Cochlear Implantation in Ménière's Disease.

IMPORTANCE: Though the effects of cochlear implantation (CI) on patients with hearing loss has been well described, its effect on the auditory and vestibular symptoms in patients with Ménière's disease (MD) has not been firmly established. OBJECTIVES: To determine whether CI in patients with MD improves hearing performance as measured by standard post-CI audiometric testing and to describe the impact of CI on subjective auditory and vestibular quality-of-life measures. DESIGN, SETTING, AND PARTICIPANTS: Retrospective review of all patients with MD 18 years or older who met diagnostic criteria for definite MD and underwent CI at Wake Forest University Baptist Medical Center, a tertiary care academic medical center, from 2000 through 2012. INTERVENTIONS: Patients were invited to answer questions regarding their current hearing ability, subjective perception of symptoms, and functional status related to their MD before and after CI. Patient records were reviewed for pertinent information. Results were then analyzed for statistical significance. MAIN OUTCOMES AND MEASURES: Pre-CI and post-CI sentence testing scores, frequency and severity of MD vestibular and auditory symptoms via the MD Functional Level Scale (FLS), and hearing quality of life via the Hearing Handicap Index were reviewed. RESULTS: Eleven CIs were performed in 10 patients. Mean long-term follow-up was 41 months. The mean age at first implantation was 64 years. The mean sentence testing scores in quiet improved from 22.8% before CI to 77.0% after CI (P < .001) at the most recent follow-up. Mean (SD) pre-CI and post-CI MD-FLS vestibular scores were similar: 3.9 (1.9) and 3.4 (1.9) (P = .52). CONCLUSIONS AND RELEVANCE: Patients with MD who undergo CI are capable of achieving substantial receptive communication improvement comparable to the gains experienced by patients without MD. Implantation seems to neither adversely alter the natural history of vestibular function nor notable exacerbate auditory symptoms.

A comparison of tympanometry with 226 Hz and 1000 Hz probe tones in children with Down syndrome.

BACKGROUND: For children with Down syndrome, the incidence of hearing loss may be as high as 78% [1], therefore the American Academy of Pediatrics recommends regular screening for the presence of hearing abnormalities. Tympanometry is used as an indication of middle ear pathology. In our experience, Down's patients' tympanograms do not always correlate with otoscopic findings. Down's patients have joint laxity, small ear canals, anterior tympanic membrane orientation and softer tissue composition, all factors thought to affect tympanogram results in infants. Because the use of the 1000 Hz tympanometry is widely recognized as standard procedure in the evaluation of infants aged 0-6 months, we propose it may have greater reliability in testing patients with Down syndrome. OBJECTIVE: Compare the results of visual inspection of the tympanic membrane by a Pediatric Otolaryngologist to the results of tympanometry at traditional probe tone (226 Hz) and at the infant probe tone (1000 Hz). METHODS: Institutional Review Board - approved prospective study of 26 subject-ears in patients with Down syndrome aged 6 months-18 years but recent stable middle ear/Eustachian tube function using physical examination and tympanometric probe tones at 226 Hz and 1000 Hz. Subject-ears were examined with record of "clear of effusion," showed the presence of "fluid," or were to be "excluded." Blinded to ear exam results, tympanometry was then completed with record of which Jerger classification tympanogram was found at each frequency. RESULTS: Although the sensitivity of each test was 1, the specificity of the 1000 Hz tympanometry (100%) in this study was markedly improved compared to the specificity of the 226 Hz tympanometry (71%) (p=0.016). CONCLUSIONS: This pilot study demonstrated evidence that tympanometry in children with Down syndrome may be more reliable at 1000 Hz than at 226 Hz in detecting the presence of middle ear effusion beyond infancy. Use of the 1000 Hz probe tone yielded fewer false positives for disease (type B tympanograms in the setting of absent middle ear disease). Further studies of a larger patient population are needed to corroborate these results.