ArticlePDF Available

Outcome of Cochlear Implantation in Post-Meningitis Deaf Children

January 2013
Iranian Red Crescent Medical Journal 15(1):15-7

DOI:10.5812/ircmj.3394

Source
PubMed

License
CC BY 4.0

Authors:

Mahdiyeh Hasanalifard

Baqiyatallah university of medical sciences, Tehran,Iran

Mohammad Ajalloueyan

Baqiyatallah University of Medical Sciences

Susan Amirsalari

Baqiyatallah University of Medical Sciences

Amin Saburi

Baqiyatallah University of Medical Sciences

Background Bacterial meningitis is the most common cause of secondary sensori-neural hearing loss in pediatrics. Due to concomitant neurological squeal such as seizure, visual impairment and hydrocephalus the successful outcome of cochlear implantation is doubtful. The aim of this survey is assessment of cochlear implantation outcomes in post meningitis deaf children. Methods Patients who were implanted at Baqiyatallah Cochlear Implant Center, during the years 2008–2010 due to post meningitis deaf childrenwere enrolled. the intraoperatively and Postoperative auditory and speech abilities were explored and compared. Results Two hundred eighty-four children with hearing loss were evaluated and eight children who were diagnosed as Post Meningitis Deafness were enrolled. The mean age of children at the meningitis diagnosis was 15.75±6.77 months and the mean age at cochlear implantation was 31.12±1.27 months. Electrode insertion in 6 out of eight patients was complete but 2 children required cochlear drill-out and in one child short electrode was used. The survey shows that auditory and language skills improved as well as expected. Improvement of auditory and speech abilities after 6 months was statistically significant (P.value< 0.05). Conclusions It seems that cochlear implantation outcome in post meningitis deaf children is not the same as non meningitis deaf children but the cochlear implantation is the only and in most cases the best way of helping these children, particularly if the gap time between deafness and surgery is minimized and the ossification is limited. Variation in outcome is not constant reason to restrict cochlear implantation in children with post meningitis deafness.

Content uploaded by Amin Saburi

Content may be subject to copyright.

Iranian Red Crescent Medical Journal

Outcome of Cochlear Implantation in Post-Meningitis Deaf Children

Mahdiyeh Hasanalifard 1, Mohammad Ajalloueyan 1, Susan Amirsalari 1, Amin Saburi 1, *

1 New Hearing Technologies Research Center, Baqiyatallah University of Medical Sciences, Tehran, IR Iran

* Corresponding author: Susan Amirsalari, New Hearing Technologies Research Center, Baqiyatallah University of Medical Sciences, Tehran, IR Iran. Tel.:

+98-2188600067 , E-mail: susanamirsalari@yahoo.com

Keywords: ENT; Pediatrics; Pediatrics Surgery

Article type: Letter; Received: 01 Nov 2011, Revised: 10 Mar 2012, Accepted: 06 Apr 2012; DOI: 10.5812/ircmj.3394

Please cite this paper as:

Hasanalifard M, Ajalloueyan M, Amirsalari S, Saburi A. Outcome of Cochlear Implantation in Post-Meningitis Deaf Children. Iran Red

Cres Med J. 2013:15(1).15-7. DOI: 10.5812/ircmj.3394

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which per-

mits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Dear Editor,

Cochlear implantation (CI) is an eﬀective procedure for

treatment of children with severe to profound Sensori-

neural hearing loss (SNHL). In spite of suitable outcome in

many patients, choosing the candidates should be regard-

ing to the child age and etiology (congenital or acquired

SNHL) (1-4). Bacterial meningitis (BM) is one of the most

common etiologies of acquired SNHL which estimated ap-

proximately 60 - 90% of all cases of secondary SNHL at chil-

dren (5). Due to concomitant neurological sequelae such

as seizure, visual impairment and hydrocephalus, the suc-

cessful outcome of CI in these cases remained doubtful.

We introduce a case series about outcome of cochlear im-

plantation at children with SNHL due to BM as the prelimi-

nary report from Iran. Two hundred eighty-four children

with hearing loss presenting to the cochlear implantation

center of the Baqiyatallah Hospital between 2008 and

2010 were evaluated and ﬁnally, eight children with Post

Meningitis deafness (PMD) were enrolled. Profound SNHL

was conﬁrmed based on the average of pre-implantation

unaided pure-tone thresholds over 90 dB. There were com-

plementary investigations for overruling other cause of

SNHL. The Nucleus 22 channel device and a speech proces-

sors device was used, routinely although other option has

been considered in special subjects. Each cases assessed by

Nerve Response Telemetry (NRT) intra-operatively and 45

days after surgery. Speech Intelligibility Rating (SIR) and

Categories of Auditory Perception scale (CAP) tests was

conducted in the best-aided situation both before and af-

ter implantation.6 All cases were assessed at three, six, 12,

and 24 months after CI. This investigation was approved by

the ethical review board. The mean age of children at the

meningitis diagnosis was 15.75 ± 6.77 (Mean ± SD) months

and the mean age at cochlear implantation was 31.12 ± 1.27

months. Two patients was male (patients number 1&4).

The microorganism cultured from the CSF was identi-

ﬁed in 3 (37.5%) patients. In three patients (No. 1, 3&4) the

causative microorganism was Streptococcus Pneumoniae

(Pneumococcus) and in other subjects causative microor-

ganisms were unknown. Electrode insertion in 6 out of

eight patients was complete but two children required co-

chlear drill-out and in one child short electrodes was used.

There was no serious complication after operation during

6 months follow up. The mean of NRT at the baseline, 3 and

6 months later was 69.37 ± 96.78, 187.37 ± 19.24 and 184.62

± 17.32, respectively ( Table 1 ). We used SPSS version 16 and

repeated measured ANOVA test to compare the CAP and

SIR ﬁndings. By using this test we were able to compare

the CAP and SIR score between more than two stages (0,

3 and 6 month after implantation). Three months after CI,

the mean score of CAP test developed from 0.62 ± 0.74 at

the baseline to 3.00 ± 1.41 and also increased to 3.75 ± 1.16 at

the 6-months after CI (P < 0.001). Also, SIR scored a mean

of 1.25 ± 0.46 at the baseline improved to 1.37 ± 0.74 at 3

months after implantation (P = 0.351) and a mean of 2.25

± 0.88 at 6 months later (P < 0.001) ( Table 2 ). Previously,

the CI success and eﬃcacy in children with additional

disability such as PMD compared to children with pure

SNHL were debatable (6, 7). This supposition was because

of having concomitant neurological squeal. The electrode

may be inserted incompletely due to ossiﬁed cochlea (8),

although, results of several previous studied were equivo-

cal. Howard et al recommended that neurologic squeal of

cochlear implantation and post-meningitis deafness

Hasanalifard M et al.

Iran Red Crescent Med J. 2013:15(1)16

BM annoy the improvement of speech perception after CI

in patients with PMD (9) El-Kashlan et al. showed children

with cochlear ossiﬁcation due to BM have signiﬁcant lower

speech perception improvement than a matched control

children with congenital SNHL at both the 6 and 24-month

follow-up after CI but with extended follow-up, some chil-

dren with ossiﬁcation had speech perception partially

(10). Eshragi et al. revealed children with PMD and those

with cochlear ossiﬁcation who undergo CI may require

frequent programming adjustments to obtain the opti-

mal performance because levels of stimulation increase

over the time (11). Partial insertion is more suitable and

comfortable than complete insertion in ossiﬁed cochlea

or labyrinth for surgeons (12). Age and causative microor-

ganism are important factors to determinate the outcome

in children with post meningitis deafness (13). Also, the

role of time between PMD and implantation is arguable.

Some survey recommended that CI should be performed

after diagnosis of PMD as soon as possible and other sug-

gested late approach (14).Young et al. showed that early

bilateral simultaneous CI in children with PMD increases

the likelihood of binaural hearing and ensures implanta-

tion of the better ear in this population of children whose

course is often complicated by formation of scar tissue

and ossiﬁcation within the cochlea (15). Regarding to the

results of present study and similar studied we conclude

that children with post meningitis deafness could be Bene-

ﬁted from CI. However, Studies with larger sample size and

a control group with longer follow-up period for conﬁrm-

ing the prognostic factors are recommended.

Table1. Nerve Response Telemetry (NRT) Findings

No. Electrodes inser-

tion

NRT (at the base-

line)

NRTa(45 days after

CI)aNRT (3 months

after CI)

NRT (6 months

after CI)

1Suitable 0 205 195 194

2Suitable 155 145 140 143

3Drill & Short elec-

trodes

0 201 196 197

4Suitable 0 196 194 190

5Suitable 195 186 190 190

6Drill & normal

electrodes

0 195 196 193

7Suitable 205 187 195 185

8Suitable 0 195 193 185

a Abbreviations: CI, cochlear implantation; NRT, nerve response telemetry

Table 2. CAP and SIR Score in Patient Before and After CI

No. CAPa( before

CI)

CAP (3 months

after CIa)

CAP (6 months

after CI)

SIRa(before CI) SIR (3 months

after CI)

SIR (6 months

after CI)

10 1 2 1 1 2

22 6 6 2 3 4

30 3 3 1 1 2

40 3 4 1 1 2

50 2 3 1 1 1

61 3 4 1 1 2

71 3 4 1 1 2

81 3 4 2 2 3

a Abbreviations: CAP, Categories of Auditory Perception; CI, cochlear implantation; SIR, speech intelligibility rating

Acknowledgements

The authors acknowledge the parents of children who

participated kindly in this survey.

Financial Disclosure

None declared.

Funding Support

None declared.

References

1. Bacciu A, Ormitti F, Pasanisi E, Vincenti V, Zanetti D, Bacciu S.

Cochlear implantation in pontine tegmental cap dysplasia. Int J

Pediatr Otorhinolaryngol. 2010;74(8):962-6.

cochlear implantation and post-meningitis deafness Hasanalifard M et al.

Iran Red Crescent Med J. 2013:15(1)

2. Bacciu A, Pasanisi E, Vincenti V, Ormitti F, Di Lella F, Guida M, et

al. Cochlear implantation in children with cerebral palsy. A pre-

liminary report. Int J Pediatr Otorhinolaryngol. 2009;73(5):717-21.

3. Nikolopoulos TP, Kiprouli K. Cochlear implant surgery in chal-

lenging cases. Cochlear Implants Int. 2004;5 Suppl 1:56-63.

4. Rizer FM, Burkey JM. Cochlear implantation in the very young

child. Otolaryngol Clin North Am. 1999;32(6):1117-25.

5. Durisin M, Arnoldner C, Stover T, Lenarz T, Lesinski-Schiedat A.

Audiological performance in cochlear implanted patients deaf-

ened by meningitis depending on duration of deafness. Eur Arch

Otorhinolaryngol. 2008;265(4):381-8.

6. Amirsalari S, Ajallouyean M, Saburi A, Haddadi Fard A, Abed

M, Ghazavi Y. Cochlear implantation outcomes in children

with Waardenburg syndrome. Eur Arch Otorhinolaryngol.

2012;269(10):2179-83.

7. Amirsalari S, Youseﬁ J, Radfar S, Saburi A, Tavallaie SA, Hosseini

MJ, et al. Cochlear implant outcomes in children with motor de-

velopmental delay. Int J Pediatr Otorhinolaryngol. 2012;76(1):100-3.

8. Liu X, Yao Y, He G, Zhai L. [Magnetic resonance imaging study and

cochlear implantation in post-meningitic deaf patients]. Lin Ch-

uang Er Bi Yan Hou Ke Za Zhi. 2004;18(7):399-401.

9. Francis HW, Pulsifer MB, Chinnici J, Nutt R, Venick HS, Yeagle JD,

et al. Eﬀects of central nervous system residua on cochlear im-

plant results in children deafened by meningitis. Arch Otolaryn-

gol Head Neck Surg. 2004;130(5):604-11.

10. El-Kashlan HK, Ashbaugh C, Zwolan T, Telian SA. Cochlear implan-

tation in prelingually deaf children with ossiﬁed cochleae. Otol

Neurotol. 2003;24(4):596-600.

11. Eshraghi AA, Telischi FF, Hodges AV, Odabasi O, Balkany TJ.

Changes in programming over time in postmeningitis cochlear

implant users. Otolaryngol Head Neck Surg. 2004;131(6):885-9.

12. Rotteveel LJ, Snik AF, Vermeulen AM, Mylanus EA. Three-year

follow-up of children with postmeningitic deafness and partial

cochlear implant insertion. Clin Otolaryngol. 2005;30(3):242-8.

13. Mitchell TE, Psarros C, Pegg P, Rennie M, Gibson WP. Performance

after cochlear implantation: a comparison of children deafened

by meningitis and congenitally deaf children. J Laryngol Otol.

2000;114(1):33-7.

14. Allegro J, Papsin BC, Harrison RV, Campisi P. Acoustic analysis of

voice in cochlear implant recipients with post-meningitic hear-

ing loss. Cochlear Implants Int. 2010;11(2):100-16.

15. Young NM, Tan TQ. Current techniques in management of post-

meningitic deafness in children. Arch Otolaryngol Head Neck Surg.

2010;136(10):993-8.

Auditory and speech outcomes of cochlear implantation in post-meningitis deafness

Article

Jan 2022
Int J Pediatr Otorhinolaryngol

Purpose This study aims to identify the auditory, speech, and surgical outcomes of cochlear implantation in patients with profound SNHL following bacterial meningitis. Methods Subjects with bilateral severe to profound SNHL who underwent unilateral cochlear implantation from 2003 to 2020 were included in this historical cohort study. The main outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Ratings (SIR) scores. The CAP and SIR outcomes were collected as three-time points after surgery: 6, 12, and 24 months. In order to achieve the strength of the relationship and for computing the Risk Ratio (RR) by log-binominal regression method, we used two binary categorizations of CAP and SIR in our analyses. Results The mean of age at implantation of the study and control group were 144.30 (156.90) and 121.10 (133.70) months, respectively. In the study group, 19 of 35 (54.3%) patients were male, and 16 (45.7%) were female. In the control group, 34 of 81 (42.0%) patients were male and 47 (58.0%) were female. The mean scores of CAP and SIR in our study improved significantly during the time in both groups. All p-values (p) were significant in both groups (T2 vs T1, T3 vs T1, and T3 vs T2). Our analysis by log-binomial regression and computing the RR based on the first and second categorization of CAP and SIR showed moderate to strong relationships between the presence of a history of meningitis and inappropriate CAP and SIR outcomes in these patients. Conclusions Although subjects who were deafened due to meningitis benefit significantly from cochlear implantation, we found moderate to strong relationships between the history of meningitis and inappropriate CAP and SIR outcomes in these patients.

A Comparison of Surgical Auditory Nerve Response and Speech Outcomes in Patients with Post-meningitic Deafness and Without Cochlear Osteogenesis Who Underwent Cochlear Implantation

Article

Full-text available

Sep 2019

Objective Patients with post-meningitis deafness remain challenging candidates for cochlear implantation (CI) which can be difficult due to fibrosis or ossification of the inner ear, and their outcomes remain doubtful. We assessed the surgical and audiological outcomes of CI in patients with profound sensorineural hearing loss caused by meningitis and compared those outcomes to patients without cochlear ossification. Methods This retrospective cross-sectional study was carried out at King Fahad General Hospital, Jeddah, Saudi Arabia. Among 246 patients who underwent cochlear implantation, 13 patients with post-meningitic deafness were identified (Group 1). A matched control group, including patients with deafness due to other causes who did not have cochlea osteogenesis, was selected (Group 2). For all patients, data were collected from medical records, including surgical and audiological outcomes. Results Sclerosis of the cochlea was high in Group 1 (46.2%). There were no postoperative surgical complications in either group. Responses of the auditory nerve action potential obtained through auditory response telemetry (ART) or the neural response telemetry (NRT) were recorded. There was no significant difference between the two groups regarding the intraoperative and the postoperative ART or NRT at selected electrodes representing the entire cochlea. Likewise, no significant difference regarding the speech recognition test (SRT) was detected. Conclusions Cochlear implantation is a safe procedure without surgical complications in post-meningitis patients. Furthermore, early CI in children was associated with favorable outcomes in terms of preservation of the auditory nerve response, restoration of speech discrimination, and recognition to levels comparable to patients with deafness due to other causes. Early audiological assessment in meningitis patients is recommended to identify hearing loss and eventually to offer CI.

Dysmorphic features and cochlear implantation outcome in children with sensorineural hearing loss; the headmost study

Article

Jan 2018

Address for correspondence: Prevalence of anxiety and depression in mothers of children with profound bilateral sensorineural hearing loss: A brief report

Article

Full-text available

Dec 2014

Background: Stress caused by physical disorders of offspring, such as deafness, is the potential reason of depression and anxiety in their parents. This study assessed the prevalence of depression and anxiety in mothers of children with sensorineural hearing disorders. Materials and Methods: An analytical cross-sectional study was conducted on 59 mothers of children diagnosed with profound sensorineural deafness who were referred to Baqiyatallah university hospitals in 2009. Depression and anxiety were assessed by depression and anxiety questionnaire (Beck Second edition). Results:The mean ages of children and mothers were 18.31 ± 4.72 months and 29.42 ± 4.39 years, respectively. The prevalence of depression and anxiety were 39 out of 59 (66.10%) and 47 out of 59 (79.66%), respectively. Conclusion: Depression and anxiety in mothers of children with profound sensorineural hearing loss have a high incidence and psychological complications must be considered substantially in mothers of children with hearing disorders.

Bacterial Meningitis after Cochlear Implantation among Children without Polyvalent Conjugate Vaccine: A Brief Report of an Iranian Cohort Study on 371 Cases

Article

Full-text available

Aug 2014

Regarding risk of bacterial meningitis (BM) after Cochlear implantation (CI), it was suggested to receive polyvalent conjugate vaccine. We aimed to estimate the prevalence of BM post CI in child recipients who do not receive polyvalent vaccine. We enrolled 371 children who had received cochlear implants from 2007 to 2010. None of them received pre or post implantation polyvalent conjugate vaccine for BM. We followed all of them for BM for 2 years after implantation. We detected only one female case of BM (0.3% of patients) with the age of 24 months. The mean age of noninfected children was 36.7 ± 23.2 months. The education level of parents was "college level or higher" in less than half of them, and about 65% of patients were products of consanguineous marriage. Our findings indicated that the incidence of BM was not higher in our cochlear implanted children who did not receive immunization than patients from countries in which routine vaccination is done. We suggest that although proper immunization is recommended before surgery, this procedure could be performed without vaccination, especially in developing countries that face financial problems for preparing vaccines.

Systematic Review and Meta-Analysis Cochlear Implantation in Post-Meningitis Deafness: Audiological, Imaging, and Postoperative Outcomes: A Systematic Review With Qualitative Synthesis

Article

Aug 2024
OTOL NEUROTOL

Introduction Post-meningitis deafness (PMD) is a potentially devastating cause of hearing loss among pediatric and adult patients, for which hearing rehabilitation with cochlear implants (CIs) remains the standard of care. To date, there have been limited systematic studies on the impact of cochlear ossification (CO) and time-to-implantation (TTI) on audiological outcomes. Methods An online database search was performed on the PubMed, Embase, and Scopus databases for articles within the past 20 years pertaining to audiological outcomes among pediatric and adult patients with PMD. Information on study characteristics, patient demographics, clinical outcomes, and postoperative complications was collected and analyzed. Results From 8,325 articles generated in the original search, 11 were included in the final analysis, representing 376 patients in total. Of the articles discussing TTI, the majority (3 of 4) found that a shorter TTI of 6 months on average led to improved audiological outcomes compared with control groups with a longer TTI. Of the articles that discussed the impact of preoperative CO, the majority (4 of 6) found that the presence of CO had a detrimental effect on postoperative audiological outcomes after CI. Finally, of the articles that discussed long-term audiological outcomes for PMD compared with the non-PMD control group after CI, the majority (4 of 7) found that PMD patients had inferior long-term outcomes. Conclusion CI is a safe and effective treatment modality for PMD, with the majority of literature demonstrating improved long-term outcomes for patients without CO and a reduced TTI.

Functional Outcomes from Cochlear Implants in Children with Acquired Hearing Loss: Case Studies

Article

Apr 2024

Purpose: Early identification of postlingual hearing loss (HL) and knowing its probable cause is of great significance. Causes that result in irreversible profound HL require immediate radiological investigation and intervention to decide the best listening device.Methods: Case reports of three children with acquired HL using cochlear implant (CI) are being presented to understand the functional outcomes in domains of audition and language skills after 12 months of CI use. The age of onset of deafness and implantation were not identical. Etiology of deafness was meningitis in two cases and viral infection in the other. Consent was obtained from the parents for participating in the study. Case history review was conducted to collect demographic and audiological details of the participants. Functional benefits from CI in those children were assessed in the domains of auditory and language skills using standardized assessment tools respectively.Results: Improvements in all domains of auditory and language skills were observed. However, the degree of benefits depended on the duration of deafness, etiology of deafness, age at onset of deafness, age at implantation and effectiveness of intervention.Conclusions: The results of the study can be used during pre-implant CI counselling to facilitate development of real expectations about the functional benefits of CI in children with acquired HL due to various reasons and showed the importance of early cochlear implantation following the diagnosis. These case presentations give an understanding of the various factors that can affect the functional outcome from CI in children with post-lingual HL.

Universal Newborn Hearing Screening in Italy

Article

Full-text available

Jan 2008
Arch Dis Child

The aim of this paper is to provide data on the degree of implementation and coverage of UNHS in Italy. Data were collected through a Questionnaire that was sent to all Birthing Hospitals active in Italy in 2006. Results revealed a quick diffusion of UNHS in Italy from 29.3% in 2003 to 48.4% in 2006. The majority of UNHS programs were implemented in the North West, 79.5%, and North East, 57.2%, while there remains a limited diffusion in some areas, typically in the Islands (11.3%). Our results suggest that three conditions seem to play a crucial role in the implementation of UNHS in Italy: the size and location of the hospital, and the presence of an audiologist in the UNHS coordinating team.

Hearing Status in Neonatal Hyperbilirubinemia by Auditory Brain Stem Evoked Response and Transient Evoked Otoacoustic Emission

Article

Full-text available

Feb 2011

Hyperbilirubinemia at neonatal period is one of the major deteriorating factors of the auditory system. If left untreated, it may cause certain cerebral damage. This study aims to evaluate the impact of hyperbilirubinemia on the hearing of neonate. This study was conducted on 35 newborn babies with jaundice (bilirubin more than 20 mg/dL). Auditory brainstem response (ABR) and transient evoked otoacoustic emission (TEOAE) tests were performed, after treatment and one year after. ABR test results indicated that 26 children (74.3%) had normal hearing but 9 (25.7%) suffered from an impairment. As for TEOAE test, 30 children (85.7%) passed whereas the remaining (14.3%) seemed to be failures. The comparative results of the two tests pointed to autonomic neuropathy /autonomic dysreflexia symptoms in 5 babies. Due to the high incidence of autonomic neuropathy/autonomic dysreflexia among hyperbilirubinemic babies, screening in this regard seems reasonable. Our result emphasizes the necessity of more experiments on the afflicted areas.

Universal neonatal audiological screening: Experience of the University Hospital of Pisa

Article

Full-text available

Apr 2011
Riv Ital Pediatr Ital J Pediatr

The early identification of pre-lingual deafness is necessary to minimize the consequences of hearing impairment on the future communication skills of a baby. According to the most recent international guidelines the deafness diagnosis must occur before the age of three months and the prosthetic-rehabilitative treatment with a traditional hearing aid should start within the first six months. When a Cochlear implant becomes necessary, the treatment should start between the age of 12 months and 18 months. The only way to diagnose the problem early is the implementation of universal neonatal audiological screening programs. Transient evoked otoacoustic emissions (TEOAE) is the most adequate test because it's accurate, economic and of simple execution. Automatic auditory brainstem response (AABR) is necessary to identify patients with auditory neuropathy but it is also important to reduce the number of false-positives.The 20-30% of infant hearing impairment is represented by progressive or late-onset hearing loss (HL) so it's also necessary to establish an audiological follow up program, especially in infants at risk. From November 2005 all neonates born in the University hospital of Pisa undergo newborn hearing screening. From 2008 the screening program follows the guidelines for the execution of the audiological screening in Tuscany which have been formulated by our group according to the 2007 JCIH Position Statement and adaptated to our regional reality by a multidisciplinary effort. From November 2005 to April 2009 8113 neonates born in the Neonatal Unit of Santa Chiara Hospital (Pisa) have undergone newborn hearing screening. 7621 neonates (93.9%) without risk factors executed only the TEOAE test. 492 (6.1%) neonates had audiological risk factors and thus underwent TEOAE and AABR. 84 patients (1,04%) failed both TEOAE and AABR tests. 78 of them underwent further investigations. 44 patients resulted falsepositives (the 0,54% of the screened newborns). 34 neonates (4,2 ‰) had a final diagnosis of hearing impairment. 8 patients (0.99 ‰) had unilateral hearing loss (HL). 26 patients (3,2 ‰) had bilateral hearing impairment. In our screening program the percentage of false-positives was quite low (0.54%) while the incidence of bilateral HL (3.2 ‰) is a little higher than that found in literature reports. In most of our patients premature birth or neonatal suffering represent the main cause of HL.

The pilot study of a nationwide neonatal hearing screening in Iran: Akbarabadi and Mirzakouchak- Khan hospitals in Tehran (June 2003-October 2004)

Article

Oct 2006

Saeid Mahmoudian

Introduction: This study investigates the conditions under which neonatal hearing screening is useful in Iran. The objectives of the study were to identify prevalence of significant bilateral SNHL and then to demonstrate whether newborn mass screening is feasible and cost effective and would result in early detection and treatment of significant hearing impairment Methods: Babies born at Akbarabadi and Mirzakouch-Khan university maternity hospitals from June 2003 to October 2004 were screened at birth in three stages by using transient evoked otoacoustic emissions (TEOAEs) and ABR. All newborns were tested under standard conditions before discharge from the hospital. According to the screening results, full audiological evaluations were performed when necessary. The screening method used was the TEOAEs-acoustic method. There were three stages in this study: initial screening, re-screening and diagnostic tests with ABR/OAEs. High risk neonates, especially those hospitalized in NICU, were scheduled for follow-up even if they were normal in the initial screening. Results: The result from 8490 live newborns indicated that 89.7% of newborns proved normal in the primary screening in the maternity ward, and 10.3% were abnormal. Out of 873 infants scheduled for follow-up study only 571 neonates returned. Using follow- up letters and phone calls, we managed to improve the response rate to 56% of bilateral failures. Using Poison's distribution for frequency rate of hearing impairment and normal estimate for this distribution and 95% confidence intervals, significant bilateral sensorineural hearing loss was present in ~1 to 4 per 1000 live births in the well baby nursery population (12 neonates), and in ~2.5 to 4.6 per 100 infants in the intensive care unit. The outcome of this program has stimulated the development of a nationwide (universal) newborn screening program which will be started in near future. Conclusion: Mass newborn screening for hearing impairment in our population was found to be feasible, beneficial, cost beneficial and justified.

Comparison of Hearing Screening Programs between One Step with Transient Evoked Otoacoustic Emissions (TEOAE) and Two Steps with TEOAE and Automated Auditory Brainstem Response

Article

Nov 2005
LARYNGOSCOPE

Objective: To compare the efficacy between one step with transient evoked otoacoustic emissions (TEOAE) and two steps with TEOAE and automated auditory brainstem response (AABR) in a newborn hearing screening program. We investigated their differences in referral rate, the accurate identification rate of congenital hearing loss (HL), and cost effectiveness. Method: From November 1998 to December 2004, a total of 21,273 healthy newborns were screened for HL in Mackay Memorial Hospital, Taipei. In the periods from November 1998 to January 2004 and from February 2004 to December 2004, the screening tools used were TEOAE alone (n = 18,260) and TEOAE plus AABR (n = 3,013), respectively. Results: A statistically significant decrease of referral rate was achieved in the group using TEOAE and AABR as screening tools when compared with TEOAE alone (1.8% vs. 5.8%). The accurate identification rate of congenital HL was 0.45% in TEOAE protocol and 0.3% in TEOAE and AABR protocol, which was not statistically significant. The total direct costs per screening were 10.1 U.S. dollars for the program using TEOAE alone and 8.9 U.S. dollars for the TEOAE plus AABR program. The intangible cost, however, was much higher in the earlier program because of the higher referral rate. Conclusion: In terms of the efficacy of a hearing screening program using the one step TEOAE and two step TEOAE and AABR programs, the later significantly decreased the referral rate from 5.8% to 1.8%. No significant difference was noted between the accurate identification rates of congenital HL. The total costs, including expenditures and intangible cost, were lower in the protocol with TEOAE plus AABR.

Cochlear implantation outcomes in children with Waardenburg syndrome

Article

Dec 2011
Eur Arch Oto Rhino Laryngol

Waardenburg syndrome (WS) is an autosomal dominant disease, characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia iridis, white forelock, and congenital sensori-neural hearing loss (SNHL). The aim of this study was to evaluate the outcome of cochlear implantation in children with WS and compare it with children with pure SNHL. In a prospective study we evaluated 336 cochlear implanted children from 2008 to 2010. The WS was diagnosed by its established criteria and for control group children without any dysmorphic features, anatomical, behavioral, and developmental disorders were also enrolled. We evaluated children of both groups 1 year after cochlear implantation by categories of auditory performance (CAP) and speech intelligibility rating (SIR) tests. Eighty-one children out of the total 336 who had SNHL were included in study. Out of these 75 (22.3%) were healthy and six (1.78%) had WS. Of the 75 healthy children 40 (53.3%) were girls, while of the six children with WS, three (50%) were girls. There was a significant difference in SIR between WS and cases with pure SNHL (2.67 ± 1.03 vs. 3.79 ± 1.11, p = 021) however, the difference was not significant in CAP (4 ± 1.26 vs. 5.13 ± 1.13, p = 0.082). Prevalence of WS was 1.78% at Baqiyatallah Cochlear Implant Center. One year after implantation there was no significant difference in auditory outcome; however, the difference in speech outcome was significant between WS and cases with pure SNHL.

Cochlear implant outcomes in children with motor developmental delay

Article

Nov 2011

Multiple handicapped children and children with syndromes and conditions resulting additional disabilities such as cerebral palsy, global developmental delay and autistic spectrum disorder, are now not routinely precluded from receiving a cochlear implant. The primary focus of this study was to determine the effect of cochlear implants on the speech perception and intelligibility of deaf children with and without motor development delay. In a cohort study, we compared cochlear implant outcomes in two groups of deaf children with or without motor developmental delay (MDD). Among 262 children with pre-lingual profound hearing loss, 28 (10%) had a motor delay based on Gross Motor Function Classification (GMFC). Children with severe motor delays (classification scale levels 4 and 5) and cognitive delays were excluded. All children completed the Categories of Auditory Perception Scales (CAP) and Speech Intelligibility Rating (SIR) prior to surgery and 24 months after the device was activated. The mean age for the study population was 4.09 ± 1.86 years. In all 262 patients the mean CAP score after surgery (5.38 ± 0.043) had a marked difference in comparison with the mean score before surgery (0.482 ± 0.018) (P=0.001). The mean CAP score after surgery for MDD children was 5.03, and was 5.77 for normal motor development children (NMD). The mean SIR score after surgery for MDD children was 2.53, and was 2.66 for NMD children. The final results of CAP and SIR did not have significant difference between NMD children versus MDD children (P>0.05). Regarding to the result, we concluded that children with hearing loss and concomitant MDD as an additional disabilities can benefit from cochlear implantation similar to those of NMD.

Current Techniques in Management of Postmeningitic Deafness in Children

Article

Oct 2010

To determine pneumococcal vaccination status of children with recent postmeningitic deafness and to review our current approach for achieving early implantation in this special population that is at significant risk for cochlear ossification. Review of imaging studies and test results. Tertiary care/referral children's hospital. Five children ranging in age from 15 months to 10 years who experienced recent onset of profound bilateral sensorineural hearing loss due to pneumococcal meningitis. All children underwent preoperative magnetic resonance imaging with 3-dimensional heavily T2-weighted steady-state free precession sequences. Four children underwent auditory steady-state response testing. All underwent bilateral cochlear implantation. Degree of electrode insertion using standard surgical procedures. All children developed meningitis despite a history of pneumococcal vaccination. Complete electrode insertion in both ears was achieved. Pneumococcal vaccination has reduced but not eliminated childhood deafness secondary to pneumococcal disease. Auditory steady-state response testing and 3-dimensional steady-state free precession imaging are modalities that expedite candidacy evaluation of this population. Early bilateral simultaneous implantation increases the likelihood of binaural hearing and ensures implantation of the better ear in this population of children whose course is often complicated by formation of scar tissue and ossification within the cochlea.

Cochlear implantation in pontine tegmental cap dysplasia

Article

Aug 2010

Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months' postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person's speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration.

Newborn hearing screening in a developing country: Results of a pilot study in Abidjan, Côte d'Ivoire

Article

Dec 2009

To investigate the feasibility of neonatal hearing impairment in newborn babies in Abidjan, Côte d'Ivoire. It is a cross-sectional study in which all infants aged from 3 to 28 days, attending for Bacille Calmette-Guerin (BCG) immunization in primary care centers or hospitalized in neonatal intensive care units (NICU), between July 2007 and March 2008, were included. Screening followed a two-stage strategy with transient evoked otoacoustic emissions (TEOAE). Infants referred after the second-stage screening were scheduled for diagnostic evaluation by diagnostic auditory brainstem response (ABR). The variables analyzed were: screening coverage, referral rates; return rates for second-stage screening and diagnostic evaluation, incidence of permanent hearing loss and age at diagnosis. 1306 newborns, of a total of 1495, were successfully screened, giving a screening coverage of 87.4%. The average age was 4.5 days (S.D.: 2.7), with 5.85 days (S.D.: 3.17) for the immunization group and 3.20 days (S.D.: 0.40) for the neonatal unit group. In total, 286 out of the 1306 infants (21.9%) were referred after the first-stage screening; out of which 193 (67.5%) return for the second stage. After the second-stage screening, 48 (16.8%) were scheduled for diagnostic evaluation (45 from NICU and 3 from primary care centers). The overall referral rate for diagnostic evaluation was 3.7% (48/1306). Only 18.75% of those referred (9/48) returned for evaluation, and seven of them (77.8%) were confirmed with hearing loss (2 from immunization group and 5 from neonatal unit group). The prevalence of permanent hearing loss in this screened population was 5.96 per 1000 (7/1174 babies who completed the screening) [95% I.C.: 5.62-6.30 per 1000]. The mean age at diagnosis was 22 weeks (S.D.: 8.3). The reasons for non-completed screening were, according to 62 mothers: no financial means, absence of hearing loss, fear of spouse reactions, lack of information about this test and deafness. The incidence of permanent and early hearing impairment identified by this screening program was about 6 per 1000. Routine hearing screening of infants for the early detection of hearing loss is necessary in Côte d'Ivoire. It is possible to implement such a hearing screening, targeting all newborns, in primary health care centers and neonatal intensive care units.