These results indicate that the processing of pendrin mutant protein is determined by mutant specific mechanisms, and that a mutant specific method would be required to rescue the conformational defects of each folding mutant.

T inniTus can be subjective, i.e., when the sound is perceived by the individual alone, or objective, i.e., when an observer can recognize the tinnitus sound of the patient.2 Subjective tinnitus comes from the inner ear, ascending auditory pathway, or auditory/nonauditory cortical regions. 9,[22][23][24][26][27][28]30 The origin of objective tinnitus is vascular or nonvascular. Of them, vascular objective tinnitus is also known as pulsatile tinnitus (PT), featuring perception of a pulse-synchronous or rhythmical sound. obJective A dominant sigmoid sinus with focal dehiscence or thinning (DSSD/T) of the overlying bony wall is a commonly encountered, but frequently overlooked, cause of vascular pulsatile tinnitus (VPT). Also, the pathophysiological mechanism of sound perception in patients with VPT remains poorly understood. In the present study, a novel surgical method, termed transmastoid SS-reshaping surgery, was introduced to ameliorate VPT in patients with DSSD/T. The authors reviewed a case series, analyzed the surgical outcomes, and suggested the pathophysiological mechanism of sound perception. The theoretical background underlying VPT improvement after transmastoid SS-reshaping surgery was also explored. methods Eight patients with VPT that was considered attributable to DSSD/T underwent transmastoid SS-reshaping surgery between February 2010 and February 2015. The mean postoperative follow-up period was 9.5 months (range 4-13 months). Transmastoid SS-reshaping surgery featured simple mastoidectomy, partial compression of the SS using harvested cortical bone chips, and reinforcement of the bony SS wall with bone cement. Perioperative medical records, imaging results, and audiological findings were comprehensively reviewed. results In 7 of the 8 patients (87.5%), the VPT abated immediately after surgery. Statistically significant improvements in tinnitus loudness and distress were evident on numeric rating scales. Three patients with preoperative ipsilesional low-frequency hearing loss exhibited postoperative improvements in their low-frequency hearing thresholds. No major postoperative complications were encountered except in the first subject, who experienced increased intracranial pressure postoperatively. This subsided after a revision operation for partial decompression of the SS. coNclusioNs Transmastoid SS-reshaping surgery may be a good surgical option in patients with DSSD/T, a previously unrecognized cause of VPT. Redistribution of severely asymmetrical blood flow, reinforcement of the bony SS wall with bone cement to reconstruct a soundproof barrier, and disconnection of a problematic sound conduction route via simple mastoidectomy silence VPT.

Forceful contractions of neck and jaw muscles have consistently been shown to modulate tinnitus and can be used to screen patients who are responsive to somatic stimulation and, therefore, optimal candidates for somatosensory-based treatment. To identify the factors associated with somatic modulation of tinnitus, 163 patients underwent 19 neck and jaw maneuvers after an extensive physiological and audiological profile was compiled. Overall, tinnitus was modulated in 57.1% of ears tested. Unilateral tinnitus showed greater prevalence of modulation. Neck maneuvers generally decreased tinnitus loudness, whereas jaw maneuvers increased loudness. Female gender and buzzing tinnitus were associated with a high prevalence of modulation and a decrease in tinnitus loudness. Loud tinnitus and low-pitched tonal tinnitus were associated with exacerbation of the condition as a result of somatic testing. Use of these characteristics to select optimal candidates for somatosensory-based tinnitus therapies may be essential for the development of an effective approach for tinnitus treatment.

Background and Objectives: Tinnitus frequency-filtered music therapy aims to restore lateral inhibition to reverse tonotopic reorganization in the auditory cortex. Although the tinnitus-relieving effect of this therapy has been investigated, the results remain controversial. We performed a prospective, randomized, controlled double-blind study to determine the tinnitussuppressing effect of tinnitus frequency-filtered music therapy. Subjects and Methods: The study included 90 participants who were randomly categorized into an experimental group that listened to tinnitus frequency-filtered music and a control group that listened to music from which a random frequency was removed. The Tinnitus Handicap Inventory (THI) score and measures of tinnitus loudness, daily awareness, and tinnitus-induced annoyance were evaluated at the initial visit and at 3 and 6 months (final follow-up). The rates of improvement in THI scores in the two groups were also recorded. Results: All measured variables showed significant improvement in both groups, except the matched tinnitus loudness and minimal masking level. However, no significant intergroup differences were observed in the amount of improvement in THI scores and any other variable. The rates of improvement in THI scores were higher in the control group at 3 and 6 months. Conclusions: Listening to tinnitus frequency-filtered music reduced tinnitus-induced handicaps; however, this approach was not significantly better than listening to music from which a random frequency was removed.

Objective: The aims of this multicenter study were to prospectively evaluate the prevalence of dead regions (DRs) in sudden sensorineural hearing loss (SSNHL) and compare the clinical characteristics and hearing outcomes of SSNHL according to the presence of DRs. Study Design: Prospective study. Setting: Multicenter study. Patients and Methods: The threshold-equalizing noise (TEN) test was prospectively performed on a total of 130 patients diagnosed with SSNHL. All patients received systemic steroid therapy and/or intratympanic steroid injection within 1 month after onset. Pure-tone audiograms and the TEN test were conducted before and after steroid treatment. Age, sex, side of affected ear, recurrence, onset of symptoms, presence of dizziness, and comorbid diseases were also collected. The prevalence of DRs in SSNHL and clinical factors related to the DRs were assessed. Hearing outcomes for SSNHL according to DRs were evaluated in 68 patients who followed a pure-tone audiogram. Results: The overall prevalence of one or more DRs in SSNHL evaluated using the TEN test was 20.8% (27/130 subjects) and the overall frequency-specific prevalence of DR was 6.7% (61/910 DRs). Although the DRs (+)and DR (−) groups had similar initial pure-tone thresholds, the DRs (+) group had significantly worse initial WRS compared to the DRs (−) group (p = 0.015). The presence of DRs was not associated with hearing recovery in a multivariate logistic regression model, but it was significantly associated with the degree of hearing gain in a multivariate linear regression model (p = 0.018). Conclusions: The presence of DRs can be considered one of the poor prognostic factors for SSNHL and the TEN test may contribute to assess the prognosis of SSNHL in clinical settings.