Serial audiometry in a clinical trial of AIED treatment.

OBJECTIVE: We analyzed pure-tone and speech audiometric results from a prospective trial of anti-inflammatory treatment of subjects with active autoimmune inner ear disease (AIED). We sought to characterize the pattern and size of the treatment effect as reflected in clinical audiometry and to identify audiometric predictors of response to steroid treatment of AIED. SUBJECTS: Adult participants demonstrated clinically established criteria for AIED (n = 116). Eligibility required audiometric evidence of active AIED as indicated by idiopathic sensorineural hearing loss with threshold elevations within 3 months of enrollment. METHODS: We evaluated audiometric changes after 4 weeks of treatment with pharmacologic doses (60 mg/day) of prednisone. We examined the relationship between audiometric pure-tone thresholds at baseline and changes in word intelligibility score (WIS) using parametric and nonparametric analyses. Magnitudes of change were assessed using independent or paired t-tests. Separate analyses were performed on subgroups that did or did not show improved WIS score with steroid treatment. RESULTS: Overall mean pure-tone averages improved from baseline to closeout of prednisone treatment in better hearing ears from 52.4 to 48.3 dB (p < .0001). Mean WIS improved in the better ear from 71.4% to 78.1% (p < .0001). Of pure-tone measures, only the six-tone average showed significant correlation with both the absolute improvements in WIS and with the percentage change in WIS after treatment. Individual frequencies at baseline showed no significant relationship with changes in WIS score after treatment. In 69 (59.5%) of 116 subjects, WIS improved (range, 2-80%) in the better ear. In these subjects, the baseline pure-tone thresholds and pure-tone averages correlated significantly and positively with improvement in WIS. CONCLUSIONS: Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

Improved speech perception in adult congenitally deafened cochlear implant recipients.

OBJECTIVE: To determine whether congenitally deafened adults achieve improved speech perception when auditory and visual speech information is available after cochlear implantation. STUDY DESIGN: Repeated-measures single subject analysis of speech perception in visual-alone, auditory-alone, and audiovisual conditions. SETTING: Neurotologic private practice and research institute. SUBJECTS: Eight subjects with profound congenital bilateral hearing loss who underwent cochlear implantation as adults (aged 18-55 years) between 1995 and 2002 and had at least 1 year of experience with the implant. MAIN OUTCOME MEASURES: Auditory, visual, and audiovisual speech perception. RESULTS: The median for speech perception scores were as follows: visual-alone, 25.9% (range, 12.7-58.1%); auditory-alone, 5.2% (range, 0-49.4%); and audiovisual, 50.7% (range, 16.5-90.8%). Seven of eight subjects did as well or better in the audiovisual condition than in either auditory-alone or visual-alone conditions alone. Three subjects had audiovisual scores greater than what would be expected from a simple additive effect of the information from the auditory-alone and visual-alone conditions alone, suggesting a superadditive effect of the combination of auditory-alone and visual-alone information. Three subjects had a simple additive effect of speech perception in the audiovisual condition. CONCLUSION: Some congenitally deafened subjects who undergo implantation as adults have significant gains in speech perception when auditory information from a cochlear implant and visual information by lipreading is available. This study shows that some congenitally deafened adults are able to integrate auditory information provided by the cochlear implant (despite the lack of auditory speech experience before implantation) with visual speech information.

Hearing changes after diagnosis in neurofibromatosis type 2.

OBJECTIVE: Describe the changes in hearing ability and progression of disease over time in subjects with neurofibromatosis Type 2 enrolled in a multicenter natural history study of vestibular schwannomas in neurofibromatosis Type 2. STUDY DESIGN: Retrospective clinical study. SETTING: International neurofibromatosis Type 2 tertiary care centers. PATIENTS: Study participants had a clinical diagnosis of neurofibromatosis Type 2, at least one untreated vestibular schwannoma, and were at least 5 years old. Sixty-three subjects (108 ears) with audiology data at either short-term follow-up (7 mo-2 yr) or long-term follow-up (3-5 yr) after diagnosis were examined in this study. MAIN OUTCOME MEASURES: Changes in four-frequency pure-tone average and speech discrimination score before any treatment intervention for both follow-up intervals. RESULTS: Within 2 years of the diagnosis of neurofibromatosis Type 2, 27% of the ears experienced a significant loss in pure-tone average relative to diagnosis, and 73% of the ears experienced no significant change in hearing. CONCLUSION: Newly diagnosed neurofibromatosis Type 2 patients who do not require immediate treatment of both vestibular schwannomas are likely to have stable hearing in the unoperated ear(s) for approximately 1 to 2 years.