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 Table of Contents  
Year : 2020  |  Volume : 40  |  Issue : 4  |  Page : 149-161

Hearing loss in children: A review of literature

1 Department of Oral and Maxillofacial Surgery, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania
2 Department of Surgery, Division of Ear, Nose and Throat, Ahmadu Bello University Teaching Hospital, Zaria, Kaduna, Nigeria

Date of Submission05-Sep-2019
Date of Decision15-Oct-2019
Date of Acceptance18-Dec-2019
Date of Web Publication21-Feb-2020

Correspondence Address:
Dr. Karpal Singh Sohal
Department of Oral and Maxillofacial Surgery, Muhimbili University of Health and Allied Sciences, P.O. Box 65014, Dar es Salaam
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmedsci.jmedsci_166_19

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Childhood hearing loss (HL) can be attributed to both environmental and genetic factors, therefore it can be congenital or acquired in nature. The effect of childhood HL is on language development, literacy, self-esteem, and social skills. Taking into account the negative impact of HL in children, this review article aims to bring into attention of the medical community the different causes of HL in children and the methods of screening and diagnosing HL in children.

Keywords: Audiometry, causes of hearing loss, children, infections

How to cite this article:
Sohal KS, Moshy JR, Owibingire SS, Shuaibu IY. Hearing loss in children: A review of literature. J Med Sci 2020;40:149-61

How to cite this URL:
Sohal KS, Moshy JR, Owibingire SS, Shuaibu IY. Hearing loss in children: A review of literature. J Med Sci [serial online] 2020 [cited 2022 Aug 19];40:149-61. Available from: https://www.jmedscindmc.com/text.asp?2020/40/4/149/282526

  Introduction Top

Hearing allows one to identify and recognize objects in the world based on the sound he/she produce, hence making communication using sound possible.[1] The process of normal human hearing requires the proper function of the external ear, middle ear, inner ear (cochlea), and ascending brainstem pathways,[2] therefore anything which interferes with the proper functioning of these structures can lead to hearing loss (HL). HL is the most prevailing birth defect, and its prevalence increases as the child reaches adolescence.[3] According to 2018 WHO estimates, children account for 7% (34 million) of all persons living with disabling HL in the world.[4] While the most obvious effect of childhood HL is on language development, it also has an impact on literacy, self-esteem, and social skills, which, in turn, can lead to reduced employment opportunities later in life and psychological consequences that can lead to feelings of isolation, loneliness, and depression.[5],[6] Permanent childhood hearing impairment is defined as a confirmed permanent bilateral hearing impairment ≥40 dBHL (hearing level) averaged over the frequencies of 0.5, 1, 2, and 4 kHz in the better hearing ear.[7] It can be attributed to both environmental and genetic factors, therefore it can be congenital or acquired in nature.[3],[6] Taking into account the negative impact of HL in children, in the present article, an overview of normal hearing, causes of HL in children, and diagnosis of HL in children have been discussed.

Overview of normal hearing

The process of normal human hearing is initiated as sound pressure waves travel through the external auditory canal and vibrate the tympanic membrane.[1],[2] The ossicular chain in the middle ear space then transmits the acoustic energy to the fluid-filled chambers within the cochlea, consequently imitating electrical and chemical gradients between the endolymph and perilymph, which function to power the cochlea.[2],[8] When a sound pressure wave is applied by the stapes to the oval window at the base of the cochlea, a traveling wave is generated that vibrates the basilar membrane maximally at the region tuned to the frequency of the sound stimulus.[2] The vertical movements of the basilar and tectorial membranes generate shearing forces that deflect the hair cell stereo-ciliary bundles in the organ of Corti.[9] Bending of the stereocilia opens mechanosensitive channels near their tips and allows the influx of cations from the endolymph into the hair cell.[2],[8] In the inner hair cells, the resultant depolarization triggers synaptic neurotransmission to afferent auditory neurons. In contrast, the outer hair cells generate unique forces that modify the physical properties of the organ of Corti and lead to frequency-selective amplification of the inner hair cell response.[10] Bending of the stereocilia opens mechanosensitive channels near their tips, allows the influx of sound information to travel through the auditory nerve to the cochlear nucleus, and follows an organized path along multiple brainstem nuclei, ultimately conveying a signal to the auditory cortex, which lies within the temporal lobe adjacent to the Sylvian fissure.[2]

Classification of hearing loss

There are three basic types of HL: conductive HL (CHL), sensorineural HL (SNHL), and mixed HL respectively basing on which part of the auditory system is affected.[11],[12] CHL occurs when sound is not conducted efficiently through the external ear canal to the middle ear. Whereas, SNHL occurs when there is damage to the inner ear or to the nerve pathways from the inner ear to the brain. Anything that disrupts sound getting to the cochlea can be considered CHL, and loss at the point of the cochlea or proximal to the cochlea is considered SNHL.[13],[14] Mixed HL is defined as CHL and SNHL.[11] While the SNHL is far more common in adults, CHL accounts for 90%–95% of all childhood HL.[12],[15]

HLs may be classified according to laterality, symmetry, clinical characteristic (syndromic or not), time of onset (congenital, perinatal, or postnatal), hereditary (genetic or not), time of manifestation (prelingual, perilingual, or post-lingual), and intensity (mild, moderate, severe, and profound).[14]

Causes of hearing loss in children

Hereditary and environmental factors are involved in the etiology of pediatric HL. More than forty genes are associated with the inheritance of HL, among which involves mutations in the gap junction protein beta 2 gene (GJB2) gene. Among environmental factors, viral infection is thought to play a major role in HL.[16] The causes of HL can be broadly grouped into congenital and acquired, such as infections, traumatic, autoimmune, and neoplastic, to name a few [Figure 1].
Figure 1:Some of the causes of hearing loss in children

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Congenital HL (hearing loss present at birth) occurs when the ability of the ear to convert the vibratory mechanical energy of sound into the electrical energy of nerve impulses is impaired.[11] Congenital hearing impairment affects nearly 1 in every 1000 live births; it is one of the most distressing disorders and the most frequent birth defect in developed societies.[5] In the majority of hearing-impaired children, HL is due to genetic factors, most often a single-gene defect.[11] These defects can have different modes of inheritance and different prevalence. HL is classified to reflect the presence (syndromic HL) or absence (nonsyndromic) of coinherited physical or laboratory findings.[11] Autosomal recessive nonsyndromic HL accounts for 80% of genetic cases and is often congenital, whereas approximately 20% of nonsyndromic HL is inherited as autosomal dominant and is usually of delayed onset.[5],[11] Although the frequency of causative genes varies across different populations and ethnicities, the most frequent genetic cause of severe-to-profound autosomal recessive nonsyndromic HL is mutation in the GJB2;[11],[16] other genes that are common causes of HL include GJB6, SLC26A4, and OTOF.[5]

Other congenital causes of HL include malformations of the middle ear which can range from the altered configuration and size of the tympanic cavity to variation in the number, size, and configuration of ossicles. Anomalies of the round window and, more rarely, of the oval window may still occur.[17] The most common malformation is isolated ossicular deformity involving the stapes superstructure and the long apophysis of the incus, and the most common congenital ossicular fixation is stapes fixation, also known as otosclerosis.[18] Raveh et al.[19] presented the results of exploratory tympanotomy performed at a large pediatrics otolaryngology center in patients with nonserous congenital CHL, and they found that 42 children had malformation of one or more ossicles without fixation of the stapes and 19 children had fixed stapes. Other congenital malformations of the middle ear that can cause HL have been summarized by Teunissen and Cremers[20] basing on the surgical approach, dividing them into the following four main groups: isolated stapes ankylosis, stapes ankylosis associated with other ossicular malformations, deformity of the ossicular chain with mobile stapes footplate, and severe aplasia or dysplasia of oval or round windows.

Dysplasias have been attributed to be among the causes of HL in children. In one of the largest studies of hearing health in skeletal dysplasia patients, Tunkel et al.[21] found HL in over one-fourth of children with skeletal dysplasias. Children with skeletal dysplasias were more likely to have abnormal tympanometry, reflecting the greater likelihood of middle ear disease. Another dysplastic condition is Mondini dysplasia, an inner ear abnormality characterized by the development of an incomplete cochlea due to helical cavitation of otic mesenchyme.[22] It is thought to be due to the arrest of neural tube development during the 7th week of gestational age.[23] Arellano et al.[22] reported on a series of cases with HL secondary to Mondini dysplasia. Similarly, Lin et al.[23] reported HL in a 10-month-old girl with Mondini dysplasia.


When an organism's body is invaded by a disease-causing microorganism, it ultimately responds to such invasion, and this is termed as an infection. Infection may be caused by viruses, bacteria, fungi, and/or parasites. There is a well-established association between some congenital infections and HL.[2],[24],[25],[26] The most frequent infectious agents associated with HL are Cytomegalovirus (CMV), rubella virus, Toxoplasma gondii, and the herpes virus and of recent, HIV has also been included in this risk group.[14] HIV infection can lead to CHL through bacterial and fungal infections, secondary to immunosuppression.[27]

Bacterial meningitis, particularly from Streptococcus pneumoniae, has been reported to be one of the most common postnatal causes of HL in children.[24],[28] Meningogenic labyrinthitis is most frequently due to bacterial meningitis and is usually bilateral. The offending pathogens are believed to invade the membranous labyrinth through the cochlear aqueducts or the lamina cribrosa of the vestibule, resulting in suppurative labyrinthitis, where the organisms gain access into the inner ear through the round window, the oval window, or via an anomalous connection between the middle and inner ear. In a study from Pakistan,[29] the frequency of HL was found to be at 22% following an episode of acute bacterial meningitis. A study from England by Fortnum and Davis[30] indicated that 7.4% of the children who had suffered bacterial meningitis had some degree of SNHL or mixed HL as a direct consequence of the disease. The impairments ranged from mild unilateral to profound bilateral and the affected children were aged between 0 (i.e., infection at birth) and 15 years, and hence, they concluded that bacterial meningitis of any type can result in sensorineural hearing impairment of any degree in a child of any age. In another study, Richardson et al.[24] concluded that SNHL developed during the earliest stages of meningitis. Permanent deafness was rare, but 10% of the patients had a rapidly reversible cochlear dysfunction which may have progressed to permanent deafness if the patients had not been treated promptly.

Congenital syphilis (CS), a condition caused by transplacental transmission of Treponema pallidum, is initially characterized by meningoneuritis and labyrinthitis and progresses to interstitial keratitis, SNHL, and peg teeth (Hutchinson's triad).[31] Eight-nerve deafness occurs in about 3% of CS cases and is secondary to luetic involvement of the temporal bone. Eight-nerve involvement can be unilateral or bilateral, and it often occurs in the first decade of life.[32],[33] Pessoa and Galvão[34] reported on the case of HL in a 7-year-old girl who was diagnosed with CS when she was 2 years old. In another case report, Marfatia et al.[33] reported HL in a 14-year-old girl.

Of the viral infections that are linked with HL, CMV infection has been extensively studied. CMV infection is omnipresent in the general population and rarely produces symptoms in the immunocompetent infant, child, or adult. CMV-induced illness may, in contrast, be serious in individuals with impaired immune systems, including HIV-infected individuals, solid organ and hematopoietic transplant patients, and infants infected in utero.[35] CMV infection has an estimated overall birth prevalence of approximately 0.3%–2.4% and approximately 10% of infected infants are born with the clinical symptoms of congenital CMV infection.[36],[37] HL associated with symptomatic CMV infection is often progressive in about 50% of patients and ultimately becomes severe to profound in the affected ear in 78% of patients.[36] The association between congenital CMV infection and HL, especially SNHL, has been known for decades, although the mechanism by which the virus causes hearing impairment in some children and not others is still not fully understood.[38] Furutate et al.[37] collected preserved dried umbilical cords from 134 children with bilateral (46 children) or unilateral (88 children) SNHL and extracted CMV DNA which was detected by quantitative polymerase chain reaction. They found that CMV DNA from the dried umbilical cords was detected in 8.7% of the bilateral SNHL and 9.1% of unilateral SNHL. In another study, Fowler and Boppana[38] established that congenital CMV infection significantly contributes to SNHL in many infant populations. Although most children with congenital CMV infection do not develop HL, it is difficult to predict which children with congenital CMV infection will develop HL and among those who do develop loss, whether or not the loss will continue to deteriorate. Similarly, a retrospective analysis of the etiology of SNHL by Ogawa et al.[16] demonstrated that congenital CMV infection is responsible for a substantial proportion of early-childhood SNHL.

Herpes simplex virus (HSV) exposure is often quoted as a risk factor for the development of SNHL in a newborn child.[39] HSVs are large, enveloped viruses containing DNA and are of two major types. Type 1 (HSV) usually involves the face and skin above the waist. Type 2 (HSV) usually involves the genitalia and skin below the waist in adults.[40] Reports of seroconversion of HSV antibody titers in idiopathic sudden SNHL and the finding of HSV-specific DNA in human spiral and vestibular ganglia of asymptomatic individuals support the role of herpes viruses in the pathogenesis of HL.[41] HSV is most frequently transmitted to an infant during passage through an infected maternal lower genital tract during birth.[40] In their study, Al Muhaimeed and Zakzouk[40] reported that 46 of the 82 infected children (56%) with HSV were found to have bilateral SNHL. According to Cohen et al.,[27] the degree of HL caused by viruses varies depending on the type of virus. CMV causes severe HL, Rubella virus causes mild-to-moderate HL, and HSV causes moderate-to-profound HL.

HL has been reported in about 20% of the congenital toxoplasmosis cases, especially in untreated children or those treated for a very short period.[14] Toxoplasmosis is a systemic infection caused by the protozoan parasite T. ondii, and congenital toxoplasmosis is caused by vertical transmission from the mother to the fetus.[42] Salviz et al.[43] postulated that the possible pathophysiology of the HL in congenital toxoplasmosis is due to postnatal inflammatory response to the tachyzoite form of T. ondii found in the internal auditory canal and in the temporal bone, specifically in the spiral ligament, the stria vascularis, and the saccular macula or the internal auditory canal. In a study from Brazil, it was observed that congenital toxoplasmosis was a risk factor for HL.[14] On the contrary, Austeng et al.[44] found no association between maternal T. ondii infection in pregnancy and subsequent HL in the offspring. Hence, they concluded that T. ondii infection in pregnancy was not an important cause of childhood HL. In addition, in another study by Leite Filho et al.,[45] it was observed that children exposed to toxoplasmosis during pregnancy did not differ from nonexposed children in relation to the occurrence of HL.

A spectrum of inflammatory disorders affects the middle ear cavity, with acute otitis media and otitis media with effusion (OME) being the most prevalent in children.[46],[47] Otitis media has multifactorial etiological factors including adenoids hypertrophy, infection (viral or bacteria), allergy, and environmental and social factors.[47] OME frequently leads to CHL as a result of reduced air pressure in the middle ear, fluid retained in the middle ear cavity, increased stiffness and mass of the tympanum, pathological changes in the tympanic membrane or ossicles such as destruction of the ossicles, or fibrosis and cholesteatoma in the middle ear.[48],[49],[50] Abdullah et al.[47] assessed fifty ears of children who had otitis media and found that 24 ears had moderate CHL, 16 ears had mild HL, while 4 ears had severe HL. In a study that was carried out among Saudi preschoolchildren,[51] acute OME was the major cause of deafness, followed by chronic otitis media. Anggraeni et al.[50] investigated on otitis media-associated HL in schoolchildren in Indonesia and found that otitis media contributed to 57% of mild-to-moderate HL cases and 79% of bilateral HL.


Of the several etiologies of HL in children, head trauma is cited to be among the acquired causes, regardless of its severity.[52],[53] Damage to the peripheral and/or central auditory pathways can occur as a primary or secondary insult after closed head injury. Primary audiological deficits after a head injury can appear as a result of direct trauma to the middle and inner ear (e.g., in fractures of the base of the skull) or due to rupture or tearing of central neuronal pathways.[53] Early secondary impairment can result from raised intracranial pressure due to bleeding and hematoma and later in the course because of diffuse axonal degeneration.[53],[54] Experimental studies have shown electrophysiological and histopathological changes in the inner ear following head injury, and the postmortem histopathologic studies following head injury revealed changes in the internal auditory canal, the tissues of the inner ear, the vestibulocochlear nerve, and the brainstem.[54]

Temporal bone fractures constitute a significant bulk of basilar skull fractures, which, in turn, constitute the most common skull fractures following head injury,[55] and fracture of the temporal bone can cause SNHL and/or CHL.[12] Up to 82% of children with temporal bone fractures have HL at presentation; of these cases, 67% will be CHL, 21% will be SNHL, and 12% will be mixed.[28] Zimmerman et al.[56] studied fifty children who had sustained head injuries and found that the incidence of CHL was 32% against 16% for SNHL. Cockrell and Gregory[57] performed audiological evaluation in 62 children who had suffered from traumatic brain injuries and found that 16% of the children had CHL, 13% had SNHL, and 16% had central auditory processing problems.

Perforation of the tympanic membrane following trauma is another cause of HL. In their study, Olowookere et al.[58] found that trauma to the ear accounted for only 3% of the cases of tympanic membrane perforation. Tympanic membrane perforation reduces the total ratio of the surface area, allowing the sound waves to directly pass through the middle ear.[59] Tympanic membrane perforation leads to a varying degree of CHL[60] and the size of perforation may define the severity of HL.[59] Traumatic membrane perforation can be due to direct trauma, acoustic trauma, barotrauma, and iatrogenic causes[60],[61] such as unskilled instrumentation or syringing of the ear, sudden air compression as in boxing, hand-slap, and blasts.[58] Another sequela of trauma can be rupture of the round or oval window membranes.[12]


Neoplasms/tumors can cause HL through one or more of the following mechanisms: direct compression of the cochlear nerve by the tumor; occlusion or vascular compression of the internal auditory artery; intratumor bleeding; internal auditory canal occlusion; and toxic substance produced by the tumor that causes degeneration of the inner ear.[62],[63] These tumors include schwannomas[64],[65] and tumors of neuroepithelial origin such as pylocytic astrocytomas (PAs) and medulloblastomas (MBs).[63],[66],[67] Schwannomas represent 0.8% of all childhood tumors and 2.08% of all unilateral acoustic neuromas, and occasionally, early deafness in childhood due to schwannoma may pass unnoticed because it often causes unilateral HL.[68] Some authors have described that vestibular schwannomas can present with HL, particularly in individuals with neurofibromatosis type 2.[28] HL secondary to schwannoma has been reported by Thomas et al.[64] and Massinger et al.[69] in a 10-year-old boy and 12-year-old girl, respectively.

PA accounts for 5%–10% of all gliomas and is the second most common pediatric brain tumor.[63],[66] Although rarely do PA cause HL, these tumors can produce changes in the blood supply to the auditory nerve, destroy cochlear nerve fibers, and disturb the inner ear fluids with biochemical changes, all of which can result in a HL.[63] These tumors commonly occur around the third and fourth ventricles, in optic chiasm and hypothalamus, however cases in which PA arise from the VIII nerve complex have been reported by Mirone et al.[66] On the other hand, Schneider et al.[67] reported a case of PA in the left cerebellopontine angle that caused HL due to stretching or compression of the auditory nerve and mechanical stress on the nerve blood supply.

Another neoplastic condition that is attributed to cause HL in children is MB. MB is said to be a highly malignant neuroepithelial tumor of the posterior fossa that accounts for 10% of all intracranial neoplasms and 29% of all pediatric fossa tumors in children.[70] HL because of MB in children has been well documented in literature.[70],[71] Other tumors that have been documented to be the cause of HL in children, though rare, include middle ear lipomas,[72],[73] middle ear lymphoma,[74] congenital cholesteatoma,[75] and aneurysmal bone cyst of the temporal bone.[76]


McCabe had proposed the existence of autoimmune SNHL (ASNHL) about 40 years ago, and ever since, evidence has confirmed the existence of autoimmune-mediated inner ear disease (AIED), a potentially treatable cause of SNHL.[77],[78] ASNHL is characterized by bilateral disease, often with the severity of HL being asymmetric.[79] AIED is defined as primary when the pathology is restricted to the ear, however, in up to a third of cases, AIED occurs in the context of systemic autoimmune disease and is defined as secondary.[80] Some of the identified autoimmune diseases affecting hearing include systemic lupus erythematosus, rheumatoid arthritis, myasthenia gravis, ASNHL, and Hashimoto's thyroiditis.[81]

For many years, it has been known that the inner ear communicates with the systemic immune system and can rapidly mount an immune response against pathogens and foreign proteins to which it has been sensitized.[82] Although the inner ear is believed to be an immune-privileged site protected by the blood–labyrinthine barrier, cochlear innate immunity has been proposed to contribute to the initiation of an adaptive immune response in the cochlea by promoting a response to antigen challenge.[81],[83] A number of inner ear antigens have been proposed as targets of antibodies due to an autoimmune response, including type 2 and type 9 collagen, beta-actin, cochlin, and beta-tectorin.[84] Baek et al.[85] attributed the etiopathogenesis of ASNHL to the cochlin-specific interferon gamma (IFN-γ)-producing T-cells. Cochlin is among the most abundant protein of the inner ear, thus it serves as a prominent candidate antigen for targeting inner ear inflammation and autoimmune-mediated HL. Baek et al.[85] found that cochlin autoreactivity was significantly enhanced in patients with ASNHL. This cochlin responsiveness involves IFN-γ-producing CD4+ and CD8+ T cells as well as elevated cochlin-specific serum antibodies. In another study, Nair et al.[86] reported on antibodies against choline transporter-like protein 2 (CTL2), an inner ear glycoprotein. They described that sensory cells die after binding of an antibody to CTL2 molecules. Their postulation was that antibody acts by blocking the transporter function of the molecule either by blocking the substrate receptor of the carbohydrate or by causing steric hindrance in the transporter pore and in either case, leads to antibody-mediated HL.

Although autoimmune inner ear disease most commonly affects people in the third to sixth decades of life,[80] cases of autoimmune-mediated HL in children have been well documented in literature. Huang and Sataloff[77] reported cases of seven children with HL secondary to AIED, and Marsili et al.[87] reported a case of HL in a 15-year-old boy diagnosed with AIED.

Ototoxic medications and substances

Ototoxicity has been described as a functional impairment and cellular degeneration of the tissues of the inner ear caused by therapeutic agents.[88] The hearing impairment in children caused by ototoxic drugs is usually bilateral, is symmetric, and of variable severity.[25] Some of the identified ototoxic drugs include aminoglycoside antibiotics, platinum-based chemotherapeutic agents (cisplatin and carboplatin), loop diuretics, macrolide antibiotics, and antimalarials.[88],[89]

Children may be exposed to ototoxic drugs/substances prenatally or postnatally. It has been well documented in literature that ingestion of ototoxic drugs/substances by pregnant women can result in HL in the offspring.[90],[91],[92] The period of gestation when the use of ototoxic medications/substances is most likely to cause damage to the auditory system is in the first trimester, especially in the 6th and 7th weeks.[25] Among these substances is alcohol. Alcohol by itself is a toxin to the fetus, as is its major metabolite, acetaldehyde. Alcohol can cause a direct effect on cells and can also cause damage due to hypoxia and disturbances in prostaglandin physiology.[90] For example, a meta-analysis aimed at identifying the comorbid conditions that co-occur in individuals with fetal alcohol syndrome Disorder, deduced that the pooled prevalence of SNHL and CHL was estimated to be up to 129 times higher in individuals with FAS than the prevalence of moderate-to-severe HL in the general population.[93]

The use of ototoxic drugs during pregnancy may as well lead to HL in children. In one case report, severe bilateral perceptive HL in a neonate was diagnosed, after the mother was treated for cervical cancer with cisplatin and paclitaxel from 26th to 34th weeks of pregnancy.[94] In a case–control study that evaluated the risk of HL in children due to in utero exposure to drugs, it was observed that HL was associated with valproic acid and low-dose acetylsalicylic acid exposure during pregnancy.[95] Aminoglycosides are said to cross the blood–placenta barrier, and animal studies have shown them to cause HL in children. Czeizel et al.[91] studied the teratological effect of aminoglycoside antibiotic treatment during pregnancy and concluded that the risk of deafness in children cannot be excluded, but the magnitude is estimated to be small.

Postnatally, treatment-induced HL has been documented. Many therapeutics can be classified as “ototoxic,” despite that, such drugs remain in current use, either because the risk of damage to the ear is small, or because there simply are no comparable alternatives.[96] Aminoglycosides are among the most commonly used antibiotics for empirical treatment of neonatal sepsis or to treat febrile neutropenia or Gram-negative infections; however, their ototoxicity depends on the total dose administered, the duration of treatment, as well as serum concentrations.[97],[98] Although they are dose dependent, there is no clear dose or duration threshold at which the risk of HL increases significantly, as some children do not develop noticeable HL. Despite high exposure to aminoglycosides, there are also reports of irreversible, profound HL after very low exposures.[97]

Platinum-based chemotherapeutical agents are important alkylating agents that are effective in the management of childhood cancers.[99] Unfortunately, these drugs commonly cause SNHL that is progressive, bilateral, and irreversible.[100] The incidence of HL in patients receiving platinum-based chemotherapy varies widely in the literature.[101] Bertolini et al.[102] observed that deterioration of hearing of Grade 2 or above was in 37% of children treated with cisplatin and 43% of patients treated with cisplatin plus carboplatin, and had concluded that carboplatin, at a standard dose, does not appear to be a significant risk factor for ototoxicity even in patients who have already been treated with cisplatin. Cisplatin which works by the disruption of DNA replication and repair can cross the blood–labyrinth barrier to gain access to cochlear tissues.[100] Subsequently, it generates reactive oxygen species that induce mitochondrial damage, and apoptosis of the cochlear outer hair cells depletes natural antioxidants such as glutathione and damages the stria vascularis and reparative stem cells of the inner ear.[100],[103]

Other causes

There are several other causes of HL in children. Hospitalization for more than 5 days in neonatal intensive care unit is reported to increase HL incidence by about 5–10 times if compared with that in the general newborn population.[104],[105] In addition, several syndromes have been described to be associated with inner ear malformation.[28] A study by Mehta et al.[106] reported that of the individuals with syndromic manifestations, Usher and Waardenburg syndromes were most commonly observed.

Diagnosing hearing loss in children

The diagnosis of HL in children may be done at any stage of their development, and the type may be dictated by the cause of HL. In newborns and infants, HL can be screened using different methods. For diagnostic purposes, there are several investigations that can be carried out such as audiometry, genetic tests, imaging studies, otoscopy, and tympanometry.

Screening newborns and infants for hearing loss

Accurate diagnosis of HL in infants is challenging, and an attempt of doing so had led to the establishment of the Universal Newborn Hearing Screening and Intervention (UNHSI) program, which has been widely adopted throughout North America, Europe, and in most other developed regions, primarily as a result of technological advances in screening and intervention modalities.[107] In the UNHSI program, diagnosis of hearing disorders in newborns and infants is generally a two-stage process that entails measuring otoacoustic emissions (OAE) or performing automated auditory brainstem response (A-ABR) audiometry, or both.[108] OAEs are forms of energy, measured as sound, generated by the outer hair cells of the human cochlea, in response to received auditory input, while A-ABR test records brainstem electrical activity in response to sounds presented to the infant via earphones.[107]

In two-stage screening, OAE measurement is followed by A-ABR audiometry.[108] In this system, if an infant passes the OAE, no additional testing is done, but when fails the OAE, he/she is next screened with A-ABR. Infants who fail the A-ABR screening are referred for diagnostic testing to determine whether they have permanent HL (PHL) or not.[109] A weakness of OAE measurement is that it does not detect fluctuating hearing impairments or those due to auditory neuropathy.[108] Wolff et al.[110] did a systematic review of the literature to assess the accuracy, effectiveness, and effects of interventions after screening and found that eight diagnostic studies comparing OAE with ABR showed sensitivity which varied between 50% and 100% and the specificity from 49.1% to 97.2%. In another study, Johnson et al.[109] concluded that if all infants were screened for HL using the two-stage OAE/A-ABR system, then approximately 23% of those with PHL at approximately 9 months of age would have passed the A-ABR. Despite some limitations of this screening program, there is strong evidence indicating that two-step screening is highly effective in identifying infants with HL.[107]

Genetic tests

Genetic screening is defined as the analysis of human DNA in order to detect heritable-related mutations.[111] The advancements in the molecular genetics of hearing impairment have demonstrated that more than 50% of children with SNHL have attributable genetic factors, thus genetic testing may be considered to be a powerful tool for addressing hearing impairment in children.[5],[112] Abnormalities in many different single genes or gene pairs can cause deafness, with some studies reporting that defects in a single gene, called GJB2, encode for connexin 26 being responsible for more than half of the genetic causes of HL.[113],[114] Mutations in the gene coding for connexin 26, account for about half of the autosomal recessive cases, therefore particularly important as a cause of genetic childhood HL.[114] Establishing the etiology of HL through genetic testing eliminates further expenditure in diagnostic health-care costs and also it provides invaluable information that can guide medical management and intervention of babies with HL.[115] Regarding the genetic testing, it is recommended that it may be guided by the case history, phenotype, physical examination, audiometry, and the relative prevalence of a gene in a clinical population.[113],[116]

Imaging studies

Computed tomography (CT) and magnetic resonance imaging (MRI) have become an essential part of the evaluation of pediatric SNHL, with anomalies being found in up to 40% of patients, ranging from major anatomic abnormalities to subtle dysplasia.[117] These imaging modalities are helpful in identifying the potential etiology for HL, to define the anatomy of the temporal bone and the central auditory pathway, to identify abnormalities that may predict HL progression or prognosis, and to identify additional intracranial abnormalities that may require further workup and/or intervention.[118] Regarding which imaging modality is best, some physicians are proponents of MRI over CT, while others note that CT may be superior yet, some other both. Other clinicians decide according to age, severity, or laterality of a given patient's presentation. However, better understanding of the relative and diagnosis-specific potential yields of each imaging modality allows sophistication with which these images may be ordered.[119]


Audiometry, which is performed using an audiometer, is the science of measuring hearing acuity and variations in a sound and is considered to be a comprehensive baseline ear health screening tool.[120],[121] There are several techniques of performing audiometry, including pure tone audiometry (PTA), speech audiometry, visual reinforcement audiometry (VRA), and play audiometry (PA).

The PTA assesses the type (CHL, SNHL, and mixed) and degree of HL.[53],[120] PTA consists of air-conduction and bone-conduction tests. The entire range of human hearing is from 20 to 20,000 Hz. Air-conduction hearing thresholds are measured for tonal stimuli at the range of frequencies from 0.125 to 8 kHz with the use of headphones and the patient gets a pure tone of one frequency. Then, bone-conduction hearing thresholds are measured for tonal stimuli at the range of frequencies from 0.25 to 4 kHz, with the use of a headband with oscillator.[120],[122] In the CHL, air-conduction thresholds worsen, so the air-conduction curve is shifted down, whereas bone-conduction thresholds remain unchanged.[122] According to the International Classification of HL, to calculate the degree of HL, it is necessary to summarize the four values, i.e., the lowest audible sound intensity using the frequencies of 500, 1000, 2000, and 4000 Hz, and then to divide the sum by 4 to get the arithmetic average.[120]

Speech audiometry assesses auditory discrimination as opposed to auditory acuity, requiring the individual to repeat standard word lists delivered through headphones at varying intensities, which, therefore, provides information on word recognition and about discomfort or tolerance to speech stimuli.[53],[123],[124] Speech audiometry is performed to obtain the speech recognition (reception) threshold (SRT) or speech detection (awareness) thresholds (SDTs) using spondee words and supra-threshold speech recognition. The SRT measures the lowest dBHL at which 50% of the time spondee can be correctly repeated or identified, whereas, SDT assesses the lowest dBHL at which the presence of speech can be correctly detected.[125] It is recommended to rule out ear diseases if unilateral or asymmetrically poor speech discrimination scores (a difference >15% between ears) or a bilateral speech discrimination scores of < 80% are observed.[125] The setback of speech audiometry lies in that there are certain measures that can be affected by language differences; thus, an individual's ability to hear discriminating differences and repeat certain words can be affected.[126]

VRA is routinely used to evaluate hearing in infants and children aged 6 months to 2 years.[127] It capitalizes on a child's natural instinct to respond to auditory stimuli by turning toward the stimuli.[128] A limitation of VRA is that the head turn response can extinguish or habituate before a full hearing assessment can be completed as it is an operant-conditioned response. That is, the desired behavior is rewarded, thus increasing the likelihood that the behavior will continue.[127],[128]

Treatment of hearing loss in children

The management of children who have HL requires a multidisciplinary team-based approach and should encompass counseling of the parents as well. The overall objectives of management are to minimize the effect that the HL will have on speech and language development and to provide appropriate strategies for communication.[13] The initial approach of managing HL should be to treat the underlying etiology. The treatment modalities of HL may be medical (antimicrobial therapies and anti-inflammatory therapies), surgical treatment (e.g., tumor excision and stapedectomy), and amplification of sound.[129]

Medical treatment entails the use of steroids, antivirals, and antibiotics. In a study by Övet et al.,[130] it was reported that significant hearing improvement may be obtained with the use of systemic steroids alone in pediatric patients with sudden SNHL. Antivirals have been used in cases of HL caused by viral infection. Ganciclovir (administered intravenously) is the treatment for both early and delayed SNHL resulting from congenital CMV infection. It prevents SNHL progression and sometimes can improve hearing status.[27] Acyclovir has been used to treat HL caused by HSV-1.[27],[41]

In the cases of HL during bacterial meningitis, parental penicillin has been shown to be effective.[24] In cases of otitis media in children, antibiotics should be considered if symptoms persist or do not improve within 4 days, or in those younger than 2 years with bilateral acute otitis media, or who have a tympanic membrane perforation.[131]

The surgical treatment of HL can be grouped into reparative procedures (in case of managing infectious or traumatic causes) and restorative procedures if HL cannot be treated with conventional amplification.[129] Surgical treatment is considered to be beneficial in cases where there is an air–bone gap that is amenable to correction by surgical intervention.[11]

Restoration of hearing is achieved by using hearing devices which are either implantable or nonimplantable. These devices include conventional air-conduction hearing aids, bone-anchored hearing aids (BAHAs), and cochlear implants.[11],[132] The conventional air-conduction hearing aids are incorporated with a microphone that converts sounds into electrical signals, returned to the ear as amplified sound. They are useful for patients with mild-to-severe HL.[132]

BAHA devices incorporate a titanium plate that is surgically anchored to the skull on the hearing-impaired side, to directly stimulate the inner ear by conducting sound vibrations through the bone.[132] Some of the indications for BAHA include severe CHL, congenital ear canal atresia, SNHL, and difficulty wearing a conventional air-conduction aid with an ear mold, due to recurrent ear infections.[132],[133]

The cochlear implant is a biomedical device that is surgically placed into the cochlea. It converts sound to an electrical signal, which is then conducted to the spiral ganglion cells in the cochlea via electrodes.[134] This conduction ultimately produces an auditory sensation to the individual that allows the detection of sounds, especially speech sounds.[135]

  Conclusion Top

HL in children is among the most prevalent health-related issue globally. There is a vast array of etiological factors of HL in children. HL in children affects both the child and his/her parents/caretakers. The effect on the child is in terms of speech and language development, communication, literacy, and learning, whereas, the parents/caretakers usually are often at greater risk of stress and have increased cost in terms of seeking for their children's well-being. It is the duty of a physician to take a thorough history of the children who present with HL, followed by ordering appropriate investigations to establish the cause of HL. The management of children who have HL requires a multidisciplinary team-based approach and should encompass counseling of the parents as well.

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Conflicts of interest

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  References Top

Dobie RA, Van Hemel S, editors. Hearing Loss: Determining Eligibility for Social Security Benefits. Washington (DC): National Academic Press; 2004.  Back to cited text no. 1
Cristobal R, Oghalai JS. Hearing loss in children with very low birth weight: current review of epidemiology and pathophysiology. Arch Dis Child Fetal Neonatal Ed 2008;93:F462-8.  Back to cited text no. 2
Jasper KM, Jamshidi A, Reilly BK. Pediatric otolaryngology, molecular diagnosis of hereditary hearing loss: Next-generation sequencing approach. Curr Opin Otolaryngol Head Neck Surg 2015;23:480-4.  Back to cited text no. 3
World Health Organization. Prevention of Blindness and Deafness: Estimates. Geneva: World Health Organization. Available from: https://www.who.int/pbd/deafness/estimates/en/. [Last accessed on 2019 Oct 24].  Back to cited text no. 4
Egilmez OK, Kalcioglu MT. Genetics of Nonsyndromic Congenital Hearing Loss. Scientifica (Cairo) 2016;2016:7576064.  Back to cited text no. 5
Krug E, Cieza A, Chadha S, Sminkey L. Childhood Hearing Strategies for Prevention and Care. Geneva: World Health Organization; 2016.  Back to cited text no. 6
Chris H, Sue A, Tony S, Soumit D. Management of the hearing impaired child. In: Watkinson J, Clarke R, editors. Scott-Brown's Otorhinolaryngology Head and Neck Surgery. 8th ed. New York: CRC Press; 2019. p. 75-90.  Back to cited text no. 7
Brownell WE. How the ear works – Nature's solutions for Listening. Volta Rev 1997;99:9-28.  Back to cited text no. 8
Oghalai JS. The cochlear amplifier: Augmentation of the traveling wave within the inner ear. Curr Opin Otolaryngol Head Neck Surg 2004;12:431-8.  Back to cited text no. 9
Fettiplace R. Hair cell transduction, tuning, and synaptic transmission in the mammalian cochlea. Compr Physiol 2017;7:1197-227.  Back to cited text no. 10
Korver AM, Smith RJ, Van Camp G, Schleiss MR, Bitner-Glindzicz MA, Lustig LR, et al. Congenital hearing loss. Nat Rev Dis Primers 2017;3:16094.  Back to cited text no. 11
Isaacson JE, Vora NM. Differential diagnosis and treatment of hearing loss. Am Fam Physician 2003;68:1125-32.  Back to cited text no. 12
Grindle CR. Paediatric hearing loss. Pediatr Rev 2014;35:456.  Back to cited text no. 13
Andrade GM, Resende LM, Goulart EM, Siqueira AL, Vitor RW, Januario JN. Hearing loss in congenital toxoplasmosis detected by newborn screening. Braz J Otorhinolaryngol 2008;74:21-8.  Back to cited text no. 14
Crowson MG, Hertzano R, Tucci DL. Emerging therapies for sensorineural Hearing loss. Otol Neurotol 2017;38:792-803.  Back to cited text no. 15
Ogawa H, Suzutani T, Baba Y, Koyano S, Nozawa N, Ishibashi K, et al. Etiology of severe sensorineural hearing loss in children: Independent impact of congenital cytomegalovirus infection and GJB2 mutations. J Infect Dis 2007;195:782-8.  Back to cited text no. 16
Esteves SD, Silva AP, Coutinho MB, Abrunhosa JM, Almeida e Sousa C. Congenital defects of the middle ear—uncommon cause of pediatric hearing loss. Braz J Otorhinolaryngol 2014;80:251-6.  Back to cited text no. 17
Djalilian HR. Symptom: Congenital conductive hearing loss. Hear J 2014;67:10-2.  Back to cited text no. 18
Raveh E, Hu W, Papsin BC, Forte V. Congenital conductive hearing loss. J Laryngol Otol 2002;116:92-6.  Back to cited text no. 19
Teunissen EB, Cremers WR. Classification of congenital middle ear anomalies. Report on 144 ears. Ann Otol Rhinol Laryngol 1993;102:606-12.  Back to cited text no. 20
Tunkel D, Alade Y, Kerbavaz R, Smith B, Rose-Hardison D, Hoover-Fong J. Hearing loss in skeletal dysplasia patients. Am J Med Genet A 2012;158A: 1551-5.  Back to cited text no. 21
Arellano B, Ramírez Camacho R, García Berrocal JR, Villamar M, del Castillo I, Moreno F. Sensorineural hearing loss and Mondini dysplasia caused by a deletion at locus DFN3. Arch Otolaryngol Head Neck Surg 2000;126:1065-9.  Back to cited text no. 22
Lin CY, Lin HC, Peng CC, Lee KS, Chiu NC. Mondini dysplasia presenting as otorrhea without meningitis. Pediatr Neonatol 2012;53:371-3.  Back to cited text no. 23
Richardson MP, Reid A, Tarlow MJ, Rudd PT. Hearing loss during bacterial meningitis. Arch Dis Child 1997;76:134-8.  Back to cited text no. 24
Roizen NJ. Nongenetic causes of hearing loss. Ment Retard Dev Disabil Res Rev 2003;9:120-7.  Back to cited text no. 25
Fortnum HM. Hearing impairment after bacterial meningitis: A review. Arch Dis Child 1992;67:1128-33.  Back to cited text no. 26
Cohen BE, Durstenfeld A, Roehm PC. Viral causes of hearing loss: A review for hearing health professionals. Trends Hear 2014;18. pii: 2331216514541361.  Back to cited text no. 27
Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 2: Syndromic and acquired causes. AJNR Am J Neuroradiol 2012;33:399-406.  Back to cited text no. 28
Zeeshan F, Bari A, Dugal MN, Saeed F. Hearing impairment after acute bacterial meningitis in children. Pak J Med Sci 2018;34:655-9.  Back to cited text no. 29
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. 30
Hone SW, Orl F, Smith RJ. Medical evaluation of pediatric hearing loss and genetic testing. Science 2002;35:751-64.  Back to cited text no. 31
De Santis M, De Luca C, Mappa I, Spagnuolo T, Licameli A, Straface G, et al. Syphilis Infection during pregnancy: Fetal risks and clinical management. Infect Dis Obstet Gynecol 2012;2012:430585.  Back to cited text no. 32
Marfatia Y, Singhal P, Patel P. A case of congenital syphilis with Hutchinson's triad. Indian J Sex Transm Dis AIDS 2011;32:34.  Back to cited text no. 33
Pessoa L, Galvão V. Clinical aspects of congenital syphilis with Hutchinson's triad. BMJ Case Rep 2011;2011. pii: bcr1120115130.  Back to cited text no. 34
Schleiss MR. Congenital cytomegalovirus infection: Update on management strategies. Curr Treat Options Neurol 2008;10:186-92.  Back to cited text no. 35
Suganuma E, Oka A, Sakata H, Adachi N, Asanuma S, Oguma E, et al. 10-year follow-up of congenital cytomegalovirus infection complicated with severe neurological findings in infancy: A case report. BMC Pediatr 2018;18:369.  Back to cited text no. 36
Furutate S, Iwasaki S, Nishio SY, Moteki H, Usami S. Clinical profile of hearing loss in children with congenital cytomegalovirus (CMV) infection: CMV DNA diagnosis using preserved umbilical cord. Acta Otolaryngol 2011;131:976-82.  Back to cited text no. 37
Fowler KB, Boppana SB. Congenital cytomegalovirus (CMV) infection and hearing deficit. J Clin Virol 2006;35:226-31.  Back to cited text no. 38
Westerberg BD, Atashband S, Kozak FK. A systematic review of the incidence of sensorineural hearing loss in neonates exposed to Herpes simplex virus (HSV). Int J Pediatr Otorhinolaryngol 2008;72:931-7.  Back to cited text no. 39
al Muhaimeed H, Zakzouk SM. Hearing loss and herpes simplex. J Trop Pediatr 1997;43:20-4.  Back to cited text no. 40
Rabinstein A, Jerry J, Saraf-Lavi E, Sklar E, Bradley WG. Sudden sensorineural hearing loss associated with herpes simplex virus type 1 infection. Neurology 2001;56:571-2.  Back to cited text no. 41
Corrêa CC, Maximino LP, Weber SA. Hearing disorders in congenital toxoplasmosis: A literature review. Int Arch Otorhinolaryngol 2018;22:330-3.  Back to cited text no. 42
Salviz M, Montoya JG, Nadol JB, Santos F. Otopathology in congenital toxoplasmosis. Otol Neurotol 2013;34:1165-9.  Back to cited text no. 43
Austeng ME, Eskild A, Jacobsen M, Jenum PA, Whitelaw A, Engdahl B. Maternal infection with Toxoplasma gondii in pregnancy and the risk of hearing loss in the offspring. Int J Audiol 2010;49:65-8.  Back to cited text no. 44
Leite Filho CA, Lagreca LCC, Jesus NO de, Corvaro CP, Ferrarini MAG, Monteiro AIMP, et al. Hearing loss in children exposed to toxoplasmosis during their gestation. Rev CEFAC 2017;19:330-9.  Back to cited text no. 45
Cai T, McPherson B. Hearing loss in children with otitis media with effusion: A systematic review. Int J Audiol 2017;56:65-76.  Back to cited text no. 46
Abdullah B, Hassan S, Sidek D. Clinical and audiological profiles in children with chronic otitis media with effusion requiring surgical intervention. Malays J Med Sci 2007;14:22-7.  Back to cited text no. 47
Manche SK, Jangala M, Koralla RM, Akka J. Prevalence of otitis media and its hearing loss in children of South Indian population. Int J Infect Dis 2016;45:334.  Back to cited text no. 48
Cai T, McPherson B, Li C, Yang F. Pure tone hearing profiles in children with otitis media with effusion. Disabil Rehabil 2018;40:1166-75.  Back to cited text no. 49
Anggraeni R, Carosone-Link P, Djelantik B, Setiawan EP, Hartanto WW, Ghanie A, et al. Otitis media related hearing loss in Indonesian school children. Int J Pediatr Otorhinolaryngol 2019;125:44-50.  Back to cited text no. 50
Al-Rowaily MA, AlFayez AI, AlJomiey MS, AlBadr AM, Abolfotouh MA. Hearing impairments among Saudi preschool children. Int J Pediatr Otorhinolaryngol 2012;76:1674-7.  Back to cited text no. 51
Yenigun A. Sudden post-traumatic sensorineural hearing loss reverted to normal by sneezing. SAGE Open Med Case Rep 2014;2:2050313X14564774.  Back to cited text no. 52
Munjal SK, Panda NK, Pathak A. Audiological deficits after closed head injury. J Trauma 2010;68:13-8.  Back to cited text no. 53
Bergemalm PO, Borg E. Long-term objective and subjective audiologic consequences of closed head injury. Acta Otolaryngol 2001;121:724-34.  Back to cited text no. 54
Maradi N, Somanath B. Hearing loss following temporal bone fractures- a study on classification of fractures and the prognosis. Int J Otorhinolaryngol Head Neck Surg. 2017;3:390-4.  Back to cited text no. 55
Zimmerman WD, Ganzel TM, Windmill IM, Nazar GB, Phillips M. Peripheral hearing loss following head trauma in children. Laryngoscope 1993;103:87-91.  Back to cited text no. 56
Cockrell JL, Gregory SA. Audiological deficits in brain-injured children and adolescents. Brain Inj 1992;6:261-6.  Back to cited text no. 57
Olowookere SA, Ibekwe TS, Adeosun AA. Pattern of tympanic membrane perforation in ibadan: A retrospective study. Ann Ib Postgrad Med 2008;6:31-3.  Back to cited text no. 58
Dessai TD, Philip R. Influence of Tympanic Membrane Perforation on Hearing Loss. Glob J Otolaryngol 2017;5:8-11.  Back to cited text no. 59
Pannu KK, Chadha S, Kumar D, Preeti. Evaluation of hearing loss in tympanic membrane perforation. Indian J Otolaryngol Head Neck Surg 2011;63:208-13.  Back to cited text no. 60
Adegbiji WA, Olajide GT, Olajuyin OA, Olatoke F, Nwawolo CC. Pattern of tympanic membrane perforation in a tertiary hospital in Nigeria. Niger J Clin Pract 2018;21:1044-9.  Back to cited text no. 61
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Celis-Aguilar E, Lassaletta L, Torres-Martín M, Rodrigues FY, Nistal M, Castresana JS, et al. The molecular biology of vestibular schwannomas and its association with hearing loss: A review. Genet Res Int 2012;2012:856157.  Back to cited text no. 62
Nachman AJ. Retrocochlear hearing loss in infants: A case study of juvenile pilocytic astrocytoma. Int J Audiol 2012;51:640-4.  Back to cited text no. 63
Thomas JA, Bank WO, Myseros JS. Glossopharyngeal schwannoma in childhood. J Neurosurg Pediatr 2008;2:130-2.  Back to cited text no. 64
Meiteles LZ, Liu JK, Couldwell WT. Hearing restoration after resection of an intracanalicular vestibular schwannoma: A role for emergency surgery? Case report and review of the literature. J Neurosurg 2002;96:796-800.  Back to cited text no. 65
Mirone G, Schiabello L, Chibbaro S, Bouazza S, George B. Pediatric primary pilocytic astrocytoma of the cerebellopontine angle: A case report. Childs Nerv Syst 2009;25:247-51.  Back to cited text no. 66
Schneider F, Kompis M, Ozdoba C, Beck J, Caversaccio M, Senn P. Pilocytic astrocytoma of the cerebellopontine angle in a child presenting with auditory neuropathy spectrum disorder. Otol Neurotol 2015;36:e101-3.  Back to cited text no. 67
Mazzoni A, Dubey SP, Poletti AM, Colombo G. Sporadic acoustic neuroma in pediatric patients. Int J Pediatr Otorhinolaryngol 2007;71:1569-72.  Back to cited text no. 68
Massinger C, Gawehn J, Keilmann A. Acoustic schwannoma with progressive hearing loss in children. A case report. Laryngorhinootologie 2003;82:92-6.  Back to cited text no. 69
Boukobza M, Polivka M. Medulloblastoma of the cerebellopontine angle in a child: A case report and review of the literature. J Mol Biomark Diagn 2016;7:284.  Back to cited text no. 70
Alnaami I, El-Hakim H, Aronyk K, Pugh J, Lu JQ, Mehta V. A pediatric medulloblastoma presenting as an isolated unilateral sensory neural hearing loss. ZUMJ 2016;22:209-13.  Back to cited text no. 71
Selesnick SH, Edelstein DR, Parisier SC. Lipoma of the middle ear: An unusual presentation in a 4-year-old child. Otolaryngol Head Neck Surg 1990;102:82-4.  Back to cited text no. 72
Tachibana T, Nishizaki K, Fujisawa M, Ogawara Y, Matsuyama Y, Abe I, et al. A Case of a Child with a Lipoma of the Middle Ear and a Concomitant Chondroma of the External Auditory Canal. J Case Reports Med. 2013;1:1–4.  Back to cited text no. 73
Lang EE, Walsh RM, Leader M. Primary middle-ear lymphoma in a child. J Laryngol Otol 2003;117:205-7.  Back to cited text no. 74
McGill TJ, Merchant S, Healy GB, Friedman EM. Congenital cholesteatoma of the middle ear in children: A clinical and histopathological report. Laryngoscope 1991;101:606-13.  Back to cited text no. 75
Sawin PD, Muhonen MG, Sato Y, Smith RJ. Aneurysmal bone cyst of the temporal bone presenting as hearing loss in a child. Int J Pediatr Otorhinolaryngol 1995;33:275-84.  Back to cited text no. 76
Huang NC, Sataloff RT. Autoimmune inner ear disease in children. Otol Neurotol 2011;32:213-6.  Back to cited text no. 77
Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum 2004;34:544-8.  Back to cited text no. 78
Buniel MC, Geelan-Hansen K, Weber PC, Tuohy VK. Immunosuppressive therapy for autoimmune inner ear disease. Immunotherapy 2009;1:425-34.  Back to cited text no. 79
Mijovic T, Zeitouni A, Colmegna I. Autoimmune sensorineural hearing loss: The otology-rheumatology interface. Rheumatology (Oxford) 2013;52:780-9.  Back to cited text no. 80
Bovo R, Aimoni C, Martini A. Immune-mediated inner ear disease. Acta Otolaryngol 2006;126:1012-21.  Back to cited text no. 81
Ma C, Billings P, Harris JP, Keithley EM. Characterization of an experimentally induced inner ear immune response. Laryngoscope 2000;110:451-6.  Back to cited text no. 82
Agrup C, Luxon LM. Immune-mediated inner-ear disorders in neuro-otology. Curr Opin Neurol 2006;19:26-32.  Back to cited text no. 83
Greco A, Fusconi M, Gallo A, Marinelli C, Macri GF, De Vincentiis M. Sudden sensorineural hearing loss: an autoimmune disease? Autoimmun Rev 2011;10:756-61.  Back to cited text no. 84
Baek MJ, Park HM, Johnson JM, Altuntas CZ, Jane-Wit D, Jaini R, et al. Increased frequencies of cochlin-specific T cells in patients with autoimmune sensorineural hearing loss. J Immunol 2006;177:4203-10.  Back to cited text no. 85
Nair TS, Kozma KE, Hoefling NL, Kommareddi PK, Ueda Y, Gong TW, et al. Identification and characterization of choline transporter-like protein 2, an inner ear glycoprotein of 68 and 72 kDa that is the target of antibody-induced hearing loss. J Neurosci 2004;24:1772-9.  Back to cited text no. 86
Marsili M, Marzetti V, Lucantoni M, Lapergola G, Gattorno M, Chiarelli F, et al. Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: A case report. Ital J Pediatr 2016;42:81.  Back to cited text no. 87
Rybak LP, Ramkumar V. Ototoxicity. Kidney Int 2007;72:931-5.  Back to cited text no. 88
Robertson CM, Tyebkhan JM, Peliowski A, Etches PC, Cheung PY. Ototoxic drugs and sensorineural hearing loss following severe neonatal respiratory failure. Acta Paediatr 2006;95:214-23.  Back to cited text no. 89
Cone-Wesson B. Prenatal alcohol and cocaine exposure: Influences on cognition, speech, language, and hearing. J Commun Disord 2005;38:279-302.  Back to cited text no. 90
Czeizel AE, Rockenbauer M, Olsen J, Sørensen HT. A teratological study of aminoglycoside antibiotic treatment during pregnancy. Scand J Infect Dis 2000;32:309-13.  Back to cited text no. 91
Church MW, Kaltenbach JA. Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review. Alcohol Clin Exp Res 1997;21:495-512.  Back to cited text no. 92
Popova S, Lange S, Shield K, Mihic A, Chudley AE, Mukherjee RAS, et al. Comorbidity of fetal alcohol spectrum disorder: A systematic review and meta-analysis. Lancet 2016;387:978-87.  Back to cited text no. 93
Geijteman EC, Wensveen CW, Duvekot JJ, van Zuylen L. A child with severe hearing loss associated with maternal cisplatin treatment during pregnancy. Obstet Gynecol 2014;124:454-6.  Back to cited text no. 94
Foch C, Araujo M, Weckel A, Damase-Michel C, Montastruc JL, Benevent J, et al. In utero drug exposure and hearing impairment in 2-year-old children A case-control study using the EFEMERIS database. Int J Pediatr Otorhinolaryngol 2018;113:192-7.  Back to cited text no. 95
Schacht J, Talaska AE, Rybak LP. Cisplatin and aminoglycoside antibiotics: Hearing loss and its prevention. Anat Rec (Hoboken) 2012;295:1837-50.  Back to cited text no. 96
Merchant TE, Boop FA, Kun LE, Sanford RA, Butler WE, Khan A, et al. Hearing Loss and Vestibular Dysfunction among Children with Cancer after receiving Aminoglycosides. Pediatr Blood Cancer 2013;60:1772-7.  Back to cited text no. 97
Fuchs A, Zimmermann L, Bickle Graz M, Cherpillod J, Tolsa JF, Buclin T, et al. Gentamicin Exposure and Sensorineural Hearing Loss in Preterm Infants. PLoS One 2016;11:e0158806.  Back to cited text no. 98
Li Y, Womer RB, Silber JH. Predicting cisplatin ototoxicity in children: The influence of age and the cumulative dose. Eur J Cancer 2004;40:2445-51.  Back to cited text no. 99
Minasian LM, Frazier AL, Sung L, O'Mara A, Kelaghan J, Chang KW, et al. Prevention of cisplatin-induced hearing loss in children: Informing the design of future clinical trials. Cancer Med 2018;7:2951-9.  Back to cited text no. 100
Dean JB, Hayashi SS, Albert CM, King AA, Karzon R, Hayashi RJ. Hearing loss in pediatric oncology patients receiving carboplatin-containing regimens. J Pediatr Hematol Oncol 2008;30:130-4.  Back to cited text no. 101
Bertolini P, Lassalle M, Mercier G, Raquin MA, Izzi G, Corradini N, et al. Platinum Compound-Related Ototoxicity in Children: Long-Term Follow-Up Reveals Continuous Worsening of Hearing Loss. J Pediatr Hematol Oncol 2004;26:649-55.  Back to cited text no. 102
Chirtes F, Albu S. Prevention and restoration of hearing loss associated with the use of cisplatin. Biomed Res Int 2014;2014:925485.  Back to cited text no. 103
Ciorba A, Corazzi V, Negossi L, Tazzari R, Bianchini C, Aimoni C. Moderate-severe hearing loss in children: A diagnostic and rehabilitative challenge. J Int Adv Otol 2018;13:407-13.  Back to cited text no. 104
Kumar S, Sharma R, Gautam P, Taneja V. Etiological factors for pediatric sensorineural hearing loss. Indian J Otol 2012;17:162.  Back to cited text no. 105
Mehta D, Noon SE, Schwartz E, Wilkens A, Bedoukian EC, Scarano I, et al. Outcomes of evaluation and testing of 660 individuals with hearing loss in a pediatric genetics of hearing loss clinic. Am J Med Genet A 2016;170:2523-30.  Back to cited text no. 106
Patel H, Feldman M. Universal newborn HS – H. Patel. Paediatr Child Health 2011;16:301-5.  Back to cited text no. 107
Ptok M. Early detection of hearing impairment in newborns and infants. Dtsch Arztebl Int 2011;108:426-31.  Back to cited text no. 108
Johnson JL, White KR, Widen JE, Gravel JS, James M, Kennalley T, et al. A multicenter evaluation of how many infants with permanent hearing loss pass a two-stage otoacoustic emissions/automated auditory brainstem response newborn hearing screening protocol. Pediatrics 2005;116:663-72.  Back to cited text no. 109
Wolff R, Hommerich J, Riemsma R, Antes G, Lange S, Kleijnen J. Hearing screening in newborns: Systematic review of accuracy, effectiveness, and effects of interventions after screening. Arch Dis Child 2010;95:130-5.  Back to cited text no. 110
Angeli S, Lin X, Liu XZ. Genetics of hearing and deafness. Anat Rec (Hoboken) 2012;295:1812-29.  Back to cited text no. 111
Wu CC, Hung CC, Lin SY, Hsieh WS, Tsao PN, Lee CN, et al. Newborn genetic screening for hearing impairment: A preliminary study at a tertiary center. PLoS One 2011;6:1-9.   Back to cited text no. 112
Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss. Genetic Evaluation of Congenital Hearing Loss Expert Panel. ACMG statement. Genet Med 2002;4:162-71.  Back to cited text no. 113
Ali Z, Babar ME, Ahmad J, Shah SA. The study of gene GJB2/DFNB1 causing deafness in humans by linkage analysis from district Peshawar. Indian J Hum Genet 2012;18:217-21.  Back to cited text no. 114
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Rao A, Schnooveld C, Schimmenti LA, Vestal L, Ferrello M, Ward J, et al. Genetic Testing in Childhood Hearing Loss: Review and Case Studies. Audiol Online 2011:1-11.  Back to cited text no. 115
Shearer AE, Smith RJ. Genetics: Advances in genetic testing for deafness. Curr Opin Pediatr 2012;24:679-86.  Back to cited text no. 116
Licameli G, Kenna MA. Is computed tomography (CT) or magnetic resonance imaging (MRI) more useful in the evaluation of pediatric sensorineural hearing loss? Laryngoscope 2010;120:2358-9.  Back to cited text no. 117
Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists. Am J Neuroradiol 2012;33:211-7.  Back to cited text no. 118
Kachniarz B, Chen JX, Gilani S, Shin JJ. Diagnostic yield of MRI for pediatric hearing loss: A systematic review. Otolaryngol Head Neck Surg 2015;152:5-22.  Back to cited text no. 119
Kapul AA, Zubova EI, Torgaev SN, Drobchik VV. Pure-tone audiometer. J Phys Conf Ser 2017;881:012010.  Back to cited text no. 120
Coates H, Kong K, Mackendrick A, Lannigan F, Vijayasakaran S, Bumbak P. Aboriginal Ear Health Manual. 1st ed. Perth, WA: Abbott & Co Printers; 2012.  Back to cited text no. 121
Lotti M, Bleecker M, Sliwinska-Kowalska M. Pure tone audiometry. Occup Neurol 2015;131:341.  Back to cited text no. 122
Eggermont JJ. Types of Hearing Loss. Hearing loss; Causes, Prevention, Treatment. 1st ed. London: Academic Press.; 2017. p. 129-73.   Back to cited text no. 123
Davies RA. Audiometry and other hearing tests. Handbook of clinical neurology. Vol. 137. Elsevier, 2016. p. 157-76.  Back to cited text no. 124
Tanaka C, Taniguchi LD, Lew HL. Diagnosis and Rehabilitation of Hearing Disorders in the Elderly. Geriatr Rehabil. 1st editio. St. Louis: Missouri: Elsevier Inc.; 2018. p. 145-59.   Back to cited text no. 125
Wyatt T. Assessment of multicultural and international clients with communication disorders. 4th editio. Commun. Disord. Multicult. Int. Popul. St. Louis: Missouri: Elsevier Inc.; 2012.   Back to cited text no. 126
Schmida MJ, Peterson HJ, Tharpe AM. Visual reinforcement audiometry using digital video disc and conventional reinforcers. Am J Audiol 2003;12:35-40.  Back to cited text no. 127
Widen JE, O'Grady GM. Using visual reinforcement audiometry in the assessment of hearing in infants. Hear J 2002;55:28-36.  Back to cited text no. 128
Lasak JM, Allen P, McVay T, Lewis D. Hearing loss: Diagnosis and management. Prim Care 2014;41:19-31.  Back to cited text no. 129
Övet G, Alataş N, Güzelkara F. Sudden pediatric hearing loss: Comparing the results of combined treatment (intratympanic dexamethasone and systemic steroids) with systemic steroid treatment alone. Otol Neurotol 2016;37:742-7.  Back to cited text no. 130
Nieto H, Dearden J, Dale S, Doshi J. Paediatric hearing loss. BMJ 2017;356:j803.  Back to cited text no. 131
Kulkarni K, Hartley DE. Recent advances in hearing restoration. J R Soc Med 2008;101:116-24.  Back to cited text no. 132
Saliba I, Woods O, Caron C. BAHA results in children at one year follow-up: A prospective longitudinal study. Int J Pediatr Otorhinolaryngol 2010;74:1058-62.  Back to cited text no. 133
Egilmez OK, Kalcioglu MT. Cochlear implant: indications, contraindications and complications. Scr Sci Medica 2015;47:9.  Back to cited text no. 134
Lima A De, Moret M, Tabanez L. The implications of the cochlear implant for development of language skills: A literature review. Rev CEFAC 2015;17:1643-55.  Back to cited text no. 135


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