Fibrous incudostapedial joint in congenital aural atresia.

OBJECTIVES: (1) Determine the prevalence of a non-bony or fibrous incudostapedial (IS) joint in the setting of congenital aural atresia. (2) Assess this anomaly's impact on surgical management and associated hearing outcomes. STUDY DESIGN: Retrospective chart review. SETTING: Subspecialty private practice. SUBJECTS AND METHODS: Operative reports and audiometric data of patients who underwent congenital aural atresia repair by a single surgeon from 2007 to 2011 were reviewed for operative anatomic findings and audiometric outcomes. RESULTS: Two hundred twenty-eight operations on 206 ears were performed. Median age was 5 years old. Fifty-five (26.7%) of these ears had a fibrous IS joint. The severity of this anomaly was graded as mild in 23 ears, moderate in 20 ears, and severe in 12 ears. Mean postoperative pure tone air conduction (PTA2) in the severely fibrous group was 51 compared to 46 in the moderate group (P = .03) and 41 in the mild group (P = .006). Patients with a fibrous IS joint who underwent successful ossicular chain reconstruction (OCR) had a mean postoperative PTA2 of 30, which was a significantly better outcome than in patients with moderately or severely fibrous IS joints who did not have OCR (P < .05). CONCLUSION: A fibrous IS joint was seen in 27% of patients undergoing repair of congenital aural atresia. The severity of this anomaly has important implications for postoperative hearing results. These findings suggest that ossicular chain reconstruction should be performed in moderately or severely fibrous cases.

HEAR MAPS a classification for congenital microtia/atresia based on the evaluation of 742 patients.

OBJECTIVE: Describe anatomical and radiological findings in 742 patients evaluated for congenital aural atresia and microtia by a multidisciplinary team. Develop a new classification method to enhance multidisciplinary communication regarding patients with congenital aural atresia and microtia. METHODS: Retrospective chart review with descriptive analysis of findings arising from the evaluation of patients with congenital atresia and microtia between January 2008 and January 2012 at a multidisciplinary tertiary referral center. RESULTS: We developed a classification method based on the acronym HEAR MAPS (Hearing, Ear [microtia], Atresia grade, Remnant earlobe, Mandible development, Asymmetry of soft tissue, Paralysis of the facial nerve and Syndromes). We used this method to evaluate 742 consecutive congenital atresia and microtia patients between 2008 and January of 2012. Grade 3 microtia was the most common external ear malformation (76%). Pre-operative Jahrsdoerfer scale was 9 (19%), 8 (39%), 7 (19%), and 6 or less (22%). Twenty three percent of patients had varying degrees of hypoplasia of the mandible. Less than 10% of patients had an identified associated syndrome. CONCLUSION: Patients with congenital aural atresia and microtia often require the intervention of audiology, otology, plastic surgery, craniofacial surgery and speech and language professionals to achieve optimal functional and esthetic reconstruction. Good communication between these disciplines is essential for coordination of care. We describe our use of a new classification method that efficiently describes the physical and radiologic findings in microtia/atresia patients to improve communication amongst care providers.

Anatomical facial nerve findings in 209 consecutive atresia cases.

OBJECTIVE: Describe intraoperative facial nerve findings in 209 consecutive atresia cases. Identify preoperative and intraoperative anatomical variants that should alert the surgeon to potential high-risk facial nerve anatomy. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care subspecialty private practice. METHODS: Retrospective review of 209 consecutive atresia cases treated between 2007 and 2011. Descriptive analysis of intraoperative findings. Logistical regression models with generalized estimating equations were used to examine the effect of preoperative variables over the operative findings. RESULTS: Two hundred and nine consecutive patients (ages 2-48) underwent atresia repair between 2007 and 2011. Preoperative Jahrsdoerfer Scale was 9 (23%), 8 (42%), 7 (19%), 6 (2%), 5 or less (2%). The facial nerve was found to have an abnormal course in 39% of the cases and not identified in 1%. It was congenitally dehiscent in 53% of cases and was surgically exposed in 10%. The most common site of congenital dehiscence was in the tympanic segment (57%). Facial-stapes contact was found in 11% of cases. The stapedius tendon was absent in 30% of cases. A single patient had a mild transient postoperative paresis (House-Brackmann 2). CONCLUSION: Atresia repair remains one of the most challenging procedures in otology. In spite of modern preoperative imaging, the facial nerve remains at risk. When performing surgery on patients with preoperative facial nerve paresis and/or lower Jahrsdoerfer scores, the surgeon should be aware of a higher incidence of facial nerve abnormalities. Thorough knowledge of anatomical variations and meticulous surgical technique are mandatory to safely perform these surgeries.

Scalp as split thickness skin graft donor site for congenital atresia repair.

OBJECTIVE: Describe the use of the scalp as a donor site for split thickness skin grafts in otologic surgery. MATERIALS AND METHODS: Review of the literature with detailed description of surgical technique and authors' experience. RESULTS: One hundred and forty- four scalp split thickness skin grafts were obtained between 2007 and 2011. No significant complications were observed with optimal cosmetic results. CONCLUSIONS: The scalp is a viable option as a split thickness skin graft donor site for otologic and other head and neck surgery. Advantages include improved cosmetic results at the donor site, quick healing with low complication rates, and easy accessibility in the operative field.

Gene expression profiling of nasal polyps associated with chronic sinusitis and aspirin-sensitive asthma.

OBJECTIVE: To identify genes whose expression is most characteristic of chronic rhinosinusitis and aspirin-sensitive asthma through genome-wide transcriptional profiling of nasal polyp tissue. STUDY DESIGN: Prospective, controlled study conducted at a tertiary care institution. METHODS: Thirty genome-wide expression microarrays were used to compare nasal polyp tissue from patients with chronic rhinosinusitis alone (CRS, n = 10) or chronic rhinosinusitis and a history of aspirin-sensitive asthma (ASA, n = 10) to normal sinonasal mucosa from patients who underwent surgery for non-sinus related conditions (controls, n = 10). Genes found to be most characteristic of each polyp phenotype, as determined from bioinformatic analyses, were validated using real-time quantitative polymerase chain reaction (RT-PCR) and immunohistochemistry in different patient sets. RESULTS: The transcriptional signature of the control mucosa was distinctly different from that of either polyp phenotype. Genes most characteristic of the CRS phenotype included two upregulated genes--met proto-oncogene (MET) and protein phosphatase 1 regulatory subunit 9B (PPP1R9B)-and two downregulated genes--prolactin-induced protein (PIP) and zinc alpha2-glycoprotein (AZGP1). The gene most characteristic of the ASA phenotype was periostin (POSTN), which was upregulated relative to controls. Differences between the CRS and ASA phenotypes were associated with alterations in the 6p22, 22q13, and 1q23 chromosomal regions. CONCLUSIONS: Nasal polyps appear to have characteristic transcriptional signatures compared to normal sinonasal mucosa. The five genes identified in this study likely play roles in the pathogenesis of polyps associated with CRS and ASA, and are therefore attractive targets for novel medical therapies for these common debilitating diseases.

The frontal wishbone: anatomic and clinical implications.

BACKGROUND: The interfrontal septal cell, which often takes the shape of a wishbone on axial CT scan, was first described by Van Alyea more than 50 years ago; however, its potential role in disease of the frontal sinus was only recently recognized. The purpose of this study was to correlate the incidence and dimensions of this "frontal wishbone" (FWB) with the presence of frontal sinus disease. METHODS: The dimensions, drainage pathways, and disease state of the FWB were measured in 150 consecutive sinus CT scans performed at a tertiary care referral center. Statistical analysis was performed to correlate the FWB configuration to the presence of frontal sinus disease. RESULTS: A FWB was present in 77.3% of patients. The wishbone drained through a discrete ostium into either the left (54.8%), the right (45.2%), or the bilateral (0.8%) frontal sinuses. In those scans with mucosal thickening or opacification of the frontal sinus (36%), concurrent FWB disease was found in 82%. Patients with a large (>1 cm) FWB in the anterior-posterior dimension were more likely to have a diseased frontal sinus (p = 0.02). CONCLUSION: The term FWB is used to describe an air cell within the interfrontal septum, which may play a role in the pathogenesis of frontal sinus disease.