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W Potsic

Publications and source records attributed to W Potsic.

8 recordsLinked to original sources

Homozygosity for the V37I Connexin 26 mutation in three unrelated children with sensorineural hearing loss.

Mutations in the Connexin 26 (Cx26) gene have been found to account for approximately 20% of all childhood deafness. This number approaches 50% in documented recessive cases of hearing loss. Two mutations, 35delG and 167delT, account for the majority of reported mutations in this gene, but to date, more than 60 mutations have been described. No other single gene has yet been identified that contributes this significantly to the aetiology of hearing loss. Several mutations in this gene have been found to predominate in specific ethnic populations (167delT in Ashkenazi Jews and 235delC in Japanese individuals). While the majority of mutations found in Cx26 result in frame shifts and premature terminations, a number of missense mutations have also been identified. The V37I missense mutation has been reported as both a polymorphism and as a potentially disease-causing missense mutation. The present authors have identified three unrelated individuals with sensorineural hearing loss who are homozygous for this mutation. One individual is of Philippine ancestry, another is from a Chinese and Cambodian background, while the third is of Chinese ancestry, raising the possibility that this mutation may be more frequent among populations in eastern Asia.

Child↗

Hearing rehabilitation using the BAHA bone-anchored hearing aid: results in 40 patients.

OBJECTIVE: This study evaluates the U.S. experience with the first 40 patients who have undergone audiologic rehabilitation using the BAHA bone-anchored hearing aid. STUDY DESIGN: This study is a multicenter, nonblinded, retrospective case series. SETTING: Twelve tertiary referral medical centers in the United States. PATIENTS: Eligibility for BAHA implantation included patients with a hearing loss and an inability to tolerate a conventional hearing aid, with bone-conduction pure tone average levels at 60 dB or less at 0.5, 1, 2, and 4 kHz. INTERVENTION: Patients who met audiologic and clinical criteria were implanted with the Bone-Anchored Hearing Aid (BAHA, Entific Corp., Gothenburg, Sweden). MAIN OUTCOME MEASURES: Preoperative air- and bone-conduction thresholds and air-bone gap; postoperative BAHA-aided thresholds; hearing improvement as a result of implantation; implantation complications; and patient satisfaction. RESULTS: The most common indications for implantation included chronic otitis media or draining ears (18 patients) and external auditory canal stenosis or aural atresia (7 patients). Overall, each patient had an average improvement of 32+/-19 dB with the use of the BAHA. Closure of the air-bone gap to within 10 dB of the preoperative bone-conduction thresholds (postoperative BAHA-aided threshold vs. preoperative bone-conduction threshold) occurred in 32 patients (80%), whereas closure to within 5 dB occurred in 24 patients (60%). Twelve patients (30%) demonstrated 'overclosure' of the preoperative bone-conduction threshold of the better hearing ear. Complications were limited to local infection and inflammation at the implant site in three patients, and failure to osseointegrate in one patient. Patient response to the implant was uniformly satisfactory. Only one patient reported dissatisfaction with the device. CONCLUSIONS: The BAHA bone-anchored hearing aid provides a reliable and predictable adjunct for auditory rehabilitation in appropriately selected patients, offering a means of dramatically improving hearing thresholds in patients with conductive or mixed hearing loss who are otherwise unable to benefit from traditional hearing aids.

Acoustic Stimulation↗

Parental values in the decision about cochlear implantation.

Parents of children who are deaf are required to make decisions shortly after diagnosis that will affect the child's method of communication and educational placement. This study examines the role of parental values and preferences in this decision making process. Twenty families with a deaf child participated in this study. Parents from 19 of these families completed a multi-attribute preference study, which assesses their preference for different outcomes in four domains, i.e. academic achievement, social life, communication, and emotional well being. In ten of these families, the child had received a cochlear implant. Results indicate wide variability in parental preferences, which do not appear to correlate with the parents' decisions to choose cochlear implantation. Interview data from the study provides some insight into the parents' motivations. The small size of the study limits further analysis of factors that influence parents' choices and suggests further study with larger numbers of families.

Adult↗

The efficacy of cefaclor vs amoxicillin on recovery after tonsillectomy in children.

Tonsillectomy and adenoidectomy continues to be one of the most commonly performed operations in the pediatric age group. The morbidity from tonsillectomy can be severe and includes throat and ear pain, fever, lethargy, and poor oral intake. A previous study at the Children's Hospital of Philadelphia (Pa) demonstrated the efficacy of amoxicillin therapy in minimizing some of these postoperative symptoms. However, some children continue to have a prolonged recovery even while receiving this antibiotic regimen. Because of these children and the high incidence of Staphylococcus found in tonsillar core tissue, a randomized, prospective study was undertaken at the Children's Hospital of Philadelphia to evaluate the efficacy of cefaclor vs amoxicillin in patients recovering from tonsillectomy. The patients received either ampicillin or cefazolin intravenously at the time of surgery and for 12 to 24 hours postoperatively. When oral intake was adequate, they received either amoxicillin or cefaclor orally for 7 additional postoperative days. Intraoperative cultures of the oropharynx and tonsillar tissue were obtained, as well as cultures of the tonsillar fossa 7 to 14 days postoperatively. The patients were evaluated for severity and duration of postoperative symptoms as well as complications. The results of this study showed no difference between the two groups of patients. We conclude that there is no justification for routine use of cefaclor over amoxicillin in the posttonsillectomy patient.

Amoxicillin↗

Fibrous dysplasia.

The controversy is this case centers around the management of this lesion, not the differential diagnosis. All the consultants agreed that the history, physical, and CT findings were consistent with various fibroosseous lesions, the most likely being fibrous dysplasia. The need for additional tests varied with a bone scan (Dr. Kearns), a bone scan and CT scan (Dr. McGill), and MRI, MRI angiogram, bone survey, BUN, creatinine, calcium, and phosphorus (Dr. Potsic). Cosmetic and functional changes were considered priorities for the consultants, with orbital compression, malocclusion, tooth eruption, nasal obstruction, and sinusitis (Dr. Kearns), nasolacrimal duct obstruction and orbital compression (Drs. McGill and Potsic) being the concerns. Because this lesion is benign and slow-growing, the consensus is that surgery should be reserved for functional or cosmetic compromise. But how aggressive should one be and what approach should be used? The approaches varied with midface degloving or lateral rhinotomy (Dr. Kearns), midface degloving (Dr. McGill), or a Caldwell-luc and lateral rhinotomy (Dr. Potsic). Assessment of this tumor postoperatively should be with patient examinations and serial CT scans. None of the consultants worried about sarcomatous changes in this tumor.

Facial Bones↗