Bedside Injection Medialization Laryngoplasty in Immediate Postoperative Patients.

OBJECTIVES: The morbidity of glottic insufficiency resulting from unilateral vocal fold immobility may significantly compromise postoperative recovery in patients with decreased pulmonary reserve or inability to protect their airway. Injection medialization laryngoplasty is an effective means of treating glottic insufficiency due to unilateral vocal fold immobility. The purpose of this study is to present our experience with bedside transoral injection medialization laryngoplasty in the immediate postoperative period. STUDY DESIGN: Case series with chart review. SETTING: Academic tertiary care hospital. SUBJECTS AND METHODS: Patient demographics, clinical characteristics, procedural details, and short-term outcome measures of oral intake were recorded in a cohort of 68 patients over 5 years with unilateral vocal cord paralysis who underwent injection medialization as a bedside procedure in the immediate postoperative period. RESULTS: Mean time to injection was 8.2 days postoperatively. At the time of injection, 40 of 68 patients were nil per os. Seventy percent (28 of 40) had their diet advanced to adequate oral intake within 5 days of injection. Greater than half of the injections (36 of 68) were performed in the intensive care unit. No complications were noted, and all patients in this cohort were able to tolerate the bedside injection. CONCLUSION: Bedside injection medialization laryngoplasty in the immediate postoperative period via the transoral approach can be performed in patients, even in the intensive care unit, while on anticoagulation, and may be of benefit for hospitalized patients with unilateral vocal fold immobility. Further studies quantifying improvement in voice and swallowing data are merited.

Cricotracheal separation after gunshot to the neck: report of a survivor with recovery of bilateral vocal fold function.

BACKGROUND: Traumatic tracheal injury via blunt or penetrating mechanism comes with a grave prognosis. Cricotracheal separation is a rare entity among these injuries and even more infrequent by means of penetrating trauma. Resultant airway discontinuity subsequent to these insults causes immense global hypoxia and tends to be uniformly fatal. OBJECTIVE: Our aim was to discuss emergent and surgical management of traumatic airway injury. CASE REPORT: We report the case of a 28-year-old male who sustained a gunshot wound to the neck resulting in laryngeal fracture and cricotracheal separation. We review the initial stabilization of his airway and detail the successive surgical management of his injury in the context of the current available literature, with an emphasis on timely airway stabilization when high suspicion for cricotracheal separation exists based on traumatic mechanism. CONCLUSIONS: Emergent management and stabilization of the airway is critical to survival in the context of trauma involving the neck and airway structures.

Giant cell tumor of the larynx: a case of malignant sarcomatous transformation.

We report what is to the best of our knowledge the first case of malignant transformation of a giant cell tumor of the larynx. The patient, a 34-year-old man, presented to our tertiary care university teaching hospital where he underwent hemilaryngopharyngectomy with radial forearm free flap reconstruction and 11 of 15 cycles of chemotherapy. He remained disease-free at approximately 6 years and 4 months of follow-up. The patient is decannulated and continues to have a good voice with excellent quality of life to this day. We discuss the patient's clinical course and subsequent treatment within the context of a review of the current literature regarding this disease entity. Our experience demonstrates that giant cell tumor of the larynx may present as a malignant neoplasm without adversely affecting the patient's prognosis when treated aggressively with surgical resection and adjunct chemotherapy.

Audiologic and genetic features of the A3243G mtDNA mutation.

BACKGROUND: Mitochondrial mutations have been shown to be responsible for syndromic and nonsyndromic hearing impairment. AIM: To assess the genotypic-phenotypic correlation of mitochondrial DNA mutations in three generations of a single family. METHODS: A single family with maternally inherited diabetes and hearing loss was recruited. Genomic DNA was subject to polymerase chain reaction-restriction fragment length polymorphism analysis (ApaI) for A3243G mutation detection and confirmation with direct DNA sequencing. The degree of heteroplasmy for the A3243G mutation in blood DNA samples was quantified. In addition, we reviewed audiological data of A3243G-associated hearing loss cases from the literature to provide details of audiologic features. RESULTS: Six of 11 family members were recruited. All affected members harbored the A3243G mutation. Four of six members had diabetes. Five of five affected members demonstrated hearing loss ranging from mild to severe. The degree of heteroplasmy ranged from 5.51% to 27.74%. CONCLUSIONS: Patients with a greater percentage of heteroplasmy have a trend toward more severe phenotypic presentations. Hearing loss is bilateral, sensorineural, and symmetric. The main audiogram shapes found were sloping. Additional studies are necessary to clarify the relationship between degree of heteroplasmy and phenotypic presentation.

Considerations for initial dosing of botulinum toxin in treatment of adductor spasmodic dysphonia.

OBJECTIVES: To assess the effect on voice improvement and duration of breathiness based on initial dose of onabotulinum toxin A (BTX-A) in the management of adductor spasmodic dysphonia (SD) and to compare voice outcomes for initial bilaterally injected doses of 1.25 units (group A) vs 2.5 units (group B) of BTX-A. STUDY DESIGN: Case series with chart review of patients with adductor SD treated at a tertiary care facility from 1990 to 2011. SETTING: Academic subspecialty laryngology practice. METHODS: Demographic data (age and sex), voice rating, duration of voice improvement, and breathiness were evaluated and compared between groups A and B using the Student t test and χ(2) analysis. RESULTS: Of 478 patients identified, 305 (223 in group A, 82 in group B) patients met inclusion criteria. The average age was 56.2 years in group A and 57.4 years in group B (P = .5). The female to male ratio was 2.91 for group A vs 3.56 for group B (P = .61). Good voice outcomes (grade 3 or 4) were reported by 91% of group A patients vs 94% of group B (P = .75). The average duration of voice improvement was 99.7 days for group A and 108.3 days for group B (P = .54). The average duration of breathiness was 10.88 days for group A vs 15.42 days for group B (P = .02). CONCLUSION: Patients injected with 1.25 units bilaterally had a statistically significant shorter duration of breathiness without a statistically significant difference in clinical effectiveness or voice outcome. It is therefore recommended that a relatively low initial BTX-A dose be used with subsequent titration to achieve improved voice outcomes.

High-resolution computed tomography analysis of the frontal sinus ostium: a pilot study.

PURPOSE: Identification and exposure of the frontal sinus recess (FSR) during endoscopic sinus surgery (ESS) are challenging due to the variable anatomy, the narrow opening of the frontal sinus ostium (FSO), and the proximity of vital anatomic structures. Hence, a strong understanding of frontal sinus anatomy is required to prevent intracranial entry. Consistent and easily identifiable landmarks and measurements could assist safe entry into the FSO. In this study, we determine the distances from the columella and anterior nasal spine (ANS) to the nasofrontal beak (NFB) and anterior skull base (ASB) using high-resolution computed tomography (HRCT) scans. METHODS: A radiographic analysis was performed at a tertiary care medical center. Measurements from the ANS to the NFB and ASB, and from the columella to the NFB and ASB were made using sagittal HRCT. Thirty-two HRCT scans were analyzed by three observers, and the mean distances and standard deviations were calculated. RESULTS: The mean distance from the ANS to the NFB was 52.3±3.4mm in men and 47.7±3.5mm in women (p<0.0001). Mean distance from the ANS to the ASB was 61.8±4.1mm in men and 56.5±4.1mm in women (p<0.0001). Mean distance from the columella to the NFB was 58.9±2.3mm in men and 53.0±3.3mm in women (p<0.0001), and from the columella to the ASB was 67.9±3.7 mm in men and 61.3±4.1mm in women (p<0.0001). CONCLUSION: While performing FSR exposure in ESS, it is recommended to stay a distance of less than 66.9 mm in men and 60.6mm in women from the columella to minimize intracranial complications.

Laryngeal involvement of multiple myeloma.

The objectives of this paper are to discuss a rare cause of laryngeal multiple myeloma, to review unique pathologic findings associated with plasma cell neoplasms, to discuss epidemiology, differential diagnosis, and treatment options for plasma cell neoplasms of the larynx. Laryngeal multiple myeloma, also noted in the literature as "metastatic" multiple myeloma, presenting as a de novo laryngeal mass is extremely rare with few reported cases. Laryngeal involvement of extramedullary tumors is reported to be between 6% and 18% with the epiglottis, glottis, false vocal folds, aryepiglottic folds, and subglottis involved in decreasing the order of frequency. We present the case of a 58-year-old male with a history of IgA smoldering myeloma who presented to a tertiary care laryngological practice with a two-month history of dysphonia, which was found to be laryngeal involvement of multiple myeloma. We review the classification of and differentiation between different plasma cell neoplasms, disease workups, pathologic findings, and treatment options.

Mitochondrial DNA mutation screening in an ethnically diverse nonsyndromic deafness cohort.

Deafness is a heterogeneous trait with many known genetic and environmental causes. Hereditary hearing loss is an extremely common disorder in the general population. Mutations in mitochondrial DNA (mtDNA) are known to be associated with nonsyndromic deafness (NSD) and syndromic deafness. The objective of this article is to investigate the frequency of common mitochondrial mutations (A1555G, G7444A, and A3243G) in an ethnically diverse cohort of probands with NSD from South Florida. These patients were ascertained at the University of Miami. Polymerase chain reaction-restriction fragment length polymorphism analysis and direct sequencing methods were used for mutation screening in a cohort of 217 patients with NSD. The frequency of common mitochondrial mutations is 1.84% (4/217) in this cohort. A1555G and G7444A accounted for four patients with NSD. Our mutation frequencies are comparable with those previously reported in other populations, indicating that mutations in mtDNA are an important cause of NSD in our patient cohort.

Efficacy of transnasal endoscopic resection for malignant anterior skull-base tumors.

BACKGROUND: Craniofacial resection (CFR) has been the standard of care for malignant tumors of the anterior skull base (ASB). However, during the past 2 decades, transnasal endoscopic resection (TER) has gained significant popularity. The purpose of this study is to compare CFR and TER with respect to perioperative and oncologic outcomes. METHODS: Retrospective analysis at a tertiary care medical center of 82 consecutive patients undergoing resection of tumors of the ASB between 1997 and 2011. RESULTS: Thirty-four patients underwent TER, while 48 patients underwent CFR. There was no statistical difference in major complications between the two groups (p = 0.29). However, TER patients had shorter operating room times (284 minutes for TER, 620 minutes for CFR; p < 0.001), lower intraoperative blood loss (675 mL for TER, 1000 mL for CFR; p = 0.005), shorter intensive care unit (ICU) stays (0 days for TER, 3 days for CFR; p < 0.001), and shorter hospital stays (4.5 days for TER; 7 days for CFR; p < 0.001). There were no differences for the rates of en bloc resection, negative margins, or disease-specific mortality. Subanalysis yielded a median follow-up of 5 years postoperatively. There were no differences in disease-specific mortality or recurrences in this group. CONCLUSION: Patients undergoing TER for tumors of the ASB are more likely to leave the ICU and the hospital earlier than their CFR counterparts. Furthermore, for carefully selected patients undergoing TER, excellent oncologic outcomes with survival and recurrence rates similar to patients undergoing CFR may be achieved. Comparison of oncologic outcomes, however, may be limited by discrepancy in histologic grade and clinical stage between the two groups. Nonetheless, TER seems to be an excellent alternative to CFR in appropriately selected patients.

L-N-Acetylcysteine treatment is associated with improved hearing outcome in sudden idiopathic sensorineural hearing loss.

CONCLUSION: Combination therapy corticosteroids plus an oral antioxidant L-N-acetylcysteine (LNAC) was associated with improved hearing over corticosteroids alone, particularly at the 6-month follow-up and at high frequencies (i.e. 4000 Hz). This is the first report of a beneficial effect of LNAC in sudden idiopathic sensorineural hearing loss (SISNHL). OBJECTIVE: To determine the association between antioxidant treatment and functional outcomes in SISNHL. METHODS: This was a case-control study of adult patients with SISNHL, treated with oral prednisone plus intratympanic dexamethasone either alone or in combination with LNAC. The outcome measure was change in pure-tone thresholds at 500-4000 Hz. Hearing recovery was also recorded as the percentage of subjects with final pure-tone threshold average (PTA) within 50% of the difference between the initial value of the affected ear and the value of the unaffected ear. Comparisons were made between combination (corticosteroids plus LNAC) and single (no LNAC use) therapy groups. RESULTS: At 6 months, the mean PTA improvements were 26.1 dB and 15.1 dB for the combination and single therapy groups, respectively (p = 0.046). Higher gains at 4000 Hz were noted with LNAC use. The percentage of patients with at least 50% recovery was 63% and 35% for the combination and single therapy groups, respectively (p = 0.0319).

Primary sclerosing fibroinflammatory pseudotumor of the maxillary sinus.

Inflammatory pseudotumor is a well known but poorly understood pathologic entity. It is associated with an unusual growth of fibrotic tissue admixed with varying amounts of inflammation that displaces and compresses normal anatomic structures, resulting in dysfunction. Pseudotumors have been found in multiple locations-the orbit in particular-but to the best of our knowledge, none has previously been reported as an isolated sinus lesion. We describe a case of primary sclerosing fibroinflammatory pseudotumor of the maxillary sinus that manifested as recurrent unilateral maxillary sinusitis in a 47-year-old woman. The patient was managed with surgery and oral steroids with full resolution of her symptoms. We also review the presentation, diagnosis, and treatment of fibroinflammatory pseudotumors within the context of the current literature.

Cochlear implantation in common forms of genetic deafness.

Genetic factors are among the main etiologies of severe to profound hearing loss and may play an important role in cochlear implantation (CI) outcomes. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this review article, current knowledge of cochlear implants for hereditary hearing loss will be discussed with an emphasis on relevant clinical genotype-phenotype correlations.

Airway implications of congenital sternal agenesis.

Congenital agenesis of the sternum is an extremely unusual malformation rarely encountered by the practicing Otolaryngologist. It typically arises in conjunction with other midline ventral congenital anomalies, including abdominal, diaphragmatic, and cardiac malformations. We report a case series of two patients managed with tracheotomy placement due to prolonged intubation. The first patient was a 63-day-old infant born at 34 weeks gestation with dysmorphic features, cleft lip and palate, and skeletal dysplasia, including absence of the sternum. The second patient was a 31-day-old infant born with ectopic cordis and diaphragmatic hernia. The inadequate closure of the anterior chest wall secondary to manubrium malformation or a variant contributes significantly to a child's inability to generate adequate ventilatory pressures. As a result, airway management must be considered not only to ensure airway patency but also an appropriate physiological environment to allow for adequate air exchange in the lungs.

Comparison of transnasal endoscopic and open craniofacial resection for malignant tumors of the anterior skull base.

OBJECTIVE/HYPOTHESIS: Craniofacial resection (CFR) represents the traditional approach for resection of anterior skull base (ASB) malignancies. However, this past decade has witnessed the emergence of transnasal endoscopic ASB resection (TER) as a feasible alternative. The aim of this study was to compare TER and CFR for ASB malignancy resection. METHODS: Retrospective analysis at a tertiary care medical center on 66 patients undergoing ASB malignant tumor resection between September 1997 and December 2006. RESULTS: Eighteen patients were treated with TER, whereas 48 patients underwent CFR. The two groups showed no significant difference in complication rate (27.8% for TER and 25.0% for CFR, P > 0.05), survival (94.4% for TER and 83.3% for CFR, P > .05), and metastasis (11.1% for TER and 8.3% for CFR, P > .05). A significant difference was observed in hospital stay with an average of 3.8 days in the TER group compared to 8.1 days in the CFR group (P < .05). Local recurrence approximated significance (5.6% for TER and 29.2% for CFR, P = .051) CONCLUSIONS: TER of ASB malignancy is associated with a decreased hospital stay and faster recovery when compared to open CFR. Lower local recurrence rate in the TER group may reflect a discrepancy in histology and clinical stage. We found no significant differences in survival, metastatic, or complication rates in the two groups, whereas patients in the TER group had the added benefit of a desirable cosmetic outcome. Overall, TER seems to be an excellent alternative to CFR in properly selected cases.

Dexamethasone base conserves hearing from electrode trauma-induced hearing loss.

OBJECTIVE/HYPOTHESIS: Local treatment of the cochlea after electrode insertion trauma with dexamethasone base conserves hearing against trauma-induced loss. STUDY DESIGN: Laboratory animal study. METHODS: A guinea pig model of electron insertion trauma (EIT)-induced hearing loss (HL) used 44 guinea pigs sub-divided into four groups: 1) unoperated, controls (Controls, n = 44); 2) EIT, untreated (EIT, n = 15); 3) EIT plus artificial perilymph (EIT + AP, n = 15); and 4) EIT plus dexamethasone base (EIT + DXMb, n = 14). Cochleae that received EIT were randomly selected with contralateral, unoperated cochleae as internal controls. Auditory brainstem responses (ABRs) in response to 0.5 to 16 kHz pure tones were obtained before surgery, immediately after surgery (0 day), and on post-EIT days 3, 7, 14, and 30. Hair cell counts were obtained from stained organ of Corti specimens from all four groups (n = 3/group). Data were analyzed using analysis of variance and a Tukey-Kramer honestly significant difference post hoc test with significance alpha set at <0.05 (hearing) and <0.001 (hair cells). RESULTS: There were significant differences (<0.05) between the ABR thresholds of unoperated (control) and contralateral operated (experimental) ears of EIT and of EIT + AP groups for all tested frequencies. There was no statistical difference (>0.05) in ABR thresholds in the EIT + DXMb versus control groups for 0.5 to 4 kHz tones. DXMb treatment protected hair cells from EIT-induced damage and loss while AP treatment did not. CONCLUSION: The absence of significant differences in hearing thresholds between the EIT + DXMb group and control ears in response to 0.5 to 4 kHz tones demonstrates that DXMb is as effective as the aqueous form of dexamethasone in conserving hearing against EIT-induced loss.

Adenotonsillar pathology in post-transplant patients.

OBJECTIVE/HYPOTHESIS: The purpose of this study is to evaluate the efficacy of adenotonsillar hypertrophy, Epstein-Barr virus (EBV) titers, and flow cytometry in the evaluation of post-transplant lymphoproliferative disease (PTLD) in an at-risk pediatric population and to propose flow cytometry is the most effective means of diagnosis and directed treatment. STUDY DESIGN/METHODS: The authors conducted a retrospective chart review of all pediatric transplant patients referred for adenotonsillectomy at a tertiary care center between May 2000 and May 2003. Thirteen patients were identified. All tonsils were evaluated for follicular lymphoid hyperplasia and atypical lymphoid cells. Seven of thirteen samples were stained for EBV RNA, and twelve of thirteen samples were tested using flow cytometry. Positive flow cytometry diagnosis was based on the presence of atypical lymphocytes, gene arrangement, or both. RESULTS: Seven of thirteen (7/13) examined patients were found to have adenotonsillar hypertrophy. Ten of thirteen (10/13) patients had elevated EBV serum titers. Four of thirteen (4/13) patients were found PTLD positive by flow cytometry. Of the four positive patients, all four patients were found to have elevated EBV titers and only two patients had adenotonsillar hypertrophy. CONCLUSIONS: Adenotonsillar hypertrophy is not a sensitive indicator of PTLD. EBV serum titers are sensitive but nonspecific. Flow cytometry is the most effective diagnostic modality and allows physicians to tailor treatment to specific forms of PTLD.

Facial cutaneous mucormycosis in a full-term infant.

Mucormycosis is a rare and potentially fatal fungal infection that most commonly affects the immunocompromised population. Although originally described by Paltauf in 1885 (D. G. Finn as cited by Vessely et al(1)((p573)) and A. M. Marchevsky as cited by Oh and Notrica(2)((p1607))), it was not until 1955 that Harris (as cited by Vessely et al(1)((p573))) reported the first case of a mucormycosis survivor. In recent years, the number of immunosuppressed patients has increased partly owing to the widespread implementation of organ transplantation and the increasing prevalence of human immunodeficiency virus infection. Consequently, the incidence of mucormycosis has also increased, especially in pediatric patients. In the setting of immunocompromise, a high index of suspicion is required to accurately diagnose and treat this potentially lethal infection in a timely fashion. To our knowledge, we report the first case of facial cutaneous mucormycosis in an infant, who also represents the first reported neonate or infant to survive a cutaneous mucormycosis infection of the head and neck.