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Year : 2012  |  Volume : 18  |  Issue : 4  |  Page : 193-195

Inner ear infections as cause of perinatal deafness

Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences,New Delhi, India

Date of Web Publication19-Dec-2012

Correspondence Address:
Kapil Sikka
Department of Otorhinolaryngology and Head and Neck Surgery, 4th floor, Room No 4057, Academic Block, AIIMS, Ansari Nagar, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-7749.104797

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Objective: To assess the role of infective agents as cause of sensorineural hearing loss (SNHL) in children. Setting: Tertiary care center actively involved in management of hearing impairment through cochlear implant program and other rehabilitation program. Materials and Methods: Retrospective chart review of 213 patients who underwent cochlear implantation at our center from 2007 to 2011 was carried out. Out of these, 185 were children. We have done the data analysis with regard to etiology of hearing loss in these 185 children. An etiology for SNHL could be established in 100 out of these 185 cases. Out of these 100 cases, we have further segregated cases where an infectious etiology was implicated. Results: Out of 185 prelingual cochlear implantees, etiology could be determined in 100 cases. Etiology was of infective origin in 26 of these 100 cases. Infective agents implicated in congenital acquired hearing loss were Toxoplasma, Rubella, Cytomegalovirus, and Herpes (TORCH) infections ( n = 9) including Rubella ( n = 7) and Cytomegalovirus (CMV) ( n = 2). Meningitis ( n = 11) and other infections ( n = 6) were responsible for secondary acquired hearing loss. Conclusions: Results showed that among the identified causes, infective agents were responsible in one-fourth cases of profound SNHL. Building awareness about such existence and their major role in causing SNHL among the otolaryngologists, pediatricians, obstetricians, physicians, audiologists and public is considered essential so that such preventable and controllable maladies are reduced by combined efforts from all these stakeholders.

Keywords: Congenital, Perinatal deafness, Sensorineural hearing loss

How to cite this article:
Gupta V, Sikka K, Kumar R, Deka RC. Inner ear infections as cause of perinatal deafness. Indian J Otol 2012;18:193-5

How to cite this URL:
Gupta V, Sikka K, Kumar R, Deka RC. Inner ear infections as cause of perinatal deafness. Indian J Otol [serial online] 2012 [cited 2021 Apr 10];18:193-5. Available from: https://www.indianjotol.org/text.asp?2012/18/4/193/104797

  Introduction Top

Development of language, speech, and communication is one of the most gifted skills in humans. Achieving this skill is directly dependent on hearing function. Sensorineural hearing loss (SNHL) can thus cause extensive social, economic, and medical implications in patients based on the degree of hearing loss. Pathologies causing SNHL include hereditary, congenital, or acquired disorders in perinatal period or during infancy and childhood. [1],[2],[3],[4]

In developing countries, SNHL secondary to the infective diseases is a major public health problem. Various infective etiologies causing perinatal deafness include maternal Toxoplasma, Rubella, Cytomegalovirus, and Herpes (TORCH) infections, meningitis, mumps, measles, and others. Meningitis and other infections are either themselves responsible for SNHL or due to other factors like intensive care unit (ICU) care, ototoxic medication, electrolyte imbalance, etc., which might be associated with these conditions and diseases. [3],[4],[5]

SNHL secondary to these conditions is largely preventable and controllable. This can be achieved by implementing targeted immunization program, following recommended treatment protocols and above all, creating awareness among stakeholders. The pediatricians, the obstetricians, the physicians and the public must be made aware about possibility of developing SNHL in these situations and settings. [3]

  Materials and Methods Top

A retrospective chart review and data analysis of the patients who underwent cochlear implants at our center from 2007 to 2011, was done. The data of 213 patients including 185 prelingual and 28 postlingually deaf implantees were available. The data of 185 prelingual cochlear implantees were carefully assessed and analyzed with regard to possible causes of hearing loss in this group of patients. Attempts are being made in this communication to bring important public health issues through these data.

  Data and Results Top

While reviewing the data regarding the etiology of hearing loss in 185 prelingual cochlear implantees (2007-2011), we have observed that in about 46% (n0 = 85) of these cases etiology was unknown or could not be determined. Out of 100 cases in which etiology was determined, in 26 cases, hearing loss was of infective origin and thus possibly preventable. This constitutes 14% of the total cases (n = 185) and about one-fourth of the cases with known etiology ( n = 100 ). These were subdivided as follows:

Congenital acquired hearing loss

Nine of these cases were of congenital hearing loss due to maternal TORCH infections with a male to female ratio of 4:5 and an average age of 5.77 years (age range: 2-8 years). Out of these nine cases, seven cases were due to maternal Rubella infection and in two cases, Cytomegalovirus (CMV) was implicated. One of these nine cases also had birth asphyxia.

Secondary acquired hearing loss

A total of 17 cases developed hearing loss in infancy or in early childhood, which was either due to meningitis ( n = 11) or infections other than meningitis (n = 6).

Out of 11 cases of SNHL secondary to meningitis, 8 were male and 3 were female with an average age of 5.11 years (age range: 2-12 years); 2 of these 11 cases also had history suggestive of ototoxicity.

Six cases with male female ratio of 1:1 and average age of 5.56 years (age range: 2.5-12 years) were due to other viral or bacterial agents. These include measles, mumps, pneumonia, chicken pox, enteric fever, and labyrinthitis, possibly of bacterial origin.

The details of infective etiology are provided in [Table 1].
Table 1: Details of infections causing deafness

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  Conclusions Top

Availability of newer more effective vaccines and wider reach and implementation of immunization program have decreased the prevalence of many previously common nongenetic infective causes of SNHL, such as measles, mumps, rubella, and Haemophilus injuenzue type B meningitis. But, in India these still appear to be among the significant etiological factors for SNHL.

A decrease in incidence of acquired sensorineural hearing loss with a relative increase in genetic forms of hearing loss has been reported from developed countries, in past few decades. But same does not stand true for developing countries where acquired as well as genetic hearing loss still counts high. [3],[4]

This is contributed by poor socioeconomic status, lower health awareness, lesser institutional deliveries, inadequate neonatal care, poor literacy level, and high rate of consanguinous marriages. Incidence of congenital hearing loss is much higher in less developed or developing countries. [5],[6],[7]

TORCH agents are still an important cause of congenital SNHL in less developed countries. Pregnant women exposed to these agents carry a significantly increased risk of developing SNHL, visual, and other neurological defects in their offsprings.

After the introduction of rubella vaccine, the past few decades have seen a consistent decrease in congenital rubella incidence in developed countries. But, in developing countries, which lack a rubella vaccination program, congenital rubella syndrome remains a major cause of acquired congenital SNHL. [8] In our study rubella induced SNHL contributed 7% to the total cases with known etiology.

Only one-tenth of congenitally infected neonates have clinical signs of CMV infection at birth. Clinical signs include hepatosplenomegaly, jaundice, a petechial or purpuric rash, intrauterine growth retardation, or respiratory distress. [9],[10]

A total of 50% of the neonates with clinical signs of CMV infection at birth also have SNHL and there occurs a progressive increase in hearing thresholds in many of them. [11]

A total of 8-10% of neonates with silent CMV infection may later develop some degree of SNHL. [4] We found two (2%) cases of CMV infection as a cause of SNHL.

Bacterial or viral meningitis has a high incidence of permanent damage to the labyrinth, especially in the very young. Meningitis, as a cause of SNHL has dual implications. One, it causes deafness and two, it causes labyrinthine ossification that makes successful cochlear implantation difficult, and at times, impossible.

Haemophillus influenza type B and pneumococcus are the common pathogens causing meningitis. Hearing loss may occur during the early phase of meningitis but is mostly noted late. SNHL due to meniningitis may affect either one ear or both ears with bilateral loss being commoner and constitutes around 6% of all cases of SNHL in children. [12],[13]

Introduction of better antibiotics and better intensive care facilities has significantly reduced the mortality associated with meningitis and thus we see more postmeningitic subjects surviving with various morbidities including SNHL. [14]

Important predictors for bacterial-meningitis-associated hearing loss include: duration of symptoms longer than 2 days, absence of petechiae, glucose concentrations in cerebrospinal fluid of 0.6 mmol/L or lower, S pneumoniae as the cause, and ataxia. If presence of any one of these risk factors is used to identify children for hearing screening, no child with hearing loss will go undetected. [13] Although, a simpler strategy is to screen all children diagnosed with bacterial meningitis. [12]

Advocacy and implementation of newborn hearing screening has played a significant role in reducing the age of identification of hearing loss from few years to few months. [15] Our review of infective etiological factors therefore emphasizes the need of awareness among doctors and the patients to understand the pattern of the conditions and diseases, which cause such human maladies and deafness. The factors are still rampant in our scenario, and fortunately, still preventable. On the basis of our data and data published previously, [3],[16],[17] we suggest:

  1. Primary prevention of mumps, rubella, meningitis, measles, pneumonia through immunization. Wider coverage of these vaccine preventable infectious diseases would reduce the incidence of acquired SNHL in addition to prevention of serious infections.
  2. Emphasis should be laid on that more and more pregnancies should be registered and deliveries to be institutional. Health care personnels at these centers should be sensitized and trained to identify the deafness-related infections like TORCH.
  3. Physicians, pediatricians and intensivists should be guided through meetings, lectures; to be cautious while treating the child with meningitis. Use of steroids in the early part of the treatment should be considered. [13] Moreover, hearing tests should be done at the earliest possible, so that hearing loss if any should be recognized and managed.

  References Top

1.Davis AC. The prevalence of hearing impairment and reported hearing disability among adults in Great Britain. Int J Epidemiol 1989;18:911-7.  Back to cited text no. 1
2.Wilson DH, Walsh PG, Sanchez L, Davis AC, Taylor AW, Tucker G, et al. The epidemiology of hearing impairment in an Australian adult population. Int J Epidemiol 1999;28:247-52.  Back to cited text no. 2
3.Deka RC. Management of hearing impaired children. Indian Pediatr 1993;30:977-80.  Back to cited text no. 3
4.Smith RJ, Bale JF Jr, White KR. Sensorineural hearing loss in children. Lancet 2005;365:879-90.  Back to cited text no. 4
5.Lin HC, Shu MT, Chang KC, Bruna SM. A universal newborn hearing screening program in Taiwan. Int J Pediatr Otorhinolaryngol 2002;63:209-18.  Back to cited text no. 5
6.Vega Cuadri A, Alvarez Suarez MY, Blasco Huelva A, Torrico Roman P, Serrano Berrocal MA, Trinidad Ramos G. Otoacoustic emissions screening as early identification of hearing loss in newborns. Acta Otorrinolaringol Esp 2001;52:273-8.  Back to cited text no. 6
7.Maisoun AM, Zakzouk SM. Hearing screening of neonates at risk. Saudi Med J 2003;24:55-7.  Back to cited text no. 7
8.Banatvala JE, Brown DW. Rubella. Lancet 2004;363:1127-37.  Back to cited text no. 8
9.Istas AS, Demmler GJ, Dobbins JG, Stewart JA. Surveillance for congenital cytomegalovirus disease: A report from the National Congenital Cytomegalovirus Disease Registry. Clin Infect Dis 1995;20:665-70.  Back to cited text no. 9
10.Boppana SB, Pass RF, Britt WJ, Stagno S, Alford CA. Symptomatic congenital cytomegalovirus infection: Neonatal morbidity and mortality. Pediatr Infect Dis 1992;11:93-9.  Back to cited text no. 10
11.Rivers LB, Boppana SB, Fowler KB, Britt WJ, Stagno S, Pass RF. Predictors of hearing loss in children with symptomatic congenital cytomegalovirus infection. Pediatrics 2002;110:762-7.  Back to cited text no. 11
12.Fortnum H, Davis A. Hearing impairment in children after bacterial meningitis: Incidence and resource implications. Br J Audiol 1993;27:43-52.  Back to cited text no. 12
13.Koomen I, Grobbee DE, Roord JJ, Donders R, Jennekens-Schinkel A, van Furth AM. Hearing loss at school age in survivors of bacterial meningitis: Assessment, incidence, and prediction. Pediatrics 2003;112:1049-53.  Back to cited text no. 13
14.Roos KL. Dexamethasone and non-steroidal anti-inflammatory agents in the treatment of bacterial meningitis. Clin Ther 1990;12:290-6.  Back to cited text no. 14
15.Mehl AL, Thomson V. The Colorado newborn hearing screening project, 1992-1999: On the threshold of effective population-based universal newborn hearing screening. Pediatrics 2002;109:E7.  Back to cited text no. 15
16.Ramakrishna TB, Deka RC, Kacker SK. Psychological problems in the hearing handicaps. Indian J Otolaryngol 1987;39:67-9.  Back to cited text no. 16
17.Venkatakarthikeyan C, Sikka K, Deka RC, Kumar R, Shyam KS, Mohnish G, et al. Etiology of sensorineural hearing loss in children undergoing cochlear implantation. Indian J Otol 2007;13:10-3.  Back to cited text no. 17


  [Table 1]

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[Pubmed] | [DOI]


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