Cochlear Implantation in Charcot-Marie-Tooth Patients: Speech Perception and Quality of Life.

OBJECTIVES: There is a limited understanding of the impact of cochlear implantation (CI) in patients with Charcot-Marie-Tooth disease (CMT), given the scarcity of reported cases. We aim to evaluate the audiological outcomes and quality of life (QoL) after CI in CMT. METHODS: Multi-institutional, university-affiliated, tertiary-referral centers, retrospective chart review.Our cohort includes 5 patients with CMT. Patients' charts were reviewed for demographic characteristics, operation notes, and pre- and post-implantation audiology evaluation. Patients completed the Cochlear Implant Quality of Life-10 (CIQOL-10) Global questionnaire. RESULTS: Pre-implantation, the mean pure tone average was 84.1 ± 7.2 dB, and the mean word recognition score was 2.4% in the implanted ear. AzBio sentence test was performed in quiet, revealing a mean of 4 ± 1.4% in the implanted ear. Post-implantation, PTA results were all within the mild hearing loss range (mean 33.0 ± 5.9 dB). Post-CI, AZ-Bio test results were 5%, 65%, and 74% (for 3 patients), and HINT scores were 55% and 58% (for 2 patients). The mean score of the CIQOL-10 questionnaire was 42.7 ± 10.47 (range 1-100). Patients were most satisfied with their ability to listen to the television or radio, have conversations in a quiet environment, and feel comfortable being themselves. CONCLUSION: To the best of our knowledge, this is the most extensive series of CI in CMT-associated sensorineural hearing loss and auditory neuropathy. Our cohort suggests that CI is a safe and reliable method for hearing rehabilitation that can achieve good speech performance and improve QoL in CMT patients.

Predicting complications of pediatric temporal bone fractures.

OBJECTIVE: Our aim is to characterize complications of pediatric temporal bone fractures and identify predictive risk factors associated with fracture complications. METHODS: A retrospective review was conducted of all temporal bone fractures diagnosed in children (age less than or equal to 18 years) from a single academic institution between 2003 and 2017. Demographics, mechanism of injury, fracture characteristics, computed tomography evaluation and follow-up duration were recorded on each patient. Outcomes measured include facial nerve injury (FNI), cerebrospinal fluid (CSF) leak, sensorineural hearing loss (SNHL), and conductive hearing loss (CHL). RESULTS: One-hundred-seventeen patients with 129 temporal bone fractures were included in the study. Most fractures were otic capsule sparing (OCS) (96%, n = 124) and longitudinal (71%, n = 91). Otic capsule violating (OCV) fractures were associated with higher CSF leak rates (20% versus 2%, p = 0.14) and FNI rates (60% versus 5%, p = 0.002) compared to OCS fractures. Audiograms were available in 37 patients (34%). Patients with Glasgow coma scale (GCS) consistent with a mild traumatic brain injury (TBI) (GCS > 13) had significantly fewer complications (FNI and CSF leaks) compared to the group with moderate and severe TBI (GCS < 13), 5% versus 23% (p = 0.03). CONCLUSIONS: Higher complication rates are seen with OCV fractures and transverse fractures. Moderate and severe TBI as measured by GCS is predictive of FNI and CSF complications in pediatric temporal bone fractures.

State of the art regeneration of the tympanic membrane.

PURPOSE OF REVIEW: One of the most common diseases of the tympanic membrane is a perforation, and tympanoplasty is one of the more common procedures in otolaryngology. Tympanic membrane regeneration and bioengineering aim to improve the success rate of the procedure, increase the availability of different scaffolds and provide innovative tools that will simplify the surgical technique and make it accessible for surgeons with varying expertise level. This review aims to raise awareness of current tissue engineering developments in tympanic membrane regeneration and how they may augment current clinical practices. We focus here on achievements in tympanic membrane cell cultures and on innovations in development of new scaffolds and growth factors that enhance regeneration of patient's native tympanic membranes. RECENT FINDINGS: In recent years, great achievements were reached in the field of tympanic membrane regeneration in the three hallmarks of bioengineering: cells, scaffolds and bioactive molecules. New techniques for modeling normal tympanic membrane proliferation were developed, as well as for isolation and expansion of normal tympanic membrane keratinocytes from miniature samples of scarred tissue. Ongoing clinical trials aim to seal the perforation by applying different scaffolds infiltrated by growth factors on the tympanic membrane. SUMMARY: Research efforts in tympanic membrane regeneration continue to seek the ideal single tissue-engineered substitute. Recent advances in tympanic membrane bioengineering include new types of scaffolds that may augment and provide a safe and effective alternative to the current gold-standard autograft. New bioactive molecules may simplify the surgical procedure and reduce surgical time by augmenting the native tympanic membrane regeneration. Several groups of bioengineering scientists and neurotologists are continuing to move forward and develop new strategies, seeking to create a fully functional tissue-engineered tympanic membrane.

State-of-the-art methods in clinical intracochlear drug delivery.

PURPOSE OF REVIEW: Increasing awareness and prevalence of disorders in hearing and balance have placed emphasis on treatment strategies. With the rapid evolution in molecular, gene, and nanotechnology, alternate delivery methods have advanced intracochlear drug delivery. This review aims to raise awareness of recent developments in technologies to augment current clinical practices. RECENT FINDINGS: Intracochlear drug delivery research has expanded with the familiarity and accessibility to cochlear implantation. Various therapeutics are closely studied for both safety and efficacy as well as biologic effect. Agents including neurotrophins, antiapoptotics, cell therapy, gene therapy, and anti-inflammatory drugs are on the forefront of preclinical research. Cochlear implant electrode modification and drug administration at the time of implantation is a major focus of research. Improvements in study design have focused on overcoming barriers including elucidating the role of the blood-perilymph barrier. SUMMARY: Inner ear drug delivery methods include systemic, intratympanic, and intracochlear administration. Therapeutic technologies aim to overcome delivery barriers and to improve overall biologic effect while minimizing toxicity. Precision of drug application through intratympanic and intracochlear administration with minimal trauma is the future of inner ear drug development.

Optimizing the Chin and Jawline Appearance: Does Surgery or Injection Make Sense?

The chin is a crucial landmark and mandates careful consideration for facial appearance. Optimal chin appearance requires evaluation and treatment in three dimensions: anteroposterior, transverse, and vertical planes. The optimization of chin deficiencies requires understanding of bony anatomy as well as the relationship of the adjacent facial structures including the lip, teeth, and nose. The practitioner should be familiar with techniques in injection, placement of alloplastic implants, as well bony genioplasty.

Quantifying Lateral Frontal Sinus Access: The Upper Blepharoplasty Approach.

OBJECTIVE: Despite advances in technology and instrumentation, access to the lateral frontal sinus remains a challenge for surgeons. We sought to quantify the reach and applicability of the upper blepharoplasty approach (UBA) to the frontal sinus. METHODS: Twelve cadaveric specimens were obtained for anatomic research and frontal sinuses divided into 3 zones. Zone 1 was defined as medial to the supraorbital neurovascular bundle (SON). The remaining orbit was then bisected to define zone 2 (centrally) and zone 3 (laterally). Twenty-four UBAs were performed followed by 12 modified endoscopic Lothrop procedures (MELP). The ability to instrument each wall of the frontal sinus was recorded for the MELP, UBA, and combined approach. RESULTS: The UBA provided excellent access to the lateral frontal sinus in zones 2 and 3 (89% and 100%). The MELP provided poorer access in zone 3 (67%) but improved access in zone 1 (83%-100%). Access for zone 1 through the UBA was limited. The combined approach yielded 100% access to each frontal sinus boundary. CONCLUSION: The MELP in combination with the UBA/lateral trephination provides excellent access to each frontal sinus boundary. The UBA provides excellent access to the lateral frontal sinus but is limited medially by the SON.

A Population Based Analysis of Melanoma of the External Ear.

OBJECTIVE: Malignant melanoma accounts for nearly 75% of all skin cancer deaths, and the incidence is on the rise in the United States. External ear melanoma (EEM) is rare, and there is little long-term data regarding the clinical behavior of this melanoma site. This study analyzes the demographic, clinicopathologic, and survival characteristics of EEM. METHODS: The SEER database was queried for EEM cases from 1973 to 2012 (8,982 cases). Data analyzed included patient demographics, incidence trends, and survival outcomes. RESULTS: External ear melanoma occurred most frequently in the sixth and seventh decades of life. Mean age at diagnosis was 65.5 (±16.8) years. However, the incidence of EEM in adolescents and young adults (ages 15-39 yr) has increased by 111.9% from 1973 to 2012. There was a strong male predilection with a male-to-female ratio of 6.40:1. The most common histologic subtype was malignant melanoma, NOS (46.8%), followed by superficial spreading melanoma (21.4%), and lentigo maligna melanoma (17.9%). The majority of cases were localized at the time of presentation (88.0%), with rare distant metastasis (1.9%). The most common treatment modality was surgery alone (97.6%), followed by surgery with radiotherapy (2.3%). Ten-year disease-specific survival was better among those treated with surgery alone (90.7%), than those treated with surgery with radiotherapy (37.1%) (p < 0.0001). Increasing Breslow's thickness and presence of an ulcerating lesion were both associated with poorer survival (p < 0.0001). CONCLUSION: This study represents the largest cohort of EEM. It has an excellent survival outcome with surgery being the treatment of choice.

Laryngeal chondrosarcoma: A systematic review of 592 cases.

OBJECTIVES/HYPOTHESIS: Laryngeal chondrosarcomas are rare entities that arise from the cartilaginous structures of the larynx, including the cricoid, thyroid cartilage, epiglottis, and arytenoid cartilages. These tumors represent a minority of malignancies involving the larynx and can be mistaken for benign pathologies. The treatment has historically been surgical excision, often by total laryngectomy. This review investigates treatment modalities and patient outcomes. STUDY DESIGN: Systematic review using PubMed/MEDLINE and EMBASE database. METHODS: The databases were used to identify articles reporting cases of chondrosarcomas occurring exclusively in the larynx. Variables analyzed included patient demographics, presenting symptoms, grade, therapeutic approach, patient outcomes, and follow-up. RESULTS: Five hundred and ninety-two cases were identified. The average age reported was 62.5 years. There was a 3:1 male to female ratio. The most common surgical approach was local excision in 178 cases, followed by total laryngectomy in 174 cases. Nonsurgical treatment such as radiotherapy and chemotherapy was only used in 0.8% and 0.2%, respectively. Disease-specific survival rates for 1, 5, 10, and 20 years were 97.7%, 91.4%, 81.8%, and 68.0%, respectively, with no differences when comparing 5-year survival rates for location, grade, and therapy. CONCLUSION: Laryngeal chondrosarcomas are rare with a good prognosis. Various surgical approaches exist, with no difference noted in 5-year survival outcomes. Nonsurgical approaches were rarely used for these lesions. LEVEL OF EVIDENCE: N/A. Laryngoscope, 2016 127:430-439, 2017.

Sphenoid sinus malignancies: a population-based comprehensive analysis.

BACKGROUND: Sinonasal malignancies are rare, representing less than 1% of all cancers, with the sphenoid sinus accounting for 1% to 2% of these cases. Sphenoid sinus malignancies exhibit very poor outcomes. There is a paucity of literature describing their histopathological features, incidence trends, treatment, and survival. We seek to elucidate these factors using a national population-based resource. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify malignant sphenoid sinus tumors. The results were analyzed for demographics, incidence, and clinicopathologic trends. Survival was calculated using Kaplan-Meier analysis. RESULTS: The search identified 472 cases. The mean and median age at diagnosis was 60.0 years. Males represented 54.9% of cases. By race/ethnicity, 82.4% were white and 8.5% were black. The four most common histopathologies were squamous cell neoplasms (29.4%), adenocarcinomas (14.4%), non-Hodgkin's mature B-cell lymphomas (13.1%), and unspecified epithelial neoplasms (11.0%). The overall incidence from 2000 to 2012 was 0.030 per 100,000. Kaplan-Meier analysis demonstrated an overall 5-year disease-specific survival (DSS) of 48.1%. Of the most common histopathological subtypes, 5-year DSS was best for mature B-cell NHL (64.0%) and worst for unspecified epithelial neoplasms (25.6%). CONCLUSION: Sphenoid sinus malignancies are rare, with high prevalence in white males. The most common histopathology is squamous cell neoplasms. They exhibit significant locoregional extension. Of the common sphenoid sinus malignant subtypes, 5-year DSS is best for mature B-cell NHL and worst for unspecified epithelial neoplasms.

Utility of Surgery/Radiotherapy in Distant Metastatic Head and Neck Squamous Cell Carcinoma: A Population-Based Approach.

OBJECTIVES: The aim of this study is to analyze the survival benefits of surgery and/or radiation therapy over no therapy in patients with metastatic (M1) squamous cell carcinoma of the head and neck region (HN-SCC). STUDY DESIGN: Retrospective administrative database analysis. SUBJECTS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried for M1 HN-SCC cases from 1988 to 2012 (6663 patients). Patient demographics, initial treatment, and survival outcomes were analyzed. Survival was analyzed with the Kaplan-Meier model. RESULTS: Of the 6663 patients identified with M1 HN-SCC in the SEER database, 1669 patients received no therapy; 2459 patients, radiotherapy; 570 patients, surgery; and 1100 patients, surgery with adjuvant radiotherapy. The mean survival was 8.44 months for patients who did not undergo any therapy. In comparison, patients who underwent radiotherapy alone, surgery alone, or surgery with radiotherapy had mean survivals of 18.03 (P < .0001), 31.07 (P < .0001), and 39.93 (P < .0001) months, respectively. The 5-year disease-specific survival rates were 6.35% for no therapy, 17.51% for radiotherapy alone, 30.50% for surgery alone, and 33.75% for surgery with radiotherapy (P < .0001). Site-specific analysis revealed that surgery and/or radiation provides disease-specific survival benefit as compared with no therapy at all subsites within the head and neck region. CONCLUSIONS: Surgery and/or radiation-which has been shown to improve quality of life in patients with advanced cancer-is associated with an increased survival when utilized in patients with distant metastatic disease.

Internal carotid artery injury in endoscopic endonasal surgery: A systematic review.

OBJECTIVES/HYPOTHESIS: Internal carotid artery (ICA) injury during endoscopic endonasal surgery (EES) is a known and feared complication of paranasal sinus and skull base procedures. These ICA injuries can result in stroke, cranial nerve palsies, and death. This review examines the setting of injury along with the treatment approaches, and patient outcomes. STUDY DESIGN: Systematic review using PubMed/MEDLINE and EMBASE. METHODS: The databases were searched for articles reporting cases of ICA injury during EES. Variables analyzed included patient demographics, operative approach, preoperative diagnosis, setting of injury, repair method, imaging studies, patient outcomes, and follow-up. RESULTS: Twenty-five articles with 50 cases were included in this review. The EES approach was used for skull base procedures in 34 cases and for inflammatory disease in 16 cases. The most commonly injured ICA segment was the cavernous (34 cases), followed by the ophthalmic (three cases). Injuries occurred more commonly on the left (1.3:1). Injury occurred in the setting of various steps during EES with instruments. Stereotactic image guidance was reported in two cases. Initial hemostasis was achieved with packing in 35 cases, endoscopic clip sacrifice in four cases, bipolar coagulation with the intent to seal defect in three cases, and bipolar coagulation with the intent to sacrifice the ICA in one case. Intraoperative or immediate postoperative angiography was reported in 27 cases. CONCLUSIONS: The incidence of reported cases of ICA injury during EES remains low. Left-sided injuries to the cavernous segment of the ICA occurred more frequently than injuries on the right. LEVEL OF EVIDENCE: NA Laryngoscope, 126:582-590, 2016.

Laryngeal Adenoid Cystic Carcinoma: A Systematic Review.

OBJECTIVE: Adenoid cystic carcinoma is a malignant minor salivary gland tumor that represents <1% of all laryngeal tumors. The submucosal location of laryngeal adenoid cystic carcinoma (LACC) results in delayed presentation. Here, we present the first systematic review of reported cases of LACC to determine trends in presentation, diagnostic and treatment modalities, and patient outcome. DATA SOURCES: PubMed, Web of Science, MEDLINE, and EMBASE databases. METHODS: A search of the above databases was done to identify articles reporting cases of LACC. The variables included in the analysis were patient demographics, presenting symptoms, tumor location, imaging, treatment, follow-up time, recurrence, and outcome. RESULTS: A total of 50 articles and 120 cases were included in the review. The most common presenting symptom was dyspnea (48.8%), followed by hoarseness (43.9%). LACC arose most frequently from the subglottis (56.7%). At presentation, 14.6% (13 of 89) of patients had regional disease. The average follow-up time was 54.0 months. At follow-up, distant metastasis was reported in 30 cases (33.3%). Surgery alone (43.3%) and surgery with radiotherapy (43.3%) were used most frequently and resulted in 57.1% and 55.3% of patients alive with no evidence disease at follow-up, respectively. CONCLUSION: LACC was most often located in the subglottis. Patients commonly presented with dyspnea and hoarseness. In this systematic review, surgery with radiotherapy and surgery alone were the most commonly employed treatment modalities, and both resulted in slightly more than 50% of patients alive with no evidence of disease at follow-up.

Nasolabial cysts: A systematic review of 311 cases.

OBJECTIVES/HYPOTHESIS: Nasolabial cysts are rare nonodontogenic cysts arising commonly in the nasal alar region. Intraoral sublabial resection of nasolabial cysts is considered the standard treatment modality. In the past 16 years, transnasal endoscopic marsupialization has emerged as an alternative approach for these lesions. A systematic review on this entity was performed to evaluate management and outcomes. DATA SOURCES: PubMed/MEDLINE Database. METHODS: A search of literature including nasolabial cysts, nasoalveolar cysts, and Klestadt's cysts was performed using the PubMed/MEDLINE database. Parameters regarding presentation, treatment, imaging, histology, and complications were extracted and subsequently analyzed. RESULTS: Seventy-nine articles with 311 patient cases were included in this analysis. In cases reporting location, nasolabial cysts occurred 46.9%, 37.5%, and 10.9% on the left, right, and bilaterally, respectively. The most common presenting complaint was facial swelling in 168 out of 237 patients (70.9%). Computed tomography was the most commonly employed imaging study (n = 170). Surgical excision of nasolabial cysts was performed using an intraoral sublabial approach in 184 patients. Seventy-nine patients underwent endoscopic transnasal marsupialization. The most common complication following surgical treatment of nasolabial cysts was postoperative facial/perinasal swelling. Three and two recurrences were reported in the sublabial excision group and transnasal marsupialization group, respectively. Mean follow-up time was 20.0 months. CONCLUSIONS: Endoscopic transnasal marsupialization is a relatively new alternative to intraoral sublabial resection of nasolabial cysts, with similar efficacy. The rates of postoperative swelling, pain, and recurrence are similar in both groups.