Comparative Treatment Outcomes for Idiopathic Subglottic Stenosis: 5-Year Update.

The North American Airway Collaborative (NoAAC) previously published a 3-year multi-institutional prospective cohort study showing variation in treatment effectiveness between 3 primary surgical techniques for idiopathic subglottic stenosis (iSGS). In this report, we update these findings to include 5 years of data evaluating treatment effectiveness. Patients in the NoAAC cohort were re-enrolled for 2 additional years and followed using the prespecified published protocol. Consistent with prior data, prospective observation of 487 iSGS patients for 5 years showed treatment effectiveness differed by modality. Cricotracheal resection maintained the lowest rate of recurrent operation (5%), followed by endoscopic resection with adjuvant medical therapy (30%) and endoscopic dilation (50%). These data support the initial observations and continue to provide value to providers and patients navigating longitudinal decision-making. Level of evidence: 2-prospective cohort study.

Nontuberculous Mycobacterial Infection of Larynx and Cervical Trachea.

OBJECTIVES: To present a patient with the first case of NTM (nontuberculous mycobacteria) infection of the larynx extending to cervical trachea, and the first case of subglottic stenosis associated with an NTM infection. METHODS: Case report and review of the literature. RESULTS: A 68-year-old female with history of prior smoking, gastroesophageal reflux disease, asthma, bronchiectasis, and tracheobronchomalacia presented with a 3-month history of shortness of breath, exertional inspiratory stridor, and hoarseness. Flexible laryngoscopy demonstrated ulceration of medial aspect of right vocal fold and subglottic tissue abnormality with crusting and ulceration extending through the upper trachea. Microdirect laryngoscopy with tissue biopsies and carbon dioxide (CO2) laser ablation of disease completed, and intraoperative culture revealed positive Aspergillus and acid-fast bacilli with Mycobacterium abscessus (type of NTM). Patient began antimicrobial treatment of cefoxitin, imipenem, amikacin, azithromycin, clofazimine, and itraconazole. Fourteen months after initial presentation, patient developed subglottic stenosis with limited extension into the proximal trachea prompting CO2 laser incision, balloon dilation, and steroid injection of the subglottic stenosis. Patient remains disease free without further subglottic stenosis. CONCLUSION: Laryngeal NTM infections are exceedingly rare. Failure to consider NTM infection in the differential diagnosis when presented with an ulcerative, exophytic mass in patients with increased risk factors (structural lung disease, Pseudomonas colonization, chronic steroid use, prior NTM positivity) may result in insufficient tissue evaluation, delayed diagnosis, and disease progression.

Jet flow rate and needle position govern distal airway pressures during low-frequency jet ventilation.

OBJECTIVES: Although jet ventilation is frequently used during surgery for airway stenosis, little is known about distal airway pressures during jet ventilation. The objective of the study is to determine how jet pressure, flow rate, and position of the ventilation needle relate to distal airway pressure magnitude and homogeneity. METHODS: Two 3D models of the first five generations of the human airway tree were created. One is a duplicate of a human airway from a 15-year-old healthy male's computed tomography scan, and the other is an idealized symmetric model of human lung morphometry. Pressure transducers measured fifth-generation distal airway pressures in both models. A computer-controlled jet needle positioning system was used to ventilate the lung casts. The effects of jet needle position, jet pressure, and jet flow rate on distal airway pressure and homogeneity were measured. RESULTS: Total entrained jet flow rate was the most reliable predictor of distal airway pressure. Pressure supplied to the jet ventilation needle had a positive linear relationship with distal airway pressure; however, this relationship was dependent on the jet needle flow resistance. As the ventilation needle moved closer to the tracheal wall, ventilation homogeneity decreased. Depth into the trachea was positively correlated with sensitivity of the needle to the tracheal wall. CONCLUSION: In this model, total entrained jet flow rate is a more robust predictor of distal airway pressure than jet inlet pressure. More homogeneous ventilation was observed in our model with the ventilation needle centered in the proximal region of the trachea.

Superior Laryngeal Nerve Block for Treatment of Neurogenic Cough.

OBJECTIVES: This study aimed to add to the body of evidence for efficacy of Superior Laryngeal Nerve (SLN) blocks for treatment of neurogenic cough. Efficacy at short- and long-term intervals are presented as well as relationships with laryngoscopic findings. METHODS: A retrospective chart review of patients treated with SLN block between 2018 and 2020 was conducted. Patient demographics, videostroboscopic findings, and patient-subjective perception of outcomes were recorded and analyzed. Cough Severity Index (CSI) scores from pre-injection, short-term follow-up, and long-term follow-up were compared. RESULTS: Twenty patients underwent SLN block in the clinic setting. Four patients were excluded for incomplete records. The indication was neurogenic cough refractory to medical management and/or cough suppression therapy. Patients with short-term follow-up (n = 13) had statistically significant decrease in CSI scores, with a mean baseline CSI of 24.3 decreasing to 16.15 (P = .006). Patients with evidence of Vocal Fold Motion/Vibratory Abnormalities (VFA) (n = 8) showed improvement in short-term CSI scores, with a mean baseline CSI of 24.13 decreasing to 14.5 (P = .004). Those without evidence of VFA did not have statistically significant improvement in short-term CSI scores. At long-term follow-up, patients with VFA had improvements that approached statistical significance with a mean baseline CSI of 22.56 decreasing to 14.56 (P = .057), while patients without VFA showed no improvement. CONCLUSIONS: Our results are consistent with previous literature indicating efficacy of SLN block. The presence of VFA may be an indicator of patients who experience increased therapeutic effect. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2676-E2680, 2021.

Association of Social Determinants of Health with Time to Diagnosis and Treatment Outcomes in Idiopathic Subglottic Stenosis.

OBJECTIVES: To examine whether social determinants of health (SDH) factors are associated with time to diagnosis, treatment selection, and time to recurrent surgical intervention in idiopathic subglottic stenosis (iSGS) patients. METHODS: Adult patients with diagnosed iSGS were recruited prospectively (2015-2017) via clinical providers as part of the North American Airway Collaborative (NoAAC) and via an online iSGS support community on Facebook. Patient-specific SDH factors included highest educational attainment (self-reported), median household income (matched from home zip code via U.S. Census data), and number of close friends (self-reported) as a measure of social support. Main outcomes of interest were time to disease diagnosis (years from symptom onset), treatment selection (endoscopic dilation [ED] vs cricotracheal resection [CTR] vs endoscopic resection with adjuvant medical therapy [ERMT]), and time to recurrent surgical intervention (number of days from initial surgical procedure) as a surrogate for disease recurrence. RESULTS: The total 810 participants were 98.5% female, 97.2% Caucasian, and had a median age of 50 years (IQR, 43-58). The cohort had a median household income of $62 307 (IQR, $50 345-$79 773), a median of 7 close friends (IQR, 4-10), and 64.7% of patients completed college or graduate school. Education, income, and number of friends were not associated with time to diagnosis via multivariable linear regression modeling. Univariable multinominal logistic regression demonstrated an association between education and income for selecting ED versus ERMT, but no associations were noted for CTR. No associations were noted for time to recurrent surgical procedure via Kaplan Meier modeling and Cox proportional hazards regression. CONCLUSIONS: Patient education, income, and social support were not associated with time to diagnosis or time to disease recurrence. This suggests additional patient, procedure, or disease-specific factors contribute to the observed variations in iSGS surgical outcomes.

Comparative Treatment Outcomes for Patients With Idiopathic Subglottic Stenosis.

Importance: Surgical treatment comparisons in rare diseases are difficult secondary to the geographic distribution of patients. Fortunately, emerging technologies offer promise to reduce these barriers for research. Objective: To prospectively compare the outcomes of the 3 most common surgical approaches for idiopathic subglottic stenosis (iSGS), a rare airway disease. Design, Setting, and Participants: In this international, prospective, 3-year multicenter cohort study, 810 patients with untreated, newly diagnosed, or previously treated iSGS were enrolled after undergoing a surgical procedure (endoscopic dilation [ED], endoscopic resection with adjuvant medical therapy [ERMT], or cricotracheal resection [CTR]). Patients were recruited from clinician practices in the North American Airway Collaborative and an online iSGS community on Facebook. Main Outcomes and Measures: The primary end point was days from initial surgical procedure to recurrent surgical procedure. Secondary end points included quality of life using the Clinical COPD (chronic obstructive pulmonary disease) Questionnaire (CCQ), Voice Handicap Index-10 (VHI-10), Eating Assessment Test-10 (EAT-10), the 12-Item Short-Form Version 2 (SF-12v2), and postoperative complications. Results: Of 810 patients in this cohort, 798 (98.5%) were female and 787 (97.2%) were white, with a median age of 50 years (interquartile range, 43-58 years). Index surgical procedures were ED (n = 603; 74.4%), ERMT (n = 121; 14.9%), and CTR (n = 86; 10.6%). Overall, 185 patients (22.8%) had a recurrent surgical procedure during the 3-year study, but recurrence differed by modality (CTR, 1 patient [1.2%]; ERMT, 15 [12.4%]; and ED, 169 [28.0%]). Weighted, propensity score-matched, Cox proportional hazards regression models showed ED was inferior to ERMT (hazard ratio [HR], 3.16; 95% CI, 1.8-5.5). Among successfully treated patients without recurrence, those treated with CTR had the best CCQ (0.75 points) and SF-12v2 (54 points) scores and worst VHI-10 score (13 points) 360 days after enrollment as well as the greatest perioperative risk. Conclusions and Relevance: In this cohort study of 810 patients with iSGS, endoscopic dilation, the most popular surgical approach for iSGS, was associated with a higher recurrence rate compared with other procedures. Cricotracheal resection offered the most durable results but showed the greatest perioperative risk and the worst long-term voice outcomes. Endoscopic resection with medical therapy was associated with better disease control compared with ED and had minimal association with vocal function. These results may be used to inform individual patient treatment decision-making.

Voice outcomes after endoscopic sinus surgery in patients with chronic rhinosinusitis with nasal polyposis.

OBJECTIVES/HYPOTHESIS: The purpose of this study was to assess voice outcomes after endoscopic sinus surgery (ESS) in patients with chronic rhinosinusitis with nasal polyposis (CRSwNP). STUDY DESIGN: Individual cohort study. METHODS: A study was performed of patients with CRSwNP who underwent ESS from July 2015 to May 2016 at a tertiary referral medical center. The main outcome measures were subjective improvement in patient-reported outcomes, using the 22-item Sino-Nasal Outcome Test (SNOT-22) and 10-item Voice Handicap Index (VHI-10). RESULTS: A total of 50 patients (age 47.9 years; 48% female), 66% revision and 34% primary, were available at a follow-up of 3 months. At the 3-month follow-up, both SNOT-22 and VHI-10 scores were improved from preoperative values (-41.8 and -14.7, respectively). CONCLUSIONS: Successful treatment of sinonasal disease can help to improve voice outcomes in patients with CRSwNP. There are inherent differences in the long-term outcomes of the results beyond 3 months based on practice patterns of ongoing medical management, but our results are interesting in that they show early improvement in voice outcomes. LEVEL OF EVIDENCE: 2b Laryngoscope, 129:299-302, 2019.

Effect of Increased Body Mass Index on Complication Rates during Laryngotracheal Surgery Utilizing Jet Ventilation.

Objective Jet ventilation has been used for >30 years as an anesthetic modality for laryngotracheal surgery. Concerns exist over increased risk with elevated body mass index (BMI). We reviewed our experience using jet ventilation for laryngotracheal stenosis to assess for complication rates with substratification by BMI. Study Design Case series with chart review. Setting Tertiary care center. Subjects and Methods A total of 126 procedures with jet ventilation were identified from October 2006 to December 2014. Complications were recorded, including intubation, unplanned admission, readmission, dysphonia, oral trauma, pneumothorax, pneumomediastinum, and tracheostomy. Lowest intraoperative oxygen saturation and maximum end-tidal CO2 (ETCO2) levels were recorded. Results Among 126 patients, 43, 77, and 6 had BMIs of <25, 25-35, and 36-45, respectively. In the BMI <25 group, there was 1 unplanned intubation. Mean maximum ETCO2 was 36.51 with no hypoxemia observed. In the BMI 25-35 group, 2 patients required intubation, and 1 sustained minor oral trauma. The mean maximum ETCO2 was 38.85, with 4 patients having oxygen saturation <90%. In the BMI 36-45 group, 2 patients required intubation. The mean maximum ETCO2 was 41 with no hypoxemia observed. BMI and length of stenosis were statistically significant variables associated with incidence of intraoperative intubation. Conclusion Increased BMI was associated with an increase in highest ETCO2 intraoperatively. However, this was not associated with an increase in major complications. Jet ventilation was performed without significant adverse events in this sample, and it is a viable option if used with an experienced team in the management of laryngotracheal stenosis.

Safety and efficacy of laryngeal mask airway ventilation in obese patients with airway stenosis.

OBJECTIVE: To assess the efficacy of laryngeal mask airway (LMA) ventilation in obese patients with airway stenosis. STUDY DESIGN: A retrospective chart review was conducted in an academic practice in a tertiary care center. METHODS: We retrospectively reviewed our experience using LMA ventilation in obese patients with airway stenosis. Lowest intraoperative O2 saturation and maximum-end tidal carbon dioxide (CO2 ) levels were recorded. Complications including intubation, unplanned admission, re-admission, postoperative pain, dysphonia, oral trauma, pneumothorax, pneumomediastinum, and tracheostomy were recorded. RESULTS: Fourteen bronchoscopies with laser incisions and dilation were performed in patients with airway stenosis exclusively using LMA ventilation. Thirteen of 14 procedures were performed on patients who had body mass index (BMI) > 30 kg/m2 . Mean BMI was noted to be 38 kg/m2 (range 25-54). All patients underwent successful laser incisions and dilation via LMA anesthesia without major or minor adverse events. The mean lowest O2 saturation level was noted to be 92%; the mean highest CO2 level was noted to be 56 mm Hg; and no patients required endotracheal intubation. CONCLUSION: In this small series of obese patients with airway stenosis, LMA anesthesia was effectively used without major or minor complications. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2582-2584, 2017.

Functional Outcomes after Salvage Transoral Laser Microsurgery for Laryngeal Squamous Cell Carcinoma.

OBJECTIVES: Transoral laser microsurgery (TLM) has been increasingly used in lieu of total laryngectomy to treat malignancy after definitive radiation. There are few data in the literature regarding functional outcomes. We retrospectively reviewed voice and swallowing outcomes in patients who underwent TLM for recurrent laryngeal carcinoma. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: Forty-two patients were identified with recurrent squamous cell carcinoma of the larynx after definitive radiation therapy from 2001 to 2013: 28 patients with glottic recurrence and 14 with supraglottic recurrence. Swallowing outcomes were evaluated by gastrostomy tube dependence, the MD Anderson Dysphagia Inventory, and the Functional Oral Intake Scale. Voice outcomes were evaluated by the Voice Handicap Index and observer-rated perceptual analysis. RESULTS: No significant difference was noted between mean pre- and postoperative MD Anderson Dysphagia Inventory scores: 78.25 and 74.9, respectively (P = .118, t = 1.6955). Mean Functional Oral Intake Scale scores after TLM for supraglottic and glottic recurrences were 6.4 and 6.6, respectively. Of 42 patients, 17 (40.5%) required a gastrostomy tube either during radiation or in conjunction with the salvage procedure. Of 17 patients, 15 resumed sufficient oral diet for tube removal. Patients' mean Voice Handicap Index score did increase from 34.3 to 51.5 (P = .047), and their mean perceptual score did decrease from 60.0 to 45.3 (P = .005). However, at 1-year follow-up, there was no significant difference in perceptual score: 61.1 to 57.1 (P = .722). CONCLUSIONS: TLM is a successful surgical option for recurrent laryngeal cancer with acceptable functional outcomes.

Injection Laryngoplasty Using Micronized Acellular Dermis for Vocal Fold Paralysis: Long-term Voice Outcomes.

OBJECTIVES: Micronized acellular dermis has been used for nearly 15 years to correct glottic insufficiency. With previous demonstration of safety and efficacy, this study aims to evaluate intermediate and long-term voice outcomes in those who underwent injection laryngoplasty for unilateral vocal fold paralysis. Technique and timing of injection were also reviewed to assess their impact on outcomes. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: Patients undergoing injection laryngoplasty from May 2007 to September 2012 were reviewed for possible inclusion. Pre- and postoperative Voice Handicap Index (VHI) scores, as well as senior speech-language pathologists' blinded assessment of voice, were collected for analysis. The final sample included patients who underwent injection laryngoplasty for unilateral vocal fold paralysis, 33 of whom had VHI results and 37 of whom had voice recordings. Additional data were obtained, including technique and timing of injection. RESULTS: Analysis was performed on those patients above with VHI and perceptual voice grades before and at least 6 months following injection. Mean VHI improved by 28.7 points at 6 to 12 months and 22.8 points at >12 months (P = .001). Mean perceptual voice grades improved by 17.6 points at 6 to 12 months and 16.3 points at >12 months (P < .001). No statistically significant difference was found with technique or time to injection. CONCLUSION: Micronized acellular dermis is a safe injectable that improved both patient-completed voice ratings and blinded reviewer voice gradings at intermediate and long-term follow-up. Further investigation may be warranted regarding technique and timing of injection.

Subjective and objective voice outcomes after transoral laser microsurgery for early glottic cancer.

OBJECTIVES/HYPOTHESIS: Transoral laser microsurgery (TLM) continues to gain popularity as a treatment modality for early glottic cancer. Oncologic outcomes have been well-defined, but there are little data to date describing functional outcomes accounting for stage of resection. STUDY DESIGN: Retrospective review. METHODS: We retrospectively reviewed patient-rated voice handicap and observer-rated vocal quality of patients who underwent TLM for early glottic carcinoma. Patients were grouped by European Laryngological Society (ELS) resection type, and the data were combined for ELS type I-III and compared with advanced resections (ELS IV-VI). The Voice Handicap Index (VHI) was used for patient-rated voice outcomes, and voice recordings were graded by two senior speech-language pathologists. Voice recordings and VHI scores were taken preoperatively and at least 1 month postoperatively. RESULTS: No major complications were encountered. Six of 49 patients underwent repeat resection for suspicious findings with pathology, demonstrating moderate dysplasia in two cases, carcinoma in situ in two cases, and inflamed mucosa only in two cases. There was no significant difference in preoperative VHI scores or objective voice grades among patients who underwent limited (ELS I-III) and those who required more advanced (ELS IV-VI) resection. There was a significant improvement in VHI scores in patients after ELS type I to III resection, from 38.77 to 22.86 (P = .006). There was no significant difference between mean preoperative and postoperative perceptual evaluation scores in patients who underwent ELS type I, II, or III resections (62.25 and 64.32 respectively, P = .621). CONCLUSIONS: Patients who undergo limited ELS resections can be assured of having a similar to improved voice after healing. Patients who undergo extended resections have poorer vocal outcomes. LEVEL OF EVIDENCE: 4.

Secondary tracheoesophageal puncture using transnasal esophagoscopy in gastric pull-up reconstruction after total laryngopharyngoesophagectomy.

BACKGROUND: There is debate about the optimal voice restoration method and technique for patients who have undergone total laryngopharyngectomy, esophagectomy, and gastric pull-up. The purpose of this study was to report a series of patients who underwent awake, secondary tracheoesophageal puncture (TEP) after this procedure. METHODS: A retrospective chart review was performed at a tertiary referral center. All subjects who underwent TEP placement under transnasal esophagoscopy guidance between 2003 and 2013 were included. RESULTS: All patients underwent uncomplicated TEP in the clinic. At the time of last follow-up, all patients had functional TEP speech that they were using preferentially over an available electrolarynx. CONCLUSION: In-office placement of secondary TEP using transnasal esophagoscopy is an efficient means of providing a conduit for voice prostheses in patients who have undergone laryngopharyngectomy with gastric pull up reconstruction. This procedure can be performed with minimal complications and with expectation of voice outcomes comparable to that seen with standard laryngectomy.

Repair of pharyngocutaneous fistula after total laryngectomy: A novel endoscopic approach.

BACKGROUND: Pharyngocutaneous fistula (PCF) is a common and challenging complication of laryngectomy. The purpose of this study was to evaluate the results of endoscopic repair of postlaryngectomy PCF. METHODS: Five patients who underwent salvage laryngectomy complicated by PCF underwent endoscopic repair at a regional medical center between 2006 and 2013. Data were compiled through a manual chart review and demographic data, adjuvant treatment, comorbidities, and clinical course were all recorded. RESULTS: Five patients underwent endoscopic repair of PCF. All patients had previously received radiation or chemoradiation therapy and underwent salvage total laryngectomy. The mean number of days to resumption of oral intake after endoscopic repair was 22 days. One patient required reoperation. No complications were noted. CONCLUSION: Endoscopic suture repair can be an effective method for treating PCF with less morbidity than flap reconstruction or conservative wound management.

Stimulation threshold greatly affects the predictive value of intraoperative nerve monitoring.

OBJECTIVES/HYPOTHESIS: Using a standardized, graded, intraoperative stimulation protocol, we aimed to delineate the effects of various stimulation levels applied to the recurrent laryngeal nerve on the postoperative predictive value of intraoperative nerve monitoring. STUDY DESIGN: A total of 917 nerves at risk were included for analysis. Intraoperatively, patients underwent stimulation of the recurrent laryngeal nerve at 0.3, 0.5, 0.8, and 1.0 mA followed by postoperative laryngoscopy for correlation with intraoperative findings. METHODS: Sensitivity, specificity, positive predictive value, and negative predictive value were calculated at each stimulation level. RESULTS: Sensitivity, specificity, positive predictive value, and negative predicative values ranged from 100% to 37%, 6% to 99%, 2% to 39%, and 100% to 99%, respectively at 0.3 to 1.0 mA. No demographic variables affected sensitivity or specificity. Receiver operating characteristic analysis identified 0.5 mA as the level of stimulation that optimizes sensitivity and specificity. CONCLUSIONS: The predictive value of intraoperative nerve monitoring varies greatly depending on the stimulation levels used. At low amplitudes of stimulation, nerve monitoring has high sensitivity and negative predictive value but low specificity and positive predictive value, related to the high rate of false positives. At high levels of stimulation, specificity and negative predictive value are high, sensitivity is low, and the positive predictive value rises as the rate of false negatives increase and the rate of false positives decrease. A stimulation level of 0.5 mA optimizes the predictive value of nerve monitoring; however, stimulation at multiple levels significantly improves the predictive value of intraoperative nerve monitoring. LEVEL OF EVIDENCE: 2b.

Effects of 2 different swallowing exercise regimens during organ-preservation therapies for head and neck cancers on swallowing function.

BACKGROUND: This study investigated the effects of 2 different swallowing exercise regimens performed during radiation therapy with/without chemotherapy on swallowing function. METHODS: Fifty patients were recruited prospectively to 2 groups (the exercise group, n = 26 and the repetitive swallow group, n = 24). Functional Oral Intake Scale (FOIS) scores and percutaneous endoscopic gastrostomy (PEG) placements were compared at posttreatment and at 3 months. RESULTS: The exercise group eliminated significantly more PEG tubes at 3 months posttreatment compared to the swallow group (16% vs 50%). Among patients who received both radiation and chemotherapy, the exercise group had significantly less PEG tubes immediately posttreatment as well as 3 months posttreatment (35% and 10%), compared to the swallow group (69% and 50%). CONCLUSION: Findings indicate significant benefits of the exercise group's exercise regimen in reducing PEG dependence and oral intake difficulties.

Interarytenoid botulinum toxin injection for recalcitrant vocal process granuloma.

OBJECTIVES/HYPOTHESIS: This study evaluated the efficacy of botulinum toxin type A injected into the interarytenoid muscle to treat recalcitrant vocal process granulomas. STUDY DESIGN: Retrospective clinical review at a tertiary care center. METHODS: Eight patients with vocal process granulomas refractory to a variety of prior treatments including surgical resection, proton pump inhibitor therapy, and voice therapy underwent percutaneous injection of botulinum toxin type A into the interarytenoid muscle, performed in an office setting. Doses ranged from 5 U to 25 U in one to two injections. RESULTS: One patient demonstrated no improvement, two patients demonstrated partial resolution, and five patients demonstrated complete resolution of their granulomas. Four patients noted transient breathiness. There were no other side effects. All patients tolerated the injections without difficulty. CONCLUSION: Botulinum toxin injection into the interarytenoid muscle appears to be a safe and effective modality for treating recalcitrant vocal process granuloma.

Paraganglioma of the hypoglossal nerve.

OBJECTIVES: To report a case of paraganglioma arising from the hypoglossal nerve and review the anatomy, clinical features, and literature to date. STUDY DESIGN: Case report and review of the literature. METHODS: Case records including paper and electronic chart and imaging reports were reviewed and summarized for the index case. A literature search was performed using pubmed keywords paraganglioma, hypoglossal, chemodactoma, carotid body, and glomus tumor. The available literature on the topic was reviewed and summarized. RESULTS: Paragangliomas associated with the hypoglossal nerve were reported in 4 cases over the past 47 years. Our index case presented with similar clinical features compared to those reported in the literature. Imaging with computed tomography and angiography showed a hypervascular mass at the carotid bifurcation, splaying the internal and external carotid arteries. As in previously reported cases, the source of the paraganglioma was only identified intraoperatively. The current case differs from prior reported literature in that the tumor was dissected from the associated hypoglossal nerve which was preserved. The patient clinically had no deficits in articulation or deglutination following excision of the lesion and was able to return to a normal diet within 24 hours of surgery. CONCLUSIONS: Hypoglossal paraganglioma is a neck mass that may not be distinguishable from more common carotid body or vagus tumors despite the use of multiple imaging modalities. Although XIIth nerve sacrifice may be requires in some instances, nerve preserving surgery, when possible, allows for complete recovery without functional deficits.