Prospective Evaluation of a Smartphone Otoscope for Home Tympanostomy Tube Surveillance: A Pilot Study.

OBJECTIVES: Recent technological advances have led to the capability of performing high resolution imaging of the tympanic membrane. Smartphone technologies and applications have provided the opportunity to capture digital images and easily share them. The smartphone otoscope device was developed as a simple system that can convert a smartphone into a digital otoscope. This device has the prospective ability to improve physician-patient communication and assist with the diagnosis and management of ear disease. Our objective was to evaluate the feasibility and physician/parental satisfaction using the Cellscope® smartphone attachment for at home tympanostomy tube monitoring. METHODS: Children between 6 months and 15 years of age at an urban tertiary children's hospital that were scheduled for bilateral tympanostomy tube insertion or underwent bilateral tympanostomy tube surgery were prospectively enrolled in the study. Comparisons were made between parental home-recorded videos and findings during in-office otoscopy. Two independent otolaryngologists reviewed the videos and concordance between inter-rater agreements was calculated. Acceptability and use questionnaires were administered to physicians and parents. RESULTS: There was good intra-rater agreement between traditional otoscopy and video-otoscopy for tube extruding, tube blocked and tube extruded with at least 80% agreement (P < .05) and excellent inter-rater agreement between physicians for nearly all tube variables (P < .0001) There was a high degree of satisfaction with this mode of surveillance. Parents and physicians agreed that the CellScope® smartphone was easy to use, helpful with the occurrence of acute events, and appeared to improve quality of care. CONCLUSIONS: The CellScope® smartphone is feasible for use in tympanostomy tube surveillance. Use of the device may allow otolaryngologists to easily follow a child's tympanostomy tube remotely over time and offer greater parental satisfaction.

Prevalence of Tinnitus and Associated Factors Among Asian Americans: Results From a National Sample.

OBJECTIVES: A previous study demonstrated low rates of high-frequency hearing loss and noise exposure among Asian Americans (AAs). This study examined the prevalence of tinnitus and associated factors among AAs using the first nationally representative sample of AAs. STUDY DESIGN: Cross-sectional analysis of a national health survey. METHODS: We analyzed data from the 2011 to 2012 National Health and Nutritional Examination Survey, during which 3,669 participants aged 20 to 69 years completed questionnaires on tinnitus (AA participants, n = 530). Tinnitus was defined as bothersome ringing lasting 5 minutes or more in the past 12 months. Prevalence of any tinnitus in the past 12 months was calculated incorporating sampling weights. Logistic regression was used to examine the associations with relevant factors. RESULTS: The prevalence of any tinnitus was 6.6% (95% confidence interval [CI]: 4.5-8.6) among AAs, significantly lower than the prevalence among the overall population at 16.5% (95% CI: 14.3-18.8]. After adjusting for audiometric hearing loss and demographics, AAs were less likely to report any tinnitus than whites (odds ratio [OR] = 0.43 [95% CI: 0.24-0.75]), blacks (OR = 0.60 [95% CI: 0.37-0.95]), and Hispanics (OR = 0.60 [95% CI: 0.45-0.78]). Rates of tinnitus were not different from whites or blacks once comorbidities, noise exposure, and depression were taken into account. Among AAs, reporting exposure to work-related noise (OR = 2.92 [95% CI: 1.15-7.41]) and having a major depressive disorder (OR = 6.45 [95% CI: 1.15-36.12]) were significant factors associated with tinnitus in a multivariate model. CONCLUSIONS: The prevalence of tinnitus is significantly lower among AAs in comparison to other racial groups. Noise exposure and depression were significant factors associated with tinnitus among AAs. LEVEL OF EVIDENCE: 2b Laryngoscope, 2020.

Speech and Communicative Participation in Patients With Facial Paralysis.

Importance: Problems with speech in patients with facial paralysis are frequently noted by both clinicians and the patients themselves, but limited research exists describing how facial paralysis affects verbal communication. Objective: To assess the influence of facial paralysis on communicative participation. Design, Setting, and Participants: A nationwide online survey of 160 adults with unilateral facial paralysis was conducted from March 1 to June 1, 2017. To assess communicative participation, respondents completed the Communicative Participation Item Bank (CPIB) Short Form questionnaire and the Facial Clinimetric Evaluation (FaCE) Scale. Main Outcomes and Measures: The CPIB Short Form and the correlation between the CPIB Short Form and FaCE Scale. In the CPIB, the level of interference in communication is rated on a 4-point Likert scale (where not at all = 3, a little = 2, quite a bit = 1, and very much = 0). Total scores for the 10 items range from 0 (worst) to 30 (best). The FaCE Scale is a 15-item instrument that produces an overall score ranging from 0 (worst) to 100 (best), with higher scores representing better function and higher quality of life. Results: Of the 160 respondents, 145 (90.6%) were women and 15 were men (mean [SD] age, 45.1 [12.6] years). Most respondents reported having facial paralysis for more than 3 years. Causes of facial paralysis included Bell palsy (86 [53.8%]), tumor (41 [25.6%]), and other causes (33 [20.6%]), including infection, trauma, congenital defects, and surgical complications. The mean (SD) score on the CPIB Short Form was 0.16 (0.88) logits (range, -2.58 to 2.10 logits). The mean (SD) score of the FaCE Scale was 40.92 (16.05) (range, 0-83.3). Significant correlations were observed between the CPIB Short Form and overall FaCE Scale scores, as well as the Social Function, Oral Function, Facial Comfort, and Eye Comfort subdomains of the FaCE Scale, but not with the Facial Movement subdomain. Conclusions and Relevance: Patients with facial paralysis in this study sample reported restrictions in communicative participation that were comparable with restrictions experienced by patients with other known communicative disorders, such as laryngectomy and head and neck cancer. We believe that communicative participation represents a unique domain of dysfunction and can help quantify the outcome of facial paralysis and provide an additional frame of reference when assessing treatment outcomes.

Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids.

OBJECTIVE: Imaging characteristics and hearing outcomes in children with cochleovestibular or cochleovestibular nerve (CVN) abnormalities. STUDY DESIGN: Retrospective, critical review. SETTING: Tertiary referral academic center. PATIENTS: Twenty-seven children with CVN abnormalities with magnetic resonance (MRI) and/or computed tomography (CT). Study Intervention(s): None. MAIN OUTCOME MEASURE(S): Determine the likely presence or absence of a CNV and auditory stimulation responses. RESULTS: Two of 27 cases had unilateral hearing loss, and all others had bilateral loss. Eleven (46%) were identified with a disability or additional condition. Twenty-two (42%) ears received a cochlear implant (CI) and 9 ears (17%) experienced no apparent benefit from the device. MRI acquisition protocols were suboptimal for identification of the nerve in 22 (42%) ears. A likely CVN absence was associated with a narrow cochlear aperture and internal auditory canal and cochlear malformation. Thirteen (48%) children with an abnormal nerve exhibited normal cochleae on the same side. Hearing data were available for 30 ears, and 25 ears (83%) exhibited hearing with or without an assistive device. One child achieved closed set speech recognition with a hearing aid, another with a CI. One child achieved open set speech recognition with a CI. CONCLUSIONS: Current imaging cannot accurately characterize the functional status of the CVN or predict an assistive device benefit. Children who would have otherwise been denied a CI exhibited auditory responses after implantation. A CI should be considered in children with abnormal CVN. Furthermore, imaging acquisition protocols need standardization for clear temporal bone imaging.

Early Communication Development of Children with Auditory Brainstem Implants.

The auditory brainstem implant (ABI) is an auditory sensory device that is surgically placed on the cochlear nucleus of the brainstem for individuals who are deaf but unable to benefit from a cochlear implant (CI) due to anatomical abnormalities of the cochlea and/or eighth nerve, specific disease processes, or temporal bone fractures. In the United States, the Food and Drug Administration has authorized a Phase I clinical trial to determine safety and feasibility of the ABI in up to 10 eligible young children who are deaf and either derived no benefit from the CI or were anatomically unable to receive a CI. In this paper, we describe the study protocol and the children who have enrolled in the study thus far. In addition, we report the scores on speech perception, speech production, and language (spoken and signed) for five children with 1-3 years of assessment post-ABI activation. To date, the results indicate that spoken communication skills are slow to develop and that visual communication remains essential for post-ABI intervention.

Assessing the Benefit-Risk Profile for Pediatric Implantable Auditory Prostheses.

BACKGROUND/AIMS: Children with congenital cochleovestibular abnormalities associated with profound hearing loss have few treatment options if cochlear implantation does not yield benefit. An alternative is the auditory brainstem implant (ABI). Regulatory authority device approvals currently include a structured benefit-risk assessment. Such an assessment, for regulatory purposes or to guide clinical decision making, has not been published, to our knowledge, for the ABI and may lead to the design of a research program that incorporates regulatory authority, family, and professional input. METHODS: Much structured benefit-risk research has been conducted in the context of drug trials; here we apply this approach to device studies. A qualitative framework organized benefit (speech recognition, parent self-report measures) and risk (surgery- and device-related) information to guide the selection of candidates thought to have potential benefit from ABI. RESULTS: Children with cochleovestibular anatomical abnormalities are challenging for appropriate assessment of candidacy for a cochlear implant or an ABI. While the research is still preliminary, children with an ABI appear to slowly obtain benefit over time. A team of professionals, including audiological, occupational, and educational therapy, affords maximum opportunity for benefit. CONCLUSIONS: Pediatric patients who have abnormal anatomy and are candidates for an implantable auditory prosthetic require an individualized, multisystems review. The qualitative benefit-risk assessment used here to characterize the condition, the medical need, potential benefits, risks, and risk management strategies has revealed the complex factors involved. After implantation, continued team support for the family during extensive postimplant therapy is needed to develop maximum auditory skill benefit.

Prevalence of Hearing Loss and Hearing Care Use Among Asian Americans: A Nationally Representative Sample.

OBJECTIVE: To assess the prevalence of hearing loss and factors affecting hearing care use among Asian Americans, using the first nationally representative sample of Asian Americans. STUDY DESIGN: National cross-sectional survey. SETTING: Ambulatory examination centers. PATIENTS: Three thousand six hundred twelve adults (522 Asian American) aged 20 to 69 in the 2011 to 2012 National Health and Examination Survey with pure-tone audiometry. MAIN OUTCOME MEASURE(S): Percentage with hearing loss, undertaking a hearing test before the study, and hearing aid use. Hearing loss was defined as better hearing ear speech frequency pure-tone average ≥25 dBHL. Analyses incorporated sampling weights to account for complex sampling design. RESULTS: The prevalence of hearing loss was 6.0% [95% CI 3.1-8.9%] among Asian Americans, comparable to White, Black, and Hispanic groups, and increased substantially with age (OR: 2.25 [95% CI: 1.6-3.2]). After adjusting for age and pure-tone average, Asian Americans with hearing loss were less likely to have received a hearing test compared with White (OR: 0.27 [95% CI: 0.20-0.36, p = <0.001]) and Black groups (OR: 0.26 [95% CI: 0.16-0.38, p<0.001]), less likely to use hearing aids compared with Whites (OR: 0.06 [95% CI: 0.01-0.64], p = 0.02), and less likely to self-report poor hearing compared with Whites (OR: 0.30 [95% CI: 0.10-0.90], p = 0.03). Among Asian Americans, using more non-English than English, being foreign-born, less education, being married, and not having insurance were associated with lower levels of receiving a hearing test. CONCLUSION: The nationally representative sample of Asian Americans with hearing data suggests that hearing loss prevalence is similar to other races/ethnicities. However, hearing aid adoption by Asian Americans tends to be less frequent.

Age-related histologic and biochemical changes in auricular and septal cartilage.

OBJECTIVES/HYPOTHESIS: To characterize the histologic and biochemical properties of auricular and septal cartilage and analyze age-related changes in middle-aged to older adults. STUDY DESIGN: Cross-sectional study of auricular and septal cartilage from 33 fresh cadavers. METHODS: Auricular and septal cartilage specimens were stained using Safranin O for glycosaminoglycans, Verhoeff's stain for elastin, and Masson's trichrome for collagen. Percentage of tissue stained, cell density and size were quantified. Relationships between donor characteristics and histologic properties were evaluated using mixed model analyses. RESULTS: The average donor age was 75 years (standard deviation = 11 years; range, 55-93 years). In auricular cartilage, each 1-year increase in age was associated with a 0.97% decrease in glycosaminoglycans (P < .001) and a 0.98% decrease in elastin (P < .001). In septal cartilage, glycosaminoglycans decreased 2.4% per year (P < .001). Age did not affect collagen content significantly in auricular (P = .417) or septal cartilage (P = .284). Cell density and cell size declined with age in auricular (both P < .001) and septal cartilage (P = .044, P = .032, respectively). Compared to septal cartilage in patients of all ages, auricular cartilage had more glycosaminoglycans, less collagen, higher cell density, and smaller cells. CONCLUSIONS: In auricular and septal cartilage, glycosaminoglycans, elastin, cell density, and cell size decrease significantly with age in patients over 55 years of age. Glycosaminoglycan content declines faster with age in septal cartilage than auricular cartilage. These age-related changes may affect biomechanical properties and tissue viability, and thereby have implications for graft choice in functional, aesthetic, and reconstructive nasal surgery. LEVEL OF EVIDENCE: NA. Laryngoscope, 127:E399-E407, 2017.

Initial Results of a Safety and Feasibility Study of Auditory Brainstem Implantation in Congenitally Deaf Children.

OBJECTIVE: To determine the safety and feasibility of the auditory brainstem implant (ABI) in congenitally deaf children with cochlear aplasia and/or cochlear nerve deficiency. STUDY DESIGN: Phase I feasibility clinical trial of surgery in 10 children, ages 2 to 5 years, over a 3-year period. SETTING: Tertiary children's hospital and university-based pediatric speech/language/hearing center. INTERVENTION(S): ABI implantation and postsurgical programming. MAIN OUTCOME MEASURE(S): The primary outcome measure is the number and type of adverse events during ABI surgery and postsurgical follow-up, including behavioral mapping of the device. The secondary outcome measure is access to and early integration of sound. RESULTS: To date, nine children are enrolled. Five children have successfully undergone ABI surgery and postoperative behavioral programming. Three children were screen failures, and one child is currently undergoing candidacy evaluation. Expected adverse events have been documented in three of the five children who received the ABI. One child experienced a cerebral spinal fluid leak, which resolved with lumbar drainage. One child demonstrated vestibular side effects during device programming, which resolved by deactivating one electrode. One child experienced postoperative vomiting resulting in an abdominal radiograph. Four children have completed their 1-year follow-up and have speech detection thresholds of 30 to 35 dB HL. Scores on the IT-MAIS/MAIS range from 8 to 31 (out of a total of 40), and the children are demonstrating some ability to discriminate between closed-sets words that differ by number of syllables (pattern perception). CONCLUSION: ABI surgery and device activation seem to be safe and feasible in this preliminary cohort.

Device Life of Two Generations of Provox Voice Prostheses.

BACKGROUND: Tracheoesophageal voice prostheses are invaluable for speech rehabilitation in patients who have received total laryngectomy, but device failure impedes communication and creates psychosocial and financial burdens. This study compares the Provox 2 and Provox Vega voice prostheses on the parameter of device life. METHODS: This was a retrospective observational study of 21 patients with 181 device replacements at an academic tertiary care medical center. Disparity in device life and factors that may influence device life were analyzed. RESULTS: The mean device life for Provox 2, at 115.6 days (SE = 5.8), was longer than for Provox Vega, at 65.1 days (SE = 7.5) (P < .001). CONCLUSIONS: Device longevity was greater for Provox 2 over Provox Vega. These results will facilitate the design of prospective studies to assess reasons for variations in device life between patients and device types.

Sentence Recognition in Quiet and Noise by Pediatric Cochlear Implant Users: Relationships to Spoken Language.

OBJECTIVE: We investigated associations between sentence recognition and spoken language for children with cochlear implants (CI) enrolled in the Childhood Development after Cochlear Implantation (CDaCI) study. STUDY DESIGN: In a prospective longitudinal study, sentence recognition percent-correct scores and language standard scores were correlated at 48-, 60-, and 72-months post-CI activation. SETTING: Six tertiary CI centers in the United States. PATIENTS: Children with CIs participating in the CDaCI study. INTERVENTION (S): Cochlear implantation. MAIN OUTCOME MEASURE (S): Sentence recognition was assessed using the Hearing In Noise Test for Children (HINT-C) in quiet and at +10, +5, and 0 dB signal-to-noise ratio (S/N). Spoken language was assessed using the Clinical Assessment of Spoken Language (CASL) core composite and the antonyms, paragraph comprehension (syntax comprehension), syntax construction (expression), and pragmatic judgment tests. RESULTS: Positive linear relationships were found between CASL scores and HINT-C sentence scores when the sentences were delivered in quiet and at +10 and +5 dB S/N, but not at 0 dB S/N. At 48 months post-CI, sentence scores at +10 and +5 dB S/N were most strongly associated with CASL antonyms. At 60 and 72 months, sentence recognition in noise was most strongly associated with paragraph comprehension and syntax construction. CONCLUSIONS: Children with CIs learn spoken language in a variety of acoustic environments. Despite the observed inconsistent performance in different listening situations and noise-challenged environments, many children with CIs are able to build lexicons and learn the rules of grammar that enable recognition of sentences.

Prognostic implications of and audiometric evidence for hearing fluctuation in Meniere's disease.

OBJECTIVES/HYPOTHESIS: 1) To establish criteria for significant hearing fluctuation by assessing the range and occurrence of hearing fluctuations over the course of Meniere's disease; 2) to determine if audiometric evidence exists to support the notion that Meniere's disease is a pathophysiologic process involving the whole cochlea; and 3) to suggest prognostic implications for initial hearing fluctuation in patients with Meniere's disease. STUDY DESIGN: Retrospective case series review. METHODS: A total of 488 patients diagnosed by 1995 American Academy of Otolaryngology-Head and Neck Surgery Meniere's disease criteria for whom audiometric data were prospectively collected (2 cohorts: 341 and 146 patients initially seen between April 2002 to July 2003 and between January to December 2010, respectively). Based on several definitions for significant hearing fluctuation, change in hearing was categorized as "same," "worse," or "better" between any two consecutive evaluations. The relationship of initial hearing fluctuation to future hearing fluctuation and future hearing loss was evaluated. RESULTS: Hearing fluctuation was evident in Meniere's disease patients with heterogeneous audiometric follow-up; and the characteristics of these hearing fluctuations, including the mean incidence, is described. Audiometric data suggests that there is a high congruence in Meniere's disease between changes in low- and high-frequency thresholds. Initial hearing fluctuation is associated with the occurrence of future and more frequent hearing fluctuations. CONCLUSION: Understanding the range of hearing fluctuations establishes a basis for determining audiometric thresholds used in evaluating future therapeutic trials aimed at the prevention of hearing loss in Meniere's disease. This knowledge will also inform the counseling directed toward patients diagnosed with Meniere's disease.

A Prospective Longitudinal Study of U.S. Children Unable to Achieve Open-Set Speech Recognition 5 Years After Cochlear Implantation.

OBJECTIVE: To identify characteristics associated with the inability to progress to open-set speech recognition in children 5 years after cochlear implantation. STUDY DESIGN: Prospective, longitudinal, and multidimensional assessment of auditory development for 5 years. SETTING: Six tertiary cochlear implant (CI) referral centers in the United States. PATIENTS: Children with severe-to-profound hearing loss who underwent implantation before age 5 years enrolled in the Childhood Development after Cochlear Implantation study, categorized by level of speech recognition ability. INTERVENTION(S): Cochlear implantation before 5 years of age and annual assessment of emergent speech recognition skills. MAIN OUTCOME MEASURE(S): Progression to open-set speech recognition by 5 years after implantation. RESULTS: Less functional hearing before implantation, older age at onset of amplification, lower maternal sensitivity to communication needs, minority status, and complicated perinatal history were associated with the inability to obtain open-set speech recognition by 5 years. CONCLUSION: Characteristics of a subpopulation of children with CIs associated with an inability to achieve open-set speech recognition after 5 years of CI experience were investigated. These data distinguish pediatric CI recipients at risk for poor auditory development and highlight areas for future interventions to enhance support of early implantation.

An open label study to evaluate the safety and efficacy of intratympanic golimumab therapy in patients with autoimmune inner ear disease.

OBJECTIVE: To evaluate the safety and efficacy of intratympanically injected golimumab (GLM), a TNF-α inhibitor, as a steroid-sparing agent for patients with steroid-dependent autoimmune inner ear disease (AIED). STUDY DESIGN: Open label. SETTING: Tertiary referral center. PATIENTS: Ten patients with steroid-dependent AIED were enrolled in Stage 2. The average patient age at enrollment was 59, with an average of 12.5 years from the start of bilateral hearing loss symptoms. The average dose of daily prednisone at the start of injections was 18 mg. INTERVENTION: Intratympanic injection of GLM. MAIN OUTCOME MEASURE: Hearing loss progression (treatment failure) was defined as either an increase in pure-tone thresholds by frequency or a decrease in word recognition score. RESULTS: There were no serious adverse events. Five of seven per-protocol subjects experienced stable pure-tone thresholds in the injected ear, whereas 4 had stable word recognition scores. Two subjects experienced an improvement in word recognition scores. The results support the hypothesis that GLM may be a promising treatment. CONCLUSIONS: The TNF-α inhibitor GLM stabilized hearing in 3 of 7 per-protocol subjects with AIED and allowed a complete tapering off of prednisone in those 7 subjects. Studies with larger samples sizes are warranted.

mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma.

BACKGROUND: Neurofibromatosis type 2 (NF2) is a rare autosomal dominant genetic disorder, resulting in a variety of neural tumors, with bilateral vestibular schwannomas as the most frequent manifestation. Recently, merlin, the NF2 tumor suppressor, has been identified as a novel negative regulator of mammalian target of rapamycin complex 1 (mTORC1); functional loss of merlin was shown to result in elevated mTORC1 signaling in NF2-related tumors. Thus, mTORC1 pathway inhibition may be a useful targeted therapeutic approach. METHODS: We studied in vitro cell models, cohorts of mice allografted with Nf2(-/-) Schwann cells, and a genetically modified mouse model of NF2 schwannoma in order to evaluate the efficacy of the proposed targeted therapy for NF2. RESULTS: We found that treatment with the mTORC1 inhibitor rapamycin reduced the severity of NF2-related Schwann cell tumorigenesis without significant toxicity. Consistent with these results, in an NF2 patient with growing vestibular schwannomas, the rapalog sirolimus induced tumor growth arrest. CONCLUSIONS: Taken together, these results constitute definitive evidence that justifies proceeding with clinical trials using mTORC1-targeted agents in selected patients with NF2 and in patients with NF2-related sporadic tumors.

Genetic evidence for an ethnic diversity in the susceptibility to Ménière's disease.

BACKGROUND: Ménière's disease (MD) is a debilitating disorder of the inner ear characterized by cochlear and vestibular dysfunction. The cause of this disease is still unknown, and epidemiological data for MD are sparse. From the existing literature, women seem to be more susceptible than men, and Caucasians seem to be more susceptible than Asians. OBJECTIVE: In this article, we characterize a large definite MD cohort for sex and age of onset of disease and use molecular genetic methodologies to characterize ethnicity. STUDY DESIGN: Medical record review for sex and age of onset. Ancestry analysis compared results from the principal component analysis of whole-genome genotype data from MD patients to self-identified ancestry in control samples. SETTING: House Clinic in Los Angeles. PATIENTS: Definitive MD patients. RESULTS: Our review of medical records for definitive MD patients reveals that women are more susceptible than men. We also find that men and women have nearly identical age of onset for disease. Lastly, interrogation of molecular genetic data with principal component analysis allowed detailed observations about the ethnic ancestry of our patients. Comparison of the ethnicity of MD patients presenting to our tertiary care clinic with the self-recollected ethnicity of all patients visiting the clinic revealed an ethnic bias, with Caucasians presenting at a higher frequency than expected and the remaining major ethnicities populating Los Angeles (Hispanics, Blacks, and Asians) presenting at a lower frequency than expected. CONCLUSION: To the best of our knowledge, this report is the first ethnic characterization of a large MD cohort from a large metropolitan region using molecular genetic data. Our data suggest that there is a bias in sex and ethnic susceptibility to this disease.

The relationship between high-frequency pure-tone hearing loss, hearing in noise test (HINT) thresholds, and the articulation index.

BACKGROUND: Speech recognition in noise testing has been conducted at least since the 1940s (Dickson et al, 1946). The ability to recognize speech in noise is a distinct function of the auditory system (Plomp, 1978). According to Kochkin (2002), difficulty recognizing speech in noise is the primary complaint of hearing aid users. However, speech recognition in noise testing has not found widespread use in the field of audiology (Mueller, 2003; Strom, 2003; Tannenbaum and Rosenfeld, 1996). The audiogram has been used as the "gold standard" for hearing ability. However, the audiogram is a poor indicator of speech recognition in noise ability. PURPOSE: This study investigates the relationship between pure-tone thresholds, the articulation index, and the ability to recognize speech in quiet and in noise. RESEARCH DESIGN: Pure-tone thresholds were measured for audiometric frequencies 250-6000 Hz. Pure-tone threshold groups were created. These included a normal threshold group and slight, mild, severe, and profound high-frequency pure-tone threshold groups. Speech recognition thresholds in quiet and in noise were obtained using the Hearing in Noise Test (HINT) (Nilsson et al, 1994; Vermiglio, 2008). The articulation index was determined by using Pavlovic's method with pure-tone thresholds (Pavlovic, 1989, 1991). STUDY SAMPLE: Two hundred seventy-eight participants were tested. All participants were native speakers of American English. Sixty-three of the original participants were removed in order to create groups of participants with normal low-frequency pure-tone thresholds and relatively symmetrical high-frequency pure-tone threshold groups. The final set of 215 participants had a mean age of 33 yr with a range of 17-59 yr. DATA COLLECTION AND ANALYSIS: Pure-tone threshold data were collected using the Hughson-Weslake procedure. Speech recognition data were collected using a Windows-based HINT software system. Statistical analyses were conducted using descriptive, correlational, and multivariate analysis of covariance (MANCOVA) statistics. RESULTS: The MANCOVA analysis (where the effect of age was statistically removed) indicated that there were no significant differences in HINT performances between groups of participants with normal audiograms and those groups with slight, mild, moderate, or severe high-frequency hearing losses. With all of the data combined across groups, correlational analyses revealed significant correlations between pure-tone averages and speech recognition in quiet performance. Nonsignificant or significant but weak correlations were found between pure-tone averages and HINT thresholds. CONCLUSIONS: The ability to recognize speech in steady-state noise cannot be predicted from the audiogram. A new classification scheme of hearing impairment based on the audiogram and the speech reception in noise thresholds, as measured with the HINT, may be useful for the characterization of the hearing ability in the global sense. This classification scheme is consistent with Plomp's two aspects of hearing ability (Plomp, 1978).

GRM7 variants associated with age-related hearing loss based on auditory perception.

Age-related hearing impairment (ARHI), or presbycusis, is a common condition of the elderly that results in significant communication difficulties in daily life. Clinically, it has been defined as a progressive loss of sensitivity to sound, starting at the high frequencies, inability to understand speech, lengthening of the minimum discernable temporal gap in sounds, and a decrease in the ability to filter out background noise. The causes of presbycusis are likely a combination of environmental and genetic factors. Previous research into the genetics of presbycusis has focused solely on hearing as measured by pure-tone thresholds. A few loci have been identified, based on a best ear pure-tone average phenotype, as having a likely role in susceptibility to this type of hearing loss; and GRM7 is the only gene that has achieved genome-wide significance. We examined the association of GRM7 variants identified from the previous study, which used an European cohort with Z-scores based on pure-tone thresholds, in a European-American population from Rochester, NY (N = 687), and used novel phenotypes of presbycusis. In the present study mixed modeling analyses were used to explore the relationship of GRM7 haplotype and SNP genotypes with various measures of auditory perception. Here we show that GRM7 alleles are associated primarily with peripheral measures of hearing loss, and particularly with speech detection in older adults.