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Pseudohypacusis in children: Circumstances and diagnostic strategy
International Journal of Pediatric Otorhinolaryngology 78 (2014) 1632–1636
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Pseudohypacusis in children: Circumstances and diagnostic strategy M. Drouillard a,b,*, N. Petroff a,b, J. Majer a,b, C. Perrot a,b, S. Quesnel a,b, M. Franc¸ois a,b ORL et CCF, CHU Robert Debre´, Paris, France a b
APHP Groupe hospitalier Robert Debre´, Pediatric Otorhinolaryngology Department, 75019 Paris, France Universite´ Paris 7, Denis Diderot, 5 rue Thomas Mann, 75013 Paris, France
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 April 2014 Received in revised form 4 July 2014 Accepted 7 July 2014 Available online 14 July 2014
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. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Non organic hearing loss Auditory brainstem response Deafness Diagnosis ASSR
1. Introduction Pseudohypacusis – also known as non-organic hearing loss (NOHL) – is defined as a discrepancy between the actual hearing thresholds of the patient and the admitted one. The word does not refer to the intentions, conscious or subconscious, of the patient who has been tested. Other words are used to describe it. They picture pseudohypacusis as malingering, functional hearing loss,
* Corresponding author at: APHP Groupe hospitalier Robert Debre´, Pediatric Otorhinolaryngology Department, Service ORL, hoˆpital Robert-Debre´, 48, Boulevard Se´rurier, 75019 Paris, France. Tel.: +33 0 1 40 03 22 22; fax: +33 0 1 40 03 22 02. E-mail address: [email protected] (M. Drouillard). http://dx.doi.org/10.1016/j.ijporl.2014.07.010 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
psychogenic hearing loss, feigning deafness, pseudohypacusis and conversion deafness. NOHL is the most neutral word to describe the situation. Since 1946, Doerfler and Stewart have started a discussion about the subclassifications of NOHL. The Austen and Lynch model makes use of three key diagnostic categories from DSM-IV linked by a continuum based on intent and gain. The first diagnosis is malingering. It refers to the intentional production of false – or grossly – exaggerated physical symptoms for self-gain (financial gain and drugs in order to avoid military service) [1,2]. The second diagnosis is factitious disorder, which states that the gain is intrapsychic rather than external. It refers to intentional production – or feigning of physical symptoms – when the motivation behind the behavior consists in assuming a sick role [1]. Finally, the third
M. Drouillard et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1632–1636
2. Methods The study concerned children from 3 to 16 year-old diagnosed with pseudohypacusis. This study was retrospective from January 1993 to November 2011 and prospective from December 2011 to April 2012. Mentally retarded children were not included in the study. A detailed history of language retardation, school difficulties, behavioral disorders, ear trauma, and family difficulties (for instance parents’ separation) was obtained. Furthermore, the purpose of children diagnosed with pseudohypacusis’ visit, the record of prior visits to our hospital or to another one for the same reason, as well as prior audiometric tests or treatments were recorded. The type and number of previous ‘‘false’’ or ‘‘faked’’ audiological evaluations was also taken into account. Every child had to go through an audiological evaluation in a sound treated booth. Tests were conducted with a Conera clinical audiometer (GN Otometrics, Denmark) for pure tone and speech audiometry through standard earphones. The hearing level was considered as being normal if the air-conduction threshold was better than 20 dB in the four speech frequencies 0.5, 1, 2 and 4 kHz. The speech stimuli were based on the Boorsma lists of words for children, and delivered by the Digivox program (Audivimedia, Paris, France) through a PC computer connected to the audiometer. Speech audiometry was considered as being normal when speech reception threshold fall under 20 dB with a 100% discrimination score. When judged necessary, tympanometry and acoustic reflex, transient evoked otoacoustic emissions (TEOE) and/or auditory brainstem responses (ABR) were recorded. We also used auditory steady state response (ASSR) for several cases in order to obtain objective responses on several frequencies. Tympanometry and acoustic reflex measurements were accomplished with an AZ7R impedance audiometer (Interacoustics, Assens, Denmark). TEOEs were recorded with the ILO92 Otodynamic analyzer measuring system (Otodynamics Ltd, London, England). The stimuli were not linear clicks, going from 69 to 85 dB SPL. TEOEs were identified on a global reproducibility greater than 75% and more than 3 dB of S/N ratio in at least 3 frequency bands [8]. Auditory brain stem responses (ABR) and auditory steady-state responses (ASSR) were recorded on an Eclipse (Interacoustics, Assens, Denmark). For ABR
recordings, the stimulus rate was 11 clicks/s, and 1600 clicks were presented for each stimulus level [9]. The disappearance of wave V while decreasing the intensity of the stimulus determined the threshold. For ASSR recordings, both ears were tested simultaneously on 4 frequencies (0.5, 1. 2, and 4 kHz), with a modulation frequency between 75 and 95 Hz. The children were divided into two groups according to the unior bilateralism of the supposed hearing loss and also according to where these children stand on a graduation going from mild to moderate, severe, profound and complete (cophosis). We contacted patient’s families. They underwent a prewritten phone interview about the hearing of their child, the wear of hearing aids after diagnosis of pseudohypacusis and the need for another doctor’s consultation. The reason and the duration of the simulation were also assessed. For those who consulted our psychologist we noted if he helped them and their child, if they thought it was important to see him and how he helped them. 3. Results During this period of time, 54 children between 3 and 16 yearold were referred to our institution because of audiological assessment. They were diagnosed with pseudohypacusis [Fig. 1]. They were 35 girls and 19 boys, with an average age of 10 years (3 months) (min: 4 years, max: 16 years). From November 2011 to April 2012, 9 children out of 145 who had been tested for deafness were diagnosed with pseudohypacusis. The incidence of pseudohypacusis during the prospective part of the study was 6.2%. 3.1. Past history of the children diagnosed with pseudohypacusis In 27 cases – half of our patients –, important events happened in the child’s life. Prior to their hearing loss complaint, two children had experienced a physical aggression, ten had difficulties at school, one had a family drama–the brutal death of one of his aunt, and one was examined within a context of abuse. Three children were experiencing parents’ separation. One child was raised in a foster family, while another one had leukemia. A girl suffered from anorexia and depression because of rheumatoid polyarthritis. Another girl had intense visceral pain. Four children had behavioral disorders (hyperactivity and aggressiveness), and two others thought of as having a bilateral hearing loss had language retardation. In our study fifteen children displayed otological antecedents. Four children had a family history of perceptive deafness, among them one had a brother with a progressive deafness since 2011. One child had already suffered a sudden unilateral deafness. Another child had an external otitis one week before the allegated hearing loss, and four children had a serous otitis media, which was [(Fig._1)TD$IG] 12 boy
Number of children
diagnosis stresses the fact that the patient unintentionally products symptoms in conversion disorder. Conflict or others stresses tend to precede the psychological change [1,3]. Pseudohypacusis also affects children [4–6]. In such cases, they are generally easier to diagnose than adults since children reproduce less consistently. However children’s diagnosis is often missed because of a lack of awareness regarding the possibility of the disease affecting them [7]. Each time a child displays poor tone thresholds, the examiner must verify that they are not the consequence of a misunderstanding or an unfamiliarity with the test procedure, or attention disorders. Children may have difficulty in paying attention to abstract stimuli such as tones. A simple method to improve the results in case an unfamiliarity occurs with the test procedure consists in repeating the test several times. Unfortunately, this is a time consuming process. The organicity of the hearing loss must be verified before any invasive examination or treatment take place. If the hearing loss is diagnosed as non-organic, the real hearing level must be evaluated. The aim of our study is to precise the circumstances under which we must pay attention to a pseudohypacusis case, as well as to evaluate the methods used to detect its presence in children and to determine their real hearing thresholds. It finally aims at analyzing the possible outcomes for these children.
1633
10
girl
8 6 4 2 0 4
5
6
7
8
9 10 11 12 13 14 15 16
Age (years) Fig. 1. Distribution of pseudohypacusis by gender and age.
[(Fig._3)TD$IG]
M. Drouillard et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1632–1636
1634
treated with tympanostomy tubes. One had a mastoiditis. Three children experienced head trauma, and one of them, who was a 14 year-old boy, complained of unilateral hearing loss after a street fight. Lastly, an 8 year-old girl complained of right hearing loss after one of her schoolmates shouted near her.
Number of children 40
35 30 25
3.2. Care pathway Out of these 54 children, 37 had priory consulted ENT private practionners (29) or ENT doctors in other hospitals (3). Five had already consulted the ENT department of our hospital. Prior to our investigation, 12 children had had a CT scan and/or a MRI of the ears (one had an appointment for it). Three children conducted a vestibular test. All these exams were evaluated as normal. Five children wore hearing aids and three received corticosteroids. Hearing aids were suggested to 7 other children. Finally, one child was enrolled in a cochlear implant program.
boy
15
girl
10 5 0
percepon conducvee mixed Fig. 3. Distribution of pseudohypacusis by type of the deafness (perceptive, conductive and mixed) and gender.
3.3. Kind of hearing loss and diagnostic tools
responses, yet TEOES were present in both ears. After explaining to the child the meaning of the presence of TEOEs, the air-conduction thresholds were also normal. In 7 cases diagnosis was made by ABR (wave V thresholds were normal in both ears), after explaining to the child the meaning of these results, another speech audiometry was performed whose responses were normal.
The supposed hearing loss was bilateral in 36 cases, while it was unilateral in 18 cases [Fig. 2].
[(Fig._2)TD$IG]
20
3.3.1. Bilateral hearing loss Thirty-six children complained of bilateral hearing loss and asked for repeat what we had just said when asked questions. The majority of simulated hearing losses were perceptive (n = 25). Four were conductive and seven mixed ones [Fig. 3]. Out of the 36 bilateral supposed HL, there were 5 mild HL, 25 moderate HL, 3 severe HL and 3 profound HL or cophosis [Figs. 4 and 5]. The ability of five children to communicate with their parents in whispered voice without looking at their lips raised the suspicion of pseudohypacusis. In 17 cases such suspicion was explained by a difference of more than 20 dB between pure tone and speech thresholds. In 7 cases the speech thresholds were normal whereas the pure tone thresholds were abnormal. In 6 cases speech and pure tone audiometry were coherent. An 8 year-old boy had ABR prior to our examination. This ABR was impossible to interpret because the child persisted in moving his head and his shoulders during the recording. Complementary examinations were routinely performed to rule out a pseudohypacusis before suggesting an invasive or expensive treatment (surgery or hearing aids). A diagnosis of 16 cases was conducted by speech audiometry and retest pure tone audiometry which were normal. Two children were retested and these latter tests displayed both speech and pure tone audiometries as being normal. In 7 cases test or retest speech audiometry elicited abnormal
3.3.2. Unilateral hearing loss In 18 cases the simulated HL was unilateral: it was perceptive in 12 cases, conductive in 2 cases, mixed in 4 cases, severe in 2 cases, moderate in 8 cases, and mild in 5 cases. Three children simulated a cophosis [Figs. 3 and 4]. One 8 year-old girl was referred to our institution for a real cophosis. Acoustic reflexes were elicited in the left ear by stimulation of the right ear at 90 dB on 250 Hz, and at 105 dB on 500, 1000 and 2000 Hz which ruled out the diagnosis of cophosis. TEOEs were present in the supposed deaf ear. One 6 year-old boy was complaining of left cophosis. Acoustic reflexes were elicited in the right ear by stimulation of the left ear at 85 dB at 250, 500, 1000 and 2000 Hz, which ruled out the diagnosis of cophosis. In the other cases, the diagnosis of pseudohypacusis was suspected on the discrepancy between speech reception and air-conduction pure tone thresholds on the supposed hearing-impaired ear. Ten children had ABR. In nine cases the wave V thresholds were normal in both ears. In the last case (an 8 year-old girl) ABR were not recordable because the child kept on moving during
[(Fig._4)TD$IG] Number of children 35
Number of children
35
30
30
25
25
20
20
boy
15
boy
15
girl
10
girl
10
5
5
0
0 unilateral deafness
bilateral deafness
Fig. 2. Distribution of pseudohypacusis by laterality and gender.
Fig. 4. Distribution of pseudohypacusis by severity (mild, moderate, severe, profound (and cophosis)) of the deafness and gender.
[(Fig._5)TD$IG]
M. Drouillard et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1632–1636
NOHL suspected : (54) -by clinical behaviour, - discrepancy of 20 dB between thresholds elicited by airconducon and vocal smulaon - when the thresholds are different when the test is repeated the same day
1635
No doubt about NOHL (clinical behaviour and audiometry ) (17)
Doubt : (37)
TEOES : (7) -hearing loss over 30dB
acousc reflex : (1) -unilateral cophosis -bilateral deafness (moderate to cophosis)
ABR +/-ASSR : (29)
Fig. 5. Means of diagnosis.
the recording. In one case there were no TEOEs because of serous otitis media but the wave V thresholds were normal. 3.4. Follow-up We met again with 10 children. While 8 had normal audiogram, two simulated again. We eventually called them on the phone over April 2012 in order to know what did these children become, and what type of follow-up they had. Twenty four families answered to our phone interview. Four children were better off after they received our support, and two other had a consultation with a psychologist who probably found the cause of the pseudohypacusis. In the case of one child, the problem had appeared after the birth of his younger brother and for the others it was due to difficulties at school. For yet another child, the psychological consultation allowed us to discovering there was a problem within the family. One child had a psychiatry consultation and an ElectroConvulsive Therapy (ECT). One child was better off, then appeared tinnitus and finally the hearing was normal. A child had a normal hearing after the diagnosis and never simulated after. Lastly, a child complained of a hearing impairment for one year. Another one wanted to keep his aids and leave them one year after and had a normal hearing. Finally a girl who had lupus and depressive symptoms had treatment in child psychiatry. She had ECT and unfortunately had to be in a wheelchair at the end. In the prospective study, we offered a psychological support to six of the present cases, for two children we informed parents concerning the pseudohypacusis and we reassured them. In regards to the last child we treated the otitis media and we also reassured the parents. 4. Discussion Non-organic hearing loss is rare in children, yet it must be ruled out in cases of hearing loss in order to avoid invasive or expensive investigation (CT-scan or MRI) or treatment (hearing aids, surgery to rule out a perilymphatic fistula) [5].
According to the literature specialized on the topic, the incidence of pseudohypacusis varies from 2% to 7%. Ioannis showed that NOHL is the most frequent etiology of sudden hearing loss: as being above 50% [10]. Our series denotes a female predominance (35 girls and 19 boys): 14 girls and 4 boys in the series of Radkowski et al. [5], 27 girls and 12 boys in the series of Yoshida et al. [6]. Moreover, the hearing loss is most of the time bilateral: it is 18 vs 12 in Radkowski’s series, 20 vs 12 in Hosoi’s series and 36 vs 18 in our series. The reported peak incidence varies from 10 to 12 year-old, and in our study the average age is 10 years (3 months) [11]. A sudden hearing loss following a conflict with parent may raise suspicion. Furthermore, we can note the importance of otological antecedents in these children (personal or familial). Two cases displayed a trauma on the ear, as shown in Rotenberg’s study [12]. Pseudohypacusis can be evoked when there is a discrepancy of 20 dB between thresholds elicited by air-conduction and vocal stimulation and/or when the thresholds are different when the test is repeated on the same day. The lack of lip-readings, asking for repeating questions, and the fact that the child whispered to his parents may evoke non-organic hearing loss or strengthen the suspicion. In order to discover children with pseudohypacusis, acoustic reflex can be tested with those who simulate an unilateral cophosis, or a bilateral deafness (moderate HL to cophosis) [13,14]. Other objective tests can be used when the diagnosis is not still confirmed by clinic and audiometry. For instance, presence of TEOES rules out a HL over 30 dB, but they can also be present in a central HL [2]. ABR are also interesting thanks to the ability to have real thresholds, and the specificity not to be affected by abnormal external and middle ear status [3]. However, ABR studies only high frequencies and requires the patient’s cooperation. It was unsuccessful in two cases because of the children’s restlessness. DPOAE can help for the diagnosis providing frequency-specific information of the cochlear functional state [3]. Finally ASSR can measure the true thresholds, particularly at low frequencies but is not performed in routine [15].
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Children underwent invasive (corticoids) or expensive treatment (hearing aids) because doctors did not do ABR and were not aware of this disease. And so practicians initiated treatments like corticosteroids and hearing aids. That’s why new tools like ABR and ASSR are important and necessary to be used before the prescription of hearing aids as soon as there is doubt and before all cochlear implants. Moreover it is important to make the diagnosis on time because it is very hard for parents to trust doctors after having gone as far as acquiring hearing aids. We can underline the importance of parental’s reassurance and information about pseuohypacusis. Some of parents deny pseudohypacusis or think that their child suffer from psychiatric disease, or do not trust their child anymore, or accuse themselves. In this case, it is very important to see a psychologist. Most of the time, pseudohypacusis translates an ill-be in the child which can often be explained by the child’s antecedents (the divorce of his parents or abuse at home for example) [16], or by a lack of attention (because of a new younger brother, sister, or a disease in the family). Thanks to our series we have found that 50% of the children with a positive personal or family history. In the study of Ioannis et al. [10], 20 on 26 children had such a story. It is important for parents and children to consult a psychologist. Parents reported that they appreciated the consultation with the psychologist because they and their child could speak freely with a neutral person. There weren’t psychiatric evaluation during the consultation. They preferred a pediatric psychologist rather than a psychiatrist. They tried together to understand why the child simulated and to make him understand that his hearing was normal. Moreover, seeing again the child three or six months later after the healing, let parents and child to achieve the process. We do not blame the child for lying, because it translates a pain and is an unconscious attitude. When the audiometric exam displays a normal result, we can be sure that the child is cured. If it is necessary, a speech and language assessment can also help, as some parents prefer a non psychological treatment at the NOHL. 5. Conclusion Non-organic hearing loss is rare among children. The incidence varies from 2 to 7%. Pseudohypoacusis must be considered when there is a discrepancy at the audiometry level, especially when speech reception thresholds are better than pure tone audiometry thresholds.
Pseudohypacusis are often under-diagnosed, because it is a poorly known disease. Therefore we must pay attention, and it must be ruled out in order to avoid invasive or expensive investigation (CT-scan or MRI) or treatment (hearing aids), above all when the hearing loss is unilateral. Providing information concerning the pathology, reassuring and psychological support are important for the parents and their child.
References [1] S. Austen, C. Lynch, Non-organic hearing loss redefined: understand, categorizing and managing non-organic behavior, Int. J. Audiol. 43 (2004) 449–457. [2] J.P. Pracy, R.M. Walsh, G.A. Mepham, D.A. Bowdler, Childhood pseudohypacusis, Int. J. Pediatr. Otorhinolaryngol. 37 (1996) 143–149. [3] S. Morita, M. Susuki, K. Iszuki, Non-organic hearing loss in childhood, Int. J. Pediatr. Otorhinolaryngol. 74 (2010) 441–446. [4] H. Hosoi, Y. Tsuta, T. Murata, H. Levitt, Suggestion audiometry for non organic hearing loss (pseudohypacusis) in children, Int. J. Pediatr. Otorhinolaryngol. 47 (1999) 11–21. [5] C. Radkowski, S. Cleveland, E.M. Friedman, Childhood pseudohypacusis in patients with high risk for actual hearing loss, Laryngoscope 108 (1998) 1534–1538. [6] M. Yoshida, A. Nogushi, T. Uemura, Functional hearing loss in children, Int. J. Pediatr. Otorhinilaryngol. 17 (1989) 287–295. [7] N. Saravanappa, Diagnostic tools in pseudohypacusis in children, Int. J. Pediatr. Otorhinolaryngol. 69 (2005) 1235–1238. [8] J.J.J. Dirckx, K. Daemers, T.H. Somers, F.E. Offeciers, P.J. Govaerts, Numerical assessment of TOAE screening results: currently used criteria and their effect on TOAE prevalence figures, Acta Otolaryngol. 116 (1996) 672–679. [9] M.P. Gorga, T.A. Johnson, J.R. Kaminski, K.L. Beauchaine, C.A. Garner, S.T. Neely, Using a combination of click-and tone burst-evoked auditory brainstem response measurements to estimate pure-tone thresholds, Ear Hear. 27 (2006) 60–74. [10] P. Ioannis, I. Psarommatis, G. Kontorinis, A. Kontrogiannis, D. Douniadakis, M. Tsakanikos, Pseudohypacusis: the most frequent etiology of sudden hearing loss in children, Eur. Arch. Otorhinolaryngol. 266 (2009) 1857–1861. [11] D.A. Bowlder, J. J. Rogers, The management of pseudohypacusis in school-age children, Clin. Otolaryngol. 14 (1989) 211–215. [12] B.W. Rotenberg, M. Makhija, B.C. Papsin, Conversion disorder in a child presenting as sudden sensorineural hearing loss, Int. J. Pediatr. Otorhinolaryngol. 69 (2005) 1261–1264. [13] J.C. Cooper, W.L. Meyerhoff, Functional deafness, in: P.P. Paparella, D.A. Shumrick (Eds.), Otorhinolaryngology, second ed., Saunders, Philadelphia, PA, 1980, pp. 1767–1776. [14] H. Zhao, C.F. Dai, F.L. Chi, et al., Non-organic hearing loss in Chinese teenagers, Auris Nasus Larynx 35 (2008) 485–492. [15] S. Kariya, K. Fukushima, A. Kawasaki, Y. Kataoka, K. Nishizaki, Auditory steadystate responses to multiple simultaneous stimuli in children with functional or sensorineural hearing loss, Eur. Arch. Otorhinolaryngol. 265 (2008) 769–773. [16] D. Erwin, L. Riedner Paul, Non organic hearing loss and child abuse: beyond the sound booth, Br. J. Audiol. 29 (1995) 195–197.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Pseudohypacusis in children: Circumstances and diagnostic strategy M. Drouillard a,b,*, N. Petroff a,b, J. Majer a,b, C. Perrot a,b, S. Quesnel a,b, M. Franc¸ois a,b ORL et CCF, CHU Robert Debre´, Paris, France a b
APHP Groupe hospitalier Robert Debre´, Pediatric Otorhinolaryngology Department, 75019 Paris, France Universite´ Paris 7, Denis Diderot, 5 rue Thomas Mann, 75013 Paris, France
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 April 2014 Received in revised form 4 July 2014 Accepted 7 July 2014 Available online 14 July 2014
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. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Non organic hearing loss Auditory brainstem response Deafness Diagnosis ASSR
1. Introduction Pseudohypacusis – also known as non-organic hearing loss (NOHL) – is defined as a discrepancy between the actual hearing thresholds of the patient and the admitted one. The word does not refer to the intentions, conscious or subconscious, of the patient who has been tested. Other words are used to describe it. They picture pseudohypacusis as malingering, functional hearing loss,
* Corresponding author at: APHP Groupe hospitalier Robert Debre´, Pediatric Otorhinolaryngology Department, Service ORL, hoˆpital Robert-Debre´, 48, Boulevard Se´rurier, 75019 Paris, France. Tel.: +33 0 1 40 03 22 22; fax: +33 0 1 40 03 22 02. E-mail address: [email protected] (M. Drouillard). http://dx.doi.org/10.1016/j.ijporl.2014.07.010 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
psychogenic hearing loss, feigning deafness, pseudohypacusis and conversion deafness. NOHL is the most neutral word to describe the situation. Since 1946, Doerfler and Stewart have started a discussion about the subclassifications of NOHL. The Austen and Lynch model makes use of three key diagnostic categories from DSM-IV linked by a continuum based on intent and gain. The first diagnosis is malingering. It refers to the intentional production of false – or grossly – exaggerated physical symptoms for self-gain (financial gain and drugs in order to avoid military service) [1,2]. The second diagnosis is factitious disorder, which states that the gain is intrapsychic rather than external. It refers to intentional production – or feigning of physical symptoms – when the motivation behind the behavior consists in assuming a sick role [1]. Finally, the third
M. Drouillard et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1632–1636
2. Methods The study concerned children from 3 to 16 year-old diagnosed with pseudohypacusis. This study was retrospective from January 1993 to November 2011 and prospective from December 2011 to April 2012. Mentally retarded children were not included in the study. A detailed history of language retardation, school difficulties, behavioral disorders, ear trauma, and family difficulties (for instance parents’ separation) was obtained. Furthermore, the purpose of children diagnosed with pseudohypacusis’ visit, the record of prior visits to our hospital or to another one for the same reason, as well as prior audiometric tests or treatments were recorded. The type and number of previous ‘‘false’’ or ‘‘faked’’ audiological evaluations was also taken into account. Every child had to go through an audiological evaluation in a sound treated booth. Tests were conducted with a Conera clinical audiometer (GN Otometrics, Denmark) for pure tone and speech audiometry through standard earphones. The hearing level was considered as being normal if the air-conduction threshold was better than 20 dB in the four speech frequencies 0.5, 1, 2 and 4 kHz. The speech stimuli were based on the Boorsma lists of words for children, and delivered by the Digivox program (Audivimedia, Paris, France) through a PC computer connected to the audiometer. Speech audiometry was considered as being normal when speech reception threshold fall under 20 dB with a 100% discrimination score. When judged necessary, tympanometry and acoustic reflex, transient evoked otoacoustic emissions (TEOE) and/or auditory brainstem responses (ABR) were recorded. We also used auditory steady state response (ASSR) for several cases in order to obtain objective responses on several frequencies. Tympanometry and acoustic reflex measurements were accomplished with an AZ7R impedance audiometer (Interacoustics, Assens, Denmark). TEOEs were recorded with the ILO92 Otodynamic analyzer measuring system (Otodynamics Ltd, London, England). The stimuli were not linear clicks, going from 69 to 85 dB SPL. TEOEs were identified on a global reproducibility greater than 75% and more than 3 dB of S/N ratio in at least 3 frequency bands [8]. Auditory brain stem responses (ABR) and auditory steady-state responses (ASSR) were recorded on an Eclipse (Interacoustics, Assens, Denmark). For ABR
recordings, the stimulus rate was 11 clicks/s, and 1600 clicks were presented for each stimulus level [9]. The disappearance of wave V while decreasing the intensity of the stimulus determined the threshold. For ASSR recordings, both ears were tested simultaneously on 4 frequencies (0.5, 1. 2, and 4 kHz), with a modulation frequency between 75 and 95 Hz. The children were divided into two groups according to the unior bilateralism of the supposed hearing loss and also according to where these children stand on a graduation going from mild to moderate, severe, profound and complete (cophosis). We contacted patient’s families. They underwent a prewritten phone interview about the hearing of their child, the wear of hearing aids after diagnosis of pseudohypacusis and the need for another doctor’s consultation. The reason and the duration of the simulation were also assessed. For those who consulted our psychologist we noted if he helped them and their child, if they thought it was important to see him and how he helped them. 3. Results During this period of time, 54 children between 3 and 16 yearold were referred to our institution because of audiological assessment. They were diagnosed with pseudohypacusis [Fig. 1]. They were 35 girls and 19 boys, with an average age of 10 years (3 months) (min: 4 years, max: 16 years). From November 2011 to April 2012, 9 children out of 145 who had been tested for deafness were diagnosed with pseudohypacusis. The incidence of pseudohypacusis during the prospective part of the study was 6.2%. 3.1. Past history of the children diagnosed with pseudohypacusis In 27 cases – half of our patients –, important events happened in the child’s life. Prior to their hearing loss complaint, two children had experienced a physical aggression, ten had difficulties at school, one had a family drama–the brutal death of one of his aunt, and one was examined within a context of abuse. Three children were experiencing parents’ separation. One child was raised in a foster family, while another one had leukemia. A girl suffered from anorexia and depression because of rheumatoid polyarthritis. Another girl had intense visceral pain. Four children had behavioral disorders (hyperactivity and aggressiveness), and two others thought of as having a bilateral hearing loss had language retardation. In our study fifteen children displayed otological antecedents. Four children had a family history of perceptive deafness, among them one had a brother with a progressive deafness since 2011. One child had already suffered a sudden unilateral deafness. Another child had an external otitis one week before the allegated hearing loss, and four children had a serous otitis media, which was [(Fig._1)TD$IG] 12 boy
Number of children
diagnosis stresses the fact that the patient unintentionally products symptoms in conversion disorder. Conflict or others stresses tend to precede the psychological change [1,3]. Pseudohypacusis also affects children [4–6]. In such cases, they are generally easier to diagnose than adults since children reproduce less consistently. However children’s diagnosis is often missed because of a lack of awareness regarding the possibility of the disease affecting them [7]. Each time a child displays poor tone thresholds, the examiner must verify that they are not the consequence of a misunderstanding or an unfamiliarity with the test procedure, or attention disorders. Children may have difficulty in paying attention to abstract stimuli such as tones. A simple method to improve the results in case an unfamiliarity occurs with the test procedure consists in repeating the test several times. Unfortunately, this is a time consuming process. The organicity of the hearing loss must be verified before any invasive examination or treatment take place. If the hearing loss is diagnosed as non-organic, the real hearing level must be evaluated. The aim of our study is to precise the circumstances under which we must pay attention to a pseudohypacusis case, as well as to evaluate the methods used to detect its presence in children and to determine their real hearing thresholds. It finally aims at analyzing the possible outcomes for these children.
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10
girl
8 6 4 2 0 4
5
6
7
8
9 10 11 12 13 14 15 16
Age (years) Fig. 1. Distribution of pseudohypacusis by gender and age.
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treated with tympanostomy tubes. One had a mastoiditis. Three children experienced head trauma, and one of them, who was a 14 year-old boy, complained of unilateral hearing loss after a street fight. Lastly, an 8 year-old girl complained of right hearing loss after one of her schoolmates shouted near her.
Number of children 40
35 30 25
3.2. Care pathway Out of these 54 children, 37 had priory consulted ENT private practionners (29) or ENT doctors in other hospitals (3). Five had already consulted the ENT department of our hospital. Prior to our investigation, 12 children had had a CT scan and/or a MRI of the ears (one had an appointment for it). Three children conducted a vestibular test. All these exams were evaluated as normal. Five children wore hearing aids and three received corticosteroids. Hearing aids were suggested to 7 other children. Finally, one child was enrolled in a cochlear implant program.
boy
15
girl
10 5 0
percepon conducvee mixed Fig. 3. Distribution of pseudohypacusis by type of the deafness (perceptive, conductive and mixed) and gender.
3.3. Kind of hearing loss and diagnostic tools
responses, yet TEOES were present in both ears. After explaining to the child the meaning of the presence of TEOEs, the air-conduction thresholds were also normal. In 7 cases diagnosis was made by ABR (wave V thresholds were normal in both ears), after explaining to the child the meaning of these results, another speech audiometry was performed whose responses were normal.
The supposed hearing loss was bilateral in 36 cases, while it was unilateral in 18 cases [Fig. 2].
[(Fig._2)TD$IG]
20
3.3.1. Bilateral hearing loss Thirty-six children complained of bilateral hearing loss and asked for repeat what we had just said when asked questions. The majority of simulated hearing losses were perceptive (n = 25). Four were conductive and seven mixed ones [Fig. 3]. Out of the 36 bilateral supposed HL, there were 5 mild HL, 25 moderate HL, 3 severe HL and 3 profound HL or cophosis [Figs. 4 and 5]. The ability of five children to communicate with their parents in whispered voice without looking at their lips raised the suspicion of pseudohypacusis. In 17 cases such suspicion was explained by a difference of more than 20 dB between pure tone and speech thresholds. In 7 cases the speech thresholds were normal whereas the pure tone thresholds were abnormal. In 6 cases speech and pure tone audiometry were coherent. An 8 year-old boy had ABR prior to our examination. This ABR was impossible to interpret because the child persisted in moving his head and his shoulders during the recording. Complementary examinations were routinely performed to rule out a pseudohypacusis before suggesting an invasive or expensive treatment (surgery or hearing aids). A diagnosis of 16 cases was conducted by speech audiometry and retest pure tone audiometry which were normal. Two children were retested and these latter tests displayed both speech and pure tone audiometries as being normal. In 7 cases test or retest speech audiometry elicited abnormal
3.3.2. Unilateral hearing loss In 18 cases the simulated HL was unilateral: it was perceptive in 12 cases, conductive in 2 cases, mixed in 4 cases, severe in 2 cases, moderate in 8 cases, and mild in 5 cases. Three children simulated a cophosis [Figs. 3 and 4]. One 8 year-old girl was referred to our institution for a real cophosis. Acoustic reflexes were elicited in the left ear by stimulation of the right ear at 90 dB on 250 Hz, and at 105 dB on 500, 1000 and 2000 Hz which ruled out the diagnosis of cophosis. TEOEs were present in the supposed deaf ear. One 6 year-old boy was complaining of left cophosis. Acoustic reflexes were elicited in the right ear by stimulation of the left ear at 85 dB at 250, 500, 1000 and 2000 Hz, which ruled out the diagnosis of cophosis. In the other cases, the diagnosis of pseudohypacusis was suspected on the discrepancy between speech reception and air-conduction pure tone thresholds on the supposed hearing-impaired ear. Ten children had ABR. In nine cases the wave V thresholds were normal in both ears. In the last case (an 8 year-old girl) ABR were not recordable because the child kept on moving during
[(Fig._4)TD$IG] Number of children 35
Number of children
35
30
30
25
25
20
20
boy
15
boy
15
girl
10
girl
10
5
5
0
0 unilateral deafness
bilateral deafness
Fig. 2. Distribution of pseudohypacusis by laterality and gender.
Fig. 4. Distribution of pseudohypacusis by severity (mild, moderate, severe, profound (and cophosis)) of the deafness and gender.
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NOHL suspected : (54) -by clinical behaviour, - discrepancy of 20 dB between thresholds elicited by airconducon and vocal smulaon - when the thresholds are different when the test is repeated the same day
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No doubt about NOHL (clinical behaviour and audiometry ) (17)
Doubt : (37)
TEOES : (7) -hearing loss over 30dB
acousc reflex : (1) -unilateral cophosis -bilateral deafness (moderate to cophosis)
ABR +/-ASSR : (29)
Fig. 5. Means of diagnosis.
the recording. In one case there were no TEOEs because of serous otitis media but the wave V thresholds were normal. 3.4. Follow-up We met again with 10 children. While 8 had normal audiogram, two simulated again. We eventually called them on the phone over April 2012 in order to know what did these children become, and what type of follow-up they had. Twenty four families answered to our phone interview. Four children were better off after they received our support, and two other had a consultation with a psychologist who probably found the cause of the pseudohypacusis. In the case of one child, the problem had appeared after the birth of his younger brother and for the others it was due to difficulties at school. For yet another child, the psychological consultation allowed us to discovering there was a problem within the family. One child had a psychiatry consultation and an ElectroConvulsive Therapy (ECT). One child was better off, then appeared tinnitus and finally the hearing was normal. A child had a normal hearing after the diagnosis and never simulated after. Lastly, a child complained of a hearing impairment for one year. Another one wanted to keep his aids and leave them one year after and had a normal hearing. Finally a girl who had lupus and depressive symptoms had treatment in child psychiatry. She had ECT and unfortunately had to be in a wheelchair at the end. In the prospective study, we offered a psychological support to six of the present cases, for two children we informed parents concerning the pseudohypacusis and we reassured them. In regards to the last child we treated the otitis media and we also reassured the parents. 4. Discussion Non-organic hearing loss is rare in children, yet it must be ruled out in cases of hearing loss in order to avoid invasive or expensive investigation (CT-scan or MRI) or treatment (hearing aids, surgery to rule out a perilymphatic fistula) [5].
According to the literature specialized on the topic, the incidence of pseudohypacusis varies from 2% to 7%. Ioannis showed that NOHL is the most frequent etiology of sudden hearing loss: as being above 50% [10]. Our series denotes a female predominance (35 girls and 19 boys): 14 girls and 4 boys in the series of Radkowski et al. [5], 27 girls and 12 boys in the series of Yoshida et al. [6]. Moreover, the hearing loss is most of the time bilateral: it is 18 vs 12 in Radkowski’s series, 20 vs 12 in Hosoi’s series and 36 vs 18 in our series. The reported peak incidence varies from 10 to 12 year-old, and in our study the average age is 10 years (3 months) [11]. A sudden hearing loss following a conflict with parent may raise suspicion. Furthermore, we can note the importance of otological antecedents in these children (personal or familial). Two cases displayed a trauma on the ear, as shown in Rotenberg’s study [12]. Pseudohypacusis can be evoked when there is a discrepancy of 20 dB between thresholds elicited by air-conduction and vocal stimulation and/or when the thresholds are different when the test is repeated on the same day. The lack of lip-readings, asking for repeating questions, and the fact that the child whispered to his parents may evoke non-organic hearing loss or strengthen the suspicion. In order to discover children with pseudohypacusis, acoustic reflex can be tested with those who simulate an unilateral cophosis, or a bilateral deafness (moderate HL to cophosis) [13,14]. Other objective tests can be used when the diagnosis is not still confirmed by clinic and audiometry. For instance, presence of TEOES rules out a HL over 30 dB, but they can also be present in a central HL [2]. ABR are also interesting thanks to the ability to have real thresholds, and the specificity not to be affected by abnormal external and middle ear status [3]. However, ABR studies only high frequencies and requires the patient’s cooperation. It was unsuccessful in two cases because of the children’s restlessness. DPOAE can help for the diagnosis providing frequency-specific information of the cochlear functional state [3]. Finally ASSR can measure the true thresholds, particularly at low frequencies but is not performed in routine [15].
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Children underwent invasive (corticoids) or expensive treatment (hearing aids) because doctors did not do ABR and were not aware of this disease. And so practicians initiated treatments like corticosteroids and hearing aids. That’s why new tools like ABR and ASSR are important and necessary to be used before the prescription of hearing aids as soon as there is doubt and before all cochlear implants. Moreover it is important to make the diagnosis on time because it is very hard for parents to trust doctors after having gone as far as acquiring hearing aids. We can underline the importance of parental’s reassurance and information about pseuohypacusis. Some of parents deny pseudohypacusis or think that their child suffer from psychiatric disease, or do not trust their child anymore, or accuse themselves. In this case, it is very important to see a psychologist. Most of the time, pseudohypacusis translates an ill-be in the child which can often be explained by the child’s antecedents (the divorce of his parents or abuse at home for example) [16], or by a lack of attention (because of a new younger brother, sister, or a disease in the family). Thanks to our series we have found that 50% of the children with a positive personal or family history. In the study of Ioannis et al. [10], 20 on 26 children had such a story. It is important for parents and children to consult a psychologist. Parents reported that they appreciated the consultation with the psychologist because they and their child could speak freely with a neutral person. There weren’t psychiatric evaluation during the consultation. They preferred a pediatric psychologist rather than a psychiatrist. They tried together to understand why the child simulated and to make him understand that his hearing was normal. Moreover, seeing again the child three or six months later after the healing, let parents and child to achieve the process. We do not blame the child for lying, because it translates a pain and is an unconscious attitude. When the audiometric exam displays a normal result, we can be sure that the child is cured. If it is necessary, a speech and language assessment can also help, as some parents prefer a non psychological treatment at the NOHL. 5. Conclusion Non-organic hearing loss is rare among children. The incidence varies from 2 to 7%. Pseudohypoacusis must be considered when there is a discrepancy at the audiometry level, especially when speech reception thresholds are better than pure tone audiometry thresholds.
Pseudohypacusis are often under-diagnosed, because it is a poorly known disease. Therefore we must pay attention, and it must be ruled out in order to avoid invasive or expensive investigation (CT-scan or MRI) or treatment (hearing aids), above all when the hearing loss is unilateral. Providing information concerning the pathology, reassuring and psychological support are important for the parents and their child.
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