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Acoustic neuromas presenting with normal or symmetrical hearing: factors associated with diagnosis and outcome.

OBJECTIVE: To evaluate the clinical features leading to diagnosis in patients with acoustic neuroma (AN) who present with normal or symmetrical hearing. Underlying tumor characteristics are also studied to identify a possible explanation for this unique presentation in the AN population. STUDY DESIGN: Retrospective case review comprising patients who were identified as having AN that presented with normal audiometry. SETTING: A tertiary referral center. PATIENTS: Patients with AN who met the criteria for normal were included in the report. For this study, abnormal audiometry is defined as an interaural difference of > or =15 dB at a single frequency or > or =10 dB at two or more frequencies, and an interaural speech reception threshold difference of > or =20 dB, or a speech discrimination score of > or =20%. MAIN OUTCOME MEASURES: Presenting symptoms and signs, clinical features that led to the diagnosis of AN, auditory brain stem response results, tumor location, size and relationship to temporal bone landmarks, surgical intervention, surgical outcome, and results of hearing preservation attempts were tabulated for each patient. RESULTS: A total of 29 patients (5%) were identified who had normal or symmetrical pure-tone audiograms between 500 and 4,000 Hz. The average difference in speech reception threshold between tumor and nontumor ear was 3.2 dB, and the average difference in speech detection score was 2.6%. The most common presenting symptoms that led to the diagnosis of the AN were dysequilibrium/vertigo (12 cases), cranial nerve V and VII abnormalities (11 cases), routine screening for families with neurofibromatosis type 2 (5 cases), asymmetrical tinnitus (4 cases), headaches (4 cases), unilateral subjective hearing difficulty (4 cases), and incidental finding during evaluation for another problem (4 cases). The average tumor size was 19 mm, with five cases presenting with tumors of size > or =30 mm. Nineteen patients underwent a hearing preservation procedure (middle fossa or retrosigmoid), 11 of whom had useful hearing postoperatively. CONCLUSIONS: Despite normal audiometry, patients presenting with imbalance or vertigo, Vth or VIIth cranial nerve deficits, or unilateral hearing complaints may warrant further evaluation to rule out the possibility of AN or other retrocochlear lesion. To seek an explanation for this phenomenon, the incidence of various tumor characteristics (e.g., depth of penetration into the internal auditory canal and degree of porous erosion) is discussed and compared with the entire AN population.

Adult↗

Seventh survey of audiometric practices in the United States.

A 5-page questionnaire was sent to 500 audiologists who were randomly selected from the directory of members of the American Academy of Audiology to assess the clinical practices most commonly used by certified audiologists in the United States. Survey content was limited to only those practices and procedures routinely used by audiologists across the nation. The current results were then compared to those obtained in similar studies conducted in 1971, 1972, 1978, 1985, 1989, and 1994 to determine which clinical procedures are being retained, modified, or replaced.

American Speech-Language-Hearing Association↗

Hearing loss with cochlear modiolar defects and large vestibular aqueducts.

OBJECTIVE: Defects of the cochlear modiolus have been found to be associated with most cases of large vestibular aqueduct. The clinical significance of these modiolar defects has not been studied previously. The purpose of this article is to correlate clinical (functional) parameters, such as hearing outcomes, with the severity of the radiographic findings in these dysplastic inner ears. STUDY DESIGN: The study design was a retrospective chart review, supplemented with telephone interviews and clinic visits. SETTING: The study was conducted at an academic, tertiary care center. PATIENTS: Thirty consecutive patients with large vestibular aqueducts participated. RESULTS: Scores of modiolar deficiencies yielded inconsistent correlations with hearing loss. Vestibular aqueduct morphology and thickness correlated very strongly with the severity of hearing loss. CONCLUSIONS: These observations support the hypothesis that large vestibular aqueduct-related hearing loss may be caused by transmission of subarachnoid pressure forces into the inner ear. However, the thickness and morphology of the vestibular aqueduct may simply be markers for more subtle cochlear dysplasia manifest by modiolar deficiency.

Adolescent↗

Preliminary experience with neural response telemetry in the nucleus CI24M cochlear implant.

OBJECTIVE: This study aimed to compare recordings of the electrically evoked whole nerve action potential (EAP) made using the reverse telemetry system of the Nucleus CI24M device with those recorded from individuals who use the Ineraid cochlear implant system. STUDY DESIGN: Data were collected in a prospective fashion from Nucleus CI24M cochlear implant users and compared with retrospective data collected from patients who use the Ineraid device. SETTING: All data were collected at the Department of Otolaryngology-Head and Neck Surgery, University of Iowa Hospitals and Clinics. PATIENTS: Data are reported from 8 patients who use the Nucleus CI24M cochlear implant and 20 patients who use the Ineraid cochlear implant system. INTERVENTIONS: The interventions described in this study were diagnostic in nature. MAIN OUTCOME MEASURES: EAP growth and refractory recovery data are reported. EAP thresholds recorded from patients who use the Nucleus CI24M device also are compared with behavioral thresholds for the stimulus used to evoke the EAP as well as the stimulation levels needed to program the speech processor. RESULTS: EAP morphology, growth, and refractory recovery functions recorded using the Nucleus CI24M reverse telemetry system compared favorably with similar measures recorded from Ineraid cochlear implant users. CONCLUSIONS: Reasonable EAP responses can be recorded using the Nucleus CI24M device. More data are needed to determine whether the information about neural responsiveness available with this device will be clinically useful.

Cochlear Implantation↗

Audiologic presentation of vestibular schwannomas in neurofibromatosis type 2.

OBJECTIVE: The audiologic presentation of vestibular schwannoma (VS) associated with neurofibromatosis type 2 (NF2) has not been well characterized. The goal of this study was to investigate the audiologic features of NF2-associated VS and to determine their relationship to the size of the tumor. STUDY DESIGN: A retrospective case review. SETTING: Quaternary governmental medical research institute evaluating patients fitting specific criteria for ongoing clinical studies. PATIENTS: Audiologic and magnetic resonance imaging data were available for 40 patients (25 males, 15 females), with an average age of 32 years, who had been recruited for ongoing clinical and genetic studies of NF2. MAIN OUTCOME MEASURES: The audiologic profile and magnetic resonance imaging characteristic of tumor were retrospectively reviewed. RESULTS: The average size of the tumor at presentation was 7.26 +/- 16.58 cm3 and measured 1.2, 1.6, and 1.1 cm in the anterior/posterior, lateral/medial, and superior/inferior dimensions, respectively. An increase in lateral/medial size of the tumor most significantly correlated with deterioration in mid- (1,000-2,000 Hz) and high- (4,000-8,000 Hz) frequency hearing levels, elevated speech reception threshold, and prolonged auditory brain stem response waves III and V latency. CONCLUSIONS: Patients with NF2 demonstrate a more predictable audiologic profile for a given size tumor than has been previously described with spontaneous or sporadic VS.

Adult↗

Single-channel to multichannel conversions in adult cochlear implant subjects.

OBJECTIVE: This study aimed to compare open-set speech perception of single-channel with multichannel cochlear implants in the same ear of postlingually deafened adults. STUDY DESIGN: The study design was a retrospective case and literature review. SETTING: The study was conducted at a tertiary referral center with an associated veterans administration hospital. PATIENTS: Postlingually deafened adults with at least 6 months of experience with a single-channel cochlear implant were studied. INTERVENTION: Replacement of a single-channel with a multichannel cochlear implant in the same ear was performed. MAIN OUTCOME MEASURES: Open-set word and sentence perception scores at least 6 months after single-channel implantation and multichannel reimplantation were measured. RESULTS: Six of six patients had substantial improvement in open-set speech recognition after reimplantation of the same ear. CONCLUSIONS: Removal of a functioning single-channel implant and replacement with a multichannel device are appropriate in postlingually deafened adults who desire better speech recognition.

Adult↗

[Language comprehension by children with profound congenital deafness after cochlear implant].

The consequences of profound deafness on oral language development in children are drastic and well-known. Modern multichannel cochlear implant (CI) has been proven to enhance speech production skills in prelingually deaf children. Speech production skills, however, are known not to be a reliable reflection of oral language competence as a whole. Language is an acquired common code in a specific group, enabling exchange of ideas, feelings and knowledge. In humans, speech is one of the channels conveying language. Assessing language development in CI children is more difficult than simply assessing speech production skills. Many factors may contribute to a poor or an excellent outcome, making it difficult to compare groups of children wearing or not wearing CI. The present study compared receptive language levels in paired matched children from CI and non-CI groups. The main conclusion of this study is that language comprehension scores grow significantly higher over time post-surgery in CI than in paired-matched non-CI children, despite better initial pure tone audiometric thresholds of the latter.

Audiometry, Pure-Tone↗

Impaired speech perception in noise in patients with a normal audiogram.

Patients with normal hearing acuity but complaining of impaired speech perception in noisy conditions were submitted to a test battery including the measurement of the speech reception threshold in noise and central auditory tests. In 95% of the cases the complaint was corroborated by an abnormal speech reception threshold (SRT) in noise. Central auditory tests were abnormal in 65% of the patients. Auditory brainstem-evoked potentials were measured in some of these patients and showed normal results in almost all cases. No specific relationship could be found between an elevated SRT in noise or an abnormal central test result.

Acoustic Stimulation↗

Effect of set size and method on speech reception thresholds in noise.

OBJECTIVE: The threshold for speech is known to improve as signal (Miller, Heise, & Lichten, 1951) or response (Pollack, 1959) uncertainty is decreased. The definition of threshold as the signal level or signal to noise ratio (S/N) at which a fixed percent-correct score is obtained, therefore, becomes problematic when set size, M, is varied through the range for which threshold varies systematically (M = 2 to M = 16) because the meaning of that fixed percent changes as M is increased or decreased. The goal of the present study is to examine the effect of set size, M, on speech reception thresholds (SRTs) under two testing strategies. DESIGN: SRTs were obtained in the presence of 80 dB SPL white noise for sets containing 2, 4, 8, and 16 words using two different procedures, one in which threshold was based on a fixed percent correct (American Speech-Language-Hearing Association, 1988) and one in which threshold was defined in terms of d' = 1.00. The subjects were 12 young women with normal hearing and little or no experience with audiologic testing procedures. RESULTS: When threshold was based on a fixed percent correct, S/N at threshold was found to be dependent on set size (F = 3.333; df = 3, 33; p = 0.031). When threshold was defined in terms of d' = 1.00, S/N at threshold was found to be independent of set size. CONCLUSIONS: If a smaller set size than that recommended by the American Speech-Language-Hearing Association (1988) guidelines is to be used for obtaining SRTs in a clinical setting, thresholds should be based on a criterion-free measure that is independent of the size of the set of words being tested, if possible.

Adult↗

A tangibly reinforced speech reception threshold procedure for use with small children.

Forty normal-hearing children between 17 and 56 months were tested using a new method for determining speech reception thresholds. The words employed were names for parts of a device that was in the form of a colorful clown. The device automatically rewarded the child with a small piece of candy each time he pressed the part of the clown that had been announced through the sound field system of a speech audiometer. Results showed that operant conditioning speech audiometry using tangible reinforcers is feasible for small children. Both boys and girls can be tested accurately by this method down to age two and one-half years.

Audiometry↗

Alterations in auditory processing of speech stimuli during aging in healthy subjects.

Studies of auditory processing during aging in man have not provided a consensus on whether aging affects the ability to process speech stimuli. To evaluate the relationship between speech recognition tasks and age, we examined 36 male subjects between the ages of 21 and 83 years, who were screened for the absence of disease, particularly in the cardiovascular and neurologic systems. Measures were obtained on the following tests: pure tone thresholds, speech reception threshold, speech discrimination, low-pass filtered speech, and binaural fusion. A statistically significant correlation was found between pure tone thresholds and age for all frequencies. When the effect of peripheral hearing loss was taken into account, speech measures did not correlate with age, with the exception of low-pass filtered speech in the left ear. Our findings suggest that the aging process in healthy man is not necessarily accompanied by deficits in the processing of speech stimuli beyond those which are due to peripheral hearing loss.

Adult↗

Design, optimization and evaluation of a Danish sentence test in noise.

The Danish sentence test DANTALE II was developed in analogy to the Swedish sentence test by Hagerman and the German Oldenburg sentence test as a new Danish sentence test in noise to determine the speech reception threshold in noise (SRT, i.e. the signal-to-noise ratio (SNR) that yields 500% intelligibility). Each sentence is generated by a random combination of the alternatives of a base list. This base list consists of 10 sentences with the same syntactical structure (name, verb, numeral, adjective, object). The test sentences were recorded and segmented in such a way that the coarticulation effects were taken into account in order to achieve a high perceived sound quality of the resynthesized sentences: 100 sentences were recorded, each coarticulation between each word and the 10 possible following word alternatives were recorded, and the correct coarticulation was used to generate the test sentences. Word-specific speech recognition curves were measured for each recorded word to optimize the homogeneity of the speech material and the measurement accuracy. Level corrections of particular words and a careful selection of the test lists produced a noticeable reduction in the variation in the distribution of word-specific SRT (standard deviation 1.75 dB instead of 3.78 dB). Therefore, the slope of the total intelligibility function was expected to increase from 8.30%/dB (raw test material) to 13.2%/dB (after modification). These theoretical expectations were evaluated by independent measurements with normal-hearing subjects, and, for the most part, confirmed. The reference data for the DANTALE II are: SRT=-8.43 dB SNR; slope at SRT, s50 = 13.2%/dB. The training effect was 2.2 dB and could be reduced to less than 1 dB if two training lists of 20 sentences were performed prior to data collection.

Adult↗

Measurements and calculations on the simple up-down adaptive procedure for speech-in-noise tests.

The simple up-down adaptive procedure is a common method for measuring speech reception thresholds. It is used by the Dutch speech-in-noise telephone screening test [National Hearing test; Smits and Houtgast Ear Hear. 26, 89-95 (2005)]. The test uses digit triplets to measure the speech reception threshold in noise by telephone (SRTT(n)). About 66 000 people took this test within four months of its introduction and details were stored of all individual measurements. Analyses of this large volume of data have revealed that the standard deviation of SRTT(n) estimates increases with hearing loss. This paper presents a calculation model which--using an intelligibility function as input--can determine the standard deviation of SRTT(n) estimates and the bias for the simple up-down procedure. The effects of variations in the slope of the intelligibility function, the guess rate, the starting level, the heterogeneity of the speech material, and the possibilities of optimizing SRTT(n) measurements were all explored with this model. The predicted decrease in the standard deviation of SRTT(n) estimates as a result of optimizing the speech material was confirmed by measurements in 244 listeners. The paper concludes by discussing possibilities for optimizing the development of comparable tests.

Aging↗

Evaluation of a wide range of amplitude-frequency responses for the hearing impaired.

The long-term average frequency spectrum of speech was modified to 25 target frequency spectra in order to determine the effect of each of these spectra on speech intelligibility in noise and on sound quality. Speech intelligibility was evaluated using the test as developed by Plomp and Mimpen (1979), whereas sound quality was examined through judgments of loudness, sharpness, clearness, and pleasantness of speech fragments. Subjects had different degrees of sensorineural hearing loss and sloping audiograms, but not all of them were hearing aid users. The 25 frequency spectra were defined such that the entire dynamic range of each listener, from 5 dB above threshold to 5 dB below UCL, was covered. Frequency shaping of the speech was carried out on-line by means of Finite Impulse Response (FIR) filters. The tests on speech reception in noise indicated that the Speech-Reception Thresholds (SRTs) did not differ significantly for the majority of spectra. Spectra with high levels, especially at low frequencies (probably causing significant upward spread of masking), and also those with steep negative slopes resulted in significantly higher SRTs. Sound quality judgments led to conclusions virtually identical to those from the SRT data: frequency spectra with an unacceptably low sound quality were in most of the cases significantly worse on the SRT test as well. Because the SRT did not vary significantly among the majority of frequency spectra, it was concluded that a wide range of spectra between the threshold and UCL levels of listeners with hearing losses is suitable for the presentation of speech energy. This is very useful in everyday listening, where the frequency spectrum of speech may vary considerably.

Adult↗

Bilateral bone-anchored hearing aids (BAHAs): an audiometric evaluation.

OBJECTIVES: Since the technique to implant bone-anchored hearing aids (BAHAs) with the use of osseointegrated implants was developed in 1977, more than 15,000 patients have been fitted with BAHAs worldwide. Although the majority have bilateral hearing loss, they are primarily fitted unilaterally. The main objective of this study was to reveal benefits and drawbacks of bilateral fitting of BAHAs in patients with symmetric or slight asymmetric bone-conduction thresholds. The possible effects were divided into three categories: hearing thresholds, directional hearing, and binaural hearing. STUDY DESIGN: Prospective study of 12 patients with bilateral BAHAs. METHODS: Baseline audiometry, directional hearing, speech reception thresholds in quiet and in noise, and binaural masking level difference were tested when BAHAs were fitted unilaterally and bilaterally. RESULTS: Eleven of the 12 patients used bilateral BAHAs on a daily basis. Tests performed in the study show a significant improvement in sound localization with bilateral BAHAs; the results with unilateral fitting were close to the chance level. Furthermore, with bilateral application, the improvement of the speech reception threshold in quiet was 5.4 dB. An improvement with bilateral fitting was also found for speech reception in noise. CONCLUSIONS: Overall, the results with bilateral fitted BAHAs were better than with unilaterally fitted BAHA; the benefit is not only caused simply by bilateral stimulation but also, to some extent, by binaural hearing. Bilateral BAHAs should be considered for patients with bilateral hearing loss otherwise suitable for BAHAs.

Adult↗

Validation of parameters for assessing Obscure Auditory Dysfunction--robustness of determinants of OAD status across samples and test methods.

About 5% of those presenting at an ENT clinic with ear or hearing complaints are found to have normal pure-tone thresholds. Many of this group complain specifically of difficulties understanding speech in background noise. We have termed this complaint 'Obscure Auditory Dysfunction' (OAD). Previous research on OAD (Saunders and Haggard, 1992) on a sample of 50 patients and 50 matched controls showed that 83% of the deviance (binary variance) in membership of group (OAD/control) could be explained by four variables, with three underlying factors. These four variables are performance threshold in noise for speech; discrepancy between this and a self-adjusted signal-to-noise ratio value (i.e. a measure of auditory confidence); dichotic listening performance; and performance on a psychoacoustic test, the threshold for detecting a tone in a spectrally notched noise. This paper reports a study on a further 59 new OAD patients and 64 unmatched controls, using the tests in the form present in the test package, plus certain others. For routine practice, slightly differing implementations of the auditory test techniques were used. Compared to the earlier sample, the new sample had poorer measures of speech reception threshold in noise and they considered themselves more handicapped by their OAD state; however there was no significant difference between the two samples on the other performance variables. Overall, the results demonstrate the robustness of the conclusions reached from the previous study, as the test-variable determinants of OAD status (i.e. case-control differences) were similar. The items on the OAD interview questionnaire which had shown significant differences between OADs and controls were also similar for the two OAD samples, giving a consistent picture of patient profile. These were: difficulties learning to read or write as a child (which relates to dichotic listening scores); adult ear problems; and tinnitus (which relate to the psychoacoustic scores). This necessary replication confirms the validity of the three-factor model of OAD status and the utility of the clinical test package based upon it.

Adolescent↗