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.

Cochlear implant patients' localization using interaural level differences exceeds that of untrained normal hearing listeners.

Bilateral cochlear implant patients are unable to localize as well as normal hearing listeners. Although poor sensitivity to interaural time differences clearly contributes to this deficit, it is unclear whether deficits in terms of interaural level differences are also a contributing factor. In this study, localization was tested while manipulating interaural time and level cues using head-related transfer functions. The results indicate that bilateral cochlear implant users' ability to localize based on interaural level differences is actually greater than that of untrained normal hearing listeners.

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).

The effect of different cochlear implant microphones on acoustic hearing individuals' binaural benefits for speech perception in noise.

OBJECTIVES: Cochlear implant microphones differ in placement, frequency response, and other characteristics such as whether they are directional. Although normal-hearing (NH) individuals are often used as controls in studies examining cochlear implant users' binaural benefits, the considerable differences across cochlear implant microphones make such comparisons potentially misleading. The goal of this study was to examine binaural benefits for speech perception in noise for NH individuals using stimuli processed by head-related transfer functions (HRTFs) based on the different cochlear implant microphones. DESIGN: HRTFs were created for different cochlear implant microphones and used to test participants on the Hearing in Noise Test. Experiment 1 tested cochlear implant users and NH individuals with HRTF-processed stimuli and with sound field (SF) testing to determine whether the HRTFs adequately simulated SF testing. Experiment 2 determined the measurement error and performance-intensity function for the Hearing in Noise Test with NH individuals listening to stimuli processed with the various HRTFs. Experiment 3 compared NH listeners' performance across HRTFs to determine how the HRTFs affected performance. Experiment 4 evaluated binaural benefits for NH listeners using the various HRTFs, including ones that were modified to investigate the contributions of interaural time and level cues. RESULTS: The results indicated that the HRTFs adequately simulated SF testing for the Hearing in Noise Test. They also demonstrated that the test-retest reliability and performance-intensity function were consistent across HRTFs, and that the measurement error for the test was 1.3 dB, with a change in signal-to-noise ratio of 1 dB reflecting a 10% change in intelligibility. There were significant differences in performance when using the various HRTFs, with particularly good thresholds for the HRTF based on the directional microphone when the speech and masker were spatially separated, emphasizing the importance of measuring binaural benefits separately for each HRTF. Evaluation of binaural benefits indicated that binaural squelch and spatial release from masking were found for all HRTFs, and binaural summation was found for all but one HRTF, although binaural summation was less robust than the other types of binaural benefits. In addition, the results indicated that neither interaural time nor level cues dominated binaural benefits for the NH participants. CONCLUSIONS: This study provides a means to measure the degree to which cochlear implant microphones affect acoustic hearing with respect to speech perception in noise. It also provides measures that can be used to evaluate the independent contributions of interaural time and level cues. These measures provide tools that can aid researchers in understanding and improving binaural benefits in acoustic hearing individuals listening via cochlear implant microphones.

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.

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.

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.

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.

Hearing preservation surgery for neurofibromatosis Type 2-related vestibular schwannoma in pediatric patients.

OBJECT: The authors reviewed the proportion of pediatric patients with neurofibromatosis Type 2 (NF2) in whom hearing was preserved after middle fossa resection of vestibular schwannoma (VS). METHODS: In this retrospective chart review the authors examined the cases of 35 children with NF2 who had undergone middle fossa resection (47 surgeries) between 1992 and 2004 in a neurotological tertiary care center. Surgical outcome was assessed using pure-tone average (PTA) thresholds obtained before and immediately after resection. Speech discrimination scores (SDSs) and pre- and postfacial nerve grades were also recorded. In 55% of surgeries, hearing of less than or equal to 70 dB PTA was maintained postoperatively. The American Academy of Otolaryngology-Head and Neck Surgery Class A hearing (PTA < or = 30 dB and SDS > or = 70%) was preserved in 47.7%. Facial nerve function was good (House-Brackmann Grades I or II) in 81% of the patients. Twelve patients had bilateral middle fossa resections; in nine (75%) of these patients hearing was maintained postoperatively in both ears. CONCLUSIONS: More than half of the children with NF2 in the authors' cohort experienced hearing preservation after middle fossa resection was performed for VS. The authors recommend this approach for preserving hearing in children with NF2.

Facial nerve function after translabyrinthine vestibular schwannoma surgery.

OBJECTIVES: To evaluate the long-term facial function of patients after translabyrinthine vestibular schwannoma [VS] surgery and identify factors that influence these outcomes. STUDY DESIGN AND SETTING: A retrospective review was performed that included 580 consecutive patients who underwent translabyrinthine craniotomy for removal of VS at a tertiary referral neurotologic practice between February 2000 and July 2004. A total of 512 patients who underwent primary microsurgical treatment of sporadic unilateral VS met inclusion criteria. Patient and tumor characteristics as well as perioperative complications are described. Perioperative and long-term facial function were evaluated in 392 patients who had at least 1-year follow-up. RESULTS: Complication rates after translabyrinthine craniotomy for VS are low. Patients with smaller tumors have significantly better postoperative facial function than those with larger tumors. CONCLUSIONS: Excellent long-term facial function can be expected in the majority of patients who undergo microsurgical removal of VS via the translabyrinthine approach. Alternative treatment strategies may need to be developed for the treatment of VS > 3.5 cm in order to maximize postoperative facial function.

Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2.

OBJECTIVE: To describe the prevalence and location of cranial nerve schwannomas, other than bilateral vestibular schwannoma, in patients with neurofibromatosis 2 (NF2). The NF2 Natural History Consortium prospectively gathered cranial magnetic resonance imaging for 83 patients across 3 annual evaluations. The time between the first and last evaluation was approximately 3 years. RESULTS: Forty-two patients (51%) had nonvestibular cranial nerve schwannomas (NVSs). Of these, 25 (60%) also had cranial meningiomas. Twenty-one of those without NVS (25% of 83) had at least 1 meningioma. The average size of the NVS was 0.4 cubic centimeters. Overall, there was no significant change in NVS size from Year 1 to Year 3 or from Year 1 to Year 2. The most common locations of the NVS were occulomotor and trigeminal. A family history of NF2 did not predict NVS location or growth. CONCLUSION: Nonvestibular cranial nerve schwannoma usually affect cranial nerves III and V, as was the case in our NF2 sample. Fortunately, neuropathies associated with these tumors are rare. In contrast, lower cranial nerve schwannomas, although also rare, are associated with swallowing difficulty, aspiration, and other sequelae.

Evaluation of facial function with a questionnaire: reliability and validity.

OBJECTIVES: To analyze the validity and reliability of assigning House-Brackmann facial nerve grade based on a patient-administered questionnaire. Specifically, the use of a questionnaire was studied for evaluation of the long-term facial function after microsurgical resection of vestibular schwannoma (VS). STUDY DESIGN: Three neurotologists with more than 25 years of experience and 3 neurotology clinical fellows rated facial nerve grade in a blinded fashion based on the questionnaire. The standard to which the questionnaires were compared was the facial nerve grade assigned to the patient during an office visit near the same time as the completed patient questionnaire. METHODS: Thirty-four patients had an office evaluation of facial function at least 1 year after microsurgical resection of a VS. Thirty patients had a sporadic VS, whereas 4 patients had neurofibromatosis 2. The average tumor size was 2.2 cm. A postoperative questionnaire regarding facial function was completed within 2 months of the office evaluation. Statistical analyses were performed to compare the office evaluation rating with the questionnaire rating (validity) and to analyze interobserver reliability. RESULTS: The association between the facial nerve grade assigned by the office evaluation and the questionnaire was highly significant (p<0.01), with a mean correlation (Spearman [rho]) equal to 0.94. The interobserver reliability was high, with coefficients ranging from 0.93 to 0.99 (p<0.01; mean=0.97). Because a large proportion of patients had normal (House-Brackmann Grade I) facial function, an additional analysis was performed with these patients excluded. The high levels of interobserver reliability persisted (average=0.96). CONCLUSION: When compared with office evaluation, a patient-administered questionnaire provides a valid and reliable measure of long-term facial function after VS surgery.

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.

Meniere's disease: prevalence of contralateral ear involvement.

OBJECTIVE: Determine the prevalence and time interval for conversion from unilateral to bilateral involvement in Meniere's disease and cochlear hydrops. STUDY DESIGN AND SETTING: Retrospective chart review in a tertiary otologic referral center. PATIENTS: 232 patients diagnosed with Meniere's Disease (n=186) or cochlear hydrops (n=46) between 1959 and 2001, who visited the clinic over a five-year period between 1997-2001 and have at least 2 audiograms more than 12 months apart. MAIN OUTCOME MEASURES: Prevalence of cochlear hydrops relative to Meniere's Disease, rate of progression from unilateral to bilateral involvement; interval between unilateral onset of symptoms and bilateral involvement; and rate of progression from cochlear hydrops to Meniere's disease. RESULTS: Initial diagnosis was Meniere's disease in 71% and cochlear hydrops in 29% of all 950 hydropic patients presenting between 1997 and 2001. In the study sample, Meniere's disease was bilateral at presentation in 11%; an additional 12% (14% of unilaterals) became bilateral during the follow-up period. At presentation, 6.5% of cochlear hydrops patients were bilateral, with another 26% becoming bilateral. Conversion from cochlear hydrops to Meniere's disease occurred in 33% and some of these are included among the bilateral. The average time interval for conversion from unilateral to bilateral Meniere's was 7.6 years (SD=7.0 years). CONCLUSION: Most otologists are aware of the potential for contralateral ear involvement and conversion from cochlear hydrops to Meniere's disease after diagnosis. These changes are significant, require long-term follow-up for detection, and may necessitate further treatment. Patients should be counseled regarding this potential when interventions are considered, especially with respect to ablative treatments.

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.

Oral steroid regimens for idiopathic sudden sensorineural hearing loss.

OBJECTIVE: To determine hearing recovery in patients with idiopathic sudden hearing loss treated with varying amounts of oral steroids. STUDY DESIGN AND SETTING: A retrospective chart review (n = 75) in a tertiary care clinic examined sudden hearing loss patients treated with 1 60-mg prednisone taper, 1 course of steroid less than a 60-mg taper, or any 2 courses of oral steroid. RESULTS: Overall, 35% of the patients recovered a clinically significant amount of hearing. Recovery was associated with immediate treatment (within 2 weeks from onset), better hearing at the onset of treatment, and treatment with the higher dose of prednisone in patients with just 1 additional symptom (dizziness or tinnitus). Patients tended to continue to experience some recovery in hearing up to 4 months after treatment. CONCLUSION: Immediate treatment of patients with unilateral idiopathic sudden hearing loss and additional symptoms (dizziness or tinnitus) with a 14-day course of 60 mg prednisone (with taper) is recommended. EBM RATING: B-3.