Cochlear implantation in posttraumatic bilateral temporal bone fracture.

Temporal bone (TB) fractures can cause loss of audiovestibular function. Four cases of profound hearing impairment following bilateral TB fracture are presented in this report. All patients received a cochlear implant. All 4 patients became regular users of their implants. None of the patients experienced facial nerve stimulation. Implant-aided audiometry demonstrated a hearing threshold of 28 dB HL. The performance in speech understanding was comparable to standard postlingual adult patients implanted. We believe that cochlear implantation in patients suffering from profound sensorineural hearing losses secondary to TB fractures can be an effective tool for rehabilitation.

Therapeutic approach in managing patients with large vestibular aqueduct syndrome (LVAS).

OBJECTIVE: To discuss the clinical approach in managing patients with large vestibular aqueduct syndrome. METHODS: Over the period from November 1997 to March 2005, 106 children have been fitted with cochlear implants in Antwerp University Hospital (UZA). The Hospital University of Maastricht (azM) had implanted 36 children since 1999 to March 2005. The present study focuses on nine children and one adult patient with large vestibular aqueduct syndrome (LVAS). The medical, report and radiology report were retrospectively analysed. RESULTS: Eight out of nine children with LVAS had been implanted and one child was on the waiting list. One adult patient was implanted with Nucleus 24M at the age of 22 years old. Nine out of 10 patients had bilateral large vestibular aqueduct. There were no intraoperative or postoperative complications encountered. CONCLUSION: Cochlear implantation is a safe operation for patients with LVAS.

Good speech recognition and quality-of-life scores after cochlear implantation in patients with DFNA9.

OBJECTIVE: To compare audiometric and quality-of-life results in DFNA 9 patients who received a cochlear implant with cochlear implant patients with adult-onset progressive sensorineural hearing loss. STUDY DESIGN: Prospective comparative design; results were collected cross-sectionally. SETTING: Tertiary referral center. PATIENTS: Eleven DFNA 9 patients were included in the study as well as a comparative group of 39 post-lingually deafened cochlear implant subjects with adult-onset progressive sensorineural hearing loss. INTERVENTIONS: All patients received a cochlear implant. Subjects were implanted with either the Nucleus 24 M/RCS or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, or CIS+ coding strategies. MEAN OUTCOME MEASURES: Speech recognition was determined by means of phonetically balanced monosyllabic word lists. The Hearing Handicap Inventory for Adults, the Glasgow Benefit Inventory, and the Scale for the Prediction of Hearing Disability in Sensorineural Hearing Loss were used to quantify the quality of life. RESULTS: The results show that the speech perception and the quality of life of the DFNA 9 patients do not differ significantly from the control group (p=0.179; p=0.56). CONCLUSION: In spite of the fact that DFNA 9 is a disease that is known to involve cochlear dendrites, cochlear implantation is a good option for treatment of deafness in DFNA 9.

Interpretation of pure-tone thresholds in sensorineural hearing loss (SNHL): a review of measurement variability and age-specific references.

When assessing a patient with a sensorineural hearing impairment, the most simple and most widely available technical investigation is pure-tone audiometry. Although it is a subjective measure, the test is very reliable if the patient is cooperative. In this paper we review standards and test-retest reliability for pure-tone audiometry. A pure-tone threshold measurement at a single frequency has a chance of 90% to be repeated between -10 dB and +10 dB compared to the first measurement, assuming no real change in hearing thresholds has occurred. It is also of great importance to use correct gender- and age-specific reference values when interpreting pure-tone threshold measurements. Several large-scale epidemiological studies have been conducted during recent years, and have provided us with reliable gender- and age-specific references. A method to take into account the age-related deterioration is presented. In this method, Z-score audiograms represent traditional thresholds in an age- and gender-independent way. At each frequency, the Z-score value is the number of standard deviation units that the threshold differs from the median value for the otologically normal population (ISO 7029).