Hearing loss in individuals with osteogenesis imperfecta in North America: Results from a multicenter study.

Hearing loss (HL) is an extra-skeletal manifestation of the connective tissue disorder osteogenesis imperfecta (OI). Systematic evaluation of the prevalence and characteristics of HL in COL1A1/COL1A2-related OI will contribute to a better clinical management of individuals with OI. We collected and analyzed pure-tone audiometry data from 312 individuals with OI who were enrolled in the Linked Clinical Research Centers and the Brittle Bone Disorders Consortium. The prevalence, type, and severity of HL in COL1A1/COL1A2-related OI are reported. We show that the prevalence of HL in OI is 28% and increased with age in Type I OI but not in Types III and IV. Individuals with OI Types III and IV are at a higher risk to develop HL in the first decade of life when compared to OI Type I. We also show that the prevalence of SNHL is higher in females with OI compared to males. This study reveals new insights regarding prevalence of HL in OI including a lower general prevalence of HL in COL1A1/COL1A2-related OI than previously reported (28.3 vs. 65%) and high prevalence of SNHL in females. Our data support the need in early routine hearing evaluation in all types of OI that can be adjusted to the severity of the skeletal disease.

BPPV Viewer: A downloadable 3D BPPV model for study of otolith disease.

OBJECTIVE: To develop a downloadable three-dimensional (3D) study tool of the membranous labyrinth in order to facilitate the study of benign paroxysmal positional vertigo (BPPV). BACKGROUND: The diagnosis and treatment of BPPV depend on an understanding of the anatomy of the vestibular labyrinth and its position relative to the head. To date, many illustrations have been made to explain principles of diagnosis and treatment of BPPV, but few have been based on anatomical studies of the membranous labyrinth. METHODS: A previously reported 3D model of a human labyrinth was transposed to a 3D development software to allow the creation of markers along the semicircular ducts and utricle. These markers represent otoliths at different positions during movement of the model within the 3D environment. User-friendly tools were created to navigate the model, to allow clear documentation and communication of studied problems, and to study the model across relevant planes. The final model can be downloaded and is available for general useat https://bppvviewer.com/download/. RESULTS: The model allows visualization of true membranous labyrinth anatomy in both ears simultaneously. The dependent portion of each semicircular duct, the planes of the cristae, and the position of the utricle can easily be visualized in any head position. Moveable markers can mark the expected progress of otolith debris with changes in head position and images can be captured to document simulations in various draw styles. CONCLUSION: This simple model could offer insights that lead to more accurate diagnosis and treatment of BPPV. It may also be useful as a tool to teach BPPV.

HPV-related oropharyngeal cancer prevalence in a middle eastern population using E6/E7 PCR.

BACKGROUND: Given the paucity of data and widely variable rates that have been reported, the main objective of this study was to examine the prevalence of HPV-positivity in oropharyngeal squamous cell carcinoma (OPSCC) in Middle Eastern patients presenting to one of the region's largest tertiary care centers using polymerase chain reaction (PCR) amplification of the HPV E6/E7 oncogenes, a highly sensitive and specific method of detection. METHODS: Medical charts and archived pathological specimens were obtained for patients diagnosed with biopsy proven oropharyngeal cancer who presented to the American University of Beirut Medical Center between 1972 and 2017. DNA was extracted from paraffin-embedded specimens and tested for 30 high-risk and low-risk papilloma viruses using the PCR-based EUROarray HPV kit (EuroImmun). RESULTS: A total of 57 patients with oropharyngeal cancer were initially identified; only 34 met inclusion/exclusion criteria and were included in the present study. Most patients were males (73.5%) from Lebanon (79.4%). The most common primary tumor site was in the base of tongue (50%), followed by the tonsil (41.2%). The majority of patients (85.3%) tested positive for HPV DNA. CONCLUSION: The prevalence of HPV-positivity amongst Middle Eastern OPSCC patients, specifically those from Lebanon, may be far greater than previously thought. The Lebanese population and other neighboring Middle Eastern countries may require a more vigilant approach towards HPV detection and awareness. On an international level, further research is required to better elucidate non-classical mechanisms of HPV exposure and transmission.

Impact of Infant Supraglottoplasty on Quality of Life.

Objectives The objective of the present study is to examine the impact of supraglottoplasty on the quality of life (QOL) of caregivers and infants with severe laryngomalacia and moderate laryngomalacia with feeding difficulties. Study Design Prospective cohort study. Setting Tertiary children's hospital. Subjects and Methods Thirty-nine infants who underwent supraglottoplasty were examined. The primary caregiver answered the 47-item short form of the Infant and Toddler Quality of Life Questionnaire-47 pre- and postoperatively; the subsection scores were compared. A 1-way analysis of variance was performed to analyze the effect of age and sex. A comparison was made between our cohort and a general population of healthy children. Results The average age at surgery was 4.0 months, and 53% of the patients were male. There was significant postoperative improvement in overall health, physical ability, growth and development, bodily pain, temperament, emotional impact on the caregiver, impact on caregiver's time, and family cohesion scores ( P < .05). The same subscale scores remained significantly improved postoperatively after age and sex were controlled. Preoperative QOL scores were significantly worse than those of the general population in nearly all categories. Postoperative physical ability ( P = .009) and temperament ( P = .011) QOL scores were higher than the those of the general population. Scores for growth and development ( P = .132), bodily pain ( P = .481), and family cohesion ( P = .717) were equivalent to those of the general population. Conclusion QOL was significantly improved after supraglottoplasty for infants with severe laryngomalacia and moderate laryngomalacia with feeding difficulties. After supraglottoplasty, QOL was similar to that of the general infant population in most categories.

Qualitative analysis of the Dix-Hallpike maneuver in multi-canal BPPV using a biomechanical model: Introduction of an expanded Dix-Hallpike maneuver for enhanced diagnosis of multi-canal BPPV.

INTRODUCTION/OBJECTIVE: Multiple canal BPPV can be a diagnostic challenge to the clinician. This is due in part to the complex anatomy of the labyrinth but also to complex and often simultaneous ocular responses that result from stimulation of multiple canals during traditional diagnostic testing. Our objective was to analyze the Dix-Hallpike maneuver used in the diagnosis of BPPV to look for patterns of simultaneous canal response and to develop a diagnostic maneuver that will allow separation of canal responses in multiple canal BPPV. METHODS: A previously created and published 3D biomechanical model of the human labyrinths for the study of BPPV was used to analyze and compare the position and movement of otoliths in the Dix-Hallpike maneuver as well as in a proposed expanded version of the traditional Dix-Hallpike maneuver. RESULTS: The traditional Dix-Hallpike maneuver with the head hanging may promote movement of otoliths in 5 of the six semicircular canals. The Dix-Hallpike maneuver with the head lowered only to the horizontal position allows for otoconia in only the lowermost posterior canal to fall to the most gravity dependent position. This position allows for minimal or no movement of otoconia in the contralateral posterior canal, or in either superior canal. Turning the head ninety degrees to the opposite side while still in the horizontal position will provoke otolith movement in only the contralateral posterior canal. The superior canals can then be examined for free otolith debris by extending the neck to a head-hanging position. These positions may be assumed directly from one to the next in the lying position. There seems to be no advantage to sitting up between positions. CONCLUSION: The Dix-Hallpike maneuver may cause simultaneous movement of otoliths present in multiple canals and create an obstacle to accurate diagnosis in multi-canal BPPV. An expanded Dix-Hallpike maneuver is described which adds intermediate steps with the head positioned to the right and left in the horizontal position before head-hanging. This expanded maneuver has helped to isolate affected semi-circular canals for individual assessment in multiple canal BPPV.

Adverse Reaction to Hyaluronic Acid Injection Laryngoplasty: A Case Report.

INTRODUCTION: Injection laryngoplasty using hyaluronic acid is a safe procedure commonly performed on patients with glottic insufficiency. STUDY DESIGN: This is a descriptive study of a case of adverse reaction to hyaluronic acid in a patient who underwent injection laryngoplasty for the treatment of unilateral vocal cord paralysis. DISCUSSION: The patient was treated with antibiotics and corticosteroids and had a full recovery. The authors recommend close observation following injection laryngoplasty using hyaluronic acid and diligent investigation of persistent postoperative laryngopharyngeal symptoms.

Changing Trends in the Management of Epistaxis.

Epistaxis is a very common complaint seen by many types of physicians including otolaryngologists, family physicians, and others. Management of epistaxis is often challenging and requires many types of intervention. The following review describes the different types of past and current treatment modalities including cautery, nasal packing, maxillary artery ligation, anterior artery ligation, and sphenopalatine artery ligation. The paper also proposes an algorithm for managing such cases.

Basaloid Squamous Cell Carcinoma of the Ethmoid Sinus with Invasion into the Skull Base Treated with Craniofacial Resection and Adjuvant Intensity-Modulated Radiation Therapy: A Case Report.

Basaloid squamous cell carcinoma (BSCC) is a rare variant of squamous cell carcinoma (SCC), which is highly aggressive, with a tendency for multifocality, local invasion, and with a high metastatic potential. Less than forty cases of BSCC of the sinonasal tract have been reported in the literature, and no reports were found on sinonasal BSCC arising from the ethmoid sinus. We report the case of a 78-year-old man who presented with BSCC arising from the ethmoid sinus with extensive bone destruction and intracranial extension. He was treated with craniofacial resection followed by adjuvant intensity-modulated radiation therapy to the tumor bed (60 Gy in 30 fractions), and the upper neck lymph nodes (50 Gy in 25 fractions). At the patient's last follow-up, four months after diagnosis, there was no evidence of disease. Aggressive management using craniofacial resection followed by adjuvant radiation therapy with or without radiosensitizing chemotherapy seems to be a reasonable approach to this challenging disease.