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Richard J Kennedy

Publications and source records attributed to Richard J Kennedy.

6 recordsLinked to original sources

Ventilation tubes and cochlear implants: what do we do?

OBJECTIVE: To determine current practice management with respect to ventilation tubes and cochlear implants. STUDY DESIGN: Questionnaire. SETTING: All members of the American Neurotology Society were sent questionnaires. MAIN OUTCOME MEASURES: Members were asked a series of questions including how they deal with ventilation tubes before cochlear implantation, how they manage serous otitis media in patients undergoing cochlear implantation, and how they manage otitis prone children with cochlear implants. RESULTS: Two hundred and twenty members returned questionnaires. Surgeons who replied perform an average of 25 implants per year: 15 in adults and 10 in children. Analysis of the data revealed a wide practice variation between surgeons. Fifty-six percent of surgeons will place a cochlear implant in a patient with a clean, dry ventilation tube in place. More than half the surgeons will place a ventilation tube in a child with serous otitis media, let the ear settle down, and perform the cochlear implant at a second operation. Wide variation in the management of otitis prone children with cochlear implants exists with respect to placement of ventilation tubes. Only 5% of surgeons reported any complications with cochlear implants that they attributed to ventilation tubes. There were a number who suggested their practice had changed since the recent identification of issues involving meningitis in implantees. CONCLUSION: Wide practice variation exists with the management of ventilation tubes in cochlear implant patients. On the basis of the results of this survey, it is acceptable to place cochlear implants in patients with clean, dry ventilation tubes. It also acceptable to place ventilation tubes in otitis prone children with cochlear implants. Despite theoretic concerns, the reported incidence of complications is low.

Cochlear Implantation↗

Stapedectomy outcomes: titanium versus teflon wire prosthesis.

OBJECTIVE: To compare the effectiveness of two stapes prostheses in hearing improvement of patients undergoing stapes surgery for otosclerosis. STUDY DESIGN: Retrospective chart review. METHODS: Titanium and Teflon wire stapes prostheses were compared with regard to effectiveness in closing the air-bone gap. The charts of 461 stapedectomies performed by one surgeon from 1996 to 2001 were reviewed. Patients who underwent stapedectomy for reasons other than otosclerosis, revision cases, and those with inadequate preoperative or postoperative bone-conduction threshold data were excluded. Small fenestra technique using either laser or drill was used for all patients. Inclusion criteria were met by 218 patients. Patients were then grouped according to type of prosthesis used, and hearing outcomes were compared. Measured outcomes were four frequency air-bone gap closure, pure-tone threshold, and rate of sensorineural hearing loss (SNHL). RESULTS: The study group was comprised of 35 titanium and 183 Teflon wire prostheses. Closure of the air-bone gap to less than 10 dB was achieved in 86% of the patients with Teflon prosthesis compared with 71% of those with titanium prostheses. The groups were equivalent in regard to site of otosclerotic disease as well as technique, laser or drill, used to create the fenestra. Rate of SNHL was low for both groups and not significantly different. CONCLUSIONS: Both prostheses provided comparable results, although the Teflon platinum wire prosthesis was slightly superior. The smaller numbers in the titanium group may confound these results. The design of the titanium prosthesis provides a crimp that is circumferential around the incus, and that prosthesis was selected in cases with a narrow incus. The selection bias may also influence the results seen in this study.

Adolescent↗

Dominant optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia: a syndrome caused by a missense mutation in OPA1.

PURPOSE: To describe the clinical features of and identify the disease-causing mutation in a large Utah family segregating a dominantly inherited syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia. DESIGN: Observational case series. METHODS: Thirty individuals at risk for a syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia in a single family underwent clinical examinations and venipuncture. Linkage analysis and mutation screening of the optic atrophy 1 gene (OPA1) were performed. RESULTS: Eighteen individuals demonstrated characteristics of the syndrome. Genetic analysis identified a G-->A substitution at nucleotide position 1334 in exon 14 of OPA1 causing an arginine-to-histidine change (R445H) in all affected members of the family. This change segregated with the disease phenotype in the study family with a LOD score of 7.02 at theta; = 0 and was not found in 200 normal control subjects. Analysis of an unrelated Belgian family with a similar phenotype revealed the same R445H mutation segregating with the disease phenotype. CONCLUSIONS: This study describes a mutation in OPA1 causing a unique syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia. These results expand the spectrum of human disease associated with mutations of OPA1 and indicate that ophthalmologists caring for patients with optic atrophy should inquire about possible associated hearing loss. Although OPA1 is a nuclear gene, the gene product localizes to mitochondria, suggesting that mitochondrial dysfunction may be the final common pathway for many forms of syndromic and nonsyndromic optic atrophy, hearing loss, and external ophthalmoplegia.

Adolescent↗

Intralabyrinthine schwannomas: diagnosis, management, and a new classification system.

OBJECTIVE: To outline the diagnosis and management of intralabyrinthine schwannomas and to propose a new classification system to further define them. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center at a university hospital. PATIENTS: Twenty-eight patients seen at the institution with intralabyrinthine schwannomas between 1996 and 2002 were included in the study. INTERVENTIONS: Diagnosis was made with magnetic resonance imaging in all but one case. Our ability to detect these tumors has been greatly enhanced by the introduction of high-resolution T2-weighted magnetic resonance imaging. Treatment options were observation with serial magnetic resonance imaging versus complete surgical removal via a translabyrinthine or transotic approach. Surgery was indicated for dizziness caused by the tumor or extralabyrinthine growth. MAIN OUTCOME MEASURES: Clinical features, audiology, radiology, and management outcomes were evaluated. RESULTS: Eight patients have undergone successful surgery with removal of their tumors and resolution of symptoms. One patient chose to have stereotactic radiotherapy. Of the 20 patients who were managed with observation and serial magnetic resonance imaging, only 1 has shown significant growth requiring surgical removal. CONCLUSION: Intralabyrinthine schwannomas are uncommon tumors that mimic the clinical features of many other neurotologic conditions. A high index of suspicion and precise imaging are often required to detect these tumors. Surgical treatment is indicated for specific indications and will be needed in the minority of patients with this disorder. The classification system that we propose is helpful in both the diagnosis and the management of these tumors.

Adult↗

External auditory canal cholesteatoma: clinical and imaging spectrum.

BACKGROUND AND PURPOSE: Cholesteatoma is an inflammatory lesion of the temporal bone that uncommonly involves the external auditory canal (EAC). In this large case series, we aimed to define its imaging features and to determine the characteristics most important to its clinical management. METHODS: Thirteen cases of EAC cholesteatoma (EACC) were retrospectively reviewed. Clinical data were reviewed for the history, presentation, and physical examination findings. High-resolution temporal bone CT scans were examined for a soft-tissue mass in the EAC, erosion of adjacent bone, and bone fragments in the mass. The middle ear cavity, mastoid, facial nerve canal, and tegmen tympani were evaluated for involvement. RESULTS: Patients presented with otorrhea, otalgia, or hearing loss. Eight cases were spontaneous, and five were postsurgical or post-traumatic. CT imaging in all 13 cases showed a soft-tissue mass with adjacent bone erosion. Intramural bone fragments were identified in seven cases. This mass most often arose inferiorly (n = 8) or posteriorly (n = 8), but it was circumferential in two cases. We noted middle ear extension (n = 5), mastoid involvement (n = 4), facial canal erosion (n = 2), and tegmen tympani dehiscence (n = 1). CONCLUSION: Temporal bone CT shows EACC as a soft-tissue mass within the EAC, with adjacent bone erosion. Bone fragments may be present within the mass. The cholesteatoma may extend into the mastoid or middle ear, or it may involve the facial nerve canal or tegmen tympani. Recognition of this entity and its possible extension is important because it may influence clinical management.

Adolescent↗