A, Sentence recognition testing in quiet (at most recent follow-up). All sentence testing was performed in best-aided conditions both before and after cochlear Implantation (CI). The percentages listed on the y-axis represent the percent correct on AzBio and Hearing in Noise Test (HINT) sentence testing. The difference between the 2 groups was significant (P < .001). B, The Ménière’s Disease Functional Level Scale (MD-FLS) is a patient-administered 6-point scaling system that determines degree of functionality related to vestibular symptoms of Ménière’s disease. Data from 10 patients are included. The difference between the 2 groups, recorded at the most recent follow-up, was not significant (P = .52).
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Fife TA, Lewis MP, May JS, Oliver ER. Cochlear Implantation in Ménière’s Disease. JAMA Otolaryngol Head Neck Surg. 2014;140(6):535–539. doi:https://doi.org/10.1001/jamaoto.2014.550
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.
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.
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.
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.
Ménière’s disease (MD) is characterized by cochleovestibular dysfunction that manifests as fluctuating sensorineural hearing loss (SNHL), episodic vertigo, aural fullness, and tinnitus.1 Estimates of the prevalence of MD in the United States vary widely, but recent data suggest it is likely to be approximately 190 per 100 000.2 Patients with MD classically develop a unilateral, low-frequency SNHL; however, bilateral involvement may manifest in up to 50% of patients.2 As the disease progresses, severe SNHL may occur bilaterally in 1% to 6% of those diagnosed as having MD.2 Cochlear implantation (CI) has been approved by the Food and Drug Administration for rehabilitation of bilateral severe to profound SNHL since 1984, and as of 2011, more than 60 000 patients in the United States have undergone CI.3
A review of the available literature revealed that only a handful of English-language articles investigating CI in the setting of MD have been published.4-8 Most of these are single case reports and opinion articles and focus primarily on the effects of CI on hearing performance in patients with MD. Only 1 study has addressed the effects of CI on the nonhearing aspects of MD.4
Patients with severe to profound bilateral SNHL and poor word recognition capability due to MD stand to experience considerable benefit from CI. The objectives of this study are to (1) determine whether CI in patients with MD improves hearing performance as measured by standard CI audiometric testing and (2) describe the impact of CI on subjective auditory and vestibular quality-of-life measures.
This study is a retrospective review and was approved by the institutional review board at Wake Forest University Baptist Medical Center. All subjects provided written consent prior to participating in the study. Subjects were identified by querying the Otolaryngology and Audiology patient databases for all patients who have undergone CI since 2000. All CI recipients who also carried the diagnosis of MD were included in this group. Patient medical charts were then reviewed for pertinent information, including demographics, medical and surgical history, and audiometric data. Only patients with MD 18 years or older who met American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) diagnostic criteria1 for definite MD were included.
Eligible patients were contacted via letter and invited to answer a series of questions regarding their current hearing ability via the Hearing Handicap Inventory for adults (HHI),9 subjective perception of MD symptoms before and after CI, and functional status related to their MD before and after CI by the MD Functional Level Scale (MD-FLS).1 The HHI is a validated 25-question, hearing-specific, self-administered survey, which measures the social and emotional impact of hearing loss on a patient’s life. The HHI is scored from 0 to 100, with 100 representing a maximal hearing handicap. The MD-FLS is a patient-administered 6-point scaling system, designed by the AAO-HNS, which determines degree of functionality related to MD vestibular symptoms. A score of 1 represents no impact from vestibular dysfunction, whereas a score of 6 indicates severely disabling vestibular impairment. Hearing in Noise Test (HINT)10 and AzBio sentence testing scores,11 frequency and severity of vestibular and auditory symptoms, and hearing quality of life were compared before and after CI. Pertinent results were then analyzed for statistical significance.
Pre-CI and post-CI sentence testing took place in a sound booth with the speaker at 0° azimuth and with the patient 3 feet from the speaker. The signal was presented at 60 dB sound pressure leve (SPL). Pre-CI sentence testing was performed binaurally with the patients wearing a well-fit hearing aid in each ear. Post-CI, binaural (CI and contralateral hearing aid) and ear-specific hearing testing was performed. While most patients were initially tested with HINT sentences, all were eventually transitioned to using the AzBio sentence test, as the minimum battery for CI changed in 2011.
Of 154 adult, CIs performed at our institution between 2000 and 2012, 20 operations were performed in 17 patients with MD who met study inclusion criteria (Table 1). Five patients were eventually excluded because it was believed that their symptoms were more consistent with autoimmune vestibular dysfunction rather than MD. One patient with MD was excluded because the hearing loss in the implanted ear was attributed to sudden profound idiopathic SNHL rather than MD. One patient met inclusion criteria but was lost to follow-up immediately following implantation and was excluded from data analysis. One patient (patient 1) received bilateral CIs and another (patient 3) experienced a device failure and required explantation and reimplantation. Ultimately, this left 11 CIs in 10 patients. The male to female ratio was 1.5:1. The mean age at first implantation was 64 years (range, 40-84 years). Patients reported a mean of 22 years of MD symptoms prior to implantation (range, 7-40 years). All patients had severe or profound bilateral SNHL attributable to their MD.
Two of 10 patients (patients 2 and 6) underwent pre-CI ablative procedures for their vestibular symptoms (surgical or chemical labyrinthectomy), and another 2 (patients 3 and 6) had pre-CI endolymphatic sac decompressive surgery. Of those who had ablative procedures, none had the procedure performed on the implanted ear.
The better hearing ear was implanted in 6 of 10 patients. The better hearing ear was chosen for 2 patients because they had previously undergone a labyrinthectomy on the contralateral side. One patient received a second implant in what was previously the better hearing ear. In the remaining 3, the interaural difference in hearing ability was negligible and the side of implantation was determined by patient preference.
The Cochlear Nucleus 5 Contour Advance (Cochlear Corp) was used in 8 of 12 procedures, and the Cochlear Nucleus Freedom Contour Advance (Cochlear Corp) was used in 3. The Cochlear Nucleus 24 Contour (Cochlear Corp) was used in 1 operation. There were no reported intraoperative complications. One patient (patient 3) had a postoperative course complicated by device failure 1 year following implantation. The patient complained of episodic pain, headache, vertigo, and “electrical shocks” every time her cochlear implant was activated. After further investigation, it was revealed the patient’s problems began following hip surgery, where monopolar electrocautery was used. Device explantation and reimplantation were performed with good result.
Preoperative and postoperative audiograms and sentence testing (HINT and AzBio) were available for all 10 patients and compared. Mean long-term follow-up was 41 months, and follow-up sentence testing scores are reported (Table 2). Mean sentence testing improved from 22.8% before CI to 77.0% after CI (P < .001) at the most recent follow-up (Figure, A).
Patient questionnaires were sent to all 10 patients and all were returned (response rate, 100%). Results from the postoperative HHI demonstrated a mean (SD) total score of 55.8 (25.3). Although patients reported over an 8-fold reduction in the number of annual vertigo episodes before CI (P = .09), the mean (SD) pre-CI and post-CI MD-FLS vestibular scores were similar: 4.0 (2.0) and 3.4 (2.1), respectively (P = .52) (Figure, B).
Three patients reported persistent hearing fluctuation in their implanted ear and 2 reported hearing fluctuation in their nonimplanted ear. Seven patients reported persistent tinnitus after implantation (2 in the implanted ear alone, 2 in the nonimplanted ear alone, and 3 in both). All but 1 patient (patient 8) with tinnitus rated their tinnitus as the “same” or “better” after implantation. Five patients reported persistent aural fullness after implantation (1 in the implanted ear alone, 2 in the nonimplanted ear alone, and 2 in both). All but 1 patient (patient 3) with aural fullness rated their perception of the symptom as the “same” or “better” after implantation. The patient with worsened tinnitus and the patient with worsened aural fullness both reported these findings in the nonimplanted ear.
This study offers additional evidence that CI can provide substantial hearing rehabilitative benefit for patients with severe to profound SNHL attributable to MD. Furthermore, CI does not appear to negatively influence the vestibular and nonhearing symptoms of MD.
On the basis of currently published data, patients with MD who undergo CI may obtain hearing performance outcomes similar to cochlear implant recipients who have other more common acquired forms of severe to profound SNHL.12 There are, of course, multiple factors that predict how an individual will perform following CI, such as duration of deafness, age at implantation, motivation, and primary mode of communication, to name a few.13
In 2004, Firszt et al14 reported on a group of 78 unilateral cochlear implant recipients who underwent sentence testing. At 60 dB SPL, average HINT scores were 73%.14 In a 2008 study, Gifford et al15 reported that in a cohort of 156 individuals with postlingual deafness, up to 71% achieved greater than 85% accuracy on HINT sentences in quiet. Our results compare favorably with these numbers, suggesting that patients with MD who undergo CI can expect a similar hearing result as patients without MD. The HHI has been used previously to assess patients’ perception of hearing ability following CI in several studies.12,16-18 Looking at 29 patients, Vermiere et al12 reported a mean post-CI HHI score of 48, comparable to our data.
In 2003, Lustig et al4 reviewed 9 patients with severe to profound bilateral SNHL attributable to MD who underwent CI. Their study reported a statistically significant improvement in hearing based on hearing testing at 1 year, which continued to improve with time. Furthermore, they also noted that patients with MD appeared to outperform a similar cohort of adults with postlingual deafness without MD on hearing performance measures.
The ability of Lustig and colleagues4 to draw conclusions on the nonhearing symptoms of post-CI patients with MD was limited owing to the uniform absence of active preimplant vestibular symptoms in their sample. This study also reported the persistence of fluctuating aural fullness in 3 patients and tinnitus in 5 patients after CI. Two patients maintained that they were still having alterations in the hearing of their implanted ear.
Hearing fluctuation persists even with electrical stimulation of the cochlea for a certain segment of MD cochlear implant recipients, as one-third of patients subjectively noted hearing performance fluctuation in the present study. This fluctuation was also revealed on audiometric testing. However, these changes were not severe, and the fluctuations were easily overcome by minor programming modifications of the cochlear implant, resulting in excellent long-term overall stability in sentence testing scores.
On the basis of our analysis, CI does not appear to have any notable negative effect on the aural fullness, tinnitus, or vestibular symptoms experienced by patients with MD. Cochlear implantation has been advocated by several authors as a treatment for tinnitus in patients with hearing loss.19,20 The theory behind this intervention is that CI addresses the changes in neural activity caused by reduced auditory input, thought to be responsible for tinnitus. In a prospective study of 142 patients, Amoodi et al20 reported that CI exerted a significant suppressive effect on tinnitus in 66% of their population (including 7 patients with deafness attributed to MD).
Regarding vestibular symptoms, it is encouraging that the introduction of a current emitting electrode within the cochlear microenvironment does not induce vertiginous symptoms in patients with a tendency toward endolymphatic hydrops, as vestibular dysfunction has been reported in up to one third of cochlear implant recipients.21 This finding is most likely a result of the natural history of vertigo symptoms in longstanding end-stage MD (one of relative resolution). The known histologic changes of intracochlear fibrosis after standard implantation could conceivably affect the endolymphatic space associated with the vestibular end-organs, since the cochlear endolymphatic space may be constricted secondary to adjacent fibrotic changes, making the vestibular endolymphatic space more vulnerable to endolymphatic hydrops. However, this theoretic pathophysiology is not supported by these data, and CI did not seem to appreciably degrade vestibular function in this sample.
Patients with MD with disabling vestibular symptoms may undergo ablative procedures (chemical labyrinthectomy with gentamycin or vestibular neurectomy) to alleviate these symptoms. Both procedures are effective,22 and their use in patients with intractable vertigo has been advocated. However, there is concern that these interventions may have an impact on the success of subsequent CI. Case reports have demonstrated successful hearing rehabilitation with CI after surgical labyrinthectomy,23,24 and similar results have been reported after intratympanic gentamycin therapy.5 Our cohort included 2 (patients 2 and 6) who underwent such procedures in the contralateral ear. Nevertheless, labyrinthectomy, either surgical or pharmacologic, should not be a contraindication to CI.
With current CI efforts aimed at preserving the intracochlear anatomy as much as possible to preserve residual hearing, future investigations may also look at how preservation of residual hearing (by using more forgiving electrode arrays and insertion techniques) may affect the overall symptom spectrum in MD cochlear implant recipients.
This study has several limitations. First, this is a retrospective review with a small sample size. However, given the low prevalence of bilateral MD culminating in audiometric CI candidacy, it would prove difficult to gather enough subjects into the study to adequately power it. The retrospective nature of the patient surveys also likely introduces a recall bias in their responses. The use of both HINT and AzBio sentence testing to assess post-CI performance is also a limitation. Owing to the ceiling effect of HINT sentences, the minimum cochlear implant test battery was amended in 2011 to include AzBio sentence testing, which is considered to be more challenging (and realistic) than the HINT test because it uses both male and female voices and uses more difficult vocabulary than HINT sentences.15 Patients who were initially tested with HINT sentences and then switched to AzBio during their follow-up may have decreased scores at some postoperative time intervals owing to the different tests being used.
Patients who undergo CI for severe to profound SNHL attributed to MD are capable of achieving a significant improvement in their hearing performance, comparable to the gains experienced by patients without MD who undergo CI. The introduction of the implant electrode into the cochlea adjacent to the endolymphatic space neither seems to adversely alter the natural history of vestibular function, nor notably exacerbate auditory symptoms in cochlear implant recipients who have MD. Cochlear implantation remains an excellent option for patients with MD who meet the threshold for candidacy.
Submitted for Publication: December 9, 2013; final revision received March 4, 2014; accepted March 13, 2014.
Corresponding Author: Eric R. Oliver, MD, Department of Otolaryngology—Head and Neck Surgery, Wake Forest University Baptist Health, Medical Center Blvd, Winston-Salem, NC 27157 (firstname.lastname@example.org).
Published Online: May 1, 2014. doi:10.1001/jamaoto.2014.550.
Author Contributions: Drs Fife and Oliver had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: Fife, Lewis, May, Oliver.
Acquisition, analysis, or interpretation of data: Fife, Lewis, May.
Drafting of the manuscript: Fife.
Critical revision of the manuscript for important intellectual content: Fife, Lewis, May, Oliver.
Statistical analysis: Fife, Oliver.
Administrative, technical, or material support: Lewis.
Study supervision: May.
Conflict of Interest Disclosures: None reported.
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