Creating a framework for data sharing in cochlear implant research.

OBJECTIVES: To summarize the development process of a national database that was designed to facilitate communication and collaboration, improve care, and create a framework for aggregate data sharing in cochlear implant (CI) research. METHODS: A group of nationally represented, multidisciplinary CI providers cooperated to define a standard set of data elements to incorporate into a database built by them in association with a group of computer scientists and software designers. CI centers across the USA, then, joined the non-profit Auditory Implant Initiative to use the database for their own clinical purposes and to help contribute to the national de-identified dataset for research and analytics. RESULTS: Approximately 12 months after the full release of the database, clinical information on 373 patients has been entered from 17 different CI centers representing 61 hearing professionals. A blend of six academic, seven private, and four non-profit CI centers participated in this phase of the data sharing network. DISCUSSION: The adoption of a single, standardized database by 17 centers throughout the USA has begun a framework for data sharing in CI research. Future steps include (1) expanding adoption, (2) scaling the database to include more patients, (3) streamlining the legal hurdles required for adoption, and (4) integrating the database with other software platforms (e.g. electronic health records, processors). CONCLUSION: A standardized clinical outcomes database that is utilized by a growing network of CI centers can help strengthen research through aggregate data sharing.

The management of pediatric cholesteatoma.

Pediatric cholesteatoma can be congenital or acquired. The two types appear to be separate and distinct entities based on molecular studies and clinical course. Pediatric cholesteatoma behaves differently from cholesteatomas in the adult. This may have more to do with anatomic and physiologic differences than with the molecular structure of the cholesteatoma. Treatment requires an individualized approach taking into account the experience of the operative surgeon and the high risk of recidivism of this disease.

Epstein-Barr virus-associated smooth muscle tumors in ataxia-telangiectasia: a case report and review.

Chromosomal breakage syndromes, including ataxia-telangiectasia (AT), are autosomal recessive disorders in which DNA repair mechanisms are defective resulting in chromosomal instability. Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important.