Faculty Bibliography

Autoimmune Inner Ear Disease (AIED) is poorly characterized clinically, with no definitive laboratory test. All patients suspected of having AIED are given glucocorticoids during periods of acute hearing loss, however, only half initially respond, and still fewer respond over time.We hypothesized that AIED is a systemic autoimmune disease characterized by dysfunctional peripheral blood mononuclear cells (PBMC) responses to a unique cochlear antigen(s). To test this hypothesis, we examined end-stage AIED patients undergoing cochlear implant surgery and compared autologous perilymph stimulated PBMC from AIED patients to controls. We determined that autologous perilymph from AIED patients was unable to induce expression of a long membrane-bound Interleukin-1 Receptor Type II (mIL1R2) transcript in PBMC as compared with controls, despite similar expression of the short soluble IL1R2 (sIL1R2) transcript (p<0.05). IL1R2 is a molecular decoy that traps interleukin-1beta (IL-1beta) and does not initiate subsequent signaling events, thereby suppressing an inflammatory response. IL1R2 transcript length is regulated by alternate splicing, and the major inhibitory function is attributed to the full-length mIL1R2. In addition, IL1R2 expression is induced by dexamethasone.Separately, we prospectively examined patients with newer onset glucocorticoid-responsive AIED. Immediately prior to clinical treatment for acute deterioration of hearing thresholds, their PBMC demonstrated a robust induction of mIL1R2 in PBMC in response to dexamethasone in vitro that correlated with a clinical response to prednisone in vivo (p<0.0001) as measured by hearing restoration. In contrast, clinically steroid unresponsive patients demonstrated high basal levels of mIL1R2 in their PBMC and only minimally augmented expression in response to dexamethasone. Thus, induced expression of mIL1R2 appears to be a protective mechanism in hearing homeostasis and warrants further investigation in a large prospective clinical trial to determine if IL1R2 can be used as a specific biomarker for AIED.

OBJECTIVE: The treatment for head and neck cancer (HNC) often involves radiotherapy. Many HNC patients are treated at the academic center (AC) where the initial surgery or diagnosis was made. Because of the lengthy time course for radiotherapy, some patients are treated at community radiation facilities (non-AC) rather than the AC despite potential AC advantages in terms of experience and technology. Our goal is to determine if these potential AC advantages correspond to a difference in treatment outcome. STUDY DESIGN: Historical cohort study. SETTING: University of Kansas Medical Center, Kansas City, Kansas. SUBJECTS AND METHODS: Review of records of patients with HNC cancers evaluated at the otolaryngology (ENT) department of an AC. Each patient's information and treatment characteristics were recorded, including radiotherapy treatment venue and treatment outcome. RESULTS: Three hundred seventy-four patients were analyzed, 263 were treated at an AC and 101 at a non-AC. Patients treated at a non-AC were more likely to present with earlier stage tumors, be treated with radiation alone rather than chemoradiotherapy, and be treated with adjuvant rather than primary radiotherapy. There was no difference in overall survival or recurrence rates between AC and non-AC. CONCLUSION: Patients treated at an AC are more likely to have advanced stage tumors and receive chemoradiotherapy as their primary treatment. In analyses of matching patient subsets, there was no significant difference in patient outcomes. Patients can be treated at a non-AC without affecting outcome compared with treatment at an AC.

Ulnar head arthroplasty has been an emerging alternative for salvage of resection arthroplasty at the distal radioulnar joint (DRUJ) since the early 1990s. Recently, it has been offered for initially treating painful arthrosis or instability of the DRUJ. This follow-up study reports a surgeon's experience treating DRUJ disorders with ulnar head arthroplasty. Twenty-two wrists in 20 patients (11 females, 9 males), 6 with no prior wrist procedures were treated between 1995 and 2006 for painful DRUJ disorders with either Herbert-Martin (Martin Medizin-Technik, Tuttingen, Germany) or Avanta (Small Bone Innovations, New York, NY) head prosthesis. Follow-up averaged 54.3 months. A standardized telephone survey determined preoperative /postoperative verbal analog pain scores and a modified Mayo Wrist Score (delineating poor, fair, good, or excellent outcomes). Data suggest that ulnar head implant arthroplasty is a reasonable treatment option for DRUJ-related pain, loss of function, or salvage of failed distal ulna resection procedures. The analog pain score statistically significant decreased by 1.68 points when comparing preoperative to postoperative scores. Average Modified Mayo Wrist Scores were good, independent of whether the procedure was primary or salvaged. No significant difference was seen between the primary or salvage group modified Mayo Wrist Scores. Whereas averaged modified Mayo scores for both the primary and salvage groups were in the good category, prostheses used as primary procedures may be associated with fewer poor or fair outcomes. Two good and 1 excellent outcome of 3 wrists requiring revision procedures suggest that even with revision of the implant arthroplasty, satisfactory results may be expected.

OBJECTIVE: We hypothesize that increasing regulation of human subject research has reduced the number of published reports of surgical innovation in otolaryngology. STUDY DESIGN: A systematic review of a predetermined subset of otolaryngology literature. METHODS: We randomly selected half of the articles published in 1988 and 2006 in three prominent journals. After identifying information was concealed, abstracts were classified independently by two authors into three groups: innovation, modification, or neither. Disagreements were resolved by a third author who read the entire article. Proportions were compared with chi(2) analysis. RESULTS: The proportion of articles classified as innovation or modification decreased significantly from 67 of 367 (18.3%) in 1988 to 59 of 548 (10.8%) in 2006 (P = 0.001). The elimination of radiology and pathology quiz cases yielded similar results: 11.1 percent vs 18.9 percent, P = 0.001. Innovation or modification in animal model research also decreased (0.9% vs 3.8%, P = 0.003). Overall, only 4 articles were actually classified as true innovation. CONCLUSIONS: The frequency of true innovation was low in both years, but articles that described surgical modifications decreased significantly over this 18-year period. One reason may be increased administrative barriers for clinical research approval. Although our analysis cannot determine cause and effect, it is an important finding that deserves further study.

OBJECTIVE: To better understand the diagnosis, treatments, and outcomes of congenitally imperforate salivary ducts. METHODS: A systematic literature review was performed using the following terms: imperforate submandibular duct, imperforate Wharton's duct, congenital atresia submandibular duct, congenital atresia sublingual duct, and congenital ranula. Only papers focusing on congenital floor of mouth cysts arising from imperforate salivary ducts were included. Two additional patients from our own experience are also discussed. RESULTS: Seventeen papers published since 1950 met inclusion criteria. A total of 26 patients underwent therapy ranging from mere observation to complete excision of the cyst with the sublingual gland. Both of our 2 additional patients both underwent simple sialodochostomy (excision of the imperforate sublingual caruncle) with cyst decompression. For all 28 patients, there have been no reported recurrences, with mean follow-up of 24.8 months and median follow-up of 14.8 months. CONCLUSIONS: Congenital floor of mouth cysts arising from imperforate salivary ducts are rare, but they may mimic the appearance of a ranula. Unlike ranulas, these cysts may be safely and effectively cured by simple sialodochostomy. To date, 26 other cases have been described in the literature, and nearly all have been successfully treated with simple incision or marsupialization without recurrence. Care should be taken to exclude ranula as a diagnosis, as incomplete excision may likely result in recurrence.