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Simon I Angeli

Publications and source records attributed to Simon I Angeli.

9 recordsLinked to original sources
PubMed2006

Inner ear dysplasia with agenesis of the corpus callosum.

In this case report we describe a patient with agenesis of the corpus callosum and asymmetric bilateral sensorineural hearing loss, who was found to have a bilateral inner ear dysplasia. Both of these anomalies have been associated with different members of the Pax gene family. However, to our knowledge this is the first report demonstrating the coexistence of these two unusual entities. By uncovering the inner ear malformation, we are able to better counsel this patient with regard to avoidance of high-risk activities such as contact sports and recommend prophylactic vaccination for meningitis prevention.

Adolescent↗
PubMed2006

Hyaluronate gel stapedotomy.

OBJECTIVE: To evaluate hearing results and postoperative dizziness after stapedotomy with application of sodium hyaluronate gel (HG) to the oval window niche during fenestration of the footplate. STUDY DESIGN: Case-control study comparing 27 stapedotomies with HG and 32 stapedotomies without HG. OUTCOME MEASURE: Preoperative and postoperative audiometric data, postoperative dizziness (vertigo or dysequilibrium), and nystagmus. RESULTS: Postoperative hearing results were not statistically different for the 2 groups. There were no cases of postoperative sensorineural hearing loss greater than 10 dB. Early postoperative dizziness (chi(2) = 4.08, P = 0.043) and nystagmus (chi(2) = 5.05, P = 0.024) were reduced in the HG group. CONCLUSIONS: HG stapedotomy is safe and effective and results in less early postoperative dizziness and nystagmus when compared with the standard technique of stapedotomy. The application of HG to the oval window niche to prevent blood from entering or perilymph from escaping the vestibule during fenestration of the stapes footplate may contribute to a quicker recovery in outpatient otosclerosis surgery.

Adjuvants, Immunologic↗
PubMed2006

Conservation of residual acoustic hearing after cochlear implantation.

OBJECTIVE: This study was designed to test the hypothesis that partial hearing conservation is attainable after cochlear implantation with a long perimodiolar electrode. Surgical strategies for hearing conservation during cochlear implantation are described. STUDY DESIGN: Prospective, single-subject, repeated-measures design. SETTING: Academic tertiary care center. PATIENTS: Twenty-eight severely to profoundly hearing-impaired adult cochlear implant recipients who had some measurable hearing preoperatively. INTERVENTION: Cochlear implantation using Nucleus Freedom Contour Advance electrode. MAIN OUTCOME MEASURES: Preimplant and postimplant pure-tone thresholds and speech recognition scores were obtained to determine the incidence and degree of conserved hearing at a mean interval of 9 (+/-3.9) months. RESULTS: Thirty-two percent of subjects experienced complete conservation of hearing (0- to 10-dB loss), and 57% experienced partial conservation of hearing (>11 dB) after implantation. However, open-set speech recognition was partially conserved in only one subject. Cochlear implant performance was not better in patients with conservation of residual hearing. CONCLUSION: Conservation of pure-tone hearing was possible in 89% of implanted patients; however, residual speech perception was not conserved with this long perimodiolar electrode. A ceiling effect tends to inflate the prevalence of hearing conservation in implantation studies of severely to profoundly hearing-impaired patients.

Adult↗
PubMed2006

Lateral tympanoplasty for total or near-total perforation: prognostic factors.

OBJECTIVE: To identify prognostic factors affecting outcome in lateral tympanoplasty for total or near-total tympanic membrane perforation. STUDY DESIGN: Retrospective case series. METHODS: Patients were those presenting with total or near-total tympanic membrane perforation undergoing lateral tympanoplasty from 1999 to 2004. We systematically collected demographic, clinical, audiologic, and outcome information. Student t test was used to determine group differences. Logistic regression analysis was used to examine the relationship between success of grafting (dependent variable) and the independent variables. Multiple regression analysis was used to examine the relationship between postoperative air-bone gap (ABG) and independent variables. RESULTS: There were seventy-seven cases (58 primary and 19 revision cases) with average follow-up of 17 months. Successful tympanic membrane grafting occurred in 91% of cases. None of the independent variables studied was predictive of the success of graft incorporation (P > .05). The mean preoperative ABG was 29.8 +/- 10 dB and improved to a postoperative ABG of 16.5 +/- 11 dB (P < .001). Smaller preoperative ABG and normal malleus handle were associated with smaller postoperative ABG. In revision cases, mastoidectomy was associated with better functional results. CONCLUSIONS: Successful grafting of near-total and total tympanic membrane perforations occurred in 91% of the cases and was independent of demographic, disease, and technical variables. Disease variables (preoperative ABG and status of malleus handle) had a greater prognostic value on postoperative ABG than other variables. In revision tympanoplasty, mastoidectomy is associated with a better functional outcome.

Adolescent↗
PubMed2005

Cochlear implantation in a patient with profound hearing loss with the A1555G mitochondrial DNA mutation and no history of aminoglycoside exposure.

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature. We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance. We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.

Aged, 80 and over↗
PubMed2005

Etiologic diagnosis of sensorineural hearing loss in adults.

OBJECTIVE: To determine the etiology of adult-onset sensorineural hearing loss. STUDY DESIGN AND SETTING: This is a prospective cohort study of 60 adult subjects with bilateral sensorineural hearing loss of no obvious etiology by medical history and physical examination. These patients were evaluated at an academic medical center and underwent evaluation by high-resolution computed tomography of the temporal bone, autoimmune panel, and DNA testing for mutations of both the GJB2 gene and the mitochondrial DNA (1555A>G and 7445A>G). RESULTS: An etiologic diagnosis was achieved in 6 patients: cochlear otosclerosis, 1 case; dilated vestibular aqueduct, 1 case; a mitochondrial DNA 7445A>G mutation, 3 cases; and a mitochondrial DNA 1555A>G mutation, 1 case. CONCLUSION: This result underscores the importance of a search for the etiology of a hearing deficit in adult patients. There are specific interventions now available for the management of hearing-impaired patients with cochlear otosclerosis and mitochondrial DNA mutations.

Adult↗
PubMed2005

Coenzyme Q-10 treatment of patients with a 7445A--->G mitochondrial DNA mutation stops the progression of hearing loss.

CONCLUSION: CoQ 10 may be helpful in delaying the progression of hearing loss in patients with the 7445A-->G mitochondrial mutation. Objective. To assess the effect of an antioxidant drug (Coenzyme Q-10) on the hearing level of patients with the mitochondrial DNA 7445A-->G mutation and associated sensorineural hearing loss (SNHL). Material and methods. We identified three patients with bilateral non-syndromic SNHL harboring the mitochondrial 7445A-->G mutation. Two patients had a family history of hearing loss with a strong matrilineal inheritance. The other patient did not have a family history of hearing loss. Two patients (1 with familial and 1 with sporadic SNHL) received treatment with 75 mg of Coenzyme Q-10 (CoQ10) twice a day for 1 year. The remaining patient with a familial form of hearing loss did not agree to take the treatment. Average bone conduction pure-tone thresholds for 0.5, 1, 2 and 4 kHz were obtained before and after diagnosis of mitochondrial hearing loss, and before and after treatment with CoQ10. Results. CoQ10-treated patients did not show any additional deterioration of their SNHL after 12 (familial case) and 13 months (sporadic case). The progression rate of SNHL was 6 dB/year in the 2 years prior to initiation of treatment in the familial case who received CoQ10 treatment. One year after being diagnosed with mitochondrial hearing loss, the patient who refused CoQ10 treatment exhibited an 11-dB deterioration of his hearing thresholds. There were no side-effects related to treatment with CoQ10.

Adult↗
PubMed2003

Systematic approach to benign paroxysmal positional vertigo in the elderly.

OBJECTIVE: We evaluated the effectiveness of a management approach that combines the canalith repositioning maneuver (CRM) and vestibular rehabilitation (VR) in the treatment of benign positional paroxysmal vertigo (BPPV) in elderly persons. STUDY DESIGN AND SETTING: Forty-seven patients (>/=70 years old) with the diagnosis of unilateral posterior semicircular canal BPPV formed the study population. This study has 2 parts. In the first part, patients were randomly assigned to 1 of 2 groups: the CRM and avoidance (no treatment). Patients were evaluated 1 month after the first visit. Those patients not responding to treatment were enrolled in the second part of the study, treated with an individualized combination of CRM and VR, and then reevaluated 3 months later. RESULTS: Statistically significant improvement of vertigo and provoked nystagmus in 64% of patients in the CRM group compared with the no-treatment group. After the addition of VR, 77% of all patients improved. CONCLUSION: A combination of CRM and VR improves BPPV in the elderly. SIGNIFICANCE: These findings suggest that although CRM is more effective than no treatment, VR can be added to improve the results in the treatment of BPPV.

Aged↗