Faculty Bibliography

Two groups of children, with (n = 7) and without (n = 7) first-year histories of otitis media, were participants in a longitudinal study that included periodic audiological and medical evaluations during the first year of life. At age 9, these children were tested on a series of speech perception and verbal short-term memory tasks using stimuli of varying degrees of phonetic contrast. Although the otitis-positive group performed less accurately than the otitis-free group, the pattern of errors was the same for the two groups. The performances of the children with and without positive histories of otitis media were negatively affected by an increase in phonetic similarity of the stimulus items. The two groups, however, did not differ on identification or on temporal-order recall when the speech sounds were differentiated by multiple features. These findings provide evidence of subtle, long-term effects of early episodes of otitis media on phonological representations and on working memory.

By a focus on three essential elements of language, phonology, semantics, and syntax, a time frame for critical/sensitive periods of language development is presented as a model of central auditory nervous system flexibility. Several studies support the hypothesis that the critical/sensitive period of phonology is from the sixth month of fetal life through the 12th month of infancy. Data indicates that the critical/sensitive periods for syntax runs through the fourth year of life, and for semantics through the 15th or 16th year of life. The data indicate that there is a time dependent series of functions in sequence that is based on responsive adaptations made by the CNS to psychophysical and electrophysiological stimuli.

There is data which indicates that the critical/sensitive period(s) for language development begins in the 6th month of fetal life and may be most susceptible to impairment during the first 2 years of life. There are observations which would indicate that the linguistic abilities which develop are dependent upon the amount of linguistic information available to the infant and are not dependent upon any particular receptive sensory mode. The challenge for the cochlear implant is to be able to transmit sufficient linguistic information--flux of information--during this period so that the resultant language is optimal. Language must be the primary outcome measure of the cochlear implant in the infant and young child.

OBJECTIVE: Malleus ankylosis is a cause of conductive hearing loss that can be difficult to assess, particularly in association with otosclerotic stapes fixation. The aim of this study is to seek the clinical and audiometrical features unique to this pathology. STUDY DESIGN: The study design was a review of malleus ankylosis cases and an analysis of functional results after ossiculoplasty. SETTING: The study was performed at Jean Causse Clinic in France. PATIENTS: The authors reviewed a series of 123 ears in 112 patients who underwent surgery for incudo-mallear ankylosis from January 1991 to September 1997. Intervention: The surgical technique depends on the type of pathology encountered. In case of associated stapedial fixation, a stapedotomy with vein graft interposition and reconstruction with a total prosthesis will be performed during a same step. MAIN OUTCOME MEASURES: Clinical evaluation, preoperative and postoperative audiometrical evaluation, operative findings, histologic examination, and postoperative functional results. RESULTS: In our series, a postoperative air-bone gap smaller than 10 dB was obtained in 77% of cases. These results confirm the possibility of managing both pathologies in a single surgical step. CONCLUSION: Incudo-mallear ankylosis remains an unusual pathology but should be systematically assessed during surgery and preferably after separation of the incudo-stapedial joint. A preoperative diagnosis is difficult to ascertain; some audiometrical features allow a suspicion. In this series, it is shown that otosclerosis can be responsible for ankylosis as seems to be confirmed by the two cases presented in this study.

OBJECTIVE: To evaluate subjective and objective outcomes of surgical therapy in congenital conductive hearing loss (HL) presenting in adulthood. The impact of hearing restoration on quality of life (QOL) for these patients may differ from those with acquired HL. STUDY DESIGN: Retrospective review of five patients treated surgically. METHODS: Standard audiometry was completed. Patients completed a subjective questionnaire as well as subjective (open-set) questions. A seven-point Likert scale was used for closed-set responses with 1 representing the worst possible outcome and 7 being best. RESULTS: The average four-frequency improvements in pure-tone average and air-bone gap were 18 dB (range, 7-29 dB) and 16 dB (range, 2-30 dB), respectively. An average score was obtained for each closed-set question. The highest-scoring items were for overall improvement of QOL (average score, 6), followed by perceived improvement in hearing (average score, 5.6). Other positive responses were improved ability to use the telephone (four of five patients) and improved localization of sound (four of five patients). Negative subjective responses included prolonged dysequilibrium in two of five, prolonged adjustment period in three, and dysgeusia in one patient. CONCLUSION: Important components of outcome are likely to escape our review if only objective measures are used to evaluate surgical outcomes. These results suggest that improved hearing in this population provides specific improvements in lifestyle and is perceived as a significant improvement in overall QOL.

To understand the role cAMP phosphodiesterases (PDEs) play in the regulation of insulin secretion, we analyzed cyclic nucleotide PDEs of a pancreatic beta-cell line and used family and isozyme-specific PDE inhibitors to identify the PDEs that counteract glucose-stimulated insulin secretion. We demonstrate the presence of soluble PDE1C, PDE4A and 4D, a cGMP-specific PDE, and of particulate PDE3, activities in betaTC3 insulinoma cells. Selective inhibition of PDE1C, but not of PDE4, augmented glucose-stimulated insulin secretion in a dose-dependent fashion thus demonstrating that PDE1C is the major PDE counteracting glucose-dependent insulin secretion from betaTC3 cells. In pancreatic islets, inhibition of both PDE1C and PDE3 augmented glucose-dependent insulin secretion. The PDE1C of betaTC3 cells is a novel isozyme possessing a K(m) of 0.47 microM for cAMP and 0.25 microM for cGMP. The PDE1C isozyme of betaTC3 cells is sensitive to 8-methoxymethyl isobutylmethylxanthine and zaprinast (IC(50) = 7.5 and 4.5 microM, respectively) and resistant to vinpocetine (IC(50) > 100 microM). Increased responsiveness of PDE1C activity to calcium/calmodulin is evident upon exposure of cells to glucose. Enhanced cAMP degradation by PDE1C, due to increases in its responsiveness to calcium/calmodulin and in intracellular calcium, constitutes a glucose-dependent feedback mechanism for the control of insulin secretion.

OBJECTIVES: Multidisciplinary care of cancer patients in varied settings is well described in the literature, but there is little specifically describing the multidisciplinary care of the patient with hepatocellular carcinoma (HCC). The purpose of this article is to describe HCC and the multidisciplinary approach at the Philadelphia Veterans Affairs Medical Center (PVAMC). MATERIALS AND METHODS: HCC is one of the most common solid tumors in the world, but it is rare in North America. It is associated with environmental carcinogens identified in animal studies, hepatitis B and C, cirrhosis of any etiology, and various metabolic diseases. No reliable therapy has been established for HCC. Surgical resection is the best treatment, but it is possible only in the patient with adequate hepatic reserve and limited-stage cancer. From January 1995 to May 1998, 22 patients at PVAMC received a diagnosis of primary HCC. One patient was a candidate for surgery, two patients received radiation therapy, and one patient underwent chemoembolization. Eighteen patients presented with an advanced-stage disease and comorbidities. RESULTS: Therapy goals in these 18 patients were limited to supportive care and enhancement of quality of life. A multidisciplinary team provided care to this challenging patient population. The multidisciplinary team treating HCC at PVAMC consisted of physicians, nurses, pharmacists, social workers, and a chaplain. Most care occurred in the outpatient setting. Supportive therapy included the controlling of ascites and abdominal discomfort, hepatic encephalopathy, and pruritus. Opioids relieved abdominal pain. Psychiatric support and counseling helped patients and families cope with the poor prognosis. CONCLUSIONS: A multidisciplinary team approach helped provide care for this challenging population. Through anecdotal reports, patients and family expressed satisfaction with their care. Research is needed to systematically test interventions designed to enhance quality of life in patients with HCC.

Increased incidence of failed primary stapedectomy may be associated with the reduced stapedectomy training experiences in contemporary residency programs. It is well recognized that revision stapedectomy results are not as successful as those of the primary surgery. Nevertheless, in experienced hands, properly selected cases for revision can have highly successful outcomes in closing air-bone gaps. Fibrous adhesions and overhanging facial nerves are most refractory to revision surgery.