain injury. LEVEL OF EVIDENCE: V.

Vocal cord immobility as a cause of aphonia in a child with 3p13p12 deletion syndrome encompassing FOXP1 gene.

Congenital bilateral laryngeal paralysis/immobilization is an uncommon condition and has been described as isolated or accompanying to some recognizable syndromes. Heterozygous mutations in the FOXP1 gene (605515) are related with intellectual disability and, language impairment with or without autistic features. Expressive language is more affected than receptive language and more than half of the patients experience oromotor dysfunction and/or feeding difficulties. Here we report a child with severe developmental, speech delay and aphonia which was considered due to bilaterally abductor vocal cord immobility. Interstitial 8700 kbp deletion encompassing FOXP1 gene was detected on 3p13p12 chromosomal region. Although it is known that FOXP1 defects are related to abnormalities in vocal communication, FOXP1-associated laryngomalacia or vocal cord paralysis/immobilization cases have not been reported yet. The FOXP1 defects are considered to be a cause of delay in speech, and it is suggested that vocal cord evaluation should be conducted in suspicious cases.

Vocal fold paralysis in infants with tracheoesophageal fistula.

OBJECTIVES: We describe the clinical characteristics and management of vocal fold paralysis in infants who were born with a tracheoesophageal fistula (TEF). METHODS: This retrospective case series included all infants born with TEFs who presented to our pediatric otolaryngology unit and intensive care unit because of dyspnea or aphonia in the years 2005 and 2006, and who were found to have vocal fold paralysis. RESULTS: Five boys and 1 girl were studied. One infant had stridor before TEF repair, and 5 after it. All children underwent flexible laryngotracheobronchoscopy and were treated in the pediatric intensive care unit before diagnosis of the vocal fold paralysis (5 bilaterally and 1 unilaterally) was made. The ages at diagnosis of paralysis ranged between 14 days and 14 months. Five infants required tracheostomy. CONCLUSIONS: Vocal fold paresis in infants is difficult to diagnose. The risk for recurrent laryngeal nerve injury associated with TEF and TEF repair should be emphasized in these children. We recommend that all newborns with TEF should be examined by an otolaryngologist before operation to confirm the mobility of the vocal folds and to rule out other associated airway malformations, and examined after operation if respiratory difficulties develop.

[Foreign accent syndrome in a case of dissociative (conversion) disorder].

Psychiatric symptoms are often manifested in verbal expression. Generally, the contents of such expression are pathological. The formal abnormalities of speech are also observed in various mental disorders, as far as quantitative abnormalities are concerned. However, disturbance of intonation, namely disprosody, is more commonly observed in organic disorders of the brain. When the accent of words and the intonation of sentences changes from that of a native speaker, the speech sounds like the broken language of untrained foreigners. Such foreign accent syndrome is usually an issue of neuropsychology. In this paper, the authors report a case of foreign accent syndrome without organic brain syndrome. The patient was a 44-year-old woman, who developed panic disorder about year after her father's death. Then she developed aphonia. After aphonia was resolved, she began to speak haltingly as if a Chinese woman was trying to speak Japanese. Organic brain diseases were subsequently excluded. She had complicated familial conflicts, including a divorce from a violent and faithless husband, interpersonal difficulties with her husband's parents, and her pubertal daughter. We diagnosed her with dissociative (conversion) disorder of ICD-10. Our patient is clinically interesting, because case reports of dysprosody are unusual and often involve organic brain diseases. We suppose that foreign accent syndrome in our patient is a variant of aphonia, and the patient unconsciously assigned the symptom two ambivalent rolls: to snow that she cannot speak well, and to express her meaning. In addition, she had a Chinese-speaking aunt-in-law who was her ideal role model. We surmise that her symptom signifies identification with her aunt-in-law.

Glottic tuberculosis masquerading as early multifocal carcinoma.

The incidence of tuberculosis has progressively decreased in developed countries after the advent of antituberculous chemotherapy, but has recently been increasing again mainly due to migratory flows. Although common in the past and considered to be a prelethal event, laryngeal involvement has become a rarity. We report the case of a 52-year-old female smoker who complained of persistent aphonia. Videostroboscopy showed bilateral vocal fold erythroleukoplakias. A chest X-ray and CT scan aroused the suspicion of lung tuberculosis, which was confirmed by Ziehl-Neelsen staining for acid-alcohol-fast bacilli on bronchoalveolar lavage. Antituberculous chemotherapy rapidly improved the quality of her voice and led to complete disappearance of the vocal fold lesions. In conclusion, laryngeal tuberculosis may mimic an early glottic multifocal carcinoma and should therefore, despite its rarity, always be considered in the differential diagnosis of erythroleukoplakia-like lesions in order to avoid improper surgical treatment and voice damage.

Arytenoid adduction to treat impaired adduction of the vocal fold due to rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic inflammatory disease affecting the synovial membrane and causing joint damage and bone destruction. The symptoms of cricoarytenoid joint (CJ) arthritis often include hoarseness, and a sense of pharyngeal fullness in the throat. Sometimes, in cases with bilateral CJ involvement, an urgent tracheostomy might be required for acute airway obstruction. In this report, we describe a woman suffering from aphonia due to hampered adduction of the vocal fold which was caused by RA with unilateral CJ involvement. Arytenoid adduction surgery on the affected side was performed. She retrieved a normal voice immediately after the surgery.

Transient Aphonia After Mediastinoscopy.

The most common adverse event after cervical mediastinoscopy is recurrent laryngeal nerve (RLN) injury, which has an incidence of 0.6% [1]. We report the case of a 68-year-old man with non-small cell lung cancer (NSCLC) who experienced transient bilateral vocal cord paralysis after mediastinoscopy, which manifested in complete aphonia. This patient's ability to maintain his airway was carefully followed up, but neither endotracheal intubation nor tracheostomy was required. The vocal cord paralysis resolved without intervention after 5 hours. To our knowledge, this is the first reported case in which bupivicaine used at the end of a cervical mediastinoscopy diffused through the freshly dissected planes to paralyze both RLNs along the tracheoesophageal grooves.

Vocal fold polyp in a professional brass/wind instrumentalist and singer.

Wind instrumentalists, especially brass players, and singers share common factors, including vocal tract shape, function and pressure, vocal fold opening and closure, breath vector of force and air flow rates. To understand the mechanism and function of the vocal folds with a pathological lesion, it is necessary to visualize the differing interactions of the vocal tract during wind and brass instrument playing and in singing. A school band director, singer, wind and brass instrumentalist, was referred by musician colleagues with intermittent dysphonia, aphonia, and inability to sing high notes. Simultaneous videolaryngoscopy, with and without stroboscopy, and external video examination were documented. An hourglass glottis with a sessile, cystic polyp of the left vocal fold were recorded and studied during phonation and the playing of 3 instruments. The techniques of glottic opening, closure, configuration and function varied with the type of instrument and phonatory function. Singing was adversely affected by the vocal fold polyp but no harmful interaction occurred during wind/brass instrument playing. Down-stream loading in singers is at the laryngeal level and in wind/brass instrumentalists is at the embouchure. Preoperative voice therapy, phonomicrosurgery, and postoperative voice rest followed by voice therapy, succeeded in restoring her combined wind/brass instrumental and singing career.

[An epilepsy case presenting temporal aphonia and prosodic deficit with left hemiparesis].

A 53-year-old right-handed woman was admitted to a hospital with a seizure, exhibiting loss of consciousness and paroxysmal myoclonic movement. MRI showed no abnormalities of her brain. Electroencephalography revealed sporadic sharp and slow wave complexes, starting from the right central region. Cerebral perfusion SPECT revealed increased blood supply in the right hemisphere. She received diagnosis of epilepsy and was treated with phenytoin. After she regained her consciousness, she had no voice and presented with left hemiparesis which was interpreted to be Todd's palsy. She gradually recovered in using her voice, to full normalization of affective prosody in 11 days. The left hemiparesis recovered first in the upper and later in the lower extremities. Her symptoms could be interpreted to be vocal and prosodic disturbance, but not aphasia, because she could understand speach and communicate with writing. The lesions responsible for prosodic deficit are still controversial. The symptoms observed and the findings obtained may indicate that the deficiency of the affective prosody may be caused by the dysfunction of the medial surface of the non-dominant frontal lobe in this case.