Auditory hallucinations in adults with hearing impairment: a large prevalence study.

BACKGROUND: Similar to visual hallucinations in visually impaired patients, auditory hallucinations are often suggested to occur in adults with hearing impairment. However, research on this association is limited. This observational, cross-sectional study tested whether auditory hallucinations are associated with hearing impairment, by assessing their prevalence in an adult population with various degrees of objectified hearing impairment. METHODS: Hallucination presence was determined in 1007 subjects aged 18-92, who were referred for audiometric testing to the Department of ENT-Audiology, University Medical Center Utrecht, the Netherlands. The presence and severity of hearing impairment were calculated using mean air conduction thresholds from the most recent pure tone audiometry. RESULTS: Out of 829 participants with hearing impairment, 16.2% (n = 134) had experienced auditory hallucinations in the past 4 weeks; significantly more than the non-impaired group [5.8%; n = 10/173; p < 0.001, odds ratio 3.2 (95% confidence interval 1.6-6.2)]. Prevalence of auditory hallucinations significantly increased with categorized severity of impairment, with rates up to 24% in the most profoundly impaired group (p < 0.001). The corrected odds of hallucination presence increased 1.02 times for each dB of impairment in the best ear. Auditory hallucinations mostly consisted of voices (51%), music (36%), and doorbells or telephones (24%). CONCLUSIONS: Our findings reveal that auditory hallucinations are common among patients with hearing impairment, and increase with impairment severity. Although more research on potential confounding factors is necessary, clinicians should be aware of this phenomenon, by inquiring after hallucinations in hearing-impaired patients and, conversely, assessing hearing impairment in patients with auditory hallucinations, since it may be a treatable factor.

Otologic and audiologic findings in 22q11.2 deletion syndrome.

Hearing loss is frequently present in the 22q11.2 deletion syndrome. Our aim was to describe the audiologic and otologic features of patients with 22q11.2 deletion syndrome. We conducted a retrospective cohort study in a single tertiary referral center. We reviewed medical files of all patients with 22q11.2 deletion syndrome who visited an otolaryngologist, plastic surgeon or speech therapist, for audiologic or otologic features. Hearing loss was defined as a pure tone average (of 0.5, 1, 2, and 4 kHz) of >20 decibel hearing level. Audiograms were available for 102 of 199 included patients, out of which 163 ears were measured in the required frquencies (0.5-4 kHz). Median age at time of most recent audiogram was 7 years (range 3-29 years). In 62 out of 163 ears (38%), hearing loss was present. Most ears had conductive hearing loss (n = 58) and 4 ears had mixed hearing loss. The severity of hearing loss was most frequently mild (pure tone average of ≤40 decibel hearing level). In 22.5% of ears, otitis media with effusion was observed at time of most recent audiogram. Age was not related to mean air conduction hearing thresholds or to otitis media with effusion (p = 0.43 and p = 0.11, respectively). In conclusion, hearing loss and otitis media are frequently present in patients with 22q11.2 deletion syndrome. Moreover, our results suggest that children with 22q11.2 deletion syndrome remain susceptible for otitis media as they age.

Factors that influence outcomes in cochlear implantation in adults, based on patient-related characteristics - a retrospective study.

OBJECTIVE: Outcomes in speech perception following cochlear implantation in adults vary widely. Many studies have been carried out to identify and quantify factors that influence outcomes. This study adds a new dimension to pre-existing literature. DESIGN: Single-centre retrospective cohort study. SETTING: University Medical Center Utrecht, the Netherlands. PARTICIPANTS: A total of 428 adults with bilateral severe-to-profound sensorineural hearing loss, unilaterally implanted between February 1988 and March 2014. MAIN OUTCOME MEASURES: Univariable and multivariable linear regression analyses were carried out to identify factors that may influence outcome after cochlear implantation. Consonant-vowel-consonant word scores were recorded pre- and post-implant and were used as outcome measure in two groups of patients (prelingually and postlingually deafened adults). As an added dimension, multiple imputation was implemented and evaluated to tackle 4% (17/407) missing data. RESULTS: For postlinguals, pre-implant speech perception score and age at onset of deafness are positive predictors and meningitis and otosclerosis as cause of deafness are negative predictors of post-implant speech perception. This model accounted for 26% of variance. For prelinguals, pre-implant speech perception score is the only strong positive predictor (ß 0.524; P < 0.001). This model accounted for 31% of variance. Age at implantation was not a significant predictor in either group. CONCLUSIONS: Speech perception is predicted by pre-implant speech perception, age at onset of deafness and aetiology (meningitis and otosclerosis) for postlinguals and solely pre-implant speech perception for prelinguals. Age at implantation is of lesser importance in predicting speech perception outcome post-implant. Multiple imputation is a useful statistical technique when analysing incomplete data sets.

Agreement between health utility instruments in cochlear implantation.

OBJECTIVES: The objectives of our study were threefold: to compare health utility scores measured with different health utility instruments in adult patients with bilateral deafness, to compare the change in health utility scores after unilateral or bilateral cochlear implantation using the different health utility instruments and to assess which health utility instrument would be the most appropriate for future studies on cochlear implantation. DESIGN: A prospective study. SETTING: The data for this article were collected as part of a multicentre randomised controlled trial in the Netherlands on the benefits of simultaneous bilateral cochlear implantation compared to unilateral cochlear implantation. PARTICIPANTS: The study included 38 adult patients with severe to profound bilateral post-lingual sensorineural hearing loss. MAIN OUTCOME MEASURES: Participants completed various quality of life questionnaires (the EuroQol five-dimensional questionnaire (EQ-5D), the Health Utilities Index mark 3 (HUI3), a visual analogue scale (VAS) for general quality of life and a VAS for hearing) preoperatively, and one and two years postoperatively. The general health utility instruments (EQ-5D, HUI3 and VAS general) were compared. RESULTS: The EQ-5D, HUI3 and VAS general utility scores differed significantly. The intraclass correlation coefficients showed poor to no agreement between these instruments. A gain in health utility after cochlear implantation was found with the HUI3 and VAS general. The highest gain in health utility was found with the HUI3. CONCLUSIONS: A health utility score depends on the health utility instrument that is used in a specific patient population. We recommend using the HUI3 in future studies on cochlear implantation.

Association of Behavior With Noise-Induced Hearing Loss Among Attendees of an Outdoor Music Festival: A Secondary Analysis of a Randomized Clinical Trial.

Importance: To date, factors associated with noise-induced hearing loss at music festivals have not yet been analyzed in a single comprehensive data set. In addition, little is known about the hearing loss-associated behavior of music festival attendees. Objectives: To assess which factors are associated with the occurrence of a temporary threshold shift (TTS) after music exposure and to investigate the behavior of music festival attendees. Design, Setting, and Participants: This prospective post hoc analysis gathered data from a randomized, single-blind clinical trial conducted on September 5, 2015, at an outdoor music festival in Amsterdam, the Netherlands. Adult volunteers with normal hearing were recruited via social media from August 26 through September 3, 2015. Intention to use earplugs was an exclusion criterion. Of 86 volunteers assessed, 51 were included. This post hoc analysis was performed from October 3, 2016, through February 27, 2017. Interventions: Music festival visit for 4.5 hours. Main Outcomes and Measures: The primary outcome was a TTS on a standard audiogram for the frequencies 3.0- and 4.0-kHz. Multivariable linear regression was performed to determine which factors are associated with a TTS. A questionnaire on behavior, hearing, and tinnitus was distributed to the participants before and after the festival visit. Results: A total of 51 participants were included (18 men [35%] and 33 women [65%]) with a mean (SD) age of 27 (6) years. Mean (SD) threshold change across 3.0 and 4.0 kHz was 5.4 (5.7) dB for the right ear and 4.0 (6.1) dB for the left ear. Earplug use (absolute difference in the left ear, -6.0 dB [95% CI, -8.7 to -3.2 dB]; in the right ear, -6.4 dB [95% CI, -8.8 to -4.1 dB]), quantity of alcohol use (absolute difference per unit in the left ear, 1.1 dB [95% CI, 0.5 to 1.7 dB]; in the right ear, 0.7 dB [95% CI, 0.1 to 1.4 dB]), drug use (absolute difference in the right ear, 6.0 dB [95% CI, 0.9 to 11.1 dB]), and male sex (absolute difference in the right ear, 4.1 dB [95% CI, 0.3 to 5.9 dB]) were independently associated with hearing loss, with earplug use being the most important factor. Unprotected participants reported significantly worse subjective hearing performance and tinnitus after the festival visit than did participants using earplugs (Cramer V, 0.62 [95% CI, 0.47-0.79] and 0.39 [95% CI, 0.16-0.62], respectively). In the earplug group, the perceived loudness (r = -0.72; 95% CI, -1.00 to -0.43) and appreciation (r = 0.53; 95% CI, 0.29 to 0.78) of music and speech perception (r = 0.21; 95% CI, 0.09 to 0.35) were correlated with the duration of earplug use. Conclusions and Relevance: The present study identified nonuse of earplugs, use of alcohol and drugs, and male sex as associated with a TTS at an outdoor music festival. Physicians should consider these factors to raise awareness about the combined risk of attending music festivals without using earplugs while consuming alcohol and/or drugs. The intention to use earplugs was correlated with the loudness and appreciation of music with earplugs, which may advocate for the use of personalized earplugs. Trial Registration: trialregister.nl Identifier: NTR5401.

Costs of different strategies for neonatal hearing screening: a modelling approach.

OBJECTIVE: To compare the cost effectiveness of various strategies for neonatal hearing screening by estimating the cost per hearing impaired child detected. DESIGN: Cost analyses with a simulation model, including a multivariate sensitivity analysis. Comparisons of the cost per child detected were made for: screening method (automated auditory brainstem response or otoacoustic emissions); number of stages in the screening process (two or three); target disorder (bilateral hearing loss or both unilateral and bilateral loss); location (at home or at a child health clinic). SETTING: The Netherlands TARGET POPULATION: All newborn infants not admitted to neonatal intensive care units. MAIN OUTCOME MEASURE: Costs per child detected with a hearing loss of 40 dB or more in the better ear. RESULTS: Costs of a three stage screening process in child health clinics are 39.0 pounds (95% confidence interval 20.0 to 57.0) per child detected with automated auditory brainstem response compared with 25.0 (14.4 to 35.6) pounds per child detected with otoacoustic emissions. A three stage screening process not only reduces the referral rates, but is also likely to cost less than a two stage process because of the lower cost of diagnostic facilities. The extra cost (over and above a screening programme detecting bilateral losses) of detecting one child with unilateral hearing loss is 1500-4000 pounds. With the currently available information, no preference can be expressed for a screening location. CONCLUSIONS: Three stage screening with otoacoustic emissions is recommended. Whether screening at home is more cost effective than screening at a child health clinic needs further study.

Aspects of spontaneous otoacoustic emissions in healthy newborns.

Spontaneous otoacoustic emissions (SOAEs) are pure-tone like signals, spontaneously present in the ear canal. In normal adult ears the prevalence of SOAEs is reported to be 30-70%, probably depending on the noise floor of the recordings. In infant studies, results on the SOAE prevalence are rare. SOAEs as well as evoked otoacoustic emissions (EOAEs) were recorded in healthy newborns. Their ages varied between 1 and 10 days. The recordings were done with commercially available equipment in a separate not sound treated room of the obstetric department. The prevalence of SOAEs was 78%, which is higher than previously reported for adults as well as healthy newborns. The prevalence was not significantly different between left and right ears, or genders. The number of emissions per emitting ear amounted on average 5.5. The median number of SOAEs in boys (3.3) is significantly lower than in girls (4.6). The SOAE levels were between -2 and 42 dB SPL. The mean level per emitting ear was 8.0 dB SPL and not significantly different between right and left ears or genders. However, the level of the strongest emission per emitting ear was significantly higher for right than for left ears. In contrast with adults most of the emissions (70%) are at frequencies above 2 kHz. Comparing the levels of the EOAEs between ears with and without SOAEs we found a statistically significant higher EOAE level in ears with SOAEs. This supports our previous hypothesis that the higher EOAE level found in healthy newborns is partly due to the more frequent presence of stronger SOAEs in healthy newborns.(ABSTRACT TRUNCATED AT 250 WORDS)

Hearing loss in middle-age persons with Down syndrome.

Hearing function of 35 institutionalized persons with Down syndrome, age 35 to 62 years, was assessed by means of otoscopy, impedance audiometry, brainstem evoked response audiometry, and pure tone audiometry. Using brainstem evoked response audiometry, we determined response thresholds for 59 ears, which compares favorably with pure tone audiometry (20 ears). We found hearing losses of 20 dB to over 90 dB in 56 of these ears. Hearing loss should be considered and, whenever feasible, excluded as a contributing factor in social and mental deterioration in middle-age persons with Down syndrome.

Hearing and language in preschool very low birthweight children.

To get more insight into preschool language and hearing in high-risk very low birthweight (VLBW) children, we conducted a prospective study in a cohort of 79 children. The prevalence of language impairment and hearing loss at age 3-4 years, their relationship to each other as well as to perinatal conditions, neurodevelopmental outcome and the home environment are described. Mild hearing loss was found in 26%, moderate hearing loss in 13% and severe hearing loss in 3% of the children. None of the children was deaf. Abnormal tympanometry was found in 57% of the children. Hearing loss at age 4 years was related to a less optimal neonatal condition and was not related to the obstetrical condition or to neonatal cerebral ultrasound findings. Language impairment was found in 21% of the children at age 3.6 years. Receptive and expressive language was not related to perinatal conditions. There was no relation between the language assessments and the audiological assessments. Cognition and the home environment of the child were the only independent variables in the prediction of language in preschool VLBW children.

A pilot case control follow-up study on hearing in children treated with tobramycin in the newborn period.

OBJECTIVE: To assess the occurrence of hearing loss in children due to neonatal exposure to long courses of tobramycin and/or high tobramycin serum concentrations. METHODS: This was a pilot case-control study in 3-4-year old children. Data on tobramycin administration were abstracted from the patient files of an earlier study. Patients exposed in the neonatal period to either long courses (>7 days) or high serum concentrations of tobramycin constituted the study group. The control group consisted of patients without tobramycin exposure. Patients were matched for other risk factor according to criteria of the joint committee on infant hearing. All patients underwent the following investigations: otoscopy and pneumatic otoscopy, followed by impedance audiometry, to exclude middle ear effusion. Click-evoked oto-acoustic emissions (ce-OAE) as well as distortion product oto-acoustic emissions (dp-OAE), tested at f2 frequencies ranging from 1 to 10 kHz, were measured to assess hearing. All patients with abnormal ce-OAE results underwent brainstem electric response audiometry (BERA) as well. Since aminoglycoside ototoxicity is usually bilateral, results were compared per patient and not per ear. RESULTS: A total of 29 patients were tested. Eleven patients were excluded due to middle ear effusion. Data for 18 patients were analyzed. In the tobramycin treated group (n=9) both ce-OAE and dp-OAE (at all tested frequencies) were not detectable in six ears of three patients. All other patients had normal ce-OAE's as well as normal dp-OAE's in this frequency range. Difference between the tobramycin treated and control group for OAE as well as dp-OAE showed a trend (P=0.08). In all three patients with undetectable emissions BERA confirmed a cochlear loss of 60-70 dB at 3 kHz in both ears. These three patients had the longest total exposure to tobramycin: 20-24 days and 84-92 mg/kg, respectively. No relation to either peak or trough serum concentrations could be detected. CONCLUSION: There was no statistical relation between hearing loss and tobramycin exposure, probably due to sample size. Our results do indicate a need for a case-control follow-up study of hearing in neonates exposed to long courses of aminoglycosides.

Growth characteristics of the human nasal septum.

Using a specially designed algorithm for the measurement of the surface area of shapes with highly irregular contours, growth curves were developed for post-natal septal growth in humans using post-mortem specimens of a study population of 30 cases, distributed over the age range from birth to 62 years. From the results a rapid growth phase for the total septum is evident immediately after birth, lasting until the second year of life. Then, a gradual deceleration of growth is recognized with a plateau eventually being reached at the age of 36 years. Mathematical analysis of the growth curve shows that the curve for the total septum is the sum of two separate mathematical equations, representing the cartilaginous and bony contribution, respectively. It is demonstrated that the cartilaginous septum reaches adult dimensions (lateral surface area) at the age of two years. Subsequent growth of the septum is due to expansion of the perpendicular plate, i.e. the bony parts of the septum.

Auditory neuropathy in a low-risk population: a review of the literature.

OBJECTIVE: Collect all available published evidence on the prevalence of auditory neuropathy in the well baby population and calculate the contribution of this to the false negative rate of oto-acoustic emission based newborn hearing screening programs. METHOD: PubMed and EMBASE were searched for relevant articles published between 1996 and 2010. Medical Subject Headings terms included 'Auditory disease', 'Prevalence' and 'Child' and their relevant synonyms. Included were original studies, which focused on well babies and reported the prevalence of auditory neuropathy. RESULTS: Of 519 citations 4 articles met the inclusion criteria. The population based prevalence of auditory neuropathy in children in population hearing screening was found to vary between 0.006% (SD 0.006) and 0.03% (SD 0.02). The false negative rate, caused by missed children with auditory neuropathy, is between 4 and 17%. CONCLUSION: The available information on the prevalence of auditory neuropathy in the well baby population is poor. However, if oto-acoustic emission screening is used in the first stage of a neonatal hearing screening program, children with auditory neuropathy are missed. The cost-effectiveness of population-based screening using auditory brainstem response should be studied.

A comparison of intra- versus post-operatively acquired electrically evoked compound action potentials.

The objective of this study was to compare the electrically evoked compound action potentials, intra- versus post-operatively, in cochlear implant patients. In a prospective study twenty-five consecutively implanted adult patients received a multichannel cochlear implant. In all patients, electrically evoked compound action potentials were recorded immediately after cochlear implantation and in a post-operative setting nine months later. The threshold of the electrically evoked compound action potential was determined in both settings. A high success rate (97.4%) was found in the intra-operative setting when recording the electrically evoked compound action potential threshold per patient. The success rate per patient was significantly lower (53.4%) in the post-operative setting. Correlations between the intra- versus the post-operative ECAP thresholds were statistically significant for all electrodes tested. The ECAP thresholds were not significantly different for the two settings. The intra-operative setting is preferable for acquisition of the ECAP threshold.

GATA3 haploinsufficiency causes a rapid deterioration of distortion product otoacoustic emissions (DPOAEs) in mice.

Human HDR (hypoparathyroidism, deafness and renal dysplasia)-syndrome is caused by haploinsufficiency of zinc-finger transcription factor GATA3. The hearing loss due to GATA3 haploinsufficiency has been shown to be peripheral in origin, but it is unclear to what extent potential aberrations in the outer hair cells (OHCs) contribute to this disorder. To further elucidate the pathophysiological mechanism underlying the hearing defect in HDR-syndrome, we investigated the OHCs in heterozygous Gata3-knockout mice at both the functional and morphological level. While the signal-to-noise ratios of distortion product otoacoustic emissions (DPOAE) in wild type mice did not change significantly during the first half-year of live, those in the heterozygous Gata3 mice decreased dramatically. In addition, both light microscopic and transmission electron microscopic analyses showed that the number of OHCs containing vacuoles was increased in the mutants. Together, these findings indicate that outer hair cell malfunctioning plays a major role in the hearing loss in HDR-syndrome.

Frequency-specific auditory brainstem responses to clicks masked by notched noise.

Frequency-specific auditory brainstem responses were recorded as a function of the intensity of a click masked by notch-filtered white noise (i.e. band-stop-filtered). The bandwidth of the notch was fixed at five-thirds octave and the center frequency was 0.5, 1.0, 2.0 or 4.0 kHz. For normally hearing subjects the latency of the main peak in the responses is increasing with decreasing center frequency of the notch and with decreasing click intensity. At a click sensation level of 60 dB, the mean of 7 subjects' peak latency was 6.7 ms for the center frequency 4.0 kHz and 10.2 ms for the center frequency 0.5 kHz. The thresholds derived from the frequency-specific responses were within 20 dB above the subjective thresholds of audibility of the clicks as heard when masked by notched noise.

Spectro-temporal modulation transfer function (STMTF) for various types of temporal modulation and a peak distance of 200 Hz.

For noise which is spectrally modulated (ripple noise) as well as temporally modulated (the ripples move), the Spectro-Temporal Modulation Transfer Function (STMTF) is defined as the curve relating modulation threshold to temporal modulation frequency with ripple spacing as a parameter. A white noise stimulus was spectro-temporally modulated in the 0.2- to 1.0-kHz region with a spectral modulation frequency (peak distance) of 0.2 kHz. Three types of temporal modulation were applied: the ripples rolled upward, downward, or the peaks and troughs interchanged periodically. It is shown that the STMTFs for these types are equal. So, for a ripple spacing of 0.2 kHz the spectro-temporal modulation thresholds are independent of the type of temporal modulation. These STMTFs appeared to be low-pass functions, no minimum was found. Their plateaus were at the same modulation depth as the modulation thresholds found by previous investigators who employed stationary ripple noise.

The relation between the pure-tone audiogram and the click auditory brainstem response threshold in cochlear hearing loss.

Auditory brainstem response thresholds for 209 ears with cochlear hearing loss were compared with the pure-tone thresholds. It is shown that the pure-tone threshold in the 2- to 4-kHz region has a one-to-one relationship with the auditory brainstem response threshold. Estimating the pure-tone threshold from the auditory brainstem response threshold, the standard error of the estimate is 11 dB. A small part of this estimation error is due to errors in the measurement of the auditory brainstem response threshold and the mean of the pure-tone thresholds at 2 and 4 kHz. The major part is due to unknown factors that are involved in the physiological relationship between the two thresholds.

Brainstem electric response audiometry in preterm infants.

In the Sophia Children's Hospital in Rotterdam 108 preterm infants were examined with Auditory Brainstem Response (ABR). Ears were categorized as 1) normal sensitivity for post-conceptional age (PCA), 2) probably-mild loss of sensitivity, or 3) a moderate to severe loss of sensitivity. All infants showed ABR peaks, so no "deaf" individuals were found. A bilaterally normal sensitivity was found in 61. A probably-mild loss was found in 22 infants and a moderate to severe loss in 21 infants. The type of loss was conductive in 31 and cochlear in 12. A moderate to severe bilateral cochlear loss was found in 2. This is in line with the literature. Of the 43 infants with a (probable) loss 18 were examined again at 3 months corrected age. Only half of the conductive losses had disappeared, the other half was exacerbated. About 2 out of 3 initially moderate to severe losses were confirmed at follow-up. The moderate to severe losses of the cochlear type were all confirmed.