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Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD)
International Journal of Pediatric Otorhinolaryngology 132 (2020) 109938
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Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD)
T
Désirée Ehrmann-Müller∗, Daniela Back, Heike Kühn, Rudolf Hagen, Wafaa Shehata-Dieler Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080, Wuerzburg, Germany
A R T I C LE I N FO
A B S T R A C T
Keywords: Auditory neuropathy spectrum disorder Cochlear implantation Hearing and speech development Education
Introduction: The present article shows long-term results in the hearing and speech development of children with auditory neuropathy spectrum disorder (ANSD). Some children were followed up for nearly 20 years, monitoring their progress through childhood into adulthood. Methods: This retrospective study examined data from 10 children who were diagnosed and treated at our tertiary referral center. All children were provided with hearing aids and/or cochlear implants. The children's hearing and speech development and their social and educational development were evaluated. Results: Seven children were provided with cochlear implants on both sides; three children, one of which had single-sided deafness, received hearing aids. All children with cochlear implants on both sides used their devices full-time. Speech perception ranged between 100% and 0% on one side. Five children attended a school for the hearing impaired; four children attended a regular school. Four children attended vocational training. Conclusions: At present, there is a lack of literature on the long-term outcomes of treatment in children with ANSD. The data presented show that the hearing and speech development in children with ANSD are significantly heterogeneous. Regular school education and social integration of children with ANSD can be achieved with intensive and supportive rehabilitative methods.
1. Introduction With a prevalence of 7–10%, auditory neuropathy spectrum disorder (ANSD) is a relevant cause of sensorineural hearing loss (SNHL) [1–3]. Preserved otoacoustic emissions (OAE) and cochlear microphonics (CM) and absent or severely distorted auditory brainstem responses (ABR) are the characteristics of ANSD [4]. Lesions at different sites like presynaptic, postsynaptic or central lesions may cause ANSD [5]. The most common predisposing factor for ANSD is prematurity [6]. Perinatal hyperbilirubinemia [7–9], hypoxia and ototoxic medication [10] are further risk factors for ANSD. 40% of the children with ANSD have an underlying genetic factor [11]. In up to one third of the children affected, ANSD is caused by cochlear nerve dysplasia (CND) [7,12]. The characteristics of ANSD are specified as fluctuating hearing sensitivity in pure tone thresholds, reduced speech perception performance especially in difficult hearing situations like in noise, and reduced sound localization ability [1,2,4,13–15]. Recommendations on treatment options in the literature range from no amplification at all, frequency modulating systems (FM systems) and hearing aids (HAs) in mild to moderate hearing loss, up to the provision
∗
of a cochlear implant (CI). There are publications which report good hearing rehabilitation with HAs in children with ANSD [16–18]. Some demonstrate outcomes similar to those obtained through provision with CIs [16,17]. In contrast, there are studies which describe a lack of benefits in hearing and communication with HAs in children with ANSD [4,19]. Dean et al. showed that children with ANSD, who do not benefit from HAs, perform well if provided with CIs [20]. Most studies show a higher benefit with a CI compared to a HA in children with ANSD [21–23]. Significant improvements in speech perception, language development and communication outcomes with CIs that are comparable to results in children with cochlear SNHL have been described in the literature [6,24–30]. However, outcomes with CIs in children with ANSD vary considerably. Some authors reported poor performance with CIs [31] or a minimal benefit from CIs in 25% of the children with ANSD [32–34]. The presence of CND seems to promote the efficacy of cochlear implantation [28,30,35–39]. The study of Breneman et al., 2012 on the speech outcomes in children with CND showed similar longterm outcomes compared to matched peers with cochlear hearing loss. To date, according to our knowledge, there are no data published on
Corresponding author. E-mail address: [email protected] (D. Ehrmann-Müller).
https://doi.org/10.1016/j.ijporl.2020.109938 Received 28 October 2019; Received in revised form 27 January 2020; Accepted 6 February 2020 Available online 07 February 2020 0165-5876/ © 2020 Published by Elsevier B.V.
International Journal of Pediatric Otorhinolaryngology 132 (2020) 109938
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(author number 3). It puts the main focus on which kindergarten or school the children attended including the parents’ reasons for choosing a specific kindergarten or school. The parents had to tick which form of kindergarten or school the children attended. Another focus of the questionnaire is how the children with ANSD perform in comparison to their normal-hearing peers at school. Concerning motoric, mental speech and social development, the parents had to mark if their child was far behind, weaker, equal, better or far better than children with normal hearing. In addition they had to tick the grades their children got in the different school subjects. Furthermore, it collects information about the use of the hearing device (tick how long the hearing device is worn per day: day and night - all day - hours - not) and the social integration and acceptance of the child (mark if the children had friends, hobbies [40]. Data analysis was performed with Graph Pad Prism and Microsoft Office Excel. This retrospective study was approved by the authorized ethics committee (2019022003) and was conducted according to the guidelines established in the Declaration of Helsinki (Washington 2002) and the ISO 14155 Parts I and II for the conduct of clinical studies involving humans.
long-term hearing and speech development outcomes and the educational and social development of children with ANSD. Due to the very heterogeneous nature of ANSD, which often occurs in combination with severe accompanying disorders, there is still an ongoing discussion on the benefit of amplification in children with ANSD. Diagnosis and adequate treatment of this hearing disorder are still quite challenging, especially in children. Therefore, it is important to collect long-term outcomes of this cohort. The aim of this study was to collect and analyze long-term hearing and speech development outcomes and the social and educational development of children with ANSD. 2. Material and methods Ten children (eight females, two males) who were diagnosed with ANSD were examined at our tertiary referral center. A retrospective analysis of the data collected from the charts was performed. The children presented at the comprehensive hearing center (CHC) of the clinic because of various risk factors, a failed newborn hearing screening (NHS) or were missing speech and language development. Subjective audiological evaluation included a free/sound field or pure tone audiometry (PTA) and a speech audiometry if possible. As clinical audiometer a WESTRA CAD03 (Westra Electronic GmbH) calibrated according to DIN (Deutsches Institut für Normung) was used. Objective tests included: OAEs, impedance metry, click-evoked ABR, auditory steady state responses (ASSR), and an electrocochleography. ABR and ASSR-measurements were performed with an eclipse 1834446 (Interacoustics, Denmark) or a WESTRA ERA Q/S-02 (Westra Electronic GmbH). Electrocochleography was performed with a Nicolet Synergy EDX. All tests were conducted in a sound-treated room (IAC model 55). As inclusion criteria for ANSD missing or conspicuous responses in ABR and the presence of OAE or typical CM morphology were rated. Children with missing ABR and missing OAE and missing CM pattern in ABR or electrocochleography were excluded from the study. Radiological diagnostics included cerebral magnetic resonance imaging (cMRI) and in some cases computed tomography (CT) of the temporal bone. Age at first presentation, age at diagnosis, age of provision with HA or CI and risk factors were documented. During follow up, the hearing and speech development and the social development of these children were investigated at regular intervals. Audiometric evaluation included aided free field audiometry at 3 frequencies (500, 1000 and 2000Hz), speech discrimination tests in quiet tested with the Freiburgers' monosyllabic test, and speech discrimination in noise tested with the Hochmair-Schulz-Moser sentence test (HSM). Furthermore, the use of the hearing device and the education level of each child were investigated using a special parent questionnaire (“(Eltern)fragebogen zur Erfassung der Bildungslaufbahn hörgeschädigter Kinder”, BILDHOEKI) which focuses on the educational development of children with a hearing impairment. The questionnaire was developed and validated by a psychologist at our center
3. Results Seven children were provided with CIs on both sides. One of these had progressive hearing loss. Three children got HAs, one of them had single-sided deafness and received a HA only in one ear. Six of these children had no risk factors and four children had known risk factors for ANSD like prematurity, genetic cause (OTOF compound heterozygous, Pendred) or craniofacial syndrome. The median age at the first presentation at the clinic was 19.5 months (range: 2–53 months). Diagnosis was established shortly after, at a median age of 35 months (range: 3–53 months). In one child, it took longer to diagnose ANSD due to the presence of additional comorbidities. Provision of suitable hearing devices was obtained at a median age of 48 months (range: 4–82 months). The follow-up of the children averaged at a median of 14.6 years (11.6–19.2 years). All of the children were older than 16 at the time of the present analysis. Further demographics are available in Table 1. OAE could be found in all children initially and disappeared in two children over time. In ABR, six children had missing bilateral responses, of whom one child showed hearing thresholds between 35 and 75 dB HL without amplification. Another one had hearing thresholds between 75 and 80 dB HL. In the other children with missing ABR responses only hearing thresholds less than 100 dB or no hearing thresholds at all could be observed. The one child, who was provided with bilateral HA showed ABR responses at 100 and 90 dB nHL and her subjective hearing threshold ranged between 40 and 70 dB HL. The child with the single sided ANSD showed responses in ABR only at 100 dB nHL and the hearing threshold lay between 55 and 65 dB HL. Another child had responses in ABR at 90 and 100 dB nHLwith hearing thresholds
Table 1 Participant demographics. P
Sex
Age at definite provision (in months)
Device
Cause of ANSD
Accompanying disorders
Years of follow up
1 2 3 4 5 6 7 8 9 10
f m m f f f f f f f
56 82 40 4 49 30 72 47 39 71
MED-EL PULSARci 100 MED-EL PULSARci 100 MED-EL PULSARci 100 HA MED-EL PULSARci 100 MED-EL PULSARci 100 MED-EL PULSARci 100 HA HA C40 + r/CONCERTO flex soft l
Craniofacial syndrome Unknown Unknown Unknown Unknown OTOF comp. heterozygot Prematurity, 31st gw Unknown Unknown Pendred syndrome
Craniofacial syndrome None None None None None None None Developmental delay None
19.2 11.6 14.2 17.8 14.4 19.1 12.5 12.1 17.4 14.8
p indicates participant, f female, m male, HA hearing aid, CI cochlear implant, mo months, y years, gw gestational week, comp. compound, r right, l left. 2
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Fig. 1. Aided AAT with CI or HA, best over time, dots represent each child in the study (grey dots = HA user, light grey: child with HA on both sides, dark grey: child with single sided HA; black = CI user, black filled: children with profound hearing loss, black frame: child with severe hearing loss, black and with: child with moderate hearing loss). Fig. 2. Aided speech perception in noise with CI, best over time, number of participants shown in dots; HSM indicates Hochmair- Schulz- Moser-test, SNR signal to noise ratio.
between 70 and 100 dB HL. And the child with progressive hearing loss showed responses in ABR at 50 and 60 dB nHL. Unfortunately, no hearing thresholds without amplification were conducted.
noise and in quiet on one side, the one with the OTOF-mutation and another one with profound hearing loss (see Fig. 2).
3.1. Hearing development
3.2. Results of the BILDHOEKI questionnaire
Averaging auditory thresholds (AAT) at 500, 1000 and 2000 Hz and speech in quiet and noise was tested separately for each ear with amplification. All children except the one with single sided ANSD hat symmetric bilateral hearing loss. Without amplification four children had profound hearing loss, three children suffered from a severe hearing loss and three children had a moderate hearing loss. After provision the degree of hearing loss decreased according to the aided thresholds to moderate hearing loss in five children, mild to moderate in the child with bilateral HA and mild in the child with single sided ANSD. In three children, including the one with the OTOF mutation and the one with progressive hearing loss, no AAT improvement was observed after amplification. The median AAT at all frequencies with a HA or CI was 35–40 dB (see Fig. 1). Due to the low sample size, a reasonable comparison between AAT with CIs and AAT with HAs was not possible. The speech perception outcomes ranged from 100% on the HSM-test to 0% on one side on the Freiburgers' monosyllabic test. Since the child with the single-sided ANSD was a non-user of the HA, speech discrimination could not be tested. In another child, speech perception could not be tested because of a severe developmental delay. The aided speech perception in quiet tested with the Freiburgers' monosyllabic test at 65 dB was 50% and higher in most children with a median of 72.5%. At 80 dB, speech perception was 60% and higher in all but one child, who had a median of 85%. Due to the low sample size and only one HA user, a comparison of speech perception with HAs and with CIs was not possible. The only child that used HA on both sides had a speech perception in quiet of 95% on both sides. The child with the progressive hearing loss reached a speech perception of 95 and 75% at 65 dB, whereas the other children with CI reached a speech perception between 20 and 85% at 65 dB. There was no difference between the children with moderate or profound hearing loss. In the child with OTOF-mutation no results of Freiburgers' test were elevated. Speech perception in noise with the HSM was performed in the seven children with CIs. 70% of the children had a speech perception score in noise (at 65 dB and a SNR of 10 dB) of 68% or higher, including two children with profound hearing loss, one with moderate hearing loss and the one with progressive hearing loss. Four of the seven children had a speech perception score in noise (at 65 dB and a SNR of 5 dB) of 80% or higher. Two children had a speech perception of 0% in
The BILDHOEKI questionnaire was returned by seven of the ten parents and children (70%). In the three remaining patients, two were provided with CIs and one with bilateral hearing aids, no further information was obtained either in written form or by telephone. Only relevant items of the questionnaire concerning the topic of the present study were analyzed. 3.2.1. Speech development The native language of five children was German only, two of the children were trained bilingually. Six children used oral communication, two also used sign language (the one with OTOF-mutation and one with moderate hearing loss). One child with a severe developmental delay was not able to communicate orally and used rudimentary sign language only. In preschool, 57% of the parents estimated their children to have a speech development that was far behind or weaker than their normal hearing peers. 57% of the children (4 of 7) self-reported a speech development equal to their normal hearing peers at secondary school (see Fig. 3). The child with the progressive hearing loss always had an equal speech development as normal hearing peers. The one with the OTOFmutation was always far behind. Speech production and speech understanding were also far behind or weaker in the children with ANSD compared to their normal hearing peers (see Fig. 4). The motor activity, mental and social competence of the children with ANSD were mostly estimated by the parents to be equal or better than the ones of their normal hearing peers (see Fig. 4). Over 50% of the parents rated their children's communicative skills as good. 71% of the children were able to tell both people that they know and people that they do not know what they are concerned about. The child with the severe developmental delay was not able to do so. All children were able to get in contact with others and had no problems understanding people. 3.2.2. Education and social career As shown in Table 1, one child had an unknown craniofacial syndrome and another child had a severe developmental delay of unknown origin. 3
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Fig. 3. Estimation of speech development compared to normal hearing peers in different periods of the educational life.
teacher trained in providing support for children with hearing loss. The child with progressive hearing loss changed to a mainstream/regular grammar school after finishing junior high school. Four children attended a special secondary school for hearing-impaired children including the one with the OTOF-mutation. The child with developmental delays continued in a school for children with additional needs. For the performance of the children at primary and secondary school with grades for each subject see Fig. 5. Grade 1 is the best mark in Germany, grade 6 the worst. The child with progressive hearing loss finished school with a general qualification to enter university and two other children, the one with OTOF-mutation and the one with severe hearing loss obtained a certificate of secondary education/subject-related entrance qualification. The remaining children are still attending school. Three of the children have already begun an apprenticeship/vocational training amongst others the child with OTOF-mutation. Six children were completely integrated in all aspects of social life,
Four children attended a regular kindergarten where children with additional needs are also accepted. One of the four children had to change to an institution for hearing-impaired children. Two children started in a kindergarten for hearing-impaired children from the beginning. The child with the severe developmental delay attended a kindergarten for children with additional needs. The child with the single-sided ANSD and the one with progressive hearing loss attended a mainstream/regular primary school with occasional assistance of a teacher trained in providing additional support for children with special needs and/or hearing loss. Four children visited a special school for hearing and speech including the one with the OTOF-mutation and the child with the severe developmental delay attended a school for children with additional needs. Regarding the secondary school career, the child with the singlesided ANSD and the child with progressive hearing loss attended a mainstream/regular junior high school; the one with the single-sided ANSD attended a junior high school with occasional assistance of a
Fig. 4. Estimation of general preschool development compared to normal hearing peers. 4
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profound hearing loss due to ANSD [29,34]. Liu et al. showed an average four frequency aided hearing level with CIs in children with ANSD from 17.5 to 57.5 dB. The PTA values in the present study ranged from 20 to 60 dB (median over frequencies: 27.5–37.5 dB). The results of aided PTA are relatively good and constant in all of the children. This is in contrast to the quite heterogeneous results of speech perception, especially in noise, which is typical for ANSD and has been frequently reported in individuals with ANSD. As shown in Fig. 3, the aided median speech perception in quiet at 65 dB was 72.5% and 85% at 80 dB. These varied from 0 to 95% at 65 dB and from 0 to 100% at 80 dB. A difference between CIs and HAs could not be assessed due to the low number of children in our study. The only tested HA user had a speech perception score of 95% in quiet on both sides. These results are likely due to a moderate hearing loss (see Fig. 2: unaided pure tone audiometry). The speech perception outcomes in noise varied from 0 to 100% amongst the children. These results are in accordance with Breneman et al. who reported that 91% of 35 ANSD children with CIs had some degree of open-set word recognition and at least 80% reached an open-set speech perception in the long-term follow-up [30]. In the present study, 90% of the children had some degree of open-set word recognition and 70% reached open-set speech perception. In other studies significant improvements in speech perception and language development with the use of CIs in ANSD were described, which were comparable to results in children with cochlear hearing loss [6,24–26]. In previous publications, an early amplification is recommended, either by HAs or by CIs, because these children have a higher benefit than those provided at an older age [26]. A correlation between hearing and speech performance and age at amplification could not be calculated in the present study due to the small sample size. 7–10% of the cases of sensorineural hearing loss in children are associated with ANSD and are often caused by the previously mentioned risk factors (see Introduction and Fig. 1). In the present study, 40% of the children had risk factors. In the literature, CND is present for between 18% [47] and 33% of children with ANSD [7,12]. There was no evidence of a CND in these ten children. However, with no speech understanding with CIs on one side in two children (P1 and P6), a CND might be assumed. One of these children was not examined by a cMRI, in the other child there was no description of such a finding in cMRI, but this was some years ago. Modern radiological diagnostics, e.g. MRI including CISS ((Constructive Interference in Steady State)-sequence, a strong T2-weighted sequence which enables a high depiction of the nerves in the cerebellopontine angle) allows a better and more detailed judgement on the presence and state of the cochlear nerve (CN) [48,49]. In previous studies that have reported minimal benefit from CIs in 25% of the children with ANSD [32–34] or even very poor performances using CIs [31], accompanying disorders or a CND are often described. Indeed, Teagle et al. showed that 50% of 140 children demonstrated open set speech perception, but some had no benefit due to a lack of electrical-induced neural synchronization, CND or other associated conditions, such as syndromes or developmental delays [33]. In the present study, speech development, subjectively estimated by the parents, improved from preschool to secondary school in nearly all adolescents, whereas 57% reached a speech development equal to their normal-hearing peers according to their own estimation. Over half of the adolescents showed good communicative skills, whereof 71% were able to tell people who they do not know what they are concerned about. Only the child with the severe developmental delay was not able to communicate by oral language. These findings are in accordance with publications in which a negative impact of accompanying disorders on hearing benefit and speech and language development outcomes in comparison to an isolated ANSD was described [30,33,50,51]. The questionnaire used in the present study indeed is a merely subjective assessment by the parents, but similar results are described in previous studies: Tobey et al. and Geers and Sedey demonstrated that speech competence considerably improved between elementary school
Fig. 5. Performance at primary school (left) and secondary school (right) shown in grades (1–6).
both at school and in their free time. They participated in sports and dance clubs, were members of a firefighting or shooting club, and played an instrument. 3.2.3. Hearing device use All children who were provided with a hearing device on both sides used their hearing device every day, although two children have no speech understanding with the CI on one side. The child with singlesided ANSD, who got a HA at an early age, is a non-user due to missing benefit. 4. Discussion To date, there is no information available on the long-term outcomes in children with ANSD. Apart from the present study, there is no report on such a long surveillance period and follow-up of children with ANSD. Our study followed up some children for nearly 20 years and therefore, we monitored their progress through childhood and into adulthood. To establish a safe diagnosis of ANSD and to identify the possible site of lesion and, therefore, to be able to determine the prognosis of ANSD, it is necessary to complete different diagnostic steps. A normal function of the outer hair cells with detectable OAE [41] and CM [42] and an abnormal integration of the function of the CN with lost or conspicuous ABR responses [4] are rated as distinct diagnostic criteria. But outer hair cell function could be lost over time [43,44]. The acoustic middle ear muscle reflex is not detectable in ANSD or only detectable with high thresholds [45,46]. Furthermore, it is essential to obtain cMRI and CT images of the auditory nerve and brainstem and behavioral audiological measurements. Benefit from amplification in children with ANSD is still under discussion and depends on audiological test results and developments in hearing and speech abilities. Due to general development, and the possible presence of accompanying disorders, there is a huge variation in speech perception and language development amongst these children. Recommendations concerning amplification range from no amplification at all across HAs to provision with CIs. Some authors showed that children with ANSD had similar results with HAs compared to children with cochlear hearing loss [17] or when compared with children with ANSD and CIs [16]. Humphriss et al. demonstrated an equivalent speech recognition ability in CI and HA users with ANSD in a sample of 27 studies [18]. Other authors described no benefits in hearing and communication with HAs in ANSD [4,19]. There are several early studies, in which a higher benefit from CIs than from HAs was reported in children with ANSD [20–23]. Roush and Liu demonstrated an improved PTA in all children provided with HAs or CIs in severe to 5
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is essential to follow-up the child with routine audiological tests and radiological imaging. Until now, there is no literature available on longterm results of children with ANSD. Present data show that the hearing and speech development outcomes in children with ANSD are very heterogeneous depending on accompanying disorders. With intensive special supporting measures, regular schools can be attended and social education be achieved. More studies with a higher number of children are needed to widen the knowledge on long-term outcomes with ANSD and to compare rehabilitation results of children provided with HAs or CIs. This probably can improve the counselling of parents and children regarding treatment and prognosis and avoid unrealistic expectations especially in children with accompanying disorders.
and high school in prelingually deaf children who received a CI/CIs at an early age l [52,53]. The preferred communication mode of 85% (6 of 7) of the adolescents in the present study is oral language. This is consistent with results from the literature that 75% of students with CIs and cochlear hearing loss use spoken language to communicate [54]. Illg et al. (2017) demonstrated that the long-term educational, vocational, and occupational outcomes in people with cochlear hearing loss who received a CI/CIs in childhood are significantly poorer than the German and worldwide normal-hearing average. 65% of these children attended schools for hearing-impaired children [55]. In the present study, only two children attended a mainstream/regular primary school: the child with the single-sided ANSD and the child with progressive hearing loss. This is most likely due to normal hearing on the contralateral side in the child with single-sided ANSD. The child with the progressive hearing loss had good residual hearing in early childhood and deafened postlingually. In addition, the child with the progressive hearing loss is the only one of the adolescents who obtained a general qualification for university entrance. Four of seven children (57%) attended special schools for hearing and speech, which is in accordance with the study of Illg et al. (2017). Two of these children graduated with a qualified certificate of secondary education/subjectrelated entrance qualification. Previous studies described that children with a hearing loss are at a higher risk for adverse psychosocial outcomes such as troubled relationships, loneliness and behavior problems [56,57]. These limitations of social development in children with hearing loss or deafness are due to restricted possibilities to learn to understand others, to develop self-awareness and independent thinking and problem-solving skills [58]. However, in a recent publication by Sarant et al. (2018), the authors found no significant difference in the psychosocial development of children aged between 5 and 8 years who received CIs relatively early compared to their normal hearing peers [59]. In the present study, all but the one child with the severe developmental delay are socially well integrated, both at school and in their free time. They all participate in sport and dance clubs, are members of the firefighting or shooting club, and even play an instrument. This corresponds to the article of Moog et al. (2011) where 94% of adolescent CI users had extracurricular activities like sports in high school [54]. However, this aspect of development needs to be further investigated. The results of recent literature concerning the long-term outcomes in children with profound cochlear hearing loss or deafness and the present study on the long-term outcomes in children with ANSD are promising. However, children with ANSD are a special group within the hearing-impaired population and therefore are not generally comparable to people with cochlear hearing loss. The decision for the right hearing amplification in children with ANSD is still difficult because of the heterogeneous nature of ANSD. Behavioral audiological measures and objective measures as ABR, OAE and CM allow for a safer diagnosis of this disease pattern. However, in order to exclude CND, cMRI and eBERA are mandatory. Using cMRI, aplasia or hypoplasia of the CN can be visualized and the presence of eBERA responses strongly correlates with a positive outcome with CIs [60]. Furthermore, genetic diagnosis is recommendable in all cases. Amplification in children with ANSD must always be a case-by-case decision depending on the individual's performance, speech perception (in quiet and noise), language development, spoken language skills, global development, and the presence of accompanying disorders. For further improvements in counselling these special group of hearingimpaired children and their parents additional data on long-time outcomes are needed.
Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Declaration of competing interest The authors disclose no conflicts of interest. Acknowledgements Ursula Lehner-Mayrhofer (MED-EL) edited the English language on a version of this manuscript. The first author got a state doctorate scholarship from the Interdisciplinary Center for clinical Science (Interdisziplinäres Zentrum für Klinische Forschung (IZKF)) at the University of Wuerzburg. References [1] G. Rance, D.E. Beer, B. Cone-Wesson, R.K. Shepherd, R.C. Dowell, A.M. King, Clinical findings for a group of infants and young children with auditory neuropathy, Ear Hear. 20 (1999) 238–252. [2] G. Rance, Auditory neuropathy/dys-synchrony and its perceptual consequences, Trends Amplif. 9 (2005) 1–43. [3] A. Foerst, D. Beutner, R. Lang-Roth, A. Foerst, Prevalence of auditory neuropathy/ synaptopathy in profound hearing impaired children, Int. J. Pediatr. Otorhinolaryngol. 70 (8) (2006) 1415–1422. [4] A. Starr, T.W. Picton, Y. Sininger, L.J. Hood, C.I. Berlin, Auditory neuropathy, Brain 119 (1996) 741–753. [5] G. Rance, A. Starr, Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy, Brain 138 (2015) 3141–3158. [6] G.M. de Carvalho, Performance of cochlear implants in pediatric patients with auditory neuropathy spectrum disorder, J. Int. Adv. Otol. 12 (1) (2016) 8–15. [7] K. Rajput, M. Saeed, J. Ahmed, M. Chung, C. Munro, S. Patel, C. Leal, D. Jiang, R. Nash, Findings from aetiological investigation of Auditory Neuropathy Spectrum Disorder in children referred to cochlear implant programs, Int. J. Pediatr. Otorhinolaryngol. 116 (2019) 79–83. [8] S.B. Amin, H. Wang, N. Laroia, M. Orlando, Unbound bilirubin and auditory neuropathy spectrum disorder in late preterm and term infants with severe jaundice, J. Pediatr. 173 (2016) 84–89. [9] K. Kanga, E. Kitazumi, K. Kadama, Auditory brainstem response of kernicterus infants, Int. J. Pediatr. Otorhinolaryngol. 1 (1979) 255–264. [10] S. Sawada, N. Mori, R.J. Mount, R.V. Harrison, Differential vulnerability of inner and outer hair cell systems to chronic mild hypoxia and glutamate ototoxicity: insights into the cause of auditory neuropathy, J. Otolaryngol. 30 (2001) 106–114. [11] V.K. Manchaiah, F. Zhao, A.A. Danesh, R. Duprey, The genetic basis of auditory neuropathy spectrum disorder (ANSD), Int. J. Pediatr. Otorhinolaryngol. 75 (2011) 151–158. [12] J. Levi, J. Ames, K. Bacik, C. Drake, T. Morlet, R.C. O'Reilly, Clinical characteristics of children with cochlear nerve dysplasia, Laryngoscope 123 (2013) 752–756. [13] G. Rance, C. McKay, D. Grayden, Perceptual characterization of children with auditory neuropathy, Ear Hear. 25 (2004) 34–46. [14] G. Rance, E. Barker, M. Mok, R.C. Dowell, A. Rincon, R. Garrat, Speech perception in noise for children with auditory neuropathy/dys-synchrony type hearing loss, Ear Hear. 28 (2007) 351–360. [15] F.G. Zeng, Y.Y. Kong, J.H. Michalewski, A. Starr, Perceptual consequences of disrupted auditory nerve activity, J. Neurophysiol. 93 (2005) 3050–3063. [16] S. Pelosi, G. Wann, C. Hayes, L. Sunderhaus, D.D. Haynes, M.L. Bennett, R.F. Labadie, A. Rivas, Cochlear implantation versus hearing amplification in patients with Auditory Spectrum Disorder, Otolaryngol. Head Neck Surg. 148 (5) (2013) 815–821. [17] E.A. Walker, R.W. McCreery, M. Spratford, E.A. Walker, Children with ANSD fitted with hearing aids applying the AAA pediatric amplification guideline: current
5. Conclusion Diagnosis and treatment of ANSD in children are still challenging. To give a prognosis about the possible development of these children, it 6
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Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD)
T
Désirée Ehrmann-Müller∗, Daniela Back, Heike Kühn, Rudolf Hagen, Wafaa Shehata-Dieler Department of Otorhinolaryngology, Plastic, Esthetic and Reconstructive Head and Neck Surgery, University of Wuerzburg, Josef-Schneider-Str. 11, 97080, Wuerzburg, Germany
A R T I C LE I N FO
A B S T R A C T
Keywords: Auditory neuropathy spectrum disorder Cochlear implantation Hearing and speech development Education
Introduction: The present article shows long-term results in the hearing and speech development of children with auditory neuropathy spectrum disorder (ANSD). Some children were followed up for nearly 20 years, monitoring their progress through childhood into adulthood. Methods: This retrospective study examined data from 10 children who were diagnosed and treated at our tertiary referral center. All children were provided with hearing aids and/or cochlear implants. The children's hearing and speech development and their social and educational development were evaluated. Results: Seven children were provided with cochlear implants on both sides; three children, one of which had single-sided deafness, received hearing aids. All children with cochlear implants on both sides used their devices full-time. Speech perception ranged between 100% and 0% on one side. Five children attended a school for the hearing impaired; four children attended a regular school. Four children attended vocational training. Conclusions: At present, there is a lack of literature on the long-term outcomes of treatment in children with ANSD. The data presented show that the hearing and speech development in children with ANSD are significantly heterogeneous. Regular school education and social integration of children with ANSD can be achieved with intensive and supportive rehabilitative methods.
1. Introduction With a prevalence of 7–10%, auditory neuropathy spectrum disorder (ANSD) is a relevant cause of sensorineural hearing loss (SNHL) [1–3]. Preserved otoacoustic emissions (OAE) and cochlear microphonics (CM) and absent or severely distorted auditory brainstem responses (ABR) are the characteristics of ANSD [4]. Lesions at different sites like presynaptic, postsynaptic or central lesions may cause ANSD [5]. The most common predisposing factor for ANSD is prematurity [6]. Perinatal hyperbilirubinemia [7–9], hypoxia and ototoxic medication [10] are further risk factors for ANSD. 40% of the children with ANSD have an underlying genetic factor [11]. In up to one third of the children affected, ANSD is caused by cochlear nerve dysplasia (CND) [7,12]. The characteristics of ANSD are specified as fluctuating hearing sensitivity in pure tone thresholds, reduced speech perception performance especially in difficult hearing situations like in noise, and reduced sound localization ability [1,2,4,13–15]. Recommendations on treatment options in the literature range from no amplification at all, frequency modulating systems (FM systems) and hearing aids (HAs) in mild to moderate hearing loss, up to the provision
∗
of a cochlear implant (CI). There are publications which report good hearing rehabilitation with HAs in children with ANSD [16–18]. Some demonstrate outcomes similar to those obtained through provision with CIs [16,17]. In contrast, there are studies which describe a lack of benefits in hearing and communication with HAs in children with ANSD [4,19]. Dean et al. showed that children with ANSD, who do not benefit from HAs, perform well if provided with CIs [20]. Most studies show a higher benefit with a CI compared to a HA in children with ANSD [21–23]. Significant improvements in speech perception, language development and communication outcomes with CIs that are comparable to results in children with cochlear SNHL have been described in the literature [6,24–30]. However, outcomes with CIs in children with ANSD vary considerably. Some authors reported poor performance with CIs [31] or a minimal benefit from CIs in 25% of the children with ANSD [32–34]. The presence of CND seems to promote the efficacy of cochlear implantation [28,30,35–39]. The study of Breneman et al., 2012 on the speech outcomes in children with CND showed similar longterm outcomes compared to matched peers with cochlear hearing loss. To date, according to our knowledge, there are no data published on
Corresponding author. E-mail address: [email protected] (D. Ehrmann-Müller).
https://doi.org/10.1016/j.ijporl.2020.109938 Received 28 October 2019; Received in revised form 27 January 2020; Accepted 6 February 2020 Available online 07 February 2020 0165-5876/ © 2020 Published by Elsevier B.V.
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(author number 3). It puts the main focus on which kindergarten or school the children attended including the parents’ reasons for choosing a specific kindergarten or school. The parents had to tick which form of kindergarten or school the children attended. Another focus of the questionnaire is how the children with ANSD perform in comparison to their normal-hearing peers at school. Concerning motoric, mental speech and social development, the parents had to mark if their child was far behind, weaker, equal, better or far better than children with normal hearing. In addition they had to tick the grades their children got in the different school subjects. Furthermore, it collects information about the use of the hearing device (tick how long the hearing device is worn per day: day and night - all day - hours - not) and the social integration and acceptance of the child (mark if the children had friends, hobbies [40]. Data analysis was performed with Graph Pad Prism and Microsoft Office Excel. This retrospective study was approved by the authorized ethics committee (2019022003) and was conducted according to the guidelines established in the Declaration of Helsinki (Washington 2002) and the ISO 14155 Parts I and II for the conduct of clinical studies involving humans.
long-term hearing and speech development outcomes and the educational and social development of children with ANSD. Due to the very heterogeneous nature of ANSD, which often occurs in combination with severe accompanying disorders, there is still an ongoing discussion on the benefit of amplification in children with ANSD. Diagnosis and adequate treatment of this hearing disorder are still quite challenging, especially in children. Therefore, it is important to collect long-term outcomes of this cohort. The aim of this study was to collect and analyze long-term hearing and speech development outcomes and the social and educational development of children with ANSD. 2. Material and methods Ten children (eight females, two males) who were diagnosed with ANSD were examined at our tertiary referral center. A retrospective analysis of the data collected from the charts was performed. The children presented at the comprehensive hearing center (CHC) of the clinic because of various risk factors, a failed newborn hearing screening (NHS) or were missing speech and language development. Subjective audiological evaluation included a free/sound field or pure tone audiometry (PTA) and a speech audiometry if possible. As clinical audiometer a WESTRA CAD03 (Westra Electronic GmbH) calibrated according to DIN (Deutsches Institut für Normung) was used. Objective tests included: OAEs, impedance metry, click-evoked ABR, auditory steady state responses (ASSR), and an electrocochleography. ABR and ASSR-measurements were performed with an eclipse 1834446 (Interacoustics, Denmark) or a WESTRA ERA Q/S-02 (Westra Electronic GmbH). Electrocochleography was performed with a Nicolet Synergy EDX. All tests were conducted in a sound-treated room (IAC model 55). As inclusion criteria for ANSD missing or conspicuous responses in ABR and the presence of OAE or typical CM morphology were rated. Children with missing ABR and missing OAE and missing CM pattern in ABR or electrocochleography were excluded from the study. Radiological diagnostics included cerebral magnetic resonance imaging (cMRI) and in some cases computed tomography (CT) of the temporal bone. Age at first presentation, age at diagnosis, age of provision with HA or CI and risk factors were documented. During follow up, the hearing and speech development and the social development of these children were investigated at regular intervals. Audiometric evaluation included aided free field audiometry at 3 frequencies (500, 1000 and 2000Hz), speech discrimination tests in quiet tested with the Freiburgers' monosyllabic test, and speech discrimination in noise tested with the Hochmair-Schulz-Moser sentence test (HSM). Furthermore, the use of the hearing device and the education level of each child were investigated using a special parent questionnaire (“(Eltern)fragebogen zur Erfassung der Bildungslaufbahn hörgeschädigter Kinder”, BILDHOEKI) which focuses on the educational development of children with a hearing impairment. The questionnaire was developed and validated by a psychologist at our center
3. Results Seven children were provided with CIs on both sides. One of these had progressive hearing loss. Three children got HAs, one of them had single-sided deafness and received a HA only in one ear. Six of these children had no risk factors and four children had known risk factors for ANSD like prematurity, genetic cause (OTOF compound heterozygous, Pendred) or craniofacial syndrome. The median age at the first presentation at the clinic was 19.5 months (range: 2–53 months). Diagnosis was established shortly after, at a median age of 35 months (range: 3–53 months). In one child, it took longer to diagnose ANSD due to the presence of additional comorbidities. Provision of suitable hearing devices was obtained at a median age of 48 months (range: 4–82 months). The follow-up of the children averaged at a median of 14.6 years (11.6–19.2 years). All of the children were older than 16 at the time of the present analysis. Further demographics are available in Table 1. OAE could be found in all children initially and disappeared in two children over time. In ABR, six children had missing bilateral responses, of whom one child showed hearing thresholds between 35 and 75 dB HL without amplification. Another one had hearing thresholds between 75 and 80 dB HL. In the other children with missing ABR responses only hearing thresholds less than 100 dB or no hearing thresholds at all could be observed. The one child, who was provided with bilateral HA showed ABR responses at 100 and 90 dB nHL and her subjective hearing threshold ranged between 40 and 70 dB HL. The child with the single sided ANSD showed responses in ABR only at 100 dB nHL and the hearing threshold lay between 55 and 65 dB HL. Another child had responses in ABR at 90 and 100 dB nHLwith hearing thresholds
Table 1 Participant demographics. P
Sex
Age at definite provision (in months)
Device
Cause of ANSD
Accompanying disorders
Years of follow up
1 2 3 4 5 6 7 8 9 10
f m m f f f f f f f
56 82 40 4 49 30 72 47 39 71
MED-EL PULSARci 100 MED-EL PULSARci 100 MED-EL PULSARci 100 HA MED-EL PULSARci 100 MED-EL PULSARci 100 MED-EL PULSARci 100 HA HA C40 + r/CONCERTO flex soft l
Craniofacial syndrome Unknown Unknown Unknown Unknown OTOF comp. heterozygot Prematurity, 31st gw Unknown Unknown Pendred syndrome
Craniofacial syndrome None None None None None None None Developmental delay None
19.2 11.6 14.2 17.8 14.4 19.1 12.5 12.1 17.4 14.8
p indicates participant, f female, m male, HA hearing aid, CI cochlear implant, mo months, y years, gw gestational week, comp. compound, r right, l left. 2
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Fig. 1. Aided AAT with CI or HA, best over time, dots represent each child in the study (grey dots = HA user, light grey: child with HA on both sides, dark grey: child with single sided HA; black = CI user, black filled: children with profound hearing loss, black frame: child with severe hearing loss, black and with: child with moderate hearing loss). Fig. 2. Aided speech perception in noise with CI, best over time, number of participants shown in dots; HSM indicates Hochmair- Schulz- Moser-test, SNR signal to noise ratio.
between 70 and 100 dB HL. And the child with progressive hearing loss showed responses in ABR at 50 and 60 dB nHL. Unfortunately, no hearing thresholds without amplification were conducted.
noise and in quiet on one side, the one with the OTOF-mutation and another one with profound hearing loss (see Fig. 2).
3.1. Hearing development
3.2. Results of the BILDHOEKI questionnaire
Averaging auditory thresholds (AAT) at 500, 1000 and 2000 Hz and speech in quiet and noise was tested separately for each ear with amplification. All children except the one with single sided ANSD hat symmetric bilateral hearing loss. Without amplification four children had profound hearing loss, three children suffered from a severe hearing loss and three children had a moderate hearing loss. After provision the degree of hearing loss decreased according to the aided thresholds to moderate hearing loss in five children, mild to moderate in the child with bilateral HA and mild in the child with single sided ANSD. In three children, including the one with the OTOF mutation and the one with progressive hearing loss, no AAT improvement was observed after amplification. The median AAT at all frequencies with a HA or CI was 35–40 dB (see Fig. 1). Due to the low sample size, a reasonable comparison between AAT with CIs and AAT with HAs was not possible. The speech perception outcomes ranged from 100% on the HSM-test to 0% on one side on the Freiburgers' monosyllabic test. Since the child with the single-sided ANSD was a non-user of the HA, speech discrimination could not be tested. In another child, speech perception could not be tested because of a severe developmental delay. The aided speech perception in quiet tested with the Freiburgers' monosyllabic test at 65 dB was 50% and higher in most children with a median of 72.5%. At 80 dB, speech perception was 60% and higher in all but one child, who had a median of 85%. Due to the low sample size and only one HA user, a comparison of speech perception with HAs and with CIs was not possible. The only child that used HA on both sides had a speech perception in quiet of 95% on both sides. The child with the progressive hearing loss reached a speech perception of 95 and 75% at 65 dB, whereas the other children with CI reached a speech perception between 20 and 85% at 65 dB. There was no difference between the children with moderate or profound hearing loss. In the child with OTOF-mutation no results of Freiburgers' test were elevated. Speech perception in noise with the HSM was performed in the seven children with CIs. 70% of the children had a speech perception score in noise (at 65 dB and a SNR of 10 dB) of 68% or higher, including two children with profound hearing loss, one with moderate hearing loss and the one with progressive hearing loss. Four of the seven children had a speech perception score in noise (at 65 dB and a SNR of 5 dB) of 80% or higher. Two children had a speech perception of 0% in
The BILDHOEKI questionnaire was returned by seven of the ten parents and children (70%). In the three remaining patients, two were provided with CIs and one with bilateral hearing aids, no further information was obtained either in written form or by telephone. Only relevant items of the questionnaire concerning the topic of the present study were analyzed. 3.2.1. Speech development The native language of five children was German only, two of the children were trained bilingually. Six children used oral communication, two also used sign language (the one with OTOF-mutation and one with moderate hearing loss). One child with a severe developmental delay was not able to communicate orally and used rudimentary sign language only. In preschool, 57% of the parents estimated their children to have a speech development that was far behind or weaker than their normal hearing peers. 57% of the children (4 of 7) self-reported a speech development equal to their normal hearing peers at secondary school (see Fig. 3). The child with the progressive hearing loss always had an equal speech development as normal hearing peers. The one with the OTOFmutation was always far behind. Speech production and speech understanding were also far behind or weaker in the children with ANSD compared to their normal hearing peers (see Fig. 4). The motor activity, mental and social competence of the children with ANSD were mostly estimated by the parents to be equal or better than the ones of their normal hearing peers (see Fig. 4). Over 50% of the parents rated their children's communicative skills as good. 71% of the children were able to tell both people that they know and people that they do not know what they are concerned about. The child with the severe developmental delay was not able to do so. All children were able to get in contact with others and had no problems understanding people. 3.2.2. Education and social career As shown in Table 1, one child had an unknown craniofacial syndrome and another child had a severe developmental delay of unknown origin. 3
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Fig. 3. Estimation of speech development compared to normal hearing peers in different periods of the educational life.
teacher trained in providing support for children with hearing loss. The child with progressive hearing loss changed to a mainstream/regular grammar school after finishing junior high school. Four children attended a special secondary school for hearing-impaired children including the one with the OTOF-mutation. The child with developmental delays continued in a school for children with additional needs. For the performance of the children at primary and secondary school with grades for each subject see Fig. 5. Grade 1 is the best mark in Germany, grade 6 the worst. The child with progressive hearing loss finished school with a general qualification to enter university and two other children, the one with OTOF-mutation and the one with severe hearing loss obtained a certificate of secondary education/subject-related entrance qualification. The remaining children are still attending school. Three of the children have already begun an apprenticeship/vocational training amongst others the child with OTOF-mutation. Six children were completely integrated in all aspects of social life,
Four children attended a regular kindergarten where children with additional needs are also accepted. One of the four children had to change to an institution for hearing-impaired children. Two children started in a kindergarten for hearing-impaired children from the beginning. The child with the severe developmental delay attended a kindergarten for children with additional needs. The child with the single-sided ANSD and the one with progressive hearing loss attended a mainstream/regular primary school with occasional assistance of a teacher trained in providing additional support for children with special needs and/or hearing loss. Four children visited a special school for hearing and speech including the one with the OTOF-mutation and the child with the severe developmental delay attended a school for children with additional needs. Regarding the secondary school career, the child with the singlesided ANSD and the child with progressive hearing loss attended a mainstream/regular junior high school; the one with the single-sided ANSD attended a junior high school with occasional assistance of a
Fig. 4. Estimation of general preschool development compared to normal hearing peers. 4
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profound hearing loss due to ANSD [29,34]. Liu et al. showed an average four frequency aided hearing level with CIs in children with ANSD from 17.5 to 57.5 dB. The PTA values in the present study ranged from 20 to 60 dB (median over frequencies: 27.5–37.5 dB). The results of aided PTA are relatively good and constant in all of the children. This is in contrast to the quite heterogeneous results of speech perception, especially in noise, which is typical for ANSD and has been frequently reported in individuals with ANSD. As shown in Fig. 3, the aided median speech perception in quiet at 65 dB was 72.5% and 85% at 80 dB. These varied from 0 to 95% at 65 dB and from 0 to 100% at 80 dB. A difference between CIs and HAs could not be assessed due to the low number of children in our study. The only tested HA user had a speech perception score of 95% in quiet on both sides. These results are likely due to a moderate hearing loss (see Fig. 2: unaided pure tone audiometry). The speech perception outcomes in noise varied from 0 to 100% amongst the children. These results are in accordance with Breneman et al. who reported that 91% of 35 ANSD children with CIs had some degree of open-set word recognition and at least 80% reached an open-set speech perception in the long-term follow-up [30]. In the present study, 90% of the children had some degree of open-set word recognition and 70% reached open-set speech perception. In other studies significant improvements in speech perception and language development with the use of CIs in ANSD were described, which were comparable to results in children with cochlear hearing loss [6,24–26]. In previous publications, an early amplification is recommended, either by HAs or by CIs, because these children have a higher benefit than those provided at an older age [26]. A correlation between hearing and speech performance and age at amplification could not be calculated in the present study due to the small sample size. 7–10% of the cases of sensorineural hearing loss in children are associated with ANSD and are often caused by the previously mentioned risk factors (see Introduction and Fig. 1). In the present study, 40% of the children had risk factors. In the literature, CND is present for between 18% [47] and 33% of children with ANSD [7,12]. There was no evidence of a CND in these ten children. However, with no speech understanding with CIs on one side in two children (P1 and P6), a CND might be assumed. One of these children was not examined by a cMRI, in the other child there was no description of such a finding in cMRI, but this was some years ago. Modern radiological diagnostics, e.g. MRI including CISS ((Constructive Interference in Steady State)-sequence, a strong T2-weighted sequence which enables a high depiction of the nerves in the cerebellopontine angle) allows a better and more detailed judgement on the presence and state of the cochlear nerve (CN) [48,49]. In previous studies that have reported minimal benefit from CIs in 25% of the children with ANSD [32–34] or even very poor performances using CIs [31], accompanying disorders or a CND are often described. Indeed, Teagle et al. showed that 50% of 140 children demonstrated open set speech perception, but some had no benefit due to a lack of electrical-induced neural synchronization, CND or other associated conditions, such as syndromes or developmental delays [33]. In the present study, speech development, subjectively estimated by the parents, improved from preschool to secondary school in nearly all adolescents, whereas 57% reached a speech development equal to their normal-hearing peers according to their own estimation. Over half of the adolescents showed good communicative skills, whereof 71% were able to tell people who they do not know what they are concerned about. Only the child with the severe developmental delay was not able to communicate by oral language. These findings are in accordance with publications in which a negative impact of accompanying disorders on hearing benefit and speech and language development outcomes in comparison to an isolated ANSD was described [30,33,50,51]. The questionnaire used in the present study indeed is a merely subjective assessment by the parents, but similar results are described in previous studies: Tobey et al. and Geers and Sedey demonstrated that speech competence considerably improved between elementary school
Fig. 5. Performance at primary school (left) and secondary school (right) shown in grades (1–6).
both at school and in their free time. They participated in sports and dance clubs, were members of a firefighting or shooting club, and played an instrument. 3.2.3. Hearing device use All children who were provided with a hearing device on both sides used their hearing device every day, although two children have no speech understanding with the CI on one side. The child with singlesided ANSD, who got a HA at an early age, is a non-user due to missing benefit. 4. Discussion To date, there is no information available on the long-term outcomes in children with ANSD. Apart from the present study, there is no report on such a long surveillance period and follow-up of children with ANSD. Our study followed up some children for nearly 20 years and therefore, we monitored their progress through childhood and into adulthood. To establish a safe diagnosis of ANSD and to identify the possible site of lesion and, therefore, to be able to determine the prognosis of ANSD, it is necessary to complete different diagnostic steps. A normal function of the outer hair cells with detectable OAE [41] and CM [42] and an abnormal integration of the function of the CN with lost or conspicuous ABR responses [4] are rated as distinct diagnostic criteria. But outer hair cell function could be lost over time [43,44]. The acoustic middle ear muscle reflex is not detectable in ANSD or only detectable with high thresholds [45,46]. Furthermore, it is essential to obtain cMRI and CT images of the auditory nerve and brainstem and behavioral audiological measurements. Benefit from amplification in children with ANSD is still under discussion and depends on audiological test results and developments in hearing and speech abilities. Due to general development, and the possible presence of accompanying disorders, there is a huge variation in speech perception and language development amongst these children. Recommendations concerning amplification range from no amplification at all across HAs to provision with CIs. Some authors showed that children with ANSD had similar results with HAs compared to children with cochlear hearing loss [17] or when compared with children with ANSD and CIs [16]. Humphriss et al. demonstrated an equivalent speech recognition ability in CI and HA users with ANSD in a sample of 27 studies [18]. Other authors described no benefits in hearing and communication with HAs in ANSD [4,19]. There are several early studies, in which a higher benefit from CIs than from HAs was reported in children with ANSD [20–23]. Roush and Liu demonstrated an improved PTA in all children provided with HAs or CIs in severe to 5
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is essential to follow-up the child with routine audiological tests and radiological imaging. Until now, there is no literature available on longterm results of children with ANSD. Present data show that the hearing and speech development outcomes in children with ANSD are very heterogeneous depending on accompanying disorders. With intensive special supporting measures, regular schools can be attended and social education be achieved. More studies with a higher number of children are needed to widen the knowledge on long-term outcomes with ANSD and to compare rehabilitation results of children provided with HAs or CIs. This probably can improve the counselling of parents and children regarding treatment and prognosis and avoid unrealistic expectations especially in children with accompanying disorders.
and high school in prelingually deaf children who received a CI/CIs at an early age l [52,53]. The preferred communication mode of 85% (6 of 7) of the adolescents in the present study is oral language. This is consistent with results from the literature that 75% of students with CIs and cochlear hearing loss use spoken language to communicate [54]. Illg et al. (2017) demonstrated that the long-term educational, vocational, and occupational outcomes in people with cochlear hearing loss who received a CI/CIs in childhood are significantly poorer than the German and worldwide normal-hearing average. 65% of these children attended schools for hearing-impaired children [55]. In the present study, only two children attended a mainstream/regular primary school: the child with the single-sided ANSD and the child with progressive hearing loss. This is most likely due to normal hearing on the contralateral side in the child with single-sided ANSD. The child with the progressive hearing loss had good residual hearing in early childhood and deafened postlingually. In addition, the child with the progressive hearing loss is the only one of the adolescents who obtained a general qualification for university entrance. Four of seven children (57%) attended special schools for hearing and speech, which is in accordance with the study of Illg et al. (2017). Two of these children graduated with a qualified certificate of secondary education/subjectrelated entrance qualification. Previous studies described that children with a hearing loss are at a higher risk for adverse psychosocial outcomes such as troubled relationships, loneliness and behavior problems [56,57]. These limitations of social development in children with hearing loss or deafness are due to restricted possibilities to learn to understand others, to develop self-awareness and independent thinking and problem-solving skills [58]. However, in a recent publication by Sarant et al. (2018), the authors found no significant difference in the psychosocial development of children aged between 5 and 8 years who received CIs relatively early compared to their normal hearing peers [59]. In the present study, all but the one child with the severe developmental delay are socially well integrated, both at school and in their free time. They all participate in sport and dance clubs, are members of the firefighting or shooting club, and even play an instrument. This corresponds to the article of Moog et al. (2011) where 94% of adolescent CI users had extracurricular activities like sports in high school [54]. However, this aspect of development needs to be further investigated. The results of recent literature concerning the long-term outcomes in children with profound cochlear hearing loss or deafness and the present study on the long-term outcomes in children with ANSD are promising. However, children with ANSD are a special group within the hearing-impaired population and therefore are not generally comparable to people with cochlear hearing loss. The decision for the right hearing amplification in children with ANSD is still difficult because of the heterogeneous nature of ANSD. Behavioral audiological measures and objective measures as ABR, OAE and CM allow for a safer diagnosis of this disease pattern. However, in order to exclude CND, cMRI and eBERA are mandatory. Using cMRI, aplasia or hypoplasia of the CN can be visualized and the presence of eBERA responses strongly correlates with a positive outcome with CIs [60]. Furthermore, genetic diagnosis is recommendable in all cases. Amplification in children with ANSD must always be a case-by-case decision depending on the individual's performance, speech perception (in quiet and noise), language development, spoken language skills, global development, and the presence of accompanying disorders. For further improvements in counselling these special group of hearingimpaired children and their parents additional data on long-time outcomes are needed.
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