Search results for " Pure-Tone"

Confirmation of PDZD7 as a Nonsyndromic Hearing Loss Gene.

2016

Objective PDZD7 was identified in 2009 in a family with apparent nonsyndromic sensorineural hearing loss. However, subsequent clinical reports have associated PDZD7 with digenic Usher syndrome, the most common cause of deaf-blindness, or as a modifier of retinal disease. No further reports have validated this gene for nonsyndromic hearing loss, intuitively calling correct genotype-phenotype association into question. This report describes a validating second case for biallelic mutations in PDZD7 causing nonsyndromic mild to severe sensorineural hearing loss. It also provides detailed audiometric and ophthalmologic data excluding Usher syndrome in both the present proband (proband 1) and the…

KTP-laser stapedotomy with a self-crimping, thermal shape memory Nitinol SMart piston: 1 year follow-up results: how we do it.

2008

Evidence for a spatial bias in the perception of sequences of brief tones

2013

Listeners are unable to report the physical order of particular sequences of brief tones. This phenomenon of temporal dislocation depends on tone durations and frequencies. The current study empirically shows that it also depends on the spatial location of the tones. Dichotically testing a three-tone sequence showed that the central tone tends to be reported as the first or the last element when it is perceived as part of a left-to-right motion. Since the central-tone dislocation does not occur for right-to-left sequences of the same tones, this indicates that there is a spatial bias in the perception of sequences. © 2013 Acoustical Society of America.

Hearing loss in Fabry disease: data from the Fabry Outcome Survey

2006

Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Questionnaires were completed by 566 Fabry patients, of whom 316 reported ear-related symptoms. Pure-tone audiograms from 86 patients, performed before starting enzyme replacement therapy, were analysed and compared with age- and sex-specific normal values (International Organization for Standardization, ISO 7029). When compared to an age-matched population (ISO 70…

Management of intralabyrinthine schwannomas

2006

Abstract Objectives Our protocol to manage the intralabyrinthine schwannoma (ILS). Methods Retrospective chart review of 7 consecutive patients managed for ILS. Results Five patients underwent surgical removal of the lesions and none experienced significant complications or recurrent disease. One patient refused surgical treatment and was closely followed by serial MRI scans with no signs of tumor growth. One patient is presently managed conservatively due to a good hearing. Conclusions Diagnosis of ILS is based on high resolution MRI scans and should be included in the differential diagnosis of patients investigated for cochleovestibular symptoms. Treatment modality of ILS is controversial…

Cochlear origin of early hearing loss in vestibular schwannoma.

2007

Objective: To test whether early hearing loss (HL) is cochlear in origin in patients with vestibular schwannoma (VS). Study Design: Retrospective case review in an academic tertiary referral center. Methods: A group of 19 VS patients with normal/symmetrical hearing and a group of 20 VS patients with mild HL (threshold at any tested frequency better than 45 dB HL) on the tumor ear side. Differences of the amplitudes of the distortion products of otoacoustic emissions (DPOAEs) between the tumor ear and the nontumor ear were studied at frequencies of 1, 1.4, 2, 2.8, and 4 kHz. The Wilcoxon test was used to compare the ears for both groups and to test for possible differences in tumor size betw…

Static posturography with dynamic tests. Usefulness of biomechanical parameters in assessing vestibular patients.

2012

Abstract Introduction and objective Posturography allows us in evaluating postural control. This study showed the posturographic parameters that were useful for assessing the functional ability to maintain balance in our sample of vestibular patients. Material and methods Of a total of 89 patients, 59 were healthy subjects and 30 had a peripheral vestibular disorder. The subjects were studied using the posturographic NedSVE/IBV system, combining static (Romberg) and dynamic (stability limits and rhythmic weight shifts) tests. We then compared the measurements found in the groups. Results Normal subjects showed significantly lower oscillations than our patients in all of the posturographic p…

Increased amplitudes of distortion product otoacoustic emissions in patients with unilateral acoustic neuroma.

2004

We present a case series of 4 patients with a unilateral acoustic neuroma and increased amplitudes of the distortion products of otoacoustic emissions (DPOAEs) at the low- and middle- frequency bandwidth on the involved side compared to the uninvolved side despite a 28-dB hearing level (HL) worse (compared to the uninvolved side) pure-tone hearing threshold average for standard audiometric frequencies between 1 and 6 kHz at the involved side. In 3 of these patients, 2 with an inferior vestibular nerve origin of the acoustic neuroma and one in whom the nerve of origin could not be unequivocally defined, the tumor was extending extrameatally. One patient had a purely intrameatal acoustic neur…

Intratympanic gentamicin treatment after endolymphatic sac surgery.

2005

Interval treatment with up to three intratympanic gentamicin injections once weekly effectively controlled vertigo while preserving hearing in patients with Ménière's disease and recurrent or resistant vertigo after saccotomy.Recurrent or resistant incapacitating vertigo may occur after endolymphatic sac surgery (saccotomy) in patients with Ménière's disease. In these patients, revision saccotomy, vestibular nerve section or labyrinthectomy are the established treatment options. We advocate a once-weekly application of intratympanic gentamicin (12 mg) as an effective alternative in this group of patients.Five patients (age range 39-65 years) with definite Ménière's disease according to the …

Transient evoked otoacoustic emissions in vestibular neuritis.

2007

Objectives: Partial or total degeneration of the vestibulocochlear anastomosis at its takeoff from the saccular ganglion and regenerating efferent neural buds under the cochlear outer hair cells (OHCs) have been found in the temporal bones of human patients with a history of vestibular neuritis (VN). We sought to test whether VN has any functional impact on the ipsilateral OHCs by means of transient evoked otoacoustic emission (TEOAE) testing. Methods: We retrospectively analyzed prospectively collected TEOAE data of 28 patients (19 female, 9 male; age range, 21 to 60 years; median age, 42.5 years). The pure tone air conduction hearing thresholds at each standard audiometric frequency (0.12…