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Bruce J Gantz

Publications and source records attributed to Bruce J Gantz.

5 recordsLinked to original sources
PubMed2002

Performance of cochlear implant recipients with GJB2-related deafness.

Congenital profound hearing loss affects 0.05-0.1% of children and has many causes, some of which are associated with cognitive delay. For prelingually-deafened cochlear implant recipients, the etiology of deafness is usually unknown. Mutations in GJB2 have been established as the most common cause of heritable deafness in the United States. In this report, we identify cochlear implant recipients with GJB2-related deafness and examine the performance of these individuals. Cochlear implant recipients received a battery of perceptive, cognitive, and reading tests. Neither subjects nor examiners knew the etiology of deafness in these individuals. The implant recipients were then examined for mutations in GJB2 using an allele-specific polymerase chain reaction assay, single-strand conformation polymorphism analysis, and direct sequencing. GJB2 mutations were the leading cause of congenital deafness among the cochlear implant recipients screened. Cochlear implant recipients with GJB2-related deafness read within one standard deviation of hearing controls better than other congenitally deaf cochlear implant recipients and non-cochlear implant recipients. Individuals with congenital deafness should be offered GJB2 screening. Positive results establish an etiologic diagnosis and provide prognostic, genetic, and therapeutic information. Effective rehabilitation for profoundly deaf individuals with GJB2-related deafness is possible through cochlear implantation.

Adolescent↗
PubMed2002

National Cancer Data Base report on malignant paragangliomas of the head and neck.

BACKGROUND: Malignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985-1996. METHODS: The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome. RESULTS: The average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985-1990) to 46% (1991-1996). The 5-year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months). CONCLUSIONS: Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease.

Adult↗
PubMed2002

Three-month results with bilateral cochlear implants.

OBJECTIVES: To evaluate possible binaural listening advantages for speech in quiet, speech in noise, and for localization in a group of postlingually deafened adults with two cochlear implants functioning independently after 3 mo experience. DESIGN: Nine postlingually deafened subjects who had received a Cochlear Corporation CI24M implant in each ear were evaluated on a number of tasks. The subjects all had audiometric or biographical (e.g., duration of deafness) differences between the ears. Word and sentence materials were presented to the subjects in quiet and in noise with the signal always in the front and the noise from the front or either side. Results are reported for each ear and for both ears with the noise on either side. This allowed evaluation of head shadow and squelch effects. Additionally, localization ability was assessed for broadband noise presented either to the right or left of center at 45 degrees azimuth. Localization was assessed for each ear and for both ears. RESULTS: Results of speech testing in quiet showed a significant advantage for the binaural condition over the better ear in four subjects. In noise, with both signal and noise in front of the subject, a significant advantage of two ears over the better ear was found for four subjects. For noise to one side of the head, when the ear opposite the noise source was added to the ear ipsilateral to the noise, a significant advantage was demonstrated for seven of seven tested subjects. When the ear ipsilateral to the noise was added to the ear contralateral to the noise, a significant advantage was shown for only one of seven (noise on right) and three of seven (noise on left) tested subjects. The localization task showed that all seven tested subjects could discriminate 45 degrees left from 45 degrees right above chance with bilateral stimulation. Three subjects could perform the discrimination above chance with only one ear. However, performance with both ears was significantly better than performance with one ear for two of these latter subjects. CONCLUSIONS: We conclude that bilateral cochlear implants can provide real advantages, particularly when it is possible to utilize the ear that is away from a noise source, thus taking advantage of the head shadow effect. In addition, localization ability was generally better with two implants than with one.

Acoustic Stimulation↗
PubMed2002

Binaural cochlear implants placed during the same operation.

OBJECTIVE: To evaluate the binaural listening advantages for speech in quiet and in noise and to localize sound when independently programmed binaural cochlear implants are used, and to determine whether ears with different hearing ability and duration of profound deafness perform differently with cochlear implants as well as to what extent preimplant psychophysical and physiologic assessment could be predictive of performance. STUDY DESIGN: Prospective study in which patients were prospectively selected to undergo bilateral implantation during a single surgical procedure at a tertiary referral center. All testing was performed with patients using their right, left, or both cochlear implants. Preimplant and intraoperative measures used electrical stimulation at the round window and stimulation through the cochlear implant. RESULTS: Bilateral implantation during the same operation did not cause any postoperative problems such as severe vertigo or ataxia. At 1 year, results of speech testing in quiet demonstrated a binaural advantage for 2 of 10 subjects. Speech-in-noise testing demonstrated that two implants were beneficial for two individuals. All subjects benefited from a head shadow effect when an ear with a better signal-to-noise ratio was available. The ability to localize sound was improved with binaural implants in all subjects. Preimplant psychophysical or physiologic measures were not predictive of eventual speech perception performance. CONCLUSION: Binaural cochlear implants can assist in the localization of sounds and have the potential in some individuals to improve speech understanding in quiet and in noise.

Adult↗