The Tinnitus Primary Function Questionnaire is valid, reliable, and sensitive and can be used to determine the efficacy of clinical trials.

Background Up to 7% of patients with severe-to-profound deafness do not benefit from cochlear implantation. Given the high surgical implantation and clinical management cost of cochlear implantation (> $1 million lifetime cost), prospective identification of the worst performers would reduce unnecessary procedures and healthcare costs. Because cochlear implants bypass the membranous labyrinth but rely on the spiral ganglion for functionality, we hypothesize that cochlear implant (CI) performance is dictated in part by the anatomic location of the cochlear pathology that underlies the hearing loss. As a corollary, we hypothesize that because genetic testing can identify sites of cochlear pathology, it may be useful in predicting CI performance. Methods 29 adult CI recipients with idiopathic adult-onset severe-to-profound hearing loss were studied. DNA samples were subjected to solution-based sequence capture and massively parallel sequencing using the OtoSCOPE® platform. The cohort was divided into three CI performance groups (good, intermediate, poor) and genetic causes of deafness were correlated with audiometric data to determine whether there was a gene-specific impact on CI performance. Results The genetic cause of deafness was determined in 3/29 (10%) individuals. The two poor performers segregated mutations in TMPRSS3, a gene expressed in the spiral ganglion, while the good performer segregated mutations in LOXHD1, a gene expressed in the membranous labyrinth. Comprehensive literature review identified other good performers with mutations in membranous labyrinth-expressed genes; poor performance was associated with spiral ganglion-expressed genes. Conclusions Our data support the underlying hypothesis that mutations in genes preferentially expressed in the spiral ganglion portend poor CI performance while mutations in genes expressed in the membranous labyrinth portend good CI performance. Although the low mutation rate in known deafness genes in this cohort likely relates to the ascertainment characteristics (postlingual hearing loss in adult CI recipients), these data suggest that genetic testing should be implemented as part of the CI evaluation to test this association prospectively.

Objectives Few studies have examined the long-term effect of age at implantation on outcomes using multiple data points in children with cochlear implants. The goal of this study was to determine if age at implantation has a significant, lasting impact on speech perception, language, and reading performance for children with prelingual hearing loss. Design A linear mixed model framework was utilized to determine the effect of age at implantation on speech perception, language, and reading abilities in 83 children with prelingual hearing loss who received cochlear implants by age 4. The children were divided into two groups based on their age at implantation: 1) under 2 years of age and 2) between 2 and 3.9 years of age. Differences in model specified mean scores between groups were compared at annual intervals from 5 to 13 years of age for speech perception, and 7 to 11 years of age for language and reading. Results After controlling for communication mode, device configuration, and pre-operative pure-tone average, there was no significant effect of age at implantation for receptive language by 8 years of age, expressive language by 10 years of age, reading by 7 years of age. In terms of speech perception outcomes, significance varied between 7 and 13 years of age, with no significant difference in speech perception scores between groups at ages 7, 11 and 13 years. Children who utilized oral communication (OC) demonstrated significantly higher speech perception scores than children who used total communication (TC). OC users tended to have higher expressive language scores than TC users, although this did not reach significance. There was no significant difference between OC and TC users for receptive language or reading scores. Conclusions Speech perception, language, and reading performance continue to improve over time for children implanted before 4 years of age. The current results indicate that the effect of age at implantation diminishes with time, particularly for higher-order skills such as language and reading. Some children who receive CIs after the age of 2 years have the capacity to approximate the language and reading skills of their earlier-implanted peers, suggesting that additional factors may moderate the influence of age at implantation on outcomes over time.

Purpose-To determine (a) changes in the Tinnitus Handicap Questionnaire (THQ) for patients using cochlear implants, (b) differences between patients who receive total or partial relief, and (c) identifiable characteristics of those who report tinnitus after implantation.Method-Pre-and postoperatively, 244 adults were administered the THQ when they reported tinnitus.Results-Of the 153 patients who had tinnitus preoperatively, 94 (61%) patients reported total suppression and 59 (39%) reported a partial reduction. In 91 patients who did not have tinnitus before implantation, 11 (12%) reported tinnitus postimplantation. The THQ score decreased from 41% preimplant to 30% postimplant. The largest reductions involved social handicap and hearing. Patients with a more severe hearing loss might be more likely to experience an exacerbation of their tinnitus. We were not able to clearly identify differences between patients who received total or partial relief and the characteristics of patients who reported tinnitus after implantation. Those who acquired tinnitus had the shortest duration hearing loss (5.6 years) and were the oldest (63 years). The average THQ score of patients getting tinnitus was 29%. Conclusions-Most tinnitus patients benefit from receiving a cochlear implant.© American Speech-Language-Hearing Association Contact author: Tao Pan, University of Iowa, Department of Otolaryngology, 21200 PFP, 200 Hawkins Drive, Iowa City, IA 52242-1009. tao-pan@uiowa.edu. Copyright of American Journal of Audiology is the property of American Speech-Language-Hearing Association and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. NIH Public Access Author ManuscriptAm J Audiol. Author manuscript; available in PMC 2010 December 1. (Demajumdar, Stoddart, Donaldson, & Proops, 1999;Di Nardo et al., 2007; Gibson, Aran, & Dauman, 1992;Ito, 1997;Miyamoto, Wynne, McKnight, & Bichey, 1997;Mo, Harris, & Lindbaek, 2002;Quaranta, Fernandez-Vega, D'elia, Filipo, & Quaranta, 2008;Tyler, 1994;Tyler & Kelsay, 1990). However, reviews by Dauman et al. (2000), Rubinstein, Tyler, Johnson, and Brown (2003), Rubinstein, Wilson, Finley, and Abbas (1999), Quaranta, Wagstaff, and Baguley (2004), and Baguley and Atlas (2007 have questioned the nature of tinnitus reduction by electrical stimulation.Although previous studies have employed categorical ratings of the effect of CIs on perceived tinnitus severity, those data have not addressed specific aspects of benefit, nor have they provided an indication of any gradation of benefit. Other studies have provided category visual analog scales or attempted interval visual analog or magnitude estimation scales (e.g., Newman, Jacobson, & Spitzer, 1996). These metrics have the potential to distinguish between those who receive total relief of their tinnitus and those who receive partial relief. However, no study has attempted to explor...

We studied the relationship between tinnitus pitch and the audiogram in 195 patients. Patients with tone-like tinnitus reported a higher pitch (mean = 5385 Hz) compared to those with a noise-like quality (mean = 3266 Hz). Those with a flat audiogram were more likely to report: a noise-like tinnitus, a unilateral tinnitus, and have a pitch < 2000 Hz. The average duration of bilateral tinnitus (12 years) was longer than that of unilateral tinnitus (5 years). Older subjects reported a less severe tinnitus handicap questionnaire score. Patients with a notched audiogram often reported a pitch or=8000 Hz. We failed to find a relationship between the pitch and the edge of a high frequency hearing loss. Some individuals did exhibit a pitch at the low frequency edge of a hearing loss, but we could find no similar characteristics among these subjects. It is possible that a relationship between pitch and audiogram is present only in certain subgroups.