Hearing Loss

Vibrant Soundbridge System: Round window stimulation with the vibroplasty technique

March 31, 2011     Willis S.S. Tsang, MD, Terence K.C. Wong, MSc, and Michael C.F. Tong, MD
article

Glomus jugulare

January 1, 2011     Jeffrey D. Suh, MD, Ashley E. Balaker, MD, Brian D. Suh, MD, and Keith E. Blackwell, MD
article

Treatment-refractory autoimmune sensorineural hearing loss: Response to infliximab

January 1, 2011     Yi-Chun Liu, MD, Rene Rubin, MD, and Robert T. Sataloff, MD, DMA, FACS
article

Abstract

We conducted a retrospective study to assess the effects of infliximab, a chimeric monoclonal antibody, on hearing in patients with autoimmune sensorineural hearing loss who had previously not responded to steroid therapy and/or treatment with other immunosuppressive drugs such as methotrexate and cyclophosphamide. We reviewed the rec-ords of 8 such patients. Our objective measures of outcome were pure-tone averages at 500, 1,000, 2,000, and 3,000 Hz and speech discrimination scores. At the completion of treatment, no patient exhibited a positive response to infliximab therapy by objective measurements, and only 1 patient (12.5%) reported subjective improvement.

Malleus head fixation

December 17, 2010     Julia Vent, MD, PhD and Dirk Beutner, MD
article

The effects of glutathione enhancement on sensorineural hearing loss

August 31, 2010     Robert T. Sataloff, MD, DMA, FACS, Therese Bittermann, MD, Linda Marks, RN, Deborah Lurie, PhD, and Mary Hawkshaw, BSN, RN, CORLN
article

Abstract

Previous research has demonstrated the benefits of anti-oxidant treatment in the prevention of hearing loss in animals. Our study tested the effects of an undenatured whey protein supplement rich in glutathione on human patients with hearing loss. Over an average of 36 months, 30 patients with hearing loss and who had data sufficient for analysis were treated with a glutathione supplement and were compared with 30 retrospective controls selected from the same otologic patient population. Patients were followed using regular hearing tests. Linear regression analysis was used to determine whether study group, baseline audiometric score, time followed, and autoimmune etiology modified the rate of hearing loss. Treatment with the glutathione supplement failed to modify significantly the progression of hearing loss in the treated population. The baseline audiometric score was most predictive of the final audiometric score (p < 0.0005). Although glutathione supplementation was not shown to be helpful in slowing hearing loss in the patients studied, our research model proved valid as it demonstrated an overall decline in hearing in both the treated and control groups over time of sufficient magnitude to permit detection of a treatment effect if a substantial effect had occurred. We suggest that this model be applied to future studies investigating the effects of antioxidants on hearing loss.

Vibrant Soundbridge implantable hearing device: Critical review and single-surgeon short- and long-term results

August 31, 2010     Charles M. Luetje, MD, FACS, Sandra A. Brown, MA, CCC-A, and Robert D. Cullen, MD
article

Abstract

We conducted a retrospective descriptive study of a series of 31 consecutively presenting patients who had been implanted with the Vibrant Soundbridge middle ear hearing device. All implantations had been performed by the senior author. Three of these patients had undergone bilateral implantation, and 4 others had undergone subsequent explantation and reimplantation in response to known or suspected device failure, giving us a total of 34 ears and 38 implants. Our goal was to ascertain short- and long-term outcomes as measured by conventional audiometry (pure-tone average at 1 to 6 kHz) and long-term benefit as defined by the use or nonuse of the device. We found that at the initial activation session 2 months postoperatively, the average hearing thresholds were within 3 dB of the preoperative thresholds in all 34 ears and all 38 implants. The mean short-term gain at activation in the 38 implants was 28.1 dB. Nineteen patients (20 ears) were available for long-term evaluation, with the length of follow-up ranging from less than 1 year to 11 years (mean: 7.3). Of these 20 ears, 9 demonstrated further gain (mean: 10.8 dB) despite any natural hearing deterioration; of the remaining 11 ears, gain was unchanged in 2, diminished in 7 (mean: -3.6 dB), and gain data were unavailable in 2. In the final analysis, there were 20 user ears and 10 nonuser ears; 4 ears were lost to all follow-up. We conclude that direct-drive hearing with the Vibrant Soundbridge middle ear hearing device is beneficial and provides sustained audiometric gain. Factors that have a significant impact on patient use or nonuse include difficulty in obtaining audiologic support and the direct and indirect costs of the device. Without audiologic or financial support, some patients may choose to become nonusers and to either switch to conventional hearing aid amplification or become apathetic about hearing improvement.

Hearing impairment in persons with the hemoglobin SC genotype

June 30, 2010     Paul A. Onakoya, FWACS, Onyekwere G.B. Nwaorgu, FWACS, and Wuraola A. Shokunbi, FWACP
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Abstract

The hemoglobin (Hb) SC genotype is seen in persons who have inherited the gene for hemoglobin S from one parent and the gene for hemoglobin C from the other. Some people with this genotype develop Hb SC disease, a variant of sickle cell disease. Hb SC disease, a compound heterozygous condition, is the most common of the hemoglobinopathies and the least severe, although it is still serious. One of the documented complications of the presence of the Hb SC genotype is sensorineural hearing loss (SNHL). We conducted a prospective case-control study of 43 subjects, aged 15 to 65 years, who had the Hb SC genotype to determine the incidence of SNHL and to determine if the hearing loss in these subjects was correlated with sex or age. Our control group was made up of 100 generally healthy, sex- and age-matched subjects with the normal Hb AA genotype. SNHL was defined as a loss of more than 25 dB HL at two or more frequencies in the same ear or at one or more frequencies in both ears. We found that SNHL was present in 12 of the 43 subjects (27.9%) in the Hb SC group (17 of 86 ears [19.8%]) and in 17 of the 100 subjects (17.0%) in the Hb AA group (21 of 200 ears [10.5%]; the difference between the two groups was not statistically significant (χ2 = 1.589; p = 0.105). We found that in the Hb SC group, SNHL was more common among females than males (38.5 vs. 11.8%), although the difference was not quite significant statistically (χ2 = 2.435; p = 0.056); in the Hb AA group, the incidence was fairly equal-15.4 and 18.8%, respectively (χ2 = 0.033; p = 0.427). Therefore, we conclude that the hearing loss in the subjects of this study was not correlated with the presence of the Hb SC genotype in either sex. In terms of age, SNHL was significantly more common in subjects aged 41 to 65 years than in those aged 15 to 40 years in both genotype groups. In the Hb SC group, SNHL was present in 4 of the 33 younger subjects (12.1%) and in 8 of the 10 older subjects (80.0%) (χ2 = 14.354; p < 0.001). In the Hb AA group, the corresponding figures were 7 of 85 (8.2%) and 10 of 15 (66.7%) (χ2 = 26.840; p < 0.001). Therefore, we conclude that the hearing loss in the subjects of this study was a function of age and was not associated with the presence of the Hb SC genotype.

Acute ototoxicity from a single infusion of oxaliplatin

May 31, 2010     Narinder K. Malhotra, MD, Rizwan Aslam, DO, Sydney P. Lipman, MD, and Vicki J. Bilski, MS, CCC-A
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Abstract

Ototoxicity secondary to the administration of the chemotherapeutic agent oxaliplatin is uncommon. We report a case of unilateral hearing loss that occurred immediately after the infusion of a single dose of oxaliplatin in a 70-year-old woman who was undergoing postoperative chemotherapy for rectal adenocarcinoma. To the best of our knowledge, no such case has been previously reported in the literature. The hearing loss in our patient was severe enough to limit her daily activities, and she experienced only minimal improvement during the next 2 years of follow-up.

Dissection of the incus long process by the chorda tympani nerve

March 31, 2010     Julia Vent, MD, PhD and Dirk Beutner, MD
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Cerebellopontine angle ganglionic hamartoma: Case report

March 1, 2010     Sidrah M. Ahmad, BS, Fernando Garcia, MD, and Robert T. Sataloff, MD, DMA, FACS
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Abstract

Cerebellopontine angle (CPA) ganglionic hamartomas are rare. We present a case of a 49-year-old woman who presented with left-sided hearing loss of more than 4 years' duration and vertigo since childhood. Magnetic resonance imaging revealed a mass on the intracanalicular segment of cranial nerve VIII on the left side, consistent with acoustic schwannoma. She opted for observation of the tumor. The patient returned 21 months after the initial evaluation with new-onset left facial weakness. At this time, a working diagnosis of facial nerve neuroma was made. The lesion was excised, and pathology confirmed a diagnosis of left-sided CPA ganglionic hamartoma. Otolaryngologists should be familiar with this uncommon tumor and include it in the differential diagnosis of CPA lesions.

HLA-B27-associated bilateral Ménière disease

March 1, 2010     Shamila G. Rawal, MD, Kunal H. Thakkar, MD, Kasra Ziai, BS, Peter A. Santi, MD, and Hamid R. Djalilian, MD
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Abstract

We describe 2 cases of bilateral Ménière disease with features resembling autoimmune inner ear disease in patients who were found to be carriers of human leukocyte antigen (HLA) B27. For immunohistochemical analysis, mouse inner ear sections were used as the tissue substrate for reaction with serum. Both patients demonstrated an increased immunofluorescence reaction compared with a normal control. We suggest that an antibody-mediated mechanism may be responsible for HLA-B27-associated Ménière disease.

Myxoma of the temporal bone: An uncommon neoplasm

March 1, 2010     Deepika Sareen, MBBS, Ashwani Sethi, MS, Sumit Mrig, MBBS, Sonu Nigam, MD, and A.K. Agarwal, MS
article

Abstract

We report the case of an 11-year-old girl who presented with a soft-tissue mass that filled the left external auditory canal and a discharge that resembled chronic suppurative otitis media. The patient underwent mastoid exploration with complete excision of the mass. Findings on the excision biopsy were consistent with a myxoma of the temporal bone. At follow-up 2 years postoperatively, the patient remained disease-free. To the best of our knowledge, this is only the 12th case of a myxoma of the temporal bone to be reported in the English-language literature.

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