Auditory processing and neuropsychological profiles of children with functional hearing loss.

OBJECTIVES: This paper compares structured history, auditory processing abilities and neuropsychological findings of children with functional hearing loss (FHL) to those with suspected auditory processing disorder without FHL (control). The main aim was to evaluate the value of a holistic assessment protocol for FHL used in a routine pediatric audiology clinic. The protocol incorporated a commercially available test battery for auditory processing disorder (APD), non-verbal intelligence (NVIQ) and tools to screen for common co-existing neurodevelopmental conditions such as attention deficit hyperactivity disorder (ADHD), language impairment (LI) and developmental coordination disorder (DCD). The outcome of such holistic assessment was expected to help in understanding the nature of FHL and to provide individualized support to mitigate their difficulties. METHODS: This retrospective study compared two groups, 40 children (M = 17, F = 23) in each group between seven and sixteen years of age, one group with a history of FHL and the other with suspected APD without FHL (control). The groups were matched against age, gender, hand use, diagnosis of APD or non-APD (31 with APD and 9 without APD in each group) and non-verbal intelligence. All the children were healthy English speaking children attending mainstream schools with no middle or inner ear abnormalities. Structured history was obtained from parents regarding different nonacademic and academic concerns. The SCAN-3:C and SCAN-3:A test batteries were used to assess auditory processing abilities; Lucid Ability test for NVIQ; Children's Communication Checklist-2 (CCC-2) for language ability; Swanson Nolan and Pelham-IV Rating Scale (SNAP-IV) for ADHD; and the manual dexterity components of the Movement Assessment Battery for Children-2 (MABC-2) as a screening tool for DCD. RESULTS: About 60% of children in both the groups had concerns regarding listening in noisy background. In the history, poor attention was reported in 45% of children in the FHL group compared to 82.5% in the control group (p < 0.01). Hyperacusis was present in 35% of children in the FHL group and in 62% of children in the control group (p < 0.05). Concerns about overall academic abilities were present in 59% of children in the FHL group and 75% of the controls (p > 0.05). Only 15% of children in the FHL group had concerns with numeracy skills in contrast to 41% of the controls (p < 0.05). Significantly fewer (p < 0.01) children in the FHL group (41%) received additional support at school than the controls (75%). Fewer children performed poorly in Filtered Words (FW) test of the SCAN-3 batteries, 30% in the FHL group and 17.5% in the control group, in contrast to Auditory Figure Ground 0 (AFG0), 85% in FHL and 80% in the control group. The number of children performing poorly in AFG0 was significantly higher compared to all the other SCAN-3 tests in FHL (P < 0.05), in contrast to FW and Competing Sentences (CS) only in the control group (p < 0.05). The control group had higher prevalence of atypical ear advantage (AEA) in left directed Competing Words (CW) (32.5%) and Time Compressed Sentences (TCS) (32.5%) compared to FW (7.5%). In contrast, FHL group had higher prevalence of AEA in AFG0 (48.7%) compared to CS (21%). High proportions of children in both the groups had LI (80% in FHL and 82.5% in the control group), with significantly lower (p < 0.05) levels of ADHD symptoms in the FHL group (39.5%) compared to the control group (72.5%). Impaired manual dexterity was present in 30.7% of children in FHL group and 47.5% in the controls. CONCLUSIONS: The prevalences of APD and language impairment are high compared to ADHD symptoms in children with FHL, and holistic assessment is recommended. Despite some similarities in the auditory and neuropsychological profiles between children with FHL and those with suspected APD without FHL some differences were noted. The results suggest that children with FHL have genuine difficulties that need to be identified and addressed. Future research is required to identify the neural pathways which could explain the similarities and dissimilarities between the two groups.

Author Response to Peck (2018), "Questionable Use of 'Nonorganic' in 'Estimating Nonorganic Hearing Thresholds'".

PURPOSE: The purpose of this letter is to respond to Dr. Peck's (2018) letter to the editor regarding the use of the term "nonorganic" to describe hearing loss, demonstrated by the pure tone audiogram that cannot be explained or is greater than what can be explained by a physiological auditory disorder. CONCLUSIONS: We prefer the term "nonorganic" rather than the term "false and exaggerated hearing loss." "Nonorganic," in our view, is a nonjudgmental term and, as stated by Austen and Lynch (2004), implies "as little as possible about its cause" (p. 450).

Questionable Use of "Nonorganic" in Norrix et al. (2017), "Estimating Nonorganic Hearing Thresholds".

PURPOSE: The author of this letter to the editor expresses concern about the use of the word "nonorganic" as a source of confusion in terminology. Specifically, this is in response to the December 2017 American Journal of Audiology article, "Estimating Nonorganic Hearing Thresholds Using Binaural Auditory Stimuli" (Norrix, Rubiano, & Mueller, 2017). "Nonorganic" is a source of confusion in terminology, because it can be used in two different ways. One way can mean to say there is no hearing loss. When used in this sense, it is illogical because it is qualifying a hearing loss believed not to exist. The second usage means there is a real disorder of function, but the organs themselves are not damaged and the basis is unknown. In the place of "nonorganic," I have proposed "false hearing loss." "Nonorganic" might carry a negative connotation that "false" might not. Many instances of false hearing loss stem from physical-mental health disturbances. Audiologists must stay alert to signs of psychosocial difficulty and refer for further evaluation accordingly. CONCLUSION: "False" hearing loss is a more appropriate term than "nonorganic" hearing loss.

[Pseudohypoacusis associated with Munchausen's syndrome]. / Psevdogipoakuziia pri sindrome Miunkhgauzena.

The authors describe a rare clinical case of psychogenic hearing loss in a female patient presenting Munchausen's syndrome with special reference to the objective and subjective methods designed to evaluate the hearing ability and used for diagnostics of this condition.

Estimating Nonorganic Hearing Thresholds Using Binaural Auditory Stimuli.

PURPOSE: Minimum contralateral interference levels (MCILs) are used to estimate true hearing thresholds in individuals with unilateral nonorganic hearing loss. In this study, we determined MCILs and examined the correspondence of MCILs to true hearing thresholds to quantify the accuracy of this procedure. METHOD: Sixteen adults with normal hearing participated. Subjects were asked to feign a unilateral hearing loss at 1.0, 2.0, and 4.0 kHz. MCILs were determined. Subjects also made lateralization judgments for simultaneously presented tones with varying interaural intensity differences. RESULTS: The 90% confidence intervals, calculated for the distributions, indicate that the MCIL in 90% of cases would be expected to be very close to threshold to approximately 17-19 dB poorer than the true hearing threshold. How close the MCIL is to true threshold appears to be based on the individual's response criterion. CONCLUSIONS: Response bias influences the MCIL and how close an MCIL is to true hearing threshold. The clinician can never know a client's response bias and therefore should use a 90% confidence interval to predict the range for the expected true threshold. On the basis of this approach, a clinician may assume that true threshold is at or as much as 19 dB better than MCIL.

Management of non-organic hearing loss in children - A case study.

A 10 year-old girl was admitted due to the claim of progressively developing hearing loss. The impedance audiometry showed no abnormalities but it was impossible to obtain reliable outcomes during pure tone audiometry assessment. The girl was additionally sent for speech audiometry, indicating a bilateral hearing loss and objective evaluations such as distortion product otoacoustic emissions and auditory brainstem responses, which results indicated a normal hearing. On the second day, repeated subjective audiometric tests showed also normal hearing, despite constantly reported hearing loss. After the psychological consultation and exclusion of neurologic pathology, the diagnosis of non-organic hearing loss was stated and the girl was scheduled for regular appointments with psychologist.

Childhood psychogenic hearing loss: Identification and diagnosis.

Psychogenic hearing loss, formerly known as functional or non-organic hearing loss, is a classic cause of consultation in infantile audiology. Risk factors include female gender, and age 8 or 12 years. Onset is relatively sudden, without impact on schooling or voice quality. Audiometric signs comprise non-superimposable audiometric thresholds (variable audiometric results), bilaterality, flat mean audiometric curve, and discrepancy between pure-tone and speech audiometry. The child needs reassuring during audiometric examination: attention-diversion techniques may be effective. Objective audiometry allows positive diagnosis, followed by rehabilitation and psychological care.

Inside implant criteria or not? - Detection of non-organic hearing loss during cochlear implant assessment.

OBJECTIVES: The diagnosis of non-organic hearing loss (NOHL) is a difficult but important issue during the assessment process for cochlear implantation (CI). We aim to identify the key factors in identifying patients with NOHL during CI assessment and present our local screening protocol for NOHL. METHODS: A retrospective review of patients referred to the Yorkshire Auditory Implant Service (YAIS) between 2003 and 2015 who were subsequently diagnosed with NOHL during the assessment. Patient demographic data, audiological and functional assessments were assessed. RESULTS: Thirty-two patients were included in the study. Mean age was 43 years (range 14-82 years). Male to female ratio was 1:1.7. Indicators of possible NOHL included a sudden deterioration in hearing (n = 21; 66%), mismatches in observed behaviour and either pure-tone audiogram (PTA) (n = 27; 84%) or functional testing (n = 20; 80%) and stapedial reflexes below reported audiological thresholds (n = 12; 46%). A mismatch in functional hearing and PTA was seen in 72% of patients. Patients with suspected NOHL were referred for further objective testing. All 23 patients who underwent objective testing had better hearing levels compared to reported hearing thresholds thus placing them outside of implant criteria. Five candidates were found to have normal hearing thresholds. DISCUSSION: NOHL can present a significant challenge to the implant team, particularly in the subgroup with a pre-existing organic hearing loss with non-organic overlay. We discuss the common features in this cohort of patients. CONCLUSIONS: To facilitate the identification of patients with NOHL, the YAIS has developed a screening protocol.

Assessment of the latest NGS enrichment capture methods in clinical context.

Enrichment capture methods for NGS are widely used, however, they evolve rapidly and it is necessary to periodically measure their strengths and weaknesses before transfer to diagnostic services. We assessed two recently released custom DNA solution-capture enrichment methods for NGS, namely Illumina NRCCE and Agilent SureSelect(QXT), against a reference method NimbleGen SeqCap EZ Choice on a similar gene panel, sharing 678 kb and 110 genes. Two Illumina MiSeq runs of 12 samples each have been performed, for each of the three methods, using the same 24 patients (affected with sensorineural disorders). Technical outcomes have been computed and compared, including depth and evenness of coverage, enrichment in targeted regions, performance in GC-rich regions and ability to generate consistent variant datasets. While we show that the three methods resulted in suitable datasets for standard DNA variant discovery, we describe significant differences between the results for the above parameters. NimbleGen offered the best depth of coverage and evenness, while NRCCE showed the highest on target levels but high duplicate rates. SureSelect(QXT) showed an overall quality close to that of NimbleGen. The new methods exhibit reduced preparation time but behave differently. These findings will guide laboratories in their choice of library enrichment approach.

Cochlear implant candidates with psychogenic hearing loss.

CONCLUSIONS: Specific requests for cochlear implantations by persons with psychogenic hearing loss are a relatively new phenomenon. A number of features seems to be over-represented in this group of patients. The existence of these requests stresses the importance of auditory brainstem response (ABR) measurements before cochlear implantation. OBJECTIVE: To describe the phenomenon of patients with psychogenic hearing losses specifically requesting cochlear implantation, and to gain first insights into the characteristics of this group. METHODS: Analysis of all cases seen between 2004 and 2013 at the University Hospital of Bern, Switzerland. RESULTS: Four cochlear implant candidates with psychogenic hearing loss were identified. All were female, aged 23-51 years. Hearing thresholds ranged from 86 dB to 112 dB HL (pure-tone average 500-4000 Hz). ABRs and otoacoustic emissions (OAEs) showed bilaterally normal hearing in two subjects, and hearing thresholds between 30 and 50 dB in the other two subjects. Three subjects suffered from depression and one from a pathologic fear of cancer. Three had a history of five or more previous surgeries. Three were smokers and three reported other close family members with hearing losses. All four were hearing aid users at the time of presentation.

Effective identification of functional hearing loss using behavioral threshold measures.

PURPOSE: Four functional hearing loss protocols were evaluated. METHOD: For each protocol, 30 participants feigned a hearing loss first on an audiogram and then for a screening test that began a threshold search from extreme levels (-10 or 90 dB HL). Two-tone and 3-tone protocols compared thresholds for ascending and descending tones for 2 (0.5 and 1.0 kHz) and 3 (0.5, 1.0, and 2.0 kHz) frequencies, respectively. A noise-band protocol compared an ascending noise-band threshold with that for 2 descending tones (0.5 and 1.0 kHz). A spondee protocol compared an ascending spondee threshold with that for 2 descending tones (0.5 and 1.0 kHz). These measures were repeated without the participants feigning losses. RESULTS: With nonfeigning participants, ascending and descending threshold differences were minimal for all protocols. When the participants feigned a loss, the spondee protocol produced the largest average threshold difference (30.8 dB), whereas the other protocols produced smaller differences (19.6-22.2 dB). CONCLUSIONS: Using both the screening test and a comparison of the initial audiogram with the screening test, the spondee and 3-tone protocols resulted in 100% true positives and 0% false positives for functional hearing loss. Either of these protocols could be used clinically or in occupational hearing conservation programs.

Pseudohypacusis in children: circumstances and diagnostic strategy.

OBJECTIVES: The study attempts to specify the circumstances under which we should pay attention to children's pseudohypacusis. It evaluates the methods used to detect such cases and to determine hearing thresholds, according to the uni-or bilateralism of hearing loss. The study finally deals with the future of children diagnosed with pseudohypacusis. METHODS: The study was retrospective from January 1993 to November 2011 and prospective from December 2011 to April 2012. We included all the children between 3 and 16 years who were diagnosed with pseudohypacusis. We observed the reasons for them to consult, whether they had already been tested or had treatment, and what kind of hearing loss they displayed. All children were tested using standard pure tone audiometry and speech audiometry. Depending on the first results, other tests were conducted. They included transient evoked otoacoustic emissions (TEOEs), auditory brainstem responses (ABR) and auditory steady state responses. Families were finally contacted by phone over April 2012 in order to let them know about their child's results. RESULTS: Fifty-four children were included: 19 boys and 35 girls, with an average age of 10 year-old (±3). The simulated hearing loss (HL) was bilateral (36), unilateral (18), of perception (37), moderate HL (33), cophosis (5). Fifteen cases were linked to a family or personal history of hearing loss, while 27 cases were due to important events like adoption, abuse, verbal aggression, school problems. Before diagnosing a pseudohypacusis, 13 children had had imaging studies, 3 had been treated with corticosteroids, and 5 had hearing aids. Most of the time the presence of pseudohypacusis was suspected a discrepancy between speech reception and air-conduction pure tone thresholds, as shown by the medical test (answer on whispered voice). The diagnosis was confirmed by ABR or TEOEs, except in cases where clinic was obvious. Then family's patient and patient were reassured and informed. An audiological follow-up during either 6 months or 1 year was proposed, as well as a psychological consultation. CONCLUSION: Complementary examinations have to be performed to rule out a pseudohypacusis case before suggesting an invasive or expensive treatment (surgery or hearing aids) of children.

Nonorganic hearing loss in children: audiometry, clinical characteristics, biographical history and recovery of hearing thresholds.

OBJECTIVES: The term "nonorganic hearing loss" (NOHL) (pseudohypacusis, functional or psychogenic hearing loss) describes a hearing loss without a detectable corresponding pathology in the auditory system. It is characterized by a discrepancy between elevated pure tone audiometry thresholds and normal speech discrimination. The recommended audiological management of NOHL in children comprises history taking, diagnosis, and counseling. According to the literature, prognosis depends on the severity of the patient's school and/or personal problems. Routine referral to a child psychiatrist is discussed as being controversial. METHODS: The clinical history of 34 children with NOHL was retrospectively evaluated. In 15 children, follow up audiometry was performed. Results of biographical history, subjective and objective audiometry, additional speech and language assessment, psychological investigations and follow up audiometry are presented and discussed. RESULTS: The prevalence of NOHL was 1.8% in children with suspected hearing loss. Mean age at diagnosis was 10.8 years. Girls were twice as often affected as boys. Patient history showed a high prevalence of emotional and school problems. Pre-existing organic hearing loss can be worsened by nonorganic causes. Children with a fast recovery of hearing thresholds (n=6) showed a high rate (4/6) of family, social and emotional problems. In children with continuous threshold elevation (n=9), biographical history showed no recognizable or obvious family, social or emotional problems; learning disability (4/9) was the most frequently presented characteristic. CONCLUSIONS: Due to advances in objective audiometry, the diagnosis of NOHL is less challenging than management and counseling. Considering the high frequency of personal and school problems, a multidisciplinary setting is helpful. On the basis of our results, drawing conclusions from hearing threshold recovery on the severity of underlying psychic problems seems inappropriate. As a consequence, a referral to a child psychiatrist can be generally recommended.

[Clinical features associated with sudden hearing loss in children].

Sudden sensorineural hearing loss is usually unilateral, and the cause is not identified in most adult cases. However, a specific cause has frequently been found in the case of children, in whom idiopathic sudden sensorineural hearing loss (ISHL) is comparatively rare. We investigated 20 cases of acute unilateral sensorineural hearing loss in children associated with a certain disease, but which was first diagnosed as ISHL. Thirteen patients aged 6 to 16 years old were diagnosed as having psychogenic (functional) hearing loss. Discrepancies in behavioral and objective tests are most valuable when functional hearing loss is suspected. Elevated pure-tone thresholds associated with normal distortion product otoacoustic emissions (DPOAEs) enable prompt further investigation. There are several conditions that may mimic functional hearing loss, so auditory brainstem response (ABR) testing is necessary to verify the actual cause. The unilateral profound hearing loss in 2 patients aged 7 and 11 years old was due to asymptomatic mumps proven by detecting the mumps IgM antibody. Total hearing recovery in the 125-1000 Hz frequencies occurred in one case. In 5 patients aged 6 to 12 years old with acute hearing loss and vertigo, high resolution CT imaging showed an abnormally enlarged vestibular aqueduct on the affected side. Large vestibular aqueduct syndrome should be considered in acute high-frequency sloping hearing loss with an A-B gap at low frequencies.

Clinical features of functional hearing loss with inattention problem in Japanese children.

OBJECTIVE: Functional hearing loss is a condition in which hearing is lost without actual pathology. In children, inattention during pure tone audiometry may be due in part to functional hearing loss. This study examined the issue of inattention as a psychological trait by analyzing the clinical features of functional hearing loss children in Japan. METHODS: Using the ADHD-Rating Scale IV, 97 functional hearing loss children were screened for inattention (27 boys, 70 girls; mean age 9 years 5 months, range 5-17 years). Those with high levels of inattention (Inattention group) were compared with others (Attention group) for clinical features statistically. Furthermore observed psychological clinical features were described. RESULTS: 36.1% were categorized in the Inattention group, which had more boys, lower in age, and had more physical, developmental, and environmental problems than the Attention group. Two groups had very different psychological feature. CONCLUSIONS: In children's functional hearing loss, there exists a group with psychological trait of inattention. Three younger children 5-6 years old with attention problems showed no psychological problems, their FHL was considered to be caused by generalized maturation and development. Nearly all of the rest children showed psychological problems, supporting the notion that FHL in children is psychogenic in nature. Because clinical features in Inattention group children were different from the Attention group significantly, it was concluded that distinguishing the Inattention group as a subtype of functional hearing loss in children would be effective for both diagnosis and treatment. Larger scale studies with many angles needed for the inattention problem in FHL children.

Pure tone auditory thresholds can change according to duration of interrupted tones in patients with psychogenic hearing loss.

CONCLUSION: Pure tone auditory thresholds can change according to duration of interrupted tones in patients with mild to severe psychogenic hearing loss (PHL). OBJECTIVES: To examine how the duration of stimulus tones affects the hearing thresholds of patients with PHL. METHODS: Twelve patients with PHL (21 ears) were enrolled in this study. We initially measured their hearing thresholds using interrupted tones with a duration of 2 s and equal length of on-time and off-time, 225 ± 35 ms, respectively. After a 10 min interval, we measured their hearing thresholds using the same interrupted tones conditions lasting 5 s. The average threshold gains (2 s thresholds minus 5 s thresholds) were compared to those of 15 control subjects with normal hearing (25 ears), 15 patients with cochlear hearing loss (23 ears), and 4 patients with retrocochlear lesions (4 ears). Patients with profound PHL (4 patients, 6 ears) were analyzed separately. RESULTS: The average threshold gain of PHL patients (excluding profound PHL patients) at all frequencies was 18.3 dB, which was significantly larger than that of other groups: 0.3 dB (profound PHL patients), 3.8 dB (controls with normal hearing), 3.0 dB (patients with cochlear hearing loss), and 3.2 dB (patients with retrocochlear lesions).

Non-organic hearing loss: new and confirmed findings.

Although non-organic hearing losses are relatively rare, it is important to identify suspicious findings early to be able to administer specific tests, such as objective measurements and specific counseling. In this retrospective study, we searched for findings that were specific ti or typical for non-organic hearing losses. Patient records from a 6 year period (2003-2008) from the University ENT Department of Bern, Switzerland, were reviewed. In this period, 40 subjects were diagnosed with a non-organic hearing loss (22 children, ages 7-16, mean 10.6 years; 18 adults, ages 19-57, mean 39.7 years; 25 females and 15 males). Pure tone audiograms in children and adults showed predominantly sensorineural and frequency-independent hearing losses, mostly in the range of 40-60 dB. In all cases, objective measurements (otoacoustic emissions and/or auditory-evoked potentials) indicated normal or substantially better hearing thresholds than those found in pure tone audiometry. In nine subjects (22.5%; 2 children, 7 adults), hearing aids had been fitted before the first presentation at our center. Six children (27%) had a history of middle ear problems with a transient hearing loss and 11 (50%) knew a person with a hearing loss. Two new and hitherto unreported findings emerged from the analysis: it was observed that a small air-bone gap of 5-20 dB was typical for non-organic hearing losses and that speech audiometry might show considerably poorer results than expected from pure tone audiometry.

Acute-onset unilateral psychogenic hearing loss in adults: report of six cases and diagnostic pitfalls.

We encountered 6 rare cases of acute-onset unilateral psychogenic hearing loss in adults. All were women in their 20s and 30s. Three cases had severe hearing impairment characterized by hearing loss at every frequency; 2 cases had profound hearing impairment, and 1 case had low-frequency hearing impairment. Of the 6 cases, 3 had a history of hearing loss, and 1 had a history of psychogenic visual disturbance. All 6 cases were initially diagnosed as having idiopathic sudden sensorineural hearing loss; all subsequently received steroid therapy. Three cases were not diagnosed as being psychogenic in origin until otoacoustic emissions and auditory brain responses were performed. Although the presence of distinctive clinical features (age, gender, and past history) is important for suspecting psychogenic hearing loss, objective audiological tests such as otoacoustic emissions are essential for diagnosing some cases. Compared to the existing reports of similar cases, our cases had a poorer prognosis (only 2 cases were cured).