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.

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.

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.

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.

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.

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.

Prenatal exposure to polychlorinated biphenyls and breastfeeding: opposing effects on auditory P300 latencies in 9-year-old Dutch children.

Effects of perinatal exposure to polychlorinated biphenyls (PCBs) on auditory P300 latencies and amplitudes were evaluated in children from a Rotterdam cohort. From this cohort of healthy, term babies, the 26 lowest and 26 highest prenatally PCB-exposed children from the breastfed and the formula-fed groups (n=104) were invited for P300 assessment when they were 9 years of age. For P300 assessment an auditory simple odd-ball paradigm was used. In the 83 participating children, 60 assessments (32 males, 28 females) satisfied the measurement criteria and were included in the data analyses. After adjusting for confounding variables, children with high prenatal exposure were found to have longer P300 latencies than children with low prenatal exposure. Lactational exposure to PCBs through breastfeeding milk was not related to P300 latencies. P300 latencies were shorter in children breast-fed for at least 16 weeks than in children breastfed for 6 to 16 weeks and formula-fed children. P300 amplitudes were not related to perinatal PCB exposure nor breastfeeding. Results of this exploratory study suggest that prenatal exposure to environmental levels of PCBs and related compounds delays mechanisms in the central nervous system that evaluate and process relevant stimuli, whereas breastfeeding accelerates these mechanisms.

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.

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.

A cephalometric analysis of nasal septal growth.

In a study using lateral cranial radiographs of 454 males and 475 females (age 0-20 years) the anterior nasal spine-nasion (ANS-N) and ANS-posterior nasal spine (ANS-PNS) distance were measured and related to age and gender. The development of both dimensions is best described by negative exponential functions. ANS-PNS distance to the age of 5 and the ANS-N distance to the age of 2, develop linearly without gender difference. After this, deceleration, statistically more significant in females (P < 0.01, ANCOVA non-linear regression analysis) occurs for both parameters. Although a study of this type does not have maximal sensitivity, no significant calendar-age-related 'growth spurts' were detected.

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.

Neonatal hearing screening.

UNLABELLED: Severe congenital hearing impairment is an important handicap affecting 0.1% of live-born infants and 1%-2% of graduates of Neonatal Intensive Care Units. The prognosis for intellectual, emotional, language and speech development in the hearing-impaired child is improved when the diagnosis is made early and intervention is begun before the age of 6 months. The usual age at diagnosis of hearing impairment is at least 18-30 months (or even later in cases of less severe hearing impairment) where there are no screening programmes. When screening is carried out using distraction methods at the age of approximately 9 months some hearing-impaired infants are missed and those discovered are at least 15-18 months before intervention begins. Neonatal screening could give hearing-impaired children the best chances for optimal care and development. Universal neonatal hearing screening is necessary, because, when neonatal hearing screening is restricted to high risk groups 30%-50% of infants with hearing loss are not discovered. The methods available for neonatal hearing screening are discussed in this paper. CONCLUSION: In our view automated measurement of auditory brainstem responses is the most valuable method for universal neonatal hearing screening.

Click-evoked oto-acoustic emissions in very-low-birth-weight infants: a cross-sectional data analysis.

For the purposes of studying the phenomenon of evoked oto-acoustic emissions (EOAEs) in very-low-birth-weight (VLBW) infants, and the conditions affecting the utility of EOAE ear screening in this population, click EOAEs were repeatedly recorded in ears of 144 VLBW infants, at different postconceptional ages of the infants and at two different test sites, i.e. in the neonatal high-care unit (ward), or at the neonatal outpatient clinic. The postconceptional age of the infants examined in the ward was 30-49 weeks and 37-66 weeks for the infants examined at the outpatient clinic. Overall 840 recording attempts were done. In the ward 86% of these attempts (388) were successful against 60% (of 452 attempts) at the outpatient clinic. In the latter group of infants the success rate of recording was only 33% at the corrected age of 6 months, which is significantly less than the 66% until the corrected age of 3 months. For a cross-sectional analysis of age effects one ear of each successfully recorded infant was selected. Analysis of the 127 successful recordings revealed that the EOAE prevalence was 71% in the ward (54% for infants receiving extra oxygen per naso) and 91% at the outpatient clinic. Compared with healthy newborns, VLBW infants are much more difficult to test, especially at the outpatient clinic. However, the EOAE prevalence at this test site is the highest and approaches that in healthy newborns. At the outpatient clinic response levels of EOAEs recorded approach levels found in healthy newborns. The higher success rate of recording in the ward and the lower EOAE prevalence are two counteracting factors as to the utility of EOAE-based ear screening of VLBW infants.

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)

Click-evoked oto-acoustic emissions in 1036 ears of healthy newborns.

Click-evoked oto-acoustic emissions (EOAEs) were recorded in 1036 ears of healthy newborns and in 71 normal-hearing adult ears. Newborns aged between 3 and 238 h were examined in a separate but not silent room of the obstetric ward. The adults were tested in a quiet but not sound-treated room. The recordings were more difficult in the newborn than in the adult, which was mirrored in recording parameters such as the time required for measurement (up to 7 min in newborns vs. 1-2 min in adult ears). Recording was always successful in adults, while retests were necessary in 4% of newborns. Also the artefact-rejection level and the stimulus stability were more favourable in adults. Still, EOAE recording for screening purposes in newborns seems feasible. Response levels in newborns (range 1.6-38.6; mean 20.2 dB SPL) appear to be higher than in adults (range 2.7-20.6; mean 12.8 dB SPL). The overall prevalence of EOAEs in newborns amounted to 93.4% and appeared to be age related. It rises from 78% in ears from newborns younger than 36 h to 99% in ears of newborns older than 108 h. This rise may be related to the middle ear clearance of amniotic fluid in the first days post partum. The prevalence in newborns older than 3-4 days is comparable with the prevalence of 97.2% in adults. Therefore, newborns should not be screened before the age of 4 days. In search of an objective EOAE detection variable, the prevalence of EOAEs for different age groups was calculated for various criterion values of reproducibility. These prevalences were compared to subjectively scored EOAE prevalences in the same age groups. A reproducibility criterion of about 50% appears to be useful for mass screening in newborns.

The masked threshold to noise ratio in brainstem electric response audiometry: assessment of the conductive loss component by bone-conducted masking.

The aim of this study was to assess the conductive loss component (CLC) by brainstem electric response audiometry. A bone-conducted noise was used to mask out the response to a conventional air-conducted click stimulus. The difference between the levels of the click and the noise is defined as the masked threshold to noise ratio (MTNR). This MTNR was determined for 21 normal ears (MTNR = -13 +/- 5 dB). The increase in MTNR compared to this normative value is a measure of the CLC. For 10 ears with an artificially induced purely conductive loss, the increase in MTNR is in good agreement with the results of conventional pure-tone and brainstem electric response audiometry.