Effects of SoundBite Bone Conduction Hearing Aids on Speech Recognition and Quality of Life in Patients with Single-Sided Deafness.

Objectives: To analyze the clinical application of SoundBite bone conduction hearing aids by assessing the improvement of speech recognition and the scores of the benefit scale questionnaire for patients with single-sided deafness (SSD). Design: Nine patients aged 24 to 61 years with SSD for more than 3 months were enrolled in this study. The patients could understand and repeat Mandarin and have good compliance with the study. The measurements were evaluated before and after one month of wearing hearing aids using the pure tone audiometry threshold, speech recognition in quiet and in noise, and the Glasgow Benefit Inventory (GBI) benefit scale score. Results: Pure tone audiometry results showed that the average hearing threshold of good ears and bad ears was 11.4 ± 2.6 dB HL and 89.9 ± 6.4 dB HL, respectively. The average hearing threshold of bad ears after wearing hearing aids was 23.5 ± 9.0 dB HL. Statistical analysis showed that the hearing improvement for the bad ears after wearing hearing aids was significant. The speech audiometry results showed that the disyllable word recognition score of the bad ears in quiet increased significantly at 50 dB SPL by 40 ± 12 percentage points and at 65 dB SPL by 71 ± 15 percentage points. As for the speech recognition in noise, when the signal sound came from the bad ear side and the noise from the good ear side (SSSDNAH), the speech recognition score (SRS) significantly increased by 17 ± 6 and 9 ± 4 at a signal-to-noise ratio (SNR) of -2 dB and -5 dB, respectively, after wearing the hearing aids. When the signal sound came from the front of the patient and the noise from the bad ear side (S0NSSD), the SRS scores were reduced by 5 ± 5 and 7 ± 5 percentage points at SNR equal to -2 dB and -5 dB, which was significantly different from that before wearing the hearing aids. When the signal and noise both came from the front of the patients (S0N0), the SRS was not significantly increased by 5 ± 4 percentage points at SNR equal to -2 dB compared to before wearing hearing aids. However, the SRS was significantly increased by 5 ± 2 percentage points at SNR equal to -5 dB compared to before wearing hearing aids. The average total GBI score was 31 ± 12 for the nine patients, with an average score of 32 ± 10, 31 ± 8, and 30 ± 7 for general conditions, social support, and physical health, respectively. The results of the questionnaires showed that patients' quality of life was improved after wearing SoundBite bone conduction hearing aids. Conclusions: SoundBite bone conduction hearing aids are a good choice for patients with SSD, as it could improve the speech recognition ability of patients both in a quiet and noisy environment and improves the quality of life after wearing hearing aids.

l-Thyroxine does not prevent immunemediated sensorineural hearing loss in autoimmune thyroid diseases.

OBJECTIVE: This is the first report dealing with immune-mediated inner ear disease (IMIED) hearing loss in a group of patients affected with autoimmune thyroid disease (AITD), whose treatment required corticosteroids, despite being treated with levothyroxine. Immunopathology linking the inner ear and the thyroid gland is also presented. PATIENTS: A total of 220 patients were selected with sensorineural hearing loss (SNHL) of causes other than presbycusis. Audiometry was performed and pure tone average was calculated before and after treatment with corticosteroids. RESULTS: Eighty-four (84) patients had SNHL of autoimmune origin, and 15 patients were diagnosed with AITD (Hashimoto's disease). Bilateral hearing loss was observed in 10 patients (66.5%). Sudden sensorineural hearing loss was the most frequent clinical form of presentation. Nine patients showed a hearing recovery greater than 10dB after corticosteroid treatment. CONCLUSIONS: Acquired hypothyroidism is thought to affect hearing due to different mechanisms. Although specific hormonal therapy may improve peripheral or central auditory disorders associated with hypothyroidism, the presence of IMIED in AITD patients requires another approach. Altered immune regulatory mechanisms involving Treg cells and CD4+CD45RO cells have been suggested in patients with AITD and IMIED. In the present study, although all the patients with hypothyroidism and subclinical hypothyroidism were being treated with levothyroxine, immune-mediated hearing loss was observed. Therapy with corticosteroids could achieve hearing recovery. Since inner ear and thyroid gland share possible antigen targets, we highlight the existence of IMIED in AITD patients and the importance of implementing appropriate therapy with corticosteroids.

Unilateral hearing loss following shunt placement for normal pressure hydrocephalus with a unilateral patent cochlear aqueduct.

Hearing loss in patients who have undergone shunt placement for a hydrocephalus is perhaps an underestimated complication rather than a rare event. There appears to be a correlation between the occurrence of hearing loss and patient characteristics consistent with excessive drainage of cerebrospinal fluid (CSF) and patent cochlear aqueduct (CA). We present the case of a 77-year-old man with unilateral dominant sensorineural hearing loss after a shunt placement for normal pressure hydrocephalus (NPH) combined with a patent cochlear aqueduct. Based on our experience and a review of the literature, we suggest an early restoration of the reduced CSF pressure using a programmable valve as a treatment strategy, which might prevent the persistent hearing loss.

Giant cell reparative granuloma of the temporal bone successfully resected with preservation of hearing.

OBJECTIVE: To describe a case of giant cell reparative granuloma of the temporal bone which extended into the middle-ear cavity, and which was successfully treated surgically via a transmastoid approach, with hearing preservation. CASE: A 37-year-old man presented with a one-year history of right-sided hearing loss, complicated by a three-month history of otalgia and a sensation of aural fullness. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumour lesion in the right temporal bone. The diagnosis was confirmed by biopsy from the mastoid lesion. INVESTIGATION AND INTERVENTION: Pure-tone audiometry, computed tomography and magnetic resonance imaging were conducted, followed by total resection. RESULT: The giant cell reparative granuloma of the temporal bone was completely resected, with preservation of hearing. CONCLUSION: Although this patient's giant cell reparative granuloma of the temporal bone extended into the middle-ear cavity, total resection was achieved, with preservation of hearing. To the best of our knowledge, hearing preservation following resection of giant cell reparative granuloma of the temporal bone has not previously been reported.

Long-term hearing outcome after retrosigmoid removal of vestibular schwannoma.

Although many investigators have reported the hearing function in the immediate postoperative period in patients with vestibular schwannoma (VS), little is known about the long-term outcomes of the postoperative hearing. The aim of this study was to analyze the long-term hearing outcomes at a mean follow-up of 5 years in patients with unilateral VS treated via the retrosigmoid approach. Twenty-four patients with immediate postoperative serviceable hearing who underwent repeated audiogram or phone interview were included in this study. During the mean follow-up period (68.8 ± 30.2 months, range 14-123 months), serviceable hearing was preserved in 20 out of the 24 patients (83%). Pure tone average (PTA) was reevaluated within 6 months in seven patients. In the two patients whose PTA deteriorated ≥ 5 dB in 6 months after surgery, their PTA worsened ≥ 15 dB compared to the immediate postoperative PTA. In the remaining five patients whose PTA deteriorated < 5 dB in 6 months after surgery, PTA was maintained within a 15-dB deterioration at the final follow-up (p = 0.04, Fisher's exact test). According to Kaplan-Meier survival plots, the 5-year or 7-year preservation rate of serviceable hearing was 86.2% or 71.8%, respectively. Further study will be needed to clarify the mechanism underlying the long-term decline of serviceable hearing; however, the deterioration of PTA in the early postoperative period may help to predict the long-term outcomes of hearing.

Bevacizumab treatment for vestibular schwannomas in neurofibromatosis type two: report of two cases, including responses after prior gamma knife and vascular endothelial growth factor inhibition therapy.

OBJECTIVES: Vestibular schwannomas are the hallmark of neurofibromatosis type two. They are difficult to treat, due to their bilateral presentation and the quest for hearing preservation. Our report describes a new treatment approach in this clinical scenario. CASE REPORT: We report two cases which confirm that bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor, causes regression of vestibular schwannomas in patients with a previous history of gamma knife radiosurgery or failed treatment with another form of vascular endothelial growth factor targeted therapy. CONCLUSION: In 2009, Plotkin et al. reported the volumetric response of vestibular schwannomas to bevacizumab treatment, both in untreated patients and in patients previously treated with erlotinib, an epidermal growth factor receptor inhibitor. The presented cases support the use of bevacizumab to treat vestibular schwannomas. Given the extremely slow growth of these tumours, we note the rapidity of volume reduction following bevacizumab therapy.