Study on characteristics of wideband acoustic immittance in patients with Inner Ear Malformations.

OBJECTIVE: To compare the differences in wideband absorbance and the resonance frequency (RF) between patients with inner ear malformations and normal control, and to explore the auditory diagnostic value of wideband acoustic immittance (WAI). METHODS: A total of 38 patients (59 ears) with enlarged vestibular aqueduct (EVA), 13 patients (14 ears) with incomplete partition type I (IP-I) and 13 patients (26 ears) with incomplete partition type II (IP-II) were included. 50 normal control (100 ears). All subjects underwent WAI tests to compare the absorbance configuration and resonance frequency. RESULTS: All the group showed lower absorbance at ambient pressure than at peak pressure in certain frequencies under 2000Hz. Under 1000Hz, the absorbance of EVA was higher than that of other groups. The average absorbance and highest absorbance of IP-I were the lowest(P＜0.05). However, IP-II and normal group had similarity on some characteristics. The three IEM groups mainly different at low and high frequencies, but not at medium frequencies. The highest absorbance of all the groups were appeared around 3000Hz. The RF of all the groups from low to high were EVA＜IP-II＜normal control＜IP-I, and the lowest was EVA(P＜0.05). CONCLUSION: Inner ear malformations can affect energy absorbance and RF. WAI is sensitive and non-invasive to provide useful information about inner ear status and facilitate detection of ear pathology.

Effects of aging on ocular vestibular-evoked myogenic potential using ER-3A insert earphone and B81 bone vibrator.

Purpose: Aging is a process associated with degeneration and dysfunction of peripheral vestibular system or apparatus. This study aimed to investigate the influence of aging on ocular vestibular-evoked myogenic potential (oVEMP) response rates and recording parameters using the B81 bone vibrator and compare them with air conduction stimuli (ACS) oVEMP response characteristics. Methods: In 60 healthy participants aged 10-71 years (mean age 39.9; 29 male participants), the oVEMP response was elicited using a B81 bone vibrator and an ER-3A insert earphone. The effects of age and stimulus on oVEMP response rates and recording parameters were evaluated. Results: Response rates and amplitudes declined with aging using either ACS or bone-conducted vibration (BCV) stimulation, particularly in individuals over 60 years of age, whereas thresholds increased and N1 latencies were prolonged. BCV showed fewer risks of absent oVEMP response than ACS (p = 0.002). BCV acquired higher amplitudes (p < 0.001), lower thresholds, and shorter N1 and P1 latencies (all p < 0.001) than ACS. Conclusions: The absence of an oVEMP response may be attributed to aging rather than a concurrent vestibular disorder. B81-BCV likely produces higher mechanical drives to the vestibular hair cells at safer and non-traumatic levels compared with ACS and therefore may be more likely to evoke a response in the elderly cohort, whose vestibular function and mechanical sensitivity have declined. Thus, B81-BCV stimulation is more effective and safer to elicit oVEMPs, and it should be recommended when ACS fails in the clinic, particularly in the elderly population.

[Perioperative management of cochlear implantation for CHARGE syndrome].

Objective:To explore the perioperative period characteristics of paediatric cochlear implant recipients of CHARGE syndrome with complex deformities. Methods:Retrospective case series of CHARGE syndrome were included. Radiological results, intraoperative findings, surgical planning and post-operative complications were analyzed. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre and post-operatively to measure auditory speech rehabilitation outcomes. Results:Five prelingual profoundly deaf children were identified, aged from 14 months to 60 months. All patients had congenital heart disease and underwent surgery before cochlear implantation. Upper airway abnormalities were detected as choanal atresia, laryngomalacia and tracheal stenosis. All ten ears showed cochlear abnormalities（Incomplete partition Ⅱ）, eight of them combined with secretory otitis media and/or middle ear deformity. All patients underwent single side surgery using standard transmastoid facial recess approach. Full insertion of the electrode was achieved in two cochleas, while partial insertion was done in three cochleas. Three ears with absent auditory nerves in MRI showed no response in the neural remote test. All patients had improved audio-speech performance with CAP scores 3.0±0.7 and 3.6±0.9, SIR scores 1.2±0.4 and 1.8±0.8, IT-MAIS scores 18.8±9.1 and 26.2±10.0, MUSS scores 2.2±2.4 and 7.2±8.3 after twelve months and twenty-four months follow up. Conclusion:Cochlear implantation in patients with CHARGE syndrome is a challenge in both its surgical and rehabilitation aspects due to multiple abnormalities. Adequate treatment planning is necessary for safe and effective surgery, including airway structures and intricate temporal bone landmarks.

Audiological characteristics and cochlear implant outcome in children with cochlear nerve deficiency.

Objectives: This study aimed to examine the audiological characteristics and validity of predicting outcomes of cochlear implants (CIs) in children with cochlear nerve deficiency (CND) based on the internal auditory meatus (IAM) nerve grading system. Methods: The audiological characteristics of 188 ears in 105 children diagnosed with CND were analyzed based on the IAM nerve grading system. In addition, 42 children with CND who underwent CI were also divided into four groups based on the system, and their auditory and speech performance at baseline (preoperative) and 6, 12, and 24 months after CI were analyzed and compared with those of the control group (n = 24) with a normal cochlear nerve (CN) and CI. Results: The audiological test results showed no significant differences among the four CND groups in terms of elicited rates of distortion product otoacoustic emission (DPOAE) (p = 1.000), auditory brainstem response (ABR) (p = 0.611), and cochlear microphonic (CM) (p = 0.167). Hearing in the CND IV group was significantly better than that in the CND I group (p < 0.05). In children with CI, the auditory and speech performance of the control group was significantly higher than all CND groups from 6 to 24 months (p < 0.05) and 12 to 24 months (p < 0.05), respectively. Meanwhile, there were no significant differences between each pair group in the four CND groups (p > 0.05). Conclusion: Children with CND, including those in whom the CN was not visualized by MRI, can benefit from CI. Additionally, the IAM nerve grading system could not predict the outcomes of CI in children with CND.

Spoken word recognition in noise in Mandarin-speaking pediatric cochlear implant users.

OBJECTIVE: The purpose of the present study was to compare spoken word recognition performance in the presence of speech spectrum-shaped noise and four-talker babbles in Mandarin-speaking children with cochlear implants (CIs). METHODS: Participants included 33 children with unilateral CIs (with a mean age of 10.4 ± 2.9 years old and a mean length of CI use of 7.5 ± 3.0 years). The Standard Chinese version of Lexical Neighborhood Test was implemented in quiet, speech-spectrum-shaped noise (SSN), and four-talker babble (FTB). The signal-to-noise ratios (SNRs) were set at +5 and + 10 dB for both types of maskers. Participants responded by verbally repeating each word they heard and the response was scored as the percentage accuracy of recognition performance. A Generalized Linear Model (GLM) fitting, correlational tests, and a two-way repeated-measures ANOVA were conducted on the percent-correct data. RESULTS: Word recognition in quiet was on average 74.5% correct but dropped to 57.3% and 48.8% correct for SSN and FTB at 10 dB SNR, respectively, and 44.4% and 32.6% correct for SSN and FTB at 5 dB SNR, respectively. In both quiet and noise conditions, the participants showed lower recognition accuracy for the hard words than for the easy words. Disyllabic words were recognized with higher accuracy rates than were the monosyllabic words. The GLM analysis revealed that all four tested factors (masker type, SNR, lexical neighborhood feature, and lexical type) showed significant impacts on word recognition in children with CIs. Word recognition scores in the two types of maskers were significantly correlated for the disyllabic words at both SNRs and monosyllabic words at 10 dB SNR. CONCLUSIONS: The present study demonstrated that the lexical features such as the lexical neighborhood characteristics and lexical type had significant effects on speech recognition performance in both quiet and noise conditions in pediatric CI users. Children with years of experience of CI use still encountered remarkable difficulties in everyday listening environment although their speech recognition in quiet reached relatively desired level. Fluctuating noise, such as speech babbles, caused greater challenge than steady-state noise for speech recognition in children with CIs.

Effects of nonlinear frequency compression on the acoustic properties and recognition of speech sounds in Mandarin Chinese.

The present study examined the change in spectral properties of Mandarin vowels and fricatives caused by nonlinear frequency compression (NLFC) used in hearing instruments and how these changes affect the perception of speech sounds in normal-hearing listeners. Speech materials, including a list of Mandarin monosyllables in the form of /dV/ (12 vowels) and /Ca/ (five fricatives), were recorded from 20 normal-hearing, native Mandarin-speaking adults (ten males and ten females). NLFC was based on Phonak SoundRecover algorithms. The speech materials were processed with six different NLFC parameter settings. Detailed acoustic analysis revealed that the high front vowel /i/ and certain compound vowels containing /i/ demonstrated positional deviation in certain processed conditions in comparison to the unprocessed condition. All five fricatives showed acoustic changes in spectral features in all processed conditions. Fourteen Mandarin-speaking, normal-hearing adult listeners performed phoneme recognition with the six NLFC processing conditions. When the cut-off frequency was set relatively low, recognition of /s/ was detrimentally affected, whereas none of the NLFC processing configurations affected the other phonemes. The discrepancy between the considerable acoustic changes and the negligible adverse effects on perceptual outcomes is partially accounted for by the phonology system and phonotactic constraints in Mandarin.

[The research of rehabilitation effect of cochlear implantation for deaf children with gene mutation].

OBJECTIVE: To assess the evaluation on auditory rehabilitation effect for 42 deaf children with GJB2 gene mutation after cochlear implantation to provide a reference for the cochlear implant effect evaluation of such patients. METHOD: To conduct the detection on common genetic deafness gene mutation hotspots of hearing impaired children with cochlear implantation. To conduct auditory rehabilitation effect evaluation on 42 cases of patients with GJB2 genetic deafness after 3 months, 6 months and 12 months of the operation respectively. The single factor repeated measure ANOVA was applied to analyze whether there were significant difference among the results of initial consonant of a Chinese syllable recognition at 3 different stages after the operation, the results of vowel of a Chinese syllable recognition at 3 different stages after the operation, and the results of two-syllable recognition at 3 different stages after the operation. RESULT: 235delC is the high-incidence mutational site in 42 cases of patients with GJB2 genetic deafness, the total detection rate is up to 90.48%. There were significant differences in the initial consonant of a Chinese syllable recognition rate, the vowel of a Chinese syllable recognition rate, the two-syllable recognition rate as well as the vowel of a Chinese syllable recognition rate after 3 months, 6 months and 12 months of the operation (P < 0.01). CONCLUSION: Cochlear implantation is a safe and effective measure for auditory reconstruction, it can help patients with GJB2 hereditary severe sensorineural deafness to improve auditory speech recognition.

Lexical and age effects on word recognition in noise in normal-hearing children.

OBJECTIVES: The purposes of the present study were (1) to examine the lexical and age effects on word recognition of normal-hearing (NH) children in noise, and (2) to compare the word-recognition performance in noise to that in quiet listening conditions. METHODS: Participants were 213 NH children (age ranged between 3 and 6 years old). Eighty-nine and 124 of the participants were tested in noise and quiet listening conditions, respectively. The Standard-Chinese Lexical Neighborhood Test, which contains lists of words in four lexical categories (i.e., dissyllablic easy (DE), dissyllablic hard (DH), monosyllable easy (ME), and monosyllable hard (MH)) was used to evaluate the Mandarin Chinese word recognition in speech spectrum-shaped noise (SSN) with a signal-to-noise ratio (SNR) of 0dB. A two-way repeated-measures analysis of variance was conducted to examine the lexical effects with syllable length and difficulty level as the main factors on word recognition in the quiet and noise listening conditions. The effects of age on word-recognition performance were examined using a regression model. RESULTS: The word-recognition performance in noise was significantly poorer than that in quiet and the individual variations in performance in noise were much greater than those in quiet. Word recognition scores showed that the lexical effects were significant in the SSN. Children scored higher with dissyllabic words than with monosyllabic words; "easy" words scored higher than "hard" words in the noise condition. The scores of the NH children in the SSN (SNR=0dB) for the DE, DH, ME, and MH words were 85.4, 65.9, 71.7, and 46.2% correct, respectively. The word-recognition performance also increased with age in each lexical category for the NH children tested in noise. CONCLUSIONS: Both age and lexical characteristics of words had significant influences on the performance of Mandarin-Chinese word recognition in noise. The lexical effects were more obvious under noise listening conditions than in quiet. The word-recognition performance in noise increased with age in NH children of 3-6 years old and had not reached plateau at 6 years of age in the NH children.