- Email: [email protected]
Universal newborn hearing screening in the Champagne-Ardenne region: A 4-year follow-up after early diagnosis of hearing impairment
International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Universal newborn hearing screening in the Champagne-Ardenne region: A 4-year follow-up after early diagnosis of hearing impairment T. Langagne *, M. Le´veˆque, P. Schmidt, A. Chays Department of Otolaryngology and ENT Surgery, Robert Debre´ University Hospital, 125 avenue du Ge´ne´ral Koenig, 51100 Reims, France
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 May 2010 Received in revised form 27 June 2010 Accepted 3 July 2010 Available online 31 July 2010
Introduction: Permanent congenital hearing loss is one of the most frequent congenital anomaly at birth. Universal newborn hearing screening (UNHS) was introduced in numerous countries in order to allow an early diagnosis and intervention for congenital hearing impairment. Objective: First aim of this study is to evaluate the accuracy of early diagnosis of hearing impairment after UNHS. Second aim is to discuss the auditory intervention proposed after this diagnosis. Last aim is to evaluate the relevance of UNHS for early diagnosis and intervention. Materials and methods: Prospective study. UNHS program was introduced in the entire French region of Champagne-Ardenne in January 2004. Forty-one children have benefited of an early diagnosis of hearing impairment until June 2007. They were included in an intervention program consisting of an audiometric follow-up and an auditory intervention. This program was conducted until June 2008. Results: There were 28 males patients and 13 females patients. The diagnosis of hearing aid impairment was carried at an average age of 3.2-month. The auditory follow-up allowed confirming the initial diagnosis of deafness for the majority of the children as for their degree of hearing loss. Auditory intervention was heterogeneous depending on degree of hearing loss of the children. Conclusion: This UNHS program demonstrates its validity and feasibility for early diagnosis and intervention of congenital hearing impairment. It brought a major impact on the management of congenital hearing impairment in Champagne-Ardenne. ß 2010 Elsevier Ireland Ltd. All rights reserved.
Keywords: Universal hearing screening Congenital deafness Diagnosis Hearing aid fitting
1. Introduction Permanent congenital hearing loss is one of the most common congenital anomaly. Bilateral, medium to profound hearing impairment is estimated to occur in 1 infant per 1000 [1]. The impact of such hearing loss on speech and language acquisition, academic achievement and social development is well known. The goal of universal newborn hearing screening (UNHS) is thus to allow an early diagnosis of congenital deafness and an early intervention for these children. In Champagne-Ardenne, UNHS has been effective from January 2004; its aim is to identify all children with permanent bilateral hearing impairment more than 35 dB. UNHS was applied to 54790 infants from January 2004 to June 2007. Forty-one children were diagnosed with hearing impairment. In this work, we describe the epidemiological data of this group, their audiologic status and the modality of auditory intervention. First aims of this study are to evaluate the accuracy of early diagnosis and auditory intervention. Second purpose is to evaluate
* Corresponding author. Tel.: +33 326787125; fax: +33 326788982. E-mail address: [email protected] (T. Langagne). 0165-5876/$ – see front matter ß 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2010.07.005
the relevance of UNHS for both early diagnosis and intervention for hearing aid impairment. 2. Methods UNHS has already been described [2]. The method consists of a two-stage screening. Every child being born in ChampagneArdenne benefits of a first test in the third day of life before hospital discharge. The first test can either be automated Transient Evoked Otoacoustic Emission (TEOAE) in maternities or automatic Auditory Brainstem Response (aABR) in neonatal intensive care unit. The procedure is considered a positive screening test in case of bilateral absence of TEOAE/aABR. In that case, a second test (retest) is performed by a physician 15 days after hospital’s discharge. When both ears fail to pass the retest, TEOAE or aABR, the baby is referred to the regional referent centre, based in Reims, for diagnostic tests. The diagnostic tests include behavioural tests and a clickevoked ABR. ABR is realized under general anaesthesia, or light sedation. Indeed, the child has to remain calm to avoid interferences. Analysed ABR parameters are essentially the auditory thresholds and the latencies of wave I, III and V. The tests’ results are exposed in presence of various actors of the
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
intervention: otologist, speech therapist, psychologist and hearing-aid specialist. It is a long discussion during which the diagnosis of deafness, the project of intervention and perspectives of evolution for the child are presented. From January 2004 to June 2007, 41 children were diagnosed with a hearing loss; they were all enrolled in a prospected study conducted until June, 2008.
1165
The data presented in this study are the one of a hearing-aid specialist in Champagne-Ardenne, dealing with the great majority of children with congenital hearing loss. These data concern 221 children, born between 1987 and 2003. All the data were stored in the database system.
3. Results 2.1. Profile of cases A medical evaluation was realized for every child in the referent centre. It includes a detailed pedigree analysis for congenital hearing loss, medical history, associated handicaps and research for risk factors, as identified by the guidelines of the Joint Committee on Infant Hearing 2000 position statement [3]. A combination of ear, nose, and throat examination was also realized. A work of data collection was thus made in the referent centre. These data concern various characteristics of the children: year of birth, sex, congenital hearing loss risk factors, associated handicaps and various characteristics of the family circle, especially type of communication used at home. Data were stored in a database system (Filemaker Pro 7, Filemaker Inc.). 2.2. Age at diagnosis Age at identification and degree of hearing loss were gathered in the referent centre. Age at identification of hearing loss presented in this study is the chronologic age of the infants at time of diagnostics tests. The data were stored in the database system. 2.3. Audiometric follow-up After diagnosis, an early intervention program was scheduled for all children. It includes an audiometric follow-up and a speech therapist intervention. The audiometric follow-up was realized by audiologists, specially trained for testing infants. An audiometric evaluation was realized every 3 months. The assessments included pure-tone audiometry, for air and bone-conduction thresholds, and vocal audiometry. Evaluations were repeated successively, depending on the infant’s age and abilities to be tested. The audiologists sent the exams, realized from January 2004 till June 2008, to the referent centre of Reims. The data were then stored in the database system. 2.4. Auditory intervention A conventional hearing aid fitting was proposed to some children depending on their hearing status and language acquisitions. Thus, it was initially recommended to some infants or realized secondarily during follow-up. Cochlear implantation was suggested according to the children’s conventional hearing aid benefits. An inventory work of data was realized among hearing-aid specialists of the region and at the referent centre of Reims. The data concerns type of auditory intervention proposed to the children. Age at hearing aid fitting and at cochlear implantation is also reported. All the data were stored in the database system. 2.5. Relevance of UNHS on early diagnosis and intervention of hearing aid impairment A work of data collection was made concerning age at diagnosis of a congenital bilateral hearing loss, and at hearing aid fitting, in Reims, before beginning of UNHS.
There were 54,790 births in Champagne-Ardenne from January 2004 till June 2007. Among these children, 53,930 newborns (98.4%) benefited from a first test which was negative for 53,339 (98.9%) of them. Only 591 (1.1%) children had a first positive test and were thus sent for a retest. The physician received 569 children. The retest’s result was positive for 46 of them. These 46 infants benefited from the diagnostic procedure, which conducted to the diagnosis of 41 hearing aid impairment. 3.1. Profile of cases 3.1.1. Sex, years of birth, congenital hearing loss risk factors and associated handicaps This population consists of 28 males (68.3%) and 13 females (31.7%) which represent a gender ratio of 2. Years of birth of the children are represented in Table 1. Seventeen children (41.5%) had no risk factors, and 24 children (58.5%) had at list one risk factor. Table 2 provides the distribution of risk factors among these 24 children. Family history of congenital deafness and neonatal pathologies were the two most frequent risks factors concerning each 11 and 10 children. Among the children with family history of deafness, 3 of them belong to the same consanguineous family (2 brothers and 1 cousin). The neonatal pathologies are diverse: artificial breathing, low Apgar score or preterm birth. Isolated deafness represents a majority of cases, concerning 28 children (68.3%), while 13 children (31.7%) present an associated deficit. Table 3 reports the details of the deficits. In 9 out those 13 children, the associated deficit was already identified before diagnosis of deafness. In the other 4 cases, associated pathologies were secondarily discovered: a West syndrome, an encephalopathy, a Congenital Disorders of Glycosylation (CDG syndrome) and a probable mitochondrial disease. Two children died during follow-up, the child with the mitochondrial disease (death at 3-month), and the child with an encephalopathy (death at 18-month). 3.1.2. Type of communication used at home Ninety-five percents of parents make use of an oral communication at home. Only 2 families make use of a gestural communication with a little of oral communication. In those 2 families, parents are carriers of a congenital hearing loss diagnosed during early childhood. Table 1 Years of birth. Year
N
2004 2005 2006 January 2007 to June 2007
10 12 13 6
Total
41
1166
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
Table 2 Distribution of risk factors.
Table 4 Age at diagnosis after UNHS. Age
N
%
<6 month 6–11 month
35 5
Total
40
87.5 12.5 100
Table 5 Degree of hearing loss after initial diagnosis.
Table 3 Details of deficit.
Degree
N
Mild Moderate Severe Profound
5 16 10 9
%
Total
40
12.5 40 25 22.5 100
Normalization of audition was noticed for 5 children. Among them, initial hearing impairment was explained by serous otitis media for 4 children, reversible after trans-tympanic tubes insertion and follow-up. The last case concerns a child with auditory neuropathy, presenting with large variation of his hearing thresholds. Thus, among 36 children followed regularly, we found 7 profound, 6 severe, 15 moderate and 3 mild deafness. Five children normalized their audition. 3.4. Auditory intervention
3.2. Age at diagnosis Among the 41 children of this study, diagnostic procedure took place in the referent centre of Reims for 40 of them. A child moved out of region right after the retest and thus benefited of a diagnosis in his home region, Brittany, at 10-month. Thirty-five children (85.4%) benefited of a diagnosis before age of 6 months (at an average age of 2 months 1/2). For 5 other children, the diagnostic was made between 6 and 11 months. Table 4 reports age at diagnosis after UNHS. The average age at diagnosis of a bilateral hearing impairment in Champagne-Ardenne is thus 3.2 months since beginning of UNHS. Diagnosis delays after 6 months are explained by social problems in 2 cases (no answers to convocations and not respected appointments), prolonged hospitalizations for 2 children and family fear of general anesthesia for the last one. 3.3. Audiometric follow-up Degrees of hearing loss after initial diagnosis are provided by Table 5. They are based on the better hearing ear threshold. The audiometric follow-up was realized for 36 children. Four infants were not followed regularly, because of 2 deaths, a nonattendance at follow-up and a change of region just after diagnosis. Table 6 reports degree of hearing loss before and after follow-up. Follow-up approved the diagnosis of hearing impairment for 31 out of the 36 children. Indeed, the degree of hearing loss for 24 children was confirmed, while it was found lowered or raised for 7 children.
3.4.1. Type of auditory intervention As a child left the region before intervention, an enrollment in early intervention was made for 39 children. Hearing aid fitting was set up for 25 children (64%) while an initial follow-up, without hearing aid fitting, was set up for the other 14 children (36%). Initial hearing aid fitting was proposed to all infants with severe or profound hearing loss, and to 6 infants with moderate hearing loss. Out of these 25 children, 23 benefited from a regular followup in the region, 1 child died and the last child did not attend the follow-up. The management conducted during follow up appears in Table 7. Cochlear implantation was realized in 6 cases out of the 25 children, which concern only 16.2% of all children followed regularly in the region. Cochlear implantation was proposed to all
Table 6 Degree of hearing loss before and after follow-up. Initial diagnosis
Diagnosis after follow-up
5 mild
3 mild 1 moderate 1 death
16 moderate
1 severe 10 moderate 4 normalization 1 change of region
10 severe
1 5 3 1
profound severe moderate normalization
9 profound
6 1 1 1
profound moderate death nonattendance at follow-up
40
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170 Table 7 Final diagnosis and auditory intervention after follow-up of children with initial hearing aid fitting. Initial diagnosis
Final diagnosis and auditory intervention after follow-up
6 moderate
1 severe (pursuit of hearing aid) 3 moderate (pursuit of hearing aid) 2 normalization (stop of hearing aid)
10 severe
1 5 3 1
9 profound
6 profound (cochlear implantation for 5 infants, pursuit of hearing aid for 1 infant) 1 moderate (pursuit of hearing aid) 1 death 1 nonattendance at follow-up
25
profound (cochlear implantation) severe (pursuit of hearing aid) moderate (pursuit of hearing aid) normalization (stop of hearing aid)
25
Table 8 Final diagnosis and auditory intervention after follow-up of children without initial hearing aid fitting. Initial diagnosis
Final diagnosis and auditory intervention after follow-up
5 mild
3 mild (no hearing aid fitting) 1 moderate (no hearing aid fitting) 1 death
9 moderate
7 moderate (hearing aid fitting) 2 normalization (no hearing aid fitting)
14
14
Table 9 Age at diagnosis before UNHS. Hearing loss
Age
Mild Moderate Severe Profound
6 years 1/2 4 years 1/2 25-month 18-month
children with profound hearing loss. One family refused implantation as they are orientating toward a gestural communication. The implantations were realized at an age between 8 and 26 months for an average age of 17.15 months. The hearing aid was maintained for 14 out of the 25 children. Hearing aid was suspended in 3 cases out the 25 children: the child with auditory neuropathy and 2 children with serous otitis media. [(Fig._1)TD$IG]
1167
The early intervention began without hearing aid fitting in 14 cases out of the 39 children (35%). It concerns children with all mild hearing impairment and some with moderate hearing impairment. Table 8 shows management conducted during follow-up of these children. Hearing aid implement was decided for 7 children with moderate hearing impairment during follow-up. No hearing aid was proposed in 7 cases because of 1 death, 2 normalization of audition and 4 satisfying language developments. 3.5. Age at hearing aid fitting Twenty-five children benefited of an initial hearing aid fitting, which was set up at a median age of 5 months. Indeed, this one took place before age of 6 months for 21 children, with a median age of 3.8 months, and after the age of 6 months for 4 children. Among the 14 other children, a hearing aid fitting was proposed during follow-up for 5 of them, at an age going from 6 months to 35 months, for an average age of 18.2 months. Thus, age at hearing aid fitting varies according to the degree of hearing loss. 3.6. Relevance of UNHS on early diagnosis and intervention for hearing impairment 3.6.1. Age at diagnosis Before UNHS, the average age at diagnosis of a bilateral deafness, in the centre of Reims, was 3 years and 9-month. Diagnosis was made earlier as degree of hearing loss was important and as there were risk factors of congenital deafness. Indeed, some targeted screenings were sometimes realized. Age at diagnosis before UNHS is shown in Table 9. 3.6.2. Age at hearing aid fitting Before UNHS, only 26.5% of the children with profound hearing loss benefited of a hearing aid fitting before age of 12-month. Fig. 1 shows the distribution of age at hearing aid fitting for profound hearing loss before and after UNHS. Among children with severe hearing loss, hearing aid fitting was realized for only 10.5% of them age of 12-month. Fig. 2 shows the distribution of age at hearing aid fitting for severe hearing loss before and after UNHS. Finally, before UNHS, 30% of the children with moderate hearing loss were set up with hearing aid before age of 4 years old. Fig. 3 shows the distribution of age at hearing aid fitting before and after UNHS for children with moderate hearing losses.
Fig. 1. Age at hearing aid fitting of profound hearing losses before and after UNHS.
[(Fig._2)TD$IG]
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
[(Fig._3)TD$IG]
Fig. 2. Age at hearing aid fitting of severe hearing losses before and after UNHS.
1168
Fig. 3. Age at hearing aid fitting of moderate hearing losses before and after UNHS.
4. Discussion 4.1. Congenital hearing loss factors Among all children, 58.5% of them had at least one risk factor of congenital deafness. The risk factors most frequently encountered, family history of congenital deafness and neonatal pathologies, are expected risk factors [4,5]. Nevertheless, this percentage is still questionable. Indeed, family histories of neonatal deafness are found among close relatives for 7 children and distant relatives for 4 others. Thus, 4 family history of neonatal deafness were found only after an exhaustive examination during diagnostic procedure and not during screening procedure. So, only 50% of children present with easily identifiable risk factors at birth. It is consistent with various studies. The percentage of children with risk factors of neonatal hearing varies from 37% to 67% [6–10]. One of these teams also underlines the difficulty of knowing with certainty the existence of these risk factors, as this process requires time and is generating additional costs [6]. These observations therefore emphasize the value of universal screening, rather than a risk factor’s screening. 4.2. Relevance of UNHS program on early diagnosis and intervention Before UNHS, we report an average age at diagnosis of a neonatal bilateral deafness of 3 years and 9-month-old.
Appreciably identical results are found in the medical literature. Age at diagnosis was 3 years old in United States, according to the National Institute of Health [8]. In United Kingdom, according to Davis et al. [4], before UNHS, half of neonatal deafness was diagnosed before age of 18 months and another quarter before age of 42 months. These results get closer to the one of the European investigation of 1979 [11] which stated only 50% of children seeing confirming their neonatal deafness before age of 3 years old. With an average age of 3.2 months at diagnosis, UNHS has brought a significant effect on the precocity of diagnosis of neonatal hearing loss in Champagne-Ardenne. This impact is found by many teams in United States with an age at diagnosis before 6month of age [6,7,8,12]. Same experiences are described in Europe, with an average age at time of diagnosis, after screening, of 2 months [9,13,14]. Naturally, as age at diagnosis is now 3 months 1/2, age at intervention considerably decreased since the beginning of UNHS. Indeed, for children with profound and severe hearing losses, hearing aid fitting was proposed and realized at a mean age of 5month. Whereas before starting up of UNHS, only 26.5% of the children with profound hearing losses and 10.5% of the children with severe hearing losses benefited from hearing aid fitting before 12month of age. Contrary to these children to whom was proposed an initial hearing aid fitting, the management proposed to the children with a moderate hearing loss was not homogeneous. So, the average age in hearing aid impairment is about 9.9-month. Before UNHS, 70%
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
of the children with a moderate hearing loss were set up with a hearing aid after age of 4 years old. Besides early age at diagnosis, early age at hearing aid fitting is also explained by the short delay diagnosis and intervention. Indeed, the period between diagnosis and hearing aid fitting is of 1.8 months for a profound hearing loss whereas it is of 2.3 months for a severe one. We explain this short delay by the fact that the team announcing the diagnosis is also the one who insures the early intervention. Interest of UNHS for an early intervention is found by other teams. Indeed, age at the beginning of the intervention varies from 4 to 7 months 1/2 [6,7,9,10,12,14,15]. The average age reported is of 5.7 months in Rhode Island [12], of 5.8 months in Hawaii [7] and of 7 months 1/2 in the state of New York [6]. Teams in United Kingdom report ages of 4 months [9,14]. Our experience underlines these results and provides evidence for UNHS to carry an early diagnosis and intervention for congenital hearing loss. 4.3. Age at diagnosis Since the beginning of UNHS in Champagne-Ardenne, the average age at diagnosis of a neonatal hearing impairment is 3 months 1/2. Indeed, the diagnostic’s announcement takes place before age of 6 months for 87.5% of children. The precocity of this diagnosis does not hold any more on the degree of hearing loss. In our experience, reasons of diagnosis delays are essentially due to social problems and to prolonged hospitalizations for children. This early age at diagnosis can be discussed. First it does not represent age at final diagnosis as regular audiometric follow-up shows variability in hearing threshold for some children. Diverse directives, and publications, insist on the importance to carry the most premature diagnosis as possible, before age of 3 months to allow an early intervention before age of 6 months [16,17,18]. The justifications are found in the profits of an early intervention for the language development of these children. It is for that reason, as well as to bring answers to the parents, that we chose to realize the most premature diagnostic announcement. As a result, even if early intervention is initiated, parents and professional are aware of possible auditory status change. Secondly, this early age at diagnosis does not take into account possible diagnoses of other deafness realized in early childhood. During this period of study, late diagnoses of hearing impairment were made among 6 young children with negative screening at birth. Two cases are explained by an incomplete screening procedure. Two others children were diagnosed at 18 months with moderate and profound hearing loss with obvious change in auditory comportment. These 2 cases are thus strongly suspect of congenital deafness of progressive appearance which is described in the medical literature [19]. Finally, 2 cases of deafness were diagnosed as a consequence of meningitis at 14 months and 2 years 1/2 old. Late diagnoses are reported by other teams practicing UNHS [10,12,14,20,21]. Fortnum et al. [22] give several explanations for these late diagnoses: incomplete procedure of screening or erroneous result, congenital hearing loss of progressive appearance and acquired deafness. This may explain variations of prevalence of hearing loss which, still according to Fortnum and al, could be 1.06 children per 1000 at birth and reach 1.65 children per 1000, and even 2.05 children per 1000 at age of 9 years old. The percentages of late diagnosis, without taking account of acquired deafness, vary from 6% to 25% [10,12,14,21]. In consequence, complete information is given to the parents, at time of screening, through a brochure. It especially reminds them to be attentive to hearing and language development of their child, even with a negative screening test at birth.
1169
4.4. Reliability of early diagnosis The modalities of hearing impairment diagnosis are little described in the literature but request ABR, associated with other audiometric evaluations. The data obtained with ABR, collected and analysed by an ENT, coupled with behavioural auditory tests, bring us sufficient arguments for diagnosis. Indeed, the audiometric follow-up of the children allowed confirming initial diagnosis for the majority of children, even if degree of hearing loss may vary in a few case. All children with initial diagnosis of profound and severe hearing impairment benefited of a confirmation of their hearing loss diagnosis after audiometric follow-up. The diagnosis is more difficult in case of mild and moderate hearing loss due to serous otitis media. These temporary conductive hearing losses are found frequently within our children’s troop. Final diagnosis is more lately established in this population. The analysis of the wave I, III and V latencies during diagnostic ABR is crucial. Indeed, a lengthening of wave’s latencies is found among all children with conductive hearing loss. Serous otitis media, sometimes as only cause of deafness, is reported by other teams [15,23]. Yoshinaga-Itano [24] find 10 serous otitis media related hearing impairment among 70 children with neonatal deafness. Nevertheless, our experience attests the fact that an early diagnosis of congenital deafness, after UNHS, is possible at a very young age. 4.5. Auditory intervention All carriers of profound and severe hearing losses benefited from an initial hearing aid fitting. A cochlear implantation was proposed to all children with a profound hearing loss and accepted by all but 1 family. No child with severe hearing loss benefited from a cochlear implantation. Thus, for both children with profound or severe hearing losses, the auditory management seems codified. The auditory intervention for children with moderate hearing loss is more heterogeneous. Hearing aid fitting was implemented at different time during follow-up. This can be explained by multiples factors. First, definitive hearing status cannot be achieved in presence of serous otitis media which requires specific treatments. Secondly, the interest of premature hearing aid fitting in moderate hearing loss has not been demonstrated. Furthermore, some parents are not convinced of the relevance of hearing aid fitting for those children. Thus, the referent center’s team progressively changed his management of moderate hearing impairment. While at the beginning of UNHS, every children with moderate hearing loss were proposed hearing aid fitting, the current attitude is to start with an initial period of follow-up to estimate at best the audiometric profile of the children. These difficulties in auditory management of children with moderate hearing losses are underlined by teams quoted previously [6,9]. At last, no child carrier of a mild deafness benefited of hearing aid equipment. The follow-up is even more difficult as parent’s hesitations, already found in follow-up of the children with moderate hearing losses, are sharply more important for these children. 5. Conclusion Our UNHS program demonstrates its validity for early diagnosis and intervention for congenital hearing loss. Early diagnosis is reliable in most cases and possible before 6 months old. This one is in return more difficult to be so precocious for children with moderate hearing loss, owing to serous otitis media, or children with incomplete cerebral maturation. Teams practicing UNHS
1170
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
must be aware of this as for the risk of diagnosis of neonatal deafness during early childhood, even with a negative screening test at birth. Complete information must be given to parents in that purpose. The intervention may be precocious as diagnosis is realized early and a short delay separates diagnosis from early intervention. This one seems codified for children with severe or profound hearing losses, with a hearing aid fitting realized as soon as possible, eventually followed by a cochlear implantation. On the other hand, the auditory management may be more difficult for children with moderate hearing losses, to whom time at hearing aid fitting is not clearly defined. Even with these difficulties, UNHS brought a considerable impact on the management of the congenital deafness in Champagne-Ardenne. A more generalized practice of UNHS in France is now desirable. References [1] H.M. Fortnum, Epidemiology of permanent childhood hearing impairment: implications for neonatal hearing screening, Audiol. Med. 1 (3) (2003) 155–164. [2] M. Le´veˆque, P. Schmidt, B. Leroux, J.B. Danvin, T. Langagne, M. Labrousse, et al., Universal newborn hearing screening: a 27-month experience in the French region of Champagne-Ardenne, Acta Paediatr. 96 (August (8)) (2007) 1150–1154. [3] Joint Committee on Infant Hearing, American Academy of Audiology, American Speech-Language-Hearing Association, Directors of Speech and Hearing Program in State Health and Welfare Agencies. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. 2000, 106 (4) 798817. [4] A. Davis, J. Bamford, I. Wilson, T. Ramkalawan, M. Forshaw, S. Wright, A critical review of the role of neonatal hearing screening in the detection of congenital hearing impairment, Health Technol. Assess. 1 (10) (1997), i–iv, 1–176. [5] H. Fortnum, A. Davis, Epidemiology of permanent childhood hearing impairment in Trent Region, 1985–1993, Br. J. Audiol. 31 (6) (1997) 409–446. [6] L. Dalzell, M. Orlando, M. Macdonald, A. Berg, M. Bradley, A. Cacace, et al., The New York State universal newborn hearing screening demonstration project: ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention, Ear Hear. 21 (2) (2000) 118–130. [7] J.A. Mason, K.R. Herrmann, Universal infant hearing screening by automated auditory brainstem response measurement, Pediatrics 101 (2) (1988) 221–228.
[8] A. Mehl, V. Thomson, The Colorado newborn hearing screening project 1992– 1999: on threshold on effective population-based universal newborn hearing screening, Pediatrics 109 (2002) 1–8. [9] K. Uus, J. Bamford, Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases, Pediatrics 117 (5) (2006) e887–e893. [10] Wessex Universal Neonatal Hearing Screening Trial Group, Controlled trial of universal neonatal screening for early identification of permanent childhood hearing impairment, Lancet 352 (9145) (1998) 1957–1964. [11] J.A. Martin, O. Bentzen, J.R. Colley, D. Hennebert, C. Holm, S. Iurato, et al., Childhood deafness in the European community, Scand. Audiol. 10 (3) (1981) 165–174. [12] B.R. Vohr, L.M. Carty, P.E. Moore, K. Letourneau, The Rhode Island Hearing Assessment Program: experience with statewide hearing screening (1993– 1996), J. Pediatr. 133 (3) (1998) 353–357. [13] M. Harrison, J. Roush, J. Wallace, Trends in age of identification and intervention in infants with hearing loss, Ear Hear. (2003) 89–95. [14] P.M. Watkin, Neonatal otoacoustic emission screening and the identification of deafness, Arch. Dis. Child. Fetal Neonatal Ed. 74 (1) (1996) F16–F25. [15] J. Bamford, K. Uus, A. Davis, Screening for hearing loss in childhood: issues, evidence and current approaches in the UK, J. Med. Screen. 12 (3) (2005) 119–124. [16] F. Declau, A. Doyen, T. Robillard, S.J. De Varebeke, Universal newborn hearing screening, B-Ent (Suppl. 1) (2005) 16–21, quiz 22–3. [17] Joint committee of infant hearing, 1994 position statement, Pediatrics 100 (1995) 152–156. [18] National Institutes of Health, Early identification of hearing impairment in infants and young children, NIH Consens. Statement 11 (1) (1993) 1–25. [19] K.P. Steel, Science, medicine, and the future: new interventions in hearing impairment, BMJ 320 (7235) (2000) 622–625. [20] T. Wada, T. Kubo, T. Aiba, H. Yamane, Further examination of infants referred from newborn hearing screening, Acta Otolaryngol. Suppl. 554 (2004) 17–25. [21] V. Weichbold, D. Nekahm-Heis, K. Welzl-Mueller, Universal newborn hearing screening and postnatal hearing loss, Pediatrics 117 (4) (2006) e631– e636. [22] H.M. Fortnum, A.Q. Summerfield, D.H. Marshall, A.C. Davis, J.M. Bamford, Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study, BMJ 323 (7312) (2001) 536–540. [23] A. Young, H. Tattersall, Universal newborn hearing screening and early identification of deafness: parents’ responses to knowing early and their expectations of child communication development, J. Deaf Stud. Deaf Educ. 12 (2) (2007) 209– 220. [24] C. Yoshinaga-Itano, Levels of evidence: universal newborn hearing screening (UNHS) and early hearing detection and intervention systems (EHDI), J. Commun. Disord. 37 (September–October (5)) (2004) 451–465.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Universal newborn hearing screening in the Champagne-Ardenne region: A 4-year follow-up after early diagnosis of hearing impairment T. Langagne *, M. Le´veˆque, P. Schmidt, A. Chays Department of Otolaryngology and ENT Surgery, Robert Debre´ University Hospital, 125 avenue du Ge´ne´ral Koenig, 51100 Reims, France
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 May 2010 Received in revised form 27 June 2010 Accepted 3 July 2010 Available online 31 July 2010
Introduction: Permanent congenital hearing loss is one of the most frequent congenital anomaly at birth. Universal newborn hearing screening (UNHS) was introduced in numerous countries in order to allow an early diagnosis and intervention for congenital hearing impairment. Objective: First aim of this study is to evaluate the accuracy of early diagnosis of hearing impairment after UNHS. Second aim is to discuss the auditory intervention proposed after this diagnosis. Last aim is to evaluate the relevance of UNHS for early diagnosis and intervention. Materials and methods: Prospective study. UNHS program was introduced in the entire French region of Champagne-Ardenne in January 2004. Forty-one children have benefited of an early diagnosis of hearing impairment until June 2007. They were included in an intervention program consisting of an audiometric follow-up and an auditory intervention. This program was conducted until June 2008. Results: There were 28 males patients and 13 females patients. The diagnosis of hearing aid impairment was carried at an average age of 3.2-month. The auditory follow-up allowed confirming the initial diagnosis of deafness for the majority of the children as for their degree of hearing loss. Auditory intervention was heterogeneous depending on degree of hearing loss of the children. Conclusion: This UNHS program demonstrates its validity and feasibility for early diagnosis and intervention of congenital hearing impairment. It brought a major impact on the management of congenital hearing impairment in Champagne-Ardenne. ß 2010 Elsevier Ireland Ltd. All rights reserved.
Keywords: Universal hearing screening Congenital deafness Diagnosis Hearing aid fitting
1. Introduction Permanent congenital hearing loss is one of the most common congenital anomaly. Bilateral, medium to profound hearing impairment is estimated to occur in 1 infant per 1000 [1]. The impact of such hearing loss on speech and language acquisition, academic achievement and social development is well known. The goal of universal newborn hearing screening (UNHS) is thus to allow an early diagnosis of congenital deafness and an early intervention for these children. In Champagne-Ardenne, UNHS has been effective from January 2004; its aim is to identify all children with permanent bilateral hearing impairment more than 35 dB. UNHS was applied to 54790 infants from January 2004 to June 2007. Forty-one children were diagnosed with hearing impairment. In this work, we describe the epidemiological data of this group, their audiologic status and the modality of auditory intervention. First aims of this study are to evaluate the accuracy of early diagnosis and auditory intervention. Second purpose is to evaluate
* Corresponding author. Tel.: +33 326787125; fax: +33 326788982. E-mail address: [email protected] (T. Langagne). 0165-5876/$ – see front matter ß 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2010.07.005
the relevance of UNHS for both early diagnosis and intervention for hearing aid impairment. 2. Methods UNHS has already been described [2]. The method consists of a two-stage screening. Every child being born in ChampagneArdenne benefits of a first test in the third day of life before hospital discharge. The first test can either be automated Transient Evoked Otoacoustic Emission (TEOAE) in maternities or automatic Auditory Brainstem Response (aABR) in neonatal intensive care unit. The procedure is considered a positive screening test in case of bilateral absence of TEOAE/aABR. In that case, a second test (retest) is performed by a physician 15 days after hospital’s discharge. When both ears fail to pass the retest, TEOAE or aABR, the baby is referred to the regional referent centre, based in Reims, for diagnostic tests. The diagnostic tests include behavioural tests and a clickevoked ABR. ABR is realized under general anaesthesia, or light sedation. Indeed, the child has to remain calm to avoid interferences. Analysed ABR parameters are essentially the auditory thresholds and the latencies of wave I, III and V. The tests’ results are exposed in presence of various actors of the
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
intervention: otologist, speech therapist, psychologist and hearing-aid specialist. It is a long discussion during which the diagnosis of deafness, the project of intervention and perspectives of evolution for the child are presented. From January 2004 to June 2007, 41 children were diagnosed with a hearing loss; they were all enrolled in a prospected study conducted until June, 2008.
1165
The data presented in this study are the one of a hearing-aid specialist in Champagne-Ardenne, dealing with the great majority of children with congenital hearing loss. These data concern 221 children, born between 1987 and 2003. All the data were stored in the database system.
3. Results 2.1. Profile of cases A medical evaluation was realized for every child in the referent centre. It includes a detailed pedigree analysis for congenital hearing loss, medical history, associated handicaps and research for risk factors, as identified by the guidelines of the Joint Committee on Infant Hearing 2000 position statement [3]. A combination of ear, nose, and throat examination was also realized. A work of data collection was thus made in the referent centre. These data concern various characteristics of the children: year of birth, sex, congenital hearing loss risk factors, associated handicaps and various characteristics of the family circle, especially type of communication used at home. Data were stored in a database system (Filemaker Pro 7, Filemaker Inc.). 2.2. Age at diagnosis Age at identification and degree of hearing loss were gathered in the referent centre. Age at identification of hearing loss presented in this study is the chronologic age of the infants at time of diagnostics tests. The data were stored in the database system. 2.3. Audiometric follow-up After diagnosis, an early intervention program was scheduled for all children. It includes an audiometric follow-up and a speech therapist intervention. The audiometric follow-up was realized by audiologists, specially trained for testing infants. An audiometric evaluation was realized every 3 months. The assessments included pure-tone audiometry, for air and bone-conduction thresholds, and vocal audiometry. Evaluations were repeated successively, depending on the infant’s age and abilities to be tested. The audiologists sent the exams, realized from January 2004 till June 2008, to the referent centre of Reims. The data were then stored in the database system. 2.4. Auditory intervention A conventional hearing aid fitting was proposed to some children depending on their hearing status and language acquisitions. Thus, it was initially recommended to some infants or realized secondarily during follow-up. Cochlear implantation was suggested according to the children’s conventional hearing aid benefits. An inventory work of data was realized among hearing-aid specialists of the region and at the referent centre of Reims. The data concerns type of auditory intervention proposed to the children. Age at hearing aid fitting and at cochlear implantation is also reported. All the data were stored in the database system. 2.5. Relevance of UNHS on early diagnosis and intervention of hearing aid impairment A work of data collection was made concerning age at diagnosis of a congenital bilateral hearing loss, and at hearing aid fitting, in Reims, before beginning of UNHS.
There were 54,790 births in Champagne-Ardenne from January 2004 till June 2007. Among these children, 53,930 newborns (98.4%) benefited from a first test which was negative for 53,339 (98.9%) of them. Only 591 (1.1%) children had a first positive test and were thus sent for a retest. The physician received 569 children. The retest’s result was positive for 46 of them. These 46 infants benefited from the diagnostic procedure, which conducted to the diagnosis of 41 hearing aid impairment. 3.1. Profile of cases 3.1.1. Sex, years of birth, congenital hearing loss risk factors and associated handicaps This population consists of 28 males (68.3%) and 13 females (31.7%) which represent a gender ratio of 2. Years of birth of the children are represented in Table 1. Seventeen children (41.5%) had no risk factors, and 24 children (58.5%) had at list one risk factor. Table 2 provides the distribution of risk factors among these 24 children. Family history of congenital deafness and neonatal pathologies were the two most frequent risks factors concerning each 11 and 10 children. Among the children with family history of deafness, 3 of them belong to the same consanguineous family (2 brothers and 1 cousin). The neonatal pathologies are diverse: artificial breathing, low Apgar score or preterm birth. Isolated deafness represents a majority of cases, concerning 28 children (68.3%), while 13 children (31.7%) present an associated deficit. Table 3 reports the details of the deficits. In 9 out those 13 children, the associated deficit was already identified before diagnosis of deafness. In the other 4 cases, associated pathologies were secondarily discovered: a West syndrome, an encephalopathy, a Congenital Disorders of Glycosylation (CDG syndrome) and a probable mitochondrial disease. Two children died during follow-up, the child with the mitochondrial disease (death at 3-month), and the child with an encephalopathy (death at 18-month). 3.1.2. Type of communication used at home Ninety-five percents of parents make use of an oral communication at home. Only 2 families make use of a gestural communication with a little of oral communication. In those 2 families, parents are carriers of a congenital hearing loss diagnosed during early childhood. Table 1 Years of birth. Year
N
2004 2005 2006 January 2007 to June 2007
10 12 13 6
Total
41
1166
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
Table 2 Distribution of risk factors.
Table 4 Age at diagnosis after UNHS. Age
N
%
<6 month 6–11 month
35 5
Total
40
87.5 12.5 100
Table 5 Degree of hearing loss after initial diagnosis.
Table 3 Details of deficit.
Degree
N
Mild Moderate Severe Profound
5 16 10 9
%
Total
40
12.5 40 25 22.5 100
Normalization of audition was noticed for 5 children. Among them, initial hearing impairment was explained by serous otitis media for 4 children, reversible after trans-tympanic tubes insertion and follow-up. The last case concerns a child with auditory neuropathy, presenting with large variation of his hearing thresholds. Thus, among 36 children followed regularly, we found 7 profound, 6 severe, 15 moderate and 3 mild deafness. Five children normalized their audition. 3.4. Auditory intervention
3.2. Age at diagnosis Among the 41 children of this study, diagnostic procedure took place in the referent centre of Reims for 40 of them. A child moved out of region right after the retest and thus benefited of a diagnosis in his home region, Brittany, at 10-month. Thirty-five children (85.4%) benefited of a diagnosis before age of 6 months (at an average age of 2 months 1/2). For 5 other children, the diagnostic was made between 6 and 11 months. Table 4 reports age at diagnosis after UNHS. The average age at diagnosis of a bilateral hearing impairment in Champagne-Ardenne is thus 3.2 months since beginning of UNHS. Diagnosis delays after 6 months are explained by social problems in 2 cases (no answers to convocations and not respected appointments), prolonged hospitalizations for 2 children and family fear of general anesthesia for the last one. 3.3. Audiometric follow-up Degrees of hearing loss after initial diagnosis are provided by Table 5. They are based on the better hearing ear threshold. The audiometric follow-up was realized for 36 children. Four infants were not followed regularly, because of 2 deaths, a nonattendance at follow-up and a change of region just after diagnosis. Table 6 reports degree of hearing loss before and after follow-up. Follow-up approved the diagnosis of hearing impairment for 31 out of the 36 children. Indeed, the degree of hearing loss for 24 children was confirmed, while it was found lowered or raised for 7 children.
3.4.1. Type of auditory intervention As a child left the region before intervention, an enrollment in early intervention was made for 39 children. Hearing aid fitting was set up for 25 children (64%) while an initial follow-up, without hearing aid fitting, was set up for the other 14 children (36%). Initial hearing aid fitting was proposed to all infants with severe or profound hearing loss, and to 6 infants with moderate hearing loss. Out of these 25 children, 23 benefited from a regular followup in the region, 1 child died and the last child did not attend the follow-up. The management conducted during follow up appears in Table 7. Cochlear implantation was realized in 6 cases out of the 25 children, which concern only 16.2% of all children followed regularly in the region. Cochlear implantation was proposed to all
Table 6 Degree of hearing loss before and after follow-up. Initial diagnosis
Diagnosis after follow-up
5 mild
3 mild 1 moderate 1 death
16 moderate
1 severe 10 moderate 4 normalization 1 change of region
10 severe
1 5 3 1
profound severe moderate normalization
9 profound
6 1 1 1
profound moderate death nonattendance at follow-up
40
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170 Table 7 Final diagnosis and auditory intervention after follow-up of children with initial hearing aid fitting. Initial diagnosis
Final diagnosis and auditory intervention after follow-up
6 moderate
1 severe (pursuit of hearing aid) 3 moderate (pursuit of hearing aid) 2 normalization (stop of hearing aid)
10 severe
1 5 3 1
9 profound
6 profound (cochlear implantation for 5 infants, pursuit of hearing aid for 1 infant) 1 moderate (pursuit of hearing aid) 1 death 1 nonattendance at follow-up
25
profound (cochlear implantation) severe (pursuit of hearing aid) moderate (pursuit of hearing aid) normalization (stop of hearing aid)
25
Table 8 Final diagnosis and auditory intervention after follow-up of children without initial hearing aid fitting. Initial diagnosis
Final diagnosis and auditory intervention after follow-up
5 mild
3 mild (no hearing aid fitting) 1 moderate (no hearing aid fitting) 1 death
9 moderate
7 moderate (hearing aid fitting) 2 normalization (no hearing aid fitting)
14
14
Table 9 Age at diagnosis before UNHS. Hearing loss
Age
Mild Moderate Severe Profound
6 years 1/2 4 years 1/2 25-month 18-month
children with profound hearing loss. One family refused implantation as they are orientating toward a gestural communication. The implantations were realized at an age between 8 and 26 months for an average age of 17.15 months. The hearing aid was maintained for 14 out of the 25 children. Hearing aid was suspended in 3 cases out the 25 children: the child with auditory neuropathy and 2 children with serous otitis media. [(Fig._1)TD$IG]
1167
The early intervention began without hearing aid fitting in 14 cases out of the 39 children (35%). It concerns children with all mild hearing impairment and some with moderate hearing impairment. Table 8 shows management conducted during follow-up of these children. Hearing aid implement was decided for 7 children with moderate hearing impairment during follow-up. No hearing aid was proposed in 7 cases because of 1 death, 2 normalization of audition and 4 satisfying language developments. 3.5. Age at hearing aid fitting Twenty-five children benefited of an initial hearing aid fitting, which was set up at a median age of 5 months. Indeed, this one took place before age of 6 months for 21 children, with a median age of 3.8 months, and after the age of 6 months for 4 children. Among the 14 other children, a hearing aid fitting was proposed during follow-up for 5 of them, at an age going from 6 months to 35 months, for an average age of 18.2 months. Thus, age at hearing aid fitting varies according to the degree of hearing loss. 3.6. Relevance of UNHS on early diagnosis and intervention for hearing impairment 3.6.1. Age at diagnosis Before UNHS, the average age at diagnosis of a bilateral deafness, in the centre of Reims, was 3 years and 9-month. Diagnosis was made earlier as degree of hearing loss was important and as there were risk factors of congenital deafness. Indeed, some targeted screenings were sometimes realized. Age at diagnosis before UNHS is shown in Table 9. 3.6.2. Age at hearing aid fitting Before UNHS, only 26.5% of the children with profound hearing loss benefited of a hearing aid fitting before age of 12-month. Fig. 1 shows the distribution of age at hearing aid fitting for profound hearing loss before and after UNHS. Among children with severe hearing loss, hearing aid fitting was realized for only 10.5% of them age of 12-month. Fig. 2 shows the distribution of age at hearing aid fitting for severe hearing loss before and after UNHS. Finally, before UNHS, 30% of the children with moderate hearing loss were set up with hearing aid before age of 4 years old. Fig. 3 shows the distribution of age at hearing aid fitting before and after UNHS for children with moderate hearing losses.
Fig. 1. Age at hearing aid fitting of profound hearing losses before and after UNHS.
[(Fig._2)TD$IG]
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
[(Fig._3)TD$IG]
Fig. 2. Age at hearing aid fitting of severe hearing losses before and after UNHS.
1168
Fig. 3. Age at hearing aid fitting of moderate hearing losses before and after UNHS.
4. Discussion 4.1. Congenital hearing loss factors Among all children, 58.5% of them had at least one risk factor of congenital deafness. The risk factors most frequently encountered, family history of congenital deafness and neonatal pathologies, are expected risk factors [4,5]. Nevertheless, this percentage is still questionable. Indeed, family histories of neonatal deafness are found among close relatives for 7 children and distant relatives for 4 others. Thus, 4 family history of neonatal deafness were found only after an exhaustive examination during diagnostic procedure and not during screening procedure. So, only 50% of children present with easily identifiable risk factors at birth. It is consistent with various studies. The percentage of children with risk factors of neonatal hearing varies from 37% to 67% [6–10]. One of these teams also underlines the difficulty of knowing with certainty the existence of these risk factors, as this process requires time and is generating additional costs [6]. These observations therefore emphasize the value of universal screening, rather than a risk factor’s screening. 4.2. Relevance of UNHS program on early diagnosis and intervention Before UNHS, we report an average age at diagnosis of a neonatal bilateral deafness of 3 years and 9-month-old.
Appreciably identical results are found in the medical literature. Age at diagnosis was 3 years old in United States, according to the National Institute of Health [8]. In United Kingdom, according to Davis et al. [4], before UNHS, half of neonatal deafness was diagnosed before age of 18 months and another quarter before age of 42 months. These results get closer to the one of the European investigation of 1979 [11] which stated only 50% of children seeing confirming their neonatal deafness before age of 3 years old. With an average age of 3.2 months at diagnosis, UNHS has brought a significant effect on the precocity of diagnosis of neonatal hearing loss in Champagne-Ardenne. This impact is found by many teams in United States with an age at diagnosis before 6month of age [6,7,8,12]. Same experiences are described in Europe, with an average age at time of diagnosis, after screening, of 2 months [9,13,14]. Naturally, as age at diagnosis is now 3 months 1/2, age at intervention considerably decreased since the beginning of UNHS. Indeed, for children with profound and severe hearing losses, hearing aid fitting was proposed and realized at a mean age of 5month. Whereas before starting up of UNHS, only 26.5% of the children with profound hearing losses and 10.5% of the children with severe hearing losses benefited from hearing aid fitting before 12month of age. Contrary to these children to whom was proposed an initial hearing aid fitting, the management proposed to the children with a moderate hearing loss was not homogeneous. So, the average age in hearing aid impairment is about 9.9-month. Before UNHS, 70%
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
of the children with a moderate hearing loss were set up with a hearing aid after age of 4 years old. Besides early age at diagnosis, early age at hearing aid fitting is also explained by the short delay diagnosis and intervention. Indeed, the period between diagnosis and hearing aid fitting is of 1.8 months for a profound hearing loss whereas it is of 2.3 months for a severe one. We explain this short delay by the fact that the team announcing the diagnosis is also the one who insures the early intervention. Interest of UNHS for an early intervention is found by other teams. Indeed, age at the beginning of the intervention varies from 4 to 7 months 1/2 [6,7,9,10,12,14,15]. The average age reported is of 5.7 months in Rhode Island [12], of 5.8 months in Hawaii [7] and of 7 months 1/2 in the state of New York [6]. Teams in United Kingdom report ages of 4 months [9,14]. Our experience underlines these results and provides evidence for UNHS to carry an early diagnosis and intervention for congenital hearing loss. 4.3. Age at diagnosis Since the beginning of UNHS in Champagne-Ardenne, the average age at diagnosis of a neonatal hearing impairment is 3 months 1/2. Indeed, the diagnostic’s announcement takes place before age of 6 months for 87.5% of children. The precocity of this diagnosis does not hold any more on the degree of hearing loss. In our experience, reasons of diagnosis delays are essentially due to social problems and to prolonged hospitalizations for children. This early age at diagnosis can be discussed. First it does not represent age at final diagnosis as regular audiometric follow-up shows variability in hearing threshold for some children. Diverse directives, and publications, insist on the importance to carry the most premature diagnosis as possible, before age of 3 months to allow an early intervention before age of 6 months [16,17,18]. The justifications are found in the profits of an early intervention for the language development of these children. It is for that reason, as well as to bring answers to the parents, that we chose to realize the most premature diagnostic announcement. As a result, even if early intervention is initiated, parents and professional are aware of possible auditory status change. Secondly, this early age at diagnosis does not take into account possible diagnoses of other deafness realized in early childhood. During this period of study, late diagnoses of hearing impairment were made among 6 young children with negative screening at birth. Two cases are explained by an incomplete screening procedure. Two others children were diagnosed at 18 months with moderate and profound hearing loss with obvious change in auditory comportment. These 2 cases are thus strongly suspect of congenital deafness of progressive appearance which is described in the medical literature [19]. Finally, 2 cases of deafness were diagnosed as a consequence of meningitis at 14 months and 2 years 1/2 old. Late diagnoses are reported by other teams practicing UNHS [10,12,14,20,21]. Fortnum et al. [22] give several explanations for these late diagnoses: incomplete procedure of screening or erroneous result, congenital hearing loss of progressive appearance and acquired deafness. This may explain variations of prevalence of hearing loss which, still according to Fortnum and al, could be 1.06 children per 1000 at birth and reach 1.65 children per 1000, and even 2.05 children per 1000 at age of 9 years old. The percentages of late diagnosis, without taking account of acquired deafness, vary from 6% to 25% [10,12,14,21]. In consequence, complete information is given to the parents, at time of screening, through a brochure. It especially reminds them to be attentive to hearing and language development of their child, even with a negative screening test at birth.
1169
4.4. Reliability of early diagnosis The modalities of hearing impairment diagnosis are little described in the literature but request ABR, associated with other audiometric evaluations. The data obtained with ABR, collected and analysed by an ENT, coupled with behavioural auditory tests, bring us sufficient arguments for diagnosis. Indeed, the audiometric follow-up of the children allowed confirming initial diagnosis for the majority of children, even if degree of hearing loss may vary in a few case. All children with initial diagnosis of profound and severe hearing impairment benefited of a confirmation of their hearing loss diagnosis after audiometric follow-up. The diagnosis is more difficult in case of mild and moderate hearing loss due to serous otitis media. These temporary conductive hearing losses are found frequently within our children’s troop. Final diagnosis is more lately established in this population. The analysis of the wave I, III and V latencies during diagnostic ABR is crucial. Indeed, a lengthening of wave’s latencies is found among all children with conductive hearing loss. Serous otitis media, sometimes as only cause of deafness, is reported by other teams [15,23]. Yoshinaga-Itano [24] find 10 serous otitis media related hearing impairment among 70 children with neonatal deafness. Nevertheless, our experience attests the fact that an early diagnosis of congenital deafness, after UNHS, is possible at a very young age. 4.5. Auditory intervention All carriers of profound and severe hearing losses benefited from an initial hearing aid fitting. A cochlear implantation was proposed to all children with a profound hearing loss and accepted by all but 1 family. No child with severe hearing loss benefited from a cochlear implantation. Thus, for both children with profound or severe hearing losses, the auditory management seems codified. The auditory intervention for children with moderate hearing loss is more heterogeneous. Hearing aid fitting was implemented at different time during follow-up. This can be explained by multiples factors. First, definitive hearing status cannot be achieved in presence of serous otitis media which requires specific treatments. Secondly, the interest of premature hearing aid fitting in moderate hearing loss has not been demonstrated. Furthermore, some parents are not convinced of the relevance of hearing aid fitting for those children. Thus, the referent center’s team progressively changed his management of moderate hearing impairment. While at the beginning of UNHS, every children with moderate hearing loss were proposed hearing aid fitting, the current attitude is to start with an initial period of follow-up to estimate at best the audiometric profile of the children. These difficulties in auditory management of children with moderate hearing losses are underlined by teams quoted previously [6,9]. At last, no child carrier of a mild deafness benefited of hearing aid equipment. The follow-up is even more difficult as parent’s hesitations, already found in follow-up of the children with moderate hearing losses, are sharply more important for these children. 5. Conclusion Our UNHS program demonstrates its validity for early diagnosis and intervention for congenital hearing loss. Early diagnosis is reliable in most cases and possible before 6 months old. This one is in return more difficult to be so precocious for children with moderate hearing loss, owing to serous otitis media, or children with incomplete cerebral maturation. Teams practicing UNHS
1170
T. Langagne et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 1164–1170
must be aware of this as for the risk of diagnosis of neonatal deafness during early childhood, even with a negative screening test at birth. Complete information must be given to parents in that purpose. The intervention may be precocious as diagnosis is realized early and a short delay separates diagnosis from early intervention. This one seems codified for children with severe or profound hearing losses, with a hearing aid fitting realized as soon as possible, eventually followed by a cochlear implantation. On the other hand, the auditory management may be more difficult for children with moderate hearing losses, to whom time at hearing aid fitting is not clearly defined. Even with these difficulties, UNHS brought a considerable impact on the management of the congenital deafness in Champagne-Ardenne. A more generalized practice of UNHS in France is now desirable. References [1] H.M. Fortnum, Epidemiology of permanent childhood hearing impairment: implications for neonatal hearing screening, Audiol. Med. 1 (3) (2003) 155–164. [2] M. Le´veˆque, P. Schmidt, B. Leroux, J.B. Danvin, T. Langagne, M. Labrousse, et al., Universal newborn hearing screening: a 27-month experience in the French region of Champagne-Ardenne, Acta Paediatr. 96 (August (8)) (2007) 1150–1154. [3] Joint Committee on Infant Hearing, American Academy of Audiology, American Speech-Language-Hearing Association, Directors of Speech and Hearing Program in State Health and Welfare Agencies. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. 2000, 106 (4) 798817. [4] A. Davis, J. Bamford, I. Wilson, T. Ramkalawan, M. Forshaw, S. Wright, A critical review of the role of neonatal hearing screening in the detection of congenital hearing impairment, Health Technol. Assess. 1 (10) (1997), i–iv, 1–176. [5] H. Fortnum, A. Davis, Epidemiology of permanent childhood hearing impairment in Trent Region, 1985–1993, Br. J. Audiol. 31 (6) (1997) 409–446. [6] L. Dalzell, M. Orlando, M. Macdonald, A. Berg, M. Bradley, A. Cacace, et al., The New York State universal newborn hearing screening demonstration project: ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention, Ear Hear. 21 (2) (2000) 118–130. [7] J.A. Mason, K.R. Herrmann, Universal infant hearing screening by automated auditory brainstem response measurement, Pediatrics 101 (2) (1988) 221–228.
[8] A. Mehl, V. Thomson, The Colorado newborn hearing screening project 1992– 1999: on threshold on effective population-based universal newborn hearing screening, Pediatrics 109 (2002) 1–8. [9] K. Uus, J. Bamford, Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases, Pediatrics 117 (5) (2006) e887–e893. [10] Wessex Universal Neonatal Hearing Screening Trial Group, Controlled trial of universal neonatal screening for early identification of permanent childhood hearing impairment, Lancet 352 (9145) (1998) 1957–1964. [11] J.A. Martin, O. Bentzen, J.R. Colley, D. Hennebert, C. Holm, S. Iurato, et al., Childhood deafness in the European community, Scand. Audiol. 10 (3) (1981) 165–174. [12] B.R. Vohr, L.M. Carty, P.E. Moore, K. Letourneau, The Rhode Island Hearing Assessment Program: experience with statewide hearing screening (1993– 1996), J. Pediatr. 133 (3) (1998) 353–357. [13] M. Harrison, J. Roush, J. Wallace, Trends in age of identification and intervention in infants with hearing loss, Ear Hear. (2003) 89–95. [14] P.M. Watkin, Neonatal otoacoustic emission screening and the identification of deafness, Arch. Dis. Child. Fetal Neonatal Ed. 74 (1) (1996) F16–F25. [15] J. Bamford, K. Uus, A. Davis, Screening for hearing loss in childhood: issues, evidence and current approaches in the UK, J. Med. Screen. 12 (3) (2005) 119–124. [16] F. Declau, A. Doyen, T. Robillard, S.J. De Varebeke, Universal newborn hearing screening, B-Ent (Suppl. 1) (2005) 16–21, quiz 22–3. [17] Joint committee of infant hearing, 1994 position statement, Pediatrics 100 (1995) 152–156. [18] National Institutes of Health, Early identification of hearing impairment in infants and young children, NIH Consens. Statement 11 (1) (1993) 1–25. [19] K.P. Steel, Science, medicine, and the future: new interventions in hearing impairment, BMJ 320 (7235) (2000) 622–625. [20] T. Wada, T. Kubo, T. Aiba, H. Yamane, Further examination of infants referred from newborn hearing screening, Acta Otolaryngol. Suppl. 554 (2004) 17–25. [21] V. Weichbold, D. Nekahm-Heis, K. Welzl-Mueller, Universal newborn hearing screening and postnatal hearing loss, Pediatrics 117 (4) (2006) e631– e636. [22] H.M. Fortnum, A.Q. Summerfield, D.H. Marshall, A.C. Davis, J.M. Bamford, Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study, BMJ 323 (7312) (2001) 536–540. [23] A. Young, H. Tattersall, Universal newborn hearing screening and early identification of deafness: parents’ responses to knowing early and their expectations of child communication development, J. Deaf Stud. Deaf Educ. 12 (2) (2007) 209– 220. [24] C. Yoshinaga-Itano, Levels of evidence: universal newborn hearing screening (UNHS) and early hearing detection and intervention systems (EHDI), J. Commun. Disord. 37 (September–October (5)) (2004) 451–465.