Auditory dysfunction in Stickler syndrome.

OBJECTIVES: To characterize the natural history and possible mechanisms of hearing loss in Stickler syndrome (OMIM 108300; or hereditary progressive arthro-ophthalmopathy) and to determine if the auditory phenotype is a useful discriminating feature for the differential diagnosis of this group of disorders. DESIGN: Multifamily study. SETTING: Outpatient audiology and otolaryngology clinics at the Warren Grant Magnuson Clinical Center of the National Institutes of Health, Rockville, Md. SUBJECTS: Forty-six affected individuals from 29 different families segregating Stickler syndrome. INTERVENTIONS: Clinical audiologic and otolaryngological examinations were performed on all individuals, including pure-tone audiometry, speech audiometry, and middle ear immittance testing. Otoacoustic emissions, auditory brainstem response, infrared video electronystagmography, and temporal bone computed tomography were performed on a subset of participants. RESULTS: The hearing loss was most often sensorineural in adults, and approximately 28 (60%) of the 46 adult patients had 2 or more thresholds greater than the corresponding 95th percentile values for an age-matched, otologically normal population. The hearing loss most often affected high frequencies (4000-8000 Hz) and was generally no more progressive than that due to age-related hearing loss. Type A(D) tympanograms (classification using the Jerger model), indicating hypermobile middle ear systems, were observed in 21 (46%) of the 46 affected individuals. Computed tomography of the temporal bones revealed no inner ear malformations in 19 affected individuals. CONCLUSIONS: The hypermobile middle ear systems observed in ears with normal-appearing tympanic membranes represent a novel finding for Stickler syndrome and are likely to be a useful diagnostic feature for this disorder. The overall sensorineural hearing loss in type I Stickler syndrome is typically mild and not significantly progressive. It is less severe than that reported for types II and III Stickler syndrome linked to COL11A2 (OMIM 120290) and COL11A1 (OMIM 120280) mutations, respectively, or the closely related Marshall syndrome. This difference will be a useful discriminatory feature in the differential diagnosis of this group of disorders.

Using endosseous dental implants for patients with ectodermal dysplasia.

Congenitally missing teeth and poorly developed or absent alveolar ridges are signs often associated with various types of ectodermal dysplasia. Endosseous dental implants may be used to support fixed mandibular prostheses in patients with ectodermal dysplasia. Anatomical factors and age considerations require careful attention to treatment planning.

Body and orofacial cathexis in edentulous complete-denture-wearing clients.

This description cross-sectional study examines cathexis, or satisfaction, with the body and the orofacial region in relationship to eight major demographic and health status variables in a nonprobability sample of 34 male and 47 female edentulous subjects aged 36 to 77 years. Kiyak's 27-item Body Cathexis Scale (BCS), including an innovative five-item Orofacial Cathexis Scale (OFCS), was shown to be reliable for use in elderly clients at least to age 77 (Cronbach's alpha = .95). More research is needed to validate the OFCS. Overall scores were in the neutral range, with teeth being the most negatively cathected body part. Longer periods of edentulism were associated with more dissatisfaction with the teeth. Body and orofacial cathexis were highly positively correlated. Chronic illness, education level, race, and religion were not significantly related to cathexis. Edentulism, especially recent edentulism in the elderly, female gender, and divorced marital status could be important indicators for body image disturbance.

Review of tracheostomy videos for staff education.

Tracheostomy care is a basic nursing skill. While it is a matter of routine procedure in the daily practice of otolaryngology and critical care nurses, general nurses in other areas may perform it infrequently. Therefore, many otolaryngology nurses provide staff education when a tracheostomy patient is present in a nonspecialty area. This educational effort can be time consuming and repetitious for the specialty nurse. Audiovisual materials can be useful as a basis for an educational session or as a substitute when the specialty nurse is unavailable. The author conducted a thorough search for available videorecordings on nursing care of adults with tracheostomies. Four videos were identified and acquired. Each one was viewed twice, and then subjectively rated for completeness and accuracy of content in a comparative, "Consumer Reports"-like manner. Recommendations are made considering content, cost, and availability. The review is written for experienced clinicians and presumes a thorough knowledge of tracheostomy care.

A review of Delphi surveys conducted to establish research priorities by specialty nursing organizations from 1985 to 1995.

Many nursing specialty organizations have completed Delphi surveys to establish their research priorities. The Society of Otorhinolaryngology-Head and Neck Nurses (SOHN) is currently conducting its own Delphi survey to the same end. This paper reviews the various Delphi methods employed by specialty nursing organizations on their members between 1985 and 1995. The studies reviewed are carefully limited to those commissioned by specialty nursing organizations and exclusive of those conducted by independent researchers who may have utilized specialty organization members. It presents an introduction to and definition of the Delphi methodology, as well as delving into methodologic and practical aspects of implementing a Delphi survey. It adds to the literature some information previously lacking on cost and time lines for studies, and will serve as a useful guide for future specialty nursing organizations who undertake a Delphi study as a novice effort. It intentionally does not recount the research priorities identified because it does not want to influence, in any way, responses of SOHN members involved in the ongoing Round I Delphi.

Overcoming the top 10 tracheotomy self-care learning barriers.

Multispecialty inpatient units are the norm in today's acute care settings. Since few units have the luxury of seasoned otorhinolaryngology (ORL) nurses, care of the postoperative patient with a tracheotomy, including discharge teaching and planning are left to the generalists. Generalist nurses and new ORL nurses may find themselves experiencing fear and anxiety along with the tracheotomy patients they care for and teach. Experienced ORL nurses are continually challenged to find ways to share their expertise with patients and less experienced nurses in effective ways. This article presents a unique approach to preparing nurses to teach self-tracheotomy care to their patients. Medical-surgical staff nurses attended a 90-minute inservice program presenting actual clinical scenarios of temporarily learning-impaired patients with tracheotomies, and were asked to role play effective interventions. The program content is designed to address the reluctance of inexperienced, busy nurses to confront learning barriers and motivate patients to accomplish early postoperative involvement in tracheotomy self care. The widespread nature of the challenges faced by nurses and patients regarding tracheotomy care has been confirmed by discussions with colleagues on a national level. It has been confirmed further by the first author's clinical experiences in a wide variety of health care settings. Recommended nursing actions and responses to learning barriers presented here are based on expert opinion and clinical experience.

Research attitudes of otorhinolaryngology clinicians: results of the 1996/1997 SOHN Delphi Survey. Society of Otorhinolaryngology and Head-Neck Nurses.

This paper reports the research experience and attitudes of respondents in the Society of Otorhinolaryngology and Head-Neck Nurses (SOHN) Delphi Survey of Otorhinolaryngology (ORL) Nursing Research Priorities. Questions on past experience with research and highest academic level achieved were included in the demographic section of the survey. A Research Attitudes Scale (RAS) was developed, validated, and administered. Results indicate that survey respondents were relative novices to the research arena but had positive attitudes about nursing research (group X = 6.2). Research attitudes scores decreased as scale items became more personal. A discussion of the SOHN Research Committee's structure and activities, as well as suggestions for additional study of SOHN members' research attitudes are offered based on the literature review and study findings.

Otorhinolaryngology nursing research priorities: results of the 1996/1997 SOHN Delphi survey.

This study was initiated by the Society of Otorhinolaryngology and Head-Neck Nurses, Inc. (SOHN) to establish research priorities for the specialty. Otorhinolaryngology (ORL) Nurses were asked to identify, rate, and rank priorities for ORL nursing research. The survey sample was gleaned from attendees at the 20th Annual SOHN Congress and Nursing Symposium plus a follow-up mailing to active SOHN members who did not attend. A decision-making method, the reactive delphi technique, was employed. Four rounds of soliciting opinions by questionnaires were completed. Data from each round were reviewed and analyzed by three ORL nurse investigators. The respondents were largely actively employed, female SOHN members who were seasoned nurses, experienced in ORL nursing. The majority were Registered Nurses (RN) or held Bachelor of Science (BSN) degrees. They were primarily employed as staff nurses in outpatient settings and working with a general otorhinolaryngology patient population of all ages. The fourteen priorities identified fall in the areas of patient outcomes (three), patient education (three), symptom management (two), patient self-care (two), psychosocial issues (two), and nursing care delivery (two), spanning the practice spectra of all ORL nurse generalists and subspecialists. The top five research priorities identified are; care of neck stomas and related equipment, quality of life in head and neck cancer patients, impact of managed care on ORL nursing care delivery, value of ORL specialty nurses/nursing units, and effective techniques to promote cessation of substance abuse in adolescents. This priority identification provides a baseline for the newly forming SOHN Foundation.

Implant rehabilitation in Erdheim-Chester disease: a clinical report.

Successful osseointegration of endosseous titanium implants is thought to be dependent upon close apposition of bone to the implant surface. The integration of implants in this patient was achieved despite the lipid-laden histiocytic infiltration of the bone marrow. Presumably, enough unaffected stromal cells were present to allow sufficient bone formation for osseointegration of the implant fixtures. This result invites speculation regarding both the mechanism of osseointegration and the minimum surface area of bone-implant interface necessary for achieving and maintaining osseointegration of titanium implants. This patient is periodically examined to determine if the loaded fixtures will remain clinically immobile for a prolonged period.