Diagnostic and surgical challenge: middle ear dermoid cyst in 12 month old with branchio-oto-renal syndrome and multiple middle-ear congenital anomalies.

Described is the first case report, to our knowledge, of a middle-ear dermoid in a child with branchio-oto-renal (BOR) syndrome. Radiographic, pathologic, and intraoperative figures are shown. This was a diagnostic and surgical challenge as the presentation was similar to a congenital cholesteatoma and the child had numerous significant temporal bone abnormalities. After the intraoperative findings suggested a non-destructive process, the treatment strategy was altered. This case reiterates the need for a cautious, flexible operative approach in a syndromic child. Included is a relevant review of the literature and a detailed clinical analysis.

Is arm span an accurate measure of height in young and middle-age adults?

Height is used to determine many important clinical measurements, but height may be difficult or impossible to measure accurately in some patients. The purpose of this study is to determine the accuracy of arm span as a measure of height in young and middle-age adults. A sample of 83 people between the ages of 20 and 61 years participated in this anthropometric study. Height and arm span were measured with a metal rule. A prediction equation was derived from regression analysis. Arm span is a valid measure of height in young and middle-age adults, and the accuracy is improved when using the prediction equation.

Acoustic mechanisms: canal wall-up versus canal wall-down mastoidectomy.

The contribution of the middle ear air spaces to sound transmission through the middle ear in canal wall-up and canal wall-down mastoidectomy was studied in human temporal bones by measurements of middle ear input impedance and sound pressure difference across the tympanic membrane for the frequency range 50 Hz to 5 kHz. These measurements indicate that, relative to canal wall-up procedures, canal wall-down mastoidectomy results in a 1 to 5 dB decrease in middle ear sound transmission below 1 kHz, a 0 to 10 dB increase between 1 and 3 kHz, and no change above 3 kHz. These results are consistent with those reported by Gyo et al. (Arch Otolaryngol Head Neck Surg 1986;112:1262-8), in which umbo displacement was used as a measure of sound transmission. A model analysis suggests that the reduction in sound transmission below 1 kHz can be explained by the smaller middle ear air space volume associated with the canal wall-down procedure. We conclude that as long as the middle ear air space is aerated and has a volume greater than 0.7 ml, canal wall-down mastoidectomy should generally cause less than 10 dB changes in middle ear sound transmission relative to the canal wall-up procedure.

Calbindin D-28k immunoreactivity in the medial nucleus of the trapezoid body declines with age in C57BL/6, but not CBA/CaJ, mice.

This study compared calbindin D-28k immunoreactivity in the medial nucleus of the trapezoid body (MNTB) in young (3-4 month old) and old (24-26 month old) CBA/CaJ mice, and young (3-4 month old), middle-aged (6.5-8.5 month old), and old (24-29 month old) C57BL/6 mice. C57BL/6 mice exhibit progressively more severe peripheral (sensorineural) hearing loss between 4 and 12 months of age, whereas CBA/CaJ mice show little change in peripheral sensitivity until very late in life. We obtained auditory brainstem response audiograms on all subject mice. Old CBA mice were selected for study whose audiograms matched those of young CBA and C57 controls. Middle-aged C57 mice showed elevated thresholds indicative of peripheral degeneration. Brain sections were reacted with anti-calbindin D-28k (CB). Staining patterns in Nissl and anti-CB material were characterized and cells were counted. We found no significant change in the number of CB+ cells or the total number of cells in the MNTB of old CBA mice compared to young controls. However, the mean number of CB+ cells decreased by 11% in middle-aged, and by 14.8% in old C57 mice. Since the decline in C57 mice was significant by 6.5-8.5 months of age, the decrease could be the consequence of a loss of input from the cochlear nucleus where cell numbers are known to decline by this age in this strain. The total number of neurons in MNTB assessed from Nissl material showed a more modest 7.1% decline with age in C57 mice, implying that the greater loss of CB immunoreactive cells with age cannot be completely attributed to a reduction in the total number of cells.

An investigation of competency to participate in legal proceedings in Canada.

OBJECTIVE: To assess fitness to stand trial, competency to plead guilty, and competency to understand Charter cautions to determine if the level of competency varies across these domains. METHODS: The Fitness Interview Test-Revised (FIT-R) and the Test of Charter Comprehension (ToCC) were administered to a group of individuals held on remand for fitness evaluations. Additionally, several questions from the FIT-R that address the ability to make a guilty plea were assessed separately and constituted an individual measure of competency to plead guilty (CoP). RESULTS: As predicted, the results indicated that the fact that an individual is competent at one juncture in the criminal proceedings does not mean that the individual necessarily is competent at all other stages of the proceedings. CONCLUSIONS: These findings suggest a need for a stage-specific approach to forensic competency assessments, requiring specialized instruments designed to assess the legal issues of competency at the various stages of legal proceedings.

The impact of Canadian Criminal Code changes on remands and assessments of fitness to stand trial and criminal responsibility in British Columbia.

OBJECTIVE: To evaluate the impact in British Columbia of the 1992 Criminal Code of Canada amendments dealing with remands for fitness to stand trial and not criminally responsible on account of mental disorder (NCRMD) assessments. METHOD: Information on 620 remands for evaluation of fitness to stand trial and/or NCRMD were collected from a sample obtained in British Columbia from 1992 to 1994. The data collected included length of remand order, length of evaluation, criminal charges, psychiatric diagnoses, and the decisions about fitness or NCRMD. RESULTS: Remands increased by about 20% in a 1993-1994 fiscal year compared with the previous year. The majority of evaluations continue to be conducted in an inpatient facility. The goal of a 5-day evaluation period is rarely met: only 12.5% of inpatients were released within 5 days of admission, and the average length of evaluation was about 3 weeks. The use and success of the NCRMD defence appears to be on the rise. In addition, there were some striking differences in remands from metropolitan and nonmetropolitan areas in terms of rates of referral and recommendations of unfitness or NCRMD. CONCLUSION: Results indicated that Bill C-30 has not yet had the anticipated impact on remands as inpatient evaluations continue to be the norm and evaluations typically take several weeks. Suggestions for policy reform and future research are presented.

Analysis of middle ear mechanics and application to diseased and reconstructed ears.

OBJECTIVE: To review current concepts of the mechanical processes of the human middle ear, and to apply them to practical issues in clinical otology and tympanoplasty surgery. BACKGROUND: The wide range of conductive hearing losses associated with middle ear pathology and reconstruction cannot be adequately explained by simple models of middle ear function. METHODS: Variables used to describe the system are sound pressure, volume velocity, and acoustic impedance. The relationship between specific middle ear structures and these variables is described such that inferences can be drawn regarding sound conduction in the normal, diseased, and reconstructed middle ear. RESULTS AND CONCLUSIONS: Sound can be transmitted from the car canal to the cochlea via two mechanisms: the tympano-ossicular system (ossicular coupling) and direct acoustic stimulation of the oval and round windows (acoustic coupling). Acoustic coupling is negligibly small in normal ears, but can play a significant role in some diseased and reconstructed ears. In the normal ear, middle ear pressure gain (which is the result of ossicular coupling) is frequency-dependent and less than generally believed. The severity of conductive hearing loss due to middle-ear disease or after tympanoplasty surgery can be predicted by the degree to which ossicular coupling, acoustic coupling, and stapescochlear input impedance are altered. Hearing after type IV and V tympanoplasty is determined solely by acoustic coupling. The difference in magnitude between the oval- and round-window pressures is more important than the difference in phase in determining cochlear input. In tympanoplasty types I, II, and III, adequate middle-ear and round-window aeration is necessary and the tympanic membrane-ossicular configuration may be less crucial.

Photosensitization and tissue distribution studies of the picket fence porphyrin, 3,1-TPro, a candidate for photodynamic therapy.

From a structurally distinct set of o-substituted tetraphenylporphyrins, the picket fence porphyrin (PFP), 3,1-meso-tetrakis(o-propionamidophenyl)porphyrin (3,1-TPro) has been selected as a potential candidate for use in the photodynamic therapy (PDT) of cancer. In this report, the time-dependent tissue distribution of 14C-labeled 3,1-TPro is described along with the results of various treatment regimens. The tissue distribution of radiolabeled 3,1-TPro is comparable to that of other porphyrin photosensitizers with the advantage of being most effective at 4 h and being cleared rapidly from most tissues. The results of the various treatment regimen experiments, as well as other studies, indicate that the 3,1-TPro mechanism of action is similar to that of other photosensitizers, but may include some minor differences. The conclusion is that 3,1-TPro and other PFP offer a class of effective photosensitizers that may be exploited for their structural versatility, straightforward synthesis leading to a compound of high purity and known structure, and stability (both in terms of shelf-life and in vivo metabolism) as potential candidates for PDT.

Fitness and competency issues in Canadian criminal courts: elucidating the standards for mental health professionals.

The issue of competency arises at various stages of criminal proceedings in an effort to protect those individuals who are unable to participate in the legal process. The competency question is a legal issue ultimately decided by a judge. However, mental health professionals are often called upon to provide insight into an individual's level of competence. To date, the standards by which an individual is found competent have remained far from clear. This creates a problem for clinicians who are requested to make competency evaluations. This article addresses the competency issues as they arise at various stages of legal proceedings in order to determine the standards that are applied by the courts. Furthermore, given the importance placed on mental health evaluations of competency, the role of mental health practitioners will be addressed as it pertains to the competency question at each juncture in the criminal justice system.