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Progress in Human Auditory and Vestibular Histopathology, pp. 5-10 edited by Salvatore Iurato and Jan E. Veldman © 1997 Kugler Publications bv/Amsterdam/New York.

An update on the NIDCD National Temporal Bone Hearing and Balance Pathology Resource Registry

Joseph B. Nadol, Jr., M.D.

The collection and study of well documented pathologic human temporal bones is essential for continued progress in elucidating disease processes of hearing, balance, and facial nerve function, for validation of clinical diagnoses during life, and to assess the efficacy of medical and surgical treatment. Newly developed techniques in molecular biology can be applied, both to freshly acquired and, in some cases, to archivally stored pathologic human temporal bones. The solicitation, documentation, collection, and study of human temporal bones is facilitated by a coordinated, national and international effort.

In the United States the National Temporal Bone Bank program (NTBB) was established in 1960 with the support of the National Institutes of Health and the Deafness Research Foundation (DRF). The principal purpose of the NTBB was the solicitation of temporal bone donors for four regional centers. On September 30, 1992, the National Temporal Bone Hearing and Balance Pathology Resource Registry was established by the National Institute on Deafness and Other Communication Disorders (NIDCD), to assume the responsibilities of the previous NTBB and, most importantly, to ambitiously add to the objectives in order to provide a more effective temporal bone network. This Registry came into being, at least in part, as a result of a workshop held in July, 1988, co-sponsored by National Institute of Neurological and Communication Disorders and Stroke (NINCDS) and the DRF.1 Although the symposium reaffirmed the importance of temporal bone collection, a decline in the number of active temporal bone collections as well as a decline in the number of active investigators in the area of temporal bone research was identified. Thus, in 1976 there were 28 active laboratories in the United States, whereas presently this number has fallen to approximately half that number. The purpose then of the contract awarded to the Massachusetts Eye and Ear Infirmary and the Deafness Research Foundation by NIDCD was to rejuvenate interest in research in the human temporal bone by establishing a computerized database of human temporal bone collections, proactively responding to inquiries from the public and researchers interested in temporal bone donation or research, dissemination of information about the human temporal bone collection, its importance for otologic practice, the implementation of professional educational activities in the field of human temporal bone and auditory brain stem study, the conservation of otopathologic collections at risk of being lost and, finally, the development and implementation of a national acquisition network to increase the yield of human temporal bone and brain tissue from donors.

Table 1: Collaborating Laboratories

Table 2:
Format of Registry Laboratory Collection Database

Table 3:
Format of Registry Temporal Bone/Brain Database

Of the 28 known human temporal bone collections in the United States, 15 are still active in the collection and study of specimens. All of the 15 active laboratories and 11 inactive temporal bone laboratories (Table 1) have collaborated with the Registry to provide a comprehensive data base with two components. The first is a "laboratory/collection" database (Table 2) consisting of demographic data from 15 active and 11 inactive collaborating laboratories. This provides information concerning location, telephone and fax number of the laboratory, the identity of the custodian, the number of specimens, both of temporal bones and human brain tissue, the average number of specimens processed per year, the staff of the laboratory, data concerning research training opportunities within the laboratory and a bibliography of published and unpublished data generated over the preceeding five years.

The second component of the data base relates specifically to temporal bone and related brain tissue (Table 3). As of February, 1995, data had been entered on 6,153 cases and over 11,500 specimens from 12 active and 6 inactive laboratories. The 7 collections that are collaborating, but for whom specific temporal bone data has not been entered, do not have written documentation of the temporal bone specimens, thus far precluding entry into the database. For each temporal bone specimen or brain tissue specimen, the Registry database contains demographic, clinical, audiologic, processing and histopathologic data and suitability of specimens for newer techniques, such as immunohistochemistry, antigen retrieval and DNA extraction.

Table 1
Collaborating Laboratories

Baylor College of Medicine, Houston, TX*
(Bobby R. Alford, M.D.)

Bowman-Gray School of Medicine, Winston-Salem, NC*
(Robert I. Kohut, M.D.)

Columbia University, New York, NY
(Jack Waen, M.D.)

Eye and Ear Institute of Pittsburgh, Pittsburgh, PA*
(Isamu Sando, M.D.)

Henry Ford Hospital, Detroit, MI
(Edwin M. Monsell, M.D., Ph.D.)

House Ear Institute, Los Angeles, CA*
(Fred H. Linthicum, Jr., M.D.)

J. Hillis Miller Health Center, Gainesville, FL
(George T. Singleton, M.D.)

Johns Hopkins Medical Center, Baltimore, MD*
(John K. Niparko, M.D.)

Massachusetts Eye and Ear Infirmary, Boston, MA*
(Harold F. Schuknecht, M.D.)

Memphis Eye and Ear Hospital, Memphis, TN
(John J. Shea, M.D.)

Mount Sinai Medical Center, New York, NY*
(Phillip W. Wackym, M.D.)

New England Medical Center, Boston, MA
(Collin Karmody, M.D.)

New York University Medical Center, New York, NY*
(H.K. Chandra Sekhar, M.D.)

Ohio State University, Columbus, OH
(Thomas F. DeMaria, Ph.D.)

Shea Clinic, Memphis, TN
(John J. Shea, M.D.)

State University of New York Health Science Center, Syracuse, NY*
(Richard R. Gacek, M.D.)

University of Chicago, Chicago, IL*
(Raul Hinojosa, M.D.)

University of Colorado, Denver, CO
(Michael J. Hart, M.D.)

UCLA, Los Angeles, CA*
(Vicente Honrubia, M.D.)

University of Iowa, Iowa City, IA*
(Bruce J. Gantz, M.D.)

University of Michigan Kresge Hearing Research Institute, Ann Arbor, MI*
(Richard A. Altschuler, M.D.)

University of Minnesota, Minneapolis, MN*(Michael M. Paparella, M.D.)

University of Oklahoma, Oklahoma City, OK
(B.Hill Britton, M.D.)

University of Texas Southwestern Medical Center, Dallas, TX*
(Charles G. Wright, Ph.D.)

University of Wisconsin, Madison, WI
(James Brandenburg, M.D., Thomas R. Pasic, M.D.)

*Active laboratories

Table 2
Format of Registry Laboratory Collection Database

Table 3
Format of Registry Temporal Bone/Brain Database

System architecture

The system runs on a 486-50 MHz IBM-compatible PC with an SCO Unix operating system. Peripherals include a high-resolution scanner, FAX/modem, floppy drive, high capacity read/write optical drive, CD/ROM drive, tape back-up drive and laser printer. Another 486-50 MHz IBM-compatible PC is used with a Microsoft Windows operating system for entry of prospective cases and for other office tasks such as word processing, statistical record keeping, generation of brochures and reports. The software employed for searching the database is the BRS Search software (BRS Software Products, McLean, VA). This is the most widely used interface for access to the National Library of Medicine (MEDLINE). It allows search of an entire document or specific field, sentences or paragraphs. Search operations include plain language, proximity, Boolean logic (and, or, with, not), and a fully integrated custom thesaurus for synonyms or related terms. The SCO Unix operating system allows simultaneous access to multiple on-site and off-site remote users. The software also provides report statistics regarding the number of records, meeting search criteria and location of search terms within the database. Report format can be individualized to provide results of specific fields desired. The specific format of the laboratory/collection database and temporal bone and related brain tissue database are shown in Table 2 and 3.

Retrospective data collected from laboratories have been entered by direct read into an editor from those collections already indexed by computer. Collections indexed on paper have been scanned into the same editor using optical character recognition software. Free text indexing and searching capability provides flexibility in the entry of pre-existing records and eliminates the need for a restrictive vocabulary and manual editing. Prospective data is entered by keyboard into a customized screen, using the BRS Search Software. Copies of the screen have been distributed to member laboratories for remote site data entry which is then transferred to the Registry by floppy disk or modem.

Access is limited to authorized personnel with various levels of password security, ranging from "read-only-access" providing data to principal investigators of member laboratories, "read-write-access" limited to those laboratories processing new temporal bones, thus allowing new data to be entered. These new entries will be verified by Registry Staff before being incorporated into the permanent database. The CD/ROM copies of the database have been distributed to individual member laboratories and require appropriate passwords. The final level of access is the read-write-and modify access and gives the authority to access the permanent database and to modify it. This is limited exclusively to the Registry investigators and its systems manager.

Interested researchers can also request a search of the database performed by Registry staff by written or telephone request. Between 1992 and February, 1995, there were a total of 163 database searches of the Registry's central office in addition to locally generated database searches in each of the member laboratories.

Because of changing departmental priority, there are valuable collections of temporal bones in the United States which are currently inactive and, therefore, at risk of being lost. Voluntary identification of an "at risk" collection results in a site visit by one of the Registry's investigators followed by a succinct description of the temporal bone collection and a solicitation for scientific proposals and conservatorship of collections which are no longer desired by the original laboratory. The solicitation is carried out in the Registry's newsletter, and the determination of conservatorship is done by vote of the Registry's Scientific Advisory Council. In the last three years two temporal bone collections, the Strauss Collection at Case Western Reserve consisting of approximately 100 neonatal and infant temporal bones, and the Brunner Collection (Los Angeles) of 45 sets of temporal bones from the pre-antibiotic era have been conserved.

For all the different ways to contact the Registry go to Contacting the Registry.

A principal objective in responding to information inquiry is to further temporal bone donorship and procurement and to respond to requests for database search. The Registry has two voice telephone lines (1-800-822-1327 and 617-573-3711), a TDD line (617-573-3888), and a fax line (617-573-3838). Non-emergent inquiries are handled in non-business hours by an answering service and emergent requests, including temporal bone procurement help, is provided by an on-call beeper system. Between September 30, 1992 and February 24, 1995 over 1,200 information inquiries were logged and processed by the Registry. In addition, as a subcontractor of the Registry, the Deafness Research Foundation, maintains an information center, including voice, TTY and fax lines. A dedicated "computer server" on the world-wide web of the Internet will disseminate information about the Registry, its mission, the database for public and researchers.

Brochures which address temporal bone donation and research in non-technical terms can be requested free-of-charge from the Registry. These brochures are available to organizations and clinics that wish to distribute or display them in their office(s).

The target audiences for this information dissemination system includes the general public and the biomedical hearing and balance research community. The Registry maintains and updates a public mailing list consisting of all known individuals and voluntary organizations interested in the study of hearing and balance disorders, including their members and sponsors. This list currently consists of over 30,000 entries. There is also a biomedical community mailing list with over 9,000 individuals including members of the American Academy of Otolaryngology-Head and Neck Surgery, Association for Research in Otolaryngology, DRF Centurions, International Otopathology Society, all otolaryngology academic departments in the United States, and all active and inactive temporal bone laboratories in the United States.

The Registry has developed and disseminates an informational brochure for the purpose of recruiting temporal bone and brain tissue donation entitled "That Others May Hear." This short form brochure answers the most common questions asked by potential temporal bone donors and contains a detachable postage prepaid mailing card requesting additional information and enrollment materials. The Registry publishes a full color non-technical informational booklet entitled "The Gift of Hearing: Learning about temporal bone donation" in conjunction with the DRF. This is a 16 page booklet providing additional information.

The Registry has developed and disseminates donor enrollment material, including a medical questionnaire entitled "Confidential medical history for research purposes," a donor consent form, a wallet size temporal bone donor card, which specifically include both temporal bone and brain tissue donations.

The Registry publishes on a semi-annual basis "The Registry" a newsletter, each approximately 6-8 pages in length with information for the research community. This is an opportunity to publish requests for conservatorship of temporal bone collections, announcements of meetings, courses and workshops, and update on new publications.

In addition, descriptive articles have been published in the Archives of Otolaryngology Head and Neck Surgery2 and a variety of other publications including the DRF Centurion Newsletter, Contact (publication of the Cochlear Implant Club International) and The Director (newsletter of the National Funeral Directors Association).

Finally, the Registry maintains an exhibit of the Registry's function and database activities at three scientific meetings annually, including the ARO, combined otolaryngologic spring meeting and the American Academy of Otolaryngology-Head and Neck Surgery. Current plans for the next year, including a scientific article updating the methods of temporal bone removal including specimens which have undergone cochlear implantation and an instruction videotape. Both the article and videotape will be used to provide instructional information for the proper collection of temporal bone material for the research community.

View a breakdown and map of the Registry's donors by state- see Temporal Bone Research/Donor Program.

The Registry's Procurement Network does not cover as many areas as the donor database, however, the network does cover 45 states. A map of network coverage can be found in Temporal Bone Research/Procurement Network.

In addition to entering archivally collected data into the temporal bone database, the donor program is equipped to encourage and accept new temporal bone and related brain tissue pledges. From 1993 to the present, 671 new pledges have been acquired. Of 1,200 pledges previously generated by the National Temporal Bone Bank Program, 500 have been renewed. The Registry serves as an entry point for interested donors and also as the provider of donation recruitment and enrollment data for member laboratories. Upon receipt of an inquiry from a potential donor, informational material including medical history questionnaire and donor consent form, is sent. If a pledge is received it is entered into the computerized database and a wallet size pledge card with a 24 hour 800 toll free number, is provided to the donor. The donor's pledge is forwarded to the active collaborating laboratory nearest the donor's address. This laboratory will receive the donor's tissue when obtained. In addition, each member laboratory is encourage to solicit its own pledges using the standardized forms and informational brochures. Locally solicited pledges are returned to the Registry for central recording and entry into mailing lists and updating mechanisms.

In addition to donor materials, a "National Tissue Procurement Network" has been established in conjunction with network member laboratories, hospital pathology departments, organ procurement organizations, and the National Disease Research Interchange (NDRI) and its network of 200 organ procurement organizations. Since January 1, 1993 to September, 1995 the Registry has successfully collected 67 temporal bones and 12 brain specimens. These specimens were sent for processing to 9 member laboratories. The National Temporal Bone Procurement Network has at its core a 24-hour toll free telephone number and on-call beeper system, in order to assist in the identity of an appropriate procuring agency. The costs associated with removal of temporal bones, brain tissues and transportation of donated tissue is born by the recipient laboratory. It ranges from $50-$360.

Periodic courses and workshops have been sponsored by the Registry. These have included the Human Temporal Bone Histopathology Update, October 6, 1993, which was a satellite function of the AAO-HNS meeting in Minneapolis; and a full day Workshop on Human Temporal Bone Histopathology, held at the Massachusetts Eye and Ear Infirmary, June 20, 1994. Current plans include courses on human temporal bone and brain histopathology, and workshops to be held at active temporal bone laboratories.

On site Registry personnel at the Massachusetts Eye and Ear Infirmary include six investigators, a full time Registry coordinator, a systems manager, budget manager and one secretary (2). DRF, is a subcontractor to the Registry for developing and disseminating informational materials. The Registry has a Scientific Advisory Council consisting of the co-principal investigators and a designee from each of the collaborating active laboratories in the country. The council meets once or twice a year. Its agenda includes an update on Registry function, improvement of the donor and procurement network, establishment of guidelines for professional courses and workshops, review of candidate member status of new laboratories, review of proposals for conservatorship of "at risk" collections, the development of new programs to enhance and increase the interest in proficiency in human temporal bone study.

1. Bluestone CD, Naunton RF, Schutz MD (eds.): Human temporal bone reserach workshop report. Ann Otol Rhinol Laryngol 98 (Suppl 3143):1-56;1989.

2. Merchant SN, Schuknecht HF, Rauch SD, McKenna MJ, Adams JC, Wudarsky R, Nadol JB Jr.: The National Temporal Bone Hearing and Balance Pathology Resource Registry. Arch Otolaryngol HNS 119:846-853;1993.

An Update on the NIDCD National Temporal Bone Hearing and Balance Pathology Resource Registry, Progress in Human Auditory and Vestibular Histopathology, pp. 5-10 edited by Salvatore Iurato and Jan E. Veldman © 1997 Kugler Publications bv/Amsterdam/New York.